Surgical management of complicated retinal detachment in a case of retinal hemangioblastoma.

Background: A 40-year-old male presented with a complaint of sudden onset diminution of vision in the left eye for 2 weeks. He was a follow-up case with retinal hemangioblastoma in both eyes. He underwent two sittings of fundus fluorescein angiography-guided trans-pupillary thermotherapy 2 years back. Since then, he was regularly followed up for 2 years with stable vision and stable retinal findings. At present, the best-corrected visual acuity (BCVA) in the right eye is 6/6, and in the left eye, it is counting fingers 2 meters. On fundus examination, he had one active hemangioblastoma in the right eye and total retinal detachment in the left eye with multiple active lesions. The right eye was treated with a single sitting of thermotherapy, and the left eye underwent pars plana vitrectomy and angioma excision, followed by silicone oil tamponade. The immediate and late post-operative periods were uneventful, with successful anatomical and functional outcomes. The left eye BCVA on late follow-up was 6/36, no further treatment was advised, and the patient was kept under follow-up and observed closely. Purpose: : To educate regarding the systemic workup, diagnosis, and surgical management of complicated retinal detachment in retinal hemangioblastoma. Synopsis: : Systemic workup, diagnosis, and surgical steps in the management of complicated retinal detachment in retinal hemangioblastoma were performed. Highlights: : Close follow-up, keen observation, and prompt treatment in the early stages of the disease are indispensable to prevent untoward sequelae of retinal hemangioblastoma. A thorough systemic workup is necessary to diagnose the systemic involvements early. Surgery, if indicated for the retinal hemangioblastoma or its associated sequelae, should be performed diligently and with careful handling of blood vessels and anomalous tissues. Online Video Link: https://youtu.be/CkoqWEnaPB8.

Suprasellar hemangioblastoma.

A 59-year-old woman presented with disturbance of consciousness and decreased visual acuity caused by a suprasellar mass identified on MRI. A bifrontal interhemispheric approach allowed removal of the top and lateral sides of the tumor from the wall of the third ventricle. The hypothalamus appeared to be the origin of the mass, which proved to be hemangioblastoma, a rare tumor in this location.

Diagnosis and surgical treatment of brainstem hemangioblastomas.

OBJECTIVE: The study aims to elucidate the advance of diagnosis and surgical treatment of brainstem hemangioblastomas (BSHs). METHODS: The data of the following patients treated in one institute were retrospectively analyzed: (1) patients with a single tumor on the brainstem which was verified by surgery and pathology; (2) patients without von Hippel-Lindau disease or multiple hemangioblastomas. RESULTS: Thirty-three patients with BSHs were identified, accounting for 15.5% of all intracranial hemangioblastomas surgically treated from August 1989 to May 2002 in Huashan Hospital. There were 17 males and 16 females. The patients were aged from 16 to 65 years with an average age of 45 years. The clinical manifestations were nonspecific. Magnetic resonance imaging and digital subtraction angiography were the major diagnostic modalities. Tumors were located on oblongata (14), ponto-oblongata (9), pons (6), and cervicomedulla (4). Tumors were solid in 29 cases, cyst in 4 cases, and had a small size in 5 (< or =3 cm), large in 19 (3.1-4 cm), and giant in 9 (>4 cm). Extra-brainstem (EBS) type (including the fourth-ventricle hemangioblastomas) was seen in 25 cases, and intrabrainstem (IBS) type in 8 cases. Preoperative embolization was performed in 12 cases since 1996. Mild hypothermia with or without hypotension was done during the operation in 10 cases. Total tumor removal was achieved in 31 patients (94%), and incomplete removal in 2 cases. Two patients with EBS type and giant solid tumors died after operation. Follow-up study (range, 1-12 years; mean, 5 years) was available in 31 patients. Karnofsky performance scale scores were > or =80 in 25 patients (80.6%), 60 to 70 in 4 patients (12.9%), and 40 to 50 in 2 patients (6.5%). CONCLUSION: Two types of BSHs can be identified. Patients with cystic IBS type could obtain excellent outcome after operations. Patients with giant or large solid BSHs remain a challenge to neurosurgeons. A combined strategy of preoperative embolization, mild hypothermia with or without hypotension, microsurgical technique, and intensive perioperative management are mandatory for removal of these kinds of tumors with acceptable morbidity and mortality.

Hypothalamic hamartoma and the Pallister-Hall syndrome.

The Pallister-Hall syndrome (PHS) was initially described as the congenital hypothalamic 'hamartoblastoma' syndrome in 1980. Cardinal manifestations of the syndrome consist of a hypothalamic hamartoma and extracranial abnormalities, initially thought to be fatal in the perinatal period. The original pathologic description of these hypothalamic lesions were from infants who died in the perinatal period and revealed small cells of variable density which resembled primitive undifferentiated germinal cells and appeared to invade the hypothalamic nuclei, suggesting a neoplastic potential. Hypothalamic lesions have now been removed from older infants and children with this syndrome and reveal a more mature histologic appearance typical of a hypothalamic hamartoma. We present 2 new cases of PHS who underwent surgery and demonstrate the maturational nature of the hypothalamic lesion and the phenotypic variability of the syndrome.

[A case of hemangioblastoma in the thalamus].

Supratentorial hemangioblastoma is encountered very rarely. About 80 cases in all have been reported. The authors present a case of supratentorial hemangioblastoma in the thalamus with gradually enlarging cysts. The patient was a 45-year-old man with complaints of left hemiparesis and headache. Computed tomographic scans of the brain showed a cystic mass with mural nodule in the right thalamus. Left vertebral angiography demonstrated a vascular tumor fed by a thalamogeniculate artery. A right temporo-parietal craniotomy was performed and the tumor was totally resected through the transcortical approach. The histological diagnosis was hemangioblastoma. Post-operative course was good except for a minor complication of the left lower quadrant homonymous hemianopsia and left hemiparesis which had disappeared at the time of discharge.