PATHOPHYSIOLOGY Hirschprung’s disease or congenital aganglionic megacolon is a b owel obstruction resulting to absence of ganglion cells in the myenteric plexus

. It is named after Harald Hirschsprung, the Danish physician who first described the disease in 1886, describing two infants who had died with swollen bellies. The ganglion cells reside in the adrenal medulla and are involved in the SNS rel ease of epinephrine and norepinephrine. They are special nerve cells in the inte stine that makes the muscles push stool to the anus. The myenteric plexus is a m ajor nerve supply in the GI system which controls GIT motility where the ganglio n cells enter. It is has two kinds namely-- Auerbach’s plexus and Meissner’s ple xus. Auerbach’s plexus provides motor innervations plus secretomotor for PNS and SNS while Meissner’s plexus works parasympathetically only. However, failure of the ganglion cells to migrate in the craniocaudal area results to absent gangli on cells in the myenteric’s plexuses in the submucosa. Parasympathetic innervati on is depressed which results to uninterrupted colonic, usually rectosigmoid, co ntraction. Decreased PNS stimulation signifies inadequate expression of synaptic neurotransmitter, Ach and NO. Due to absence of ganglion cells in the myenteric plexus, especially Meissner’s plexus, there is an increase in synaptic activity of acetylcholinesterase, an enzyme which degrades acetylcholine activity and a decrease of nitric oxide, an enzyme responsible for the relaxation of smooth mus cles in the endothelium in the recto-sigmoid segment. Both of these factors inhi bit relaxation of the contracted segments. These strings of events will result t o an ineffective peristaltic movement that causes impaction of stool in the larg e intestines. Bowel distention also results from impaction of feces and flatus. The abdominal distention and fecal impaction will result to intestinal perforati on and constipation. Moreover, it will cause bacterial overgrowth in the intesti nes and mucosal irritation of the linings of the intestines that may result to c omplications such as enterocolitis and diarrhea, which is the most common compli cation. If left untreated, hypovolemic shock will follow.

Absence of Ganglion Cells in Myenteric Plexuses Inadequate expression of PNS stimulation (↑acetylcholinesterase, ↓NO) Inhibits relaxation of contracted segment Ineffective peristaltic movement Bowel distention 2o to fecal stagnation and gas Bacterial Overgrowth Mucosal Irritation Perforation Constipation Enterocolitis and Diarrhea (most common complication)

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