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 tube.
This syndrome can be caused by genetic or hormonal problems and the symptoms appear soon after birth, which can be changes in the shape and function of the bladder, which is usually very large, decreased or absent 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 unblock the stomach and intestines, which can improve the symptoms of the disease. In addition, an alternative to increase the life expectancy and quality of the person with this syndrome is multivisceral transplantation, that is, the transplantation of the entire gastrointestinal system.
Main symptoms
The symptoms of Berdon syndrome appear shortly after birth, the main ones being:
- Constipation; Urinary retention; Dilated bladder; Belly swelling; Flabby abdomen muscles; Vomiting; Swollen kidney; Bowel obstruction.
The diagnosis of Berdon Syndrome is made by assessing the symptoms presented by the child after birth and by imaging tests, such as ultrasound. The disease can also be identified during pregnancy by performing a morphological ultrasound after the 20th week of pregnancy. Understand what the morphological ultrasound is for.
How the treatment is done
The treatment of Berdon Syndrome is not able to promote the cure of the disease, but it helps to minimize symptoms in patients and improve their quality of life.
Surgery on the stomach or intestine is recommended to unblock these organs and improve their functioning. Most patients need to be fed through a tube due to the problem in the digestive system. See how tube feeding is done.
It is also common to have surgery on the bladder, creating a connection to the skin in the belly area, which allows urine to drain.
However, these procedures have little effect on the patient, often leading to death from malnutrition, multiple organ failure and generalized infection in the body, sepsis. For this reason, multivisceral transplantation has become the best treatment option and consists of performing five surgeries at once: transplantation of stomach, duodenum, intestine, pancreas and liver.
