Cyst Masquerading as Inadvertent Bleb After a Scleral-Fixated Intraocular Lens in Marfan Syndrome: A Case Report

Introduction: To present a case of an epithelial inclusion cyst masquerading as an inadvertent bleb in a patient with Marfan syndrome.

Case report: A woman with Marfan syndrome presented with a subluxed crystalline lens in her right eye, which progressively subluxed over the following 2 years. A lensectomy was performed with placement of an anterior chamber intraocular lens (IOL); however, the patient experienced blurred vision and photopsias and preferred IOL explantation. The IOL was removed and a scleral-fixated posterior chamber IOL was placed. Vision improved with an uncomplicated postoperative course. Five months later, the patient experienced sudden onset redness and sharp pain in this eye. A 3-mm cystic lesion with tan material was found over a prior scleral incision site. Intraocular pressure was normal and no aqueous leaked from the lesion. Owing to concerns of an infected inadvertent bleb, treatment with topical and oral antibiotics was started, but the lesion did not change in appearance and the patient experienced persistent pain. The lesion was surgically excised and histopathology revealed a conjunctival epithelial inclusion cyst with intralesional keratin. A month later, another conjunctival inclusion cyst developed and was excised per patient preference.

Conclusion: Marfan syndrome is characterized by defects in the FBN1 gene and may theoretically lead to an abnormal sclera, increasing the risk of bleb formation after scleral incision. Distinguishing between a filtering bleb and an epithelial inclusion cyst is critical in patient care. Although retained keratin from a cyst may mimic a bleb with purulence, intraocular pressure, aqueous leakage, and response to topical antibiotics may help distinguish between the two.