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Enlarging corneoscleral cyst in a 2-year-old girl
Short Reports Enlarging corneoscleral cyst in a 2-year-old girl Elham AlQahtani, MD, Flavia Godoy, MD, and Christopher Lyons, MB, FRCS Cysts rarely develop within the cornea or sclera of children with no previous history of trauma or surgery. Primary corneoscleral cysts may arise from proliferation of anomalously situated epithelial or endothelial cells. We report the case of a 2-year-old girl with a primary enlarging cyst situated at the limbus of her left eye. Optical coherence tomography was used to determine whether there was any communication between the cyst and the anterior chamber and to help plan appropriate management.
Case Report
A
2 year-old girl presented at BC Children’s Hospital, Vancouver, 6 weeks after her parents noticed a cystic lesion on the surface of her left eye. There was no ocular discomfort, eye-rubbing, tearing, or discharge. There was no past history of trauma, ocular surgery, or atopy. Family history was unremarkable. On examination, visual acuity was 20/30 in each eye using single Allen pictures. Slit-lamp examination showed a left superonasal bleblike localized swelling measuring 4 4 mm at the corneal limbus (Figure 1A). There were no inflammatory signs. The anterior chambers were of equal depth and both intraocular pressures measured 9 mm Hg by rebound tonometry. Examination was otherwise unremarkable. B-scan ultrasound revealed a cystic mass but no underlying ciliary body abnormality. On examination under anesthesia, gonioscopy showed no communication into the anterior chamber; however, angle distortion was evident, with significant widening of the trabecular meshwork overlying the cyst. Over the next 10 weeks the cyst slowly enlarged in both height and diameter, gradually encroaching into the peripheral cornea (Figure 1B), prompting the decision to operate. Spectral domain optical coherence tomography (OCT) of the anterior chamber was used to outline the cyst and exclude the possibility of intraocular communication (Figure 2).
Author affiliations: Ophthalmology Department, BC Children’s Hospital and University of British Columbia, Vancouver, British Columbia, Canada Submitted February 17, 2015. Revision accepted March 24, 2015. Correspondence: Christopher Lyons, MB, FRCS, Ophthalmology Department, BC Children’s Hospital and University of British Columbia, 4480 Oak Street, Vancouver, BC, Canada V6H3V4 (email: [email protected]). J AAPOS 2015;-:1-3. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.03.020
Journal of AAPOS
FIG 1. A, Corneoscleral cyst at presentation. B, 10 weeks later, the cyst had enlarged significantly and was encroaching into the cornea.
At surgery, donor cornea was available as a precaution. The conjunctiva was incised posterior to the cyst, dissected off its surface and reflected forward onto the peripheral cornea (Figure 3). The cyst contents were aspirated using a 27-gauge needle at the peripheral cyst margin; as fluid was withdrawn, the anterior chamber depth remained unchanged. A 1 1 mm biopsy of the cyst wall was sent to pathology along with cyst contents. The cyst was then reinflated and flushed with sterile water for 5 minutes as a sclerosant, taking care to avoid contact with the rest of the operative site.1 Two 8-0 polyglactin 910 sutures were passed through the cyst, approximating its roof to its base to induce closure and scarring. The conjunctiva was closed with 8-0 polyglactin 910 suture. Histologically, the cyst wall was reported to be lined by epithelium, with sparse benign squamous epithelial cells and macrophages within the fluid content. The findings were consistent with a diagnosis of corneoscleral epithelial cyst. The patient made a rapid and complete recovery. There has been no recurrence with 5 years of follow-up.
Discussion Primary cysts of the cornea, sclera, or both (corneoscleral cyst) have been described for more than a century.2 In
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AlQahtani, Godoy, and Lyons
Volume - Number - / - 2015
FIG 3. The conjunctiva has been dissected off the surface of the corneoscleral cyst prior to aspiration of the cyst content, partial wall excision, and injection of water as a sclerosant.
