Rare Diseases Symptoms Automatic Extraction

Outcome after anal intrasphincteric botox injection in children with surgically treated hirschsprung disease.

[hirschsprung disease]

A nonrelaxing internal anal sphincter is present in a relatively large proportion of children with surgically treated Hirschsprung disease (HD) and can cause obstructive gastrointestinal symptoms. The short- and long-term outcome and adverse effects of intrasphincteric botulinum toxin (Botox) injections in children with obstruction after surgically treated HD are evaluated.The outcome of children with surgically treated HD treated with intrasphincteric Botox injections for obstructive symptoms was analyzed with a retrospective chart review between 2002 and 2013 in the University Medical Centers of Maastricht and Nijmegen.A total of 33 patients were included. The median time of follow-up was 7.3 years (range 1-24). A median of 2 (range 1-5) injections were given. Initial improvement was achieved in 76%, with a median duration of 4.1 months (range 1.7-58.8). Proportion of children hospitalized for enterocolitis decreased after treatment from 19 to 7. A good long-term response was found in 49%. Two children experienced complications: transient pelvic muscle paresis with impairment of walking. In both children symptoms resolved within 4 months without treatment.Intrasphincteric Botox injections in surgically treated HD are an effective long-term therapy in approximately half of our patients with obstructive symptoms. The possibility of adverse effects should be noticed.

Diseases presenting "gastrointestinal symptoms" symptom

  • alpha-thalassemia
  • congenital toxoplasmosis
  • cutaneous mastocytosis
  • familial mediterranean fever
  • hirschsprung disease
  • junctional epidermolysis bullosa
  • kindler syndrome
  • malignant atrophic papulosis
  • systemic capillary leak syndrome
  • typhoid

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