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[Chronic intestinal pseudo-obstruction: pathogenesis, diagnosis and therapy]
Summary
Chronic intestinal pseudo-obstruction (CIPO) is a rare motility disorder causing ileus symptoms without blockage. Diagnosis involves imaging, histology, and manometry, with treatment focusing on nutrition, motility, and complications.
Area of Science:
- Gastroenterology
- Internal Medicine
- Rare Diseases
Context:
- Chronic intestinal pseudo-obstruction (CIPO) presents as a severe intestinal motility disorder.
- Patients exhibit symptoms mimicking mechanical ileus but without physical obstruction.
- CIPO can be primary or secondary to various systemic or external factors.
Purpose:
- To outline the diagnostic approaches for CIPO.
- To detail the therapeutic strategies for managing CIPO.
- To emphasize avoiding unnecessary surgical interventions.
Summary:
- CIPO diagnosis requires ruling out mechanical obstruction via radiology and endoscopy.
- Histologic examination of full-thickness intestinal biopsies and small intestinal manometry are crucial.
- Manometry aids in diagnosing CIPO during asymptomatic periods and differentiating neuropathic from myopathic forms.
Impact:
- Effective management involves nutritional support, prokinetic agents, and addressing complications like bacterial overgrowth.
- Home parenteral nutrition and intestinal transplantation are options for severe cases, especially in children.
- Avoiding unnecessary laparotomies is critical to prevent complications and worsening of the clinical course.