FIG 2. Anterior segment optical coherence tomography series showing the cyst to be separate from the anterior chamber.
the present case, the differential diagnosis included conjunctival epithelial inclusion cyst, limbal dermoid (choristoma), and cystic extension of an intraocular neoplasm. A conjunctival inclusion cyst usually follows a history of trauma or surgery. A corneal/limbal dermoid is more opaque and usually situated inferotemporally or temporally. A thorough examination, including visualization or imaging of the anterior chamber angle, with imaging of the ciliary body and iris, ruled out malignant cystic extension of an underlying intraocular tumor. Most patients with corneoscleral cysts have a history of trauma or surgery, resulting in the introduction of surface epithelial cells into the corneal or scleral tissue planes. Typical examples are limbal traction sutures during strabismus surgery or suture tracks in lamellar keratoplasty.3 In the absence of previous surgery or a history of trauma, a developmental etiology is likely. Most reported cases in the literature are of epithelial origin, some describing the presence of conjunctival goblet cells despite absence of trauma or surgery.4 Endothelial origin, from Schlemm’s canal, for example, is also possible but less common.5 Ectopic lacrimal tissue has also been found in corneoscleral cyst lining,
supporting a developmental etiology.6 Pseudo-hypopyon may accumulate within the cyst from debris accumulation.3 Although most corneoscleral cysts are isolated, they can occasionally communicate with the intraocular space.2 Preoperative assessment should determine this and enable appropriate planning for surgery with patch-grafting as necessary. Spectral domain anterior segment OCT is a noncontact imaging modality that requires only a few seconds for image acquisition. It is well tolerated by infants7 and proved valuable for anterior segment evaluation in the present case. Structures posterior to the iris pigment epithelium, such as the ciliary body, are better imaged using ultrasound biomicroscopy, but this is challenging in infants since ocular contact and a coupling device are required. An enlarging limbal cyst is a rare but concerning presentation. Sclerosing agents described in the literature for this purpose include iodine, cocaine, trichloracetic acid, tetracycline,8 and ethanol.9 Shin and colleagues1 were the first to use sterile water to induce cyst closure. Sterile water is safer in the event of inadvertent intraocular entry. However, pure water in itself is sufficiently endothelial-toxic; therefore, extreme care is required to remain extracameral. We recommend using a cannula with a luer lock syringe, and gentle irrigation applied under constant microscopic visualization. The in vivo dose–effect of sterile water on cystic lining survival is not completely validated, and we irrigated for 5 minutes as recommended by Shin and colleagues.1 Combined with this technique, we also brought the two apposing surfaces of the scleral cyst walls together with closely lined sutures, as described by Bhatt and Ramaesh.3 Lamellar keratoplasty was not appropriate in the present case but could be helpful in select cases.10 References 1. Shin YJ, Wee WR, Kim M, Lee JH. Corneoscleral cyst treated with distilled water injection. Korean J Ophthalmol 2002;16:110-13.
Journal of AAPOS
Volume - Number - / - 2015 2. De Schweinitz GE, Crampton GS. Cyst of the corneoscleral junction communicating with the vitreous. Ophthalmic Record 1911; 20:42-3. 3. Bhatt PR, Ramaesh K. Intrastromal corneal limbal epithelial implantation cyst. Eye 2007;21:133-5. 4. Mahmood MA, Awad A. Congenital sclerocorneal epithelial cyst. Am J Ophthalmol 1998;126:740-41. 5. Custodis E. Uber Skleralcysten. Albrecht von Graefes Arch Ophthalmol 1932;128:112-18. 6. Rao SK, Fogla R, Biswas J, Padmanabhan P. Corneoscleral epithelial cysts: evidence of developmental etiology. Cornea 1998;17:446-50.
Journal of AAPOS
AlQahtani, Godoy, and Lyons
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7. Jancevski M, Foster CS. Anterior segment optical coherence tomography. Semin Ophthalmol 2010;25:317-23. 8. Graubart EB, Hubbard GB III. Giant Intrascleral cyst treated with trichloroacetic acid. Arch Ophthalmol 2008;126:438-9. 9. Ali Javadi M, Sharifi A, Hashemian SJ, Yazdani S, Parvizi G, Kanavi MR. Management of intracorneal epithelial cysts with ethanol irrigation and cyst wall excision: a clinicopathologic report. Cornea 2006;25:479-81. 10. Sano Y, Okamoto S, Nishida K, Sotozono C, Kinoshita S. Peripheral lamellar keratoplasty for corneoscleral cyst: three case reports. Cornea 1999;18:233-6.
