Surgical treatment of a 180° thoracolumbar fixed kyphosis in a young achondroplastic patient: a one-stage “in situ” combined fusion and spinal cord translocation

摘要

OpenimageinnewwindowAnachondroplasticpatientwithathoracolumbarkyphosiswasfirstseenattheageof16atourinstitution.Hisonlyconcernatthattimewastheaestheticimplicationofhisdeformity.Hisphysicalexaminationwasnormalexceptforlossoftheneurologicreflexesinthelowerlimbs.Theradiographsshowedafixed180°thoracolumbarkyphosiswithcorrectfrontalandsagittalbalances.NospinalcordanomalywasfoundonMRI.Twoyearslater,hedevelopedaprogressiveneurogenicclaudicationofthelowerlimbs.Hewasstillneurologicallyintactatrest.TheMRIshowedanabnormalcentralspinalcordsignalinfrontoftheapexofthekyphosisassociatedwiththenarrowcongenitalspinalcanal.Inregardstothisprogressiveneurologicalworsening,asurgicaltreatmentwasdecided.Wedecidedtoperformafrontandbackarthrodesiscombinedwithaspinalcorddecompressionwithoutreductionofthedeformity.Afive-levelhemilaminotomywasperformedwithaposteriorapproachatthekyphosisdeformity.Thespinalcordwasindividualisedonto10?cmandtheleftnerverootswereisolated.Adecancellationosteotomyofthethreeapexvertebraeandadiscexcisionwereperformed.Theposterioraspectofthevertebralbodywasthentranslatedforward2?cmandinassociationwiththespinalcord.Twonerverootswereseveredlaterallytoapproachtheanteriorpartofthekyphosisandaperonealstrutgraftwasinlayedanterolaterally.Acomplementaryanteriorandarightposterolateralfusionwasmadewithcancellousbone.Thepatientwasimmobilisedinacastfor3?monthsrelayedbyathoracolumbosacralorthosisfor6?months.At3?yearsfollow-up,theneurogenicclaudicationhaddisappeared.Noworseningofthekyphosiswasobserved.Hisonlycomplaintisviolentelectricshockinthelowerlimbswithanyexternalsuddenpressureonthespinalcordintheareauncoveredbybone...