Affiliation:
1. Centro Hospitalar Universitário Lisboa Norte (North Lisbon University Hospital Centre), Lisbon, Portugal.
Abstract
Case:
We describe an adult patient with bilateral Sprengel deformity (BSD) with bilateral omovertebral bone and thoracic spinal failure of segmentation (T2-T4) successfully surgically treated only at 19-year-old. After resection of the bilateral omovertebral bone and simultaneous relocation of the scapula, disabilities of the arm, shoulder, and hand score improved from 26.7 preoperatively to 2.5 at the 3-year follow-up.
Conclusion:
The surgical treatment of Sprengel deformity is typically recommended between 3 and 7 years of age when significant shoulder impairment and/or severe cosmetic concerns are present. The authors were able to achieve a successful surgical outcome in a patient with BSD despite an unusual late presentation.
Publisher
Ovid Technologies (Wolters Kluwer Health)
Subject
Orthopedics and Sports Medicine,Surgery
Reference16 articles.
1. Sprengel deformity: pathogenesis and management;Harvey;J Am Acad Orthop Surg.,2012
2. Die angeborene verschiebung des schulterblattes nach oben;Sprengel;Archiv Fur Klinische Chirurgie.,1891
3. Klippel-Feil anomaly with Sprengel anomaly, omovertebral bone, thumb abnormalities, and flexion-crease changes: novel association or syndrome?;Larson;Am J Med Genet.,2001
4. Congenital elevation of the scapula;Cavendish;J Bone Joint Surg Br.,1972
5. Sprengel deformity in biological sisters;Pargas;JAAOS.,2020