introduction · ©1997 erlanger health system tennessee craniofacial center 1(800) 418-3223...

©1997 Erlanger Health System Tennessee Craniofacial Center 1(800) 418-3223

INTRODUCTION

THE TENNESSEE CRANIOFACIAL CENTER, part of the ErlangerHealth System, is located in Chattanooga, Tennessee. TheCenter specializes in the evaluation and treatment of patientsof all ages with craniofacial deformities. Its services cover abroad range of reconstructive operations for the treatment ofdeformities of the face and skull resulting from birth defects,tumors and trauma.

Dr. Larry A. Sargent, M.D. is the Medical Director of theTennessee Craniofacial Center. He is a plastic and recon-structive surgeon specializing in craniofacial surgery andmaxillofacial trauma. Before joining The Plastic SurgeryGroup, P.C., of Chattanooga, Dr. Sargent was Director ofCraniofacial Surgery at Walter Reed Army Medical Center inWashington, D.C. He is a graduate of the Georgia Institute ofTechnology and Emory University School of Medicine. Hecompleted his general surgery and plastic surgery training atThe John Hopkins Hospital in Baltimore, Maryland andstudied Craniofacial Surgery with Dr. Paul Tessier in Paris,France. Dr. Sargent is Professor and Chairman of theDepartment of Plastic Surgery at the University of TennesseeCollege of Medicine in Chattanooga, a Fellow of theAmerican College of Surgeons, a Fellow of the AmericanAcademy of Pediatrics, and board certified in plastic surgery.He is an active member of the American Association ofPlastic Surgeons, the American Society of Plastic andReconstructive Surgeons, the American Society ofMaxillofacial Surgeons, the American Association ofPediatric Plastic Surgeons, the American Cleft Palate-Craniofacial Association, the Plastic Surgery ResearchCouncil, and numerous regional societies.

The primary purpose of this book is to inform health careprofessionals, as well as parents, of the treatment that isavailable at the Tennessee Craniofacial Center for varioustypes of craniofacial deformities. All patient examples shownin this book are the reconstructive work of Dr. Larry Sargent.


PREFACE

The Regional Craniofacial Center offers new hope andpromise to many patients with facial deformities. The effec-tive Craniofacial Center is one that has a dedicated, orga-nized team of professionals of which each member has agenuine interest in evaluating and treating these types ofdeformities. The team approach remains our standard carefor evaluation; however, the ultimate responsibility is withthe Craniofacial Surgeon for choice of operation and the finalsuccess or failure dependent on his skill, experience andjudgement. I feel, to continue to maintain this level of exper-tise, the Craniofacial Surgeon must devote the majority of hispractice to the treatment of these types of problems.Adequate volumes of patients are necessary to maintain thisexpertise, which enhances safety and insures that optimumresults are obtained. The specialized care and support facili-ties of a major medical center and children’s hospital are alsomandatory. I feel these are the criteria by which a centershould be judged.

This book is organized like a monograph, using mypatients to illustrate current surgical techniques that I use ona regular basis to treat various craniofacial deformities. Itstrongly reflects the comprehensive, coordinated treatmentplan used at our center. In putting together this material, Ihad several goals in mind. First was to educate health pro-fessionals about the new and rapidly growing field of

Dr. Larry A. Sargent


Craniofacial Surgery. Second, was to provide a reference forpatients and their families concerning treatments that areavailable and the type of results that might be expected. Andfinally, to reflect my own experience and philosophy as relat-ed to the treatment of these complex problems.

Providing informative material on craniofacial deformi-ties, ultimately results in better patient care. As the primarycare physician and the public become aware of what a centersuch as ours has to offer, expectations will and should con-tinue to rise. It is not enough to correct a deformity; the ulti-mate goal should be to create an attractive face. This requiresa thorough appreciation of facial aesthetics and the constantdesire to strive toward perfection.

Craniofacial Surgery is a unique field in Plastic Surgerythat at its best blends technical skill with artistic creativity.This type of facial reconstruction is often very hard and tir-ing work involving long operations, demanding a certaindedication from the surgeon. However, the treatment ofthese complex problems is both challenging and rewarding.Correcting a facial deformity can very often change a per-son’s entire outlook on life, giving them a new self-esteemand a second chance. As this new field of surgery grows withthe advances in technology and surgical techniques, thereshould always be that unfatigable desire that pushes one tocontinually strive for the best results possible for eachpatient. I feel this is and will continue to be my philosophy.

Larry A. Sargent, M.D., F.A.C.S., F.A.A.P.Medical Director, Tennessee Craniofacial CenterProfessor and ChairmanDepartment of Plastic SurgeryUniversity of TennesseeChattanooga, Tennessee


THE CRANIOFACIALDEFORMITY

Diagnosis: Craniofacial deformi-ties, or alterations in the natural formof the face or skull, can be congenital or acquired. For thosepatients born with craniofacialanomalies, the obstetrician or pedia-trician is the initial point of contactfor appropriate medical treatment.Referrals should be made to a cranio-facial center as soon as it is accept-able for the child to be evaluated.Accurate diagnosis at an early agenot only avoids unnecessary emo-tional distress for the parents andfamily, but it also minimizes poten-tial future problems associated withthe deformities by early correction.For those patients with acquireddeformities as the result of trauma ortumor resection, referral to a cranio-facial center may be desirable to helprestore facial function and appear-ance.

Etiology: While the pattern ofembryonic craniofacial developmenthas been well defined through exten-sive research, very little is knownabout the etiology of many craniofa-cial anomalies. Some are known tobe primarily genetic in nature, whileothers are thought to be caused byenvironmental factors. A combina-tion of both environment and genet-ics may play a role in the etiology;however, most of the time the causeis unknown.

Craniofacial Team: The care of craniofacial patients requires theexpertise of super-specialized profes-sionals from many health care fields.

New advanced computer systems enable the surgeon to performcomplex photographic and radiographic analysis that aid intreatment planning.

Computer images of patient with orbital hypertelorism.


Multidisciplinary teams have been estab-lished at regional centers to provide thecomprehensive care necessary to ade-quately evaluate and treat craniofacialpatients and their families. No singlephysician can possess the expertise toevaluate and treat all the abnormalities ofthese patients.

The benefits of the team approach arenumerous. Members of the craniofacialteam work together to ensure that thepatient is evaluated and treated in a coor-dinated manner and that all of his needs,both physical and psychosocial, are met.The team combines the expertise of eachspecialist to provide a level of compre-hensive care that cannot be provided by asingle physician, no matter how rep-utable.

These multidisciplinary craniofacialteams are found at a few major medicalcenters across the U.S. where theresources are available to provide thesafest and most advanced treatment forpatients suffering from facial anomalies.Regionalization also ensures that eachteam has a large enough patient load tomaintain the necessary expertise for prop-er treatment. The more procedures theyperform together, the better the teambecomes. Consequently, operative time isdecreased, complications are minimized,and results are improved. Craniofacialprocedures performed on an irregular oroccasional basis invite disaster and arenot in the best interest of the patient.

The craniofacial team is directed bythe craniofacial surgeon, a plastic surgeonwho has received additional trainingextensively in craniofacial techniques andwhose practice is predominantly dedicat-ed to the treatment of facial anomalies.

At the Tennessee Craniofacial Center,we adhere to Dr. Tessier’s principles that,“Craniofacial surgery should be per-

formed only if it is the main interest of that surgeon,and he has the support facilities of a major medicalcenter.”

Dr. Sargent is the medical director of the centerand the leader of the team. Other disciplines repre-sented on the team include:

• Neurosurgery• Ophthalmology• Pedodontics/Orthodontics• Otolaryngology• Anesthesiology• Speech Pathology• Pediatrics• Audiology• Psychology• Social Service• Genetics• Nursing• Prosthodontics• Clinical Coordinator


Recent Advancements: A number ofadvances have been made in surgical tech-nique and technology as applied to craniofacialsurgery. Calvarial bone grafts for the most parthave replaced rib and hip grafts. These outertable split grafts are available in an assortmentof sizes and shapes with less painful donorsites and less resorption compared to rib or hipbone.

Tremendous radiological advances havebeen made in the past ten years that haveimproved preoperative analysis of craniofacialdeformities. The use of two- and three-dimen-sional CT scans has drastically enhanced ourability to analyze these complex deformities.Computer analysis of photographs and radi-ographs is also available and can provide fur-ther information for preoperative planning.

Another major advancement has been theapplication of rigid skeletal fixation to cranio-facial surgery. The new techniques of rigidskeletal fixation combined with wide exposurehave allowed the craniofacial surgeon to obtainmuch better stability and eliminate intermaxil-lary fixation in most cases. This techniqueoffers significant advantages, particularly inchildren. It has improved our overall quality ofresults as well as decreasing morbidity.

Follow-Up Care: Treatment of craniofacialproblems does not end with surgical restora-tion, but continues for many years. This follow-up should be conducted by the cranio-facial team in order to maintain a continuity ofcare that assures the patient the best long termoutcome. As a child grows and develops,asymmetries may result if areas of the face failto develop equally; therefore, a child’s growthand development must be routinely followed.Sometimes it may be necessary to repair theseasymmetries surgically. Often, major craniofa-cial deformities require multiple, staged proce-dures performed at different ages. Oncetreatment is initiated, it is important that fol-low-up care continues.

Each patient referred to the TennesseeCraniofacial Center undergoes an evaluationby the principle members of the team, withadditional team members called in dependingupon the individual needs of that patient. Agroup conference is held following these evalu-ations to discuss each patient. The goal of theteam is to diagnose the physical and psychoso-cial problems and formulate a coordinated,comprehensive treatment plan. This is fol-lowed by execution of the treatment plan at theappropriate time, longitudinal follow-up, andcollection of data on the team’s activities andresults.

Surgical Plan Established: WhenCraniofacial Surgery is recommended as treat-ment for a specific anomaly, there are two dis-tinct goals of this surgery. First, is the attempt torestore the patient to as near normal function aspossible and to prevent future dysfunction.Secondly, surgery may be necessary to correctstructural disfigurement in order to achieve opti-mal appearance. A patient suffering from a facialdeformity may experience problems in dealing

with his disfigurement emotionally or socially.Often, improvements in appearance followingcraniofacial surgery can lead to increases in self-esteem, self-confidence and social acceptance.The psychological benefit of craniofacial surgeryis an extremely important goal of the surgery.

Computer simulated images. Top left shows chinadvancement with rhinoplasty.


ENOPHTHALMOSEnophthalmos can be defined

as the relative recession (back-ward or downward displacement)of the globe into the bony orbit.The three basic structures thatdetermine globe position are thebony orbits, the ligament systemand the orbital fat. Displacementof the orbital walls with enlarge-ment of the bony orbit may be themajor components in the produc-tion of enophthalmos in orbitalfractures. Post-traumatic enoph-thalmos is frequently seen and isthe result of disruption of thebony orbit and ligament systemwith displacement of the orbitalsoft tissue. This presents clinicallyas a sunken appearance to the eyewith pseudoptosis and deepeningof the supratarsal fold. Treatmentinvolves reconstruction of thebony orbit with restoration ofbony orbital volume and reposi-tioning of the globe. The use ofcraniofacial techniques allows thisto be accomplished with minimalcomplications.

This patient has traumatic enophthalmos of the left orbit.Note sunken-in appearance of the left eye due to unre-paired orbital fracture.

This patient has severe displace-ment of the right eye caused bytumor.

Postoperative result after tumorresection and orbital reconstruc-tion with repositioning of the right eye.

Postoperative result after orbital reconstruction and repo-sitioning of eye.

Enophthalmos

Enophthalmos

Clefts of the Lip and Palate


Each year approximately 227,500 or 7percent of births in the United States areaffected by birth defects of the head andface. The most common of these areclefts of the lip and palate which occuronce in every 700 births. Clefts occur ininfants of all races with a 2:1 male tofemale ratio. The incidence of clefts ishighest in the Asian population andlowest in African Americans. Of all oro-facial clefts, 21 percent present as cleftlip only (unilateral and bilateral), 46 per-cent present as cleft lip and palate, whilethe remaining 33 percent have cleftpalate alone.

What is a cleft? A cleft is a divisionor separation of parts of the lip or roof ofthe mouth that is formed during theearly months of development of theunborn child. All of the parts of the lipor roof of the mouth are present; theysimply failed to fuse in a normal way.Surgical intervention is necessary toalign the parts and join them. Often thebones of upper jaw (maxilla) and/or theupper gum are affected. A cleft lip can beincomplete with a variable degree ofnotching of the lip, or complete, extend-ing through the lip and into the nose.

Preoperative Postoperative

CLEFTS OF THE LIP AND PALATE

Variations in clefts of the palate.

A). Cleft of soft palate only. B). Incomplete cleftof soft and hard palate. C). Complete unilateralcleft palate. D). Complete bilateral cleft palate.


FEEDING AN INFANT WITH A CLEFT

Feeding an infant is important not only inproviding nourishment, it also provides anintimacy and closeness for both the parentand the child. Infants with a cleft of the lip orof the soft palate seldom have problems withfeeding either by bottle or breast.

In babies with clefts of the hard palate, theopening in the roof of the mouth often causesdifficulty in creating adequate pressure onthe nipple, thus creating an inability to suckwell enough to get adequate nourishment.Feeding the infant takes patience and prac-tice. At our center we recommend the use of asoft squeezable plastic bottle like MeadJohnson with an orthodontic nipple such asNuk. You can increase the flow by gentlysqueezing or putting pressure on the bottle. Itis important to feed the infant before he/shebecomes too hungry. Position the infant in anupright position with the head tilted backslightly. This position allows the milk to flowdown into the throat and less into the nose.Infants with clefts do swallow more air andneed to be burped more frequently. At first, itmay take extra time, but this will steadilydecrease . Feeding time of the newborn variesfrom 20-30 minutes. When feeding takeslonger than 45 minutes, the infant may beburning up calories necessary to gain weight.If this occurs the feeding consultant should becontacted to help with the feeding technique.

Breast feeding the newborn with a cleft ofthe hard palate is often unsuccessful.Generally the infant cannot produce enoughnegative pressure to obtain ample breast milkto provide adequate nourishment. Using abreast pump to extract the milk and feedingthe infant breast milk from a squeezable bottle is recommended.

Clefts of the palate can vary in severity.Some may involve just the uvula and the softpalate. These are incomplete clefts of thepalate. Others extend the length of the palateand are complete clefts. They may involveone side of the palate (unilateral) or bothsides (bilateral).

Etiology: The exact cause of lip andpalatal clefting is not known, but mostexperts feel that it is due to both genetic andenvironmental factors. Clefts are associatedwith abnormalities in the genes which maybe a result of inheritance or from a sponta-neous mutation during fetal development.We recommend genetics counseling to dis-cuss causes of the cleft and the recurrencerisk factors.

Team Assessment: Children born withclefts should be carefully assessed by thecraniofacial team in order to detect potential-ly serious abnormalities that can be associat-ed with clefting. There are over 150syndromes that include cleft lip or palate intheir differential diagnosis. Generally, cleft-ing is the only congenital abnormality thatthe child has, but nearly 15 percent of all cleftlip or palate patients present clinically withmultiple problems.

The team concept allows a systematic,comprehensive treatment plan to be devel-oped and allows the team members to worktogether to identify problems before theybecome significant. The most common spe-cialities involved in the care of a child with acleft are: plastic surgery, otolaryngology, den-tistry, audiology, speech pathology, geneticsand pediatrics. Once a complete assessmentof the child with a cleft has been performed,a plan for treatment can be outlined.


SURGERYCleft Lip Repair - The objec-

tive in repairing the lip is toclose the cleft to create a pleas-ing face that will develop nor-mally with minimal scarring.Closure of the lip is performedby the plastic surgeon when thebaby is approximately 3 monthsof age and weighs at least 10pounds. When there is involve-ment of the alveolus and palate,an orthodontic appliance maybe placed in the maxillary seg-ments as the first procedure.This is performed by the teamdentist as an outpatient surgicalprocedure. The appliance isused to align the alveolus so thatit can be repaired (gingivope-riosteoplasty) at the time of thelip repair or lip adhesion. Thisimproves nasal support on thecleft side and creates a tunnelthat should develop bone, clos-ing the cleft. If the alveolus is notclosed in infancy, then the alveo-lar ridges will be orthodontical-ly aligned and a bone graftperformed to stabilize the maxil-la (5-10 years of age). Correctionof the nasal deformity is usuallyperformed at the time of liprepair. Additional proceduresmay be necessary to enhance theappearance of either the lip ornose.

