Management Of Prune Belly Syndrome Research Articles

Diagnosis and management of prune belly syndrome in pediatric patients

Prune-Belly Syndrome has been defined as a rare urological congenital anomaly. Worldwide, a prevalence of 1 per 50,000 live births was described. Among the characteristics that identify this syndrome are the deficiency of the abdominal wall musculature, dilation of the urinary tract and bilateral cryptorchidism. Management requires great interdisciplinary knowledge and even urological surgery. The objective of this work was to identify the main methods of diagnosis and management of Prune Belly Syndrome in pediatric patients through a bibliographic review. The bibliographic review was carried out with articles published from 2017 to 2023 in high-impact journals, 25 articles were found, 8 had case reports and 17 were a theoretical source of information. In short, the key diagnosis is established by means of a prenatal ultrasound, with respect to management it can be conservative or surgical and multidisciplinary management with specialists is important.

Prune Belly Syndrome: A Ten-Year Single Tertiary Centre Experience in South-South, Nigeria

Background: Prune belly syndrome (PBS) is a congenital anomaly that consists of a triad of abdominal wall defect, bilateral cryptorchidism, and urinary tract dilation. The disease is of varying severity. This study aims to highlight the challenges and peculiarities in the management of PBS in a resource-poor setting. Materials and Methods: This is a ten-year retrospective study conducted at the University of Port Harcourt Teaching Hospital. Ethical approval for the study was sought and gotten from the hospital’s ethical committee. The information gotten included history, duration of symptoms, examination findings, age of the patient, category of disease, and intraoperative findings. The data from the folders were collected and evaluated. Frequencies, percentages, the mean and standard deviation were used to summarize the data as appropriate. Results: Fifteen patients were included in the study. The hospital incidence of PBS was 112/100,000, twelve males and three females. The age range was from 1 day to 15 years, mean age was 14 months ± 2.3 months. Most patients presented between 3 months and 2 years and 11 months. Twelve patients had category three PBS and five patients had associated anomalies. Eleven male patients died after 5 years of follow-up from progressive renal deterioration. The female patient fared better than the males. Conclusion: PBS is rare, most patients with the condition present late. The most common cause of mortality was progressive renal deterioration.

First experience: management of Prune Belly syndrome in Harapan Kita Women and Children’s Hospital

Prune belly is a rare congenital syndrome, consisting of congenital abdominal wall musculature absence, urogenital tract anomalies, and cryptorchidism in male patient. The incidence of this syndrome is 1 in 35,000 to 1 in 50,000 live births and rarely occurs in females (3-5%). One step operation of ureteral re-implantation, orchidopexy, and abdominoplasty is one option of treatment in managing this condition. This was our first experience, involving a multidisciplinary approach. A 30 year old Asian Woman G4P3A0 at 33-34 weeks of gestation was referred to the Urology Department with a fetal ultrasound showing bilateral hydronephrosis and undescended testis. Prune Belly was first suspected when abnormal abdominal circumference was noted by the obstetrician while doing a fetomaternal ultrasound at the 19 weeks gestation. No intervention was undertaken during gestation. The baby delivered spontaneously at 38 weeks gestation. Testes were impalpable and the abdominal contour was suggestive of Prune Belly. CT urography showed bilateral hydronephrosis and hydroureter. Micturating cysto-urethrography showed bilateral vesicoureteral reflux while renogram showing a cortical defect. Serial follow-ip ultrasounds were done showing bilateral hydroureter with 4th grade bilateral hydronephrosis. By the age of 2 the patient underwent one step operation of ureteral reimplantation, orchidopexy, and abdominoplasty. After the operation, the patient recovered with good function and cosmetic result. Prune belly is a rare and complex congenital syndrome. From our experience at Harapan Kita Women and Children’s Hospital, one step operation involving multidisciplinary approach of ureteral re-implantation, orchidopexy, and abdominoplasty has shown a good result. Further follow up is needed to evaluate either this choice of treatment could improve patient outcome.

Diagnosis and Medical Management of Prune Belly (Triad) Syndrome

Incidence of prune belly syndrome (PBS) has remained stable for several decades; advances have been made in prenatal diagnosis with the aid of advanced US and magnetic resonance technology. Use of fetal MRI as an adjunct to US, especially in the setting of oligohydramnios, anhydramnios, and maternal obesity, makes additional imaging methods potentially beneficial. MRI is currently accepted as a valuable technique for fetal anomalies assessment. Addition of three- and four-dimensional US has improved specificity to the traditional two-dimensional imaging. The following discusses the in utero assessment, neonatal and childhood evaluation, and management of PBS. This review contains 5 figures, 2 tables, and 48 references. Key Words: Eagle-Barrett, fast-scanning MRI, fetal ultrasonography, in utero hydronephrosis, prune belly syndrome, triad syndrome, vesicoamniotic shunt,pediatric renal insufficiency

Corrigendum: Vesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center.

