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REVIEW ARTICLE
Year : 2019  |  Volume : 57  |  Issue : 1  |  Page : 31-33

Pearls in the management of Stevens–Johnson syndrome


Dr. G. Sitalakshmi Memorial Clinic for Ocular Surface Disorders, Sankara Nethralaya, Chennai, Tamil Nadu, India

Date of Web Publication10-Jun-2019

Correspondence Address:
Dr. Bhaskar Srinivasan
Dr. G. Sitalakshmi Memorial Clinic for Ocular Surface Disorders, Sankara Nethralaya, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_21_19

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  Abstract 


Stevens–Johnson Syndrome, a life-threatening disorder, can lead to severe ocular morbidity and corneal blindness. The discomfort and visual loss caused leads to a considerable impact on the quality of life. The goals of treatment include measures to reduce inflammation, thereby improving comfort and results for visual rehabilitation. The issues to be addressed include dry eye, lid margin keratinization, adnexal disorders, and fornix obliteration. Measures to address these like punctal cautery and mucous membrane graft have been discussed along with their surgical technique. Visual rehabilitation, following stabilization of the ocular surface, can be achieved with optical iridectomy, cataract surgery or in end-stage disease by performing the modified osteo-odonto keratoprosthesis or Type 2 keratoprosthesis. The procedures to stabilize the ocular surface help in preventing further deterioration of the ocular surface and contribute to improved vision and comfort.

Keywords: Dry eye, lid margin keratinization, Stevens–Johnson Syndrome


How to cite this article:
Iyer G, Srinivasan B, Agarwal S. Pearls in the management of Stevens–Johnson syndrome. TNOA J Ophthalmic Sci Res 2019;57:31-3

How to cite this URL:
Iyer G, Srinivasan B, Agarwal S. Pearls in the management of Stevens–Johnson syndrome. TNOA J Ophthalmic Sci Res [serial online] 2019 [cited 2019 Aug 20];57:31-3. Available from: http://www.tnoajosr.com/text.asp?2019/57/1/31/259881




  Introduction Top


The ocular sequelae of Stevens–Johnson Syndrome (SJS) cause significant ocular morbidity and can lead to bilateral corneal blindness. Following the acute onset of the disease, during which, the course can be complicated by vision-threatening complications, in most instances, the eye tends to progress to what is termed as the chronic stage of the disease manifesting ocular sequelae of the disease. These sequelae can cause progressive detrimental damage to the ocular surface, if not addressed.[1]

The ocular sequelae of SJS can be categorized into the dry eye, lid margin keratinization (LMK), symblepharon, and adnexal conditions such as trichiasis and entropion. Limbal stem cell deficiency can rarely be seen immediately after the onset of SJS, but more commonly occurs secondary to the unaddressed ocular sequelae of SJS listed above. Hence, the procedures in this stage of the disease are aimed at stabilizing the ocular surface with intent to prevent or reduce further deterioration of the ocular surface.

Visual loss in SJS could be secondary to the limbal stem cell deficiency with or without a keratinized ocular surface, corneal opacity secondary to complications such as a persistent epithelial defect or corneal infiltration, or cataract. Rarely, the disease could be associated with glaucoma or posterior segment complications, usually related to corneal perforation.

Each of these factors, therefore, has to be addressed based on the presenting feature and can be classified into procedures to help stabilize the ocular surface, tectonic procedures, and procedures for visual rehabilitation.[2],[3],[4]


  Stabilizing the Ocular Surface Top


1. Dry eye– the most common sequelae of SJS, dry eye is often treated with topical lubricants. In severe dry eye, preservative-free lubricants are preferred. In addition, in eyes with the severe dry eye with absent emotional and reflex tearing, punctual cautery, if the nasolacrimal duct is patent as confirmed by syringing, has a significant role to play. This can be performed as an outpatient procedure using a hand held battery operated device to cauterize the punctum. The procedure can be repeated in the case of recanalization of the punctum. In cases of severe dry eye, prosthetic replacement of ocular surface ecosystem (PROSE) lenses have a role to play in improving the comfort of the patient. In eyes with corneal scar or irregularity along with dryness, both the vision and comfort improve with the PROSE lenses. Minimal reversal of an early lackluster appearance of the ocular surface can occur with continued prolonged use of the lens. It also has a role to play in eyes with lid margin keratinization (LMK) by preventing blink induced microtrauma

2. LMK leads to constant microtrauma to the ocular surface and should be addressed. For grades 2 and above, excision with mucus membrane grafting is advisable. This helps reduce the microtrauma and stabilizes the ocular surface [Figure 1]. PROSE lenses can be worn subsequently, if required. The surgical steps of mucous membrane grafting for LMK have been described herewith.
Figure 1: (a) Preoperative pictures showing lid margin keratinization in the lower and upper lid, (b) Postoperative pictures at 6 weeks showing well taken up vascularised mucous membrane graft in lower and upper lid

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Surgical technique

General anesthesia is preferred. Lid sutures are taken using 4–0 silk and lids everted with swabsticks or a flat blunt instrument placed beneath the lid to aid hemostasis.

