Clinical, laboratory and prognostic features of congenital large intestinal motor dysfunction (pseudo-Hirschsprung's disease).

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

One hundred and forty-eight cases of congenital large intestinal motor dysfunction (pseudo-Hirschsprung 's disease ) were reported by members of the Japanese Society of Pediatric Surgeons during the past 20 years . The disorder was defined as a congenital , non-mechanical obstruction of the intestine with the presence of intramural ganglia in the terminal rectum . Intramural ganglia were abnormal in 77 cases , normal in 42 , and could not be determined in 29 . Of those with abnormal intramural ganglia , 54 had immature ganglia or hypoganglionosis (oligoganglionosis ), 15 had neuronal intestinal dysplasia , and eight had a segmental anomaly . Of those with a normal myenteric plexus , 22 had chronic and twelve had suspected idiopathic intestinal pseudo-obstruction syndrome ; eight had megacystis-microcolon -intestinal hypoperistalsis syndrome . While cases with both hypoganglionosis and normal intramural ganglia had normal acetylcholine esterase activity , a significantly greater number of patients with hypoganglionosis lacked normal rectoanal reflexes . Patients with hypoganglionosis , chronic idiopathic intestinal pseudo-obstruction syndrome , and megalocystis-microcolon -intestinal hypoperistalsis syndrome had poor prognoses with an overall mortality of 36 .9 %. These findings indicate that congenital large intestinal motor dysfunction remains a serious disease of childhood .

Diseases
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Diseases presenting "chronic idiopathic intestinal" symptom

megacystis-microcolon-intestinal hypoperistalsis syndrome

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