Berdon syndrome is a rare disease that mainly affects girls and causes problems in the intestines, bladder and stomach. Generally, people with this disease do not pee or poop and need to be fed by a catheter.
This syndrome can be caused by genetic or hormonal problems, the symptoms arise soon after birth, which can be changes in the form and function of the bladder, which is usually very large, decrease or absence of bowel movements, which leads to the arrest of belly, in addition to decreasing the size of the large intestine and swelling of the small intestine.
Berdon syndrome has no cure, but there are some surgical procedures that aim to unclog the stomach and intestines, which can improve the symptoms of the disease. In addition, an alternative to increase the expectation and quality of life of the person with this syndrome is the multivisceral transplantation, that is, the transplantation of the entire gastrointestinal system.
Main symptoms
The main symptoms of Berdon's Syndrome are:
- Constipation;
- Urinary retention;
- Dilated bladder;
- Swelling of the belly;
- Muscles of the abdomen flaccid;
- Vomiting;
- Swollen kidney;
- Bowel obstruction.
The diagnosis of Berdon's Syndrome is made through the evaluation of the symptoms presented by the child after birth and by imaging tests, such as ultrasonography. The disease can also be identified during pregnancy by means of ultrasonography during routine exams. Usually this happens from the 20th week of gestation.
How is the treatment done?
Treatment of Berdon Syndrome is not able to promote the cure of the disease, but it helps to minimize the symptoms in patients and improve their quality of life.
Surgery in the stomach or bowel is recommended to unclog these organs and improve their functioning. Most patients need to be fed through a catheter due to the problem in the digestive system.
It is also common to be performed bladder surgery, creating a connection to the skin in the belly region, which allows the drainage of urine.
However, these procedures have little effect on the patient, often leading to death due to malnutrition, multiple organ failure and widespread infection in the body, sepsis. For this reason, multivisceral transplantation has become the best treatment option and consists of five surgeries at once: transplantation of the stomach, duodenum, intestine, pancreas and liver.
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