Von Hippel-Lindau disease : The first case of Songklanagarind Hospital
Sarinya Puwanant, D. of Internal Medicine, F. of Medicine, PSU.
Suwanna Sinsawaiwong, D. of Internal Medicine, F. of Medicine, PSU.
Mansing Ratanasukon, D. of Ophthalmology, F. of Medicine, PSU.
Corresponding e-mail : ssuwanna@ratree.psu.ac.th
Presented : The 16th Annual Academic Meeting, Faculty of Medicine, PSU, Hat Yai, Songkhla,
16-18 August 2000
Key words : Von Hippel-Lindau disease (VHL), central nervous system (CNS), retina
Von Hippel-Lindau disease (VHL) is an uncommon inherited disease. The inheritance is auto-somal dominant with variable penetrance. The classical feature is vascular tumor of central nervous system (CNS) and retina. The 1982-1999, Medical record of Songklanagarind Hospital revealed 9
cases of CNS hemangio-blastoma. Eye examinations were performed and in three of nine patients showed normal. The other six patients were not examined. There is no data of VHL registered in our hospital. The age of onset of the CNS hemangioblastoma in VHL is younger with multiple sites, higher recurrent rate and poorer prognosis than sporadic case. Genetic counselling and early screening tests are the most useful methods for early detection and therapy. We present a case of VHL who had a
family history and was completely investigated.
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