Uninstrumented fusion in cervical kyphosis due to neurofibromatosis type I: report of two paediatric cases.

ABSTRACT

PURPOSE: Severe cervical kyphosis (CK) in neurofibromatosis type 1 (NF-1) is associated with a high risk for progression and neurologic impairment in children. We present our surgical technique and mid-term outcomes of uninstrumented anterior tibial strut grafting for severe CK secondary to NF-1. METHODS: Case report. The Consensus-based Clinical Case Reporting Guideline Development (CARE) guidelines were followed. RESULTS: Two paediatric patients (8- and 3-year-old) presented with severe CK secondary to NF-1. A halo body jacket (HV) allowed the progressive distraction of the cervical spine, avoiding neurological compromise and deformity progression. Circumferential fusion was obtained with anterior tibial strut autograft and posterior onlay bone graft. Cervical spine fusion was successfully maintained at a minimum 4-year follow-up in both patients. CONCLUSION: In children with severe CK secondary to NF-1, cervical distraction and immobilisation with a HV followed by uninstrumented anterior tibial strut grafting and posterior bone grafting, provided spinal fusion and stability without increasing the risk of neurological injury and donor site morbidity. The reported surgical technique appears to be a valuable tool in the armamentarium of the spinal surgeon.