Maurice F. Rabb » Swollen Optic Nervehead

This 6 year old white female returned for evaluation of a swollen left optic nervehead and decreasing visual acuity O.S. that occurred approximately 15 months after she underwent biopsy, diagnosis, and radiation treatment of embryonal rhabdomyosarcoma of the left orbit.

Initial radiation through a left anterior orbital and left lateral orbital approach totaled 6040 rads over a 57-day period. Erythema of the left upper eyelid developed during treatment. Thereafter, she was on a chronic course of chemotherapy included Vincristine for the initial four months following radiotherapy. At that time, she was also started on Actinomycin D for a series of 5-day courses occurring once every three months. She was also on chronic cytoxan 25mg per day, alternating with 15mg per day for the preceding 15 months. However, because of low blood counts, she was off the drug more often than on.

At the onset of her recent visual symptoms, vision was 20/20 OD and 20/60 OS. V OS had previously been normal. There was no afferent pupillary defect. The right fundus was normal. There left fundus showed multiple cotton-wool spots around the border of the left disc. There were tiny hard exudates in the left macula and a single blotchy hemorrhage of about 150 microns superonasal to the disc and about 4 or 5 disc diameters from it. Repeat orbital ultrasound and CT-scan showed no recurence of tumor, and a diagnosis of radiation-induced optic neuropathy and mild radiation-induced retinopathy was made (see photos). All medications were stopped.

Three months after discontinuation of her antimetabolite therapy, the best corrected vision in the left eye was 20/100+. The ophthalmoscopic appearance was virtually normal. The disc was pink and did not show the cotton-wool exudates seen acutely. The macula also looked normal. A repeat angiogram was performed (see photos).