[This corrects the article DOI: 10.3389/fped.2018.00180.].

Commentary to “27 years of experience with the comprehensive surgical management of prune belly syndrome”

The authors should be congratulated for presenting this large case series on the long-term outcomes of surgical treatment for prune belly syndrome (PBS) [1]. In an era when termination of pregnancy for this condition is commonplace, thus limiting the accumulation of individual experience, reports like this can certainly assist urologists dealing with prospective cases. The question that remains unanswered in the management of these patients is what the indications for surgical intervention other than orchidopexies really are. It is unquestionable that the frequent occurrence of urinary tract infections (UTIs) in association with significant dilatation and stasis in the urinary tract set a tempting stage for offering the surgical procedures described in this paper to most patients. The toll paid for such an interventional approach is significant, as supported by the 26% morbidity rate and 37% reoperation rate reported by the authors. In a recent published series with the exact same number of patients, less than 50% of the patients had major urinary reconstruction or abdominoplasty. Moreover, in the same series the second most common procedure (35% of cases) performed on the urinary tract was an appendicovesicostomy, highlighting that adequate bladder emptying may indeed be one of the key factors in preventing stasis and UTIs [2]. That is in stark contrast to the experience of the authors reported herein, where 6 out of 46

Commentary to ‘27 years of experience with the comprehensive surgical management of prune belly syndrome’

Commentary to ‘27 years of experience with the comprehensive surgical management of prune belly syndrome’

27 years of experience with the comprehensive surgical treatment of prune belly syndrome

Prune belly syndrome (PBS) presents with three main features: abdominal wall flaccidity, urological abnormalities and cryptorchidism. As a result, urologists must consider the eventual repair of the abdominal wall flaccidity and urinary tract abnormalities, and the mandatory correction of cryptorchidism, as well as decide whether to perform the procedures in a single comprehensive approach or in multiple steps. To report experiences with comprehensive surgical management of prune belly syndrome. From 1987 to 2014, 46 children with PBS were submitted for comprehensive surgical treatment. According to individual needs, treatment aimed to correct the abdominal flaccidity, reconstruct the urinary tract, and perform bilateral orchiopexy and circumcision, which were performed in one procedure. Urinary tract reconstruction was indicated whenever pyelo-ureteral dilatation with evidence of significant stasis and/or vesicoureteral reflux was associated with recurrent urinary tract infections (UTI). Treatment for this cohort included: 44 abdominoplasties, 40 upper urinary tract reconstructions, 44 cystoplasties associated with three appendico-vesicostomies, 46 bilateral orchiopexies and 36 circumcisions. The median age at surgery was 16 months and children were followed for a median of 143 months. Abdominal appearance and tonus were improved in 90% of the children after the primary surgery and 100% after reoperation. Upper urinary tract reconstruction was performed in most children and long-term follow-up showed functional stabilization of the urinary tract in about 90% of the children, with progression to renal failure in 10%. Lower urinary tract reconstruction was performed in most children (95.6%); on late follow-up, continence was observed in 81% of them, while incontinence was present in 19% and usually associated with polyuria. Adequate bladder emptying was possible in most boys (82.6%), while the remaining required clean intermittent catheterization. Pre-operative UTI was present in 89.1% and urinary sepsis in 15.2%. Postoperatively, the incidence of laboratorial UTI was significantly reduced to 39.1%, while urinary sepsis was absent. Bilateral orchiopexy was performed in all children, with 85% of the testes becoming normal in size and well located in the scrotum. Comprehensive surgical treatment is feasible and has good long-term results. A considerable incidence of reoperations due to complications or progression of the disease was observed. The long-term results for reno-ureteral anatomy and function, bladder function, infection, testicular size and location, as well as abdominal aspect and tonus, show that comprehensive surgery is an adequate method for managing children with PBS.

Prune-Belly Sendromlu Hastada Anestezi Yaklaşımı

Prune-Belly Sendromlu Hastada Anestezi Yaklaşımı

Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population.

