Retinal angioma of Von hippel-lindau disease: A case report
O Nabih1, H Hamdani1, L El Maaloum2
1Medical Resident at Pediatric Ophthalmology Department, Hopital 20 Aout, 1953, Casablanca, Morocco.
Introduction:
Von Hippel-Lindau disease (VHL), also known as Von Hippel-Lindau syndrome, is a rare genetic disorder with multisystem involvement. It is characterised by the development of multiple vascularised tumours, particularly cerebellar, retinal and/or visceral. The disease can occur at any age and usually starts with retinal hemangioblastomas.
Case Report:
We report the case of a 45-year-old female patient with no particular pathological history, who. consulted the ophthalmology department for a change of optical correction.The funds examination showed an uncomplicated bilateral hemangioma with no other associated signs. Fluorescein angiography confirmed the diagnosis by showing in the left eye a multiple retinal hemangioma visible in the mid-periphery facing the branches of the superior temporal arches. The brain MRI showed a multifocal hemangioblastoma in the posterior cerebral fossa. A renal ultrasound returned normal. The patient had undergone photocoagulation of the retinal lesions to avoid any complications.
Discussion:
The German ophthalmologist Eugen von Hippel first described angiomas in the eye. The term Von Hippel-Lindau disease was first used in 1936; however, its use became common only in the 1970s.Tumours called hemangioblastomas are characteristic of von Hippel-Lindau syndrome. These growths are made of newly formed blood vessels and occurs in the periphery of the retina. Spontaneous progression occurs leading to visual impairment as a result of maculopathy or exudative retinal detachment.Early recognition and treatment of specific manifestations of VHL can substantially decrease complications and improve quality of life.Conventional treatment of the retinal hemangioblastomas is laser photocoagulation or cryotherapy depending on the location and size of the lesions. It must be based on the patient's visual symptoms and tumor progression.
Conclusion:
Management of patients with VHL disease often requires a multidisciplinary approach. The role of the ophthalmologist is important in the management of this condition since the ocular involvement may be indicative of the disease.
