case report anesthetic management in a gravida with type...

Case ReportAnesthetic Management in a Gravida withType IV Osteogenesis Imperfecta

Elizabeth Vue Juan Davila and Tracey Straker

Montefiore Medical Center 111 East 210th Street The Bronx NY 10467 USA

Correspondence should be addressed to Elizabeth Vue evuemontefioreorg

Received 8 January 2016 Revised 17 May 2016 Accepted 9 June 2016

Academic Editor Andre Megarbane

Copyright copy 2016 Elizabeth Vue et al This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use distribution and reproduction in any medium provided the original work is properly cited

Osteogenesis imperfecta (OI) is an inherited disorder of the connective tissues caused by abnormalities in collagen formation OImay presentmany challenges to the anesthesiologist A literature review reveals a wide range of implications from basic positioningto management of the difficult airwayWe present the anesthetic management of a 25-year-old gravid woman with OI fetal demiseand possible uterine rupture admitted for an exploratory laparotomy

1 Introduction

Osteogenesis imperfecta (OI) is a genetic connective tissuedisorder commonly known as brittle-bone disease with dif-ferent phenotypic presentations due to quantitatively insuf-ficient or qualitatively abnormal type 1 collagen [1ndash3] Itcommonly manifests with multiple bone fractures and maybe accompanied by a reduced life span Inherited autosomaldominant gene mutations in the alpha chain that comprisetype 1 collagen account for approximately 90 of OI cases[3 4]

Subtypes of OI are characterized based on genetic radio-graphic and clinical findings [4ndash6] Symptoms ranging frommild to severemay present at any age Common clinicalman-ifestations of OI include fractures with sometimes absentto minimal trauma blue sclera short stature scoliosis limbdeformities joint laxity and platelet dysfunction [4ndash11]

We present the case and discuss the anesthetic challengesin the management of a gravid patient with type IV OIpresenting with possible uterine rupture The challengesencountered include complex airway management respira-tory compromise secondary to skeletal deformity dwarfismand potential fractures from positioning

2 Case Report

A 25-year-old wheelchair bound multiparous woman at18 weeks of gestation with a history of OI scoliosis and

dwarfism presented with complaints of abdominal painnausea and vomiting The patient denied any history ofcardiac disease She did give a history of not being able tobe ventilated or intubated during her last caesarean sectionculminating in an emergent tracheostomy that was laterremoved Based on her medical history and physical examshe appeared to have a moderate form of OI

On admission to the ER no fetal heart tones were notedCT findings revealed expanding hemoperitoneum from apossible uterine rupture Initial assessment pointed to hypo-volemic shock On physical exam the patient was 41 inchestall (31015840510158401015840) weighing 37 kg with flexed upper extremities andshort bowed legs Airway examination revealed a short neckwith limited neck extension two-fingerbreadth thyromentaldistance small mouth opening Mallampati class 3 poordentition and a tracheostomy scar Her abdomen was grosslygravid with an umbilical hernia and was tender to light pal-pationThe patient was alert and oriented in visible pain anddistress while recumbent in the fetal position Preoperativevitals and pertinent labs were as follows temperature 979∘HR 110 BP 70ndash10030ndash40 RR mid-20 s and SpO

299-100

on room airWBC 209 k120583L HH 61191 platelets 243 k120583L sodium

139mEqL potassium 34mEqL chloride 109mEqLbicarbonate 10mEqL urea nitrogen 13mEqL creatinine051mgdL glucose 366mgdL calcium 71mgdL and lacticacid 57mmolL were found The venous blood gas revealed

Hindawi Publishing CorporationCase Reports in MedicineVolume 2016 Article ID 7429251 6 pageshttpdxdoiorg10115520167429251

2 Case Reports in Medicine

pH 71 partial pressure of carbon dioxide 413mmHg partialpressure of oxygen 389mmHg base excess minus153mmolLhemoglobin 46 gdL and glucose 214mgdL

A right femoral triple lumen catheter was emergentlyplaced in the ERThe patientrsquos hemodynamic status improvedwith normal saline resuscitation and packed red blood cells(PRBCs)

The patient was transported to the OR for an emergentexploratory laparotomy On patient sign-in with the anes-thesia team OR nursing staff and surgical team present thepatient confirmed the procedurewritten on the consent formincluding a possible hysterectomy if it was life-threateningShe emphasized her desire to maintain fertility for futurepregnancies if possible

