Craniotomy for cerebellar hemangioblastoma excision in a patient with von Hippel–Lindau disease complicated by uncontrolled hypertension due to pheochromocytoma

• This report describes a patient with VHL syndrome and uncontrolled hypertension due to pheochromocytoma who developed obstructive hydrocephalus and underwent craniotomy for the excision of a cerebellar hemangioblastoma combined with a laparoscopic adrenalectomy.
• A rare case of cerebellar hemangioblastoma excision in a patient with von Hippel–Lindau disease complicated by uncontrolled hypertension due to pheochromocytoma.
• There is hardly any literature on emergency craniotomy in a patient with von Hippel–Lindau disease.
• There are no clear treatment guidelines for the emergency excision of cerebellar hemangioblastomas in patients with intracranial hypertension complicated by abnormal hypertension due to pheochromocytoma whose blood pressure is not sufficiently controlled before surgery.

IntroductionThis report describes a patient with Von Hippel–Lindau (VHL) syndrome and uncontrolled hypertension due to pheochromocytoma who underwent craniotomy for the excision of a cerebellar hemangioblastoma combined with a laparoscopic adrenalectomy.Case reportA 31-year-old man presented with severe headache. MRI showed areas of abnormal enhancement in the left cerebellum that were determined to be hemangioblastoma with mass effect and obstructive hydrocephalus. His blood pressure rose abruptly and could not be controlled. CT of the abdomen revealed bilateral suprarenal tumors, and the patient was diagnosed as having VHL syndrome.On the third day, he presented with increasing headache, a decreased level of consciousness, and hemiparesis. We were not able to perform an craniotomy because abdominal compression in the prone or sitting position resulted in severe hypertension. We performed ventricular drainage to control his ICP. On the fifth day, we first performed a bilateral laparoscopic adrenalectomy to control ICP and then moved the patient to the prone position before performing a craniotomy to remove the left cerebellar hemangioblastoma.Discussion & conclusion In patients with pheochromocytoma, the effects of catecholamine oversecretion can cause significant perioperative morbidity and mortality, but these can be prevented by appropriate preoperative medical management. When carrying out an excision of cerebellar hemangioblastomas in patients with intracranial hypertension complicated by abnormal hypertension due to pheochromocytoma whose blood pressure is not sufficiently controlled, tumor resection of the pheochromocytoma prior to cerebellar hemangioblastoma excision in the same surgery may prevent increased ICP and reduce perioperative risk.