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Journal of Pediatric Gastroenterology... Jul 1997
Comparative Study Review
Topics: Abnormalities, Multiple; Adult; Child; Colon; Female; Humans; Infant, Newborn; Intestinal Obstruction; Intestines; Male; Obstetric Labor Complications; Peristalsis; Pregnancy; Prenatal Diagnosis; Radiography; Syndrome; Urinary Bladder
PubMed: 9226521
DOI: 10.1097/00005176-199707000-00002 -
Seminars in Pediatric Surgery Feb 2005Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare and the most severe form of functional intestinal obstruction in the newborn. The major... (Review)
Review
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.
Topics: Colon; Dilatation, Pathologic; Fetal Diseases; Humans; Ileum; Infant, Newborn; Intestinal Obstruction; Muscle, Smooth; Peristalsis; Radiography; Ultrasonography, Prenatal; Urinary Bladder
PubMed: 15770589
DOI: 10.1053/j.sempedsurg.2004.10.026 -
Pediatric Radiology Apr 2013Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare and frequently lethal form of severe functional intestinal obstruction more commonly found in... (Review)
Review
Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare and frequently lethal form of severe functional intestinal obstruction more commonly found in girls. Imaging features characteristic of this disease include a large dilated bladder, microcolon and intestinal dysmotility. Additional imaging findings may include intestinal malrotation, hydronephrosis and vesicoureteral reflux. It is usually fatal in the first year of life. Because presenting clinical and imaging features can mimic other causes of neonatal bowel obstruction, we compiled examples of this disorder to help the pediatric radiologist recognize imaging findings associated with MMIHS and aid in the development of a long-term management plan and in counseling the family regarding implications of this disorder. We reviewed recent and historical literature relevant to MMIHS and present the imaging and clinical features of four patients with MMIHS treated at our institution as examples of this uncommon disorder.
Topics: Abnormalities, Multiple; Colon; Female; Humans; Image Enhancement; Infant; Infant, Newborn; Intestinal Pseudo-Obstruction; Male; Tomography, X-Ray Computed; Ultrasonography; Urinary Bladder
PubMed: 22926452
DOI: 10.1007/s00247-012-2479-y -
Radiology Case Reports 2007We report a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) in a newborn female infant who presented with an abdominal mass, absent bowel...
We report a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) in a newborn female infant who presented with an abdominal mass, absent bowel sounds, and feeding intolerance with bilious emesis. MMIHS is a rare congenital bowel and bladder defect requiring surgery and chronic total parenteral nutrition in an attempt to sustain life. With few exceptions, it is predominately fatal within the first six months of life. We describe the relevant clinical and radiologic findings with ultrasound correlation of this case followed by a brief review of literature included in the discussion.
PubMed: 27303483
DOI: 10.2484/rcr.2007.v2i4.26 -
Indian Journal of Pediatrics 1999Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare cause of intestinal obstruction mainly affecting female neonates. We present a case of a... (Review)
Review
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare cause of intestinal obstruction mainly affecting female neonates. We present a case of a newborn female infant with a history of abdominal distension, bilious vomiting and decreased urine output. Barium enema showed a microcolon. Patient died soon after admission and the autopsy revealed a shortened bowel, a microcolon with abundant ganglion cells in the myenteric plexus, and an enlarged urinary bladder. An interesting finding in this case was the presence of enlarged nerve bundles containing several large ganglion cells on the lateral wall of the cervix. The salient clinical and autopsy findings in this case are presented.
Topics: Abnormalities, Multiple; Colon; Female; Humans; Ileum; Infant, Newborn; Intestinal Obstruction; Peristalsis; Syndrome
PubMed: 10798164
DOI: 10.1007/BF02723874 -
Pediatric Surgery International Oct 2011Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare and the most severe form of functional intestinal obstruction in the newborn. This congenital... (Review)
Review
PURPOSE
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare and the most severe form of functional intestinal obstruction in the newborn. This congenital condition is associated with non-obstructed urinary bladder, microcolon and decreased or absent intestinal peristalsis. This study was designed to determine the incidence and outcome of MMIHS.
