Key takeaways:
- The eyes of sufferers with SJS/TEN ought to be urgently evaluated and handled to keep away from sequelae.
- Cryopreserved amniotic membrane can promote therapeutic and restore the ocular floor.
Stevens-Johnson syndrome and its extra extreme variant, poisonous epidermal necrolysis, are uncommon and harmful immunologic ailments.
Stevens-Johnson syndrome (SJS) and poisonous epidermal necrolysis (TEN) lead to rapid-onset keratinocyte apoptosis and irritation inflicting blistering and sloughing of the pores and skin and the mucosal epithelium that strains the mouth, eyes and eyelids (Kohanim et al.).
SJS/TEN is often induced by an hostile response to medicine, mostly antibiotics, antiepileptics and allopurinol. Nevertheless, mycoplasma infections may set off an identical however clinically distinct situation referred to as Mycoplasma pneumoniae-induced rash and mucositis (Metcalfe et al.; Haseeb et al.).
Acute ocular involvement happens within the majority of SJS/TEN instances, which may result in persistent ocular sequelae reminiscent of extreme dry eyes, symblepharon and decreased imaginative and prescient in no less than one-third of sufferers (Kohanim et al.). Youngsters typically have extra extreme ocular problems than adults (Chen et al.), and in my expertise, the illness development tends to be extra fulminant in pediatric sufferers. It could be needed to put pediatric sufferers underneath sedation to get a correct analysis.
Preliminary prognosis and ocular illness development
Sufferers with SJS/TEN could initially current with flu-like signs and a rash (Shaw et al.). As a result of these signs are nonspecific, resembling higher respiratory infections or drug rashes, SJS/TEN could also be tough to diagnose in its early levels. Usually, it isn’t till a second or third journey to the emergency room that the situation will probably be acknowledged and the affected person admitted. If involvement of the mouth or airways interferes with consuming or respiratory, sufferers with SJS/TEN could also be positioned in intensive care.
Picture: Darren Gregory, MD
Ocular signs are categorized by the diploma of epithelial sloughing, as noticed by fluorescein staining. Delicate instances could solely present conjunctival hyperemia, whereas reasonable instances could have sloughing of lower than 33% of the lid margin and fewer than 1 cm of the conjunctiva. Instances are thought of extreme if they’ve sloughing of greater than 33% of the lid margin on no less than one lid, any epithelial defect greater than punctate staining on the cornea, or epithelial sloughing of greater than 1 cm on the conjunctiva. Extraordinarily extreme instances could have all of those options without delay. To reduce the danger for extreme ocular sequelae, these instances require intensive eye care, together with pressing amniotic membrane transplantation to the lids and ocular floor (Gregory).
Managing SJS/TEN
SJS/TEN could severely have an effect on the eyes even when a affected person shouldn’t be in essential situation from different features of the illness. I be sure to judge sufferers admitted with SJS/TEN every day to observe illness development. This analysis ought to embody fluorescein staining of the floor of the eyes and the backs of the eyelids, the place vital sloughing can happen even when the remainder of the attention seems comparatively unaffected (Gregory). I additionally rinse sufferers’ eyes with saline every day as a result of there could also be some therapeutic profit to cleansing off the buildup of particles and mucus. This rinsing additionally permits a cleaner and clearer examination. The eyes are handled with antibiotic and corticosteroid drops throughout the acute section of the illness.
Throughout post-discharge follow-up, I carry out guide expression of sufferers’ meibomian glands to clear any particles and mitigate any problems that will come up from meibomian gland dysfunction.
Using systemic corticosteroids and intravenous immunoglobulin has been explored in SJS/TEN to various and controversial outcomes (Gregory). Using etanercept as a monotherapy and together with steroid remedy has proven some promise in bettering remedy outcomes in sufferers with SJS/TEN, however additional analysis is required to realize a whole understanding of its efficacy (Mukherjee et al.).
Surgical administration of SJS/TEN
When surgical intervention is required, cryopreserved amniotic membrane (CAM; AmnioGraft, BioTissue) has been a sport changer for bettering remedy outcomes. CAM has potent anti-inflammatory and anti-scarring properties that promote therapeutic and assist restore the ocular floor. Early software of CAM has proven effectiveness in stopping probably the most extreme problems related to SJS/TEN (Gregory; Di Pascuale et al.).
If a affected person has vital sloughing of the conjunctiva on the again of the eyelids, the floor of the attention or the pores and skin alongside the perimeters of the eyelids, we’ll carry out amniotic membrane transplantation, often inside 24 hours. I desire to carry out this process within the working room underneath normal anesthesia, but when needed, it’s also potential to carry out this process on the bedside underneath topical and native anesthetic. Instead of sutures, cyanoacrylate glue can be utilized to fixate the CAM to the exterior lid pores and skin and permit the CAM to be utilized with no anesthetic injections (Shanbhag et al.).
When performing the amniotic membrane transplantation, we all the time cowl the lid margins and backs of the eyelids with a sheet of CAM. Usually, we’re ready to make use of a sutureless CAM/symblepharon ring machine (Prokera, BioTissue) for remedy of the ocular floor. Nevertheless, if there’s in depth sloughing of the bulbar conjunctiva on the floor of the attention, we’ll as an alternative suture a sheet of amniotic membrane over the whole ocular floor moderately than simply inserting a Prokera. To reduce long-term scarring sequelae, you could deal with the lids and palpebral conjunctiva with CAM. Merely inserting a Prokera on the ocular floor shouldn’t be ample remedy in extreme instances. In my expertise, nonetheless, performing full amniotic membrane transplantation within the first week of the sickness has been instrumental in stopping probably the most devastating sequelae of SJS/TEN.
Conclusion
Because of the rapidity with which SJS/TEN can advance and the potential severity of its sequelae, it’s crucial that ophthalmologists be acquainted with the latest remedy choices for SJS/TEN. They need to additionally pay attention to the place sufferers might be transferred, if needed, to obtain CAM transplantation in a well timed method. With early and aggressive remedy, we will considerably mitigate lots of the most damaging ocular problems of this probably blinding situation.
References:
Chen YL, et al. Am J Ophthalmol. 2023;doi:10.1016/j.ajo.2023.08.009.
Di Pascuale MA, et al. Ophthalmology. 2005;doi:10.1016/j.ophtha.2004.11.035.
Gregory DG. Ocul Surf. 2008;doi:10.1016/s1542-0124(12)70273-2.
Gregory DG. Ophthalmology. 2011;doi:10.1016/j.ophtha.2011.01.046.
Gregory DG. Ophthalmology. 2016;doi:10.1016/j.ophtha.2016.04.041.
Haseeb A, et al. Ocul Surf. 2023;doi:10.1016/j.jtos.2022.11.007.
Kohanim S, et al. Ocul Surf. 2016;doi:10.1016/j.jtos.2016.02.001.
Metcalfe D, et al. Entrance Med (Lausanne). 2021;doi:10.3389/fmed.2021.662897.
Mukherjee EM, et al. Ann Allergy Bronchial asthma Immunol. 2022;doi:10.1016/j.anai.2022.06.025.
Shaw B, et al. Am J Case Rep. 2023;doi:10.12659/AJCR.941992.
Shanbhag SS, et al. Ocul Surf. 2019;doi:10.1016/j.jtos.2019.03.001.
For extra info:
Darren Gregory, MD, an ophthalmologist training on the College of Colorado Sue Anschutz-Rodgers Eye Middle who focuses on cataract surgical procedure and remedy of ailments of the cornea and ocular floor, might be reached at darren.gregory@cuanschutz.edu.
