Juvenile xanthogranuloma of the iris in an adult.

Juvenile xanthogranuloma is a self-limited skin disorder of young children that uncommonly affects the eye. Juvenile xanthogranuloma has been described in adults, but reported intraocular involvement is extremely rare. We report a case of juvenile xanthogranuloma diagnosed in a 25-year-old man who was seen with nontraumatic hyphema and iridocyclitis. Diagnosis was made from a biopsy specimen obtained from a suspicious skin lesion. Topical and systemic steroids, radiation therapy, and finally immunosupression were required to eliminate the iris tumor clinically and resolve the patient's recurrent symptoms.

[Radiotherapy in juvenile xanthogranuloma of the iris]. / Strahlentherapie bei juvenilem Xanthogranulom der Iris.

Nevoxanthoendotheliomas (and juvenile xanthogranulomas) are very rare benign skin diseases which occur in infants. An ocular manifestation, mainly in the iris, has been found in approximately. 10% of the cases, The clinical picture is characterized by a recurrent unilateral hemorrhage of the anterior chamber, which is the reason for seeing an ophthalmologist. The clinical diagnosis of juvenile xanthogranuloma is based on the characteristic iris changes - heterochromia, circumscribed vascularized tumor, or diffuse thickening of the iris. Sometimes elevated intraocular pressure is also found. In combination with a mild local steroid, radiotherapy with 300 to 400 cGy should be started immediately in order to prevent a sometimes uncontrollable secondary glaucoma. The good results achieved in 5 of the authors' own cases underline the value of low-dose radiotherapy in this disease: the affected eye was healed in all 5 cases.

Juvenile xanthogranuloma of the iris managed with superficial radiotherapy.

A case of juvenile xanthogranuloma involving the iris successfully managed with superficial radiotherapy is presented. A review of the literature suggests that this benign condition, if progressive or extensive, is optimally treated with radiotherapy as an alternative to major surgical procedures, including enucleation.

[Results of radiotherapy in case of juvenile xanthogranuloma of the iris]. / Ergebnisse der Strahlentherapie bei juvenilen Xanthogranulomen der Iris.

The juvenile xanthogranuloma is a rare, benign, cutaneous disease occurring in infants and young children which can be situated in about 10% of the cases on the eye and preferentially on the iris. The reason for consulting an ophthalmologist is mostly a recurrent unilateral hyphemia which is typical for this disease. The diagnosis of the juvenile xanthogranuloma has to be established clinically, based on the characteristic alterations of the iris--heterochromia, vascularized tumor or diffuse thickening of the iris--and the possibly increased intraocular pressure. In order to avoid the deleting consequences of an uncontrollable secondary glaucoma, radiotherapy with a total dose of 300 to 400 cGy combined with a mild local steroid treatment has to be undertaken immediately, if possible. Taking into consideration the indications of literature, the value of a low-dose radiotherapy for this disease shall be demonstrated by five own cases in all of which the concerned eye could be healed and preserved.

[Radiotherapy of the juvenile xanthogranuloma of the iris (author's transl)]. / Zur Strahlentherapie des juvenilen Xanthogranuloms der Iris.

A report is given on an eight months old child with a juvenile xanthogranuloma of the iris and a secondary glaucoma. After radiotherapy with 6 X 0,5 Gy under conventional deep therapy conditions, a soon regression of the increased intraocular pressure was observed. In case of a juvenile xanthogranuloma of the iris, this secondary glaucoma represents in any case an acute danger to the eye, so it must be treated immediately. Radiotherapy has to be considered as the method of choice; it is also discussed with regard to the risk and the avoidance of a radiation cataract which, however, has to be tolerated in certain cases in order to prevent greater troubles.