what’s new in limb-lengthening and deformity...

Specialty Update

What’s New in Limb-Lengtheningand Deformity Correction

Sanjeev Sabharwal, MD, MPH, Kevin W. Louie, MD, and J. Spence Reid, MD

This update summarizes select articles pertaining to limb-lengthening and deformity correction that were publishedfrom January 1, 2013, to December 31, 2013.

Pediatric Lower-Extremity DisordersLimb Alignment and Guided GrowthThe optimal timing for performing growth modulation and thechoice of implants continues to be investigated. Lee et al.compared five different methods used to calculate the exacttiming of epiphysiodesis and found that, following percuta-neous epiphysiodesis in forty-four children, all techniques gavean overcorrected value of limb-length discrepancy comparedwith what was noted at skeletal maturity1. In another retro-spective series of thirty-nine children with limb-length dis-crepancy, no difference in efficacy was noted among the use ofstaples, tension-band plates, and transphyseal screws2. In arandomized clinical trial of twenty-six children with idiopathicgenu valgum, stapling and tension-band hemiepiphysiodesiswere equally effective in realigning the limb3. In the earlypostoperative period following percutaneous permanentepiphysiodesis, volume computed tomography (CT) scanningwith radiostereometric analysis may be used to confirm theanticipated physeal ablation4,5. Using this imaging technology6

and clinical evaluation7, authors have questioned the efficacy oftension-band plates in the proximal part of the tibia as aneffective means of correcting limb-length discrepancy.

Several authors have reported on the efficacy of using lessexpensive implants (3.5-mm reconstruction plates and one-third tubular plates with noncannulated screws) as a viablealternative to currently available tension-band constructs for

achieving temporary hemiepiphysiodesis around the knee8-11.Guided growth is also effective in managing lower-extremityangular deformities in children with certain skeletal dysplasias12

and in pediatric amputees13. However, caution should be ex-ercised when using tension-band plates in children with smallepiphyses or osteopenic bone, as the screw may get drawn intothe physis and cause an iatrogenic growth arrest14. In additionto being used for correction of angular deformities in thefrontal plane, guided growth can be used for correcting sagittalplane15, and perhaps rotational, deformities. On the basis oftheir work in a rabbit model, Arami et al. demonstrated thepotential feasibility of altering axial rotational growth by usingobliquely placed plates across the distal femoral physis16.

Obesity-Related IssuesIn general, genu valgum, primarily related to distal femoraldeformity, is the predominant lower-limb malalignment inobese adolescents17. Compared with their peers, children withBlount disease have advanced skeletal maturity18. The use of acircular external fixator with a proximal tibial osteotomy re-mains an effective method to achieve limb realignment inmorbidly obese adolescents19. In another study, patients tend-ed to gain weight during extended periods of externalfixation20.

Congenital Limb DeficienciesAt a mean follow-up of 21.5 years, patients who had undergonea Van Nes rotationplasty for the correction of congenitalfemoral deficiency functioned at a high level despite gaitdeviations21. Koczewski et al. reported on nine patients withlower-limb hypoplasia involving deficiencies of the interme-diate foot rays22. Predicted limb-length discrepancy at skeletalmaturity and the number of foot rays involved may influence

Specialty Update has been developed in collaboration with the Board ofSpecialty Societies (BOS) of the American Academy of Orthopaedic Surgeons.

Disclosure: None of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of anyaspect of this work. One or more of the authors, or his or her institution, has had a financial relationship, in the thirty-six months prior to submission of thiswork, with an entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. No author hashad any other relationships, or has engaged in any other activities, that could be perceived to influence or have the potential to influence what is written inthis work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.

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COPYRIGHT ! 2014 BY THE JOURNAL OF BONE AND JOINT SURGERY, INCORPORATED

J Bone Joint Surg Am. 2014;96:1399-406 d http://dx.doi.org/10.2106/JBJS.N.00369

the method of limb reconstruction in children with congenitalfibular deficiency23.