Unilateral Cleft Lip with Custom Latham

Photos to the right:

A unilateral cleft lip is shown. A customfitted Latham appliance has been fabricatedand placed to rotate alveolar segmentstogether. Postoperative result is shown afterrepair of alveolar cleft and lip.


UNILATERALCLEFT LIP REPAIR

A unilateral cleft lip results fromfailure of the union of the maxillaryand median nasal processes, thuscreating a split or cleft in the lip oneither the left or right side. It may bejust a notching of the lip or extendcompletely through the lip into thenose and palate. A number of proce-dures have been described to repairthe unilateral cleft lip. The proce-dure used at our Center is theMillard rotation advancement tech-nique. The procedure is designed toreconstruct the lip, muscle, oralmucosa, and to reposition the nose.It is performed under general anes-thesia with surgery lasting 2-3 hoursand a hospital stay of 2-4 days.Special considerations are necessaryfor feeding and positioning theinfant postoperatively. The baby’selbows are restrained from bendingto prevent him/her from disruptingthe nose or lip. Positioning the childin an infant seat keeps him/her fromrolling over and injuring the lip ornose. Pacifiers and nipples are notallowed. The baby is fed with a spe-cial syringe feeder with a soft tube. Ittakes approximately 3 weeks for thewound to gain enough strength todiscontinue the above precautions.The lip scar is initially red andswollen, but it begins to mature andimprove in appearance in six-twelvemonths.

Unilateral Cleft Lip




Photos of infants who underwent repair ofunilateral cleft lip with rotation advance-ment technique.



BILATERALCLEFT LIP

The bilateral cleft lip involvesseparation of the lip alongphiltral lines, isolating the centralsegment (prolabium). Fifteen per-cent of children born with cleftlips have bilateral clefts. Theassociated nasal deformity is usu-ally more severe than the unilat-eral cleft due to a very shortcolumella and flaring of both nos-trils. Surgical correction of thebilateral cleft lip is usually per-formed in one procedure at threemonths of age; however, the pro-cedure may be staged, closingone cleft at a time. Rotation of thenostrils to a more normal positionis performed in the first proce-dure. A second procedure is per-formed by 2-3 years of age tolengthen the columella. Patientswith complete bilateral cleft lipsfrequently require additional pro-cedures to enhance the appear-ance of the lip and nose.Performed under general anes-thesia, the operation generallyrequires 2-3 hours. A hospitalstay of 2-4 days should be expect-ed. Feeding, positioning andelbow restraints are the same asthose for repair of the unilateralcleft lip.

Bilateral Cleft Lip





Photos of infants who underwent onestage repair of bilateral cleft lip.


CLEFT PALATE REPAIRThe objective of cleft palate

surgery is to close the palate torestore normal function to eatingand drinking and to enhance thedevelopment of normal speech.

Clefts of the palate can occur asisolated deformities or in combina-tion with a cleft of the lip. Cleftpalates result from failure of fusionof the embryonic facial processesresulting in a fissure through thepalate. This may be complete(extending through the hard andsoft palates) or any degree ofincomplete (partial cleft). Thepalate forms the roof of the oralcavity and the floor of the nose;thus, a cleft causes a free communi-cation between these two cavities.As a result, treatment of palatalclefts is complex because of poten-tial problems with feeding, speech,middle ear infections, occlusionand jaw alignment.

Surgical treatment of the cleftpalate is best accomplished in onesurgical procedure before the childreaches 12-14 months of age. Thecleft palate is surgically closed byelevating two muscoperiostealflaps. The levator muscles are ele-vated, redirected and repaired; anda three layer closure of nasalmucosa, muscle and oral mucosaaccomplished. Surgery under gen-eral anesthesia usually lasts about2 hours. Special precautions asthose after the repair of the cleft lipare necessary for 2-3 weeks. Weprefer that the child be weanedfrom the bottle and pacifier prior tothe palatal repair. No hard orcrunchy foods are allowed for 3weeks post operatively.

Cleft Palate Repair:

Closure of cleft palate with pushback palatoplasty. A). Twomucoperiosteal flaps are outlined. B). Flaps are elevated off thehard palate. C,D). The abnormal levator muscle insertion to thehard palate is identified and cut free. E). The nasal lining isclosed as a separate layer and the levator muscle reapproximated.F). The palatal mucoperiosteal flaps are closed in a V-Y fashion.


PHARYNGEAL FLAPApproximately 70-80 percent of

all cleft palate patients will developvelopharyngeal competence afterpalate closure and thus the potentialfor normal speech. The remaining20-30 percent will require speechtherapy and/or an additional surgi-cal procedure called a pharyngealflap. To correct persistent hypernasalspeech, this procedure involves rais-ing a flap of tissue from the posteriorpharynx and inserting it into the softpalate. This flap is indicated whenthe repaired palate is too short or themuscles do not function properly,causing a persistent hypernasalspeech. The procedure is performedusually after the age of 4-5 whenspeech and velopharyngeal compe-tence can be thoroughly assessedand before the child begins school.

LATE CLEFT TREATMENT

The Craniofacial Center can alsohelp those individuals that havegrown up without access to a com-prehensive, coordinated teamapproach. For adults with speechproblems, the previously mentionedpharyngeal flap, combined with anintensive regimen of speech therapy,can produce significant improve-ments. Orthognatic surgery is avail-able to patients with deformities ofthe jaws to improve their appearanceas well as to correct dental occlusion.For soft tissue revision of a severelytightened or notched upper lip, anAbbe flap is the surgical option. Thisprocedure is usually indicated inbilateral cleft patients who have ashort or deficient columella and atightened upper lip. This operation

Pharyngeal Flap:

The pharyngeal flap procedure for hypernasal speech. A superior-ly based flap of tissue is raised from the posterior pharynx andsutured to the soft palate thereby decreasing the amount of airthrough the nose. Lateral ports or holes are left so that the nosewill not be obstructed.

Inadequate Closure

Adequate Closure

Corrected Closure

patients will have velopharyngeal incompe-tence or hypernasal speech after surgery, andmay require a pharyngeal flap to correct itaround the age of 4-5 years.

DENTALClefts of the palate generally have an effect

on dental development. In the area of the cleft,teeth often erupt in a crooked position withextra teeth or missing teeth being common inthe cleft area. Radiographs are often taken todetermine the exact position of the teeth.Dental problems have an effect on speech,chewing, appearance and frequently requireorthodontic treatment. Early orthodontic inter-vention may require a palatal expansion devicewith further alignment of the dental arches.Later treatment after the primary teeth haveerupted can begin at 10-12 years of age.Orthognathic surgery may be indicated if amalocclusion develops due to abnormalgrowth of the maxilla.

PIERRE ROBIN SYNDROME

This syndrome was described in 1923 byPierre Robin in which he described airwayobstruction associated with glossoptosis andhypoplasia of the mandible. Today this syn-drome is characterized by retrognathia ormicrognathia, glossoptosis, and airway obstruc-tion. An incomplete cleft of the palate is associ-ated with the syndrome in approximately 50%of these patients.

In patients with micrognathia (small jaw) orretrognathia, the chin is posteriorly displacedcausing the tongue to fall backward toward theposterior pharyngeal wall. This results inobstruction of the airway on inspiration. Cryingor straining by these children can often keepthe airway open. However, when the childrelaxes or falls asleep, airway obstructionoccurs. Due to these respiratory problems, feed-ing may become very difficult. This can lead to


can add fullness to the upper lip as well aslengthen the columella. A number of addi-tional surgical therapies, similar to the onesdescribed, are available to patients whodesire further improvements.

HEARINGChildren with cleft palate have a higher

incidence of hearing problems. TheEustachian tube connects the middle earspace to the back of the throat. It normallyopens and shuts to relieve pressure thatbuilds up behind the ear drum. If theEustachian tube does not open, then the pres-sure increases until mucus or “fluid” accu-mulates behind the eardrum. The musclesresponsible for opening the Eustachian tubedo not function as well in children with cleftpalates resulting in more frequent problemswith fluid, otitis media and ear infectionswhich can be very painful. Because of thisproblem, it is important to have the infant’shearing tested during the first few months. Ifhearing is impaired by fluid buildup orunequal pressure, it may be necessary for theotolaryngologist to place pressure equalizing(PE) tubes. Tubes are often placed at the timeof the lip or palate surgery. It is crucial thatchildren with cleft palates have regular hear-ing tests to monitor middle ear problems thatcould alter the development of normal hear-ing as well as speech. As the child grows, thefrequency of ear infections and fluid in theears seem to decrease.

SPEECHSpeech development in children with cleft

lip only should be normal. The unrepairedcleft palate causes speech to sound hyper-nasal because air passes through the nosewhile talking. Most speech sounds requirethe nose to be closed off from the mouth. Cleftpalate surgery usually remedies the problem,but speech therapy is still recommended.Approximately 20-30 percent of cleft palate


a sequence of events: glossoptosis,airway obstruction, crying or strain-ing with increased energy expendi-ture and decreased oral intake. Thisvicious cycle of events if untreatedcan led to exhaustion, cardiac fail-ure, and ultimately death.

Treatment of this syndrome canbe divided into conservative therapyversus surgical intervention. Themajority of these patients can bemanaged by placing the infant in theprone position until adequategrowth of the jaw occurs. This caus-es the jaw and the tongue to fall for-ward opening the airway. If this typeof treatment fails the infant shouldthen be considered for a tongue-lipadhesion (a procedure to pull thetongue forward) or a tracheostomy.

In children with severe underde-velopment of the lower jaw, a newtechnique called mandibular boneexpansion is now available. Thistechnique also called distractionosteogenesis involves placement ofan expansion device that is turneddaily to slowly lengthen the jaw. Anexternal incision is required to makea surgical cut through the jaw bonewith placement of pins that aresecured to the expansion device.Once the amount of expansion of thebone has been obtained (4-5 weeks)the device is then kept in place untilthe bone gap heals with new boneformation (8 weeks). This techniquecan be performed at a very early agewhich is a significant advantageover the traditional technique oflower jaw lengthening.

Results after removal of lengthening devices. Patient now readyfor removal/closure of tracheostomy.

Bilateral mandibular lengthening devices surgically placed withlengthening in progress.

Two year old with Pierre Robin and severe airway obstruc-tion requiring tracheostomy.

Pierre Robin

Clefts of the Lip and Palate


Each year approximately 227,500 or 7percent of births in the United States areaffected by birth defects of the head andface. The most common of these areclefts of the lip and palate which occuronce in every 700 births. Clefts occur ininfants of all races with a 2:1 male tofemale ratio. The incidence of clefts ishighest in the Asian population andlowest in African Americans. Of all oro-facial clefts, 21 percent present as cleftlip only (unilateral and bilateral), 46 per-cent present as cleft lip and palate, whilethe remaining 33 percent have cleftpalate alone.

What is a cleft? A cleft is a divisionor separation of parts of the lip or roof ofthe mouth that is formed during theearly months of development of theunborn child. All of the parts of the lipor roof of the mouth are present; theysimply failed to fuse in a normal way.Surgical intervention is necessary toalign the parts and join them. Often thebones of upper jaw (maxilla) and/or theupper gum are affected. A cleft lip can beincomplete with a variable degree ofnotching of the lip, or complete, extend-ing through the lip and into the nose.


CLEFTS OF THE LIP AND PALATE

Variations in clefts of the palate.

A). Cleft of soft palate only. B). Incomplete cleftof soft and hard palate. C). Complete unilateralcleft palate. D). Complete bilateral cleft palate.


FEEDING AN INFANT WITH A CLEFT

Feeding an infant is important not only inproviding nourishment, it also provides anintimacy and closeness for both the parentand the child. Infants with a cleft of the lip orof the soft palate seldom have problems withfeeding either by bottle or breast.

In babies with clefts of the hard palate, theopening in the roof of the mouth often causesdifficulty in creating adequate pressure onthe nipple, thus creating an inability to suckwell enough to get adequate nourishment.Feeding the infant takes patience and prac-tice. At our center we recommend the use of asoft squeezable plastic bottle like MeadJohnson with an orthodontic nipple such asNuk. You can increase the flow by gentlysqueezing or putting pressure on the bottle. Itis important to feed the infant before he/shebecomes too hungry. Position the infant in anupright position with the head tilted backslightly. This position allows the milk to flowdown into the throat and less into the nose.Infants with clefts do swallow more air andneed to be burped more frequently. At first, itmay take extra time, but this will steadilydecrease . Feeding time of the newborn variesfrom 20-30 minutes. When feeding takeslonger than 45 minutes, the infant may beburning up calories necessary to gain weight.If this occurs the feeding consultant should becontacted to help with the feeding technique.

Breast feeding the newborn with a cleft ofthe hard palate is often unsuccessful.Generally the infant cannot produce enoughnegative pressure to obtain ample breast milkto provide adequate nourishment. Using abreast pump to extract the milk and feedingthe infant breast milk from a squeezable bottle is recommended.

Clefts of the palate can vary in severity.Some may involve just the uvula and the softpalate. These are incomplete clefts of thepalate. Others extend the length of the palateand are complete clefts. They may involveone side of the palate (unilateral) or bothsides (bilateral).

Etiology: The exact cause of lip andpalatal clefting is not known, but mostexperts feel that it is due to both genetic andenvironmental factors. Clefts are associatedwith abnormalities in the genes which maybe a result of inheritance or from a sponta-neous mutation during fetal development.We recommend genetics counseling to dis-cuss causes of the cleft and the recurrencerisk factors.

Team Assessment: Children born withclefts should be carefully assessed by thecraniofacial team in order to detect potential-ly serious abnormalities that can be associat-ed with clefting. There are over 150syndromes that include cleft lip or palate intheir differential diagnosis. Generally, cleft-ing is the only congenital abnormality thatthe child has, but nearly 15 percent of all cleftlip or palate patients present clinically withmultiple problems.

The team concept allows a systematic,comprehensive treatment plan to be devel-oped and allows the team members to worktogether to identify problems before theybecome significant. The most common spe-cialities involved in the care of a child with acleft are: plastic surgery, otolaryngology, den-tistry, audiology, speech pathology, geneticsand pediatrics. Once a complete assessmentof the child with a cleft has been performed,a plan for treatment can be outlined.


SURGERYCleft Lip Repair - The objec-

tive in repairing the lip is toclose the cleft to create a pleas-ing face that will develop nor-mally with minimal scarring.Closure of the lip is performedby the plastic surgeon when thebaby is approximately 3 monthsof age and weighs at least 10pounds. When there is involve-ment of the alveolus and palate,an orthodontic appliance maybe placed in the maxillary seg-ments as the first procedure.This is performed by the teamdentist as an outpatient surgicalprocedure. The appliance isused to align the alveolus so thatit can be repaired (gingivope-riosteoplasty) at the time of thelip repair or lip adhesion. Thisimproves nasal support on thecleft side and creates a tunnelthat should develop bone, clos-ing the cleft. If the alveolus is notclosed in infancy, then the alveo-lar ridges will be orthodontical-ly aligned and a bone graftperformed to stabilize the maxil-la (5-10 years of age). Correctionof the nasal deformity is usuallyperformed at the time of liprepair. Additional proceduresmay be necessary to enhance theappearance of either the lip ornose.

Unilateral Cleft Lip with Custom Latham


A unilateral cleft lip is shown. A customfitted Latham appliance has been fabricatedand placed to rotate alveolar segmentstogether. Postoperative result is shown afterrepair of alveolar cleft and lip.


UNILATERALCLEFT LIP REPAIR

A unilateral cleft lip results fromfailure of the union of the maxillaryand median nasal processes, thuscreating a split or cleft in the lip oneither the left or right side. It may bejust a notching of the lip or extendcompletely through the lip into thenose and palate. A number of proce-dures have been described to repairthe unilateral cleft lip. The proce-dure used at our Center is theMillard rotation advancement tech-nique. The procedure is designed toreconstruct the lip, muscle, oralmucosa, and to reposition the nose.It is performed under general anes-thesia with surgery lasting 2-3 hoursand a hospital stay of 2-4 days.Special considerations are necessaryfor feeding and positioning theinfant postoperatively. The baby’selbows are restrained from bendingto prevent him/her from disruptingthe nose or lip. Positioning the childin an infant seat keeps him/her fromrolling over and injuring the lip ornose. Pacifiers and nipples are notallowed. The baby is fed with a spe-cial syringe feeder with a soft tube. Ittakes approximately 3 weeks for thewound to gain enough strength todiscontinue the above precautions.The lip scar is initially red andswollen, but it begins to mature andimprove in appearance in six-twelvemonths.

Unilateral Cleft Lip




Photos of infants who underwent repair ofunilateral cleft lip with rotation advance-ment technique.