The anesthetic plan potential complications and the DoNot Resuscitate (DNR) status were subsequently addressed tothe patient and the anesthesia team Both theORnursing staffand surgical staff were present for the discussionThe patientindicated her understanding and gave verbal consent for fullresuscitative measures in the perioperative period AmericanSociety for Anesthesiologist (ASA)monitors were placed anda right radial arterial line was inserted in sterile fashionin place of the noninvasive blood pressure cuff to avoidpossible bony trauma An arterial blood gas was immediatelyobtained

Fiberoptic intubation was attempted to avoid possiblecomplications from direct laryngoscopy anticipated difficultairway and use of succinylcholine No sedation was givensecondary to possible risk of aspiration hemodynamic insta-bility and desaturation in the setting of a difficult airwayThe airway was topicalized with aerosolized 4 lidocaineand a negative gag reflex was achieved The initial fiberopticintubation was attempted unsuccessfully via an OvassapianairwayTherewas limitedmouth opening and themouthwassmall as well These features made placement of the Ovass-apian airway difficult The patient had a persistent gag reflexdespite additional topicalization through an epidural catheterthreaded in the bronchoscope port A second fiberopticattemptwasmade through a slit 22 F nasopharyngeal trumpetplaced in the left nostrilThe nares were tight and edematousThese features made it difficult to pass the bronchoscopedown the correct passage to the nasopharyngeal spaceDespite the distorted anatomy the nasal fiberoptic approachproved successful A gradual inhalational induction andmaintenance with sevoflurane was instituted to avoid severehypotension

The patient remained hemodynamically stable through-out the intubation process A 14G right external jugular intra-venous line was placed The patient remained normothermicvia an upper body Bair Hugger and a fluid warmer Thebaseline intraoperative arterial blood gas (ABG) revealed pH749 partial pressure of carbon dioxide 19mmHg partialpressure of oxygen 144mmHg base excess minus81mmolLlactic acid 42 hemoglobin 62 gdL and glucose 128mgdLUpon initial surgical approach 2 L of blood was evacu-ated from the abdomen Hemostasis was obtained quicklyAggressive resuscitation instituted at the time included 3 Lof crystalloids and 4U of PRBCs Further exploration of the

abdominal contents confirmed a ruptured uterus with anextra-uterine fetal demise

The option of hysterectomy was discussed by the obste-tricians and gynecologic-oncologists due to the increased riskofmorbidity andmortality for this patientThe surgeonswereprimarily concerned about possible uterine rupture and post-partum hemorrhage in subsequent pregnancies Secondaryconcerns regarding this patient included poor prenatal careand failure to obtain anesthesiology consultation prior toadmission for delivery The anesthetic consultation was feltnecessary for team-based clinical management planning dueto the high-risk nature of this patientrsquos condition In responseto the considerations for hysterectomy while in the operatingroom the anesthesiologist advocated for the patientrsquos wishesreemphasizing her desire to maintain fertility if possibleThe uterus was repaired and conserved The patient wastransported to SICU and was successfully extubated onpostoperative day 1 without complications

3 Discussion

Osteogenesis imperfecta is one of the most common skeletaldysplasias estimated around 6-7 per 100000 births [4 6] Itis an extremely heterogeneous group of heritable connectivetissue disorders that was first classified into four major typesby Sillence et al in 1979 [7] More recent studies havecurrently identified 17 causative genes including autosomalrecessive genes [4 6] As a result the nomenclature andclassification have evolved substantially In 2009 the Inter-national Nomenclature Group for Constitutional Disordersof the Skeleton (INCDS) standardized the classification ofOI [4 6] based on the severity and clinical features of thedisorder (see Table 1)

Patients with moderate-severe OI are susceptible to bonefractures bruising and dislocation from minimal to notrauma [5 8] One should exercise care during transporta-tion placement on the operation table and positioning Forsome patients supine position with fully extended armsabducted less than 90 degrees may cause perioperative mor-bidity Pressure points should be supported and padded Theplacement of tourniquets [8] for the insertion of peripheralintravenous (IV) catheters must be approached with cautionArterial cannulation for blood pressure measurement may bepreferred to avoid repeated trauma from a blood pressure cuff[12] Bleeding and bruising tendencies in these patients arewell documented [2 11ndash14]

