Hirschsprung’s Disease (Aganglionic Megacolon)

Definition: It involves an enlargement of the colon, caused by the bowel obstruction resulting from an aganglionic section of bowel or absence of ganglion cells – nerve do not function correctly and normal relaxation of bowel cannot occur. The length of the bowel that is affected varies but seldom stretches for more than about 30 cm.

Clinical Manifestations:

1. Newborn period:

a. abdominal distention

b. failure of passage of meconium within the first 48 hours of life

c. develops disinterest in feeding

d. repeated vomiting

2. Older infants and children

a. suffers from chronic constipation

b. tympanic abdominal distention

c. poor feeding

d. failure to thrive

e. irritable and fretful

f. Diarrheal enterocolitis – common complication below 3 months

g. fever

i. passage of ribbon like stools

Diagnostic examinations:

• Barium enema may reveal megacolon
• Rectal biopsy
• Anorectal Manometry – it shows that the external sphincter contracts normally but does not relax

Etiology:

• Heredity – The incidence is sporadic, however there is an increasing incidence in the family
• Associated conditions

  -strongly associated with Down syndrome ( 5-15% of patients with Hirschsprung disease also have trisomy21)

• Waardenburg syndrome, congenital deafness, malrotation, gastric diverticulum and intestinal atresia

Medical Management:

• Initial intravenous hydration, NPO (nothing per orem) status, intestinal and gastric decompression
• Decompression – Nasogastric tube insertion or either digital rectal examination or normal saline rectal irrigations 3 to 4 times daily.
• Surgical options: Colostomy at the level of normal bowel

*Surgery done in a 3 stage approach:

1. Temporary colostomy

2. Definitive Repair / Diversion Procedure

a. Abdomino-Perineal Procedure

b. Duhamel

c. Scaves Approach- Endorectal – Pulithru

c.1. done 8 months to 1 year

c.2. Infants has weight of 20 pounds

3. Closure of colostomy

Nursing Management:

• Asses immediately for enterocolitis.
• Avoid taking body temperature rectally because it may damage the mucosal lining

• Prevention of constipation – small frequent feeding with low residue diet
• Administration of oral and systemic antibiotics
• Use of stool softeners
• Provide a comfortable position- Semi-fowler’s to relieve dyspnea of abdominal distention
• Encourage the family members to have a diet which includes corn and rice
• Encourage parents to verbalize their anxieties as well as teach them the proper care of colostomy.

Nursing Care Plan – Hirschsprung’s Disease

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