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Clinical Pediatrics
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*Intestinal Obstruction
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Neonatal-Onset Chronic Intestinal Pseudo-Obstruction Syndrome

Yhu-Chering Huang

Department of Pediatrics, Chang Gung Children's Hospital, Taoyuan

Hung-Chang Lee

Department of Pediatrics, Mackay Memorial Hospital

Fu-Yuan Huang

Department of Pediatrics, Mackay Memorial Hospital

Hsin-An Kao

Department of Pediatrics, Mackay Memorial Hospital

Ming-Lung Yeh

Department of Pediatric Surgery, Mackay Memorial Hospital

Pei-Yeh Chang

Department of Pediatric Surgery, Mackay Memorial Hospital

Jiin-Cherng Sheu

Department of Pediatric Surgery, Mackay Memorial Hospital

Sing-Lin Shih

Department of Radiology, Mackay Memorial Hospital

Be-Fung Chen

Department of Pathology, Mackay Memorial Hospital, Taipei, Taiwan, R.O.C.

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.

Clinical Pediatrics, Vol. 34, No. 5, 241-247 (1995)
DOI: 10.1177/000992289503400503
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