Congenital Aganglionic Megacolon Flashcards

1
Q

Definition

A

• Functional obstruction of the colon due to absence of ganglion cells in bowel wall

• Commonest site: rectosegmoid in 75%
. 10% of cases total —->ileostomy

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2
Q

Incidence

A
  • 1 / 5000

* Male: Females = 4: 1 (positive family history may be present).

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3
Q

Clinical picture/Presentation

A

Neonatal (80%):
Delayed passage of meconium beyond 48 hours.

• May be acute obstruction

In older child:
Chronic constipation and abdominal distension.

• Large fecal mass felt in left lower abdomen with empty rectum

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4
Q

Clinical picture/

Complicated cases:

A

🌚„ Enterocolitis:

  • Infection with clostridia difficile, staph aureus and anaerobes
  • Presented with bloody diarrhea & toxemia

🌚 Intermittent attacks of intestinal obstruction.

🌚 Failure to thrive due to protein losing enteropathy

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5
Q

Investigations

A

🌚🌝contrast enema
Barium enema:→ olderthen 1m
Gastrographin → younger then 1m

• Classic findings: an abrupt narrow transition zone between the normal dilated proximal colon and a smaller caliber obstructed distal aganglionic segment

🌚🌝 Rectal suction biopsy (from narrow segment)
• The gold standard for diagnosing Hirschsprung disease
• Reveals absent ganglia + hyper trophid neve endings

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6
Q

TX

A

pull-through procedure: surgical resection of aganglionic intestinal segment and anastomosis of remaining intestine to anus Either in newborn period or staged if extensive aganglionosis

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7
Q

Prognosis

A

Most have normal/ near-normal anorectal function Complications: fecal incontinence and constipation, postoperative enterocolitis (medical emergency if progresses to sepsis)

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