​Pharmacology - Hirschsprung Disease

​Pharmacology - Hirschsprung Disease
Caused during embrogenesis by a lack of neural crest cell migration
Is linked to Down syndrome; affects boys more than girls; occurs in 1 in 3000 births.
Gross: Colon dilatation near the aganglionic segment (megacolon): the rectum is involved.
Macroscomic: Meissner and Auerbach plexus ganglion cels absent

Clinical Signs and Symptoms 
occurs early in childhood and manifests as a colon perforation or persistent constipation with bloating in the abdomen; it can also manifest as acute enterocolitis with watery, foul-smelling stools.

Management 
Surgery and Anastomosis 
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The term "Ogilvie syndrome," also known as "acute colonic pseudo-obstruction," describes the radiological and clinical manifestation of colonic obstruction when there isn't a real obstruction. Older patients with a history of trauma, infection, or specific prescription use (such as opioids or anticholinergics) are more likely to experience it. Significant colonic dilatation with low air-fluid levels is seen on the abdominal x-ray. The course of treatment involves the installation of a nasogastric tube, maintaining NPO state, stopping any problematic medications, and, if necessary, colonoscopic decompression. If left untreated, this illness may develop into a colonic perforation.