What is Megacystis Microcolon intestinal Hypoperistalsis syndrome?
Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a severe disorder affecting the muscles that line the bladder and intestines . It is characterized by impairment of the muscle contractions that move food through the digestive tract (peristalsis) and empty the bladder.
Is megacystis fatal?
Berdon syndrome, also called Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIH syndrome), is an autosomal recessive fatal genetic disorder affecting newborns.
What causes Hypoperistalsis?
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital condition characterized by abdominal distension caused by a largely dilated non-obstructed urinary bladder (megacystis); very small colon (microcolon); and decreased or absent intestinal movements (intestinal peristalsis).
What does Hypoperistalsis mean?
(hī’pō-per’i-stal’sis), Reduced or inadequate peristalsis.
What causes megacystis?
Megacystis is most often caused by obstructive conditions that block the urethra, such as posterior urethral valves, or malformation to the urethra such as stenosis (narrowing or stiffening) or atresia (failure to develop); or persistent cloaca (a complex anatomic developmental malformation).
What is megacystis?
From Wikipedia, the free encyclopedia. Megacystis (fetal) Specialty. Urology. Fetal megacystis is a rare disease that is identified by an abnormally large or distended bladder.
Can megacystis go away?
In pregnancies with megacystis of 7–15 mm the parents can be reassured that once the fetal karyotype is found to be normal in about 90% of cases the megacystis will resolve without any obvious adverse effects on renal development and function.
Is megacystis curable?
Obstructive megacystis without other complications is treatable. Previous studies have already described the treatment methods, such as vesicoamniotic shunting,[6,7] valve resection, and urinary stent.
How is megacystis diagnosed?
In a first trimester scan (11-14 weeks), megacystis is diagnosed if the longitudinal bladder diameter is more than 7 mm. During fetal life, the fetal kidneys produce urine, which is passed to the bladder through tubes called ureters.
Can megacystis resolve itself?
In the chromosomally normal group there is spontaneous resolution of the megacystis without any obvious adverse consequence on the development of the urinary system in about 90% of cases.
What does megacystis mean?
What causes LUTO?
The two most common causes of LUTO include posterior urethral valves (PUVs), and urethral atresia [5]. In general, obstruction at the bladder outlet in males is caused by PUVs, whereas in females it is secondary to urethral atresia [4].
What are the symptoms of Megacystis microcolon syndrome?
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital condition characterized by abdominal distension caused by a largely dilated non-obstructed urinary bladder (megacystis); very small colon (microcolon); and decreased or absent intestinal movements (intestinal peristalsis).
How old is the oldest person with megacystis microcolon?
What is the age of the oldest reported person with megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS)? In a review of the literature published in 2011 including 227 cases of megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), the oldest survivors were a 19 year old female and 24 year old male. [3]
Are there any survivors of Megacystis microcolon hypoperistalsis?
The majority of survivors are either maintained by TPN or have undergone multiorgan transplantations. The survival in MMIHS in recent years has improved. The majority of survivors are either maintained by TPN or have undergone multiorgan transplantations.
What causes an enlarged bladder in Megacystis microcolon?
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is primarily characterized by abdominal distention caused by an enlarged bladder (megacystis) and intestinal pseudo-obstruction .The enlarged bladder may be detected prenatally. [2]