Case Report
A
2 year-old girl presented at BC Children’s Hospital, Vancouver, 6 weeks after her parents noticed a cystic lesion on the surface of her left eye. There was no ocular discomfort, eye-rubbing, tearing, or discharge. There was no past history of trauma, ocular surgery, or atopy. Family history was unremarkable. On examination, visual acuity was 20/30 in each eye using single Allen pictures. Slit-lamp examination showed a left superonasal bleblike localized swelling measuring 4 4 mm at the corneal limbus (Figure 1A). There were no inflammatory signs. The anterior chambers were of equal depth and both intraocular pressures measured 9 mm Hg by rebound tonometry. Examination was otherwise unremarkable. B-scan ultrasound revealed a cystic mass but no underlying ciliary body abnormality. On examination under anesthesia, gonioscopy showed no communication into the anterior chamber; however, angle distortion was evident, with significant widening of the trabecular meshwork overlying the cyst. Over the next 10 weeks the cyst slowly enlarged in both height and diameter, gradually encroaching into the peripheral cornea (Figure 1B), prompting the decision to operate. Spectral domain optical coherence tomography (OCT) of the anterior chamber was used to outline the cyst and exclude the possibility of intraocular communication (Figure 2).
Author affiliations: Ophthalmology Department, BC Children’s Hospital and University of British Columbia, Vancouver, British Columbia, Canada Submitted February 17, 2015. Revision accepted March 24, 2015. Correspondence: Christopher Lyons, MB, FRCS, Ophthalmology Department, BC Children’s Hospital and University of British Columbia, 4480 Oak Street, Vancouver, BC, Canada V6H3V4 (email: [email protected]). J AAPOS 2015;-:1-3. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.03.020
Journal of AAPOS
FIG 1. A, Corneoscleral cyst at presentation. B, 10 weeks later, the cyst had enlarged significantly and was encroaching into the cornea.
At surgery, donor cornea was available as a precaution. The conjunctiva was incised posterior to the cyst, dissected off its surface and reflected forward onto the peripheral cornea (Figure 3). The cyst contents were aspirated using a 27-gauge needle at the peripheral cyst margin; as fluid was withdrawn, the anterior chamber depth remained unchanged. A 1 1 mm biopsy of the cyst wall was sent to pathology along with cyst contents. The cyst was then reinflated and flushed with sterile water for 5 minutes as a sclerosant, taking care to avoid contact with the rest of the operative site.1 Two 8-0 polyglactin 910 sutures were passed through the cyst, approximating its roof to its base to induce closure and scarring. The conjunctiva was closed with 8-0 polyglactin 910 suture. Histologically, the cyst wall was reported to be lined by epithelium, with sparse benign squamous epithelial cells and macrophages within the fluid content. The findings were consistent with a diagnosis of corneoscleral epithelial cyst. The patient made a rapid and complete recovery. There has been no recurrence with 5 years of follow-up.
Discussion Primary cysts of the cornea, sclera, or both (corneoscleral cyst) have been described for more than a century.2 In
1
2
AlQahtani, Godoy, and Lyons
Volume - Number - / - 2015
FIG 3. The conjunctiva has been dissected off the surface of the corneoscleral cyst prior to aspiration of the cyst content, partial wall excision, and injection of water as a sclerosant.
