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Implantation pearl cyst after uncomplicated clear cornea phacoemulsification mimicking an iris tumour

      Epithelial downgrowth (ED) is a serious, although rare, complication of ocular surgery and trauma that can result in irreversible vision loss.
      • Weiner M.J.
      • Trentacoste J.
      • Pon D.M.
      • Albert D.M.
      Epithelial downgrowth: a 30-year clinicopathological review.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      • Kim S.K.
      • Ibarra M.S.
      • Syed N.A.
      • Sulewski M.E.
      • Orlin S.E.
      Development of epithelial downgrowth several decades after intraocular surgery.
      • Srinivasan S.
      • Jones D.H.
      • Jay J.L.
      • Roberts F.
      Epithelial downgrowth following clear cornea phacoemulsification in a buphthalmic eye.
      • Haller J.A.
      • Stark W.J.
      • Azab A.
      • Thomsen R.W.
      • Gottsch J.D.
      Surgical management of anterior chamber epithelial cysts.
      • Stone D.U.
      • Char D.H.
      • Crawford J.B.
      • Margolis T.P.
      • Van Gelder R.N.
      • Strauss E.C.
      Metaplastic squamous epithelial downgrowth after clear corneal cataract surgery.
      In the ocular surgery group, cataract surgery, especially in the extracapsular era, accounted for 86% of cases
      • Weiner M.J.
      • Trentacoste J.
      • Pon D.M.
      • Albert D.M.
      Epithelial downgrowth: a 30-year clinicopathological review.
      related to vitreous loss, persistent hypotony, capsule rupture, multiple surgeries, delayed wound healing, wound fistulas, and iris incarceration. According to the literature, 82% of cases presented within the first year after intraocular surgery
      • Weiner M.J.
      • Trentacoste J.
      • Pon D.M.
      • Albert D.M.
      Epithelial downgrowth: a 30-year clinicopathological review.
      • Kim S.K.
      • Ibarra M.S.
      • Syed N.A.
      • Sulewski M.E.
      • Orlin S.E.
      Development of epithelial downgrowth several decades after intraocular surgery.
      with retrocorneal membrane (48%), painful glaucoma (32%), positive Seidel test (24%), corneal edema (22%), hypotony (17%), painless glaucoma (14%), iritis (11%), and iris cyst (8%).
      • Weiner M.J.
      • Trentacoste J.
      • Pon D.M.
      • Albert D.M.
      Epithelial downgrowth: a 30-year clinicopathological review.
      Epithelial cells that gain access to the anterior chamber can grow and present in 3 classical forms: (i) opaque sheet over the cornea, iris, and angle structures; (ii) epithelial anterior chamber clear cyst; (iii) rarely as a pearl-white solid cyst, also called as iris pearl tumor.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      • Kim S.K.
      • Ibarra M.S.
      • Syed N.A.
      • Sulewski M.E.
      • Orlin S.E.
      Development of epithelial downgrowth several decades after intraocular surgery.
      • Srinivasan S.
      • Jones D.H.
      • Jay J.L.
      • Roberts F.
      Epithelial downgrowth following clear cornea phacoemulsification in a buphthalmic eye.
      Histologically, they are arranged in a multilayer structure resembling corneal or conjunctival epithelium.
      • Weiner M.J.
      • Trentacoste J.
      • Pon D.M.
      • Albert D.M.
      Epithelial downgrowth: a 30-year clinicopathological review.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      The sheet-like form is the most common presentation and has the worst prognosis, followed by the serous and pearl cysts.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      • Srinivasan S.
      • Jones D.H.
      • Jay J.L.
      • Roberts F.
      Epithelial downgrowth following clear cornea phacoemulsification in a buphthalmic eye.
      • Haller J.A.
      • Stark W.J.
      • Azab A.
      • Thomsen R.W.
      • Gottsch J.D.
      Surgical management of anterior chamber epithelial cysts.
      • Sitchevska O.
      • Payne B.F.
      Pearl cysts of the iris.
      Serous cysts are transparent and often form in a wall of conjunctiva covered by epithelium, whereas pearl cysts are solid, opaque, and most commonly associated with cilia that enter the anterior chamber.
      • Sitchevska O.
      • Payne B.F.
      Pearl cysts of the iris.
      • Song J.
      • Bi H.
      Pearl cyst treatment by surgical excision.
      Because of their potential for growth and development of serious complications, most authors favour a surgical approach.
      • Song J.
      • Bi H.
      Pearl cyst treatment by surgical excision.
      We report a case of ED presenting as an iris pearl tumour 2 years after uncomplicated clear cornea phacoemulsification.

