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Sprengel Deformity Associated with Spondylocostal Dysostosis Treated Using a Modified Wilkinson Procedure: A Case
Yuto Obataya1,2, Yuta Tsukagoshi1, Yoshiyasu Ikezawa2
1Department of Orthopaedic Surgery, Institute of Medicine, University of Tsukuba, Tsukuba, Japan.
Introduction:
Sprengel deformity is a rare congenital anomaly caused by failure of normal scapular descent during embryogenesis. Spondylocostal dysostosis (SCDO) is a genetically heterogeneous disorder characterized by vertebral segmentation defects and rib malformations. Reports describing the coexistence of these two conditions are scarce, and optimal surgical management remains unclear.
Case Report:
A 6-year-old girl presented with limited elevation of the left shoulder and visible scapular asymmetry. Imaging demonstrated elevation, medial displacement, and severe downward rotation of the scapula, associated with rib malalignment and cervicothoracic segmentation anomalies consistent with SCDO. We performed a modified Wilkinson procedure incorporating a Y-shaped scapular osteotomy to achieve both caudal repositioning and rotational correction.
Conclusion:
At 1 year postoperatively, shoulder elevation was nearly symmetric, and at 18 months, the patient had no functional limitations. In cases of Sprengel deformity associated with SCDO, severe downward rotation may necessitate a rotationally corrective osteotomy rather than descent alone.
