Megacystis microcolon intestinal hypoperistalsis syndrome.
نویسندگان
چکیده
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare and the most severe form of functional intestinal obstruction in the newborn. The major features of this congenital and usually lethal anomaly are abdominal distension, bile-stained vomiting, and absent or decreased bowel peristalsis. Abdominal distension is a consequence of the distended, unobstructed urinary bladder with or without upper urinary tract dilation. Most patients with MMIHS are not able to void spontaneously. This article reviews the pathogenesis of MMIHS as well as the clinical, radiological, surgical and histological findings in all reported cases of this syndrome.
منابع مشابه
Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome Associated With Prune Belly Syndrome: A Case Report
Megacystis Microcolon Intestinal Hypoperistalsis Syndrome is a quite rare congenital anomaly that presents with a functional obstruction of the gastrointestinal tract which is usually fatal. It is three to four times more prevalent in females. We present a case of a rare association of a male neonate with Megacystis Microcolon Intestinal Hypoperistalsis Syndrome who in addition had the classica...
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Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is now a well established entity [1,2]. Also known as Berdon syndrome [1], it is characterized by massive abdominal distension caused by a largely dilated non obstructed urinary bladder, microcolon and decreased or absent intestinal peristalsis. Isolated cases of congenital megacystis [3,4] and microcolon without megacystis [5] h...
متن کاملIntrauterine death in megacystis-microcolon-intestinal hypoperistalsis syndrome.
Megacystis-microcolon-intestinal hypoperistalsis syndrome is an uncommon condition, possibly inherited as an autosomal recessive trait. This report describes an affected sib pair with intrauterine death of one of the sibs.
متن کاملAn unusual urinary tract presentation in a case of megacystis microcolon intestinal hypoperistalsis syndrome.
Megacystis microcolon intestinal hypoperistalsis syndrome is a rare congenital anomaly. Several pathogeneses have been described so far, but there is no single mechanism that can explain all the findings of the syndrome. Affected newborns usually present clinically in the first few days of life. The mainstay of diagnosis is a pre or a postnatal imaging evaluation using ultrasound or magnetic re...
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Background. A novel mutation in the ACTG2 gene is described in a pregnant patient followed up for chronic intestinal pseudoobstruction (CIPO) during pregnancy and her fetus with megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS). Case. 24-year-old gravida 1 para 1 with CIPO and persistent nausea and vomiting in pregnancy, admitted at 28 weeks of gestation. Ultrasound revealed a f...
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ورودعنوان ژورنال:
- Seminars in pediatric surgery
دوره 14 1 شماره
صفحات -
تاریخ انتشار 2005