Background

Prune Belly Syndrome (PBS), Eagle-Barrett syndrome, or abdominal muscle deficiency syndrome, is a rare congenital disorder primarily affecting male infants. However, it can occur in females as well. It is characterized by a triad of distinctive features: partial or complete absence of abdominal muscles, urinary tract abnormalities, and undescended testicles (cryptorchidism). The exact cause of PBS remains unclear, but it is thought to result from a combination of genetic and environmental factors.

The absence or underdevelopment of abdominal muscles gives the abdomen a wrinkled appearance, which resembles a prune, hence the name “Prune Belly Syndrome.” This lack of abdominal musculature can lead to gastrointestinal and urinary complications, making PBS a complex and challenging condition to manage. Children born with Prune Belly Syndrome often require early medical intervention to address the associated health issues.

Treatment may involve surgical procedures to correct urinary tract abnormalities, hernias, and undescended testicles. Ongoing care is essential to manage complications such as urinary tract infections and kidney problems. Despite the challenges associated with Prune Belly Syndrome, advances in medical care and also surgical techniques have improved the prognosis for affected individuals, and with early and comprehensive treatment, many can lead fulfilling lives. 

Epidemiology

The Epidemiology of Prune Belly Syndrome: 

• Prevalence: Prune Belly Syndrome affects approximately 1 in 30,000 to 40,000 live births. It is more commonly observed in males than females. 
• Genetic Factors: Some cases of Prune Belly Syndrome are associated with genetic factors, while others may be sporadic. 
• Geographical Distribution: The prevalence and distribution of Prune Belly Syndrome can vary across regions. There is a notable analysis of the syndrome in Africa.

Anatomy

Pathophysiology

Prune Belly Syndrome (PBS), or Eagle-Barrett syndrome, is a complex and rare congenital disorder that primarily affects the abdominal wall, urinary tract, and genitalia. While the exact cause of PBS remains unclear, there are several critical aspects of its pathophysiology: 

• Abdominal Muscle Deficiency: One of the hallmark features of PBS is the partial or complete absence of abdominal muscles, particularly the rectus abdominis muscles. This deficiency in muscle development can occur early in fetal development and contributes to the characteristic wrinkled appearance of the abdomen, which resembles a prune. This muscular deficiency weakens the abdominal wall, leading to various issues, including abdominal distension and the inability to perform normal abdominal muscle functions like coughing and bearing down during bowel movements. 
• Urinary Tract Abnormalities: PBS often involves significant abnormalities in the urinary tract. These abnormalities can include dilated and tortuous ureters, hydronephrosis (enlargement of the kidneys due to urine buildup), and bladder dysfunction. These urinary tract problems can result in urinary stasis, infections, and potentially kidney damage if not managed and treated appropriately. 
• Cryptorchidism: Another common feature of PBS is undescended testicles (cryptorchidism), where one or both testicles fail to descend into the scrotum. This can lead to infertility issues and an increased risk of testicular cancer. 
• Gastrointestinal Complications: The lack of abdominal muscles in PBS can also affect the functioning of the gastrointestinal system. Individuals with PBS may experience digestion, bowel movements, and gastrointestinal motility difficulties. Hernias, such as umbilical hernias, are also common in individuals with this syndrome. 

The underlying cause of PBS is still not fully understood, but it is thought to involve genetic and environmental factors. Some genetic mutations and abnormalities may play a role in developing PBS, but further research is needed to pinpoint the precise genetic factors involved. Additionally, the disruption of normal fetal development during early pregnancy may contribute to the formation of these characteristic abnormalities in the abdominal wall, urinary tract, and genitalia. 

Etiology

The exact etiology of Prune Belly Syndrome (PBS), also known as Eagle-Barrett syndrome, remains incompletely understood, and it is believed to involve a combination of genetic and environmental factors. Researchers have made several hypotheses about the potential causes of PBS, but no definitive cause has been identified. Here are some key considerations regarding the etiology of PBS: 

• Genetic Factors: Evidence suggests that genetic factors may contribute to the development of PBS. Some cases of PBS appear to run in families, indicating a genetic predisposition. However, no specific genetic mutation or marker has been consistently associated with the syndrome. Researchers continue to investigate the genetic aspects of PBS to understand its inheritance patterns better and identify potential genetic contributors. 