Excessive bone fragility can be challenging for the anes-thesiologist Abnormal skeletal growth causing anatomicaldistortion of the airwaymay impede tracheal intubation [8 911] Neck and mandibular fractures may occur during laryn-goscopy Upward translocation of the cervical spine (basilarinvagination) [4 6] may disrupt vascular and cerebral spinalfluid flow and result in hindbrain herniation Presence ofdentinogenesis imperfecta (DI) in patients with OI increasesthe risk of tooth dislodgement during oral instrumentation[6 8 9 11 12] In appropriate clinical scenarios supraglotticairways [8 9 11 12] have been safely placed to preventthe complications that might arise from tracheal intubation

Case Reports in Medicine 3

Table 1 Classification of osteogenesis imperfecta and related anesthetic concerns [4 6]

Types OI syndromicnames Gene Inheritance Postnatal clinical characteristics Anesthetic concerns

Type INondeformingOI with blue

sclera

COL1A1COL1A2

ADAD

Rarely congenital fractures low bonemass deformity of spine or long bones isuncommon higher frequency of longbone fractures in presence ofdentinogenesis imperfecta (DI) nearnormal growth velocity and heightambulant blue-gray sclera susceptible toconductive hearing loss absence ofchronic bone pain or minimal paincontrolled by simple analgesics

Bone fractures during extremitymanipulation (eg positioning PIVplacements with tourniquet) dentaldamage during oropharyngealinstrumentation difficulty of hearinghyperthermia or malignanthyperthermia platelet dysfunctioncapillary fragility

Type II Perinatallylethal OI

COL1A1COL1A2CRTAPLEPRE1PPIB

ADADARARAR

Ribs with continuous or discontinuousfracture crumpled (accordion-like) longbones and multiple fractures thighsabducted and in external rotation allvertebrae hypoplasticcrushed clinicalindicators of severe chronic pain smallthorax respiratory distress leading toperinatal death

Most prenatally diagnosed pregnanciesare terminated Rarely do these patientssurvive to adulthood Pain relief isvaluable

Type III Progressivelydeforming

COL1A1COL1A2BMP1CRTAPFKBP10LEPRE1PLOD2PPIB

SERPINF1SERPINH1TMEM38BWNT1

CREB3L1

ADADARARARARARARARARARARAR

Usually near term newborn or infantpresentation with bone fragility andmultiple fractures platyspondylyvertebrae at birth thin ribs withdiscontinuous beadingfractures markedshort stature progressive kyphoscoliosisand bowing of legs generalizedosteoporosisosteopenia increasedprevalence of basilar impression possiblyhaving blue sclera at birth DI is variablehearing loss is more frequent in adultspossibly having cardiovascularcomplications such as valvulardysfunction or aortic root dilation

Bone fractures during extremitymanipulation posterior fossacompression syndromes due to basilarimpression from cervical manipulationpulmonary insufficiency or hypertensioncardiopulmonary failure hyperthermiaor malignant hyperthermia plateletdysfunction capillary fragilitypostoperative pain control

Type IV

Commonvariable OIwith normal

sclera

COL1A1COL1A2WNT1CRTAPPPIBSP7PLS3

ADADADARARARXL

Variable severity recurrent fracturesvertebral compression fracturesosteoporosis variable degrees ofdeformity of long bones and spine(thoracolumbar kyphoscoliosis) bowingof long bones short stature possiblybeing wheelchair bound normal scleraDI increased prevalence of basilarimpression (5 times higher relative risk inthose with DI) hearing impairment is notoften encountered possibly havingchronic bone pain possibly havingcardiovascular complications such asvalvular dysfunction or aortic rootdilation

Bone fracture or dislocation dentaldamage posterior fossa compressionsyndromes pulmonary insufficiency orhypertension cardiorespiratory failurehyperthermia or malignanthyperthermia platelet dysfunctionpostoperative pain control

Type V

OI withcalcification ininterosseousmembranes

IFITM5 AD

No congenital fractures distinguished bycalcification of interosseous membrane inforearms increased risk of developinghyperplastic callus restriction ofpronation and supination of forearmsradial head dislocations bowing of longbones in some patients vertebralcompression fractures no DI presencewhite sclera

Bone fractures and dislocations duringextremity manipulation indomethacinrecommended to avert callus progressionhyperthermia or malignanthyperthermia platelet dysfunctioncapillary fragility