BILATERALCLEFT LIP

The bilateral cleft lip involvesseparation of the lip alongphiltral lines, isolating the centralsegment (prolabium). Fifteen per-cent of children born with cleftlips have bilateral clefts. Theassociated nasal deformity is usu-ally more severe than the unilat-eral cleft due to a very shortcolumella and flaring of both nos-trils. Surgical correction of thebilateral cleft lip is usually per-formed in one procedure at threemonths of age; however, the pro-cedure may be staged, closingone cleft at a time. Rotation of thenostrils to a more normal positionis performed in the first proce-dure. A second procedure is per-formed by 2-3 years of age tolengthen the columella. Patientswith complete bilateral cleft lipsfrequently require additional pro-cedures to enhance the appear-ance of the lip and nose.Performed under general anes-thesia, the operation generallyrequires 2-3 hours. A hospitalstay of 2-4 days should be expect-ed. Feeding, positioning andelbow restraints are the same asthose for repair of the unilateralcleft lip.

Bilateral Cleft Lip





Photos of infants who underwent onestage repair of bilateral cleft lip.


CLEFT PALATE REPAIRThe objective of cleft palate

surgery is to close the palate torestore normal function to eatingand drinking and to enhance thedevelopment of normal speech.

Clefts of the palate can occur asisolated deformities or in combina-tion with a cleft of the lip. Cleftpalates result from failure of fusionof the embryonic facial processesresulting in a fissure through thepalate. This may be complete(extending through the hard andsoft palates) or any degree ofincomplete (partial cleft). Thepalate forms the roof of the oralcavity and the floor of the nose;thus, a cleft causes a free communi-cation between these two cavities.As a result, treatment of palatalclefts is complex because of poten-tial problems with feeding, speech,middle ear infections, occlusionand jaw alignment.

Surgical treatment of the cleftpalate is best accomplished in onesurgical procedure before the childreaches 12-14 months of age. Thecleft palate is surgically closed byelevating two muscoperiostealflaps. The levator muscles are ele-vated, redirected and repaired; anda three layer closure of nasalmucosa, muscle and oral mucosaaccomplished. Surgery under gen-eral anesthesia usually lasts about2 hours. Special precautions asthose after the repair of the cleft lipare necessary for 2-3 weeks. Weprefer that the child be weanedfrom the bottle and pacifier prior tothe palatal repair. No hard orcrunchy foods are allowed for 3weeks post operatively.

Cleft Palate Repair:

Closure of cleft palate with pushback palatoplasty. A). Twomucoperiosteal flaps are outlined. B). Flaps are elevated off thehard palate. C,D). The abnormal levator muscle insertion to thehard palate is identified and cut free. E). The nasal lining isclosed as a separate layer and the levator muscle reapproximated.F). The palatal mucoperiosteal flaps are closed in a V-Y fashion.


PHARYNGEAL FLAPApproximately 70-80 percent of

all cleft palate patients will developvelopharyngeal competence afterpalate closure and thus the potentialfor normal speech. The remaining20-30 percent will require speechtherapy and/or an additional surgi-cal procedure called a pharyngealflap. To correct persistent hypernasalspeech, this procedure involves rais-ing a flap of tissue from the posteriorpharynx and inserting it into the softpalate. This flap is indicated whenthe repaired palate is too short or themuscles do not function properly,causing a persistent hypernasalspeech. The procedure is performedusually after the age of 4-5 whenspeech and velopharyngeal compe-tence can be thoroughly assessedand before the child begins school.

LATE CLEFT TREATMENT

The Craniofacial Center can alsohelp those individuals that havegrown up without access to a com-prehensive, coordinated teamapproach. For adults with speechproblems, the previously mentionedpharyngeal flap, combined with anintensive regimen of speech therapy,can produce significant improve-ments. Orthognatic surgery is avail-able to patients with deformities ofthe jaws to improve their appearanceas well as to correct dental occlusion.For soft tissue revision of a severelytightened or notched upper lip, anAbbe flap is the surgical option. Thisprocedure is usually indicated inbilateral cleft patients who have ashort or deficient columella and atightened upper lip. This operation

Pharyngeal Flap:

The pharyngeal flap procedure for hypernasal speech. A superior-ly based flap of tissue is raised from the posterior pharynx andsutured to the soft palate thereby decreasing the amount of airthrough the nose. Lateral ports or holes are left so that the nosewill not be obstructed.

Inadequate Closure

Adequate Closure

Corrected Closure








Pierre Robin

Ear Reconstruction

Reconstruction of the ear is one ofthe most challenging problems fac-ing a reconstructive surgeon as itdemands precise technique com-bined with artistic creativity.Microtia is a congenital deformity ofthe external ear where the auricle(the external ear) is severelydeformed. There may be a spectrumof external ear deformities with var-ious degrees of involvement of themiddle and inner ear. This type ofear deformity is commonly seen inpatients with hemifacial microsomiaand Treacher-Collins syndrome.

Psychological effects of an eardeformity play a significant role intiming of reconstruction. Most sur-geons prefer to initiate treatmentwhen the patient is between 5 and 7years of age since this early inter-vention will reduce anxiety as aresult of peer pressure. This alsoallows for sufficient rib growth toprovide the quantity of cartilageneeded by the surgeon for adequateframework fabrication. Surgery atthis time can give a more consistentresult than earlier intervention dueto the fact that the child has had achance to grow, thus making it easi-er for the surgeon to balance the sizeand shape of the reconstructed earto the child’s normal ear.

MICROTIAThe treatment of microtia

involves surgical reconstruction ofthe external ear framework. Ear

EAR RECONSTRUCTION


Costal cartilage harvestedfrom left chest wall.

Cartilage carved into ear framework.

Microtia

reconstruction requires a carefully planned, stagedreconstruction that involves 3-4 operative procedures.The first procedure involves the construction of the car-tilage framework for the ear. Under general anesthesia,donor cartilage for the frame is obtained en bloc from the

Tennessee Craniofacial Center 1(800) 418-3223 ©1997 Erlanger Health System

rib area contralateral to the ear being recon-structed to take full advantage of the carti-lage’s natural curvature. Working frompre-surgical templates that have been drawnas well as from photographs, the surgeon thencarves the cartilage into its new shape andcarefully positions the graft into position. Theoverlying skin then redrapes to the newlycarved cartilage framework. Subsequent oper-ations are required to rotate the lobule and toelevate the framework into its final position.

Costal cartilage harvested fromchest wall.

Cartilage carved into earframework.

It is of significance to note that if there isnormal hearing in one ear, surgery to improvehearing in the abnormal ear is not recom-mended. Almost 90% of all patients withmicrotia have only unilateral involvement andquickly adjust to this condition followingbirth. Potential gains from working on themiddle ear are outweighed by the inherentrisks of the surgery itself. Therefore, middleear surgery should be performed only on thetrue bilateral microtia patient or the patientwith significant hearing loss in both ears.

Nine year old with left microtia.

Microtia

Postoperative result after staged ear reconstruction.




Reconstruction of traumatic ear deformity

TRAUMATIC EARDEFORMITIES

The traumatic amputation ofan ear is another circumstance inwhich this type of staged carti-lage reconstruction can be effec-tively used. The amount of earloss determines the types andstages of reconstruction needed.If only a small part of the ear islost from trauma or tumor resec-tion, then helical or rim advance-ment flaps may be used toreconstruct this portion. If largersections are lost then a stagedreconstruction is necessary.Effective ear reconstruction isdependent upon meticulous sur-gical technique and careful pre-operative planning.

Microtia

Four year old born with right microtia.

Appearance of ear after first stagereconstruction.

Postoperative result after stagedreconstruction.


Aquired Deformity (Human Bite to Ear)

Human bite to ear with loss of middle third.

Helical rim flap reconstruction. Postoperative result.

Protruding Ears

Eight year old with bilateral protruding ears.

Postoperative result after otoplasty.

OTOPLASTYAnother congenital deformi-

ty of the ears is called prominentor protruding ears. The ear’sprominence is due to lack ofdevelopment of the antihelicalfold. The primary reason to cor-rect this deformity is to elimi-nate the psychological traumathat this condition can cause.Peer ridicule can be severe withthis particular deformity. Theoperation designed to correctthis problem involves recreatingthe antihelical fold curling orsetting the ears back closer to thehead. With experience, consis-tently good results can beobtained.

Craniosynostosis


Craniosynostosis can be defined as thepremature closing of one or more of thenormally present bony gaps between thedifferent bones of the skull. These linearareas where the bones have not yet healedtogether are called cranial sutures. Theyintersect in certain areas of the skull form-ing “soft spots” or fontanelles. The cranialsuture lines or non-ossified zones accom-modate the brain’s growth and expansionduring the first years of life. The brainincreases in volume about 2-1/2 to 3 timesthe first two years of life with these non-ossified areas of bone remaining open for avariable length of time and then closing in apredictable manner. Premature synostosisor closure of the suture will stop the growthof the skull in the direction perpendicularto that suture; however, growth will contin-ue in the direction parallel to the suture.The result is that synostosis of a particularsuture will alter the shape of the skull in apredictable manner with recognizable pat-terns. With multiple suture involvement,increased intracranial pressure can occursince the skull cannot sufficiently expand toaccommodate normal brain growth. Inmoderate craniosynostosis with only onesuture involved increased intracranial pres-


3-D Scan of Sagittal Synostosis

CRANIOSYNOSTOSIS

sure has been found to be present in approxi-mately 13-23% of patients.

The surgical treatment of craniosynostosishas been transformed by the development andapplications of craniofacial techniques toreshape the skull and upper face in the infant.Functionally, the goal in treatment is to releasethe fused sutures to prevent any problems asso-ciated with increased intracranial pressure aswell as creating the potential for normal growth.Cosmetically, the goal is to obtain normal shapeof the skull and face, thus minimizing psychoso-cial problems.


together to insure that the patients are evalu-ated and treated in a coordinated manner andthat all their needs, both physical and psy-chosocial, are met. The team combines theexpertise of each specialist to provide a levelof comprehensive care that cannot be provid-ed by a single physician. The primary physi-cian or pediatrician plays a key role in earlydiagnosis and referral so that a successfultreatment is accomplished.

Surgical Treatment: Tremendousadvancements have been made in the treat-ment of infants with isolated craniosynostosisdue to the application of craniofacial surgeryprinciples. Past treatments such as stripcraniectomies which remove only the fusedsuture have resulted in unpredictable results.These procedures have often resulted in skulland facial deformities that are difficult to cor-rect in the older child. Current surgical treat-ment involves a team approach with acombined expertise of a neurosurgeon andcraniofacial surgeon. The goal of surgery is toestablish normal contour of the brow, fore-head and skull; and to allow the brain togrow and expand in a normal manner withrelease of synostosis. The timing of surgicalcorrection depends on the age at diagnosisand referral. In our experience we feel thatthe best results are obtained when infantsundergo surgical repair between 4 and 8months of age. This period has several advantages:

(1) remodeling is easier because the bone isvery malleable;

(2) rapid brain growth benefits bone remodeling;

(3) bone defects heal rapidly.Early correction also prevents further

deformity caused by unreleased, fusedsutures. Therefore, early diagnosis and refer-ral to a craniofacial center is crucial. We advo-cate extensive release of the fused suture,total reshaping of the brow, superior orbits,and skull as necessary between 4 and 8months of age. Older patients will require

Approximately one infant in every onethousand births will have a premature clo-sure of a cranial suture associated with askull deformity. Infants born with abnormalskull shapes should be suspected of havingcraniosynostosis. However, abnormal skullshapes at birth may be related to fetal headposition or birth trauma; this type of defor-mity will usually correct itself in severalmonths. True craniosynostosis will notimprove with time and usually worsens withgrowth. On examination palpation of theabnormal portion of the head often reveals aridge in the area of the fused suture. Oncecraniosynostosis is suspected, the diagnosiscan be verified with plain skull radiographsand CT scans. We routinely obtain CT scanswith three-dimensional reconstructions toconfirm the diagnosis, rule out other intracra-nial abnormalities, and to fully assess thedeformity. The 3-D scans can very clearlyshow the skull shape and help in pre-opera-tive planning. Isolated craniosynostosis mustbe distinguished from a syndrome that has asone of its components craniosynostosis. Thespecific diagnosis is an important startingpoint in treatment planning.

Etiology: While the pattern of embryoniccraniofacial development has been welldefined through extensive research, very lit-tle is known about the etiology of many cran-iofacial anomalies. There is strong evidencefor a genetic role in the occurrence of somecraniosynostosis. An inherited tendency orfamily history has been reported in manycases in the literature, however, most cases ofisolated craniosynostosis are usually spo-radic in occurrence with no known etiology.

Team Evaluation: Patients with cran-iosynostosis need to be evaluated and treatedat a recognized craniofacial center with ateam approach. We have a multidisciplinaryteam at our center to provide the comprehen-sive care necessary to adequately evaluateand treat craniofacial patients and their fami-lies. Members of the craniofacial team work


modification of thisplan due to the pres-ence of more rigid boneand slower cranialgrowth. In addition,older patients mayrequire reconstructionof secondary facialdeformities.

In the event thatintraoperative or post-operative blood trans-fusions are needed, theparents are asked toprovide designateddonor blood. In addi-tion, aspirin and othermedications that mightprolong the blood clotting time are to beavoided.

FUSED SUTURE NAME DESCRIPTION

Sagittal Scaphocephaly Boat Skull

Metopic Trigonocephaly Triangular Skull

Unilateral Coronal Plagiocephaly Asymmetric Skull

Bicoronal Brachycephaly Short Skull

Lambdoid Plagiocephaly Asymmetric Skull

TERMINOLOGY OF CRANIOSYNOSTOSIS

Multiple suture lines shown in infant skull.

Lambdoid Suture

Sagittal Suture

Coronal SutureMetopic Suture


SAGITTAL SYNOSTOSISPremature closure of the sagittal

suture, the longitudinal suture on thetop of the head, stops growth laterallyproducing a narrow head. There is acompensatory growth in the antero-posterior direction with elongation ofthe skull and a bulging of the frontand back of the head. This particulardeformity is called scaphocephalydue to the skull’s boat shaped appear-ance. Synostosis of the sagittal sutureis the most frequent type of singlesuture craniosynostosis. It makes up50-60% of all patients within NorthAmerica with craniosynostosis. Theparticular infants with sagittal synos-tosis characteristically have normalintelligence.

In sagittal synostosis or scapho-cephaly we most frequently performtotal skull reshaping. In this type ofsynostosis the forehead and back ofthe skull are usually bulging, over-projected, and the width of the skulltoo narrow. The front, back and later-al bone plates are removed and thenreshaped to a more normal contour.The forehead is tilted back (as well asthe occiput) and the entire anteropos-terior dimension of the skull is short-ened. The reshaped bones are securedin position with wires and/ormicroplates. Certain gaps between thebones are left unsecured to avoidrestriction of growth. The dura, orouter covering of the brain, may beplicated in the frontal region todecrease the prominence. Barrel stavecuts are made in the lateral bone at thebase of the skull to allow more projec-tion of the skull laterally, increasingthe transverse width. This type of pro-cedure gives a more definitive andimmediate improvement in skullshape.

Scaphocephaly




METOPIC SYNOSTOSIS

The metopic suture runs down themidline of the forehead. Premature fusionof this suture results in a triangular shapedforehead called trigonocephaly. A bonyridge is usually palpable that extends fromthe bridge of the nose to the upper part ofthe forehead. This premature fusion pro-duces a prominent midline keel with later-al recession of the brows. From the frontalview these patients typically have theappearance of hypotelorism or decreaseddistance between the eyes.

The incidence of metopic synostosis inmost cases of craniosynostosis is between5-10%. This deformity is usually obvious atbirth, but the severity of the anomaly mayvary. We prefer to correct these deformitiesbetween 4 and 8 months of age. Our cur-rent surgical treatment consists of frontalbone remodeling and supralateral orbitaladvancement. The triangular or keel-shaped forehead is removed and recon-toured to the appropriate shape or bonemay be taken from another area of theskull to replace this bone. The entire bonysupraorbital bar is removed and reshapedwith supralateral orbital advancement torestore the normal brow contour.Microplates are used to secure the frontalbar to the facial bones and maintain thenormal contour.


Intraoperative photograph of triangular shaped forehead(left) and brow (right).

Trigonocephaly


UNILATERALCORONAL SYNOSTOSIS

Premature fusion of one of thecoronal sutures results in an asym-metric forehead and brow. On theaffected side the forehead is flattenedand recessed with the brow andsupraorbital rim both elevated andrecessed. The contralateral foreheadmay exhibit compensatory bulgingor bossing. This ultimately results ina very asymmetric malformationcalled plagiocephaly. Technically,plagiocephaly refers to any type of asymmetric skull deformity.However, many authors have usedthis synonymously with unilateralcoronal synostosis.