• Disruptions in Fetal Development: Many congenital syndromes, including PBS, are thought to result from disruptions in normal fetal development during early pregnancy. In the case of PBS, these disruptions are believed to occur during the formation of the abdominal wall, urinary tract, and genitalia. The precise mechanisms leading to these developmental abnormalities are not well-defined but may involve genetic, environmental, or multifactorial factors. 
• Environmental Factors: While genetic factors likely play a significant role, some researchers have explored potential environmental factors that could contribute to the development of PBS. These factors might include exposure to certain medications, toxins, or infections during pregnancy. However, no specific environmental cause has been definitively linked to PBS. 
• Multifactorial Nature: Prune Belly Syndrome is considered a multifactorial disorder, likely resulting from a combination of genetic susceptibility and environmental influences.

Genetics

Prognostic Factors

Prune Belly Syndrome (PBS), also known as the Eagle-Barrett syndrome, is a complex and variable condition, and the prognosis for individuals with PBS can vary widely based on several factors. Prognostic factors in PBS often include: 

• Severity of Abdominal Muscle Deficiency: The extent of abdominal muscle deficiency can significantly impact an individual’s prognosis. Those with more severe muscle deficiency may face more significant challenges regarding abdominal wall function and potential complications related to this aspect of PBS. 
• Urinary Tract Abnormalities: The severity and type of urinary tract abnormalities, such as hydronephrosis, vesicoureteral reflux (VUR), and kidney damage, play a crucial role in the prognosis. Early detection and appropriate management of urinary tract issues are essential to minimize the risk of kidney damage and improve long-term outcomes. 
• Kidney Function: The health and function of the kidneys are critical factors in the prognosis. Individuals with PBS may risk kidney damage due to urinary tract abnormalities, stasis, and recurrent infections. Monitoring and managing kidney function is crucial to prevent or mitigate renal complications. 
• Associated Complications: PBS is associated with various potential complications, including urinary tract infections, gastrointestinal problems, and respiratory issues. The presence and management of these complications can influence the overall prognosis. 
• Timing of Intervention: Early diagnosis and prompt intervention, including surgical correction of urinary tract abnormalities and hernias, can positively impact the prognosis. Early intervention can help prevent or diminish the risk of complications and also improve overall quality of life. 
• Multidisciplinary Care: Access to comprehensive and multidisciplinary medical care, including pediatric urologists, nephrologists, gastroenterologists, and other specialists, is essential for managing the various aspects of PBS and improving long-term outcomes. 
• Individual Variation: Recognizing that the prognosis for individuals with PBS can vary widely is essential. Some individuals may have milder forms of the syndrome and experience fewer complications, leading to better long-term outcomes, while others may face more significant challenges. 
• Psychosocial Support: The emotional and psychological well-being of individuals with PBS and their families can also impact the overall prognosis.  

Clinical History

Age Group:  

Neonates and Infants: 

Prune Belly Syndrome is often diagnosed shortly after birth or during infancy. 

Adolescents and Adults: 

As individuals with PBS transition into adolescence and adulthood, they may face unique challenges related to their condition.

Physical Examination

Abdominal Examination: 

Abdominal Wall: Assess the appearance of the abdominal wall, which typically appears wrinkled or “prune-like” due to the absence or underdevelopment of abdominal muscles. Note the severity of this feature. 

Palpation: Gently palpate the abdomen to check for abdominal distension, masses, and tenderness. Evaluate for the presence of hernias, such as umbilical hernias. 

Urinary System Examination: 

Genitalia: Examine the genitalia, particularly in males, for undescended testicles (cryptorchidism). Evaluate the position of the testicles, as they may be palpable in the inguinal canal or higher in the abdomen. 

Kidneys: Assess the size and position of the kidneys. Check for signs of abdominal masses, hydronephrosis (enlarged kidneys), or tenderness. 

Bladder: Evaluate the bladder’s size and palpate the lower abdomen for bladder distension. Inquire about any urinary symptoms or signs of urinary tract infections. 

Gastrointestinal Examination: 

Inquire about gastrointestinal symptoms, such as constipation, difficulty with bowel movements, or abdominal discomfort. 

Examine for signs of abdominal distension, which may be related to gastrointestinal issues. 

Vital Signs: 

Measure vital signs, including BP, heart rate, and respiratory rate, to assess overall health and rule out any acute issues. 

