Surgical challenges in the management of cervical kyphotic deformity in patients with severe osteoporosis: an illustrative case of a patient with Hajdu–Cheney syndrome

[摘要] OpenimageinnewwindowPurposeNostandardstrategyexistsforthemanagementofcervicalkyphoticdeformityinpatientswithsevereosteoporosis.Infact,insuchsubpopulation,standardalgorithmscommonlyusedinpatientswithnormalbonemineraldensitymaynotbeapplicable.InthisGrandRounds,theauthorspresentachallengingcaseofapatientwithHajdu–Cheneysyndrome,araredisorderofbonemetabolisminducedbyaNotch-2mutation,whopresentedwithcervicalkyphoticdeformityandsevereosteoporosis.MethodsA65-year-oldfemalepatientwithapreviousdiagnosisofHajdu–Cheneysyndromepresentedwithcervicalmyelopathyandcervicalkyphoticdeformity.TheinitialMRidemonstratedmultilevelcervicalcanalstenosis.TheCT-scanalsorevealedmarkedspondylolisthesisofC6overC7aswellasnumerouslaminarandpediclefractures,resultinginacervicalkyphosisofapproximately50degrees.ResultsThepatientwassubmittedto360-degreedecompressionandfusionofthecervicalspineconsistingofastagedC6anteriorcorpectomyandmultilevelmicrodiscectomieswithwideopeningofthe posteriorlongitudinalligamentinordertoprovideasatisfactoryreleaseofanteriorspinalstructures,followedby24 hofcervicalhalo-traction,asecondanteriorapproachforbonegraftimplantationinthesiteofthecorpectomyaswellasinsertionofallograftsandcompletionoftheACDFC2–T1andplating,and,finally,aposteriorC2–T3pediclescrewinstrumentationusingintra-operativeCT-scan(O-arm)navigationguidance.ConclusionsThiscaseillustratessomeintra-operativenuancesaswellasspecificsurgicalrecommendationsforcervicaldeformitysurgeryinpatientswithsevereosteoporosis,suchasavoidanceofCasparpinsforinterbodydistraction,useofintra-operativefluoroscopyforachievementofbicorticalpurchaseofanteriorcervicalscrewsandplacementofpediclescrewsduringposteriorinstrumentation.Moreover,suchillustrativecasedemonstratesthat,inthesubpopulationofpatientswithsevereosteoporosis,itmaybepossibletosuccessfullyapplycervicaldistractionafteranisolatedanteriorapproachwithasatisfactoryimprovementinthecervicalalignment,possiblyavoidingmorelaborious540-degreeapproachessuchasthepreviouslydescribedback–front–backorfront–back–frontsurgicalalgorithms...

[学科分类] 骨科学

[关键词] Hajdu–Cheney syndrome ;Osteoporosis ;Cervical kyphotic deformity ;Anterior cervical discectomy and fusion ;Cervical pedicle screw fixation