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Prune belly syndrome is a birth defect affecting the abdominal muscles, genital organs and urinary tract of the infant. The muscles of the abdomen are poorly developed and the skin covering them is wrinkled, giving an appearance similar to a prune, hence the name of the disorder. The testicles of male infants may not have descended into the scrotal sack and the infant may also have a urinary tract malformation which often results in urinary infections.
The disorder is also known as Abdominal Muscle Deficiency Syndrome, Congenital Absence of the Abdominal Muscles, Eagle-Barrett Syndrome, Obrinsky Syndrome, Triad Syndrome, Urethral Obstruction Malformation Sequence and Deficiency of Abdominal Musculature.
Prune belly syndrome disorder is considered a rare birth defect. It occurs once in forty thousand live birth cases. There is a much greater risk of the disorder affecting male infants. It has been stated by the University of Rochester Medical Center that 95 % cases of the Prune Belly Syndrome occur in males while only 5% female infants are affected. It is not contagious or infectious to the caregivers.
The actual cause of the disorder is still under investigation. During the studies conducted, it has been observed that siblings are often affected by the birth defect, which gives weight to the theory that there may be a genetic component at play. However, this is as yet unsubstantiated by research.
The birth defect has also been called the “Triad Syndrome” based on the three main physical symptoms that afflict the newborn infant. These three symptoms include:
1. The abdominal muscles of the infant are not developed properly. There may only be a partial set of muscles or the abdominal muscles may be missing completely. The severity of the birth defect varies per case.
2. The testicles may fail to descend into the scrotal sac. If both testicles have not descended into the scrotum, the condition is known as bilateral cryptorchidism. In some infants, only a single testicle may have descended into the scrotum.
3. The urinary tract malformation can include enlarged or blocked ureters. Ureters are the tubes that carry urine from the bladder to the urinary tract and finally out of the body. The bladder itself may be expanded abnormally. This can cause problems with the ureters as well as the kidneys. Kidney malfunction is a common feature.
A child suffering from prune belly syndrome will have a wrinkled abdomen due to the lack of proper development of muscles there. Instead of stretching over the muscles, the skin bunches up due to the lack of the abdominal muscles.
Additionally, affected children may be unable to empty their bladder due to the missing muscular action of the abdomen, and an obstruction of the ureters which can make it difficult for the child to urinate and has the potential to foster a urinary tract infection.
The infant may also experience a delay in the milestones of growth, such as sitting up unsupported and walking, due to the lack of strength in the abdominal muscles.
Children born with prune belly syndrome are also more likely to be affected by other birth defects. These could be associated with the skeletal system, intestines, or the heart. For example, they may suffer from clubfoot, spine malformations, patent ductus arteriosus, and gastrointestinal anomalies or cardiac defects.
The disorder predominantly strikes males, but female infants could also be born with defects of the external genitalia. Urethral atresia, uterine duplication, and anorectal anomalies are frequently seen in the female infant’s cases.
The severity of the defect will determine the treatment that the infant is prescribed. Antibiotics will be required to contain upper and lower urinary tract infections. Surgery for bladder obstructions such as a vesicostomy may be required. An orchiopexy which is a surgical process to bring the testes to the scrotum may be recommended for male infants. A combination of multiple surgical procedures may be conducted to give the infant a better chance at survival.
Infants who are born with this defect may die within a few months of birth if they do not receive timely treatment. Even with proper medical treatment, the disorder remains serious and life threatening.
Although there have been cases where newborns have been treated with surgery in a timely manner and survived with close to the expected normal growth and development, most infants born with this defect will continue to struggle with medical and developmental problems for all of their lives.