The incidence of unilateral coro-nal synostosis in most series of cran-iosynostosis is between 10-20%. Ifuntreated, this type of synostosismay result in a severe deformity ofthe forehead, orbit, and nose thatpersists or worsens with growth. Wefeel the best treatment for plagio-cephaly should involve a bilateralapproach with remodeling of theentire forehead and brow betweenthe ages of 4 and 8 months. Our cur-rent surgical treatment consists ofextended bicoronal craniectomieswith bilateral frontal bone recon-struction. The supraorbital bar iscompletely removed, reshaped, andstraightened. The supraorbital bar isadvanced and lowered as necessaryon the affected side and rigidly fixedin position to the face. The recon-structed forehead is then secured tothe supraorbital bar. We feel that thebilateral approach obtains a moresymmetric forehead and brow thanthe unilateral approach.



Plagiocephaly


BICORONALSYNOSTOSIS

Bicoronal suture fusionresults in a flat retrudedforehead with increasedheight to the skull. Thiscondition is also calledbrachycephaly due to theshort anteroposteriordiameter. As a result of thisshortening there is a com-pensatory bulging of thetransverse diameter orwidth of the skull. Thebrow is usually recessedand elevated with thedeformity being symmet-ric. The incidence ofbicoronal synostosis orbrachycephaly is between10-20% in most series ofcraniosynostosis. This mayoccur as an isolated occur-rence or part of a morecomplex syndrome withfacial retrusion such asApert’s or Crouzon’s.Surgical treatment consistsof extended bicoronalcraniectomies with recon-struction of the forehead.The supraorbital bar orbrow is reshaped andadvanced forward with theforehead. The reconstruct-ed forehead and brow arerigidly fixed to the noseand lateral orbits withmicroplates. If there isexcessive height to theskull then total calvarialremodeling is also per-formed, decreasing theheight of the skull.


Intraoperative lateral view of skullbefore remodeling.

Intraoperative lateral view of skull afterremodeling.

Brachycephaly


POSTERIOR PLAGIOCEPHALY

There has been a significant increasein the past several years of infantsreferred for evaluation of occipitaldeformities. This increase in occurrenceof posterior skull deformities appears tobe related to the 1992 recommendationfor supine sleep positioning by TheAmerican Academy of Pediatrics toavoid Sudden Infant Death Syndrome.Occiput deformities can be divided intolambdoid synostosis and deformationalor positional plagiocephaly.

The lambdoid suture is a pairedstructure that transverses in a symmetric

Posterior Plagiocephaly

Postoperative result after posteriorskull remodeling.

PostoperativePreoperative Postoperative

Preoperative photo showing left posterior skull flattening.

Postoperative result after posteriorskull remodeling.


fashion across the posterior aspect of theskull. Premature fusion or synostosis of thissuture is rare but may be unilateral or bilat-eral.

Characteristically in unilateral synosto-sis the posterior skull is flattened on theinvolved side. Displacement of the ear withcompensatory skull changes may be presentin severe cases. Bilateral synostosis is char-acterized by extensive flattening across theposterior skull and increased verticalgrowth to accommodate brain enlargement.In mild cases the deformity can be coveredby hair and surgical repair may not be indi-cated. When there is significant flatteningor asymmetry, we recommend release of thefused sutures and posterior skull remodel-ing. Surgical treatment consists of extensiveposterior skull craniectomies with remodel-ing of both sides of the occiput and posteri-or advancement of the affected side. Rigidfixation with microplates is used to hold thereconstructed bones in position and main-tain the contour until healing takes place.

Occipital plagiocephaly without synos-tosis must be distinguished from lambdoidsynostosis. Most patients referred to ourcenter for posterior skull deformities aredeformational or positional plagiocephaly.In these infants, the skull becomes mis-shaped from repeated pressure on the sameposition, without the premature fusion ofthe lambdoid suture. Positional plagio-cephaly usually improves without surgery.Once the child’s sleep patterns change andhe spends more time awake, the brain’s nor-mal growth forces help to reshape the poste-rior skull. Some centers recommend acustom fitted helmet to mold the head backinto position over a period of severalmonths. However, in cases where the poste-rior skull deformity progresses or is severe, we recommend surgical remodeling asdescribed, even though a true synostosismay not be present.

CRANIOFACIAL DYSOTOSISCraniofacial dysotosis (Crouzon’s and Apert’s

diseases) are characterized by craniostenosiswith cranial dysmorphia and facial deformities(hence, the term craniofacial dysotosis).

APERT SYNDROMEApert syndrome or acrocephalosyndactyly

syndromes are rare conditions. In 1906, Apertdescribed the skull, facial, and hand deformitiesof several patients characteristic of this syn-drome that now bears his name. The incidence ofinfants born with Apert syndrome is one forevery 100,000 to 160,000 live births. Many of theinfants born with this syndrome show a sporadictransmission, which means that a family mayhave a child with Apert’s when no other mem-bers of the family are affected. The recurrent riskof having another child with Apert’s for twounaffected parents is negligible. However, if theparent is affected there is a 50% chance of eachoffspring having Apert syndrome with bothmales and females affected equally.

Clinical Features: Patients with Apert syn-drome have very distinct facial and extremityfeatures. Abnormal skull shape is due to cran-iosynostosis or premature fusion of the suturesor soft spots. The skull usually demonstrates ashort anteroposterior diameter (brachycephaly)and may be excessively tall (turricephalic) orabnormally wide (euryprosopia). The foreheadis generally always retruded, but this may not beobvious due to the hypoplasia of the midface.The orbits or bony sockets which contain theeyes are very shallow causing a bulging or prop-tosis. The orbits are usually rotated downwardand lateral causing a downward shape to the lat-eral corners of the eyes. There may be a moder-ate increased distance between the eyes(hypertelorism) with muscle imbalance.

The middle of the face in Apert’s is bothretruded and very hypoplastic. This causes thecentral midface to have a characteristic sunken-


in appearance with the nose beingthick and beaked. The upper jaw ormaxilla characteristically shows anarrow arch with an open bite anddental crowding. The maxilla issignificantly retruded compared tothe mandible with the teeth of thelower jaw projecting in front of theupper teeth. Other possible clinicalfeatures include moderate hearingloss, speech impairment, acne, anddecreased mental capability insome individuals. Intellectualpotential may be difficult to evalu-ate due to communication prob-lems. Some patients with Apertsyndrome may have normal intelli-gence. All patients with Apert syn-drome demonstrate a unique handmalformation. This is characterizedby a complex syndactyly or fusionof the skin, soft tissue, and bones ofthe fingers. Both hands are affectedequally, as are the feet. This unusu-al variation of syndactyly can beused to identify Apert’s from othersimilar syndromes.

Treatment: The treatment ofpatients with Apert syndrome isnot uniform due to significant vari-ations in the facial anomalies, ageof patients when first seen, andprevious operations. Our primaryconcern of the infant born with thissyndrome is: compression of thebrain, breathing problems, pro-truding eyes with corneal expo-sure, and lack of facial growth. Thesurgical plan must be flexible and individualized to the patient.Multiple stages or operations at different ages are usually necessary.

When we see these patients asinfants, the first stage is treatmentof the craniosynostosis with total

Apert Syndrome

Preoperative photo of infantwith Apert syndrome.

Postoperative result after fore-head and brow advancement.


Characteristic appearance of complex syndactyly of hand.


calvarial reshaping. We prefer to do thisprocedure at four to six months of age. Afrontal-orbital advancement is per-formed which increases the intracranialspace and size of both orbits. Total skullreshaping helps correct the tower skullproblem which is not addressed byfrontal-orbital advancement alone. Aventriculo-peritoneal shunt may beneeded for treatment of a hydro-cephalus. This is performed prior toskull remodeling. Occasionally a repeatcraniotomy is needed to further reshapethe calvarial vault and advance theorbits. The next stage of the reconstruc-tion is midfacial advancement. We usu-ally perform this procedure between theages of four to six years old prior tostarting school. If necessary, anintra/extracranial advancement of theentire face and forehead (monobloc) canbe performed with correction of mild

hypertelorism or lateral rotation of the orbits.This procedure, as described by Tessier, is calleda facial bipartition and corrects several deformi-ties at once. It effectively widens the maxilla andderotates the orbits, and narrows the upper face.In milder cases an extracranial LeFort IIIadvancement may be used.

The final steps in the reconstruction are max-illary/mandibular osteotomies to complete thecorrection of any further dental discrepancies.These procedures are usually performed aftereruption of permanent dentition and completionof growth (teen years). Additional proceduressuch as rhinoplasty, genioplasty and eyelidsurgery may be beneficial. Surgical separation ofthe fingers is usually started in the first year oflife and completed by three to four years of age.

The patient with Apert syndrome representsa complex combination of multiple deformities.Our goal is to treat not only the function andphysical problems but the psychosocial issues as

Postoperative result after facialadvancement.

Apert Syndrome

This little girl has the character-istic facial appearance of ApertSyndrome which is also associat-ed with complex syndactyly.

Preoperative


CROUZON SYNDROME

This syndrome was originallydescribed in 1912 by a French neuro-surgeon. He described four essentialcharacteristics: exorbitism, retromaxil-lism, inframaxillism and parodoxicretrogenia. The incidence of this syn-drome appears to be approximatelyone in 25,000 in the general popula-tion. It is inherited as an autosomaldominant pattern with variableexpression. However, approximately25% of reported cases have no familyhistory and represent a new mutation.

Clinical Features: Patients withCrouzon syndrome have very distinctfacial features with craniofacial bonemorphology similar to Apert syn-drome. Premature fusion of thebicoronal suture is the most commontype of craniosynostosis with abrachycephalic or oxycephalicappearance to the skull. The extent ofthe craniosynostosis may be variableas well as the age at onset. There isretrusion of both the forehead andbrow, with midface hypoplasia andshallow orbits with bulging eyes(proptosis). Orbital hypertelorism isless commonly seen in Crouzon’s thanApert’s syndrome. The lateral canthimay be slanted downward with somedegree of upper eyelid ptosis. Theproptosis is usually more prominentin Crouzon’s than Apert’s; however,in Apert syndrome, the other craniofa-cial deformities are more complex andsevere. In addition, hydrocephalusand developmental delay are less fre-quently seen in Crouzon’s thanApert’s. The nasomaxillary retrusionmay cause some degree of nasal air-way obstruction with mouth breath-

Apert Syndrome

Four year old girl with Apertsyndrome.

Postoperative result aftermonobloc facial advancementwith facial bipartition (medialrotation of orbits).


well. We feel these individuals are best evaluated andtreated by a craniofacial center that utilizes the multi-disciplinary team approach providing a coordinated,comprehensive, long-term treatment plan with carefulmonitoring of growth and development.


osteotomy is used to correct the dental discrepancies incombination with orthodontic intervention. This is usual-ly performed after facial growth is completed and may becombined with a genioplasty (chin reduction/advance-ment). Additional procedures such as rhinoplasty may beneeded.

ing. The chin is usually large withincreased vertical height but oftenretruded resulting in a paradoxicalretrogenia. The maxillary teeth areretruded compared to the mandiblewith a class III malocclusion. Otherpossible abnormalities include con-ductive hearing loss, inverted V-shape to palate and exposurekeratitis. Variations of this type ofcraniofacial dysostosis are seen in thesyndromes of Carpenter, Pfeiffer andSaethre-Chotzens.

Treatment: Our approach to thetreatment of Crouzon syndrome issimilar to that described for Apertsyndrome. The first stage involvestreatment of the craniosynostosiswith total calvarial reshaping asneeded and frontal-orbital advance-ment. This procedure is usually per-formed at four to six months of ageand is effective in increasing theintracranial space and enlarging theorbits. It is not unusual for a ven-triculo-peritoneal shunt to be neededfor treatment of a hydrocephalus.Occasionally, a repeat craniotomywill be needed in infancy to furtherreshape the calvarial vault andadvance the orbits. The next stage ofreconstruction is midface advance-ment performed at four to six yearsof age. This type of advancement canbe accomplished with either a LeFortIII or a monobloc (entire face andforehead) osteotomy. The position ofthe forehead and brow determineswhich procedure would be the bestalternative. If necessary, themonobloc osteotomy can be furthersplit in the midline to correct orbitalhypertelorism (facial bipartition).

The final stage of the reconstruc-tion is management of the dentalclass III malocclusion. A LeFort I

Young woman with Crouzon’sSyndrome.

Result after correction oforbital hypertelorism, maxil-lary advancement, mandibular setback and chin reduction.

Two year old boy withCrouzon’s syndrome whoexhibits severe proptosis.

Postoperative appearance afterforehead-brow advancement. ALefort III advancement will beneeded in the future.

Crouzon Syndrome


fashion across the posterior aspect of theskull. Premature fusion or synostosis of thissuture is rare but may be unilateral or bilat-eral.

Characteristically in unilateral synosto-sis the posterior skull is flattened on theinvolved side. Displacement of the ear withcompensatory skull changes may be presentin severe cases. Bilateral synostosis is char-acterized by extensive flattening across theposterior skull and increased verticalgrowth to accommodate brain enlargement.In mild cases the deformity can be coveredby hair and surgical repair may not be indi-cated. When there is significant flatteningor asymmetry, we recommend release of thefused sutures and posterior skull remodel-ing. Surgical treatment consists of extensiveposterior skull craniectomies with remodel-ing of both sides of the occiput and posteri-or advancement of the affected side. Rigidfixation with microplates is used to hold thereconstructed bones in position and main-tain the contour until healing takes place.

Occipital plagiocephaly without synos-tosis must be distinguished from lambdoidsynostosis. Most patients referred to ourcenter for posterior skull deformities aredeformational or positional plagiocephaly.In these infants, the skull becomes mis-shaped from repeated pressure on the sameposition, without the premature fusion ofthe lambdoid suture. Positional plagio-cephaly usually improves without surgery.Once the child’s sleep patterns change andhe spends more time awake, the brain’s nor-mal growth forces help to reshape the poste-rior skull. Some centers recommend acustom fitted helmet to mold the head backinto position over a period of severalmonths. However, in cases where the poste-rior skull deformity progresses or is severe, we recommend surgical remodeling asdescribed, even though a true synostosismay not be present.

CRANIOFACIAL DYSOTOSISCraniofacial dysotosis (Crouzon’s and Apert’s

diseases) are characterized by craniostenosiswith cranial dysmorphia and facial deformities(hence, the term craniofacial dysotosis).

APERT SYNDROMEApert syndrome or acrocephalosyndactyly

syndromes are rare conditions. In 1906, Apertdescribed the skull, facial, and hand deformitiesof several patients characteristic of this syn-drome that now bears his name. The incidence ofinfants born with Apert syndrome is one forevery 100,000 to 160,000 live births. Many of theinfants born with this syndrome show a sporadictransmission, which means that a family mayhave a child with Apert’s when no other mem-bers of the family are affected. The recurrent riskof having another child with Apert’s for twounaffected parents is negligible. However, if theparent is affected there is a 50% chance of eachoffspring having Apert syndrome with bothmales and females affected equally.

Clinical Features: Patients with Apert syn-drome have very distinct facial and extremityfeatures. Abnormal skull shape is due to cran-iosynostosis or premature fusion of the suturesor soft spots. The skull usually demonstrates ashort anteroposterior diameter (brachycephaly)and may be excessively tall (turricephalic) orabnormally wide (euryprosopia). The foreheadis generally always retruded, but this may not beobvious due to the hypoplasia of the midface.The orbits or bony sockets which contain theeyes are very shallow causing a bulging or prop-tosis. The orbits are usually rotated downwardand lateral causing a downward shape to the lat-eral corners of the eyes. There may be a moder-ate increased distance between the eyes(hypertelorism) with muscle imbalance.

The middle of the face in Apert’s is bothretruded and very hypoplastic. This causes thecentral midface to have a characteristic sunken-


in appearance with the nose beingthick and beaked. The upper jaw ormaxilla characteristically shows anarrow arch with an open bite anddental crowding. The maxilla issignificantly retruded compared tothe mandible with the teeth of thelower jaw projecting in front of theupper teeth. Other possible clinicalfeatures include moderate hearingloss, speech impairment, acne, anddecreased mental capability insome individuals. Intellectualpotential may be difficult to evalu-ate due to communication prob-lems. Some patients with Apertsyndrome may have normal intelli-gence. All patients with Apert syn-drome demonstrate a unique handmalformation. This is characterizedby a complex syndactyly or fusionof the skin, soft tissue, and bones ofthe fingers. Both hands are affectedequally, as are the feet. This unusu-al variation of syndactyly can beused to identify Apert’s from othersimilar syndromes.

