Berdon Syndrome

Definition

Definition of Berdon Syndrome
Berdon syndrome (medical condition) is usually lethal gastrointestinal disorder involving dysfunction of the bowel and bladder due to abnormalities of the smooth muscle. It is also listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Berdon syndrome, or a subtype of Berdon syndrome, affects less than 200,000 people in the US population.

Symptoms

Symptoms/Causes of Berdon Syndrome
Some of the symptoms of the disease are mentioned below:
Constipation
Dilated Bladder
Dilated Small Bowel
Reduced Intestinal Peristaltic Action
Absent Intestinal Peristaltic Action

Diagnosis

Diagnosis/Prognosis of Berdon Syndrome
MMIH is difficult to diagnose before the baby is born. Abnormal internal organs may be seen on fetal ultrasound, but it may not be clear that the child has MMIH, unless the child had a sibling with the disorder. Once the baby is born, physical examination and ultrasound can determine if MMIH exists.

Treatment

Treatment of Berdon Syndrome
However, there are no cure for MMIH, and cause death within a year from infection to the infants. Infants can be fed intravenously with special mixtures, but this type of therapy often causes liver failure. In the last decade physicians have attempted to replace all of the defective internal organs in infants with MMIH by transplantation.