A possible relationship between Beckwith-Wiedemann syndrome, urinary tract anomaly and prune belly syndrome

2008 ◽  
Vol 38 (6) ◽  
pp. 410-414 ◽  
Author(s):  
Hiroshi Watanabe ◽  
Tatsuhiro Yamanaka
Keyword(s):  
Urinary Tract ◽  
Prune Belly Syndrome ◽  
Urinary Tract Anomaly ◽  
Prune Belly

scholarly journals Prune -Belly Syndrome with spina Bifida and Anorectal agenesis

2004 ◽  
Vol 43 (151) ◽  
pp. 36-37
Author(s):  
Bharat D Joshi ◽  
R Prasad ◽  
R Singh ◽  
N K Bhatta ◽  
S J Bhattarai
Keyword(s):  
Urinary Tract ◽  
Spina Bifida ◽  
Birth Defect ◽  
Abdominal Muscle ◽  
Newborn Baby ◽  
Prune Belly Syndrome ◽  
Urinary Tract Anomaly ◽  
Prune Belly ◽  
Anorectal Agenesis

Prune Belly Syndrome is a rare birth defect with only few cases reported in our part. A newborn baby withPrune belly syndrome is presented along with review of literature.Key Words: Prune belly, abdominal muscle, urinary tract anomaly

Surgical Management of the Urinary Tract for the Prune-Belly (Triad) Syndrome

2019 ◽  
Author(s):  
David B. Joseph
Keyword(s):  
Urinary Tract ◽  
Prune Belly Syndrome ◽  
Ureteral Reconstruction ◽  
Prune Belly ◽  
Short And Long Term ◽  
Tract Infections ◽  
Vascular Preservation ◽  
Urinary Reconstruction

Urinary reconstruction is tempting based on the impressive abnormal findings that are revealed on imaging. The abnormal appearance of the urinary system by itself is not enough to warrant reconstruction. Reconstruction should only be undertaken when there is clear clinical evidence that stagnant urine leads to urinary tract infections and/or obstruction that is associated with renal compromise. This chapter describes temporary and permanent upper and lower urinary reconstructions. Particular consideration is given to the pathophysiology of prune belly syndrome and the disproportionate dilation and dysfunction of the distal ureter when undertaking ureteral remodeling. The techniques of ureteral folding and formal excisional ureteral tapering are described stressing the importance of vascular preservation. The role of reduction cystoplasty is placed in perspective of short- and long-term benefits. This review contains 18 references. Key Words: Eagle-Barrett syndrome, megacystis, megaureter, prune-belly syndrome, tapered ureteral reimplant, triad syndrome, ureteral reconstruction, urinary diversion, bladder reduction.

scholarly journals Prune-belly syndrome

2009 ◽  
Vol 49 (5) ◽  
pp. 304
Author(s):  
Yenny Yenny ◽  
Kusuma P. A ◽  
Damanik M. P.
Keyword(s):  
Congenital Anomaly ◽  
Prognostic Factors ◽  
Urinary Tract ◽  
Pulmonary Hypoplasia ◽  
Clinical Findings ◽  
Prune Belly Syndrome ◽  
Live Births ◽  
Prune Belly ◽  
Clinical Variants ◽  
Urinary Tract Anomalies

Prune-belly syndrome, also known as Eagle-Barretsyndrome, is a congenital anomaly comprisingthree clinical findings: deficient abdominalmusculature, urinary tract anomalies, andbilateral cryptorchidism. Other clinical findings involvingrespiratory, skeletal, digestion and cardiovascular systemmay also accompany the syndrome. The incidence isapproximately 1 : 30,000 to 40,000 live births and 95%of cases occur in boys. Pulmonary hypoplasia and kidneyfailure are important prognostic factors that contributeto 60% of mortality rate. Treatment includes surgicalcorrection of the abdominal wall and urinary tract,orchidopexy and other supportive managements.l-4 Wereport 4 cases on typical Prune-belly syndrome, togetherwith other clinical variants.

Prune-belly syndrome in Africa: An analysis and systematic review of cases, etiology, treatment, and outcomes

2020 ◽  
pp. 205141582090319
Author(s):  
Kerri Keet ◽  
Brandon Michael Henry ◽  
R Shane Tubbs
Keyword(s):  
Health Care ◽  
Urinary Tract ◽  
Access To Health Care ◽  
Cultural Beliefs ◽  
Developed Countries ◽  
Abdominal Muscles ◽  
Prune Belly Syndrome ◽  
Associated Anomalies ◽  
Prune Belly ◽  
Access To Health

