[Histiocytosis-X of the orbit (author's transl)]

After reviewing the syndrome of histiocytosis X, the special problems of an attack on the eye are discussed with regard to clinical, histological and radiological findings. By means of two cases, all aspects important for the diagnosis and treatment of histiocytosis X are discussed with reference to the literature. A histologically verified histiocytosis X in the area of the orbita is described in an almost 2 year old girl and another girl aged 7 years and 3 months. In both cases, the disease began with an edema of the upper eyelid and swelling of the lateral orbital margin. The second case showed in addition the clinical symptoms of a dacryoadenitis. The X-ray pictures of the skull revealed a large triangular osseous lesion in the area of the orbital margins. Blood chemistry, hematological tests and the bonemarrow puncture did not yield any pathological findings. The diagnosis of histiozytosis X could be made only by a biopsy from the tumor area. The swelling in the area of the eyelids and the lateral orbital margin disappeared under cytostatic therapy with Vincristin and Prednison.