The answer to yesterday’s mystery is … Prune Belly Syndrome (AKA Eagle-Barrett Syndrome)! Awesome job Ashley, F, Shorty, Rich S, Charlton Barrett, ColorMaven, Jami and Dennis at lunch, Angelita, Ken, Puffy MD, JP, MBAMom, Riley, Elizabeth, Michelle RN, Mike, and Max.
Prune Belly Syndrome is a congenital disorder characterized by absence of the abdominal muscles. Without muscle to support the abdominal wall, it caves in and gives it the wrinkly appearance of a prune. This disorder is seen almost exclusively in males, and it is due to a defect in development of the cell layer called the mesoderm. In addition to malformed abdominal muscles, PBS causes undescended testicles and abnormalities of the urinary tract. The photos from yesterday’s case show that our patient had the classic prune belly, plus a markedly distended bladder and ureters seen on ultrasound.
The lack of musculature often causes the prune belly to become a pot belly later in life, as the abdominal organs protrude. Sadly, half of children with this disease will need dialysis due to kidney failure before adulthood. Some patients require surgical correction of the urinary tract and kidney transplant. The abdominal wall can be reconstructed, in some cases by grafting muscle from the thigh into the abdomen. This is a disease with a poor prognosis, but early diagnosis (such as in utero via prenatal ultrasounds) can help by allowing early intervention.
Got a question about Prune Belly Syndrome? Let me know!