Regional anesthesia can be safe and effective [10ndash12] how-ever a thorough assessment of the airway severity of spinedeformity prior back surgery and platelet function should bedone

Avoidance of succinylcholine should be considered dueto the potential for fasciculation-induced fractures [8 11 12]and malignant hyperthermia (MH) Studies have looked intothe association of OI and hyperthermia with or withoutMH susceptibility [15 16] A review of cases involving MHand caffeine halothane contracture test (CHCT) showedresults in the context of coexisting diseases and syndromes[15] It was concluded that there is weak evidence for theassociation of OI to MH but a positive association tointraoperative hyperthermia responsive to standard coolingmethodsWithin these case seriesmost patients withOIwerefound to have normal CHCT and no reports of MH wereconfirmed in OI patients with positive CHCT In contrast aretrospective study showed no significance in intraoperativehyperthermia or end-tidal CO

2levels between patients with

OI and those undergoing general anesthesia [16] includinguse of sevoflurane Ogawa et al [17] advocates using totalintravenous infusion (TIVA) to avoid body temperatureelevation and MH however there may be a risk of propofolinfusion syndrome as reported in one case after short-termpropofol infusion for anesthesia [17 18]

Patients with OI may not tolerate general anesthesia wellSpinal and chest wall deformities predispose patients with OIto pulmonary disease ventilation-perfusion mismatch andrapid desaturation [19] Pectus carinatum and kyphoscoliosislimit thoracic movement and lung expansion resulting inrestrictive pulmonary disease These derangements includedecreased vital capacity decreased functional residual capac-ity and decreased chest wall compliance Reduced functionalresidual capacity of pregnancy adds to pulmonary compro-mise [20] Hemodynamic changes should be anticipated fromaortocaval compression from the gravid uterus combinedwith the vasodilatory effects of general anesthesia Further-more these patients may have cardiac disease includingvalvular dysfunction

Platelet dysfunction is a clinical concern commonlyencountered [11 14] Preoperative platelet transfusion shouldbe considered Studies have shown increased capillaryfragility decreased platelet retention decreased factor VIIIproduction and decreased collagen-induced platelet aggre-gation The underlying collagen abnormality [14] can resultin delicate tissues and small blood vessels that are unable toadequately constrict Pregnant women are prone to uterineatony and may result in excessive postpartum hemorrhageor disseminated intravascular coagulopathy [10 11 20] (seeTable 2)

Pregnant women with OI who have skeletal deformityand short stature should be monitored in high-risk prenatalcare centers for both maternal and fetal safety [20] Breechpresentation of the fetus is common in women with OI Inaddition pregnant women with OI usually do not toleratethe increasing size of a gravid uterus due to short statureand usually require early caesarean section [6 12 20] Spon-taneous uterine rupture has been described in women withOI [2 3] Increased risk of vaginal lacerations and uterine

Table 2 Osteogenesis imperfecta challenges in the perioperativesetting

Perioperativephase OI specific anesthetic challenge

Preoperative

Hypovolemic shockBlood lossAnticipation of difficulty of intubationNPO statusGravida status

Intraoperative

PositioningHemodynamic monitoring noninvasiveversus invasivePIV placementAirway control tracheal intubationtechniqueDental and oral trauma avoidanceGeneral anesthetic agent inhalational versusTIVAVentilation and oxygenation managementHemodynamic managementHemostasisExtubation

Postoperative Ventilation and oxygenation managementPain control

rupture have also been described in women attemptingvaginal delivery and are often treated as a trial of laborwith a scarred uterus [12] Studies have shown that womenwith OI have a decreased amount of collagen type I in themyometrium [2] This is thought to be the underlying causeof spontaneous uterine ruptures However women with OIhave also successfully delivered without complications [3]Literature reports both successful vaginal and cesarean birthsin these patients and the optimal mode of delivery should bedecided on an individual basis [20]

31 Ethical Concerns The ASA Guidelines and AmericanCollege of Surgeons recommend that prior to proceduresrequiring anesthetic care any existing directives limitingthe use of resuscitative methods should be reviewed withthe patient or designated surrogate when possible [21ndash23]Automatic suspension of DNR orders in the OR is inappro-priate without informed consent There is agreement thatopportunities for a careful informed discussion about thepotential resuscitative measures and surgical risks shouldbe discussed in order to provide the treatment that bestsupports the patientrsquos vision of care and the acute clinicalsituation If possible all physicians in the healthcare teamdirectly involved in the care of the patient during theprocedure should be present and included [21] As a resultthese directives should be clarified or modified based onthe preference of the patient and clearly documented in themedical record