      Case Report

      A 71-year-old male presented with a 3-month history of a red, painful, and photophobic left eye. His ocular history was significant for bilateral uncomplicated clear corneal phacoemulsification with posterior chamber intraocular lens (PC-IOL) implantation 2 years previously, and his family and medical history were unremarkable. There was no history of trauma. Visual acuity (VA) was 6/9 OD and 6/18 OS. OS biomicroscopy showed cilioconjunctival hyperemia, +4 anterior chamber cells, and a vascularized dome-shaped amelanotic iris mass at the 12 o’clock position, with a splashing of pigment over the surface with a slither hypopyon (Fig. 1A–D). There was also a PC-IOL and no evidence of ED from the section site of his previous cataract surgery. No vitritis was identified, and fundoscopy did not reveal any signs of posterior inflammation. Ultrasound B-scan detected an iris lesion at 12 o’clock with medium internal echogenecity and an elevation of 3.0 mm (Fig. 2A). Anterior segment optical coherence tomography confirmed these measurements (Fig. 2B). Treatment with topical steroids led to an improvement in inflammation but not in the mass dimensions. Uveitis work-up was negative, so an excisional biopsy by broad iridectomy was performed. Microbiology analysis of the aqueous aspirate was negative. Histopathology findings were of iris tissue partly covered by stratified squamous epithelium reminiscent of ocular surface epithelium.
      Fig. 1
      Fig. 1External photograph of the left eye. (A) Image shows a vascularized dome-shaped amelanotic iris mass at the 12 o’clock position, with a splashing of pigment. (B) Magnified image of the lesion and cornea, where mild edema and keratic precipitates can also be identified. (C) Image shows cilioconjunctival hyperemia with a discrete hypopyon. (D) Gonioscopic view of lesion showing an elevated lesion with no corneal contact. These features suggested a neoplastic process.
      Fig. 2
      Fig. 2(A) Ultrasonography of left eye detected an elevated iris lesion at the 12 o’clock position with medium internal echogenecity, a transverse base of 4.8 mm, a longitudinal base of 3.2 mm, and an elevation of 3.0 mm. (B) Anterior segment optical coherence tomography confirmed ocular ultrasonography measures.
      The overall appearance was suggestive of ED (Fig. 3). There was no evidence of neoplasia.
      Fig. 3
      Fig. 3Hematoxylin and eosin staining (×10 objective): cystic space containing keratin flakes and debris, lined by stratified squamous epithelium. The wall includes iris stroma and pigment epithelium.
      The eye resolved well with topical medication. At 24 months’ follow-up, VA was 6/9, and there was no evidence of inflammation or recurrence (Fig. 4A–C).
      Fig. 4
      Fig. 4Photographs of the left eye after broad iridectomy using different slit-lamp illumination techniques: (A) diffuse illumination, (B) tangential illumination, and (C) retroillumination, which show a clear cornea, residual scar tissue at the capsulorhexis margin and no recurrence of the lesion.