Growth and Development: 

Monitor growth parameters, including height, weight, and head circumference, especially in pediatric patients, to ensure appropriate growth and development. 

Neurological Examination: 

Assess neurological function as needed, particularly in associated spinal abnormalities or neurological conditions. 

Skin Examination: 

Inspect the skin for any signs of rashes, scars, or surgical incisions related to previous interventions. 

Assessment of Associated Complications: 

Screen for PBS-associated complications, such as urinary tract infections, kidney dysfunction, respiratory issues, and musculoskeletal problems. This may involve additional diagnostic tests and imaging studies. 

Psychosocial Assessment: 

Consider the psychological and emotional well-being of the individual and their family, as living with a rare condition like PBS can significantly impact mental health.

Age group

Associated comorbidity

Neonates and Infants: 

Neonates and infants with PBS may have urinary tract abnormalities, such as hydronephrosis (enlarged kidneys), dilated ureters, and bladder issues. Undescended testicles (cryptorchidism) are also common in male infants with PBS. 

Adolescents and Adults: 

Kidney function, fertility, and the psychological impact of living with a rare condition are considerations during this stage. 

Associated activity

Acuity of presentation

Neonates and Infants: 

The initial presentation may include a visibly distended, wrinkled abdomen, which resembles a prune due to the partial/complete absence of abdominal muscles. This characteristic abdominal appearance is a crucial feature of PBS. 

Adolescents and Adults: 

While the characteristic abdominal appearance persists into adulthood, the focus of care may shift to long-term management of urinary and kidney function and addressing fertility issues in males with cryptorchidism. 

Differential Diagnoses

Abdominal Wall Defects: 

Gastroschisis: Gastroschisis is a congenital disability in the abdominal wall that results in the protrusion of intestines outside the abdomen, typically to the right of the umbilical cord. Unlike PBS, it does not involve the characteristic wrinkled abdominal appearance. 

Neuromuscular Disorders: 

Amyoplasia Congenita: This condition involves severe muscle weakness or absence of muscles, but it does not typically present with the urinary tract and genital abnormalities seen in PBS. 

Myasthenia Gravis: This autoimmune neuromuscular disorder can cause muscle weakness, but it is not a congenital syndrome and does not include the abdominal and urological features seen in PBS. 

Urinary Tract Abnormalities: 

Posterior Urethral Valves: This congenital condition primarily affects males and involves obstruction of the urethra, leading to urinary tract abnormalities. However, it does not involve the abdominal muscle deficiency seen in PBS. 

Congenital Megaureter: This condition can lead to dilated ureters but does not typically involve the characteristic abdominal appearance of PBS. 

Neurogenic Bladder: Conditions causing neurogenic bladder dysfunction, such as spinal cord abnormalities or myelomeningocele (spina bifida), can present with urinary tract issues similar to those seen in PBS. However, these conditions do not include the abdominal muscle deficiency seen in PBS. 

Caudal Regression Syndrome (Sacral Agenesis): This condition is characterized by abnormalities in the lower spine, lower limbs, and genitourinary tract. While it shares some features with PBS, it is a distinct condition. 

Genitourinary and Renal Conditions: 

Vesicoureteral Reflux (VUR): VUR can lead to urinary tract issues but does not typically involve the abdominal muscle deficiency seen in PBS. 

Hydronephrosis can be a sign of various urinary tract abnormalities but does not include the characteristic abdominal appearance of PBS. 

Prune Belly-Like Syndrome: Some conditions may resemble PBS but lack certain key features. These may include cases of abdominal muscle deficiency without associated urinary tract abnormalities or vice versa. 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

The Prune Belly Syndrome (PBS) treatment paradigm, also known as Eagle-Barrett syndrome, is complex and typically involves a multidisciplinary approach. The specific treatment plan can vary based on the individual’s unique clinical presentation and needs. Here is a general outline of the treatment paradigm for PBS: 

Early Diagnosis and Assessment: 

Prompt diagnosis of PBS is essential to initiate appropriate care. The diagnosis is often made based on the characteristic physical features, including the wrinkled abdomen, and imaging studies such as ultrasound and sometimes genetic testing. 

Medical Management: 

Management of urinary tract issues: Urinary tract abnormalities are a hallmark of PBS. Treatment may include antibiotics to avert urinary tract infections, medications to manage bladder dysfunction, and interventions to address vesicoureteral reflux (VUR). 

