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

Abstract

IntroductionTo present a case of an epithelial inclusion cyst masquerading as an inadvertent bleb in a patient with Marfan syndrome.Case ReportA 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.ConclusionMarfan 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.

Management of an inadvertent late filtering bleb and hypotony in a scleral fixated intraocular lens implantation

“…Blebs, in most cases are large and more luminous than conjunctival inclusion cysts and have associated conjunctival epithelial edema. 10 Epithelial inclusion cysts are lined by non-keratinized squamous epithelium. The tan colour of these cysts due to keratin and normal IOP due to lack of connection to anterior chamber help differentiate these cysts from blebs.…”

Filtering Pseudo-Bleb Secondary to Sutured Posterior Chamber Intraocular Lens with Implications for Ocular Surgery in Marfan Syndrome

“…Conjunctival cyst was also documented to masquerade as a pseudo-bleb after sclera-fixated IOL implantation in Marfan syndrome. 1 Shanmugam et al 4 described a case of pseudo-bleb secondary to vitrectomy, but that patient was diagnosed with Traboulsi syndrome. UBM has also been employed to describe the anterior segment findings of this syndrome.…”

“…It is interesting to note that both eyes in our patient, and those of other reported cases, developed cystic ischemic pseudo-blebs despite no exposure to anti-metabolites such as mitomycin C or 5-fluorouracil. 1 , 2 , 3 , 4 , 5 This suggests aqueous dynamics in the subconjunctival space plays a role in bleb morphology. This has been reported as a risk factor for bleb leak after trabeculectomy.…”

“…Marfan syndrome is a congenital connective tissue abnormality caused by mutations in the fibrillin (FBN1) gene and affecting cardiovascular, musculoskeletal, and ocular structures. 1 Weakening of the sclera in Marfan syndrome poses a risk of primary (spontaneous scleral rupture) or secondary (following scleral incisions) pseudo-bleb.…”

A Study on Correlation of Surgeon’s Experience and Inadvertent Conjunctival Cyst Following SICS and Review of the Literature

“…The important differential diagnosis of conjunctival cysts is filtering blebs [2,16]. Gosalia NR., et al have reported a case of inclusion cyst masquerading as an inadvertent bleb in a patient with Marfan's syndrome due to abnormal sclera [23]. Gonioscopy helps differentiate the etiology of the cyst, whether due to Trabeculectomy or the mSICS surgery, by viewing the fish mouthing of the scleral tunnel [13].…”