Background: Prune-belly syndrome is a rare congenital disorder characterized by a spectrum of three anomalies: bilateral undescended testes, dilated urinary tract, and anterior abdominal muscle deficiency. Objectives: In developing countries, inadequate access to health care may affect treatment and outcomes of prune-belly syndrome. This study’s goal was to review the anatomical features, etiology, genetics, management, and outcomes of cases in Africa. Methods: PubMed was searched to identify case reports and case studies describing prune-belly syndrome in Africa. Data collected from each study included the number of cases, age at diagnosis, sex, description of the abdominal muscles, testes, and urinary tract, as well as associated anomalies, management, and long-term outcomes. Results: A total of 16 publications that reported 58 cases in African countries were included. The prevalence of female patients (15.5%) was higher than in developed countries (3%). The abdominal muscles were deficient in all cases, and bilateral cryptorchidism was present in nearly all males (96%). Distension of the bladder was common, with normal anatomy reported in only one case. Bilateral hydroureters and hydronephrosis also were present in the majority of cases. Only six cases (10.3%) had no associated anomalies, such as musculoskeletal or cardiovascular. Karyotyping was performed in only three cases (5.2%) because of limited hospital facilities. Six parents (10.3%) declined treatment for their children, 12 cases (20.7%) were managed conservatively, and 25 (43.1%) received surgical intervention. Patients’ mortality rate was higher than in developed countries. Conclusion: Diagnosis and treatment of prune-belly syndrome remains a challenge in Africa, in which multiple factors, such as access to health care and cultural beliefs, affect mortality rates and outcomes. Patient education and support groups may improve compliance with treatment. Level of evidence: Not applicable for this multicenter audit.

Urinary tract reconstruction in prune belly syndrome

Urology ◽  
1978 ◽  
Vol 12 (3) ◽  
pp. 333-335 ◽  
Author(s):  
Ronald Rabinowitz ◽  
Martin Barkin ◽  
John F. Schillinger ◽  
Robert D. Jeffs
Keyword(s):  
Urinary Tract ◽  
Prune Belly Syndrome ◽  
Prune Belly ◽  
Urinary Tract Reconstruction

scholarly journals A rare case report of prune belly syndrome with malnutrition

2021 ◽  
Vol 2021 (4) ◽  
Author(s):  
Turyalai Hakimi ◽  
Mohammad Akbar Ibrahimi
Keyword(s):  
Urinary Tract ◽  
Family Medicine ◽  
Rural Areas ◽  
Valsalva Maneuver ◽  
Unknown Etiology ◽  
Abdominal Muscles ◽  
Prune Belly Syndrome ◽  
Prune Belly ◽  
Tract Infections ◽  
Urinary Tract Malformations

ABSTRACT Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by a triad of deficient abdominal wall musculature, undescended testicles and urinary tract malformations. Most of the patients have pulmonary, cardiac, skeletal and gastrointestinal tract anomalies. Lack of abdominal muscles leads to constipation due to inability to perform Valsalva maneuver, which helps push the stool out of the rectum during the defecation. Additionally, frequent respiratory tract infections, persisting constipation and urinary tract infections lead to the development of malnutrition in children. We report this case to raise the awareness of low socioeconomic and low-resource medical settings that malnutrition could be existed or caused by PBS. We also encourage the expansion of pediatric surgery and family medicine training to increase the number of specialist (family medicine) to report and refer PBS in earlier phase, while working in rural areas and remote provinces.

scholarly journals Follow up of Patients with Eagle-Barett (Prune Belly) Syndrome Treated with Single-Stage Abdominoplasty and Total Genitourinary Tract Reconstruction

2019 ◽  
Vol 29 (01) ◽  
pp. 014-020
Author(s):  
Nicolás Fernández ◽  
Ana María Ortiz ◽  
Juan David Iregui ◽  
Andrea Estrada ◽  
Angeline Rojas ◽  
...  
Keyword(s):  
Renal Function ◽  
Urinary Tract ◽  
Abdominal Wall ◽  
Management Approach ◽  
Abdominal Muscles ◽  
Prune Belly Syndrome ◽  
Urologic Surgery ◽  
Prune Belly ◽  
Bowel Movements

Abstract Introduction The Prune Belly Syndrome (PBS) is the deficiency or congenital hypoplasia of the abdominal muscles accompanied with disorders of the urinary tract. The surgical treatment for the uropathy of the PBS seeks to correct the anatomical defects that induce damage to the urinary tract, including the improvement of the bladder emptying. The correction of the abdominal wall defect must be considered as an important part of the treatment. Objective To describe of the most relevant clinic characteristics and outcomes of a group of eight patients with PBS managed with abdominoplasty and reconstructive urologic surgery. Methods Retrospective review of the medical charts of patients with PBS at Hospital Universitario San Ignacio, Hospital Militar Central of Bogotá, Clinica Infantil Colsubsidio and Fundación Oftalmológica Ardila Lulle of Bucaramanga, (Colombia) that were managed with reconstructive surgery of the urinary tract and Monfort technique abdominoplasty (between 2006 and 2016) by one of the authors (JPN). The evaluated variables included: phenotypic appearance, renal function, bowel movements, and urinary tract infection (UTI) episodes. These variables as well as the abdominal wall aspect, were evaluated postoperatively. Results There were 11 patients identified with PBS between 2006 and 2016. Eight (8) of them underwent reconstructive urologic surgery, orchidopexy and Monfort technique abdominoplasty. After the procedures, none of the patients presented deterioration of the renal function, and they reported a better quality of the stools according to the Bristol scale; there were also less episodes of UTI. The phenotypic aspect of the abdominal wall was qualified as satisfactory in all cases. Conclusions Reconstructive urologic surgery and abdominoplasty in patients with PBS is an excellent choice of management that reduces the number of UTIs, improves bowel movements according to the Bristol scale, and preserves the renal function. This management approach also improves the phenotypic aspect of the abdominal wall. All of this leads to higher survival rate and better quality of life.

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