Abstract
PURPOSE: We describe a case of secondary open-angle glaucoma due to mucin-producing congenital iris stromal cyst in a 4 year old patient. OBSERVATIONS: A 4-year old female patient with a history of unilateral congenital iris stromal cyst presented with sudden-onset eye pain and redness, with markedly elevated intraocular pressure and evidence of early optic nerve damage. During the examination under anesthesia, the anterior chamber angle was open and there was no evidence of pupillary block. Ultrasound biomicroscopy revealed mildly echogenic substance filling the anterior chamber suspicious of mucoid material, which was verified by the inability to aspirate the material through a 25 gauge needle. The iris cyst was excised, and the intraocular pressure normalized spontaneously. Pathologic examination confirmed a mucin-secreting iris cyst lined with goblet cells and confirmed the mucogenic mechanism. CONCLUSIONS AND IMPORTANCE: This is the first reported case of mucogenic glaucoma in a pediatric patient. This rare entity should remain on the differential diagnoses of childhood glaucoma associated with nonacquired ocular anomalies. Surgical excision of the iris cyst may be curative.