Fertility is usually preserved [11 20] in patients withmild to moderate OI Though there are increased risks foruterine rupture and postpartum hemorrhage patients withOI have also successfully delivered without complicationsThere may be circumstances when adhering to the patientrsquos


wishes is likely to result in harm and cause surgical teams andanesthesiologists to hesitate in their actions [23] Howeverpatient autonomy is essential to ethical decision-making Itis the responsibility of the provider to address and advocatefor the patientrsquos right Not adhering to the patientrsquos desiremay cause harm to both the patient and family as well asto the provider Healthcare workers are often impacted bymedical errors in theworkplace and suffer as ldquosecond victimsrdquo[24 25] Studies have described damage to the providerrsquosconfidence and self-esteem exhibiting posttraumatic stresssymptoms and requiring support Second victim is a commonproblem for healthcare organizations and trainees are partic-ularly more vulnerable [25] This example reemphasizes theimportance for an open discussion addressing the patientrsquosgoal of care and the surgical and anesthetic concerns prior toa procedure If the provider finds the medical decisions forpatients to be irreconcilable with his own moral views thenthe provider should withdraw in a nonjudgmental fashionproviding an alternative plan for care in a timely fashion[21]

4 Conclusion

Patients with OI pose significant challenges for the anes-thesiologist In the past many patients with moderate tosevere OI died by the end of the second decade mainlydue to complications of skeletal chest wall deformity andcardiorespiratory failure [19] With the current therapeuticoptions available the majority of these patients will surviveinto adult life These patients may present with a wide rangeof obstacles that should be considered and managed appro-priately The ability to identify the type of OI a patient hasand to consider associated clinical conditions will help deter-mine the choice of perioperative anesthetic management Ananesthesiology consultation prior to an elective surgery andin early pregnancy is recommended In the setting of anemergent procedure it is prudent to try to attain a thoroughpreoperative assessment and devise a preinduction anestheticplan as this may improve outcomes in these patients

After a thorough literature research using PubMedGoogle Scholar and Ovid Medline and Cochrane ourrecommendations for anesthetic management of these casesinclude the following

(1) Preoperative

(a) Discontinue bisphosphonate infusions from thestart of pregnancy [20] if the patient is on theregimen Exposure could cause skeletal abnor-malities and congenital malformations

(b) Obtain detailed medical history to determinethe type and severity of the patientrsquos disease[4 6]

(c) Obtain echocardiography to evaluate cardiacanatomy and function [4] if indicated

(d) Accurately assess the airway to determine diffi-culty of intubation [8 9 11]

(e) Devise the anesthetic plan and alternative plans(f) Confirm a type and screen [11 14]

(2) Intraoperative

(a) Transport and position patient with care [5 8](b) Consider arterial cannulation in place of blood

pressure cuff to avoid bone fractures and bruis-ing [11ndash14]

(c) Avoid succinylcholine use [8 11 12] if feasible forpatient clinical management

(d) Be vigilant of hemodynamic and ventilationchanges [11 14 19 20]

(e) Be cognizant of the risk for hyperthermia andmalignant hyperthermia [15 16]

(f) Monitor for excess bleeding [11 14]

(3) Postoperative

(a) Ensure adequate oxygenation and ventilation(b) Monitor for postoperative hemorrhage(c) Pain control patients with OI may have chronic

bone pain that is not related to surgical site(d) Consider resuming cyclic intravenous pamid-

ronate therapy postoperatively [13] and for aminimum of 2 years [26] Most studies are seenin pediatrics

(e) Bisphosphonate use as analgesics have beenshown to be beneficial in CRPS osteoporosisPagetrsquos disease of the bone multiple myelomametastatic bone disease and vertebral compres-sion fractures [27 28]

Competing Interests

The authors declare that they have no competing interests

References

[1] A Gajko-Galicka ldquoMutations in type I collagen genes result-ing in osteogenesis imperfecta in humansrdquo Acta BiochimicaPolonica vol 49 no 2 pp 433ndash441 2002

