3 Capillary Hemangioma of the optic disc

The diagnosis of an angioma of the optic disc is not always easy. Up to the
1970's these cases were usually diagnosed after removal of the eye either
because of secondary complications or because the lesion was considered
clinically as a malignant melanoma. In 1972 Oosterhuis and Rubinstein [38]
described the clinical and fluoroangiographic characteristics of hemangiomas
of the disc and predicted that improved diagnostic methods would bring to
light more cases.

I. Classification of angiomatous tumors of the optic disc

Three main types of angiomatous tumors of the optic disc may be con-
sidered [3]:
1. Capillary angioma. Capillary angiomas of the optic disc mayor may not
be associated with retinal angiomatosis and are considered as a peculiar
form of von Hippel's disease.
2. Cavernous angioma. Cavernous angiomas of the optic disc are very rare.
They may also be associated with a cavernous hemangioma of the retina.
3. Angioma racemosum. In such cases the involvement of the optic disc is
only part of the fundus lesion.
In this chapter the discussion will be limited to capillary angiomas of the
disc. Cavernous angiomas will be dealt with in chapter 4 and angioma
racemosum in chapter 5.

II. Incidence

Schindler et al. [45] traced 58 cases of hemangiomas of the disc published

from 1912 to 1975 and added seven additional cases. Since then at least 30
other cases were published [1, 3, 7,12-14,21,26,27,35-37,41,47,50,52].
59% of the patients are males and 41% are females. In 52% only the
right eye is affected, in 40% only the left eye and in 8% bilateral involve-
ment was noticed [45]. Bilateral cases were described by a number of
authors [9, 18, 20, 21, 30, 48].

55

J. J. De Laey et al., Vascular Tumors and Malformations of the Ocular Fundus

© Belgian Society of Ophthalmology, Leuven, Belgium and Kluwer Academic Publishers 1990
56 Chapter 3

III. Clinical features

Capillary hemangiomas of the optic disc are usually diagnosed during the
third decade, in 53% of the cases because of visual loss and in 30% on
routine examination in patients without subjective signs. The other cases
were found after enucleation for secondary glaucoma or leucocoria or
during a systematic investigation of members of families with von Hippel-
Lindau's disease. The earliest clinical aspect is probably that of a flat vascular
lesion, better detected by fluorescein angiography [26]. An angioma of the
disc was even an incidental finding during autopsy in a patient with von
Hippel-Lindau, where the retina and optic nerve had been clinically con-
sidered as normal [35].

Figure 3 -1. Optic disc hemangioma on the right optic disc in a 62 year old woman. a. aspect
in redfree light. b, c. fluoroangiography. The uninvolved part of the optic disc does not stain in
the late sequence of the fluoroangiography .