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...

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...

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.

We report two female sibs with the megacystis-microcolon-intestinal hypoperistalsis syndrome. The parents are first cousins. These cases, together with three other published reports of affected sibs, confirm the autosomal recessive inheritance of the syndrome.

Multiple cardiac rhabdomyomata were discovered on necropsy tissue review of a previously well child with megacystis-microcolon-intestinal hypoperistalsis syndrome, who died unexpectedly at home at 40 months of age. Multiple cardiac rhabdomyomata occur rarely and have not previously been reported with this syndrome. They are most frequently associated with tuberous sclerosis. The finding of mult...

megacystis microcolon intestinal hypoperistalsis syndrome (mmihs) is a rare and the most severe form of functional intestinal obstruction in the newborn. the characteristic features of this congenital and fatal disease are abdominal distension, absent or decreased bowel peristalsis. abdominal distension is a consequence of the distended, unobstructed urinary bladder with or without hydronephros...

Normal Anatomy of the Urinary Prune-Belly Syndrome/ 289 Tract/ 255 Megacystis-Microcolon-intestinal Bilateral Renal Agenesis/ 259 Hypoperistalsis Syndrome/ 291 Infantile Polycystic Kidney Congenital Mesoblastic Disease/ 266 Nephroma/ 292 Adult Polycystic Kidney Disease/ 268 Wilms’ Tumor/ 293 Multicystic Kidney Disease/ 270 Normal Anatomy of the Adrenal Ureteropelvic junction Glands/ 295 Obstruc...

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...