Monitoring kidney function: Regular monitoring of the kidney function, including renal ultrasounds and blood tests, is crucial to detect and address kidney issues early. 

Gastrointestinal management: Individuals with PBS may experience gastrointestinal symptoms such as constipation or difficulty with bowel movements. Dietary modifications, medications, and regular follow-up with a gastroenterologist may be necessary. 

Surgical Interventions: 

Corrective surgeries: Surgical procedures are often required to address urinary tract abnormalities, such as ureteral reimplantation or valve ablation, and to correct hernias (e.g., umbilical hernia) or other abdominal issues. 

Orchidopexy: For males with cryptorchidism (undescended testicles), orchidopexy (surgery to reposition the testicles into the scrotum) is commonly performed. 

Bladder augmentation: In cases of severe bladder dysfunction, bladder augmentation surgery may be considered to increase bladder capacity and improve urinary function. 

Psychosocial Support: 

Individuals and families dealing with PBS often benefit from psychosocial support and counseling to cope with the emotional and psychological aspects of living with a rare condition. 

Long-Term Management: 

Continual follow-up: Regular follow-up with specialists, including pediatric urologists, nephrologists, gastroenterologists, and geneticists, is essential throughout life to monitor the individual’s health, address emerging issues, and adjust treatment plans. 

Addressing complications: Management includes addressing complications as they arise, such as urinary tract infections, kidney dysfunction, and potential respiratory or musculoskeletal problems. 

Fertility Considerations: 

For males with PBS, fertility assessment and options for fertility preservation may be discussed with a urologist. Cryptorchidism and potential fertility issues should be addressed in adolescence or adulthood. 

Patient and Family Education: 

Education about the condition, its management, and regular follow-up is crucial for the individual and their family. 

Research and Clinical Trials: 

Participation in research studies or clinical trials may be an option in some cases, as ongoing research can provide insights into new treatment approaches and potential therapies. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

use-of-the-non-pharmacological-therapy-for-modifying-the-environment

Prune Belly Syndrome, also known as the Eagle-Barrett syndrome, primarily involves congenital abnormalities of the abdominal wall, urinary tract, and genitalia.

While modifying the environment may not be a direct treatment for PBS, creating a supportive and accommodating environment can help individuals with PBS manage some of the challenges associated with the condition. Here are some environmental modifications and considerations for individuals with PBS: 

Hygiene and Infection Prevention: 

Maintain good hygiene practices to reduce the risk of urinary tract infections (UTIs), common in individuals with PBS. 

Encourage frequent handwashing, particularly before and after catheterization or any urinary procedures. 

Keep the genital and perineal area clean and dry. 

Accessible Restroom Facilities: 

Ensure easy access to clean, well-maintained restroom facilities, especially in public places. 

Consider the availability of changing tables and accessible stalls with support bars if needed. 

Accommodations for Catheterization: 

If catheterization is part of the individual’s routine care, provide a private and sanitary space for the procedure. 

Ensure that necessary supplies and equipment for catheterization are readily available. 

Dietary Considerations: 

Work with a healthcare provider or nutritionist to plan a diet that addresses gastrointestinal issues common in individuals with PBS, such as constipation. 

Make dietary modifications as recommended by healthcare professionals. 

Supportive Furniture and Equipment: 

Consider using supportive furniture or equipment, such as orthopedic chairs or seating cushions, to provide comfort and promote proper posture. 

Mobility Aids: 

If musculoskeletal issues are present, consider mobility aids or adaptive devices that may improve mobility and independence. 

Accessibility at Home: 

Modify the home environment to accommodate any mobility challenges or physical limitations. 

Ensure that pathways are clear and free from hazards. 

Psychosocial Support: 

Create an emotionally supportive environment with open communication and understanding among family members, caregivers, and healthcare providers. 

Consider counseling or support groups for individuals and families to cope with the psychological and emotional aspects of living with PBS. 

Education and Advocacy: 

Educate school staff, peers, and caregivers about PBS to promote understanding and reduce stigma. 

Advocate for appropriate accommodations in educational and community settings as needed. 

Regular Medical Care: 

Schedule and attend regular medical appointments with specialists to monitor and manage the condition effectively. 

Keep medical records and a list of medications and treatments up to date. 

