Upper Extremity Manifestations of Beckwith-Wiedemann Syndrome: A Unique Case Presentation and Management of a Pediatric Patient Over 9 Years

Beckwith-Wiedemann syndrome is a rare congenital genetic condition with various clinical manifestations, notably isolated lateralized overgrowth. Literature regarding hemihyperplasia of the hand and upper extremity is scarce. We present a 9-year-old right handedgirl with right upper-extremity hemihypertrophy who was followed extensively over 9 years for various soft tissue complaints. Despite adequate nerve decompressions, asymmetric growth recurred. After puberty, the patient developed wrist and finger contractures, resulting in diminished range of motion and function, which caused considerable psychosocial distress in school. The patient underwent extensor indicis proprius tendon release, dorsal wrist capsular release, proximal row carpectomy, and total wrist arthrodesis to neutralize the wrist. One-year after surgery, the patient's function and psychosocial status improved. The remaining finger contractures are being addressed with staged tendon lengthening procedures. The management of Beckwith-Wiedemann syndrome hand pathologies requires a tailored approach, likely involving individualized preoperative planning, incremental treatments, and frequent reassessments.