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Neonatal-onset chronic intestinal pseudo-obstruction syndrome
Y C Huang1, H C Lee, F Y Huang
1Department of Pediatrics, Chang Gung Children's Hospital, Taoyuan, Taiwan, R.O.C.
Clinical Pediatrics
|May 1, 1995
Summary
Neonatal chronic intestinal pseudo-obstruction syndrome (CIPS) presents with severe gastrointestinal issues and poor weight gain. This condition in newborns has a poor prognosis, often leading to early mortality.
Area of Science:
- Pediatric Gastroenterology
- Neonatal Medicine
- Gastrointestinal Motility Disorders
Background:
- Chronic intestinal pseudo-obstruction syndrome (CIPS) is a rare, severe gastrointestinal motility disorder.
- Neonatal onset of CIPS presents unique diagnostic and management challenges.
Purpose of the Study:
- To describe the clinical presentation, diagnostic findings, and outcomes of infants with neonatal-onset CIPS.
- To highlight the importance of early recognition and the poor prognosis associated with this condition in newborns.
Main Methods:
- Retrospective review of six cases of neonatal-onset CIPS identified between 1985 and 1990.
- Analysis of clinical symptoms, gastrointestinal contrast studies, laparotomy findings, and histopathological results.
- Evaluation of patient outcomes, including mortality.
Main Results:
- Common symptoms included failure to gain weight, abdominal distention, and vomiting.
- Gastrointestinal contrast studies revealed delayed transit time in all patients and colonic dilatation in four.
- Three patients underwent laparotomy, revealing no mechanical obstruction; one had hypoganglionosis. Four patients expired shortly after discharge.
Conclusions:
- Neonatal CIPS should be suspected in infants with urinary retention, abdominal distention, or constipation from birth.
- The prognosis for CIPS presenting in the newborn period is significantly worse than in older children or adults.