Use of medical care for treating Prune Belly Syndrome

Medical care for individuals with Prune Belly Syndrome: 

• Medical Evaluation and Monitoring: Diagnosis is usually made shortly after birth. Regular medical check-ups are essential to monitor the progress of the condition and address any associated health issues. Frequent ultrasounds, blood tests, and other imaging studies may be necessary to monitor kidney function and urinary tract abnormalities. 
• Surgical Intervention: Many children with Prune Belly Syndrome require surgical intervention to correct urinary tract and genital abnormalities. Common surgical procedures include orchidopexy (testicle descent surgery), ureteral reimplantation, and bladder augmentation. The timing and specific surgeries required depend on the individual case. 
• Nutritional Support: Some infants with Prune Belly Syndrome may have difficulty feeding due to the abdominal muscle weakness. Nutritional support, including feeding tubes or specialized formulas, may be necessary to ensure proper nutrition and growth. 
• Management of Urinary Tract Infections (UTIs): Children with Prune Belly Syndrome are at a higher risk of UTIs due to urinary tract abnormalities.  
• Renal Function Monitoring: Kidney function should be closely monitored throughout a person’s life. Any decline in kidney function may require further medical intervention, including possible kidney transplantation. 
• Physical Therapy: It can help improve muscle tone and mobility in children with Prune Belly Syndrome. It can also be beneficial in addressing any orthopedic issues that may arise due to the lack of abdominal muscles. 
• Psychological and Emotional Support: Living with a rare condition like Prune Belly Syndrome can be challenging. Providing emotional support to both the affected child and their family is important. Support groups and counseling can be helpful. 

Use of Surgical care for treating Prune Belly Syndrome

Surgical care plays a significant role in the treatment of Prune Belly Syndrome (PBS), addressing the urological and abdominal issues associated with this condition. The specific surgical interventions required can vary widely depending on the individual’s unique case and the severity of the syndrome.  

• Orchidopexy: This surgery is performed to correct undescended testicles (cryptorchidism), which is a common feature of PBS. Undescended testicles are relocated into the scrotum to promote normal development and reduce the risk of complications, such as testicular cancer and infertility. 
• Ureteral Reimplantation: Many individuals with PBS have abnormalities in their urinary tract, such as dilated ureters or vesicoureteral reflux (VUR). Ureteral reimplantation is a surgical procedure that involves repositioning the ureters to prevent urine from flowing backward into the kidneys, reducing the risk of kidney damage and urinary tract infections. 
• Bladder Augmentation: In some cases, PBS can lead to a small and poorly functioning bladder. Bladder augmentation involves enlarging the bladder by using a piece of the patient’s intestine or synthetic material. This procedure helps improve bladder capacity and reduce the risk of urinary incontinence. 
• Mitrofanoff Procedure: For individuals with PBS who have difficulty emptying their bladder, a Mitrofanoff procedure may be performed. This surgical technique creates a channel that allows for intermittent catheterization, enabling the individual to empty their bladder more effectively. 
• Abdominal Wall Reconstruction: As PBS is characterized by the absence or severe underdevelopment of abdominal muscles, some individuals may require abdominal wall reconstruction surgery.  
• Ventral Hernia Repair: Ventral hernias, which are common in individuals with PBS due to the weakened abdominal muscles, may require surgical repair to prevent complications. 
• Kidney Surgery: In severe cases where kidney function is compromised due to urinary tract abnormalities, kidney surgery, including nephrectomy (removal of a kidney) or kidney transplantation, may be necessary. 
• Routine Monitoring: Individuals with PBS often require ongoing monitoring and periodic surgical interventions as they grow and develop. Regular urological and abdominal assessments are essential to address any new issues or complications.

Managing the undescended testis for Prune Belly Syndrome

Managing undescended testes in individuals with Prune Belly Syndrome (PBS) is an important aspect of their care. Undescended testes, also known as cryptorchidism, is a common feature of PBS. Proper management is crucial to promote normal testicular development and reduce the risk of complications, such as infertility and testicular cancer.  

