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Chronic Intestinal Pseudo-Obstruction (CIPO) is a rare, severe motility disorder of the gastrointestinal tract characterized by signs and symptoms of intestinal obstruction without any mechanical blockage.
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It is due to failure of coordinated peristalsis from neuromuscular abnormalities affecting the enteric nervous system, smooth muscle, or both.
Etiopathology
Primary (idiopathic / genetic forms):
- Neuropathic: enteric nerve degeneration or maldevelopment (e.g., Hirschsprung-like syndromes, mitochondrial neurogastrointestinal encephalomyopathy – MNGIE).
- Myopathic: smooth muscle disorders (familial visceral myopathy, scleroderma-like).
- Mixed forms.
Secondary (acquired forms):
- Systemic diseases: scleroderma, systemic lupus erythematosus, amyloidosis, diabetes mellitus, Parkinson’s disease.
- Post-infectious neuropathies.
- Paraneoplastic syndromes (anti-Hu antibodies).
- Chronic use of drugs affecting motility (e.g., opioids, anticholinergics).
Clinical Presentation
- Chronic or recurrent episodes of bowel obstruction: abdominal pain, distension, bloating.
- Nausea, vomiting, constipation, or diarrhea.
- Malnutrition and weight loss due to poor absorption and dietary restriction.
- Pediatric cases: failure to thrive, feeding intolerance.
- Complications: bacterial overgrowth, sepsis, electrolyte imbalance, intestinal failure.