Eagle-Barrett syndrome, more commonly known as Prune Belly Syndrome (PBS), is a congenital disorder that primarily affects male-bodied children. This condition is identified by a combination of three abnormalities appearing in combination. These abnormalities include urinary tract malformations, underdeveloped abdominal muscles, and undescended testicles. We will discuss the symptoms, causes, and diagnostic procedures for identifying PBS. We will also address the treatment options available at Advanced Pediatric of Rockland.
Understanding Prune Belly Syndrome
There is no definitive cause known for prune belly syndrome. What is known is that it is rare and complex to treat and is likely the result of multiple environmental and genetic factors. The most notable feature of PBS is underdeveloped abdominal muscles. The wrinkled appearance caused by this feature is where PBS draws its name, as the abdomen is often said to have a ‘prune-like’ appearance. Those with this condition frequently have undescended testicles (cryptorchidism) along with urinary tract malformations. Despite having these symptoms in common, each case of PBS is different, with these complications varying in severity. Urinary tract infections, gastrointestinal issues, fertility problems, growth and developmental delays, and kidney dysfunction are common symptoms of the syndrome.
Symptoms and Complications
Prune Belly Syndrome has been known to manifest in a variety of ways. As mentioned above, the severity of the symptoms can vary from patient to patient. The most prominent symptom is the aforementioned wrinkled abdomen with its prune-like appearance. The following additional symptoms are common sufferers of PBS:
- Poor bladder control
- Urinary incontinence
- Urinary retention
- Enlarged Kidneys
- Acid reflux
- Kidney failure
- Delayed growth and development
- Respiratory difficulties
- Fertility issues
These represent the most well-known symptoms of prune belly syndrome. However, the list is incomplete.
Diagnosis and Evaluation of Prune Belly Syndrome
Diagnosing this condition requires undergoing a comprehensive evaluation. A review of the patient’s medical history and a physical examination will occur as part of this process. In addition, imaging may be done to identify the extent of the syndrome’s impact more clearly. MRIs, X-Rays, and ultrasounds are commonly used during this diagnostic process. Genetic testing is often used to identify potential factors associated with PBS.
Treatment and Management of Prune Belly Syndrome
Managing this condition involves addressing and mitigating the complications associated with the condition. This process typically involves a multidisciplinary approach involving a range of specialists. Some of the specialists that may become involved with the treatment and management of PBS include:
In instances where the functioning of the kidney is significantly impacted, a kidney transplace may be necessary. Monitoring of the patient’s health, including urinary tract health, kidney function, gastrointestinal health, and growth and development, will occur throughout.
Get Support From Advanced Pediatrics of Rockland
Living with PBS can present a significant challenge to individuals with the condition and their families. Receiving the support of healthcare professionals like those at Advanced Pediatrics of Rockland can make all the difference along the way. Contact our office at (845) 364-9800, or see us at our Pomona, NY, location today!