Prune Belly Syndrome - Causes, Symptoms, and Management

What Is Prune Belly Syndrome?

An uncommon illness known as prune belly syndrome manifests at birth. Three abnormalities define prune belly syndrome. It is also known as Eagle-Barrett syndrome or triad syndrome:

1. The abdominal muscles of the infant are nonexistent or very weak due to a lack of abdominal muscular development. The skin on the baby's belly is now wrinkled like a prune as a result.

2. The testicles in the infant boy did not descend out of his body and into his scrotum (the delicate sack of skin under the penis).

3. The baby's urinary system, which includes the kidneys and bladder, has developmental issues such as a distorted, enlarged bladder. Almost all children with prune belly syndrome have major urinary tract issues. Children must be watched for the onset of the chronic renal disease since the bladder typically does not drain very adequately.

Other birth abnormalities could potentially be present in a child with prune belly syndrome. The most frequently impacted organs are the heart, lungs, intestines, and skeletal system. External genitalia anomalies can also occur in females.

What Causes Prune Belly Syndrome?

It is unclear what specifically causes prune belly syndrome. Numerous theories exist.

It might be brought on by a flaw in the bladder's embryonic development. Urine buildup can cause the kidney, ureters, and bladder to enlarge. The abdominal muscles waste away (atrophy) when the bladder gets bigger. A big bladder or the obliteration of the groin (inguinal) canals may be to blame for the retention of the testes in the abdominal space or cryptorchidism. The obstruction at the bladder outlet or the urethral obstruction may have been cleared up by the time the baby was born, making it impossible to spot any mechanical obstacles later on.

Other studies believe that the inadequate development of the abdominal muscles is a secondary cause of urinary problems. Due to incomplete bladder emptying, infections and urine retention may develop. Additional potential issues include indigestion and constipation. Since the abdominal muscles are crucial for breathing, their absence could account for chest deformities.

A third option is that the urine irregularities and the muscular deficit share an unidentified underlying cause. It is possible that the early breakdown of the abdominal muscles is caused by a neural system problem. Some children have shown a correlation with a congenital open spinal canal (spina bifida), and the existence of clubfeet is also frequently linked to prune belly syndrome.

Which Population Gets Affected by Prune Belly Syndrome?

A fairly uncommon condition known as prune belly syndrome manifests at delivery. Although few female cases have been documented in the medical literature, the condition primarily affects males. Only one out of every 30,000 to 40,000 kids born experiences it.

What Are the Signs and Symptoms Observed in Prune Belly Syndrome?

There are about fifty symptoms of prune belly syndrome. From newborn to baby, symptoms differ. Based on a physical exam and other tests, the healthcare professional will be able to make the diagnosis.

The most typical signs are as follows:

1. The abdominal wall has a wrinkled or prune-like appearance. The lack of or inadequate growth of the abdominal muscles is the causative factor.

2. Swollen or blocked ureters, enlarged kidneys, enlarged bladders, and vesicoureteral reflux (in this, there is a backflow of urine in the urinary tract) are all issues with the urinary system.

3. Large abdomen with intestinal loops visible through the thin abdominal wall skin.

4. Failure of the testicles to enter the scrotum or descend into the scrotum in males.

5. Digestive and intestinal issues.

6. Cardiac structural issues.

7. Anomalies of the musculoskeletal system, including clubfoot and scoliosis.

Other signs include:

1. Frequent urinary tract infections as a result of improper urine discharge.

2. Defect in the ventricle septum.

3. Gastrointestinal malrotation.

4. Post-ejaculatory pain is a typical complaint at a later age. It lasts for around two hours and is most likely a bladder spasm.

5. Congenital joint dislocations, such as hip dislocations, are musculoskeletal anomalies. The high prevalence of concomitant musculoskeletal problems makes a full orthopedic evaluation necessary for the diagnosis of prune belly syndrome.

6. Lung lobe involvement in pneumonia, atelectasis, and pulmonary hypoplasia.

How to Diagnose Prune Belly Syndrome?

The diagnosis is typically clear from birth, but locating and counting the anomalies takes time and attention. Imaging procedures such as an ultrasound, X-ray, and intravenous pyelogram will be necessary to fully comprehend the difficulties in order to identify the amount of the genitourinary tract's involvement. An IVP or intravenous pyelogram uses a dye to map the kidneys' and their ducts' level of involvement.

How to Manage Prune Belly Syndrome?

Depending on the severity of the symptoms, each kid will have a different course of treatment for prune belly syndrome. In order to give optimal care, a kid with prune belly syndrome who also has additional birth problems needs to be monitored by a number of specializations. For the preservation of kidney and bladder drainage and function, as well as the prevention of urethral infections, a close collaboration with a nephrologist is required.

Typical interventions comprise:

• Antibiotic Prophylaxis: To avoid a urinary tract infection, a low, once-daily dose of an antibiotic may be prescribed.

• For Enhancing Urinary Drainage:

  • Endoscopic Evaluation: While the child is sedated in the operating room, the urologist may need to look within the urethra. A cystoscope, a tiny instrument with a light and a camera lens at the end, will be inserted by the urologist. He will utilize this tool to make the appropriate urethral incisions that might be blocking the urethra.

  • Vesicostomy: A vesicostomy may be advised to assist in facilitating urine bladder emptying. The bladder is opened by a vesicostomy, allowing urine to drain freely from the lower abdominal hole. An inverted portion of the bladder wall is sewn to the abdomen during surgery. The vesicostomy is a short-term solution that can be removed afterward.

  • Ureteral reimplantation: Urine can flow back into the ureter due to an anatomical defect that is fixed via ureteral reimplantation.

• For Protecting Testicular Health: If the testicles are not positioned in the scrotum by the age of six months, doctors advise having an operation known as an orchiopexy to accomplish this (also called orchidopexy). Usually, a little incision is made in the scrotum and around the groin. The testicle is removed, put in a tiny pouch in the scrotum, and stitched to the body. Since the testicles of many male infants with a prune belly are located in the abdomen, this operation may need to be performed in two stages or concurrently with the repair of the abdominal wall.

• Reconstruction of the Abdominal Wall: To give the abdominal wall a smoother appearance, the excess skin folds that resemble ‘prunes’ must be removed, and the aberrant muscles must be tightened.

Conclusion

Prune belly syndrome in children necessitates a lifetime of close monitoring. If the baby is born with prune belly syndrome, there is a wide variety of potential outcomes. The treatment strategy is determined by the kind and degree of the affected body systems. It is crucial that the care patients get continues to be efficient and successful, especially for those with complex urologic disorders like prune belly syndrome.