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Comprehensive approach to ocular consequences of Stevens Johnson Syndrome - the aftermath of a systemic condition

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Abstract

Background

Stevens Johnson Syndrome (SJS) can lead to end stage corneal blindness. This study describes the comprehensive treatment measures and their outcomes in the management of ocular sequelae and complications of SJS.

Methods

Four hundred sixty-four eyes of 232 patients of SJS who underwent surgical intervention (punctal cautery, mucus membrane grafting for lid margin keratinisation, fornix reconstructive procedures, tectonic procedures, keratoplasty and keratoprosthesis) were studied. It was a non-comparative, retrospective, interventional case series. The primary outcome was the change in the best corrected visual acuity (BCVA). Secondary outcome measures included an improvement in the ocular surface status as indicated by corneal epithelial fluorescein staining and Schirmer’s I strip wetting.

Results

The BCVA and the ocular surface status improved and/or stabilized in > 70 % of eyes following punctal cautery (n = 160) and > 80 % of eyes following lid margin mucus membrane grafting (n = 238). BCVA improved in 50 % of eyes following fornix reconstructive procedures (n = 24) with COMET (n = 6), in 63.9 % eyes with the Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) lens (n = 36), in 81.8 % of eyes after cataract surgery (n = 22). A BCVA of ≥20/200 was achieved in 72.34 % of eyes following keratoprostheses procedures (n = 47). The mean duration of follow up was 53.3 ± 15.2 months.

Conclusion

The ocular sequelae of Stevens Johnson Syndrome can be blinding. They need to be identified and addressed early to retard the continued deterioration of the ocular surface. Our study aims to highlight the problem as well as the importance of comprehensive measures in the management of this potentially blinding disorder.

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References

  1. Power WJ, Ghoraishi M, Merayo-Lloves J, Neves RA, Foster CS (1995) Analysis of the acute ophthalmic manifestations of the erythema multi- forme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum. Ophthalmology 102(11):1669–1676

    Article  CAS  PubMed  Google Scholar 

  2. Howard GM (1963) The Stevens-Johnson syndrome: ocular prognosis and treatment. Am J Ophthalmol 55:893–900

    CAS  PubMed  Google Scholar 

  3. Arstikaitis MJ (1973) Ocular aftermath of Stevens-Johnson syndrome. Arch Ophthalmol 90:376–379

    Article  CAS  PubMed  Google Scholar 

  4. Wright P, Collin JR (1983) The ocular complications of erythema multiforme (Stevens Johnson syndrome) and their management. Trans Ophthalmol Soc U K 103:338–341

    PubMed  Google Scholar 

  5. Hsu M, Jayaram A, Verner R, Lin A, Bouchard C (2012) Indications and outcomes of amniotic membrane transplantation in the management of acute stevens-johnson syndrome and toxic epidermal necrolysis: a case–control study. Cornea 31(12):1394–1402

    Article  PubMed  Google Scholar 

  6. Gregory DG (2011) Treatment of acute Stevens-Johnson syndrome and toxic epidermal necrolysis using amniotic membrane: a review of 10 consecutive cases. Ophthalmology 118(5):908–914

    Article  PubMed  Google Scholar 

  7. Shammas MC, Lai EC, Sarkar JS, Yang J, Starr CE, Sippel KC (2010) Management of acute Stevens-Johnson syndrome and toxic epidermal necrolysis utilizing amniotic membrane and topical corticosteroids. Am J Ophthalmol 149(2):203–213

    Article  CAS  PubMed  Google Scholar 

  8. Thomas John MD, Gary N, Foulks MD, Michelle E, John KC, Dean H (2002) Amniotic membrane transplantation in the surgical management of acute Toxic Epidermal Necrolysis. Ophthalmology 109:351–360

    Article  PubMed  Google Scholar 

  9. Cher I (2003) Blink-related microtrauma: when the ocular surface harms itself. Clin Experiment Ophthalmol 31:183–190

    Article  PubMed  Google Scholar 

  10. Di Pascuale MA, Espana EM, Liu DT, Kawakita T, Li W, Gao YY, Baradaran-Rafii A, Elizondo A, Raju VK, Tseng SC (2005) Correlation of corneal complications with eyelid cicatricial pathologies in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis syndrome. Ophthalmology 112(5):904–912

    Article  PubMed  Google Scholar 

  11. Iyer G, Pillai VS, Srinivasan B, Guruswami S, Padmanabhan P (2009). Mucous Membrane Grafting For Lid Margin Keratinization in Stevens Johnson Syndrome (SJS):Results. Cornea. Dec 17

  12. Gomes JA, Santos MS, Ventura AS, Donato WB, Cunha MC, Höfling-Lima AL (2003) Amniotic membrane with living related corneal limbal/conjunctival allograft for ocular surface reconstruction in Stevens-Johnson syndrome. Arch Ophthalmol 121(10):1369–1374

