Peripheral capillary haemangioma (von Hippel tumour)
 

A von Hippel's tumour showing large feeder and drainage vessels to and from the angioma

There may be macular exudate which points to the presence of peripheral retinal abnormalities or the presence  of large calibre vessels which when followed peripherally shows the presence of the vascular lesion. There is a vascular lesion (avoid using the word 'tumour' in the presence of the patient which may upset him/her)  with dilated feeder and drainage vessels. There may be exudate around the lesion or in the macula. Look for: any abnormal vascular changes in the optic disc which may be caused by optic disc haemangioma any cryotherapy or photocoagulation scars in the same or fellow eye. mention you like to measure the blood pressure as capillary haemangioma is associated with von  Hippel-Lindau's syndrome in 25% of cases mention you like to examine the abdomen (especially at the flank) for any scar which may indicate  previous operation for phaeochromocytoma or renal carcinoma (again seen in von Hippel-Lindau's syndrome).

Questions:

1. What is the mode of inheritance in von Hippel-Lindau's syndrome?

Answer
Autosomal dominant and the abnormal gene is located on chromosome 3q

2. What are the systemic features of von Hippel-Lindau's syndrome?

Answer
cerebellar haemangioblastoma which may in turn lead to polycythaemia
visceral cysts
phaeochromocytoma
renal carcinoma

3. How would you treat the haemangioma?

Answer
Untreated, the lesion will continue to grow leading to more leakage and exudate. The lesion should be treated early with either laser photocoagulation or cryotherapy.

 

Return to the main page