The incision is made immediately posterior to the gray line (if identified) or midway along the thickened keratinized lid margin to include distichiatic lashes along the entire length of the lid stopping short of the punctum using a 15 no blade on Bard Parker handle. The plane of excision is maintained posterior to the tarsal plate to include only the keratinized conjunctiva. The minimum width of excision is 5 mm or including the entire extent of the keratinized conjunctiva, whichever is larger and is left behind like a flap. Hemostasis is achieved using minimal cautery. The length and width of the dissected area in each lid are measured to determine the length and width of lip mucosa to be harvested.(approximately 20 mm × 5 mm for each lid; can vary from case to case). The lower lip is preferred. If required, the mucosa is harvested from the upper lip in addition to the lower lip. An area of approximately 40 mm × 10 mm is marked out on the stretched mucosa and submucosal infiltration of 5 ml xylocaine adrenaline (1:1,600,000) is given. The marked out area is dissected using a 15 no blade on Bard Parker handle and the harvested graft is soaked in the antibiotic solution. The edges of the raw lip mucosa are approximated using continuous 5–0 vicryl sutures. After confirming hemostasis, attention is redirected to the eye after change of gloves and surgical instruments. The harvested graft is freed of any underlying fatty tissue by sharp dissection and thinned out to the extent possible to allow good stretchability of the graft. This is facilitated by rolling the harvested graft around the index finger of the left hand. The graft is cut in to four parts for each lid measuring 20 mm × 5 mm or as per the need using straight scissors. One edge of the mucosa is sutured to the lid margin using 8–0 vicryl continuous interlocking suture with exteriorization of the knots.

The same procedure was repeated for the other lids.

Based on the availability of the mucosal graft, the keratinized conjunctival flap is excised.

Reconstituted fibrin glue is applied to the raw tarsal surface beneath the graft, the graft stretched and stuck over the tarsus. A blunt instrument like a muscle hook is used to iron out the graft to remove the excess of glue or haem trapped beneath. End-to-end approximation of the graft with the conjunctival cut edge is confirmed by pinching the two edges together with toothed-forceps. The eye is completely cleaned off any excess glue in the vicinity of the graft. The eye is patched with antibiotic eye ointment.

3. Adnexal conditions such as trichiasis and entropion should be addressed

4. Forniceal shortening.

Although can be left largely unaddressed, fornix reconstructive procedures are primarily undertaken to help stabilize the tear film in severe cases and help in the fitting of a PROSE lens when the same is precluded by the shortened fornix. This is usually combined with a mucus membrane grafting to the lid margin when associated with lid margin keratinization.


  Tectonic Procedures Top


Tectonic procedures include those procedures that are applicable in case of a persistent epithelial defect or corneal perforation. These include the use of cyanoacrylate glue, amniotic membrane grafting, lamellar or full-thickness patch grafts or keratoplasties. Conjunctival hooding and/or tarsorrhaphy have a role to play in certain cases.


  Visual Rehabilitation Top


Optical iridectomy can be attempted to improve vision in eyes with central or paracentral corneal opacities with a relatively clear zone of the peripheral cornea. Cataract extraction, phacoemulsification or extracapsular, based on the feasibility can be performed in these eyes with cataract.

These procedures are usually undertaken after performing procedures to stabilize the ocular surface.

Keratoprosthesis forms the last resort for visual rehabilitation in bilateral end-stage ocular surface disease. Modified osteo-odonto keratoprosthesis (MOOKP) forms the first choice of Kpro in eyes with SJS. Type 2 Boston Kpro or the osteo Kpro can be performed in eyes not suitable for the MOOKP.

Acknowledgment

Mucous membrane grafting for LMK in SJS-Cornea Society Newsletter April 2016.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Jain R, Sharma N, Basu S, Iyer G, Ueta M, Sotozono C, et al. Stevens-Johnson syndrome: The role of an ophthalmologist. Surv Ophthalmol 2016;61:369-99.  Back to cited text no. 1
    
2.
Iyer G, Pillai VS, Srinivasan B, Guruswami S, Padmanabhan P. Mucous membrane grafting for lid margin keratinization in Stevens-Johnson syndrome: Results. Cornea 2010;29:146-51.  Back to cited text no. 2
    
3.
Iyer G, Srinivasan B, Agarwal S, Kamala Muralidharan S, Arumugam S. Comprehensive approach to ocular consequences of Stevens Johnson syndrome – The aftermath of a systemic condition. Graefes Arch Clin Exp Ophthalmol 2014;252:457-67.  Back to cited text no. 3
    
4.
Iyer G, Srinivasan B, Agarwal S, Pillai VS, Ahuja A. Treatment modalities and clinical outcomes in ocular sequelae of Stevens-Johnson syndrome over 25 years - A paradigm shift. Cornea 2016;35:46-50.  Back to cited text no. 4
    


    Figures

  [Figure 1]



 

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