Introduction: Hemangioblastomas [75% sporadic, 25% with Von Hippel Lindau(VHL) disease] are highly vascular, benign lesions. The surgical nuances, management, and complication avoidance of brain-stem hemangioblastoma(BH) have been studied.
Methods: Over 18 years, 27(mean age:29 years; range 15-60years) consecutive cases of BH underwent microsurgical excision. All patients were assessed clinic-radiologically for neurological deficits and screened for VHL syndrome. Outcome of the patients was based on Karnofsky Performance Status(KPS).
Results: 12 out of 19(70.4%) patients with hydrocephalus underwent a cerebrospinal fluid(CSF) diversion procedure. Lower cranial nerve palsy was present in 10(37%) patients and motor weakness in 13(48%). The tumors [mean size 3.34+1.06cm, range:1.4-5.5cm; 11solid, rest solid-cystic; 18(66.7%) subpial and 9(33.33%) intramedullary] were divided into four categories based on size: A:<2cm(n=5,18.5%); B:2-3cm(n=10,37%); C:3-4 cm(n=6,22.2%); D:>4cm(n=6,22.2%). Their location was at posterior cervicomedullary junction(n=12); pontomedullary junction(n=7); pons(n=3), medulla(n=3) and ponto-mesencephalic region(n=2). Multiple flow voids were seen in >50% patients with tumor> 2cm. 5 patients had syringomyelia; and, 8 had diffuse cervical cord expansion. Two patients with a large vascular tumor underwent preoperative embolization. Six patients had VHL syndrome; one underwent bilateral adrenalectomy for refractory hypertension; and, the another, nephrectomy for renal cell carcinoma. Twenty-six patients underwent a midline suboccipital craniectomy; and, 1 with a cerebellopontine angle tumor, a retromastoid craniectomy. 15 patients underwent total excision; 10 patients, near-total (<10% remaining) excision, and 2 patients, a subtotal (>10% remaining)) excision. Three patients (2 with VHL syndrome) expired due to exsanguinating hemorrhage, spreading venous thrombosis and aspiration pneumonitis, respectively. At follow-up visit (median:25+interquartile range 2-56months), 17 patients had improved KPS, 4 remained in same status and 3 (recently operated on tracheostomy) had worsened KPS.
Conclusions: Significant improvement is achievable in neurological status in patients following successful extirpation of brain-stem hemangioblastoma, despite a turbulent perioperative period. Leaving tumor capsule adherent to the brain-stem often helps in preserving brain-stem function. Postoperatively, the patients should be monitored for their respiratory and lower cranial nerve status to prevent aspiration pneumonitis.
Patient Care: Our research identifies safe surgical practices to improve post operative outcomes in brainstem hemangioblastomas.
Learning Objectives: Significant neurological improvement is achievable in patients following e successful excision of brain-stem hemangioblastomas. The patients may require significant perioperative management. In the presence of VHL syndrome, management of abdominal masses and/or pheochromocytoma may be required. Draining veins of the tumor must be preserved until the majority of the tumor has been addressed. Leaving a small bit of the tumor capsule adherent to the brain-stem often helps in preserving brain-stem function. The patients should be regularly monitored for their respiratory and lower cranial nerve functional status.