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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 26-27

Novel management of spontaneous onset sclerocorneal cyst in a child


Pediatric Ophthalmology, National Institute of Ophthalmology, Pune, Maharashtra, India

Date of Submission21-Apr-2021
Date of Acceptance30-Jun-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Jai A Kelkar
Near Mahatma Phule Museum, 1137/80, Off Ghole Road, Pune - 411 005, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_941_21

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  Abstract 


A 2-year-old girl was referred for management of swelling on the nasal side in the right eye. On ophthalmic examination, the right eye showed a subconjunctival cyst extending into the anterior corneal stroma on the nasal side. Ultrasound biomicroscopy of the right eye showed a fluid-filled cystic lesion involving the cornea, with no communication with the anterior chamber. Examination under anesthesia revealed an intrastromal sclerocorneal cyst over the nasal half of cornea, not involving pupillary axis. The cyst was drained, anterior cyst wall excised, scleral lips glued together, and the bare corneoscleral bed covered with amniotic membrane with no recurrence.

Keywords: Amniotic membrane graft, intraoperative OCT, sclerocorneal cyst, ultrasound biomicroscopy


How to cite this article:
Kelkar JA, Kelkar AS, Singhvi PS. Novel management of spontaneous onset sclerocorneal cyst in a child. Indian J Ophthalmol Case Rep 2022;2:26-7

How to cite this URL:
Kelkar JA, Kelkar AS, Singhvi PS. Novel management of spontaneous onset sclerocorneal cyst in a child. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Jan 28];2:26-7. Available from: https://www.ijoreports.in/text.asp?2022/2/1/26/334995



Sclerocorneal cyst is a rare entity. It may be spontaneous in onset or may occur following trauma or surgery. Various etiologies have been suggested for the sclerocorneal cyst. Few believe that epithelial cells get entrapped in the limbus during scleral development and lead to the formation of a cyst,[1] whereas Rao et al.[2] stated the presence of ectopic lacrimal tissue as developmental etiology for such cysts.


  Case Report Top


A 2-year-old girl was referred for management of swelling on the nasal side in the right eye. The past medical history revealed lipomeningocele and spina bifida of L4–L5. She had undergone spinal cord cyst excision at the age of 6 months.

On ophthalmic examination, the right eye showed a subconjunctival cyst extending into the anterior corneal stroma on the nasal side, from 7 to 12 O'clock position. On streak retinoscopy, high against-the-rule astigmatism was noted (+1.0/−[email protected]). Vision could not be recorded because of the young age of the child.

Left eye examination was within normal limits. Ultrasound biomicroscopy (UBM) of the right eye showed a fluid-filled cystic lesion involving the cornea, with no communication with the anterior chamber [Figure 1].
Figure 1: Ultrasound biomicroscopy of the right eye showed a fluid-filled cystic lesion involving the cornea, with no communication with the anterior chamber

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Examination under anesthesia revealed an intrastromal sclerocorneal cyst involving the nasal half of the cornea and not involving the pupillary axis [Figure 2]. It measured 20 mm (vertical) by 15 mm (horizontal). The conjunctiva was freely mobile over the intrascleral portion of the cyst. The sclerocorneal cyst was filled with clear fluid. The surrounding stroma outside the cyst margin was clear. There was an absence of corneal vascularization or any signs of trauma. The anterior chamber was well-formed and clear. The rest of the anterior segment examination was normal along with normal fundus.
Figure 2: Right eye: Sclerocorneal cyst involving the nasal half of cornea sparing the pupillary axis

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After obtaining informed consent from the parents, surgical intervention was planned. Localized peritomy was performed to expose the underlying scleral cyst. The anterior wall of the cyst near the limbus was incised. Fluid was drained from the intrastromal part of the cyst. After a thorough wash of the intrastromal cavity with balanced salt solution, the cyst collapsed completely. The base and sidewalls of the scleral cyst were scrapped to remove the epithelial cells.

