RESUMO
Hemangioblastomas are benign World Health Organization grade 1 tumors that are relatively rare.1 They may be sporadic or found in association with von Hippel-Lindau disease. Posterior fossa hemangioblastomas arise in the cerebellar hemisphere and, less commonly, at other sites such as medullary hemangioblastomas.2-4 Their characteristic radiologic features including solid-cystic morphology and prominent vessels aid in the preoperative diagnosis.5 In this operative video, we discuss the technical nuances and steps to avoid complications while operating on a large medullary hemangioblastoma. A 19-year-old lady presented with headache, vomiting, and visual deterioration of 2 months' duration. On examination, she had visual acuity of 4/60 right side, 6/60 left side, bilateral papilledema, and truncal and gait ataxia. Since she had presented in altered sensorium and a computed tomography head scan showed hydrocephalus, a ventriculoperitoneal shunt was placed on an emergency basis. Proper radiologic workup was done. On the basis of radiologic findings, she was provisionally diagnosed to have hemangioblastoma and surgery was planned. Preoperative angiogram showed an intense tumoral blush, but embolization was deemed infeasible since the tumor had multiple feeding vessels. Definitive surgery for tumor excision was performed 5 days after the shunt surgery. The patient's family consented to the procedure and the use of images and clinical data for publication. Video 1 highlights the tumor location, its prominent blood supply and venous drainage, use of intraoperative neuromonitoring, and the technical nuances for an en bloc excision. The patient had a left lateral gaze (sixth nerve) palsy in the immediate postoperative period, which resolved over the next 3 months. Postoperative and follow-up imaging confirmed complete tumor excision. Modern microsurgical techniques and a thorough understanding of the tumor vasculature help in performing safe and complete excision of such tumors.1,2 En bloc excision remains the mainstay of surgery for hemangioblastomas.
