Use of expandable corpectomy trial and cage for reduction of neurofibromatosis associated cervical swan neck deformity

Abstract

Originally recognized by German pathologist Frederick von Recklinghausen in 1882, neurofibromatosis type I (NF1) is an autosomal dominant disease generated from pathogenic mutation in the NF gene on chromosome 17q11.2 that affects approximately 1 in 3,000. The incidence of cervical spine abnormality in patients with NF1 is not well defined as frequently patients are asymptomatic. Severe cervical spine deformity in NF1 is currently only reported on the case series and case report level. Included in these series are cases of cervical swan neck deformity (CSND). Since their introduction in the early 2000 s, expandable corpectomy implant technology has demonstrated their efficacy in reconstruction of the cervical spine and has been used to treat trauma, metastases, myelopathy, and cervical deformity. We describe a novel technique for use in reduction and reconstruction in NF1 associated CSND including caspar pin distraction, cervical traction and serial expansion of an expandable corpectomy trial.

Keywords

• Neurofibromatosis type 1
• Cervical swan neck deformity
• Cervical kyphosis
• Myelopathy
• Expandable corpectomy cage