ABSTRACT(approximately 0.2% of all cases reported in the last 20 years in Beijing Tiantan Hospital).
█ mATERIAl and mEThODS
PatientsTen pediatric patients (6 male and 4 female patients) who were admitted from January 1994 to December 2014 and diagnosed with unilateral vestibular schwannomas were █ INTRODUCTION V estibular schwannomas, which originate from the vestibular division of cranial nerve VIII, occur more often in adults than children. Identification of a sporadic or non-neurofibromatosis type 2 vestibular schwannoma in a child is extremely rare (1-7). When a vestibular schwannoma is found in a child, neurofibromatosis should be the first differential diagnosis. We reviewed 10 cases involving <16-yearold patients without clinical evidence of neurofibromatosis AIm: We investigated the clinical presentation and management issues of unilateral vestibular schwannomas in childhood without evidence of neurofibromatosis. mATERIAl and mEThODS: Ten pediatric patients with unilateral vestibular schwannoma and no evidence of neurofibromatosis were treated by the senior author at Tiantan Hospital from January 1994 to December 2014. The clinical manifestations, neuroimaging findings, treatment methods, and therapeutic results were retrospectively reviewed.
RESUlTS:The study included 6 male and 4 female patients. The mean age of the patients was 13.9 years (range, 11-15 years). Common clinical features included increased intracranial pressure, hearing loss, and ataxia. The median diameter of the tumors was 5.15 cm. Gross total resection was achieved in 9 of 10 patients via a suboccipital retrosigmoid approach, and the perioperative mortality rate was 10%. The follow-up period ranged from 1 to 12 years. One patient with facial nerve injury recovered to HouseBrackmann grade I postoperatively, 5 to grade II-III, and 2 to grade IV-V. Only 1 patient had preserved hearing.
CONClUSION:The clinical features of pediatric vestibular schwannomas are different from those of adult vestibular schwannomas, and cranial nerve preservation in children is more difficult. When a child presents with hearing loss as well as cognitive disability, a vestibular schwannoma should be highly suspected. Hypervascular vestibular schwannomas in childhood should be managed by multistaged surgeries.