Congenital Pseudarthrosis of the TibiaThe management of congenital pseudarthrosis of the tibia re-mains challenging24. Prospective clinical trials may help todiscern the role of various surgical and pharmacologic ap-proaches for congenital pseudarthrosis of the tibia25. The use ofcircular fixation and intramedullary nailing remain the pri-mary treatment modalities for this disease26,27. Spontaneoushealing of the persistent nonunion without compression wasnoted during proximal tibial lengthening over the retainedintramedullary nail in two children with congenital pseudar-throsis of the tibia28. The two-stage induced membrane tech-nique appears to be promising29.

Hip DisordersA mean shortening of 19 mm (range, 10 to 38 mm) at skeletalmaturity was noted in ninety-three (55%) of 168 patients withLegg-Calve Perthes disease, with advanced lateral pillar stagebeing the strongest predictor for future limb-length discrep-ancy30. Varus osteotomy was not associated with residualshortening of the affected extremity. The evolution andtechnical considerations of the modified pelvic support os-teotomy in pediatric patients was recently described by Choiet al.31.

Surgical Techniques and OutcomesIn a retrospective review of 125 children undergoing supra-malleolar derotational osteotomies, a complication rate of12.8% was noted with conventional plate fixation comparedwith 2.4% with cross-pinning32. The use of locking plates forstabilizing long-bone osteotomies following acute correction33

and gradual correction34 is gaining popularity. While femorallengthening over an intramedullary nail decreases the externalfixation time, Gordon et al. noted a 38% complication rate(including failure to lengthen, fractures, implant failures,subluxation of the hip and knee joints, and deep infections) in aseries of thirty-seven children35. Launay et al. reported twentyfractures (18%) following lower-limb lengthening of a total of111 limb segments in fifty-eight children. Risk factors includedcongenital etiology and less than seven days of latency periodfollowing the osteotomy36. Martin et al. described a noveltechnique of performing quadricepsplasty for managing ex-tension contracture of the knee following femorallengthening37.

OsteomyelitisDespite residual limb shortening, sequestrectomy followed bynonvascularized fibular grafting can effectively address gapnonunion of long bones secondary to osteomyelitis in chil-dren38. With some additional risks, the Ilizarov technique canallow comprehensive management of pseudarthrosis followinghematogenous osteomyelitis of the tibia39.

Pediatric TraumaZenios40 reported that, despite the prolonged use of an externalfixator, twelve skeletally immature patients with an unstabletibial fracture (eight closed, four open) had satisfactory healingand alignment with use of the Taylor Spatial Frame (Smith &Nephew, Memphis, Tennessee), with one patient developing acompartment syndrome postoperatively.

On the basis of a mean follow-up of twenty-one (range,sixteen to twenty-eight) years for individuals with a pediatricfemoral fracture, Palmu et al. reported a positive correlationbetween residual angular deformity and premature arthritis ofthe knee joint41. In a retrospective review of pediatric femoralshaft fractures treated with flexible intramedullary nailing, Parket al. reported that a low nail-canal diameter ratio may beassociated with femoral overgrowth42. The development ofpremature growth arrest following intra-articular physealfractures of the medial malleolus correlated with the magnitudeof initial displacement and a delay in surgical intervention43.

Posttraumatic Bone Defects in AdultsThe clinical outcomes, including the cost associated withcomplex posttraumatic limb reconstruction, have been re-ported44-46. Lowenberg et al.47 reviewed thirty-four patients whohad a mean tibial bone defect size of 8.7 cm and soft-tissuedefects and who were referred for a second opinion prior toamputation. These lower limbs were successfully salvaged withflap reconstruction and bone transport. The mean duration ofbone transport was 10.8 months, and one flap failed. Despite a29% reoperation rate, all gaps had healed, without recurrentinfection, at a mean follow-up time of eleven years and themajority had satisfactory functional outcomes. The averagelifetime cost per patient per year was appreciably less than thepublished cost for amputation. Two other studies48,49 reportedsatisfactory subjective outcome scores for this type of recon-struction for tibial bone defects.

