Eagle-Barrett Syndrome

Jagdish Kathwate
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Eagle-barrett Syndrome - Patient Education
What is Eagle-Barrett syndrome?
It is a group of birth defects that involve three main problems:
• Poor development of the abdominal muscles, causing the skin of the belly area to wrinkle like a prune
• Undescended testicles (cryptorchidism)
• Urinary tract problems
Also known as prune belly syndrome.
Causes
The causes of prune belly syndrome are unknown. The condition affects mostly boys.While in the womb, the developing baby's abdomen swells with fluid, often from a urinary tract abnormality. That fluid disappears after birth, leading to a wrinkled abdomen that looks like a prune. The appearance is more noticeable due to the lack of abdominal muscles.
Symptoms
Weak abdominal muscles can cause:Constipation Delay in sitting and walkingDifficulties coughing Urinary tract problems can cause difficulty urinating.
Exams and Tests
A woman who is pregnant with a baby who has prune belly syndrome may not have enough amniotic fluid (oligohydramnios). This can cause the infant to have lung problems due to the compression of the baby.
An ultrasound done during pregnancy may show that the baby has a swollen bladder or enlarged kidney.In some cases, a pregnancy ultrasound may also help determine if the baby has:
• Heart problems
• Bone/muscle (musculoskeletal) abnormalities
• Stomach and intestinal problems
• Underdeveloped lungs

Which test my doctor will do?
The following tests may be performed on the baby after birth to diagnose the condition:
• Blood tests
• Intravenous pyelogram (IVP)
• Ultrasound
• Voiding cystourethrogram (VCUG)
• X-ray
Treatment
Early surgery is recommended to fix weak abdominal muscles, urinary tract problems, and undescended testicles.The baby may be given antibiotics to treat or help prevent urinary tract infections.

What is prognosis for it?
Prune belly syndrome is a serious and often life-threatening problem.
Many infants with prune belly syndrome are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of birth problems.


Eagle-Barrett Syndrome Eagle-Barrett Syndrome 8/1/2015
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