FIG 2. Anterior segment optical coherence tomography series showing the cyst to be separate from the anterior chamber.
the present case, the differential diagnosis included conjunctival epithelial inclusion cyst, limbal dermoid (choristoma), and cystic extension of an intraocular neoplasm. A conjunctival inclusion cyst usually follows a history of trauma or surgery. A corneal/limbal dermoid is more opaque and usually situated inferotemporally or temporally. A thorough examination, including visualization or imaging of the anterior chamber angle, with imaging of the ciliary body and iris, ruled out malignant cystic extension of an underlying intraocular tumor. Most patients with corneoscleral cysts have a history of trauma or surgery, resulting in the introduction of surface epithelial cells into the corneal or scleral tissue planes. Typical examples are limbal traction sutures during strabismus surgery or suture tracks in lamellar keratoplasty.3 In the absence of previous surgery or a history of trauma, a developmental etiology is likely. Most reported cases in the literature are of epithelial origin, some describing the presence of conjunctival goblet cells despite absence of trauma or surgery.4 Endothelial origin, from Schlemm’s canal, for example, is also possible but less common.5 Ectopic lacrimal tissue has also been found in corneoscleral cyst lining,
supporting a developmental etiology.6 Pseudo-hypopyon may accumulate within the cyst from debris accumulation.3 Although most corneoscleral cysts are isolated, they can occasionally communicate with the intraocular space.2 Preoperative assessment should determine this and enable appropriate planning for surgery with patch-grafting as necessary. Spectral domain anterior segment OCT is a noncontact imaging modality that requires only a few seconds for image acquisition. It is well tolerated by infants7 and proved valuable for anterior segment evaluation in the present case. Structures posterior to the iris pigment epithelium, such as the ciliary body, are better imaged using ultrasound biomicroscopy, but this is challenging in infants since ocular contact and a coupling device are required. An enlarging limbal cyst is a rare but concerning presentation. Sclerosing agents described in the literature for this purpose include iodine, cocaine, trichloracetic acid, tetracycline,8 and ethanol.9 Shin and colleagues1 were the first to use sterile water to induce cyst closure. Sterile water is safer in the event of inadvertent intraocular entry. However, pure water in itself is sufficiently endothelial-toxic; therefore, extreme care is required to remain extracameral. We recommend using a cannula with a luer lock syringe, and gentle irrigation applied under constant microscopic visualization. The in vivo dose–effect of sterile water on cystic lining survival is not completely validated, and we irrigated for 5 minutes as recommended by Shin and colleagues.1 Combined with this technique, we also brought the two apposing surfaces of the scleral cyst walls together with closely lined sutures, as described by Bhatt and Ramaesh.3 Lamellar keratoplasty was not appropriate in the present case but could be helpful in select cases.10 References 1. Shin YJ, Wee WR, Kim M, Lee JH. Corneoscleral cyst treated with distilled water injection. Korean J Ophthalmol 2002;16:110-13.
Journal of AAPOS
Volume - Number - / - 2015 2. De Schweinitz GE, Crampton GS. Cyst of the corneoscleral junction communicating with the vitreous. Ophthalmic Record 1911; 20:42-3. 3. Bhatt PR, Ramaesh K. Intrastromal corneal limbal epithelial implantation cyst. Eye 2007;21:133-5. 4. Mahmood MA, Awad A. Congenital sclerocorneal epithelial cyst. Am J Ophthalmol 1998;126:740-41. 5. Custodis E. Uber Skleralcysten. Albrecht von Graefes Arch Ophthalmol 1932;128:112-18. 6. Rao SK, Fogla R, Biswas J, Padmanabhan P. Corneoscleral epithelial cysts: evidence of developmental etiology. Cornea 1998;17:446-50.
Journal of AAPOS
AlQahtani, Godoy, and Lyons
3
7. Jancevski M, Foster CS. Anterior segment optical coherence tomography. Semin Ophthalmol 2010;25:317-23. 8. Graubart EB, Hubbard GB III. Giant Intrascleral cyst treated with trichloroacetic acid. Arch Ophthalmol 2008;126:438-9. 9. Ali Javadi M, Sharifi A, Hashemian SJ, Yazdani S, Parvizi G, Kanavi MR. Management of intracorneal epithelial cysts with ethanol irrigation and cyst wall excision: a clinicopathologic report. Cornea 2006;25:479-81. 10. Sano Y, Okamoto S, Nishida K, Sotozono C, Kinoshita S. Peripheral lamellar keratoplasty for corneoscleral cyst: three case reports. Cornea 1999;18:233-6.