Treatment: The treatment ofpatients with Apert syndrome isnot uniform due to significant vari-ations in the facial anomalies, ageof patients when first seen, andprevious operations. Our primaryconcern of the infant born with thissyndrome is: compression of thebrain, breathing problems, pro-truding eyes with corneal expo-sure, and lack of facial growth. Thesurgical plan must be flexible and individualized to the patient.Multiple stages or operations at different ages are usually necessary.

When we see these patients asinfants, the first stage is treatmentof the craniosynostosis with total

Apert Syndrome

Preoperative photo of infantwith Apert syndrome.

Postoperative result after fore-head and brow advancement.


Characteristic appearance of complex syndactyly of hand.


calvarial reshaping. We prefer to do thisprocedure at four to six months of age. Afrontal-orbital advancement is per-formed which increases the intracranialspace and size of both orbits. Total skullreshaping helps correct the tower skullproblem which is not addressed byfrontal-orbital advancement alone. Aventriculo-peritoneal shunt may beneeded for treatment of a hydro-cephalus. This is performed prior toskull remodeling. Occasionally a repeatcraniotomy is needed to further reshapethe calvarial vault and advance theorbits. The next stage of the reconstruc-tion is midfacial advancement. We usu-ally perform this procedure between theages of four to six years old prior tostarting school. If necessary, anintra/extracranial advancement of theentire face and forehead (monobloc) canbe performed with correction of mild

hypertelorism or lateral rotation of the orbits.This procedure, as described by Tessier, is calleda facial bipartition and corrects several deformi-ties at once. It effectively widens the maxilla andderotates the orbits, and narrows the upper face.In milder cases an extracranial LeFort IIIadvancement may be used.

The final steps in the reconstruction are max-illary/mandibular osteotomies to complete thecorrection of any further dental discrepancies.These procedures are usually performed aftereruption of permanent dentition and completionof growth (teen years). Additional proceduressuch as rhinoplasty, genioplasty and eyelidsurgery may be beneficial. Surgical separation ofthe fingers is usually started in the first year oflife and completed by three to four years of age.

The patient with Apert syndrome representsa complex combination of multiple deformities.Our goal is to treat not only the function andphysical problems but the psychosocial issues as

Postoperative result after facialadvancement.

Apert Syndrome

This little girl has the character-istic facial appearance of ApertSyndrome which is also associat-ed with complex syndactyly.

Preoperative


CROUZON SYNDROME

This syndrome was originallydescribed in 1912 by a French neuro-surgeon. He described four essentialcharacteristics: exorbitism, retromaxil-lism, inframaxillism and parodoxicretrogenia. The incidence of this syn-drome appears to be approximatelyone in 25,000 in the general popula-tion. It is inherited as an autosomaldominant pattern with variableexpression. However, approximately25% of reported cases have no familyhistory and represent a new mutation.

Clinical Features: Patients withCrouzon syndrome have very distinctfacial features with craniofacial bonemorphology similar to Apert syn-drome. Premature fusion of thebicoronal suture is the most commontype of craniosynostosis with abrachycephalic or oxycephalicappearance to the skull. The extent ofthe craniosynostosis may be variableas well as the age at onset. There isretrusion of both the forehead andbrow, with midface hypoplasia andshallow orbits with bulging eyes(proptosis). Orbital hypertelorism isless commonly seen in Crouzon’s thanApert’s syndrome. The lateral canthimay be slanted downward with somedegree of upper eyelid ptosis. Theproptosis is usually more prominentin Crouzon’s than Apert’s; however,in Apert syndrome, the other craniofa-cial deformities are more complex andsevere. In addition, hydrocephalusand developmental delay are less fre-quently seen in Crouzon’s thanApert’s. The nasomaxillary retrusionmay cause some degree of nasal air-way obstruction with mouth breath-

Apert Syndrome

Four year old girl with Apertsyndrome.

Postoperative result aftermonobloc facial advancementwith facial bipartition (medialrotation of orbits).


well. We feel these individuals are best evaluated andtreated by a craniofacial center that utilizes the multi-disciplinary team approach providing a coordinated,comprehensive, long-term treatment plan with carefulmonitoring of growth and development.


osteotomy is used to correct the dental discrepancies incombination with orthodontic intervention. This is usual-ly performed after facial growth is completed and may becombined with a genioplasty (chin reduction/advance-ment). Additional procedures such as rhinoplasty may beneeded.

ing. The chin is usually large withincreased vertical height but oftenretruded resulting in a paradoxicalretrogenia. The maxillary teeth areretruded compared to the mandiblewith a class III malocclusion. Otherpossible abnormalities include con-ductive hearing loss, inverted V-shape to palate and exposurekeratitis. Variations of this type ofcraniofacial dysostosis are seen in thesyndromes of Carpenter, Pfeiffer andSaethre-Chotzens.

Treatment: Our approach to thetreatment of Crouzon syndrome issimilar to that described for Apertsyndrome. The first stage involvestreatment of the craniosynostosiswith total calvarial reshaping asneeded and frontal-orbital advance-ment. This procedure is usually per-formed at four to six months of ageand is effective in increasing theintracranial space and enlarging theorbits. It is not unusual for a ven-triculo-peritoneal shunt to be neededfor treatment of a hydrocephalus.Occasionally, a repeat craniotomywill be needed in infancy to furtherreshape the calvarial vault andadvance the orbits. The next stage ofreconstruction is midface advance-ment performed at four to six yearsof age. This type of advancement canbe accomplished with either a LeFortIII or a monobloc (entire face andforehead) osteotomy. The position ofthe forehead and brow determineswhich procedure would be the bestalternative. If necessary, themonobloc osteotomy can be furthersplit in the midline to correct orbitalhypertelorism (facial bipartition).

The final stage of the reconstruc-tion is management of the dentalclass III malocclusion. A LeFort I

Young woman with Crouzon’sSyndrome.

Result after correction oforbital hypertelorism, maxil-lary advancement, mandibular setback and chin reduction.

Two year old boy withCrouzon’s syndrome whoexhibits severe proptosis.

Postoperative appearance afterforehead-brow advancement. ALefort III advancement will beneeded in the future.

Crouzon Syndrome

Orbital Reconstruction


Encephalocele: Congenital nasalencephaloceles are complex problemsthat are best treated by the combinedefforts of a neurosurgeon and a plasticsurgeon. An encephalocele is the hernia-tion of the brain through a congenital ortraumatic opening in the cranium.Alterations and distortions of the sur-rounding facial structures, such asdeformities of the naso-orbital skeletondue to an absence or separation of bonein the midline of the face, are complica-tions of encephaloceles due to theirposition and size. Comprehensive treat-ment includes resecting the encephalo-cele, repairing the fibrous covering ofthe brain, repairing any bony defects,and reconstructing a more normal softtissue facial appearance. With recentadvances in diagnostic and surgicaltechniques, it is possible to perform athorough preoperative evaluation andto treat the lesion with definitive one-stage reconstruction at the time ofexcision.

The nasofrontal encephalocele bone defect is shown withosteotomies for mobilization of the medial orbital walls illustrated. The orbital walls are centrally mobilized and thenstabilized and the remaining defects bone grafted.

A one week old infant born withlarge nasal encephalocele.

Postoperative result at eight months. Postoperative result at five years afterdefinitive one stage construction.

ORBITAL RECONSTRUCTION

Nasal Encephalocele


Seven month old boy born with large nasal encephalocele.

Intraoperative excision of encephalocele and bony reconstruction.

Nasal Encephalocele

Postoperative result after definitive onestage reconstruction.


ORBITALHYPERTELORISM

Orbital hypertelorismrepresents an increasedinterorbital distance and ismost commonly associatedwith craniofacial dysostosis(Apert’s and Crouzon’s dis-eases), encephaloceles, facialclefts, and fronto-nasal dys-plasias. The treatment ofmoderate to severe deformi-ties involves surgery toreduce the interorbital dis-tance and to correct any nasalabnormalities by way of an intracranial surgicalapproach that releases thebony orbits of the eyes andrepositions them closertogether. Inlay bone grafts,secured in place with mini-plate fixation, are then placed

Illustration Upper Right:

Extracranial/Intracranial correctionof orbital hypertelorism. Orbitalwall osteotomies are illustrated withmobilization medially. The remain-ing defects are bone grafted. Afrontal bone flap is usually not nec-essary using this technique.

Fifteen year old male born withincomplete midline facial cleft caus-ing increased distance between eyes.

Postoperative result after extracra-nial reconstruction of hypertelorism.

Thirteen year old male with severeorbital hypertelorism and abnormalforehead contour.

Postoperative result two years after intracranial reconstruction oforbital hypertelorism with foreheadremodeling.

Orbital Hypertelorism


to provide structural supportand to fill the spaces left bymoving the orbits. The idealtiming for this surgery isbetween two and five years ofage in order for the psychologi-cal trauma involved with thedeformity to be minimizedwhile maximizing the ophthal-mological benefits. In caseswhere the deformity fromhypertelorism is less severe, thesurgery can be performed usingan extracranial approach toachieve orbital rearrangement.However, it is generally agreedthat using the intracranial tech-nique constitutes a more consis-tent and safer method ofcorrecting the malformation.


Intracranial correction of orbital hypertelorism

Four year old boy with the severestform of orbital hypertelorism.

Postoperative result

Intracranial correction of orbital hypertelorism. A frontal bone flap is raised and four wall block osteotomies illus-trated. The central bony section is removed and the orbit is mobilized medially.


Three year old girl with orbital hyper-telorism and nasal clefts.

Eight year old girl with encephaloceleand severe orbital hypertelorism.

Postoperative result after intracranialcorrection of orbital hypertelorismwith forehead reconstruction.

Postoperative result



Nine year old girl born with orbitalhypertelorism- very wide set eyes witha broad flat nose. Intraoperative photosshow cuts to be made in orbital bones.A midline segment of bone is removedand orbits advanced medially.Stabilization is obtained with mini-plates and wires.

Postoperative result after intracranialcorrection of orbital hypertelorism.Computer simulated result is comparedto actual postoperative photograph.









Enophthalmos

Enophthalmos


ExophthalmosEXOPHTHALMOSExophthalmos is an abnormal

prominence or protrusion of the eye-ball, most frequently seen in patientswith Grave’s disease (hyperthy-roidism). As with enophthalmos, surgi-cal correction is frequently necessary toachieve the desired aesthetic result. Theextent of the deformity dictates the sur-geon’s choice of treatment options.Although severe exophthalmos maypresent as a surgical emergency inwhich vision is threatened, moderateexophthalmos can also be distressing tothe patient — the wide-eyed stare, lidretraction, and proptosis are at bestunsightly and at worst psychologicallyhandicapping. By utilizing craniofacialapproaches and techniques, excellentaesthetic results can be safely obtained.Mild to moderate cases are repaired byremoving the floor and lateral wall ofthe orbit to allow for tissue decompres-sion (removing compressive pressureon the eyeball itself), while severe casesnecessitate a more aggressive approachincluding multi-wall (lateral, medial,and inferior) orbital osteotomies. Thisfunctionally enlarges the bony orbit,allowing the globe to assume a morenormal posterior position.


This patient has severe exophthalmos caused by hyperthyroidism.

Postoperative result after enlargement of bony orbits.


FACIAL BIPARTITIONThe surgical technique of

facial bipartition involves verticalsplitting or separation of the facialskeleton into two segments. Theprocedure has been used to suc-cessfully correct hypertelorismwith widening and leveling of thelower maxilla. The facial biparti-tion concept can also be designedto provide a facial advancement inaddition to enlargement of themaxilla and medial rotation of theorbits. This procedure offers newpossibilities and can be applied tocorrect a number of deformities invarious syndromes in a one-stageprocedure. With appropriate indi-cations and careful technique, thisprocedure can produce dramaticresults.

Facial Bipartition

Preoperative

Facial Bipartition

The osteotomies and bone resections are illustrated in a frontofacialmonobloc advancement with correction of hypertelorism – the facialbipartition procedure. The frontofacial complex is stabilized with miniplates.

Facial Bipartition

Dramatic changes can be made inthe profile using the bipartitiontechnique illustrated in drawing.

Postoperative


MONOBLOCADVANCEMENT

Infants born withfaciocraniosynostosis mayhave severe airway prob-lems, increased intracranialpressure, vision-threateningproptosis and a failure tothrive. Such life-threateningproblems may be treatedwith a one-stage procedureknown as a monoblocadvancement. This singlephase operation releases thestenosis and advances theforehead and facial bones enbloc to a more anterior posi-tion. This has the goal ofestablishing normal functionand appearance as early aspossible. We feel this proce-dure has increased risks ofinfection due to the likelycommunication between thenasal and intracranial cavi-ties and therefore use it cau-tiously for such indicationsas airway compromise orvision-threatening proptosis.

Monobloc Advancement

Infant born with faciocraniosynos-tosis causing severe airway prob-lems, proptosis and failure to thrive.

Postoperative result after a one-stage advancement of skull and facewith marked improvement in air-way, proptosis and overall healthstatus.








Enophthalmos

Enophthalmos


ExophthalmosEXOPHTHALMOSExophthalmos is an abnormal

prominence or protrusion of the eye-ball, most frequently seen in patientswith Grave’s disease (hyperthy-roidism). As with enophthalmos, surgi-cal correction is frequently necessary toachieve the desired aesthetic result. Theextent of the deformity dictates the sur-geon’s choice of treatment options.Although severe exophthalmos maypresent as a surgical emergency inwhich vision is threatened, moderateexophthalmos can also be distressing tothe patient — the wide-eyed stare, lidretraction, and proptosis are at bestunsightly and at worst psychologicallyhandicapping. By utilizing craniofacialapproaches and techniques, excellentaesthetic results can be safely obtained.Mild to moderate cases are repaired byremoving the floor and lateral wall ofthe orbit to allow for tissue decompres-sion (removing compressive pressureon the eyeball itself), while severe casesnecessitate a more aggressive approachincluding multi-wall (lateral, medial,and inferior) orbital osteotomies. Thisfunctionally enlarges the bony orbit,allowing the globe to assume a morenormal posterior position.


This patient has severe exophthalmos caused by hyperthyroidism.

Postoperative result after enlargement of bony orbits.


FACIAL BIPARTITIONThe surgical technique of

facial bipartition involves verticalsplitting or separation of the facialskeleton into two segments. Theprocedure has been used to suc-cessfully correct hypertelorismwith widening and leveling of thelower maxilla. The facial biparti-tion concept can also be designedto provide a facial advancement inaddition to enlargement of themaxilla and medial rotation of theorbits. This procedure offers newpossibilities and can be applied tocorrect a number of deformities invarious syndromes in a one-stageprocedure. With appropriate indi-cations and careful technique, thisprocedure can produce dramaticresults.

Facial Bipartition

Preoperative

Facial Bipartition

The osteotomies and bone resections are illustrated in a frontofacialmonobloc advancement with correction of hypertelorism – the facialbipartition procedure. The frontofacial complex is stabilized with miniplates.

Facial Bipartition

Dramatic changes can be made inthe profile using the bipartitiontechnique illustrated in drawing.

Postoperative


MONOBLOCADVANCEMENT

Infants born withfaciocraniosynostosis mayhave severe airway prob-lems, increased intracranialpressure, vision-threateningproptosis and a failure tothrive. Such life-threateningproblems may be treatedwith a one-stage procedureknown as a monoblocadvancement. This singlephase operation releases thestenosis and advances theforehead and facial bones enbloc to a more anterior posi-tion. This has the goal ofestablishing normal functionand appearance as early aspossible. We feel this proce-dure has increased risks ofinfection due to the likelycommunication between thenasal and intracranial cavi-ties and therefore use it cau-tiously for such indicationsas airway compromise orvision-threatening proptosis.

Monobloc Advancement

Infant born with faciocraniosynos-tosis causing severe airway prob-lems, proptosis and failure to thrive.

Postoperative result after a one-stage advancement of skull and facewith marked improvement in air-way, proptosis and overall healthstatus.

