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Megacystis microcolon intestinal hypoperistalsis syndrome
P S Makhija1, K F Magdalene, M K Babu
1Department of Pathology, St. John's Medical College and Hospital, Bangalore, Karnataka.
Indian Journal of Pediatrics
|May 8, 2000
Summary
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare neonatal intestinal obstruction. Autopsy revealed characteristic MMIHS findings plus unusual cervical nerve tissue, suggesting a broader neurological basis.
Area of Science:
- Neonatal Medicine
- Gastroenterology
- Neuropathology
Background:
- Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare, often fatal, congenital disorder.
- It primarily affects female neonates, presenting with intestinal obstruction, abdominal distension, and poor feeding.
Observation:
- A case report of a female neonate presenting with MMIHS symptoms including abdominal distension, bilious vomiting, and decreased urine output.
- Diagnostic imaging confirmed a microcolon, a key feature of MMIHS.
Findings:
- Autopsy revealed a shortened bowel, microcolon with abundant myenteric ganglion cells, and an enlarged urinary bladder, consistent with MMIHS.
- Notably, enlarged nerve bundles with ganglion cells were found on the cervical wall, an atypical finding.
Implications:
- This case highlights the neuropathological spectrum of MMIHS.
- The cervical nerve findings may suggest a more widespread developmental neuropathy than previously recognized in MMIHS.