Botulinum toxin is efficient to treat obstructive symptoms in children with Hirschsprung disease

Obstructive symptoms are common after pull-through for Hirschsprung disease. Botulinum toxin injection treatment may improve the bowel function if internal sphincter achalasia is the cause of obstructive symptoms. The aim of this study was to review the outcome in patients treated with intrasphincteric botulinum toxin injections after pull-through for Hirschsprung disease.

The operative records were used to identify children with Hirschsprung disease who were treated with botulinum toxin injections at Karolinska University Hospital, Stockholm, Sweden, from September 2007 to November 2014. Data on age, sex, associated syndromes, length of aganglionic segment, age at pull-through, type of pull-through, age at first botulinum toxin injection, indication for botulinum toxin injection, and effect of first botulinum toxin injection were retrieved from the case records. Bowel function at last follow-up visit or telephone contact was recorded.

Nineteen patients were identified. All had biopsy-verified Hirschsprung disease. Eighteen (15 males and 3 females) children had undergone intrasphincteric botulinum toxin injection treatment for obstructive symptoms after pull-through, which was done at 127 (18-538) days of age. Four children had total colonic aganglionosis. The first botulinum toxin injection was given at 2.4 (0.53-6.9) years of age. Thirteen children (72 %) had a good response to the first injection treatment. The children underwent 3 (1-13) injection treatments. At follow-up four patients had improved and did not need treatment for obstruction, four were scheduled for further botulinum toxin injections, eight had persistent obstructive symptoms treated with laxatives or enemas, and two children had an ileostomy.

Botulinum toxin injection treatment improves the obstructive symptoms in children after pull-through for Hirschsprung disease. The effect is reversible and a majority of patients need repeat injections. When injection treatment is not repeated, a large proportion of children need laxatives or enemas due to recurrent symptoms.