Bhuyan Mrinal, Dutta Deep, Baishya Basanta Kumar, Hussain Zakir
Department of Neurosurgery, Gauhati Medical College, Guwahati, Assam, India.
Asian J Neurosurg. 2016 Jul-Sep;11(3):311. doi: 10.4103/1793-5482.179644.
A 45-year-old gentleman presented with headache, dizziness, and unsteadiness of gait. On imaging, he was found to have a cerebellar and a spinal intramedullary hemangioblastoma. To rule out Von Hippel-Lindau (VHL) disease, we did screening of the whole abdomen with ultrasonography followed by contrast-enhanced computed tomography of the abdomen, and surprisingly, the patient was having bilateral adrenal pheochromocytoma and a hepatic cyst. A diagnosis of VHL disease was made on this basis. The patient was operated and he made a very good recovery. We are reporting this interesting case because of its typical findings and its rarity. Disease management and review of literature were also discussed at the end.
一位45岁男性因头痛、头晕及步态不稳前来就诊。影像学检查发现他患有小脑及脊髓髓内血管母细胞瘤。为排除冯·希佩尔-林道(VHL)病,我们先对全腹部进行了超声筛查,随后进行了腹部增强计算机断层扫描,令人惊讶的是,该患者患有双侧肾上腺嗜铬细胞瘤及一个肝囊肿。基于此做出了VHL病的诊断。患者接受了手术,恢复情况良好。我们报告这一有趣病例是因其典型表现及罕见性。最后还讨论了疾病管理及文献回顾。