Original Articles
Surgical treatment of children blinded by Stevens-Johnson syndrome

https://doi.org/10.1016/S0002-9394(99)00224-XGet rights and content

Abstract

PURPOSE:

The surgical treatment of severe Stevens-Johnson syndrome is considered to be very difficult, especially in children. However, ocular surface reconstruction is possible in certain cases.

METHODS:

We have performed ocular surface reconstruction by allogeneic corneal epithelial stem cell transplantation in four children blinded by Stevens-Johnson syndrome.

RESULTS:

Two cases failed, and the other two had excellent results. The successful cases had good lacrimal function and conjunctival epithelium, with clear corneal stroma and pathology limited to the superficial ocular tissue, whereas the failures did not.

CONCLUSIONS:

The successful ocular surface reconstruction has been stable for more than 1 year in two cases, suggesting that some patients with Stevens-Johnson syndrome are very good candidates for ocular surface reconstruction, especially when the patients have good tear function and healthy conjunctival epithelium.

Section snippets

Patients and methods

We have treated four children, all boys aged 8 to 10 years (Table 1), since we started performing ocular surface reconstruction for Stevens-Johnson syndrome in 1994. We used slit-lamp biomicroscopy and impression cytology7 to evaluate the ocular surface and check for healthy conjunctival epithelium. Impression cytology measures the presence of goblet cells or skin-like cells on the bulbar conjunctiva by pressing a small strip of filter paper (Millipore, Bedford, Massachusetts) after topical

Results

Among four children on whom we performed ocular surface reconstruction, two cases were unsuccessful, whereas the other two cases were completely successful, providing almost normal visual acuity. The preoperative conditions were different in the successful and unsuccessful cases, resulting in different outcomes. In the two unsuccessful cases the patients had a completely keratinized epithelium without conjunctival epithelium and no reflex tearing. The two successful cases had a healthy

Discussion

Although Stevens-Johnson syndrome is a rare disease, it can occur at any age, often causing blindness in both children and adults. Cicatricial keratoconjunctivitis has been considered difficult to treat, with poor surgical results.10, 11 Although the new approach of using epithelium stem cell transplantation has begun, the results are not yet promising.5, 11 In our latest report, ocular surface reconstruction failed in children.5 However, because such impaired vision can have a substantial

Acknowledgements

We acknowledge the referrals from and discussions with Drs Woo Chan Park (Case 3), Akitoshi Yoshida and Sho Igarashi (Case 1), Mitsuru Nakazawa and Yuko Wada (Case 4), and Takahiko Motohashi (Case 2). We also acknowledge Drs Scheffer Tseng and Edward Holland for their consultation on one of our patients.

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This study was supported by the Medical School Faculty and Alumni Grants of Keio University Medical School, Tokyo, Japan.

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