      Discussion

      The incidence of ED after cataract surgery has been reported to range from 0.08% to 0.12%
      • Weiner M.J.
      • Trentacoste J.
      • Pon D.M.
      • Albert D.M.
      Epithelial downgrowth: a 30-year clinicopathological review.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      when intracapsular and extracapsular extraction techniques were used. With advances in cataract surgery, sutureless phacoemulsification has become the procedure of choice for most surgeons. It allows for a smaller wound with better closure that results in better visual acuity with a lower rate of complications,
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      • de Silva S.R.
      • Riaz Y.
      • Evans J.R.
      Phacoemulsification with posterior chamber intraocular lens versus extracapsular cataract extraction (ECCE) with posterior chamber intraocular lens for age-related cataract.
      • Knauf H.P.
      • Rowsey J.J.
      • Margo C.E.
      Cystic epithelial downgrowth following clear-corneal cataract extraction.
      thus rendering ED very rare.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      There are few reports in the literature on this subject over the past 20 years.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      • Srinivasan S.
      • Jones D.H.
      • Jay J.L.
      • Roberts F.
      Epithelial downgrowth following clear cornea phacoemulsification in a buphthalmic eye.
      • Stone D.U.
      • Char D.H.
      • Crawford J.B.
      • Margolis T.P.
      • Van Gelder R.N.
      • Strauss E.C.
      Metaplastic squamous epithelial downgrowth after clear corneal cataract surgery.
      • Knauf H.P.
      • Rowsey J.J.
      • Margo C.E.
      Cystic epithelial downgrowth following clear-corneal cataract extraction.
      • Lee B.L.
      • Gaton D.D.
      • Weinreb R.N.
      Epithelial downgrowth following phacoemulsification through a clear cornea.
      Of these, only Knauf et al.
      • Knauf H.P.
      • Rowsey J.J.
      • Margo C.E.
      Cystic epithelial downgrowth following clear-corneal cataract extraction.
      reported the cystic form, namely, the serous cyst, presenting 3 years after cataract surgery and managed successfully by surgical excision. To the best of our knowledge, there have been no reports of pearl cyst formation after uncomplicated clear cornea phacoemulsification. In general, the cystic forms have been considered more easily managed because of prompt detection and better surgical eradication.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      • Haller J.A.
      • Stark W.J.
      • Azab A.
      • Thomsen R.W.
      • Gottsch J.D.
      Surgical management of anterior chamber epithelial cysts.
      Other reported successful treatment options for implantation anterior chamber cysts have included aspiration of cyst contents and endolaser photocoagulation of the residual cyst wall, aspiration and intralesional administration of mitomycin C, or more invasive vitrectomy and local cryoablation of adjacent cyst wall.
      • Kim S.K.
      • Ibarra M.S.
      • Syed N.A.
      • Sulewski M.E.
      • Orlin S.E.
      Development of epithelial downgrowth several decades after intraocular surgery.
      • Haller J.A.
      • Stark W.J.
      • Azab A.
      • Thomsen R.W.
      • Gottsch J.D.
      Surgical management of anterior chamber epithelial cysts.
      • Yu C.S.
      • Chiu S.I.
      • Tse R.K.
      Treatment of cystic epithelial downgrowth with intralesional administration of mitomycin C.
      The sheet-like form has been more often reported
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      • Srinivasan S.
      • Jones D.H.
      • Jay J.L.
      • Roberts F.
      Epithelial downgrowth following clear cornea phacoemulsification in a buphthalmic eye.
      • Lee B.L.
      • Gaton D.D.
      • Weinreb R.N.
      Epithelial downgrowth following phacoemulsification through a clear cornea.
      and has been found to have the worst outcome because it is more difficult to diagnose and eradicate.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      • Kim S.K.
      • Ibarra M.S.
      • Syed N.A.
      • Sulewski M.E.
      • Orlin S.E.
      Development of epithelial downgrowth several decades after intraocular surgery.
      • Srinivasan S.
      • Jones D.H.
      • Jay J.L.
      • Roberts F.
      Epithelial downgrowth following clear cornea phacoemulsification in a buphthalmic eye.
      Extensive disease was also associated with a poorer outcome.
      • Srinivasan S.
      • Jones D.H.
      • Jay J.L.
      • Roberts F.
      Epithelial downgrowth following clear cornea phacoemulsification in a buphthalmic eye.
      The current case describes the uncommon pearl cyst presentation of ED, which occurs more frequently after trauma
      • Sitchevska O.
      • Payne B.F.
      Pearl cysts of the iris.
      but uniquely now has been found after uncomplicated phacoemulsification. It presented as a solid iris lesion, as documented by imaging, suggesting a neoplastic process. The natural sequence of events has been described after implantation of cilia into the iris after trauma: formation of the pearl cyst, followed by iridocyclitis, and then by enlargement of the cyst to cause secondary glaucoma.
      • Sitchevska O.
      • Payne B.F.
      Pearl cysts of the iris.
      Other forms of ED can be associated with tumours. Stone et al.
      • Stone D.U.
      • Char D.H.
      • Crawford J.B.
      • Margolis T.P.
      • Van Gelder R.N.
      • Strauss E.C.
      Metaplastic squamous epithelial downgrowth after clear corneal cataract surgery.
      reported a case of pseudophakic chronic aggressive inflammation that later was associated with the development of a vascularized infiltrative iris mass, which was proved to be a metaplastic ED of a limbal squamous cell carcinoma with secondary intraocular inflammation. Therefore, malignant and other etiologies of iris cysts, such as congenital cysts, gummas, and tubercles, should be included in the differential diagnosis.
      • Sitchevska O.
      • Payne B.F.
      Pearl cysts of the iris.
      • Song J.
      • Bi H.
      Pearl cyst treatment by surgical excision.
      Surgical treatment was the treatment of choice, and during follow-up, no recurrence was identified. This is in accordance with previous reports that show that in patients with ED, surgical treatment led to fewer enucleations than in those treated medically or not treated.
      • Weiner M.J.
      • Trentacoste J.
      • Pon D.M.
      • Albert D.M.
      Epithelial downgrowth: a 30-year clinicopathological review.
      • Vargas L.G.
      • Vroman D.T.
      • Soloman K.D.
      • et al.
      Epithelial downgrowth after clear cornea phacoemulsification: report of two cases and review of the literature.
      • Srinivasan S.
      • Jones D.H.
      • Jay J.L.
      • Roberts F.
      Epithelial downgrowth following clear cornea phacoemulsification in a buphthalmic eye.
      • Sitchevska O.
      • Payne B.F.
      Pearl cysts of the iris.
      In summary, ED is a very rare complication of modern cataract surgery. This case shows that, in this setting, in addition to the previously described serous cystic and sheet-like growth forms, epithelial growth can also present as an implantation pearl cyst. Clinicians should have a high index of suspicion for this condition in pseudophakic patients when more usual conditions have been excluded. Appropriate treatment includes early surgical excision, which may be associated with a more favourable prognosis.

      Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

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