Surgical Management of "Kissing" Spinal Plexiform Neurofibromas in Neurofibromatosis Type 1 Patients.

BACKGROUND: "Kissing" neurofibromas (KNs) are a unique group of spinal tumors found in neurofibromatosis type 1 (NF1) patients. These are bilateral neurofibromas that approximate each other at the same level, with significant impingement compression of the cord or thecal sac. The best management options and surgical strategies for NF1 patients with KN have not been standardized.

METHODS: We conducted a retrospective study evaluating adult NF1 patients with KN. All patients are followed routinely at the Gilbert Israeli NF Center. Patients' files were reviewed for natural history, imaging features, surgical technique, and surgical outcome.

RESULTS: Twelve patients with at least 1 pair of KN were identified (6 females). Median age at spinal presentation was 24 (range 17-48). KNSs were located at the cervical (n = 8) and lumbar (n = 8) region, with no thoracic involvement. Seven of the 12 patients were operated; all underwent surgery due to cervical compression with progressive myelopathy. Four patients remained asymptomatic during the follow-up period. Three patients underwent multiple operations. Operative outcome was favorable in 71% of patients, with marked overall motor improvement or stabilization of neurologic deterioration. Two patients who entered surgery with a low functional reserve deteriorated after surgery.

CONCLUSIONS: In our series, KN caused progressive cord compression in 7 of the 8 patients with cervical tumors. No intervention was needed for lumbar tumors. Cervical tumors should be followed closely, with a low threshold for intervention. NF1 patients harboring KN should be followed both clinically and radiologically for life.