Outcomes following endoscopic-assisted Woodward procedure for Sprengel deformity

Purpose An endoscopic-assisted approach for Sprengel deformity has been previously reported. Our objective was to assess outcomes of the endoscopic Woodward procedure in a series of patients. Methods Retrospective analysis was performed of children with a Sprengel deformity treated between November 2014 and February 2018. Recorded data were demographic, pre- and postoperative active shoulder elevation and deformity severity according to Cavendish. Results A total of 12 children (four girls and eight boys, ten right-sided/two left-sided) with a mean age of nine years two months (3 years 5 months to 16 years 1 month) and mean follow-up 19.8 months (10 to 48) were assessed. Nine children were classified as Cavendish Grade III and three as grade IV, respectively. Mean preoperative active shoulder forward elevation was 100.8° (70° to 120°), while postoperatively it increased to 149.2° (100° to 170°). Mean preoperative scapular high difference was 4.5 cm (2.8 to 5.2), while postoperatively it was 1.33 cm (0 to 2.8). Conclusion The endoscopic assisted Woodward procedure is an effective technique. Further comparative studies will ascertain advantages in functional and cosmetic results compared to the standard Woodward procedure. Level of Evidence Therapeutic study, Level IV

Three-year follow-up after Woodward procedure in children with Sprengel’s deformity

Sprengel deformity is a rare congenital disorder characterized by a congenital high scapula caused by obstruction of the development of the scapula, which accompanies bone, muscle and cartilage during pregnancy. This scapular deformity can be associated with other congenital malformations such as rib abnormalities, scoliosis, and Klippel-Feil syndrome. This is a follow-up of 3-year-old children with Sprengel deformity, cavendish grade 3 who were treated with the Woodward procedure. Authors conclude that the Woodward procedure is an effective procedure for obtaining good functional and cosmetic results.

Klippel–Feil Syndrome with Sprengel Deformity and Extensive Upper Extremity Deformity: A Case Report and Literature Review

Introduction. Klippel–Feil syndrome (KFS) is a congenital anomaly resulting from fusion of cervical vertebral bodies secondary to the dysregulation of signaling pathways during somite development. It is commonly associated with scoliosis and Sprengel deformity. We present a case of KFS with commonly associated abnormalities as well as deformities that have not yet been reported in the literature. Case Presentation. A 3-year-old girl presented for further evaluation of a left upper extremity deformity following a negative genetic workup. Upon physical exam and radiographic imaging, the patient was diagnosed with KFS and associated abnormalities including cervical scoliosis, Sprengel deformity, and congenital deformity of the left upper extremity. Deformities of the left upper extremity include radioulnar synostosis, a four-rayed hand, and absent thenar musculature. The Sprengel deformity was corrected surgically with a Woodward procedure. Discussion. Congenital musculoskeletal deformities can be differentiated based upon spinal and limb embryology. The presence of extraspinal abnormalities not originating from somite differentiation may suggest a severe form of KFS. Important considerations in the workup of the KFS patient include looking for deformities of the shoulder girdle and upper extremities to identify abnormalities for intervention at a young age.

The Woodward technique for Sprengel deformation

A case of 5-year-old boy with a left congenital undescended scapula graded Cavendish III was consulted in our unit and operated successfully with the Woodward technique. It was the first case treated by this procedure for the past 10 years, in the Pediatric Surgical Unit of the Yaounde Gyneco-Obstetric and Pediatric Hospital (Cameroon). The Woodward procedure permitted to obtain a good functional and cosmetic result.