Haddad syndrome also called as Hirschprung?s disease is a congenital disorder of colon causing chronic constipation. Haddad syndrome which causes enlargement of the colon, caused by bowel obstruction resulting from an aganglionic section of bowel that starts at the anus and progresses upwards.

 

Causes

Congenital aganglionosis of the distal bowel defines Haddad syndrome.

Aganglionosis begins with the anus, which is always involved, and continues proximally or upwards for a variable distance.

Both the myenteric (Auerbach) plexus and the submucosal (Meissner) plexus are absent, resulting in reduced bowel peristalsis and function.

Haddad syndrome is associated with

• Down syndrome
• Neurocristopathy syndromes
• Piebaldism
• Goldberg-Shprintzen syndrome
• Multiple endocrine neoplasia type II
• Congenital central hypoventilation syndrome

Clinical features

Approximately 10% of patients with Haddad syndrome have a positive family history

Haddad syndrome occurs more often in males than in females, with a male-to-female ratio of approximately 4:1.

• Newborn with delayed passage of meconium or in any child with a history of chronic constipation since birth
• Bilious vomiting
• Abdominal distention
• Poor feeding
• Failure to thrive
• About 10% of children may present with diarrhea caused by enterocolitis
• On examination- distended abdomen, spasm of anus, and inability to release the flatus

Tests and diagnosis

• Complete blood count
• Serum electrolytes
• Renal function test
• Coagulation studies
• Barium enema- reveals arrowed distal colon with proximal dilation
• Anorectal Manometry- detects the relaxation reflex of the internal sphincter after distension of the rectal lumen.
• Definitive diagnosis of Haddad syndrome is by rectal biopsy- indicates absence of ganglion cells.
• Cardiac evaluation- 2D echo for cardiac anomalies

Treatment

Goals of medical care

Management of complications of unrecognized or untreated Haddad syndrome

• Nil per orally, intravenous hydration by fluids (DNS or RL)
• Correction of electrolyte imbalance (sodium and potassium correction)
• Nasogastric decompression
• Administration of broad spectrum intravenous antibiotics

Colonic lavage may also need to be used in postoperative patients who develop enterocolitis as a complication.

Surgical care

Treatment of Haddad syndrome consists of surgical resection of the abnormal section of the colon, followed by reanastomosis

Common surgical procedure- Swenson and Duhamel surgery