Clinical and Therapeutic Features of Neurofibromatosis in Ghana

ABSTRACT

Introduction. Neurofibromatosis (NF) is a generalized form of benign tumors of the peripheral nerves involving the skin; subcutaneous tissue; and bone. It comprises neurofibromatosis type 1 (NF-1); neurofibromatosis type 2 (NF-2); and schwannomatosis. Objective. To document the clinical and epidemiological features of patients with NF presenting at Komfo Anokye Teaching Hospital (KATH) in Kumasi; Ghana; from January 2009 to December 2013; and the outcome of management. Patients and methods. This is a retrospective study. Data on all patients managed within the study period were retrieved from the records of the surgical outpatient department (SOPD) of KATH and theater records and analyzed. Results. Fifteen patients underwent surgery for function impairing and disfiguring lesions; comprising five males and 10 females. Their ages ranged from three years to 55 years; with a mean age of 27.3 years. Seventeen lesions were found on the 15 patients that required surgery 10 on the head and neck; three on the trunk; two on the upper limbs; and two on the lower limbs. Three of the lesions were neurofibromas; 14 were plexiform lesions. Fourteen patients had NF-1; only one patient had NF-2; there were no cases of schwannomatosis. Debulking was done for 12 patients; this was combined with suspension in two cases and enucleation in one case. Excision was done for five patients. Eight of the lesions recurred and the patients underwent a second debulking procedure. Conclusion. NF causes disfigurement and functional deficits in affected patients. Surgery can achieve symptomatic relief. Early presentation is recommended for optimal results: