Irreducible thoracic spondyloptosis in a child with neurofibromatosis: a rationale for treatment

Abstract

Study design: A case report and review of literature are presented.

Objectives: To describe the surgical management of a midthoracic spondyloptosis associated with kyphosis in a child with neurofibromatosis, an extremely rare but a potentially high-morbidity complication.

Summary of background data: Dystrophic kyphoscoliotic spinal deformity is the most common orthopedic sequela of neurofibromatosis. Spondyloptosis is a rare complication but with the potential for high morbidity if the diagnosis is missed or undertreated. Reported cases are rare.

Methods: A severe thoracic spondyloptosis occurred in a 7-year-old girl with peripheral neurofibromatosis who presented with transient paraparesis after a fall. The kyphosis was reduced by cantilever correction forces, achieving side-to-side (bayonet) apposition rather than anatomic reduction of the spondyloptosis. This was followed by anterior spinal arthrodesis and structural grafting.

Results: Two and a half years after the surgery there is no loss of correction, and the patient has remained neurologically recovered.

Conclusions: Posterior correction of the gibbus in a bayonet apposition and stabilization with a two-rod construct followed by anterior spinal arthrodesis and structural grafting seem to offer efficient surgical treatment.