METHODS
A systematic review of the literature (1976-2011) was performed for key words "megacystis microcolon intestinal hypoperistalsis". Resulting publications were reviewed for epidemiology and outcome. Reference lists were screened for additional cases.
RESULTS
A total number of 227 MMIHS cases were reported from 1976 to 2011. A clear preponderance for female infants was found (female 70.6 vs. male 29.4%). One or more surgical interventions were reported in 115 patients (including gastrostomy, ileostomy, jejunostomy, segmental resections of small bowel, adhesiolysis and internal sphincter myectomy). For decompression of the megacystis, vesicostomy was performed in 41 patients. Outcome was reported in a total of 218 patients. Survival rate was 19.7% (survivors: n = 43, non-survivors: n = 175), the oldest survivor being 24 years old. The vast majority of the surviving patients had to be maintained by total or partial parenteral nutrition (TPN). Main causes of death were sepsis, malnutrition and multiple organ failure. Twelve multivisceral transplantations have been reported to date in patients with MMIHS. The majority of the survivors are reported to be free of parenteral nutrition and show improving gastric emptying. However, intermittent catheterisation remains obligatory due to persistent bladder dysfunction.
CONCLUSIONS
The survival in MMIHS in recent years has improved. The majority of survivors are either maintained by TPN or have undergone multiorgan transplantations.
Topics: Abnormalities, Multiple; Cause of Death; Colon; Decompression, Surgical; Enterostomy; Female; Humans; Infant, Newborn; Intestinal Pseudo-Obstruction; Male; Parenteral Nutrition; Sex Distribution; Survival Rate; Treatment Outcome; Urinary Bladder; Viscera
PubMed: 21792650
DOI: 10.1007/s00383-011-2954-9 -
Ryoikibetsu Shokogun Shirizu 2001
Review
Topics: Colon; Female; Humans; Male; Peristalsis; Syndrome; Urinary Bladder
PubMed: 11528675
DOI: No ID Found -
American Journal of Physiology.... Jun 2021Visceral smooth muscle is a crucial component of the walls of hollow organs like the gut, bladder, and uterus. This specialized smooth muscle has unique properties that... (Review)
Review
Visceral smooth muscle is a crucial component of the walls of hollow organs like the gut, bladder, and uterus. This specialized smooth muscle has unique properties that distinguish it from other muscle types and facilitate robust dilation and contraction. Visceral myopathies are diseases where severe visceral smooth muscle dysfunction prevents efficient movement of air and nutrients through the bowel, impairs bladder emptying, and affects normal uterine contraction and relaxation, particularly during pregnancy. Disease severity exists along a spectrum. The most debilitating defects cause highly dysfunctional bowel, reduced intrauterine colon growth (microcolon), and bladder-emptying defects requiring catheterization, a condition called megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). People with MMIHS often die early in childhood. When the bowel is the main organ affected and microcolon is absent, the condition is known as myopathic chronic intestinal pseudo-obstruction (CIPO). Visceral myopathies like MMIHS and myopathic CIPO are most commonly caused by mutations in contractile apparatus cytoskeletal proteins. Here, we review visceral myopathy-causing mutations and normal functions of these disease-associated proteins. We propose molecular, cellular, and tissue-level models that may explain clinical and histopathological features of visceral myopathy and hope these observations prompt new mechanistic studies.
Topics: Cytoskeleton; Humans; Intestinal Pseudo-Obstruction; Muscle, Smooth; Mutation
PubMed: 33729000
DOI: 10.1152/ajpgi.00066.2021 -
Pediatric Nephrology (Berlin, Germany) Dec 1995
Topics: Abnormalities, Multiple; Adult; Colon; Female; Humans; Infant, Newborn; Intestinal Diseases; Peristalsis; Pregnancy; Syndrome; Ultrasonography, Prenatal; Urinary Bladder
PubMed: 8747128
DOI: 10.1007/BF00868746