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Prune belly syndrome
Eagle-Barrett syndrome; Triad syndrome; Urethral obstruction malformation sequence
Prune belly syndrome is a group of birth defects that involves these 3 main problems:
- Poor development of the abdominal muscles, causing the skin of the belly area to wrinkle like a prune
-
Undescended testicles
Undescended testicles
Undescended testicle occurs when one or both testicles fail to move into the scrotum before birth.
Read Article Now Book Mark Article - Urinary tract problems
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, the cause is a problem in the urinary tract. The fluid disappears after birth, leading to a wrinkled abdomen that looks like a prune. This appearance is more noticeable due to the lack of abdominal muscles.
Symptoms
Weak abdominal muscles can cause:
- "Little Buddha" appearance
-
Constipation
Constipation
Constipation in infants and children occurs when they have hard stools or have problems passing stools. A child may have pain while passing stools o...
Read Article Now Book Mark Article - Delay in sitting and walking
- Difficulties 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 (the fluid that surrounds the fetus). This can cause the infant to have lung problems from being compressed in the womb.
An ultrasound done during pregnancy may show that the baby has a swollen bladder or enlarged kidney.
Ultrasound done during pregnancy
A pregnancy ultrasound is an imaging test that uses sound waves to create a picture of how a baby is developing in the womb. It is also used to chec...
In some cases, a pregnancy ultrasound may also help determine if the baby has:
- Heart problems
- Abnormal bones or muscles
- Stomach and intestinal problems
- Underdeveloped lungs
The following tests may be performed on the baby after birth to diagnose the condition:
- Blood tests
-
Intravenous pyelogram
(IVP)
Intravenous pyelogram
An intravenous pyelogram (IVP) is a special x-ray exam of the kidneys, bladder, and ureters (the tubes that carry urine from the kidneys to the bladd...
Read Article Now Book Mark Article - Ultrasound
-
Voiding cystourethrogram
(VCUG)
Voiding cystourethrogram
A voiding cystourethrogram is an x-ray study of the bladder and urethra. It is done while the bladder is emptying.
Read Article Now Book Mark Article - 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.
Outlook (Prognosis)
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. The cause of death is from severe lung or kidney problems, or from a combination of birth problems.
Some newborns survive and can develop normally. Others continue to have many medical and developmental problems.
Possible Complications
Complications depend on the related problems. The most common are:
- Constipation
-
Bone deformities (
clubfoot
, dislocated hip, missing limb, finger, or toe,
funnel chest
)
Clubfoot
Clubfoot is when the foot turns inward and downward. It is a congenital condition, which means it is present at birth.
Read Article Now Book Mark ArticleFunnel chest
Pectus excavatum is a medical term that describes an abnormal formation of the rib cage that gives the chest a caved-in or sunken appearance....
Read Article Now Book Mark Article - Disease of the urinary tract (may need dialysis and a kidney transplant)
Undescended testicles can lead to infertility or cancer.
When to Contact a Medical Professional
Prune belly syndrome is usually diagnosed before birth or when the baby is born.
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 a pregnancy ultrasound shows that your baby has a swollen bladder or enlarged kidneys, talk to a specialist in high-risk pregnancy or perinatology.
Prevention
There is no known way to prevent this condition. If the baby is diagnosed with a urinary tract obstruction before birth, in rare cases, surgery during the pregnancy may help prevent the problem from progressing to prune belly syndrome.
References
Caldamone AA, Woodard JR. Prune belly syndrome. In: Wein AJ, Kavoussi LR, Novick AC, Partin AW, Peters CA, eds. Campbell-Walsh Urology. 10th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 123.
Elder JS. Obstruction of the urinary tract. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds.
Nelson Textbook of Pediatrics.
20th ed. Philadelphia, PA: Elsevier; 2016:chap 540.
Review Date: 10/27/2015
Reviewed By: Chad Haldeman-Englert, MD, FACMG, Fullerton Genetics Center, Asheville, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
