1. Long term ocular manifestations of Stevens-Johnson syndrome and toxic epidermal necrolysis in children
Asim Ali, MD, FRCSC
Kamiar Mireskandari, MD, PhD
Caroline Catt, MBBS, FRANZCO
No financial disclosures to report

2. SJS and TEN SJS SJS/TEN overlap TEN <10% BSA Adult mortality 1-3%
Mean age ~25 years
SJS/TEN overlap
10-30% BSA
Intermediate form
TEN
>30% BSA
Adult mortality 10-70%
Mean age ~53 years
Rare but severe mucocutaneous blistering diseases COMMONLY INVOLVING THE OCULAR SURFACE
The clinical entity between these 2 ends of the spectrum is designated Overlap Syndrome.

3. 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
An important disease for us to understand in children. Although it has a lower incidence, survival is better, so there is potentially a greater disease burden, long-term, in this population with a long life ahead of them.
Mortality of 0-17% in TEN in children cf adults in which it is ~30%

4. METHODS Retrospective cohort review Inclusion criteria: 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
Diagnosis according to Bastuji-Garin1 consensus definition
We undertook an REB approved..
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

5. RESULTS - Demographic Information
Patient Characteristics
Mean value (range)
Total
Male
Female 36
22 (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:
SJS
SJS/TEN
TEN
20 (56%)
9 (25%)
7 (19%)

6. Severity of acute ocular involvement Severity criteria published by Power et al (1995)
We classified the severity of ocular involvement….
81% had ocular involvement acutely.
Mild involvement was defined as ocular signs requiring routine eye care, which completely resolved before discharge from hospital (eg. lid edema, conjunctival injection, requiring prophylactic antibiotics or lubricants); Moderate involvement included specific ocular complications requiring specific treatment, normal vision and near complete resolution of ocular disease prior to discharge (eg. conjunctival membranes, corneal epithelial loss of more than 30%), and; Severe involvement included sight-threatening disease with the need for ongoing specific ophthalmic treatment after discharge (eg. symblepharon, active corneal disease at the time of discharge). Where there was asymmetry between the two eyes, the more severe category was recorded.

7. 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
6 days (0-20)
Symblepharon
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)

8. Eyelid manifestations
Clinical 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
5.4 months (0.5-12)
Lid margin keratinisation
8 (22.2%)
4.8 months (0.5-21)
Entropion
5.7 months (1-13)
Distichiasis
4 (11.1%)
9.5 months (3-24)
Punctal auto-occlusion
10.8 months (1-30)
Changes in the eyelids also occurred with high frequencies and onset over a large time period ranging between…

9. 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
16 months (4 months-3 years)

10. 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%)

11. Long term follow-up group: visual acuity outcome + interventions
VA>20/40
VA <= 20/50
VA<20/200
Better eye
17 (100)
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

12. 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
Ocular manifestations are acutely present in 81% of children, and 100% of those with TENS – securing a role for the ophthalmologist in the care of these children in the acute care setting