Related Experiment Videos
[Chronic idiopathic intestinal pseudo-obstruction: visceral myopathy. Report of 4 cases]
A F de Pini1, M T de Dávila, A Marín
1Hospital Nacional de Pediatría Prof. Juan P. Garrahan, Buenos Aires, República Argentina.
Abstract:
Chronic intestinal pseudo-obstruction is the term applied to a heterogeneous group of functional motility disorders sharing a common clinical expression: signs and symptoms of bowel obstruction in absence of mechanical occlusion. It is caused by ineffective intestinal propulsion. The chronic form of intestinal pseudo-obstruction may be primary or secondary. Primary pseudo-obstruction or chronic idiopathic pseudo-obstruction (CIIP) defines a group of propulsive disorders having no recognized underlying diseases. This study presents four female patients, aged between 4 months to 7 years, and makes a review of the literature. The symptoms, very similar in three of them, were bilious vomiting, abdominal distention and constipation, alternating with diarrhea and malnutrition. The fourth patient, different from the others in the age of onset and evolution, only had severe constipation and abdominal bloating. The diagnostic was made by full thickness biopsies during laparotomy, getting specimens by mapping, at different heights of intestine and stomach. Samples were studied by optic and electronic microscopy and visceral myopathies were found. None of them had urinary disorders. Medical treatment consisted of total parental nutrition and/or enteral nutrition. Cisapride was not effective in the two patients who received it.
Insights
Chronic idiopathic intestinal pseudo-obstruction (CIIP) involves ineffective intestinal propulsion causing obstruction symptoms without mechanical blockage. This study found visceral myopathies in four pediatric patients, highlighting diagnostic and treatment challenges.
Area of Science:
- Gastroenterology
- Pediatric Motility Disorders
- Histopathology
Background:
- Chronic intestinal pseudo-obstruction (CIPO) presents as bowel obstruction symptoms without mechanical blockage, stemming from ineffective intestinal propulsion.
- CIPO can be primary (chronic idiopathic intestinal pseudo-obstruction, CIIP) or secondary to other conditions.
- CIIP is characterized by a lack of identifiable underlying diseases causing the propulsive disorder.
Observation:
- This study details four female pediatric patients (4 months to 7 years) with CIIP.
- Three patients exhibited bilious vomiting, abdominal distention, constipation alternating with diarrhea, and malnutrition.
- The fourth patient presented with severe constipation and abdominal bloating, differing in onset and progression.
Findings:
- Diagnosis was confirmed via full-thickness intestinal and stomach biopsies analyzed by light and electron microscopy.
- Visceral myopathies were identified as the underlying pathology in all four patients.
- No urinary disorders were observed in these patients.
Implications:
- The findings underscore visceral myopathies as a cause of CIIP in pediatric cases.
- Effective management strategies remain challenging, with total parenteral/enteral nutrition being a primary approach.
- Cisapride showed limited efficacy in the treated patients, suggesting a need for alternative therapeutic options.