Treacher Collins Syndrome


Treacher Collins syndrome (alsocalled mandibulofacial dysostosisand Franceschetti Syndrome) is ahighly complex disease process. Thebasic etiology is unknown, but it isgenerally thought to be inherited asan autosomal dominant trait withvariable penetrance. It is character-ized by hypoplasia of the facial bones,especially the zygoma and themandible. Facial clefting causes thishypoplastic appearance, with possi-ble deformities or deficiencies of theear, orbital, midface, and lower jawregions. The clinical appearance is aresult of the zygoma (malar bone)failing to fuse with the maxilla,frontal, and temporal bones. Highlyvariant degrees of involvement (com-plete, incomplete, and abortiveforms) can be seen, but commonfacial features may include:

(1) Hypoplastic cheeks, zygomatic arches, and mandible;

(2) Microtia with possible hearingloss;

(3) High arched or cleft palate;(4) Macrostomia (abnormally

large mouth);(5) Anti-mongoloid slant to the eyes;(6) Colobomas;(7) Increased anterior facial height;(8) Malocclusion (anterior open bite);(9) Small oral cavity and airway with

a normal-sized tongue;(10) Pointed nasal prominence.

The craniofacial team’s geneticistshould evaluate all Treacher Collins

TREACHER COLLINSSYNDROME


Severe mandibular hypoplasia in the patient with Treacher Collins syndrome.

PostoperativePreoperative

Postoperative result after mandibular reconstruction and chinadvancement to correct malocclusion and improve profile.




Identical twins with Treacher Collins syndrome.

patients and their families to determineif the disease has been caused by inheri-tance of a family trait or as the result of aspontaneous gene mutation. If the dis-ease has been inherited by one child in afamily, there is a 50% chance that the par-ents will give birth to another involvedchild. If neither parents nor other familymembers are affected and a child is bornwith the condition, then a mutation hasoccurred. There is a 50% chance that thischild will pass the trait on to future gen-erations. Fortunately, genetic advancesand careful prenatal screening havemade Treacher Collins syndromeextremely rare.

An extensive array of complicationscan affect treatment. Because of the smalljaw and airway, combined with the nor-mal size of the tongue, breathing prob-lems can occur at birth and during sleepfor a child with Treacher Collins syn-drome when the base of the tongueobstructs the small hypopharynx. Thissituation can cause serious problemsduring the induction of general anesthe-sia. Consequently, a tracheostomy maybe required to adequately control the air-way. Learning and speech difficulties canalso occur depending on the degree ofconductive hearing loss common in thesyndrome. Learning disabilities canpotentially create a significant social stig-ma for the child. As with other disfigur-ing conditions, assessing and treating thepsychological needs of the TreacherCollins patient is a vital function of thetrue craniofacial center.

Treatment of the hard and soft tissuesof the face can require a number of sur-gical interventions, the first being thecorrection of eyelid coloboma in the firstyears of life (depending on the severity).The next stage is orbital reconstructionwith calvarial bone grafts and correctionof the lateral canthal displacement.

Multi-stage ear reconstruction follows at about 5-7years of age. Correcting the lower face and jawsinvolves close coordination between the craniofa-cial surgeon and the pedodontist/orthodontist,with orthodontic intervention beginning with theeruption of the patient’s permanent teeth. After theteeth are aligned to their proper axis (or as closelyas is possible), treatment of the lower face theninvolves orthognathic surgery to reposition themandible and the maxilla, usually done during thepatient’s teen years. This can be a one- or two-stepprocedure. The combined procedure involves rotat-ing the midfacial segment around a transverse axisat the frontonasal angle (for severe maxillaryhypoplasia) and lengethening the mandibularramus. For less severe cases, a LeFort I typeosteotomy technique is used to lower the maxillarytuberosities along with the ramus lengthening pro-cedure for the mandible. As the child’s face contin-ues to grow, additional procedures may be required to correct any developing deformities.Complimentary procedures such as rib cartilagegrafts on the zygoma, closure of macrostomia, andsecondary genioplasties are performed accordingto individual cases. A well-planned treatment regi-men can produce excellent results with the ultimategoal being the complete restoration of form andfunction, thus enabling the patient to adapt to a“normal” way of life.


CANTHAL SURGERYMany congenital and acquired deformities

are associated with medial and lateral canthaldisplacement. Patients with blepharophimosis(telecanthus, epicanthal folds, ptosis), hyper-telorism, Down’s Syndrome, craniofacialsysostosis, and acquired deformities may allhave canthal deformities. Evaluation of theposition and shape of the canthal area is anecessity in the planning of all orbital surgery.The contour and position of the canthi areimportant components in the aesthetic balanceand symmetry of the face.

PreoperativeDisplacement of both medial canthi is present in thispatient due to facial trauma.

PostoperativeMedial canthopexies are performed to return the medialcanthi to their normal position.

Canthal Surgery

PostoperativeMedial orbital wall osteotomies and medial canthopexiesperformed to correct deformity.

PreoperativeDisplacement of medial canthi and medial orbital wallsdue to facial trauma.

Canthal surgery essentially consists of repo-sitioning the involved canthal tendon to thedesired position and securing it to the bone.This would seem to be a simple, straight-for-ward procedure. However, because of the com-plex anatomy of the medial canthal area andthe difficulty in obtaining normal, symmetricalsoft tissue contours, this procedure is neither aseasy nor as predictable as it would appear tobe. Attention to surgical technique, soft tissuetension and contour, bone mobilization andposition, and healing of the tendon to bone —all are important in the ultimate results of can-thal surgery.

Nasal Reconstruction


A properly proportioned, wellplaced nose can have a dramaticimpact on one’s facial appearance.Nasal surgery involves a wide spec-trum of procedures ranging fromcosmetic rhinoplasty to total nasalreconstruction. Basic rhinoplastysurgery involves the correction orreshaping of existing nasal struc-tures, whereas more extensive caseswill require that the craniofacial sur-geon actually construct a part of thenose that may be missing or badlymisshapened due to disease or trau-ma. Meticulous attention to detailwhen repairing or reconstructing thenasal lining, skeletal support, or skincovering is essential to obtain a struc-ture that is fully functional as well aspleasing to the eye.

When large defects of the noseare present from tumor resection ortrauma, flap tissue provides the bestaesthetic coverage. The most com-mon flap used for major nasal recon-struction is the forehead flap. Thisreliable flap can supply a large areaof skin with good color match mak-ing it suitable for partial or totalnasal reconstruction.

Reconstruction of the nasal skele-tal framework is frequently neces-sary in patients with congenital ortraumatic deformities. This supportis best obtained using bone or carti-lage. Outer table calvarial bonegrafts harvested from the parietal

NASAL RECONSTRUCTION


Sixteen year old girl withdeformity of the nose.

Result after reconstruction ofnose with bone graft.

Technique of using a cantilever bone graft for reconstruction of thenose. Calvarial bone is most commonly used and is rigidly fixedwith countersunk lag screws.



Resection of basal cell carcinomawith full thickness defect on left side of nose. Forehead flapoutlined.

Forehead flap rotated to closedefect on left side of nose. Tip offlap lined with skin graft.

Postoperative result after division and insetting of forehead flap.

L-shaped grafts and columella struts. The tipof the nose is best supported with cartilagegrafts. Careful attention to detail and planningis necessary in these procedures to create astructure that is both functional and aestheti-cally pleasing.

area of the skull make excellent cantileverbone struts for support of the nasal dorsum.These grafts can be rigidly fixed with lagscrews to provide good stability and dorsalcontour. Additional techniques are availableto provide nasal support such as the



Fifteen year old girl with severe nasomaxillary hypoplasia.

Postoperative result after nasomaxil-lary osteotomy with nasal lengthening

and calvarial bone grafts.


Orthognathic Surgery


Orthognathic surgery refers to thesurgical repositioning of the maxilla,mandible, and the dentoalveolar seg-ments to achieve facial and occlusalbalance. One or more segments of thejaw(s) can be simultaneously reposi-tioned to treat various types of maloc-clusions and jaw deformities.

Preoperative diagnosis and plan-ning for patients with jaw asymmetriesand deformities includes a photo-graphic analysis and a completeorthognathic work-up involvingcephalometric and panorex radi-ographs, dental impressions, and mod-els. This is done by thePedodontist/Orthodontist in coordi-nation with the craniofacial surgeon.All findings are analyzed and pre-sur-gical model surgery performed toascertain the feasibility of varioustreatment options. Additionally, com-puter analysis is done pre-surgicallyby the craniofacial surgeon to simulatesurgical results, thereby facilitatingproper planning of the case. Computeranalysis provides the craniofacial teamwith visual information and numericaldata that is a compilation of manytime-consuming calculations such asthose used in various cephalometricanalyses (Steiner, Ricketts, or Jarabak-Bjork).

Usually, pre-surgical orthodonticsare necessary to straighten the teethand align the arches so that a stableocclusion can be obtained post-opera-tively, while orthodontics followingsurgery are frequently required to

ORTHOGNATHIC SURGERY


Nineteen year old female withmaxillary hypoplasia, malocclu-sion and facial disharmony.

Postoperative result after com-bined maxillary/mandibularprocedures to correct occlusionand improve facial balance.

Twenty year old male with prognathic mandible.

Postoperative result aftermandibular set back.

revise minor occlusal discrepancies. Orthognathicsurgery is often delayed until after all of the permanentteeth have erupted unless medical conditions necessi-tate that the surgery be performed earlier. In adult



A young woman with a prog-nathic mandible and hypoplasticmaxilla.

Postoperative result after maxil-lary advancement and mandibu-lar set back.

Twenty year old woman withmicrogenia.

Postoperative result afterorthognathic surgery and chinadvancement showing improve-ment in lower facial profile.

patients, orthognathic surgery can becombined with soft tissue contouringto improve the aesthetic results.

Maxillary advancement is a type oforthognathic surgery that may be nec-essary to improve the facial contourand normalize dental occlusion whenthere is a relative deficiency of themidface region. This is done by surgi-cally moving the maxilla with sophisti-cated bone mobilization techniquesand fixing it securely into place. Formost patients, the use of screws andminiplates have replaced wiring of thebone and teeth required to hold thejaw stable. Inlay bone grafts can be uti-lized for space maintenance andsecured with screw and plate fixation,while onlay bone grafting is used toaugment the bony skeleton andimprove facial soft tissue contour.

Depending on the soft tissue pro-file of the face or the severity of anocclusal discrepancy, problems withthe lower face may require surgery onthe mandible. This can be done in con-junction with or separate from maxil-lary surgery. The mandible can beadvanced, set back, tilted or augment-ed with bone grafts. A combination ofthese procedures may be necessary.Pre-operative planning is crucial to thesuccess of the procedure and evaluatesthe surgical and orthodontic options.The surgeon chooses the type ofmandibular surgery based on his expe-rience, evaluation of the photographicand cephalometric analysis, andmodel surgery. Following any signifi-cant surgical movement of themandible, fixation may be accom-plished with miniplates and screws orwith a combination of interosseouswires and intermaxillary fixation(IMF). Rigid fixation (screws andplates) has the advantage of needing

limited or no IMF. However, if interosseous wiring isused, IMF is maintained for approximately six weeks.Nutritionally balanced, blenderized diets are importantfor proper healing in the patient in IMF.

The chin is an important component of the facialprofile as well as the aesthetic balance. The position andprojection of the chin should be evaluated in patients


considering orthognathic andfacial soft tissue contouring proce-dures. Photographic and cephalo-metric analysis help determine theamount of change necessary toobtain a well balanced face. Thechin can be augmented with suchalloplastic materials as silicone,polyethylene or hydroxyapatite.However, most craniofacial sur-geons prefer a sliding horizontalosteotomy genioplasty. This pro-cedure tends to give a more natur-al contour to the chin and avoidsthe risk of extrusion that goesalong with alloplastic implants.


Nineteen year old girl with ectodermal dysplasia. She has a hypoplastic maxilla and an enlarged mandible.

Seventeen year old patient withmicrognathia (hypoplastic lower jaw).

Postoperative result aftermandibular advancement.

Postoperative result after maxillary advancement, chin reduction and orthodontic treatment.


Trauma Reconstruction


CRANIOMAXILLOFACIALTRAUMA

Millions of people sustain trauma tothe head and face resulting in complexfractures which, if not correctly diag-nosed and treated, may cause perma-nent functional and cosmeticdeformities. During the past decade,advances in radiographic procedures,the utilization of craniofacial surgicaltechniques, and the advent of rigidminiplate fixation have tremendouslyimproved the functional and aestheticresults in facial fracture management.

The accurate diagnosis of facial frac-tures has been greatly improved by theaddition of two- and three-dimensionalCT scans which have replaced the plainradiographs for the diagnosis of manytypes of fractures. The three-dimension-al reconstructions have enhanced pre-operative bone analysis and planningby providing a life-like simulation of thefractures.

In acute trauma cases, the goal ofreconstruction is a one-stage repairwhich has been made possible by theapplication of craniofacial techniques.Delayed treatment has been replaced byearly or immediate surgical treatmentand stabilization of small bone frag-ments augmented by bone grafts andminiplate fixation. These recentadvances have allowed surgeons toapproach and often reach the goal ofrestoring preinjury facial appearanceand function while at the same timeminimizing revisional surgery.

TRAUMA RECONSTRUCTIONTrauma Reconstruction

A three-dimensional scanabove shows extensive frac-tures of the face.

Postoperative radiograph atright shows stabilization offacial buttresses with 10miniplates.

The maxilla is stabilized with four miniplates.

New miniplate and screw system.


Without treatment in a timelymanner, many individuals willdevelop future problems, theseverity and consequences ofwhich can be much greater than ifthe injury had been immediatelyrepaired. However, modern cran-iofacial surgical techniques cannow offer hope for patients withpre-existing post-traumatic facialdeformities despite considerabledelays between injury, diagnosis,and treatment. These innovativetechniques establish a higher stan-dard of care for the managementof facial injuries.

The following sectionsdescribe the different areas andtypes of facial fractures:

ZYGOMATIC FRACTURES

The zygomatic bone occupiesa prominent and important posi-tion in the facial skeleton. It playsa key role in determining facialwidth as well as acting as a majorbuttress of the midface. Its anteri-or projection forms the malar emi-nence and is often referred to asthe malar bone. The zygoma hasseveral important articulations inthe midface. The zygoma forms asignificant portion of the floor andlateral wall of the orbit. In addi-tion, the zygoma meets the lateralskull to form the zygomatic arch.

The zygoma is the main but-tress between the maxilla and theskull; but in spite of its sturdiness,its prominent location makes itprone to fracture. The mechanismof injury usually involves a blowto the side of the face from a fist,

Front and lateral three dimensional CT Scans demonstrate displaced fractures of all zygomatic buttresses.

Patient with a left displaced zygomatic fracture.

An open reduction with rigidminiplate fixation was performedwith postoperative result shown.

Zygomatic Fractures

object, or secondary to motor vehicle accidents. Moderateforce may result in minimally or nondisplaced fractures atthe suture lines. More severe blows frequently result ininferior, medial, and posterior displacement of the zygoma.Comminuted fractures of the body with separation at thesuture lines are most often the result of high-velocity motorvehicle accidents. In general, displaced fractures willinvolve the inferior orbital rim and orbital floor, the zygo-maticofrontal suture, the zygomaticomaxillary buttress,and the zygomatic arch. Occasionally, however, a directblow to the arch will result in an isolated depressed fractureof the arch only.


Radiographic evaluation of the fractureis mandatory and may include both plainfilms and a computed tomographic (CT)scan. The CT scan has now essentiallyreplaced plain films as the Ògold standardÓin both evaluation and treatment planning.If physical findings and plain films are notsuggestive of a zygomatic fracture, the eval-uation may end here. However, if they dosuggest fracture, a coronal and axial CTscan should be obtained. The CT scan willaccurately reveal the extent of orbitalinvolvement, as well as degree of displace-ment of the fractures. This study is vital forplanning the operative approach.

Historically, closed reduction was themethod of choice for nearly all zygomaticfractures. Multiple methods wereemployed, but most involved simply exert-ing pressure underneath the malar emi-nence and popping the fragments back intoalignment. Not only were these results fre-quently unsatisfactory, but they werefraught with complications including per-sistent diplopia, orbital dystopia, malunion,and significant residual deformity. In ourown experience, closed reductions yieldunpredictable results with significantchance of relapse. We feel that plate andscrew fixation is now the standard of care.

The treatment of zygomatic fractures hasdramatically progressed over the past sev-eral decades from an entirely closedapproach to the more aggressive openreduction and rigid miniplate fixation oftoday. If a zygomatic fracture is displaced,we do an open reduction and rigid stabi-lization with mini-and microplates. Thefloor of the orbit is routinely explored andreconstructed, if needed, to restore orbitalvolume. The complications of an inade-quately or unreduced zygomatic fractureare very difficult to correct secondarily andusually avoidable. We feel that early diag-nosis combined with this aggressive surgi-cal treatment yields the best results.

