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Prune Belly syndrome

Also known as: Eagle-Barrett syndrome

Background

Prune belly syndrome is an extremely rare condition which only affects boys. It is characterised by three distinctive features:

  • poor development of the abdominal muscles
  • cryptorchidism (undescended testicles in males)
  • urinary tract problems.

The cause of prune belly syndrome is not known.

Credits

Medical text written December 2011 by Imran Mushtaq, Consultant Paediatric Urologist, Great Ormond Street Hospital, London, UK.

What are the symptoms?

Prune belly syndrome is a serious and in some cases can be a life-threatening problem. As the name implies, prune belly syndrome is characterised by an abdomen with a wrinkly or 'prune-like' appearance. This is because, while in the womb, the developing baby's tummy swells with fluid. The 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.

The range of urinary tract anomalies varies widely in prune belly syndrome from an inability to completely empty the bladder to a more serious enlargement of the kidneys, ureter and bladder. A child may experience frequent urinary tract infections.

In most affected males, the testes are small and reside in the abdomen (tummy). Sperm are also thought to be absent or do not form properly. Rarely, neoplasias (malignant changes) have been reported in the testes, meaning that there is the potential for them to become cancerous.

Complications associated with prune belly syndrome may include pulmonary hypoplasia (underdevelopment of the lungs), heart anomalies, gastrointestinal abnormalities and musculoskeletal (bone/muscle) abnormalities.

How is it diagnosed?

The condition can be diagnosed prenatally. Ultrasound scans and X-rays may identify the type of urinary tract abnormalities present after birth.

How is it treated?

Early surgery may be required to fix the weak abdominal muscles, the urinary tract problems, and to bring down the undescended testicles.

The baby may be given antibiotics to treat or help prevent urinary tract infections.

Inheritance patterns and prenatal diagnosis

Inheritance patterns
The exact cause of prune belly syndrome remains unknown.

Prenatal diagnosis
Prune belly syndrome may be diagnosed by ultrasound examination from abnormal development of the bladder and urinary tract. Mothers carrying an unborn baby with prune belly syndrome may develop varying degrees of oligohydramnios (insufficient amniotic fluid) increasing the likelihood of lung problems after birth.

Is there support?

There is no support group for prune belly syndrome in the UK. Families can use Contact a Family's freephone helpline for advice, information and, where possible, links to other families.

Contact a Family's web-based linking service MakingContact.org can be accessed at community.cafamily.org.uk