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7.8.2

Hirschsprungs Disease

Malawi Standard Treatment Guidelines, 6th Edition, 2023. Chapter 7, Gastrointestinal Conditions.

Clinical Description

A congenital condition in which the rectum and part of the colon fail to develop a normal system of nerves, leading to an accumulation of faeces in the colon following birth.

Signs and Symptoms

This may be suspected in

  • Delayed passage of stools/meconium in a neonate
  • Chronic constipation since birth
  • Some present later with signs of bowel obstruction (bilious vomiting, abdominal distension), poor feeding and failure to thrive

Investigations

  • FBC
  • AXR

Non-pharmacological

  • This condition has a life-threatening complication of enterocolitis which may present with sepsis and shock from bacterial overgrowth
  • These patients need to be referred to a tertiary hospital.

Pharmacological

A rectal washout and antibiotics (Ceftriaxone 50mg/kg/dose and Metronidazole 7.5mg/kg/day) may be considered before referral in the event of:

  • Abdominal distension
  • Vomiting
  • Fever
  • Gush of stools on DRE.

*These are signs of an enterocolitis

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