Background: Hemangioblastomas are benign (World Health Organization Grade I), highly vascular neoplasms commonly associated with Von Hippel-Lindau (VHL) disease.[2] The VHL tumor-suppressor gene, located on chromosome 3, is implicated in sporadic cases and cases associated with VHL disease. Hemangioblastomas most commonly arise in the posterior fossa; however, they may also be found supratentorially or within the spinal cord.[3] Surgical intervention is indicated for symptomatic lesions with a goal of complete resection of the enhancing nodule.[1].
Case description: We demonstrate the case of a 69-year-old man with a history of multiple hemangioblastomas who had undergone two previous craniotomies and Gamma-Knife radiosurgery (Video https://drive.google.com/file/d/1lUwsb80NLmIW2Enp-DVdtM9_Oqbid3Ih/view?usp=sharing). He presented with progressive imbalance and diplopia and was found to have a new lesion within the suprasellar cistern. Digital subtraction angiography (DSA) and magnetic resonance imaging (MRI) characteristics were typical of hemangioblastoma. Surgery was determined to be indicated, with a goal of vision preservation. Preoperative embolization was not possible because preoperative DSA demonstrated vascular supply by only small perforators directly from the internal carotid artery. Hypopituitarism was identified preoperatively, although diabetes insipidus was not present. The patient underwent a right orbitozygomatic craniotomy and extradural anterior clinoidectomy for access. The tumor was noted to encapsulate the infundibulum, which necessitated its sacrifice. Postoperatively, the patient remained at his neurologic baseline. He had a positive triphasic diabetes insipidus response and was discharged home on maintenance desmopressin. Postoperative MRI demonstrated complete lesion resection.The patient gave informed consent for treatment and video recording. Institutional review board approval was deemed unnecessary.
Conclusion: This video highlights a safe and effective surgical technique for suprasellar lesions as well as the complex anatomy observed through an orbitozygomatic approach.
Keywords: Craniotomy; Hemangioblastoma; Infundibulum.
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