Ocular surface reconstruction of Steven Johnson syndrome / toxic epidermal necrolysis affected eye - A case report

Abstract

Introduction: Toxic epidermal necrolysis (TEN), also known as Steven Johnson syndrome (SJS), is a devastating disease. Patients develop blindness and symblepharon despite multiple reconstructive surgeries. We report a case of SJS/TEN with ocular involvement where treatment with hyperbaric oxygen therapy (HBOT) resulted in a significant improvement in the visual acuity after surgery.

Case presentation: A woman with SJS/TEN with severe ocular complication (SOC) had limbal stem cell deficiency and symblepharon of the superior and inferior fornix. Pannus grew over her cornea, reducing the vision to counting finger. The symblepharon produced shortening of the fornix, causing entropion. The in-turned eyelid caused her eyelashes to rub against the cornea, causing great damage to the ocular surface. Limbal stem cell deficiency led to the loss of normal corneal morphology and invasion of the pannus onto the central visual axis, resulting in poor vision. She experienced ocular inflammation for 3 months before transfer to our hospital for admission. Ophthalmic examination showed bilateral corneal opacity with conjunctivalization, and inferior and superior fornix shortening. Symblepharon-lysis with amniotic membrane transplantation was attempted but the outcome was poor, with recurrence of superior scaring and symblepharon. She finally underwent major reconstructive surgery with allogeneic limbal stem cell transplantation with her sister as the donor, autologous minor salivary gland transplantation, and oral buccal mucosa flap transplant. HBOT was given daily post-surgery for supporting the grafts and suppressing inflammation. After 17 HBOT sessions and 3 months of autoserum drops, her left eye vision increased from the initial counting finger to 0.4 due to the successful growth of the corneal epithelium from the donor corneal limbal cell line. When a scleral contact lens which vaulted over the corneal limbal area was fitted, her vision improved to 0.8 due to redressal of high order aberration and astigmatism from the cornea scar.

Conclusion: After major reconstruction of the ocular surface with multiple cell type transplants, including limbal stem cells, minor salivary gland acinar cells, and oral mucosa cells, HBOT proved useful in supporting the graft uptake and oxygenation of the donor tissues, enabling fast recovery of the grafts and cell functioning, with eventual return of the working vision of the patient.

Keywords: AMT, amniotic membrane transplantation; CLAL, conjunctival limbal allograft; CLAU, conjunctival limbal autograft; CLET, Cultivated limbal epithelial transplantation; Case report; HBOT, Hyperbaric oxygen therapy; HLA typing, human leucocyte antigen typing; Hyperbaric oxygen therapy; LSCD, Limbal stem cell deficiency; Limbal stem cell deficiency; Limbal stem cell transplantation; Minor salivary gland transplant; Oral buccal mucosa flap transplantation; SJS, Steven Johnson syndrome; SOC, severe ocular complication; Stevens-Johnson syndrome; TEN, toxic epidermal necrolysis; Toxic epidermal necrolysis; lr-CLAL, living-related conjunctival limbal allograft.

Figure 1

Profound pannus ingrowth onto the cornea after limbal stem cell deficiency (LSCD) [A] and fluorescence revealed bizarre ocular surface in her left eye [B]. Symblepharon causes superior fornix shortening [C] and vision occlusion by pannus formation [D]. After surgical procedure of allogeneic limbal stem cell transplantation (LSCT) with living-related conjunctival limbal allograft (lr-CLAL) & simple limbal epithelial transplantation (SLET), combined with 17 times of HBOT. Postoperative 4-month image showing a stable well-epithelialized ocular surface with mild neovascularization, a faint residual deep stromal scarring as seen in the left eye [E], fluorescence staining of the same eye shows no cornea epithelium defect and stable tear film [F].

Figure 2

Illustration of surgical procedure. A. Pannus removal from disease eye of bulbar conjunctiva and Palpebral conjunctiva. B. Buccal mucosal flap and minor salivary gland transplantation.B1. Oral buccal mucosa taken from patient’s right cheek, sliced into 2 pieces, then transplanted to the patient’s upper and inferior palpebral conjunctiva. B2. Minor salivary gland taken from patient’s left labial area transplanted to diseased eye’s superior conjunctiva and superior fornix. C. Lr-CLAL (living related conjunctiva limbal allograft) + SLET (Simple Limbal Epithelial Transplantation). The limbal stem cell within the conjunctiva limbal graft is taken from her sister’s donor eye, suture to the patients’ superior and inferior limbus area, extra limbus tissue was sliced into small pieces, and then transplanted to patient’s central denuded cornea vie fibrin glue (Artiss®). D. Amniotic membrane, taken from uterus, was grafted over the patient’s cornea, bulbal conjuncitiva, upper and lower fornix, palpebral conjunctiva.

Figure 3

Ocular Surface Grading Score (OSGS) and images of Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) patient. C, conjunctivalization; N, neovascularization; O, opacification; K, keratinization; S, symblepharon; U, upper conjunctival sac shortening; L, lower conjunctival sac shortening; T, total OSGS. (A–B) Pre-operation surgery OSGS status: pre-operation (C3,N3,O2,K0,S2,U2,L2:T14), (C) Post operation 4 months OSGS status: (C1,N1,O1,K0,S0,U0,L0: T3). (D) Post op 12 months OSGS status: (C2,N1,O1K0,S0, U0, L0: T4).