MAXILLARY FRACTURESThe maxilla forms the largest component of

the middle third of the facial skeleton. The maxil-la is a key bone in the midface that is closely asso-ciated with adjacent bones providing structuralsupport between the cranial base and the occlusalplane. Fractures of the maxilla occur less fre-quently than those of the mandible or nose due tothe strong structural support of this bone. Themidface consists of alternating thick and thin sec-tions of bone that are capable of resisting signifi-cant force. This structurally strong bone providesprotection for the globes and brain, projection ofthe midface, and support for occlusion.Reestablishing continuity of these buttresses is thefoundation on which maxillary fracture treatmentis based.

Renee LeFort (1901) provided the earliest clas-sification system of maxillary fractures. Hismodel described Ògreat lines of weakness in thefaceÓ using low-velocity impact forces directedagainst cadaver skulls. A discussion of fractures ofthe maxilla would not be complete without adescription of LeFortÕs work.

The Lefort I fracture, or transverse fracture,extends through the base of the maxillary sinusesabove the teeth apices essentially separating thealveolar processes, palate, and pterygoid process-es from the facial structures above. This trans-verse fracture across the entire lower maxillaseparates the alveolus as a mobile unit from therest of the midface. Fracture dislocations of seg-ments of the alveolus may be associated with thisfracture. With high-energy injuries, the palatemay be split in the midline in addition to theLeFort I fracture.

A pyramidal fracture of the maxilla is synony-mous with a LeFort II fracture. This fracture pat-tern begins laterally, similar to a LeFort I, butmedially diverges in a superior direction toinclude part of the medial orbit as well as thenose. The fracture extending across the nose maybe variable, involving only the nasal cartilage oras extensive as to separate the nasofrontal suture.


The fracture extends diagonallyfrom the pterygoid plates throughthe maxilla to the inferior orbitalrim and up the medial wall of theorbit to the nose. This separates themaxillary alveolus, medial wall ofthe orbit and nose as a separatepiece.

A LeFort III fracture or craniofa-cial dysjunction denotes a completeseparation of the midface or facialbones from the cranium. This frac-ture transverses the zygomati-cofrontal suture, continues throughthe floor of the orbit, and finallythrough the nasofrontal suture. Thebones of the orbit are separatedthrough the lateral wall, floor, andmedial wall. It is unusual to havethis fracture as a single segment ofbone; more commonly, it commin-utes with varying combinations of

LeFort classification of midfacial fractures.

(A) LeFort I or transverse fracture of the maxilla.(B) LeFort II or pyramidal fracture of the maxilla. (C) LeFort III or craniofacial dysjunction.

A. B.

C.

Trauma Reconstruction

LeFort classification of midfacial fractures.

Twenty-four year old womaninvolved in motor vehicle accidentsustaining panfacial fractures.

Postoperative photo after onestage repair using craniofacialtechniques.

zygomatic, nasoethmoid, and orbital frac-tures. The fractures may not be symmetricon both sides and minimal mobility may bepresent.

Maxillary fractures today are often theresult of motor vehicle accidents. Thesehigh-velocity injuries many times producefracture patterns not classified by the stan-dard LeFort system, but are described sim-ply by the anatomic structure fractured andthe degree of comminution present.Computerized tomographic (CT) scans andthe more recent development of three-dimensional reconstructions have aidedgreatly in the diagnosis, classification, andpreoperative planning of these complexmaxillary fractures.

Maxillary fractures are treated by reduc-tion and immobilization. Establishment ofpreinjury occlusion and midface buttressalignment provides the foundation for thistreatment. The goals of treatment of LeFort

fractures are to reestablish preinjury occlusionwith normal height and projection of the face. Toaccomplish this, the structural buttress of themaxilla must be aligned and stabilized to providethe necessary support and contour to the midface.The proper occlusal relationship between the den-tal arches is established with intermaxillary fixa-tion (IMF), or more appropriately termedmaxillomandibular fixation. Early placement ofthe patient in IMF can eliminate some of the sec-ondary deformities caused by LeFort fractures.IMF is established by securing arch bars to theupper and lower dental arches with individualwire ligatures around the teeth. The appropriateocclusion is then determined by wear facets, andthe maxillary and mandibular arch bar aresecured together. This is one of the simplest andmost effective forms of treatment. However, IMFis more commonly used in conjunction with otherimmobilization and stabilization techniques.

Recent advances in the treatment of maxillaryfractures have been the use of extended open-


Twenty-eight year old patient withpanfacial fractures.

(Top photo) Three Dimensional CTScan shows comminuted maxillaryfractures. (Lower photo) Postoperativeradiograph shows rigid fixation of allfour buttresses.

Postoperative result with restorationof preinjury appearance.

Maxillary Fractures


reduction techniques with rigid plate andscrew fixation of the facial buttresses.Bone grafts have been used to replacemissing or comminuted bone with earlytreatment of these injuries. This moreaggressive surgical approach has dramat-ically improved the aesthetic results nowobtainable with fewer secondary defor-mities.

NASOETHMOID ORBITAL FRACTURES

Trauma to the central midface fre-quently results in fractures of thenasoethmoid orbital (NOE) skeleton. Thiscomplex area consists of a union of bonesfrom the nose, orbits, maxilla, and crani-um. These fractures may be the most dif-ficult and challenging of all facialfractures to diagnose and treat. To theinexperienced examiner, NOE fracturesmay be misdiagnosed as simple nasalfractures, and a high degree of suspicionis necessary to make the diagnosis. Thesefractures may occur as isolated injuries oras part of more complex (LeFort) facialfractures. Swelling of the nose and medi-al canthal areas may mask the fracturesand make the physical findings obscure.Failure to diagnose these injuries or inad-equate treatment will result in both func-tional and cosmetic deformities that arevery difficult to correct secondarily.

The successful surgical treatment ofthese complex injuries consist of earlyopen repair with precise reduction andstabilization of bone fragments. Bonegrafts are used to restore contour andsupport to areas of extremely comminut-ed or missing bone. Soft tissue manage-ment of the naso-orbital area is crucial torestoration of preinjury appearance. Theapplication of craniofacial techniques inrecent years has greatly enhanced theresults now obtainable.

Maxillary Fractures

Twenty year old female with Lefort I maxillary fracture and malocclusion.

CT scan shows fractures of all maxillary buttresses.

Postoperative result after openreduction and rigid fixation.


The nasoethmoid region is an intricateconfluence of bones that makes up the com-plex anatomy of the central-upper midface.Identifying and understanding the structuralbuttresses are crucial to restoring the normalanatomy and appearance of the region. Bydefinition, the nasoethmoid fracture involvesdisruption of the inferior orbital rim, lateralnasal bones, medial orbital wall, and frontalprocess of the maxilla. Essentially the medialorbital rim buttress to which the medial can-thal tendon is attached is dislocated. It is theoperative reduction and stabilization of thiscentral segment of bone that is the key to suc-cessful treatment of this injury.

There are a number of potential pitfalls in

(A) Diagram illustrates a bilateral comminuted NOE fracture. (B) Closeup showing reduction of central bone frag-ments and stabilization with wires. Comminuted midline nasal bone fragments have been temporarily removed provid-ing exposure to nasal side of medial orbital wall fractures. Two circular transnasal wires are used: (1) one posterior andsuperior to the tendon insertion, and (2) one anterior and inferior to the tendon insertion. (C) Superior and inferiororbital rim fragments are reduced and stabilized with miniplates as illustrated. (D) Nasal bones are frequently recon-structed with a cantilever bone graft secured with lag screws or a miniplate.

the surgical treatment of NOE fractures.Once this injury has been diagnosed, theextent of the fracture is determined by physi-cal exam and the CT scan. A definitive earlyopen reduction with stabilization of the frac-tures utilizing the techniques of craniofacialsurgery is then accomplished.

The best results of the NOE fracture areobtained with early diagnosis and aggressivesurgical treatment. Complications resultwhen this injury has been misdiagnosed orinadequately treated. Unstable or displacedfractures that are left untreated result in per-manent deformities once healed. Late recon-struction is a difficult task that requiresrepositioning of both bone and soft tissue.

Nasoethmoid Fracture and Repair

A.

C. D.

B.


Incomplete reduction of the medi-al orbital rim fragment, which contains the canthal tendon, can result in a widening of the interor-bital distance or a telecanthus.Asymmetric repair, inadequatestabilization, and inadvertentstripping of the medial canthaltendon can all contribute to thisproblem. Inaccurate assessment ofthe nasal bones and septal supportcan lead to contour deformity ofthe nose with inadequate tip pro-jection. Close attention is neededfor reconstruction of the orbitalwall fracture to avoid the enlarge-ment of the orbital volume andresultant enophthalmos and verti-cal orbital dystopia. Although latereconstruction of these deformitiesis possible, in general, the best aes-thetic results are obtained withdefinitive repair at the time of injury, avoiding the commonpitfalls.

Nasoethmoid Fracture


(A) Fifty-four year old woman involvedin a motor vehicle accident sustainedextensive soft tissue injuries and commi-nated bilateral nasoethmoid fractures. (B) Fractures were reduced and stabilizedwith miniplates and transnasal wires. (C) Septal Support was destroyed andreconstruction with a cantilever calvarialbone graft was required. (D,E) Postoperative appearance afteracute one stage repair.

A.

C. D.

E.

B.


INTERNAL ORBITALFRACTURES

Trauma to the upper face fre-quently results in fractures of theinternal orbit. These may occur asisolated blowouts or associated withmore complex adjacent facial frac-tures. The range of potential injuriesto the internal orbit may vary from asmall orbital floor defect to destruc-tion of all four walls. Despite thispotential for wide variation of inter-nal orbital fractures, there are basicprinciples that can be applied to aidin diagnosis and treatment. The bonyorbits play a vital role in maintainingnormal function and aesthetics of theeyes. Accurate realignment oranatomic reconstruction of the bonyorbit is essential to maintain normalfunction and appearance.

A number of advances have beenmade in the past 10 to 15 years in thediagnosis and treatment of internalorbital fractures. The computerizedtomographic (CT) scan has dramati-cally enhanced the surgeonÕs abilityto diagnose and determine the extentof the internal orbital fractures.Three-dimensional scans may evenfurther enhance this radiographicevaluation. With the development ofthe field of craniofacial surgery, sur-gical techniques have also signifi-cantly improved in this area. Thesetechniques have allowed extendedexposure of the fractures, whichgreatly facilitates assessment andtreatment of orbital injuries. Betterexposure allows more precise reduc-tion and rigid stabilization of thefractures. The other major advance-ment in orbital surgery has been theuse of rigid fixation to provide a sta-ble internal orbital reconstruction.

Elderly man in motor vehicleaccident with telescoping in of nasoethmoid complex.

Postoperative result followingimmediate repair of fracturesand reconstruction of nose.

Twenty year old female withcomminuted nasoethmoidorbital fractures.

Postoperative result after openreduction and rigid fixation ofall fractures.



The use of mini- or microplates and metalmeshes in combination with bone grafts hasalso improved stabilization and enhanced thehealing of orbital fractures.

The recommended treatment of theseinjuries varies greatly in the literature withthe best method of reconstruction remainingcontroversial. Surgical treatment has rangedfrom packing of the maxillary antrum to totalorbital reconstruction with autogenous orsynthetic materials. Accurate anatomic recon-struction is essential to restore function andappearance of the eye.

Because most of the bone of the internalorbit is thin and weak, it is frequently difficultto reduce and adequately stabilize withoutthe use of autogenous or alloplastic materials.

Autogenous bone grafts have been the mate-rial most often used by craniomaxillofacial sur-geons for reconstruction of the internal orbit.Split calvarial bone has gained popularitybecause of its low rate of infection and decreasedresorption. Other autogenous materials thathave been used include iliac bone, split rib, andcartilage. Resorption may be a problem with theuse of autogenous bone. Each of these materialsalso requires a donor area, although donor sitemorbidity is usually very small.

A variety of alloplastic material such as sili-cone, teflon, tantalum mesh, polyethylene, andmethyl methacrylate have been used for orbitalreconstruction. The primary concern with theuse of alloplastic material has been the risk ofinfection with extrusion. The metal miniplatessystems have been very successful in the treat-

Internal Orbital Fractures

Titanium micromesh used to reconstruct internal orbit.

Preoperative CT Scan demonstratesright orbital blowout fracture in thirty-three year old patient that clinically exhibited enopthalmos.

Postoperative CT Scan shows recon-struction of orbital floor with metalmesh.

Postoperative three dimensional scanshows mesh extending into posteriorthird of orbit, reconstructing almostentire orbital floor.

Postoperative result with normalposition and function of eye.


ment of facial fractures. These tech-niques of rigid fixation using metalmeshes have also been applied success-fully to the treatment of internal orbitalfractures. In our experience of recon-structing over 300 internal orbital frac-tures with metal mesh, we have foundthis technique to be both safe and effec-tive with no infections as a result ofusing metal mesh.

The goal of surgical treatment ofblowout fractures is to replace orbitalcontents back into the bony orbit and torestore normal orbital volume andshape. The best method of reconstruc-tion of internal orbital fractures remainscontroversial. A number of techniqueshave been described using autogenousgrafts or alloplastic materials. We havehad good success with the use of metalmesh alone, without bone grafts, for thereconstruction in internal orbital defects.This micromesh is our preference whenalloplastic material is used. It is rigid yetmalleable and can provide excellentorbital support for large defects, particu-larly in the posterior aspect of the orbit.This is important, since accurateanatomic reconstruction of both orbitalshape and volume is crucial to obtain thebest possible results.

Significant advancements have beenmade in the evaluation and treatment ofinternal orbital fractures. CT has greatlyenhanced our ability to identify the exactlocation and extent of destruction ofinternal orbital fractures. Early opera-tive intervention combined with wide exposure, meticulous reduction, and rigid fixation has significantlyenhanced the treatment of internalorbital fractures.

Internal Orbital Fractures

This patient sustained blunt trauma to the face.

Three dimensional scan shows complex orbital fractures.

Postoperative result after extensiveopen reduction and rigid fixation.


MANDIBULARFRACTURES

The mandible is a unique bonehaving a complex role in both aes-thetics of the face and functionalocclusion. Because of the promi-nent position of the lower jaw,mandible fractures are the mostcommon fracture of the facialskeleton.

Fractures of the mandibleshould be classified according tothe site of the fracture, degree ofdisplacement, presence of com-minution and status of the denti-tion. Physical examination willtypically reveal a malocclusion,pain at fracture site, mucosalecchymosis or laceration withbleeding between teeth at the frac-ture site. Plain radiographs canhelp further define the fractures ofthe mandible. In a patient with anisolated jaw injury, a Panorex isthe ideal radiograph to visualizethe fracture and condition of theteeth. In the multisystem injuredpatient a computerized tomo-graphic scan with three dimen-sional reconstruction providesgood visualization of the frac-

Mandibular Fractures

Mandibular Fractures

Sixty-six year old female sustainingcomplex comminuted fractures to allbones of face.

This patient sustained severe com-plex fractures of all facial bones withloss of right eye.

Postoperative result after one stagerepair.

Postoperative result after one stagerepair.

Skull with mandibular plates. Rigid fixation of mandibular fractures shown onskull using titanium plates.


tures.

Reduction and stabilization of themandible fracture is the key to success-ful treatment. The method of manage-ment may vary based on the severity,location of the fracture and presence orabsence of teeth. Mandible fractures areusually treated by closed reductionwith wiring of the teeth or open reduc-tion with internal rigid fixation usingplates. Nonoperative management of amandible fracture with a soft diet israrely indicated. The technique ofclosed reduction involves wiring ofarch bars on the teeth and intermaxil-lary fixation for 4 to 6 weeks. Internalrigid fixation requires exposure of thefracture sites and stabilization withplates and/or screws. This techniquecan frequently avoid postoperativeintermaxillary fixation (wiring teethtogether) and the problems associatedwith this such as weight loss and jointstiffness.

Following the principles of accuratereduction with good stabilization canfrequently avoid complications andhelp to restore the patients primaryocclusion and facial appearance.

POST-TRAUMATICFACIAL DEFORMITIES

The best way to prevent post-trau-matic facial deformities is to obtain theappropriate treatment at the time ofthe injury. Applying the latest cranio-facial techniques can in many casesmake major secondary revisionsunnecessary. However, despite themany advances that have come fromthe field of craniofacial surgery, thereare still patients that end up with sig-nificant deformities. These are patientsthat for whatever reason are treated

This patient has longstanding post-traumatic right enophthalmos and ectropion.




