Eagle-Barrett syndrome Definition Prune belly syndrome is a group ofВ birth abnormalities marked byВ three major findings: - Lack ofВ development of abdominal muscles, causing the skin of the abdomen to wrinkle like a prune
- Undescended testicle
- Urinary tract problems
Alternative Names Eagle-Barrett syndrome Causes The underlying causes of prune belly syndrome are unknown. Boys make up 96 - 97% of cases. Expectant mothers carrying affected infants may develop varying degrees of oligohydramnios (not enough amniotic fluid) that make the infant likely to have lung problems. (See also Potter syndrome.) The newborn infant has a wrinkled abdomen that looks like a prune. This is because the abdomen swelled with fluid in the womb, then lost that fluid after birth, leading to puckering of excess skin. The appearance is more noticeable because the lack of enough abdominal muscles. Many infants are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of birth problems. Symptoms - Wrinkled skin on abdomen
- Undescended testicle
- Club foot or abnormal limbs (rare)
Exams and Tests - Oligohydramnios (decreased or absent amniotic fluid)
- EnlargedВ renal collecting structures
- EnlargedВ bladder
Other findings may include: - Underdeveloped lungs
- HeartВ problems
- Stomach and intestinalВ problems
- Musculoskeletal abnormalities
The following tests may be used to diagnose the condition: - Ultrasound
- X-ray
- Intravenous pyelogram (IVP)
- Blood tests
Treatment Outlook (Prognosis) Prune belly syndrome is a serious and often life-threatening problem. Many newborns survive with varying degree ofВ recurring problems. Others are stillborn or die in the firstВ 2 years of life. Possible Complications Complications depend on the related problems. The most common are chronic renal failure When to Contact a Medical Professional Prune belly syndrome is usually diagnosed before birth or at the time of birth. If you have a child with diagnosed prune belly syndrome, call your health care provider at the first sign of a urinary tract infection or other urinary symptoms. If, before birth,В yourВ baby isВ seen to have a distended bladder or enlarged kidneys on prenatal ultrasound, seek the advice of a specialist in high-risk pregnancy or perinatology. Prevention There is no known guaranteed prevention. If a prenatal diagnosis of urinary tract obstruction is made, it may be possible to prevent it from progressing to prune belly syndrome with prenatal surgery. |