Two groups have reported on the use of the Taylor SpatialFrame to simultaneously address the problems of bone andsoft-tissue loss in high-energy open tibial fractures. The basictechnique involves the intentional creation of a shortening andangulation deformity at the fracture site, which allows the soft-tissue envelope to be closed primarily and avoid the need for alocal or free tissue transfer. After healing of the soft tissue, butprior to bone-healing, the deformity is gradually corrected andthen lengthening or bone transport is employed to treat theremaining bone defect50. The Direct Scheduler module of theTaylor Spatial Frame software is designed to facilitate this typeof treatment without the need for frame mounting parame-ters51. Mora et al.52 reported on 120 infected tibial nonunionswith combined bone and soft-tissue loss, which were treatedwithout plastic surgical tissue transfer and with use of the soft-tissue recruitment associated with bone transport (6 to 18 cm)alone (epidermato-fascial osteoplasty). Karargyris et al.53, intheir series of seven patients, reported acceptable outcomeswith use of a similar technique combining bone transport,

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VO LU M E 96-A d NU M B E R 16 d AU G U S T 20, 2014WH AT ’ S NE W I N LI M B -LE N G T H E N I N G A N D DE F O R M I T Y CO R R E C T I O N

What’s New in Limb-Lengthening and Deformity Correction

Papineau grafting, and negative-pressure wound care withoutformal plastic surgical coverage. These demanding techniquescan provide a solution in otherwise unsalvageable situations.

The problem of achieving a successful knee arthrodesiswith a large bone defect following debridement of an infectedperiarticular fracture was addressed in four patients54. Thetechnique involved debridement and soft-tissue coverage fol-lowed by bone transport (6 to 10 cm) with a circular fixator.The authors recommend a ‘‘peg in socket’’ docking techniqueto increase mechanical stability.

The prolonged external fixation time associated with theconsolidation phase of either lengthening55 or bone trans-port56,57 can be decreased substantially with use of a techniquethat incorporates a locking plate and unilateral monorailtransport. This allows the external frame to be removed at thetime of either docking or completion of lengthening. The re-generate bone is protected by the locked implant. With use ofthis technique in a series of ten segmental posttraumatic tibialdefects (3.8 to 9.3 cm), Oh et al.57 were able to substantiallyreduce the external fixation index (average 13.4 days/cm) withexcellent healing of the docking site and consolidation of thelengthening regenerate.

Upper-Extremity ReconstructionSeveral studies have highlighted the utility of advanced tech-niques of external fixation for a variety of challenging upper-extremity pathologic conditions. Bumbasirevic et al.58 reportedon their retrospective series of fifteen patients (average age,twenty-four years) with scaphoid nonunions, which weretreated with the Ilizarov technique without bone graft. Thetreatment consisted of three stages: distraction, compression,and immobilization. Healing was achieved in all patients withan improvement in Mayo wrist score from 21 to 86.

Osteotomy at the level of congenital radioulnar synos-tosis with gradual correction by means of circular externalfixation was used to improve forearm position in four adoles-cents with severe pronation deformities (mean pronationangle, 100")59. Two patients sustained temporary radial neur-apraxia following attempts at partial acute correction intra-operatively. Hosny and Kandel60 reported on five patients(mean age, twenty-one years) with posttraumatic radial club-hand (mean shortening of radius, 4.2 cm) treated with dis-traction lengthening of the radius with use of a circular fixator.At an average follow-up of twenty-five months, all patientshealed without supplemental bone-grafting and had improvedhand function.

A hybrid monolateral fixator restored length and align-ment in a series of forty humeri (twenty-three patients)61. Themean age at surgery was fourteen years (range, ten to twenty-two years). The average duration of external fixation was 8.3months with a mean lengthening of 8.8 cm and a healing indexof twenty-eight days/cm. There were three refractures and twocases of varus angulation. Five elbows developed a flexioncontracture.

Sewell et al.62 reported on distraction osteogenesis for thetreatment of congenital clavicular hypoplasia in five patients(seven clavicles), with a mean age of fifteen years. The averagelength gained was 31 mm (24.7% of original bone length), andthe mean time in the monolateral fixator was 174 days with anexternal fixation index of fifty-six days/cm. Two patients re-quired internal fixation following fixator removal. The Oxfordshoulder scores improved from 28.5 to 41.

