Nerve.  2023 Oct;9(2):195-202. 10.21129/nerve.2023.00409.

Surgical Management of Recurrent Plexiform Neurofibroma in a Pediatric Patient with Severe Cervical Kyphotic Deformity: A Case Report

• Jung S
• Kang D
• Kim D
• Lee SH

• KMID: 2547519
• DOI: http://doi.org/10.21129/nerve.2023.00409

Abstract

Approximately 50% of patients with neurofibromatosis type 1 (NF1) develop orthopedic complications, and spinal deformities are common manifestations. Cervical kyphosis is comparatively rare in NF1; however, it results in spinal cord compression associated with paralysis and respiratory dysfunction, requiring surgical correction. Pediatric patients with NF1 usually have small and defective pedicles or lateral masses, and surgery with a single approach is limited to ensure sufficient spinal cord decompression and deformity correction. However, no reliable treatment guidelines are available for this challenging condition. This case report presents a 7-year-old patient with NF1 who had severe cervical kyphosis with intradural extramedullary neurofibromas. The tumors were removed before correcting the deformity to decompress the spinal cord and reduce the risk of spinal cord injury. Moreover, we effectively corrected and stabilized the kyphosis using the anterior-posterior-anterior approach.

Keyword

Plexiform neurofibroma; Cervical; Kyphosis; Pediatric; Correction