    Article  PubMed  Google Scholar 

  13. Solomon A, Ellies P, Anderson DF, Touhami A, Grueterich M, Espana EM, Ti SE, Goto E, Feuer WJ, Tseng SC (2002) Long-term outcome of keratolimbal allograft with or without penetrating keratoplasty for total limbal stem cell deficiency. Ophthalmology 109(6):1159–66

    Article  PubMed  Google Scholar 

  14. Koizumi N, Inatomi T, Suzuki T, Sotozono C, Kinoshita S (2001) Cultivated corneal epithelial stem cell transplantation in ocular surface disorders. Ophthalmology 108(9):1569–1574

    Article  CAS  PubMed  Google Scholar 

  15. Nakamura T, Inatomi T, Sotozono C, Amemiya T, Kanamura N, Kinoshita S (2004) Transplantation of cultivated oral mucosalepithelial cells in patients with severe ocular surface disorders. Br J Ophthalmol 88:1280–1284

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  16. Solomon A, Ellies P, Anderson DF, Touhami A, Grueterich M, Espana EM, Ti SE, Goto E, Feuer WJ, Tseng SC (2002) Long-term outcome of keratolimbal allograft with or without penetrating keratoplasty for total limbal stem cell deficiency. Ophthalmology 109(6):1159–1166

    Article  PubMed  Google Scholar 

  17. Ang LP, Sotozono C, Koizumi N, Suzuki T, Inatomi T, Kinoshita S (2007) A comparison between cultivated and conventional limbal stem cell transplantation forStevens-Johnson syndrome. Am J Ophthalmol 143(1):178–180

    Article  PubMed  Google Scholar 

  18. Omoto M, Shimmura S, Hatou S, Ichihashi Y, Kawakita T, Tsubota K (2010) Simultaneous deep anterior lamellar keratoplasty and limbal allograft in bilateral limbal stem cell deficiency. Jpn J Ophthalmol 54(6):537–543

    Article  PubMed  Google Scholar 

  19. Sotozono C, Inatomi T, Nakamura T, Koizumi N, Yokoi N, Ueta M, Matsuyama K, Miyakoda K, Kaneda H, Fukushima M, Kinoshita S (2013) Visual improvement after cultivated oral mucosal epithelial transplantation. Ophthalmology 120(1):193–200

    Article  PubMed  Google Scholar 

  20. Koizumi N, Inatomi T, Suzuki T, Sotozono C, Kinoshita S (2001) Cultivated corneal epithelial stem cell transplantation in ocular surface disorders. Ophthalmology 108(9):1569–1574

    Article  CAS  PubMed  Google Scholar 

  21. Kinoshita S, Koizumi N, Nakamura T (2004) Transplantable cultivated mucosal epithelial sheet for ocular surface reconstruction. Exp Eye Res 78:483–491

    Article  CAS  PubMed  Google Scholar 

  22. Nishida K, Yamato M, Hayashida Y, Watanabe K, Yamamoto K, Adachi E, Nagai S, Kikuchi A, Maeda N, Watanabe H, Okano T, Tano Y (2004) Corneal reconstruction with tissue-engineered cell sheets composed of autologous oral mucosal epithelium. N Engl J Med 351:1187–1196

    Article  CAS  PubMed  Google Scholar 

  23. Ang AY, Chan CC, Biber JM, Holland EJ (2013) Ocular surface stem cell transplantation rejection: incidence, characteristics, and outcomes. Cornea 32(3):229–36

    Article  PubMed  Google Scholar 

  24. Sant’ Anna AE, Hazarbassanov RM, de Freitas D, Gomes JÁ (2012) Minor salivary glands and labial mucous membrane graft in the treatment of severe symblepharon and dry eye in patients with Stevens-Johnson syndrome. Br J Ophthalmol 96(2):234–239

    Article  PubMed  Google Scholar 

  25. Rosenthal P, Cotter P (2003) The Boston Scleral Lens in the management of severe ocular surface disease. Ophthalmol Clin North Am 16:89–93

    Article  PubMed  Google Scholar 

  26. Rathi VM, Mandathara PS, Dumpati S, Vaddavalli PK, Sangwan VS (2011) Boston ocular surface prosthesis: an Indian experience. IJO 59(4):279–281

    Google Scholar 

  27. Sotozono C, Ang LP, Koizumi N, Higashihara H, Ueta M, Inatomi T, Yokoi N, Kaido M, Dogru M, Shimazaki J, Tsubota K, Yamada M, Kinoshita S (2007) New grading system for the evaluation of chronic ocular manifestations in patients with Stevens-Johnson syndrome. Ophthalmology 114(7):1294–1302

