A 13-year-old girl presented with loss of VA in both eyes (0.1 RE and 0.2 LE).
The examination of the eye fundus showed the presence of bilateral QMS.
Fluorescein angiography showed diffuse pigmentary epitheliopathy with exudative edema and secondary detachable thickening.
At 5 months of age, she had been successfully treated with Langerhans cell histiocytosis. A complete systemic study including cranial and orbital imaging tests was performed.
No significant findings were found.
Therapy with topical, peribulbar and oral corticosteroids was initiated.
Peribulbar triad injections were repeated four times during the following year, achieving only transient improvements in macular edema and serous detachments.
In September 2002, treatment with oral cyclosporine was started, and in March 2003, cyclosporine was associated with persistence of CME.
Finally, 4 mg of intravitreal triamcinolone was administered to both eyes in July 2003, with initial improvement in QMS and VA.
Injections were repeated twice in the RE and once again in the LE.
In December 2004, IOP was 27 mmHg RE and 26 mmHg LE, so topical therapy with 0.5% thymolol and 0.02% dorzolamide (Cosopt®) was indicated.
In March 2005, the patient had severe bilateral VA loss (<0.05) due to the development of intumescent cataracts.
After phacoextraction surgery with intraocular lens implantation without complications, VA is 0.3 in both eyes.