• Early Evaluation and Diagnosis: The evaluation for undescended testes usually begins shortly after birth, as part of the diagnosis of PBS. It’s important to identify the condition early to initiate appropriate management. 
• Hormone Therapy (if applicable): In some cases, hormone therapy may be considered to stimulate testicular descent, especially if the testes are partially descended. Hormone therapy typically involves the use of human chorionic gonadotropin (hCG) or gonadotropin-releasing hormone (GnRH). However, the effectiveness of hormone therapy in PBS may be limited, and surgery is often required. 
• Surgical Orchidopexy: Surgical correction of undescended testes through orchidopexy is the most common and effective treatment. This procedure involves the relocation of the undescended testes into the scrotum. The timing of surgery based on several factors, including the child’s age, the severity of PBS, and the surgeon’s recommendations. Surgery is often recommended between 6 months and 1 year of age to optimize testicular development and minimize the risk of complications. 
• Bilateral Orchidopexy: In many cases of PBS, both testes may be undescended. In such instances, bilateral orchidopexy is typically performed to correct both testes in a single surgical procedure. 
• Follow-Up Care: After orchidopexy, regular follow-up appointments with a pediatric urologist are essential to monitor the testes’ development and function. These appointments help ensure that the testes are in the scrotum, have normal growth, and are producing sperm when the child reaches puberty. 
• Fertility Counseling: Individuals with PBS, even after successful orchidopexy, may still have an increased risk of fertility issues later in life. Therefore, it’s important for patients and their families to receive fertility counseling and consider sperm banking if appropriate. 
• Long-Term Monitoring: Individuals with PBS should receive ongoing urological care and monitoring throughout their lives. This includes regular check-ups to assess testicular health and address any potential issues or complications.

Use of Blocksom vesicostomy for treating Prune Belly Syndrome

A Blocksom vesicostomy is a surgical procedure that involves creating a temporary opening (stoma) in the bladder to allow urine to be diverted out of the body. This procedure can be used in cases of Prune Belly Syndrome (PBS) when there are significant urinary tract abnormalities that require intervention.

Blocksom vesicostomy is typically considered when other treatment options, such as ureteral reimplantation or bladder augmentation, are not feasible or appropriate.  

• Indications: A Blocksom vesicostomy may be considered for individuals with PBS who have severe bladder dysfunction, high-grade vesicoureteral reflux (VUR), or other urinary tract abnormalities that prevent normal bladder emptying. It is often reserved for cases where more conservative treatments have not been effective. 
• Preoperative Evaluation: Before the surgery, a thorough evaluation of the patient’s urinary tract and overall health is conducted. This evaluation may include imaging studies, urodynamic testing, and other assessments to determine the extent of the urinary tract abnormalities and the need for surgery. 

Surgical Procedure: 

• Anesthesia: The surgery is typically performed under general anesthesia. 
• Stoma Creation: During the procedure, a small incision is made in the lower abdomen, and a segment of the bladder is brought to the surface to create a stoma. The stoma serves as an external opening through which urine is diverted from the bladder. 

• Stoma Care: After the surgery, a pouch or bag is generally attached to the stoma to collect urine. Caregivers are trained on how to manage and care for the stoma and the collection bag. 
• Benefits and Goals: The primary goal of a Blocksom vesicostomy is to relieve pressure on the bladder and prevent damage to the kidneys caused by urinary retention and reflux. By diverting urine away from the bladder. 
• Postoperative Care: After the surgery, close monitoring is essential to ensure the stoma functions properly and to watch for any signs of infection or complications. Regular follow-up appointments with a pediatric urologist are necessary to assess the stoma and overall urinary tract health. 
• Reassessment and Further Surgery: A Blocksom vesicostomy is typically considered a temporary measure. As the child grows and the urinary tract develops, the healthcare team will periodically reassess the need for further surgical interventions, such as bladder augmentation or ureteral reimplantation.

use-of-intervention-with-a-procedure-in-treating-prune-belly-syndrome

The treatment of Prune Belly Syndrome (PBS), also known as Eagle-Barrett syndrome, often involves various surgical procedures to address the congenital abnormalities associated with the condition.

