"Sprengel's Deformity in children" -A Case Series with an Evidence - Based Approach in Diagnosis and it's Surgical Management.

Introduction: Sprengel's deformity (SD) is a rare congenital condition in children characterized by failure of caudal descent, resulting in a high scapula, a cosmetic deformity with functional limitation of the shoulder movements. It is frequently associated with vertebral and other anomalies. Materials and Methods: A retrospective case series of six children aged <17 years diagnosed with SD over 10 years presented to our tertiary care referral center was included in our study. The exclusion criteria were adults with Sprengel and children with syndromic SD due to their different spectrum of possible intervention, timing of surgery, and their outcomes. Clinical assessment using Cavendish, Rigault systems, and radiological evaluation using plain radiographs, Computerized tomographic scan, and magnetic resonance imaging were performed. Surgical management by Woodward's procedure was undertaken in a 9-year-old case. The outcomes were assessed based on cosmetic improvement, shoulder abduction, and its complications. Current concepts of SD in diagnosis, different radiographic indices, and various surgical options from the systematic review were discussed. Results: The study included six children aged 2-9 years. Four patients had associated omo-vertebral anomalies, five had congenital spinal anomalies, and three had scoliosis. Cavendish Grade ranged from II to IV. Woodward's surgical corrective procedure resulted in significant improvement in shoulder abduction and cosmetic appearance. No major neurovascular complications were observed. Conclusion: Early diagnosis and appropriate surgical intervention in moderate-to-severe SD provide satisfactory cosmetic and functional outcomes. A comprehensive evaluation for associated anomalies is essential for optimal management.