Long term ocular manifestations of Stevens-Johnson syndrome and

toxic epidermal necrolysis in childrenAsim Ali, MD, FRCSC

Kamiar Mireskandari, MD, PhDCaroline Catt, MBBS, FRANZCO

No financial disclosures to report

SJS and TEN

SJS– <10% BSA– Adult mortality 1-3%– Mean age ~25 years

TEN– >30% BSA– Adult mortality 10-70%– Mean age ~53 years

SJS/TEN overlap– 10-30% BSA– Intermediate

form

SJS and TEN in children

• Compared to adults– Lower incidence– Better survival

• Acute ocular involvement in ~80%• Ocular manifestations in the acute and long

term, and visual acuity outcomes not well reported

METHODS

• Retrospective cohort review– Demographics– Admission details– Ophthalmic findings and treatment

• At every inpatient and subsequent outpatient review

• Inclusion criteria:– Admitted to Hospital for Sick Children, Toronto, Canada

from 2001 - 2011– Diagnosis according to Bastuji-Garin1 consensus definition

1. Bastuji-Garin S, Rzany B, Stern RS, et al. Clinical classification of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol 1993;129;92-6

RESULTS - Demographic Information

Patient Characteristics Mean value (range)

TotalMaleFemale

3622 (61%)14 (39%)

Age 8.8 (0.67 – 15) years

Admitted to ICU 11 (31%)

Mean duration of admission

15 (2 – 87) days

Mean duration of follow-up

13 months (day 0 – 9 years)

Diagnosis:SJSSJS/TENTEN

20 (56%)9 (25%)7 (19%)

Severity of acute ocular involvementSeverity criteria published by Power et al (1995)

SJS (n=20) SJS/TEN overlap (n=9)

TEN (n=7)0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Severe

Moderate

Mild

None

Conjunctival manifestations

Clinical Sign Number of patients affected (%)

Mean time of onset after admission (range)

Conjunctivitis 28 (77.8%) 1 day (0-9)

Conjunctival membranes 10 (27.8%) 1 day (0-5)

Bulbar conjunctival ulceration 14 (38.9%) 4 days (0-18)

Subconjunctival hemorrhage 12 (33.3%) 4 days (0-30)

Tarsal conjunctival ulceration 12 (33.3%) 6 days (0-20)

Symblepharon 10 (27.8%) 44 days (1-207)

Sub-conjunctival scarring 5 (13.9%) 3.8 months (0.6-11)

Ankyloblepharon 4 (11.1%) 5 months (0.4-18)

Eyelid manifestationsClinical Sign Number of patients

affected (%)Mean time of onset after admission (range)

Lid margin ulceration 9 (25%) 6 days (0-26)

Lid edema 14 (38.9%) 8 days (0-44)

Trichiasis 3 (8.3%) 24 days (10-31)

Blepharitis 2 (5.6%) 30 days (22-37)

Meibomian gland disease 9 (25%) 5.4 months (0.5-12)

Lid margin keratinisation 8 (22.2%) 4.8 months (0.5-21)

Entropion 3 (8.3%) 5.7 months (1-13)

Distichiasis 4 (11.1%) 9.5 months (3-24)

Punctal auto-occlusion 3 (8.3%) 10.8 months (1-30)

Corneal manifestations

Clinical Sign Number of patients affected (%)

Mean time of onset after admission (range)

SPEE 18 (50%) 3 days (0-9)

Corneal epithelial defect 9 (25%) 30 days (0-242)

Corneal opacification 4 (11.1%) 3.5 months (1.3-5)

Dry eye 10 (27.8%) 6.7 months (2-17)

Corneal vascularisation 3 (8.3%) 12.3 months (3.8-22)

Limbal stem cell failure 3 (8.3%) 16 months (4 months-3 years)

Long term follow-up group: chronic manifestations

• All patients with at least one review after discharge were analyzed separately

• 17 met these criteria (6 SJS, 6 SJS/TENS and 5 TENS)

• Complications in this group– Corneal opacification (23.5%),– Corneal neovascularization (17.6%)– Limbal stem cell failure (17.6%)– New symblephara (35.3%)

Long term follow-up group: visual acuity outcome + interventions

VA>20/40 VA <= 20/50 VA<20/200

Better eye 17 (100) 0 0

Worse eye 14 (82.4%) 2 (11.8%) 1 (5.9%)

• Mean follow-up 26.9 mo• 4 patients required a PROSE device

(Boston Foundation for Sight, Needham, MA)

• 1 patient required eyelid repair for entropion

CONCLUSIONS

• Ocular manifestations are acutely present in 81% of children, and 100% of those with TEN

• 11% of all patients required surgical intervention for their ophthalmic sequelae

• Prolonged follow-up is indicated to identify late ocular complications

• Despite high frequency of vision threatening complications, most children maintain good vision