Surgical interventions are crucial for managing the urinary tract and abdominal issues commonly seen in PBS. The specific procedures needed depend on the individual’s clinical presentation and may include the following: 

• Ureteral Reimplantation: Many individuals with PBS have vesicoureteral reflux (VUR), where urine flows backward from the bladder into the ureters and the kidneys. Ureteral reimplantation is a surgical procedure that corrects this issue by repositioning and reattaching the ureters to the bladder to prevent reflux. 
• Valve Ablation: In some cases, individuals with PBS may have obstructive valves or membranes within the urinary tract that impede urine flow. Valve ablation is a procedure to remove or open these obstructions, allowing for better urinary drainage. 
• Bladder Augmentation: Individuals with severe bladder dysfunction may require bladder augmentation surgery. This procedure involves enlarging the bladder using a section of the bowel or other tissue, increasing bladder capacity and improving its ability to store urine. 
• Hernia Repair: Umbilical hernias and other abdominal wall defects are common in PBS due to the absence of abdominal muscles. Surgical repair of these hernias may be necessary to avert complications and reduce the risk of bowel protrusion through the abdominal wall. 
• Orchidopexy: For males with cryptorchidism (undescended testicles), orchidopexy is a surgical procedure to reposition the testicles into the scrotum. This is important for potential fertility and testicular health. 
• Gastrointestinal Surgery: In some cases, individuals with PBS may require surgical interventions to manage gastrointestinal issues, such as treating bowel obstructions or correcting anatomical abnormalities. 
• Kidney Surgery: Surgical procedures on the kidneys may be necessary to address kidney issues related to urinary tract abnormalities or to treat complications such as kidney stones. 
• Other Surgical Interventions: Additional surgeries may be required to address associated complications or anomalies, such as spinal abnormalities or respiratory issues, depending on the individual’s specific presentation. 

use-of-phases-in-managing-prune-belly-syndrome

The management of Prune Belly Syndrome (PBS), also known as Eagle-Barrett syndrome, typically consists of different phases of care to address the various aspects of the condition and its associated complications. Here’s an overview of the phases of management for PBS: 

Early Diagnosis and Evaluation: 

The management process begins with early diagnosis, often shortly after birth or in infancy, when the characteristic features of PBS become evident. 

Healthcare providers conduct a thorough evaluation to assess the severity of the abdominal muscle deficiency, urinary tract abnormalities, and other associated features. 

Immediate Care and Stabilization: 

Immediate medical care may be needed to address acute issues or complications, such as urinary tract infections, respiratory distress, or gastrointestinal problems. 

Stabilization includes providing supportive care to ensure the child’s overall health and comfort. 

Multidisciplinary Team Assessment: 

A multidisciplinary team of healthcare specialists, including pediatric urologists, pediatric nephrologists, pediatric surgeons, geneticists, and other experts, is involved in assessing the individual’s needs. 

Surgical Interventions: 

Surgical procedures are often necessary to address urinary tract abnormalities, abdominal wall issues (such as hernias), and other anatomical problems associated with PBS. 

The timing of surgeries varies, but many procedures are performed during infancy or early childhood to prevent complications and optimize outcomes. 

Long-Term Medical Management: 

Following surgical interventions, ongoing medical management is crucial.  

Medical management may include using medications to prevent urinary tract infections, manage bladder dysfunction, or address gastrointestinal symptoms. 

Psychosocial Support: 

Psychological and emotional support is an essential component of PBS management. Families and individuals with PBS may benefit from counseling, support groups, and resources to help them cope with the challenges of the condition. 

Educational Support: 

Educational support may be required to ensure that the child with PBS can access appropriate schooling and accommodations to meet their unique needs. 

Fertility Assessment and Family Planning (if applicable): 

In cases of cryptorchidism (undescended testicles) in males, fertility assessment and discussions about family planning may occur during adolescence or adulthood. 

Transition to Adulthood: 

As individuals with PBS transition into adulthood, the care focus may shift from pediatric specialists to adult specialists who can provide continued care and address specific adult healthcare needs. 

Research and Clinical Trials: 

Some individuals with PBS may be able to participate in research studies or clinical trials to explore new treatment approaches and potential therapies.

Medication

Future Trends

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References

Prune Belly Syndrome – StatPearls – NCBI Bookshelf (nih.gov) 

Prune belly syndrome: current perspectives | PHMT (dovepress.com) 

Prune-belly syndrome in Africa: An analysis and systematic review of cases, etiology, treatment, and outcomes – Kerri Keet, Brandon Michael Henry, R Shane Tubbs, 2021 (sagepub.com) 

Prune Belly Syndrome – Symptoms, Causes, Treatment | NORD (rarediseases.org) 

Prune Belly Syndrome | Children’s Hospital of Philadelphia (chop.edu) 

Prune Belly Syndrome – Physiopedia (physio-pedia.com) 

Prune Belly Syndrome – Cancer Therapy Advisor