Author Affiliations: Department of Pediatric Surgery, Children's University Hospital, Inselspital, University of Bern, Switzerland (Berger S, Rufener J, Klimek P, Zachariou Z, Boillat C)

Corresponding Author: S Berger, MD, Department of Pediatric Surgery, Children's University Hospital, Inselspital, University of Bern, CH-3010 Bern, Switzerland (Tel: 0041-31-632-9274; Fax: 0041-31-632-9292; Email: steffen.berger@insel.ch)

doi: 10.1007/s12519-012-0356-2

Background: After resection of long-segment Hirschsprung's disease, severe perianal dermatitis (SPAD) may occur because of high stool frequency and elevated concentrations of fecal pancreatic proteases. We investigated prospectively the effect of potato-derived protease inhibitors (PPI) on skin conditions in children with postoperative SPAD.

Methods: Four children (aged 12 to 24 months) with therapy-resistant SPAD after transanal endorectal pull-through for long-segment Hirschsprung's disease received topical PPI (1% in 20% zinc ointment) with each diaper-change. Parents noted down a subjective dermatitis score daily. Photo documentation and outpatient visits were made to assess the treatment results.

Results: No adverse effects were observed after treatment with PPI. A remarkable improvement of the dermatitis in 3 of the 4 patients as well as improvements in pain and sleep disorders were observed during the PPI-treatment course.

Conclusion: The results of the study suggest that PPI may reduce otherwise intractable protease-induced skin irritation in infants.

Key words:  Hirschsprung's disease; perianal dermatitis; protease inhibitor; transanal endorectal pull-through

World J Pediatr 2012;8(2):173-176