A case of ultrashort-segment Hirschsprung's disease discovered after taking polyethylene glycol electrolyte solution

Yukihiro Tatekawa1

  • 1Department of Pediatric Surgery, Saku Central Hospital Advanced Care Center, Nagano 385-0051, Japan.

Insights

This case study details ultrashort-segment Hirschsprung

Area of Science:

  • Pediatric Surgery
  • Gastroenterology

Background:

  • Hirschsprung's disease (HD) is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
  • Ultrashort-segment HD involves aganglionosis limited to the rectum or rectosigmoid region.
  • Diagnosis can be challenging, especially in milder forms.

Observation:

  • A 4-year-old girl with a history of constipation since birth presented with acute abdominal distension.
  • Imaging revealed a significant stool burden and rectal/sigmoid dilation.
  • Anorectal manometry and rectal biopsy confirmed Hirschsprung's disease.

Findings:

  • The patient underwent a Soave endorectal pull-through procedure.
  • Postoperatively, stool frequency was initially high (4-10 times/day) but gradually decreased.
  • Long-term stool frequency normalized without ongoing medication.

Implications:

  • This case highlights the importance of considering ultrashort-segment HD in pediatric patients with chronic constipation.
  • Surgical intervention can be effective in managing symptoms.
  • The Soave procedure provided a favorable outcome in this patient.

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