    Article  PubMed  Google Scholar 

  28. Kaido M, Yamada M, Sotozono C, Kinoshita S, Shimazaki J, Tagawa Y, Hara Y, Chikama T, Tsubota K (2012) The relation between visual performance and clinical ocular manifestations in Stevens-Johnson syndrome. Am J Ophthalmol 154(3):499–511

    Article  PubMed  Google Scholar 

  29. Yao FU, Liu J, Tseng SCG (2011) Oral mucosal graft to correct Lid margin pathologic features in cicatricial ocular surface diseases. Am J Ophthalmol 152(4):600–608

    Article  Google Scholar 

  30. Ciralsky JB, Sippel KC, Gregory DG (2013) Current ophthalmologic treatment strategies for acute and chronic Stevens-Johnson syndrome and toxic epidermal necrolysis. Curr Opin Ophthalmol 24(4):321–328

    Article  PubMed  Google Scholar 

  31. Liu J, Sheha H, Fu Y, Giegengack M, Tseng SC (2011) Oral mucosal graft with amniotic membrane transplantation for total limbal stem cell deficiency. Am J Ophthalmol 152(5):739–747

    Article  PubMed  Google Scholar 

  32. Sayegh RR, Ang LP, Foster CS, Dohlman CH (2008) The Boston keratoprosthesis in Stevens-Johnson syndrome. Am J Ophthalmol 145(3):438–444

    Article  PubMed  Google Scholar 

  33. Iyer G, Pillai VS, Srinivasan B, Falcinelli G, Padmanabhan P, Guruswami S, Falcinelli G (2010) Modified osteo-odonto keratoprosthesis-the Indian experience-results of the first 50 cases. Cornea 29(7):771–776

    PubMed  Google Scholar 

  34. Hille K, Grabner G, Liu C, Colliardo P, Falcinelli G, Taloni M, Falcinelli G (2005) Standards for modified osteo-odonto keratoprosthesis (OOKP) surgery according to Strampelli and Falcinelli - the Rome–Vienna protocol. Cornea 24:895–908

    Article  PubMed  Google Scholar 

  35. Pujari S, Siddique SS, Dohlman CH, Chodosh J (2011) Outcomes of the Boston keratoprosthesis type II: the Massachusetts eye and ear infirmary experience (2011). Cornea 30(12):1298–1303

    Article  PubMed  Google Scholar 

  36. Maskati QB, Maskati BT (2006) Asian experience with the Pintucci Keratoprosthesis. Indian J Ophthal 54:89–94

    Article  Google Scholar 

  37. Falcinelli G, Falsini B, Taloni M, Colliardo P, Falcinelli G (2005). Modified osteo-odonto-keratoprosthesis for treatment of corneal blindness: long-term anatomical and functional outcomes in 181 cases.

  38. Tan A, Tan DT, Tan XW, Mehta JS (2012) Osteo-odonto keratoprosthesis: systematic review of surgical outcomes and complication rates. Ocul Surf 10(1):15–25

    Article  PubMed  Google Scholar 

  39. Liu C, Okera S, Tandon R, Herold J, Hull C, Throp S (2008) Visual rehabilitation in end-stage inflammatory ocular surface disease with the osteo-odonto-keratoprosthesis: results from the UK. Br J Ophthalmol 92(9):1211–1217

    Article  CAS  PubMed  Google Scholar 

  40. De La Paz MF, De Toledo JÁ, Charoenrook V, Sel S, Temprano J, Barraquer RI, Michael R (2011) Impact of clinical factors on the long-term functional and anatomic outcomes of osteo-odonto-keratoprosthesis and tibial bone keratoprosthesis. Am J Ophthalmol 151(5):829–839

    Article  Google Scholar 

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Conflict of interest

The authors have no financial or proprietary interest in any of the materials used in this study.

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Authors and Affiliations

  1. Medical Research Foundation, Sankara Nethralaya, 18 College Road, Chennai, 600006, Tamil Nadu, India

    Geetha Iyer, Bhaskar Srinivasan, Shweta Agarwal, Seema Kamala Muralidharan & Sumathi Arumugam

  2. CJ Shah Cornea Services, Dr G Sitalakshmi Memorial Clinic for Ocular Surface Disorders, Prof G Falcinelli Center for MOOKP, Medical Research Foundation, Sankara Nethralaya, 18, College Road, Chennai, 600 006, India

    Geetha Iyer

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  1. Geetha Iyer
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  2. Bhaskar Srinivasan
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  3. Shweta Agarwal
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Correspondence to Geetha Iyer.

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Iyer, G., Srinivasan, B., Agarwal, S. et al. Comprehensive approach to ocular consequences of Stevens Johnson Syndrome - the aftermath of a systemic condition. Graefes Arch Clin Exp Ophthalmol 252, 457–467 (2014). https://doi.org/10.1007/s00417-014-2568-8

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