Título / Title

RETINAL CAPILLARY HEMANGIOBLASTOMA IN VON HIPPEL DISEASE TREATED WITH LASER PHOTOCOAGULATION AND CRYOTHERAPY

Introdução / Purpose

To report a case of Von Hippel disease with retinal capillary hemangioblastoma that was treated with photocoagulation and cryotheraphy

Material e Método / Methods

Case report

Resultados / Results

A 24-years-old white male, complaining of progressive vision loss on OD for 1 year. Past medical history is unremarkable. The best corrected visual acuity (BCVA) was counting fingers on OD and 20/20 on OS. Fundus examination showed a retinal hemangioblastoma superotemporal to the macula associated with dilated feeding and draining vessels on OS (Fig.1). OCT revealed intraretinal fluid extending from the hemangioblastoma towards the macula (Fig.2). There was no alterations on OS. Fluorescein angiography showed leakage from the hemangioblastoma. Systemic investigation, brain CT scan, and abdomen US were normal. Laser photocoagulation around the lesion and cryotherapy at the base of the tumor were performed. BCVA after 2 weeks was slight worsen due to the so-called "ablatio fugax", caused by treatment-induced retinal edema, which is transient. After 5 months of follow-up, the patient returned to his initial BCVA and there was involution of the peripheral tumor and reduction of the sub and intraretinal macular fluid (Fig.3). There was no improvement of the BCVA due to the development of atrophy and interruption of the subfoveal ellipsoid zone.(Fig.4)

Discussão e Conclusões / Conclusion

Although retinal capillary hemangioblastomas can occur as an isolated condition, most occur in association with Von Hippel–Lindau disease and can be one of the earliest manifestations. Laser photocoagulation, cryotherapy, surgical excision are modalities of treatment, depending upon tumor size, location and other findings. Our case showed regression of the tumor using cry and laser. Patients should be followed for life, since new tumors in the same or the fellow eye may appear later on. Systemic evaluation also is warranty for life since the systemic disease may be fatal unless diagnosed in a timely fashion

Palavras Chave

Hemangioblastoma; Von Hippel-Lindau; tumor