Lower-Limb Reconstruction in AdultsA closing-wedge high tibial osteotomy results in negligiblechanges in limb length, whereas an opening-wedge high tibialosteotomy can increase limb length, especially in patients whorequire a large angular correction63. A ‘‘safe zone’’ has beenidentified through which a medial opening-wedge high tibialosteotomy should be directed to minimize the possibility offracturing the lateral cortex64. Medial opening-wedge hightibial osteotomy may increase patellofemoral contact pres-sure65. Compared with manual techniques, computer-assistednavigation was reported to be more accurate and reproduciblewhen high tibial osteotomy surgery is being performed66. Smalldegrees of valgus malalignment may be treated successfullywith a lateral opening-wedge high tibial osteotomy67.

A systematic review and meta-analysis suggests that totalknee arthroplasty following high tibial osteotomy provides anoutcome similar to that associated with total knee arthroplastydone in patients without a prior osteotomy68. The clinicaloutcomes (limb alignment, complications, or patient-reportedresults) were similar in patients undergoing total knee ar-throplasty following an opening-wedge or closing-wedge hightibial osteotomy69. A proximal tibial osteotomy distal to thetibial tubercle does not affect the posterior tibial slope or pa-tellar height70.The use of patient-specific guides for total kneearthroplasty resulted in success in obtaining neutral mechan-ical axes in the majority of patients71. Kinematically alignedtotal knee arthroplasty restores function without catastrophicfailure, regardless of alignment72. Compared with conventionaltechniques, robotic-assisted total knee arthroplasty improvesflexion-extension gap balance and alignment73.

The LLRS AIM index (a mnemonic indicating sevenpretreatment domains: Location and number of deformities,Leg-length inequality at maturity, Risk factors, Soft-tissuecoverage, Angular deformity, Infection/bone quality, andMotion/stability of the joints above and below) of the LimbLengthening and Reconstruction Society is a reliable classifi-cation system for lower-extremity deformities74. The domainsare rated on a 0 to 4 scale of increasing intensity, and the scoresare combined into a single index of complexity ranging fromnormal to high. Identifying and treating lower-limb malrota-tion is effective in addressing patellofemoral pain and insta-bility in adolescents and young adults75. The fusion andcomplication rates associated with use of a multiplanar, multi-axis external fixator for knee fusions are comparable with thoseof other methodologies76.

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Foot and AnkleExternal fixation has continued to be useful in arthrodesis,deformity correction, and arthroplasty of various segments ofthe foot and ankle. Subtalar distraction osteogenesis with ex-ternal fixation allows restoration of calcaneal height that isoften lost when performing subtraction subtalar arthrodesis forposttraumatic arthritis following intra-articular calcanealfractures77. The Ilizarov fixator can facilitate healing in patientsundergoing a Pirogoff amputation with ankle disarticulationand tibiocalcaneal fusion78. Ankle arthrodesis in patients withinfection, deformity, compromised soft tissues, and bone losscan be successfully achieved with a circular external fixator79.Cavovarus deformities may have concomitant problems suchas torsional malalignment and leg-length discrepancies that canbe addressed successfully with external fixation80. The Ortho-SUV Frame (OSF; Ortho-SUV Ltd, St. Petersburg, Russia) is aversatile tool in correcting forefoot and hindfoot deformities81.

Joint-preserving realignment surgery may be applicablefor patients with asymmetric valgus ankle osteoarthritis82. Asupramalleolar and lateralizing calcaneal osteotomy providesimilar redistribution of elevated ankle joint contact forces forfixed cavovarus foot deformities83.

Temporary intraoperative ankle distraction can improvesurgical exposure of lateral osteochondral lesions of the talus84.Patients with osteochondral defects of the talus with concomi-tant osteoarthritis may be amenable to twelve weeks of ankledistraction and allograft resurfacing85. Arthrodiastasis with mi-crofracture shows promising results for osteochondral lesions ofthe tarsal navicular in young athletes86. Use of a customized footplate in patients undergoing lower-extremity limb salvage al-lowed patients to engage in advanced weight-bearing activities87.

