I suffer from chronic idiopathic intestinal pseudo-obstruction, gastroparesis, diffuse esophageal spasms, achalasia, GERD and other diseases. Founder of AGMD.
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Visit the Center of Education page on our website, under the Gastroparesis section, to watch Dr. Kenneth Koch discuss Gastroparesis and Electrical Stimulation Therapy.
https://t.co/Zie4MCx6RH.
#agmdhope, #gimotility, #gastroparesis, #enterra
Chronic Intestinal Pseudo-Obstruction (CIPO): A severe motility disorder resembling mechanical obstruction. New review highlights gaps in data on surgical options, including intestinal transplants, and calls for improved research. #CIPO#GIHealth#Science https://t.co/JWyiomwTBW"
Hirschsprung Disease (Congenital Aganglionic Megacolon) is a congenital disorder of the intestine characterized by the absence of ganglion cells in the submucosal (Meissner) and myenteric (Auerbach) plexuses, most commonly affecting the distal colon and rectum. This results in a functional intestinal obstruction due to failure of relaxation of the affected bowel segment.
Etiology & Pathophysiology-
Caused by failure of neural crest cell migration during embryogenesis.
The aganglionic segment remains tonically contracted, while the proximal bowel becomes dilated (megacolon).
Can be sporadic or genetic (associated with mutations in the RET proto-oncogene).
Associated conditions: Down syndrome, congenital heart disease, MEN type 2.
Epidemiology Incidence: ~1 in 5,000 live births-Male predominance (≈4:1)
Majority present in the neonatal period
Clinical Features
Neonates -Failure to pass meconium within 24–48 hours ,Abdominal distension ,Bilious vomiting
Explosive stool on digital rectal examination
Infants & Children -Chronic constipation since birth
Poor feeding, failure to thrive
Recurrent enterocolitis (life-threatening)
Adults (rare) -Long-standing severe constipation Abdominal distension
Diagnosis -Contrast enema: Narrow distal segment with proximal dilatation (transition zone)
Anorectal manometry: Absence of recto-anal inhibitory reflex (RAIR)
Gold standard: Rectal biopsy showing: Absence of ganglion cells
Increased acetylcholinesterase
-positive. nerve fibers
Management Definitive treatment is surgical
Resection of aganglionic segment with pull-through procedures:
Swenson , Duhame , Soave
Temporary stoma may be required in sick neonates
Complications Hirschsprung-associated enterocolitis (HAEC)
Bowel perforation
Post-operative constipation or incontinence
Prognosis -Excellent with early diagnosis and appropriate surgery
Long-term bowel function usually satisfactory #MedTwitter #MedEd #MedX