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[Megabladder-microcolon-intestinal hypoperistalsis syndrome]
S Rite Gracia1, B Fernández Alvarez de Sotomayor, V Rebage Moisés
1Unidad de Neonatología. Servicio de Pediatría. Hospital Infantil Universitario Miguel Servet. Zaragoza.
Anales Espanoles De Pediatria
|November 21, 2000
Summary
Megacystis-microcolon-intestinal hypoperistalsis syndrome is a rare congenital disorder causing bladder distention and poor gut motility. Early diagnosis via imaging is crucial for suspected cases of intestinal obstruction and urine retention.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Urology
Background:
- Megacystis-microcolon-intestinal hypoperistalsis (MMIH) syndrome is a rare congenital disorder.
- It is characterized by urinary bladder distention and hypoperistalsis of the entire gastrointestinal tract.
Observation:
- Presents a case with typical clinical and radiological findings of MMIH syndrome.
- Diagnosis should be suspected in patients with intestinal obstruction and urine retention.
- Imaging techniques like abdominal plain films and urinary ultrasonography are key for confirmation.
Findings:
- Histological findings include a thinned intestinal muscle layer and connective tissue proliferation.
- These histological findings are generally non-specific for MMIH syndrome.
Implications:
- The prognosis for MMIH syndrome is typically very poor.
- Early recognition and diagnosis are critical for patient management.
- Further research may improve outcomes for this rare condition.