Prune Belly Babies

When I worked in the Emergency Department I used to encounter a lot of new syndromes, and diseases that would leave me wondering what was going on with those patients. More often than not, I would go home after my shift, and research the patient. One of the patients that I remember doing the most research for was this baby with Prune Belly Syndrome (PBS).

Prune-Belly Syndrome is a rare congenital anomaly with an incidence of 1 per 30,000–50, 000 live births and it is characterized by poor development of the abdominal muscles, causing the skin of the belly area to wrinkle like a prune, undescended testicles, and urinary tract problems (Papantoniou 2007).

Diagnosis of PBS is usually confirmed with an ultrasound which demonstrates an abdominal cavity filled with fluid. While in the womb, the developing baby's abdomen swells with fluid. 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.

Many women are advised to terminate their pregnancy if the fetus has other congenital defects. PBS is a syndrome that is more often than not accompanied by pulmonary hypoplasia, heart defects, and muscle/skeletal problems leading to a short life span.

However, many treatments are available such as surgery to help strengthen the abdominal muscles, antibiotics to treat the various respiratory and urinary tract infections. Most recently in Chicago there was a five year old patient who received a surgery to improve liquid containment of the bladder reducing urinary tract infections. The appendix was detached  from the cecum and joined to one end of the bladder, bringing the other end through an opening, or stoma, in the abdominal wall. This conduit is supported by the bladder muscle so when the bladder is full, there is no leakage.

Finally, PBS has no known cause, and further research is needed to expand the knowledge on this syndrome to expand the life spans and improve the lives of the children affected by it.  

Prenatal diagnosis of prune-belly syndrome at 13 weeks of gestation: case report and review of literature. Papantoniou, N., et al. Journal Of Maternal-Fetal & Neonatal Medicine. 2010 23: 1263-1267.

 Information about the surgery can be found at: http://www.uchicagokidshospital.org/specialties/urology/stories/graham.html