[2] A Di Lieto F Pollio M De Falco et al ldquoCollagen contentand growth factor immunoexpression in uterine lower segmentof type IA osteogenesis imperfecta relationship with recurrentuterine rupture in pregnancyrdquo American Journal of Obstetrics ampGynecology vol 189 no 2 pp 594ndash600 2003

[3] S P Chetty B L Shaffer and M E Norton ldquoManagement ofpregnancy in women with genetic disorders part 1 disordersof the connective tissue muscle vascular and skeletal systemsrdquoObstetrical and Gynecological Survey vol 66 no 11 pp 699ndash709 2011

[4] F S VanDijk andDO Sillence ldquoOsteogenesis imperfecta clin-ical diagnosis nomenclature and severity assessmentrdquo Ameri-can Journal of Medical Genetics Part A vol 164 no 6 pp 1470ndash1481 2014

[5] A Forlino W A Cabral A M Barnes and J C MarinildquoNew perspectives on osteogenesis imperfectardquoNature ReviewsEndocrinology vol 7 no 9 pp 540ndash557 2011

[6] F S VanDijk JMCobbenAKariminejad et al ldquoOsteogenesisimperfecta a review with clinical examplesrdquoMolecular Syndro-mology vol 2 no 1 pp 1ndash20 2011


[7] D O Sillence A Senn and D M Danks ldquoGenetic heterogene-ity in osteogenesis imperfectardquo Journal of Medical Genetics vol16 no 2 pp 101ndash116 1979

[8] M A L Erdogan M Sanli and M O Z Ersoy ldquoAnesthesiamanagement in a child with osteogenesis imperfecta andepidural hemorrhagerdquo Brazilian Journal of Anesthesiology vol63 no 4 pp 366ndash368 2013

[9] M L Santos C Anez A Fuentes B Mendez R Perinan andM Rull ldquoAirway management with ProSeal LMA in a patientwith osteogenesis imperfectardquo Anesthesia and Analgesia vol103 no 3 article 794 2006

[10] T M Vogel E F Ratner R C Thomas Jr and U ChitkaraldquoPregnancy complicated by severe osteogenesis imperfecta areport of two casesrdquo Anesthesia amp Analgesia vol 94 no 5 pp1315ndash1317 2002

[11] T G Lyra V A F Pinto F A B Ivo and J D S NascimentoldquoOsteogenesis imperfecta in pregnancy case reportrdquo RevistaBrasileira de Anestesiologia vol 60 no 3 pp 321ndash324 2010

[12] EDinges COrtner L Bollag J Davies andR Landau ldquoOsteo-genesis imperfecta cesarean deliveries in identical twinsrdquo Inter-national Journal of Obstetric Anesthesia vol 24 no 1 pp 64ndash842015

[13] Y R Choi N-J Yi J S Ko et al ldquoLiving donor livertransplantation for an infant with osteogenesis imperfecta andintrahepatic cholestasis report of a caserdquo Journal of KoreanMedical Science vol 29 no 3 pp 441ndash444 2014

[14] G Edge B Okafort M E Fennelly and A O Ransford ldquoAnunusual manifestation of bleeding diathesis in a patient withosteogenesis imperfectardquo European Journal of Anaesthesiologyvol 14 no 2 pp 215ndash219 1997

[15] J Benca and K Hogan ldquoMalignant hyperthermia coexistingdisorders and enzymopathies risks and management optionsrdquoAnesthesia and Analgesia vol 109 no 4 pp 1049ndash1053 2009

[16] K Bojanic J E Kivela C Gurrieri et al ldquoPerioperative courseand intraoperative temperatures in patients with osteogenesisimperfectardquo European Journal of Anaesthesiology vol 28 no 5pp 370ndash375 2011

[17] S Ogawa R Okutani and K Suehiro ldquoAnesthetic managementusing total intravenous anesthesia with remifentanil in a childwith osteogenesis imperfectardquo Journal of Anesthesia vol 23 no1 pp 123ndash125 2009

[18] C Kill A Leonhardt and H Wulf ldquoLacticacidosis after short-term infusion of propofol for anaesthesia in a child withosteogenesis imperfectardquo Paediatric Anaesthesia vol 13 no 9pp 823ndash826 2003

[19] R F Widmann F D Bitan F J Laplaza S W Burke MF DiMaio and R Schneider ldquoSpinal deformity pulmonarycompromise and quality of life in osteogenesis imperfectardquoSpine vol 24 no 16 pp 1673ndash1678 1999