Benign and Malignant TumorsIntramedullary fixation without use of supplemental bisphos-phonates was effective in treating acute and impending long-bone fractures in children with fibrous dysplasia88. Li et al.reported satisfactory healing and alignment in twenty-onepatients with fibrous dysplasia following a valgus proximalfemoral osteotomy with dynamic hip-screw fixation89.

Despite its association with a prolonged duration of ex-ternal fixation, distraction osteogenesis remains a viable bio-logic solution for addressing long-bone defects followingoncologic resection90,91. However, the use of perioperative cy-totoxic agents may delay bone-healing92. Epiphysis-preservingresection of malignant tumors of the proximal part of the tibiaalong with reconstruction that makes use of fibular autograftsand circular external fixation can preserve the native knee jointin children but may require a protracted postoperative course93.Alternatively, expandable prostheses can be utilized, althoughan implant-related complication rate of 51% was reported inthirty-two children with sarcoma of the femur94.

A computer-assisted navigation system can facilitateguided multiplanar osteotomies during resection of a well-circumscribed chondrosarcoma about the knee95. Tibial corti-

cal strut autograft interposition arthrodesis following resectionof distal radial tumors resulted in acceptable function with lowdonor-site morbidity in seventeen patients96.

Postoperative Complications and Their ManagementPin-Track InfectionIn an effort to improve the pin-bone interface duringexternal fixation, Toksvig-Larsen et al.97 studied the use ofbisphosphonate-coated stainless steel pins as compared withhydroxyapatite-coated pins and uncoated pins in adult patientsundergoing proximal tibial correction with hemicallotasis forthe treatment of medial compartment arthritis. They notedthat, in the metaphysis, the bisphosphonate-coated pinsyielded a removal torque that was similar to that associatedwith the hydroxyapatite-coated pins. In an animal model,Koseki et al.98 determined that the photocatalytic bactericidaleffect of titanium-dioxide-coated pins (as compared with thatof uncoated pins) was more effective in decreasing infection.Other potential strategies for decreasing pin-track infectioninclude the use of titanium-copper alloys and the application ofa coating of nanosilver, nitric oxide, chitosan, chlorhexidine,iodine, hydroxyapatite, and certain antibiotics99. When selectedmembers of the Limb Lengthening and Reconstruction Societywere surveyed, most respondents (81%) preferred the use ofhydroxyapatite-coated half pins100.

Lethaby et al.101 performed a meta-analysis with eleventrials (including 572 patients) to evaluate the efficacy of variouspin-site care regimens. A variety of topical solutions (salinesolution, alcohol, povidone iodine, hydrogen peroxide), ap-plication frequencies (daily, weekly), and techniques (sterile,nonsterile) were compared. The authors noted that due to theheterogeneity, confounding variables, and poor quality of evi-dence, they were unable to identify a pin-care strategy thatclearly minimized infection. In another study, the effect ofretention or removal of the crust of dried exudate that forms atthe pin-skin interface of transfixation wires was investigated102.Retention of the dried crust reduced the infection rate (36%compared with 61%), but rendered subsequently infected pinsites more refractory to treatment.

MiscellaneousThe possibility of beginning early functional weight-bearingphysical therapy during posttraumatic limb-salvage treatmentwith use of an ankle-spanning circular external fixator with acustom foot plate was reported by Blair et al.87.

Prophylactic intramedullary fixation with use of one ortwo titanium elastic nails following femoral lengthening withexternal fixation minimizes secondary interventions should thelengthening regenerate fracture103.

