A collection of three primary symptoms indicate the presence of this birth defect. These are the deficiency in the muscles of the abdomen which create the wrinkled appearance of the belly skin, the dilated urinary tract, as well as the undescended testicles in the male infants. Once Prune Belly syndrome has been diagnosed, treatment is prescribed based on the severity of the defect.
Complications Associated with Prune Belly Syndrome
Prune Belly syndrome is a rare disorder occurring once in forty thousand live births. 95% of the cases affect male infants. Besides the three clinical symptoms mentioned above, a number of other complications are also probable in infants born with this birth defect.
- Malformation of ureters, which are the tubes that transport urine from the bladder to outside the body.
- Enlargement of the bladder resulting in pressure on other internal organs in the abdominal cavity. Also known as mega-bladder.
- Problems of the kidney like renal dysplasia (the improper development of the kidney in the womb), and hydronephrosis (the swelling of kidneys due to improper drainage of urine from kidneys to bladder).
- Malformation of the prostate gland or the testes is also a possibility. One or both of the testicles may not descend into the scrotum.
Treatment Options
Treatment of Prune Belly syndrome depends on the severity of the disorder, the age of the infant, the predicted course of the disease, the child’s tolerance level for medical procedures, and the opinion of the parents of the child.
Prenatal Ultrasound and Medical Pregnancy Termination: The number of live births with Prune Belly syndrome have reduced due to detection of the birth defect on prenatal ultrasounds. Subsequently, the parents often decide to abort the fetus rather than have the child born with the defect and undergo lifelong medical problems.
Abdominoplasty: Extensive surgery required to remodel the abdominal wall and the urinal tract. The muscle from the hip may be used to reinforce the stomach muscles.
Urethral Surgery: May be performed to deal with malformation of the urethra. A vesicostomy may also be performed to create an opening in the bladder via the abdomen.
Orchiopexy: This surgery is for the male infants whose testicles have not descended into the scrotum. It helps to treat the cryptorchidism.
Cyctoplasty: A mega bladder will need to be surgically treated for it to function normally. A bladder reconstructive surgery such as cytoplasty will be recommended.
Kidney Transplantation: The infants with Prune Belly syndrome are likely to develop kidney failure in 30% cases. A kidney transplantation surgery will be essential in these cases for the child to survive.
Outlook Post Treatment
Prune Belly Syndrome is a life threatening disorder. Most infants who are not diagnosed with the birth defect will die within a few weeks of their birth. The usual cause of death is a combination of problems affecting the kidneys and ending in renal failure.
If the birth defect is detected, there is a better chance of the newborn’s survival. The severity of the disorder will determine if the child will develop normally as he grows up. Also, medical intervention in form of surgeries may be required to ensure that developmental problems are kept at bay.
In a study conducted by Vahudin Zugor, Gunter E. Schott and Apostolos P. Labanaris, 16 patients suffering from Prune Belly syndrome were analyzed. The patients were studied at the Erlangen Paediatric Urology department where they came in for treatment through the years 1970 to 2006. The study’s findings covered the symptoms of the patients, the manner of diagnosis, the treatment received, and the outcome in the long term.
The patients were aged between 1 and 13 years. All the children were found to be suffering from congenital aplasia of the abdominal wall as well as different types of urogenital malformations. All patients received varying treatment over different time periods. Most of them underwent at least one form of surgery as part of the treatment.
Mean follow up time for the study was 17 years. Survival rate after 10 years was pegged at 93.7%. This study revealed that if appropriate treatment is provided in a timely manner, the child born with Prune Belly syndrome can have a somewhat normal development into adulthood.
Prognosis
The main aim of treatment for Prune Belly syndrome is to ensure that the triad of birth defects are corrected so that the patient has a good chance of survival. Most doctors will adopt a wait and watch attitude once diagnosis is complete so that the following check ups allow them to accurately judge the severity of the disease and predict its possible course.
Reconstructive surgeries and kidney transplantation is usually required. Post the surgeries, the birth defects are rectified and the patient has a pretty good chance of returning to good health. The prognosis is good for those infants who received timely treatment.
References
- https://rarediseases.info.nih.gov/diseases/7479/prune-belly-syndrome
- https://rarediseases.org/rare-diseases/prune-belly-syndrome/
- https://medlineplus.gov/ency/article/001269.htm
- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC 3395978/
Further Reading
- All Prune Belly Syndrome Content
- Diagnosis and Treatment of Prune Belly Syndrome
- Prune Belly Syndrome
- Symptoms and Causes of Prune Belly Syndrome
Last Updated: Feb 27, 2019
Written by
Cashmere Lashkari
Cashmere graduated from Nowrosjee Wadia College, Pune with distinction in English Honours with Psychology. She went on to gain two post graduations in Public Relations and Human Resource Training and Development. She has worked as a content writer for nearly two decades. Occasionally she conducts workshops for students and adults on persona enhancement, stress management, and law of attraction.
Source: Read Full Article