Sight and comfort: complex procedures in end-stage Stevens–Johnson syndrome

Abstract

Background: We describe our complex surgical techniques in the management of a patient with end-stage ocular surface disease from Stevens-Johnson syndrome. Methods and results: Her severe discomfort due to absolute ocular dryness in the right eye was successfully treated with submandibular gland autotransplantation. Impending loss of the left eye due to repeated perforation and infection was prevented with a penetrating corneal graft covered by a new modification of a Cutler-Beard-type full-thickness lower lid skin advancement-flap. Visual rehabilitation was achieved by means of osteo-odontokeratoprosthesis. Conclusion: The procedures described allow the preservation of eyes doomed for enucleation, relief from severe discomfort and rehabilitation from corneal blindness. Due to their complex nature they require the collaborative subspecialist surgical skills of opthalmologists and maxillofacial surgeons.

Osteo-odonto-keratoprosthesis – A maxillofacial perspective

“…Of affected individuals, 35% experience permanent loss of vision (Howard, 1963;Geerling et al, 2003). In severe ocular surface disease corneal grafts fail because of compromised corneal wound healing (Tugal-Tutkun et al, 1995;Geerling et al, 2003). In addition to limbal stem cell deficiency, the absence of tears reduces epithelial viability due to lack of nutrition, mechanical and antibacterial protection.…”

“…In addition to limbal stem cell deficiency, the absence of tears reduces epithelial viability due to lack of nutrition, mechanical and antibacterial protection. Although new treatment modalities such as amniotic membrane transplantation and autologous serum drops have been tried they are not without failures (Chiou et al, 1998;Poon et al, 2001;Geerling et al, 2003). An ideal keratoprosthesis should be able to surpass the natural cornea by having an improved optical quality, with decreased aberrations and a specifiable power.…”

“…However SteveneJohnson syndrome; a drug-or infection-induced acute inflammatory reaction of skin and mucous membranes is the most common cause. Of affected individuals, 35% experience permanent loss of vision (Howard, 1963;Geerling et al, 2003). In severe ocular surface disease corneal grafts fail because of compromised corneal wound healing (Tugal-Tutkun et al, 1995;Geerling et al, 2003).…”

Transplantation of the Major Salivary Glands

“…It is only indicated in patients with absolute aqueous tear deficiency (Schirmer's test Յ1 mm) with persistent severe pain despite punctal occlusion, a trial of scleral or limbal fit rigid contact lenses, at least half-hourly application of unpreserved tear substitutes and a conjunctivalised corneal surface. For these patients the demand on time and financial resources is however justified not only by significant relief of severe symptoms and some signs of dry eye, but also by substantial gains for society [32,33]. Visual rehabilitation by means of keratoplasty nevertheless remains unsuccessful even after successful SMG transplantation.…”

Zum Stand der Keratoprothesen mit biologischer Haptik – Die Düsseldorf-Offenburger-Erfahrungen mit Osteo-Odonto- und Tibia-Keratoprothese

“…Auch das ästhetisch ungewöhnliche Ergebnis eines Auges mit dem äußeren Aspekt von Mundschleimhaut ist zumindest für Patienten und Angehörige gewöhnungsbedürftig und erfordert teilweise eine psychologische Begleitung. Aus unserer Sichtwie auch der meisten Patientenrechtfertigen aber sowohl die teilweise hervorragenden Visusergebnisse als auch die Langfristigkeit diese äußerst aufwendige Chirurgie, die zuvor dauerhaft Erblindete wieder Lesefähigkeit ermöglicht [18,19]].…”