New Tools and TechniquesEnhancing Bone-HealingOur understanding of bone-healing continues to advance. In astudy of the Masquelet technique, the osteogenesis-improving

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capabilities of the induced membrane decreased over time andthe authors suggested that the optimal time for the secondstage may be within one month following implantation of themembrane-inducing foreign material104. The use of teriparatidein the treatment of nonunions shows early promise and needsfurther investigation in human subjects105. In an animal study, thecombination of bone morphogenetic protein (BMP) andbisphosphonate as an adjunct to autograft was superior to au-tograft with BMP or autograft alone106. A quantitative assessmentof the yield of osteoblastic progenitor cells in bone-marrow as-pirate from the iliac crest, tibia, and calcaneus revealed that theiliac crest had a higher concentration than the other two sites107.In another study, the concentration and yield of osteoprogenitorcells was greater in aspirates obtained from the posterior iliaccrest as compared with the concentration obtained from theanterior iliac crest108. Optimal management of the docking site atthe conclusion of bone transport continues to be controversial109.Sala et al. noted that a minimally invasive endoscopic procedureat the docking site was a safe and viable technique, with unionrates similar to conventional open techniques, in nine patientsundergoing bone transport110.

Surgical Implants and Perioperative TechniquesExternal fixators used for deformity correction continue toevolve. When compared with the Ilizarov fixator, the SmartCorrection device (Response Ortho, Edgewater, New Jersey)was more accurate in correcting deformities, especially multi-planar deformities111.

Twenty-nine humeral shaft fractures treated surgically withexternal fixator-assisted reduction and minimally invasive plateosteosynthesis resulted in excellent reductions and high union rates,although there were two cases of transient radial nerve palsy112.

There are several imaging options for assessing patientswith limb-length discrepancy and angular deformities. Astanding-upright assessment of individuals with limb-lengthdiscrepancy with use of EOS (EOS Imaging, Paris, France)low-dose radiography was more accurate than CT scanogramsand conventional radiographs, with the added benefit ofsignificantly lower radiation exposure113. Compared withthe intraoperative navigation system, the Dugdale methodfor preoperative planning for opening-wedge high tibialosteotomy can theoretically undercorrect the angularcorrection114. Imaging software using picture archiving andcommunication system (PACS)-enhanced software methodsreliably enable direct measurement of osteotomy gaps inhigh tibial osteotomy surgery115. A novel locked-plate fixationdevice allows full weight-bearing following opening-wedge

osteotomy for varus gonarthrosis116. Malrotation errors duringinsertion of femoral intramedullary nails can be minimizedwith intraoperative measurement of femoral antetorsion withuse of the anterior cortical angle method available withsmartphones117.

Novel computer-assisted preoperative guides and surgi-cal instrumentation seem promising. A custom simulation-based surgical template facilitated the intraoperative executionof a three-dimensional corrective osteotomy for thirty patientswith posttraumatic cubitus varus deformities118. Individualizedguides were more precise, faster, and easier to use comparedwith navigated distal radial osteotomies in a laboratory ex-periment119. Virtual three-dimensional planning and patient-specific osteotomy guides for deformities about the knee wereuseful in single, double, and triple-plane osteotomies120. Acomputer-aided design and custom-made guide in conjunc-tion with a custom-made intramedullary nail may be useful incorrective osteotomy for complex femoral deformities121. In ananimal study, the use of a piezoelectric bone-cutting instru-ment enhanced bone-healing compared with conventionalsaws122. A case of 14 cm of lower-limb shortening as a latesequela of childhood meningococcemia was corrected with useof a fully implantable nail123. With the increasing availability ofvarious fully implantable intramedullary lengthening nails, amore in-depth analysis will be required to assess their efficacy,safety, and cost compared with more conventional surgicaldevices and techniques.

Upcoming EventsSpecialty Day of the LLRS (Limb Lengthening and Recon-struction Society) will be held at the Annual Meeting of theAmerican Association of Orthopaedic Surgeons (AAOS) onMarch 28, 2015, in Las Vegas, Nevada. The combined meetingof the LLRS and the International LLRS is planned for No-vember 4 through 7, 2015, in Miami, Florida. Details areavailable at the LLRS web site: www.llrs.org.

Sanjeev Sabharwal, MD, MPHKevin W. Louie, MDJ. Spence Reid, MDDepartment of Orthopedics,Rutgers-New Jersey Medical School,90 Bergen Street, Doctor’s Office Center,Suite 7300, Newark, NJ 07103.E-mail address for S. Sabharwal: [email protected]

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