The conjunctiva around the outer border of the cyst was dissected. The anterior cyst wall measuring 2 mm by 12 mm was excised. No communication was noted with the anterior chamber. The scleral bed was cauterized. The intraoperative OCT (iOCT, Artevo 800 3D, Zeiss, USA) showed thickened corneal tissue overlying the cystic area [Figure 3]. Under iOCT guidance, the cornea was incised and the excess tissue was scrapped using a crescent knife. The scleral lips were glued together using fibrin glue (Baxter, India), thus obviating the need for a scleral patch graft. Amniotic membrane was used to cover the bare scleral and corneal bed of the wound, placed in situ with help of fibrin glue. Conjunctiva was reposited over the amniotic membrane till the limbus and was closed over it with 8-0 Ethicon sutures. The anterior chamber remained formed throughout the procedure, thus confirming the absence of communication with the cyst cavity. There is no recurrence of cyst on 6 months follow-up visit [Figure 4]. Astigmatism has reduced on streak retinoscopy (+1.00/−[email protected]).
Figure 3: Intraoperative optical computed tomography of the right eye showing the sclerocorneal cyst, with thickened corneal wall and no communication with the anterior chamber

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Figure 4: Right eye: Postoperative image at 6 months follow-up

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  Discussion Top


The indications for surgical intervention are a progressive increase in the size of cyst leading to poor vision, involvement of visual axis, or astigmatism.[3],[4] Vision may get affected due to distortion and scarring caused by the lesion on and around the limbus.[5] In our case, one third of the cornea was involved on presentation. As the course is progressive, failure to treat will impair the sight by extending over the pupillary area. Considering this possibility and due to the large size of the cyst in our patient, we operated early.

Rao et al.[2] described marsupialization of the scleral portion of the cyst and chemical ablation of the lining epithelium of the corneal cyst with 20% trichloroacetic acid.

Sano et al.[6] have tried excision of the cyst with lamellar keratoplasty with frozen corneal tissue and obtained successful results. Shah et al.[4] did excision of the cyst with a patch graft to cover the scleral defect and amniotic membrane grafting without any chemical cautery.

In our case, complete deroofing of the cyst was not done, instead we chose to excise some part of the scleral tissue that was redundant after the cyst wall collapsed completely. Also, the patient's sclera was preserved and the two walls of the cyst were closed with the application of fibrin glue, thus obviating the need for scleral patch graft. Amniotic membrane grafting was done to cover the glued sclera and the corneal wound. Scrapping of the cystic cavity was done to ensure that there were no nests of epithelial tissue that were postulated to bring about a recurrence.[2]

Spina bifida is a congenital disorder with systemic and ophthalmic associations. Caines and colleagues[7] documented 20 children with spina bifida. Of these, 81% had refractive errors, 52% strabismus, 50% defective accommodation, 43% nystagmus, and 10% optic atrophy.[7] Other ophthalmological manifestations such as microphthalmia, anterior lenticonus, iris coloboma, anophthalmia, optic atrophy, hypertelorism, microcornea, absence of optic nerve and ganglion cells of the retina, and absence of the macula have been reported to be involved in spina bifida.[8]


  Conclusion Top


The occurrence of sclerocorneal cyst in spina bifida has never been reported, and whether this association is by chance or otherwise needs to be evaluated further.

Declaration of patientconsent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mahmood MA, Awad A. Congenital sclerocorneal epithelial cyst. Am J Ophthalmol 1998;126:740-1.  Back to cited text no. 1
    
2.
Rao SK, Fogla R, Biswas J, Padmanabhan P. Corneoscleral epithelial cysts: Evidence of developmental etiology. Cornea 1998;17:446-50.  Back to cited text no. 2
    
3.
Soni T, Das S. Natural course of congenital corneoscleral cyst: 10-year follow-up. Indian J Ophthalmol 2020;68:2217.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Shah SG, Matalia HP, Sangwan VS. Spontaneous onset corneoscleral hematic cyst. Indian J Ophthalmol 2006;54:118.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Akbaba M, Hacıyakupoğlu G, Uğuz A, Karslıoğlu Ş, Karcıoğlu Z. Congenital intrascleral cyst. Clin Ophthalmol (Auckland, NZ) 2011;5:583.  Back to cited text no. 5
    
6.
Sano Y, Okamoto S, Nishida K, Sotozono C, Kinoshita S. Peripheral lamellar keratoplasty for corneoscleral cyst: Three case reports. Cornea 1999;18:233-6.  Back to cited text no. 6
    
7.
Caines E, Dahl M, Holmström G. Longtermoculomotor and visual function in spina bifida cystica: A population-based study. Acta Ophthalmol Scand 2007;85:662-6.  Back to cited text no. 7
    
8.
Kara N, Yazici AT, Demirkale HI, Bozkurt E, Çakır M, Demirok A, Yılmaz ÖF. A case of spina bifida with ocular manifestations. J AAPOS 2009;13:600-1.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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