Allo-simple limbal epithelial transplant with keratoplasty – Saving the surface in xeroderma pigmentosa

Swetha Ravichandran; Radhika Natarajan

doi:10.4103/ijo.IJO_2691_21

PHOTO ESSAY

Year : 2022 | Volume : 2 | Issue : 3 | Page : 804-805

Allo-simple limbal epithelial transplant with keratoplasty – Saving the surface in xeroderma pigmentosa

Swetha Ravichandran, Radhika Natarajan
Department of Cornea and Refractive Surgery, Sankara Nethralaya, Medical Research Foundation, Chennai, Tamil Nadu, India

Date of Submission	19-Oct-2021
Date of Acceptance	11-Feb-2022
Date of Web Publication	16-Jul-2022

Correspondence Address:
Dr. Radhika Natarajan
Sankara Nethralaya, Medical Research Foundation, 41, College Road, Chennai - 600 006, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2691_21

Abstract

Keywords: AlloSLET, LSCD, xeroderma pigmentosa

How to cite this article:
Ravichandran S, Natarajan R. Allo-simple limbal epithelial transplant with keratoplasty – Saving the surface in xeroderma pigmentosa. Indian J Ophthalmol Case Rep 2022;2:804-5

How to cite this URL:
Ravichandran S, Natarajan R. Allo-simple limbal epithelial transplant with keratoplasty – Saving the surface in xeroderma pigmentosa. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 19];2:804-5. Available from: https://www.ijoreports.in/text.asp?2022/2/3/804/351145

Xeroderma pigmentosa (XP) is a rare autosomal recessive disease characterized by faulty DNA repair. Ocular manifestations include lid abnormalities, ocular surface inflammation, limbal stem cell deficiency (LSCD), epithelial neoplasia, corneal scarring, cataracts, and tear film abnormalities, in addition to extreme photosensitivity, hyperpigmentation, and neurological manifestations systemically.^[1] Primary LSCD due to XP makes the patients affected poor candidates for corneal surgery, causing epithelial irregularity and stromal scarring, resulting in visual loss and graft failure.^[2]

Our XP patient had undergone multiple penetrating keratoplasties in the left eye which was pseudophakic. It was her only seeing eye and she was non ambulant. She had a failed graft with epithelial roughening, stromal scarring, and 360° LSCD with limbal vascularization [Figure 1].

Figure 1: LSCD with vascularization (pink arrowheads) and failed corneal graft in XP. LSCD = limbal stem cell deficiency, XP = xeroderma pigmentosa

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It was decided to do an allogenic simple limbal epithelial transplant (alloSLET) alongside her third full-thickness transplant to prevent postoperative epithelial failure. Four simple limbal epithelial transplant (SLET) bits, 3 mm long, were harvested from the same donor corneal graft and fixed on amniotic membrane with fibrin glue, around the host limbus [Figure 2]. She was treated with topical steroids and systemic immunosuppression to prevent rejection of both allografts.

Figure 2: Intraoperatively, alloSLET bits placed (blue arrows) after minimal debridement at the limbus, avoiding damage to the donor graft epithelium. alloSLET = allogenic simple limbal epithelial transplant

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SLET bits were found to be well integrated at the first week follow-up [Figure 3] and a clear graft with an intact ocular surface was seen at 4 months [Figure 4]. She became self-ambulant and is on follow-up for her systemic disease. Though no marker studies were done to prove that SLET benefitted the re-epithelization, combining the two procedures gave both anatomical and functional outcome in our single-eyed patient.

Figure 3: Integrated SLET bits (orange arrow) in the first postoperative week. SLET = simple limbal epithelial transplant

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Figure 4: Clear graft with good re-epithelization and some remnant alloSLET bits (pink arrowheads) at 3 months. alloSLET = allogenic simple limbal epithelial transplant

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Discussion

Such combined or staged procedures have been described in LSCD in mucus membrane pemphigoid, Steven Johnson syndrome, and ocular chemical injury.^[3] Simultaneous keratoplasty with alloSLET was described as an emergency procedure in sterile keratolysis following bilateral chemical injury.^[4] Ours may be the first case report on combining alloSLET with keratoplasty for re-epithelization in an XP patient, with multiple graft failures in the past.

Declaration of patient consent

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

1.	Brooks BP, Thompson AH, Bishop RJ, Clayton JA, Chan CC, Tsilou ET, et al. Ocular manifestations of xeroderma pigmentosum: Long-term follow-up highlights the role of DNA repair in protection from sun damage. Ophthalmology 2013;120:1324-36.
2.	Jalali S, Boghani S, Vemuganti GK, Ratnakar KS, Rao GN. Penetrating keratoplasty in xeroderma pigmentosum. Case reports and review of the literature. Cornea 1994;13:527-33.
3.	Gupta N, Farooqui JH, Patel N, Mathur U. Early results of penetrating keratoplasty in patients with unilateral chemical injury after simple limbal epithelial transplantation. Cornea 2018;37:1249-54.
4.	Kunapuli A, Fernandes M. Successful outcome of simultaneous allogeneic simple limbal epithelial transplantation with therapeutic Penetrating keratoplasty (PKP) for limbal stem cell deficiency and sterile keratolysis after chemical injury. Cornea 2021;40:780-2.