[20] M Cozzolino F Perelli L Maggio et al ldquoManagement ofosteogenesis imperfecta type I in pregnancy a review ofliterature applied to clinical practicerdquo Archives of Gynecologyand Obstetrics vol 293 no 6 pp 1153ndash1159 2016

[21] American Society of Anesthesiologists Ethical guidelines forthe anesthesia care of patients with do-not-resuscitate orders orother directives that limit treatment

[22] American College of Surgeions ldquoStatement on advance direc-tives by patients lsquodo not resuscitatersquo in the operating roomrdquoBulletin of the American College of Surgeons vol 99 no 1 pp42ndash43 2014

[23] D Dugan and J Riseman ldquoDo-not-resuscitate orders in anoperating room setting 292rdquo Journal of PalliativeMedicine vol18 no 7 pp 638ndash639 2015

[24] A W Wu and R C Steckelberg ldquoMedical error incidentinvestigation and the second victim doing better but feelingworserdquoBMJQuality and Safety vol 21 no 4 pp 267ndash270 2012

[25] S D Scott L E Hirschinger K R Cox M McCoig JBrandt and L W Hall ldquoThe natural history of recovery for thehealthcare provider lsquosecond victimrsquo after adverse patient eventsrdquoQuality and Safety in Health Care vol 18 no 5 pp 325ndash3302009

[26] M A El Sobky A A Zaky Hanna N E Basha Y N TarrafandMH Said ldquoSurgery versus surgery plus pamidronate in themanagement of osteogenesis imperfecta patients a comparativestudyrdquo Journal of Pediatric Orthopaedics Part B vol 15 no 3 pp222ndash228 2006

[27] B Kosharskyy W Almonte N Shaparin M Pappagallo andH Smith ldquoIntravenous infusions in chronic painmanagementrdquoPain Physician vol 16 no 3 pp 231ndash249 2013

[28] M Pappagallo B Breuer H-M Lin et al ldquoA pilot trial ofintravenous pamidronate for chronic low back painrdquo Pain vol155 no 1 pp 108ndash117 2014

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Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014


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Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom


pH 71 partial pressure of carbon dioxide 413mmHg partialpressure of oxygen 389mmHg base excess minus153mmolLhemoglobin 46 gdL and glucose 214mgdL

A right femoral triple lumen catheter was emergentlyplaced in the ERThe patientrsquos hemodynamic status improvedwith normal saline resuscitation and packed red blood cells(PRBCs)

The patient was transported to the OR for an emergentexploratory laparotomy On patient sign-in with the anes-thesia team OR nursing staff and surgical team present thepatient confirmed the procedurewritten on the consent formincluding a possible hysterectomy if it was life-threateningShe emphasized her desire to maintain fertility for futurepregnancies if possible

The anesthetic plan potential complications and the DoNot Resuscitate (DNR) status were subsequently addressed tothe patient and the anesthesia team Both theORnursing staffand surgical staff were present for the discussionThe patientindicated her understanding and gave verbal consent for fullresuscitative measures in the perioperative period AmericanSociety for Anesthesiologist (ASA)monitors were placed anda right radial arterial line was inserted in sterile fashionin place of the noninvasive blood pressure cuff to avoidpossible bony trauma An arterial blood gas was immediatelyobtained

Fiberoptic intubation was attempted to avoid possiblecomplications from direct laryngoscopy anticipated difficultairway and use of succinylcholine No sedation was givensecondary to possible risk of aspiration hemodynamic insta-bility and desaturation in the setting of a difficult airwayThe airway was topicalized with aerosolized 4 lidocaineand a negative gag reflex was achieved The initial fiberopticintubation was attempted unsuccessfully via an OvassapianairwayTherewas limitedmouth opening and themouthwassmall as well These features made placement of the Ovass-apian airway difficult The patient had a persistent gag reflexdespite additional topicalization through an epidural catheterthreaded in the bronchoscope port A second fiberopticattemptwasmade through a slit 22 F nasopharyngeal trumpetplaced in the left nostrilThe nares were tight and edematousThese features made it difficult to pass the bronchoscopedown the correct passage to the nasopharyngeal spaceDespite the distorted anatomy the nasal fiberoptic approachproved successful A gradual inhalational induction andmaintenance with sevoflurane was instituted to avoid severehypotension

