Irreducible Thoracic Spondyloptosis in a Child with Neurofibromatosis: A Rationale for Treatment.
Hanna, B. Guirguis MD *; Pill, Stephan G. MS, PT *+; Drummond, Denis S. MD *+
[Report] Spine. 27(14):E342-E347, July 15, 2002.

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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.

(C) 2002 Lippincott Williams & Wilkins, Inc.