Neonatal-onset chronic intestinal pseudo-obstruction syndrome
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Y C Huang1, H C Lee, F Y Huang
1Department of Pediatrics, Chang Gung Children's Hospital, Taoyuan, Taiwan, R.O.C.
Insights
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.
Abstract:
Between January 1985 and January 1990, six cases of neonatal-onset chronic intestinal pseudo-obstruction syndrome (CIPS) were identified. Failure to gain weight in six cases, abdominal distention in five, and vomiting in five were the most common presenting symptoms. The contrast studies of the gastrointestinal tract demonstrated delayed transit time in 6/6, jejunal or ileal dilatation in 1/6, megaduodenum in 1/6, dilatation of the colon with barium retention in 4/6, and microcolon in 1/6. Urinary tract involvement was noted in three patients. Laparotomy, performed in three patients, revealed no mechanical obstruction. Except for hypoganglionosis in Patient 4, no recognizable neuropathy or myopathy was noted histopathologically. Four patients expired within 2 months after discharge. We conclude that CIPS with neonatal onset should be suspected when infants have urinary retention and abdominal distention or constipation beginning at birth or soon after. The prognosis of CIPS presenting in the newborn period appears worse than that presenting in childhood or adulthood.