Functional shoulder girdle reconstruction in paediatric desmoid tumour

Abstract

Desmoid tumours are rare fibroblastic neoplasms that, although benign, may be locally aggressive, invasive, and disabling. In children with Gardner syndrome, they typically arise in the abdominal wall, whereas scapular involvement is uncommon and surgically challenging because of the risk of scapular winging after resection of stabilizing muscles. We report the case of a 4-year-old boy with Gardner syndrome and multifocal desmoid disease, including a large tumour of the left shoulder girdle. Radical resection required sacrifice of several stabilizers, and scapular stability was preserved using a dynamic reconstructive technique: a trapezius flap elongated with lumbosacral fascia, passed through a bone tunnel in the scapula and anchored to adjacent muscles. Despite recurrence requiring re-excision and chemotherapy, the patient remained disease-free at 4-year follow-up, with preserved scapular stability. This case underscores the importance of dynamic reconstruction techniques, avoidance of mutilating resections, and multidisciplinary management in paediatric multifocal desmoid disease.