To review the clinical manifestations and operative management of a large contemporary pediatric cohort of patients with prune-belly syndrome (PBS). PBS patients aged <21 years followed up in our pediatric urology clinic were identified by the International Classification of Diseases, Ninth Revision code (756.71). Demographics, concomitant diagnoses, surgical history, imaging studies, and renal or bladder function were evaluated. Data were available for 46 pediatric patients (44 boys and 2 girls). Mean age was 7.6 ± 4.7 years (range, 0.9-20 years). Average length of clinical follow-up was 6.8 ± 5 years. Forty-five children (97.8%) had hydroureteronephrosis, and 36 of them (78.3%) had vesicoureteral reflux. Five patients (10.9%) had significant pulmonary insufficiency, and 2 patients (4.3%) were oxygen dependent. Eighteen children (39.1%) had other congenital malformations, including cardiac in 4 patients (8.7%) and musculoskeletal anomalies in 10 patients (21.7%). Orchidopexy was the most common surgery, with all boys aged ≥3 years having undergone the procedure. Twenty-two patients (47.8%) had a history of ureteral surgery, 22 (47.8%) had bladder surgery, 11 (23.9%) had renal surgery, and 6 (13%) had urethral procedures. Nineteen patients (41.3%) underwent abdominoplasty. Eighteen children (39.1%) had documented chronic kidney disease, and 8 children (17.4%) underwent renal transplantation. Average age at transplantation was 5.1 ± 2.9 years. The mean nadir creatinine level for patients with end-stage renal disease was 1.4 mg/dL compared with 0.4 mg/dL for those not requiring transplantation (P <.001). Children with PBS have significant comorbidities and require frequent operative intervention, with disease heterogeneity necessitating an individualized management approach. Early end-stage renal disease is prevalent, with approximately 15% of children requiring kidney transplantation.

Prune Belly Syndrome

Prune belly syndrome, a rare congenital anomaly, exists almost exclusively in males and consists of genital and urinary abnormalities with partial or complete absence of abdominal wall musculature. The syndrome, caused by urethral obstruction early in development, is the result of massive bladder distention and urinary ascites, leading to degeneration of the abdominal wall musculature and failure of testicular descent. The impaired elimination of urine from the bladder leads to oligohydramnios, pulmonary hypoplasia, and Potter's facies. The syndrome has a broad spectrum of affected anatomy with different levels of severity. The exact etiology of prune belly syndrome is unknown, although several embryologic theories attempt to explain the anomaly. With advances in prenatal screening techniques, the diagnosis and possible treatment of prune belly syndrome can occur before birth, although controversy exists on the proper management of prune belly syndrome. This article explores the theories behind the pathophysiology and embryology of prune belly syndrome and its multisystemic effects on the newborn infant. Specific attention is paid to presentation, clinical features, head-to-toe physical assessment, family support, and nursing care of infants with prune belly syndrome.

Comprehensive surgical treatment of prune belly syndrome: 17 years' experience with 32 patients

Objectives To describe the results of comprehensive surgical management of prune belly syndrome. Methods Thirty-two patients were evaluated and treated for abdominal and genitourinary abnormalities. The surgical procedure, including simultaneous abdominoplasty, bilateral orchiopexy, and urinary tract reconstruction, was performed according to individual needs. At follow-up, the urinary tract, abdominal, and genital anatomy and function were evaluated. Results The upper urinary tract anatomy and function were stabilized in 30 patients, and no vesicoureteral reflux was found postoperatively. The bladder drainage was adequate in 29 patients, and 4 presented with recurrent bacteriuria. Abdominoplasty improved flaccidity in 29 patients. Adequate testicular position and consistency were obtained in 54 testes. Complications included acute tubular necrosis (1 patient) and ureterovesical obstruction (3 patients). Conclusions Individualized comprehensive surgical management of prune belly syndrome is effective, with an acceptable incidence of complications and excellent long-term results.

Editorial comment

The appropriate treatment of patients with PBS has produced controversy between those who advocate early surgical rehabilitation and those who believe that surgical remodeling of the urinary tract should be considered only when required by the patient's deteriorating clinical course. The resolution of this controversy has been confounded by the rarity and broad variation in the severity of this disorder. Comparisons of the relative merits of these approaches remain based on patient series in which one or the other method has been applied. This report describes the management of a diverse group of patients and cannot fully resolve this debate. However, this large patient series does confirm that a comprehensive surgical approach can yield very good long-term results. Improved upper tract drainage and elimination of reflux in this series translated into less urinary stasis and a lower incidence of urinary tract infection. Of the 32 patients, 30 demonstrated stable or improved renal function at a mean follow-up of 5 years. Of note, these results were achieved only after several patients had undergone multiple procedures. The authors' approach may have been more aggressive than that applied by others in that 16 of these patients were considered to display category III features. Most often these patients still require orchiopexy but are less likely to require urinary tract reconstruction. Reflux, particularly when associated with urinary tract infection, progressive dilation of the urinary tract, or worsening renal function, would represent the usual indication for intervention and may have emerged in this group. Comprehensive surgical treatment of prune belly syndrome: 17 years' experience with 32 patientsUrologyVol. 64Issue 4PreviewTo describe the results of comprehensive surgical management of prune belly syndrome. Full-Text PDF Reply by the authorsUrologyVol. 64Issue 4PreviewWe thank the reviewer for his very thoughtful comments. It is important to stress that most of the patients underwent surgery at the time of their referral, and our main objective was to treat all abnormalities during only one procedure that included tailored urinary tract reconstruction, orchiopexy, abdominoplasty, and circumcision, as soon as possible. Full-Text PDF

Recent trends in the management of prune-belly syndrome

Recent trends in the management of prune-belly syndrome