The patient remained hemodynamically stable through-out the intubation process A 14G right external jugular intra-venous line was placed The patient remained normothermicvia an upper body Bair Hugger and a fluid warmer Thebaseline intraoperative arterial blood gas (ABG) revealed pH749 partial pressure of carbon dioxide 19mmHg partialpressure of oxygen 144mmHg base excess minus81mmolLlactic acid 42 hemoglobin 62 gdL and glucose 128mgdLUpon initial surgical approach 2 L of blood was evacu-ated from the abdomen Hemostasis was obtained quicklyAggressive resuscitation instituted at the time included 3 Lof crystalloids and 4U of PRBCs Further exploration of the

abdominal contents confirmed a ruptured uterus with anextra-uterine fetal demise

The option of hysterectomy was discussed by the obste-tricians and gynecologic-oncologists due to the increased riskofmorbidity andmortality for this patientThe surgeonswereprimarily concerned about possible uterine rupture and post-partum hemorrhage in subsequent pregnancies Secondaryconcerns regarding this patient included poor prenatal careand failure to obtain anesthesiology consultation prior toadmission for delivery The anesthetic consultation was feltnecessary for team-based clinical management planning dueto the high-risk nature of this patientrsquos condition In responseto the considerations for hysterectomy while in the operatingroom the anesthesiologist advocated for the patientrsquos wishesreemphasizing her desire to maintain fertility if possibleThe uterus was repaired and conserved The patient wastransported to SICU and was successfully extubated onpostoperative day 1 without complications

3 Discussion

Osteogenesis imperfecta is one of the most common skeletaldysplasias estimated around 6-7 per 100000 births [4 6] Itis an extremely heterogeneous group of heritable connectivetissue disorders that was first classified into four major typesby Sillence et al in 1979 [7] More recent studies havecurrently identified 17 causative genes including autosomalrecessive genes [4 6] As a result the nomenclature andclassification have evolved substantially In 2009 the Inter-national Nomenclature Group for Constitutional Disordersof the Skeleton (INCDS) standardized the classification ofOI [4 6] based on the severity and clinical features of thedisorder (see Table 1)

Patients with moderate-severe OI are susceptible to bonefractures bruising and dislocation from minimal to notrauma [5 8] One should exercise care during transporta-tion placement on the operation table and positioning Forsome patients supine position with fully extended armsabducted less than 90 degrees may cause perioperative mor-bidity Pressure points should be supported and padded Theplacement of tourniquets [8] for the insertion of peripheralintravenous (IV) catheters must be approached with cautionArterial cannulation for blood pressure measurement may bepreferred to avoid repeated trauma from a blood pressure cuff[12] Bleeding and bruising tendencies in these patients arewell documented [2 11ndash14]

Excessive bone fragility can be challenging for the anes-thesiologist Abnormal skeletal growth causing anatomicaldistortion of the airwaymay impede tracheal intubation [8 911] Neck and mandibular fractures may occur during laryn-goscopy Upward translocation of the cervical spine (basilarinvagination) [4 6] may disrupt vascular and cerebral spinalfluid flow and result in hindbrain herniation Presence ofdentinogenesis imperfecta (DI) in patients with OI increasesthe risk of tooth dislodgement during oral instrumentation[6 8 9 11 12] In appropriate clinical scenarios supraglotticairways [8 9 11 12] have been safely placed to preventthe complications that might arise from tracheal intubation





sclera

COL1A1COL1A2

ADAD





ADADARARAR






CREB3L1




Type IV


sclera


ADADADARARARXL



Type V


IFITM5 AD













Preoperative


Intraoperative








4 Conclusion



(1) Preoperative






(2) Intraoperative







(3) Postoperative





Competing Interests


References



































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of




















sclera

COL1A1COL1A2

ADAD





ADADARARAR






CREB3L1




Type IV


sclera


ADADADARARARXL



Type V


IFITM5 AD













Preoperative


Intraoperative








4 Conclusion



(1) Preoperative






(2) Intraoperative







(3) Postoperative





Competing Interests


References



































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of


























Preoperative


Intraoperative








4 Conclusion



(1) Preoperative






(2) Intraoperative







(3) Postoperative





Competing Interests


References



































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of


















4 Conclusion



(1) Preoperative






(2) Intraoperative







(3) Postoperative





Competing Interests


References



































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of












































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of
















case report anesthetic management in a gravida with type...

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