Post-Traumatic Facial Deformities

Post-traumatic facial deformity with displaced nasoethmoid complex.


inadequately or because of life-threatening other injuries, do notreceive any treatment of theirfacial injuries.

Unrepaired or inadequatelyreduced facial fractures can resultin a wide range of severe cosmet-ic and functional deformities.Unrepaired fractures around theorbits can cause a sunken-inappearance of the eyes, nasaldeformities, as well as functionalproblems with vision (doublevision). Unrepaired injuries of themiddle and lower face can causecontour deformities, flattening ofthe cheeks, or malocclusion of theteeth with difficulty chewing.These are but a few of the manyproblems associated with post-traumatic facial deformities.

Late repair or reconstructionafter the soft tissue and boneshave healed is much more difficult than repair at the time of initial injury. Reconstructionrequires wide exposure whichenables the surgeon to have directvisualization of the bony defor-mities. Bone cuts are then made toreposition the displaced bone andreattach the soft tissue back intoits normal position. Missing or severely deformed bone mayrequire replacement with bonegrafts. These operative proce-dures all use the standard tech-niques of craniofacial surgery.

A seventeen year old patient with inadequately treated nasoethmoid orbital fractures.


Postoperative result one year after late reconstruction.

Preoperative

Post-Traumatic Facial Deformities


SOFT TISSUE DEFORMITIES

Soft tissue deformities may involvethe skin, subcutaneous tissue, underly-ing muscle or a combination of any ofthese elements. There are a number ofsyndromes such as hemifacial microso-mia in which asymmetry of the soft tis-sue may be a component. However, thenumber one cause of facial soft tissuedeformities is trauma. Traumatic facialinjuries may be blunt, penetratingand/or avulsive in nature. An avulsionor loss of soft tissue may create a sig-nificant deformity which requiresreconstruction. Generally the facialskeletal deformities are reconstructedfirst followed by correction of soft tis-sue problems.

One example of a severely deform-ing and psychologically cripplinginjury is the scalp avulsion. This pre-sents a very challenging problem par-ticularly if the avulsed portion is verylarge and cannot be replanted bymicrovascular technique. A new andinnovative approach to this type ofproblem has been the use of the tissueexpander. These expanders are muchlike deflated balloons placed beneathadjacent normal tissue. Over a periodof weeks these expanders are graduallyinflated, stretching the normal skin foruse in reconstruction. An example ofthis technique is shown for reconstruc-tion of the scalp.

Soft tissue tumors are another causeof facial deformities. Resection of eitherbenign or malignant facial tumors canresult in a significant cosmetic deformi-ty. Craniofacial techniques can be usedto help reconstruct these defects andrestore facial appearance.

Thirty-five year old male with softtissue injuries and complex facialfractures.

Postoperative result after one-stagereconstruction of soft tissue and bone.

Five year old boy was attacked by a pit bulldog and lost almost all of his scalp and forehead.

This injury was initially repairedwith skin grafts.

Using tissue expanders his entirehair-bearing scalp has been restored.

Soft Tissue Deformities


FACIAL BONE CONTOURING

The face is basically a soft tis-sue representation of the under-lying skeletal structure. Facialbone contouring is the surgicalchange in this bony support toproduce a more aesthetic appear-ance. It is not only desirable tocorrect a particular deformity,but also to improve the entire aesthetic facial harmony.Correction in contour of thefacial skeleton can be achievedby osteotomies or by augment-ing the skeleton with bone orother materials. After a maxillaryor mandibular repositioning,additional bone contouring isoften necessary to correct slightasymmetries. Augmentation ofsuch areas as the cheek, chin, ornose can be done in combinationwith osteotomies or alone.

Stereotypical appearancesassociated with particular dis-ease processes can often bemarkedly improved by bonycontouring. Patients with facialclefts commonly have a flat max-illary profile due to the deficien-cy of the facial skeleton over thefront of the maxilla and thelower bony rims of the orbits.Contouring with graft materialscan give a more normal appear-ance, thus removing some of thepsychological stigmas associatedwith such deformities.

In many cases, however,facial contouring is requested bypatients whose appearance fallswell within the normal range.These individuals seek surgeryto enhance their appearance, not

Seventeen year old female with facial imbalance and notching of upper lip secondary to bilateral cleft lip and palate.

Postoperative result after Abbe Flap (lip switch) upper lip reconstruction, advancement of maxilla, chin, and rhinoplasty.

Facial Contouring

to radically change it. Cheek augmentation is a goodexample. The aesthetic refinements of bony contouringused in combination with osteotomies when necessaryallow the craniofacial surgeon to obtain the optimumaesthetic results.


The term hemifacial microsomia(HFM) is used to describe a spec-trum of complex anomalies charac-terized by underdevelopment ofone side of the face. A variety ofterms have been used to identifythese facial deformities that resultfrom a developmental malforma-tion of the first and secondbranchial arches. Such names ascraniofacial microsomia, lateralfacial dysplasia, first and secondbranchial arch syndrome, and oculoauriculovertebral dysplasia(Goldenhar’s syndrome) have beenused to describe this syndrome.

The incidence of HFM is in therange of 1 in 3500 to 4500. The causeof this deformity has not been satisfactorily identified to date.Transmission is usually sporadic,however, reports do exist of the syndrome being inherited. HFM may occur by itself or in asso-ciation with other anomalies such as Goldenhar’s syndrome (oculoauriculovertebral dysplasia).Goldenhar’s includes, in additionto a HFM, a bony malformation ofthe spine and superficial eye lesionscalled epibulbar dermoids.

The collected group of deformi-ties that make up this syndromemay vary greatly in extent anddegree covering a wide spectrumranging from mild underdevelop-ment of the lower jaw to severedeformity of the skull and face.Characteristically this deformity, asthe name implies (hemifacial),involves one side of the face, how-ever involvement of both sides to

Young girl with a left hemifacialmicrsomia. Microsomia resulted in airway obstruction requiringtracheostomy.

Postoperative result aftermandibular lengthening.Tracheostomy was able to beremoved due to improved positionof mandible.


Hemifacial Microsomia

HEMIFACIAL MICROSOMIA


some degree can be observed in ashigh as 15% of all cases. The mostobvious deformity involves thelower jaw and ear, but soft tissuedeficiency, maxillary hypoplasiaand even orbit and skull anomaliesmay also be present. Lack of devel-opment of the external ear is a com-mon feature with the severity of theear deformity proportional to thejaw deformity.

These complex problems requirea thorough evaluation by the cran-iofacial team. When we see thesepatients as infants they are classi-fied according to the severity of themandibular deformity. Subsequenttreatment is individualized beingbased on this classification and theirage at time of evaluation. The goalsof treatment are to re-establishfacial symmetry (bony and soft tis-sue), normal occlusion, and hope-fully normal joint function. There issome controversy concerning theage in which surgical interventionshould be started, however we rec-ommend early surgical correction.In patients born with absent struc-tures, reconstruction of the jaw jointis begun early (before age 6).Children with mild deformitiesmay benefit from the use of anorthodontic device (activator) thatattempts to stimulate mandibulargrowth.

Soft tissue augmentation is per-formed when necessary after bonyskeletal symmetry has beenobtained. Ear construction is usual-ly performed at 5-6 years of age.However, in cases of HFM withmarked asymmetry of the lowerface, ear construction is delayed

Twenty year old woman with lefthemifacial microsomia.

Result after surgical correction of jaw and chin deformity andaugmentation of cheek.

Hemifacial Microsomia

until bony symmetry is obtained so that the appropriateposition of the new ear can be determined.

It is important to remember that HFM is a progressivebony and soft tissue deformity with the earliest and mostconsistent abnormalities noted in the mandible. In gener-al, we recommend early surgical intervention in anattempt to maximize normal growth forces, minimizesecondary deformities, and give the patient the psycho-logical benefits that result from early improved appear-ance and self esteem.

Möbius syndrome or congenital facialparalysis is a rare deformity. The exact fre-quency of occurrence is not known, with onlyseveral hundred cases being reported in theliterature. This type of facial paralysis hasbeen classically described as bilateral, com-plete, and permanent. However, the paralysisoccasionally may be asymmetric, in which theupper face is effected and the lower lip move-ment spared. Although uncommon, this syn-drome is very distinct.

Bilateral congenital facial paralysis hastraditionally been associated with Paul JuliusMöbius. In 1888, Möbius described a clinicalentity involving bilateral paralysis of thesixth and seventh cranial nerves. He was thefirst to single out these particular palsies as aseparate entity. Hence, the hallmark clinicalfeatures of Möbius syndrome are paralysis ofthe sixth and seventh cranial nerves. The sev-enth cranial nerve or facial nerve innervatesthe muscles of facial expression. Paralysis ofthis nerve results in the mask-like facies andthe inability to smile. The paralysis of thesixth cranial nerve results in the inability toabduct the eyes or simply to look to the sideby just moving the eyes. Because of thisinability to move the eyes laterally, the childfollows objects by turning his head from sideto side. Patients with Möbius syndrome mayalso have additional problems. The paralysismay involve other cranial nerves such as thenerve that innervates the tongue. Limb anom-alies have been reported in 25% of cases withthe most common problem being the clubfoot. Additional problems can be lack ofdevelopment of chest musculature such asthe pectoral muscles which was present in15% of the reported cases.

The etiology of this syndrome remainsunclear. One hypothesis is that nuclear

hypoplasia or agenesis of the sixth and seventhfacial nerves causes the anomaly. Anotherhypothesis is primary aplasia or lack of devel-opment of the muscles leading to atrophy ofthe nerve. Also in doubt is the role that heredi-ty plays. While many feel heredity plays norole, there have been reports of a small numberof cases occurring in families indicating a pos-sible hereditary tendency.

The diagnosis of Möbius syndrome is notalways made in the newborn nursery and isoften not recognized as Möbius syndrome forseveral months. The parents usually show themost concern over the mask-like facies which isdue to the paralysis of the facial nerve. Thesixth nerve paralysis becomes obvious whenthe child follows objects by turning his headfrom side to side. The child may experience eat-ing problems when food becomes lodged in thecheeks requiring manual removal. However, inthe congenital form of facial paralysis, func-tional problems are often not as significant asmight be expected. Corneal protection which isrelated to the ability to close the eyes is usuallyadequate with problems related to cornealexposure uncommon. It is the lack of facialexpression or inability to smile that poses themajor problem. This can severely inhibit psy-chosocial development as the child approachesschool age and thus becomes aware that theyare different. We feel this obstacle to interper-sonal relationships and social developmentshould play a role in the timing of surgicalintervention.

The timing of recommended surgical cor-rection is of course influenced by the age of thepatient at referral. Ideally, one would want toevaluate Möbius syndrome patients as infants.Due to the psychosocial problems that canresult from bilateral facial paralysis, we recom-mend early surgical intervention if possible. It


MÖBIUS SYNDROME:CONGENITAL FACIAL PARALYSIS

is preferable to have the child’s reconstruc-tion complete before he starts school. Thiswould mean starting the reconstruction atfour to five years of age. However, the ageof the patient does not effect the operativeoutcome; successful reconstruction can beperformed in adults as well.

The treatment of patients with Möbiussyndrome is directed toward restoration offacial motion. Acquired facial paralysis dif-fers from the congenital type found inMöbius syndrome, and its treatment willnot be discussed in this article. In the con-genital cases, corneal exposure is seldom aproblem and therefore does not require sur-gical treatment. However, in acquired caseseye problems are frequent and generallyrequire surgical intervention. In bilateralcongenital facial paralysis the major goal isto restore motion in the appropriate direc-tion to the upper lip and commissure. Inunilateral cases, a nerve graft from the func-tioning facial nerve to the effected side canbe performed. However, in bilateral casessuch as Möbius syndrome, there is no func-tioning facial nerve available. The surgicaloptions in these cases are a local muscletransfer procedure or free muscle transplan-tation. Local regional muscles such as thetemporalis or masseter can be used torestore facial movement. Either of thesemuscles of mastication can be moved main-taining their innervation to restore move-ment of the upper lip and commissure.Retraining and practice is necessary to getthe maximum benefit.

A newer procedure is microvascularmuscle transplantation. This involves mov-

ing a muscle from another part of the bodyalong with its nerve and blood supply. Thenerve and vessels to the muscle are recon-nected with the aid of a microscope. Themuscle is oriented in such a fashion to pro-vide motion to the upper lip and commissure.

In each of these procedures the familyneeds to have realistic expectations of whatcan be accomplished. If successful, these pro-cedures can provide tone to the upper lip andcommissure at rest, with muscle action thatcan produce a smile. Fine movements or vari-ation in expression are usually not possible.However, these procedures can make dra-matic improvements in the mask-like facies ofMöbius syndrome. They provide the poten-tial benefits of facial animation withimproved social interaction.



SUMMARY

CRANIOFACIAL SURGERY encompasses the reconstruction ofa broad spectrum of facial deformities. This field is in itsinfancy and continues to develop and grow rapidly. Thefunctions of a craniofacial center have been described and avariety of examples used to demonstrate the type of recon-structive procedures routinely performed at our center. Thisbook is not meant to be comprehensive in describing themultitude of craniofacial deformities that occur or every sur-gical option available. We have described the more commondeformities and syndromes with our current approach totreatment. However, it is important to remember that treat-ment plans vary and must be individualized for each patientdepending on the type and severity of the particular defor-mities present. We feel this customized approach is a crucialstep in obtaining the best results possible.

Such centers offer great potential and hope to manydeformed patients. We feel that providing informative mate-rial about the treatment of these disorders results in betterpatient education and ultimately better care. We hope thisbook has answered many of the questions asked by bothhealth professionals and parents concerning the evaluationand treatment of craniofacial deformities.


T. C. THOMPSON CHILDREN’S HOSPITAL

T. C. Thompson Children’s Hospital is a full service teaching hospi-tal affiliated with the University of Tennessee College of Medicine. Itprovides the most comprehensive pediatric care available, featuring thestate’s only Level I Pediatric Trauma Center, one of five PerinatalCenters of Excellence, and a Level III Neonatal Intensive Care Unit.State of the art facilities are available to meet the special needs of chil-dren. Additional subspecialties include cardiology, neurophysiology,genetics, physical therapy, allergy/immunology, endocrinology, hema-tology, oncology, infectious disease, neonatology, neurology, nephrolo-gy, gastroenterology, pulmonology, pediatric intensive care, pediatricsurgery, and pediatric anesthesiology.

CRANIOFACIALFOUNDATION OF AMERICA

The Craniofacial Foundation of America (CFA) is a non-profit orga-nization dedicated to helping people with facial deformities lead nor-mal lives. Established in 1989, the CFA provides support for theTennessee Craniofacial Center.

The foundation provides financial assistance for food, travel, andlodging expenses to qualified families traveling to the TennesseeCraniofacial Center for evaluation and treatment. It also provides net-working opportunities for craniofacial patients and their families forthe rights of those with facial disfigurement, and as a source for educa-tional material on craniofacial anomalies.

For information, call (423)778-9192 or (800)418-3223


ABOUT THE ERLANGER HEALTH SYSTEM

Erlanger Health System is owned and operated by theChattanooga-Hamilton County Hospital Authority and governed byits 11-person Board of Trustees. Today, Erlanger is the only compre-hensive health system in the region, recognized as providing the high-est quality of care with a commitment to the communities it serves.

Erlanger features:

• Erlanger Medical Center, the oldest, largest and most compre-hensive hospital in the region.

• T. C. Thompson Children’s Hospital, the only healthcare facilitydedicated to complete care for children in the region.

• Erlanger North, a 57-bed community hospital located in RedBank; and Erlanger East, a state-of-the-art ambulatory care center.

• The James L. Fowle Regional Cancer Center.• The Regional Kidney Transplant Center, the region’s only organ

transplant program.• The Regional Trauma Center, the region’s only Level I

trauma center.• LIFE FORCE, the region’s only air ambulance program.• The Willie E. Miller Eye Center, the first hospital in Tennessee

dedicated solely to eye care.• The Regional Heart Center, site of the first open-heart surgery

ever performed in Chattanooga.• The region’s only Level III Neonatal Intensive Care Unit.• The Chattanooga Unit of the University of Tennessee College of

Medicine, training the next generation of physicians.• The Erlanger Community Partnership, a philanthropic

program aimed at meeting needs in the community served by Erlanger Health System.

• Membership in the Galaxy Health Alliance, a partnership of hospitals aimed at reducing healthcare costs.

• LifeForce Health Plans, an affiliate of Cariten Healthcare, aprovider-owned insurance company.

For answers to health questions or for help in locating an Erlangerphysician, call The Professionals, 24 hours a day, at 778-8181 or 1-800-333-8181.

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