title: Severe Sprengel deformity associated with Klippel-Feil syndrome and a complex vascular abnormality that determined the corrective surgery technique.
abstract: Sprengel deformity (SD), a congenital condition characterized by elevation of the scapula, is a cause of functional and aesthetic defects that can be improved by surgical correction. Many cases of SD are associated with Klippel-Feil syndrome (KFS), in which there may be abnormalities of the supra-aortic vessels. We present the case of an 11-year-old girl with severe SD and KFS. The left vertebral artery arose from the subclavian artery in a very high cervical location, which made surgical descent of the scapula unfeasible. The patient was treated using a Mears procedure, with osteotomy of the scapula and tenotomy of the long head of the triceps. The appearance and range of motion of the shoulders improved considerably, and there were no vascular complications. A morphologic vascular assessment is essential in children with SD and concomitant KFS to avoid potentially serious iatrogenic vascular injury when performing a scapular-descending surgical technique.
authors.affiliation: aVall d'Hebron Hospital, University of Barcelona, Barcelona bJosep Trueta University Hospital, Girona cHospital Sant Joan De Deu, University of Barcelona, Barcelona, Spain.
authors.forename: Cesar G
authors.initials: CG
authors.lastname: Fontecha
authors.forename: Ester
authors.initials: E
authors.lastname: Navarro Cano
authors.forename: Francisco
authors.initials: F
authors.lastname: Soldado
authors.forename: Ignasi
authors.initials: I
authors.lastname: Barber
mesh_terms: D002648:Child; D000013:Congenital Abnormalities / Q000601:surgery*; D005260:Female; D006801:Humans; D007714:Klippel-Feil Syndrome / Q000150:complications*; D016059:Range of Motion, Articular; D012540:Scapula / Q000002:abnormalities* / Q000601:surgery; D012785:Shoulder Joint / Q000002:abnormalities* / Q000601:surgery; D013348:Subclavian Artery / Q000002:abnormalities*; D016896:Treatment Outcome; D014711:Vertebral Artery / Q000002:abnormalities*