Prune -Belly Syndrome with spina Bifida and 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

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

Clinical Genetics ◽

10.1111/j.1399-0004.1990.tb03605.x ◽

2008 ◽

Vol 38 (6) ◽

pp. 410-414 ◽

Cited By ~ 12

Author(s):

Hiroshi Watanabe ◽

Tatsuhiro Yamanaka

Keyword(s):

Urinary Tract ◽

Prune Belly Syndrome ◽

Urinary Tract Anomaly ◽

Prune Belly

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Pathogenesis of the Prune-Belly Syndrome: A Functional Urethral Obstruction Caused by Prostatic Hypoplasia

PEDIATRICS ◽

10.1542/peds.73.4.470 ◽

1984 ◽

Vol 73 (4) ◽

pp. 470-475 ◽

Cited By ~ 1

Author(s):

Philippe Moerman ◽

Jean-Pierre Fryns ◽

Paul Goddeeris ◽

Joseph M Lauweryns

Keyword(s):

Urinary Tract ◽

Abdominal Wall ◽

Abdominal Distension ◽

Abdominal Muscle ◽

Urethral Obstruction ◽

Prune Belly Syndrome ◽

Urinary Tract Abnormalities ◽

Prune Belly ◽

Urogenital Anomalies

Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy—ie, the degeneration of already formed muscle—and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prunebelly syndrome.

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PRUNE BELLY SYNDROME: A CASE REPORT

Ukrainian Journal of Nephrology and Dialysis ◽

10.31450/ukrjnd.1(45).2015.09 ◽

2014 ◽

pp. 65-69

Author(s):

M.I. Solodkiy ◽

K.A. Dzhuma ◽

R.V. Zhezhera ◽

O.J. Vylkov

Keyword(s):

Congenital Anomaly ◽

Case Report ◽

Urinary Tract ◽

Abdominal Muscle ◽

Prune Belly Syndrome ◽

Urinary Tract Abnormality ◽

Rare Congenital Anomaly ◽

Prune Belly ◽

Ultrasound Investigation ◽

Different Levels

Prune belly syndrome (PBS) is a rare congenital anomaly almost exclusive to males defined by the triad of abdominal muscle deficiency, severe urinary tract abnormality and cryptorchidism. The syndrome has a broad spectrum of affected anatomy with different levels of severity. We report a newborn boy with PBS. Diagnosis was confirmed by karyotyping, ultrasound investigation and intraoperative findings.

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Current Management of the Dilated Urinary Tract in Prune Belly Syndrome

Urologic Clinics of North America ◽

10.1016/s0094-0143(21)00899-5 ◽

1990 ◽

Vol 17 (2) ◽

pp. 407-418 ◽

Cited By ~ 2

Author(s):

John R. Woodard ◽

Ira Zucker

Keyword(s):

Urinary Tract ◽

Prune Belly Syndrome ◽

Current Management ◽

Prune Belly

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

10.2310/uro.11043 ◽

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.

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Prune-belly syndrome

Paediatrica Indonesiana ◽

10.14238/pi49.5.2009.304-8 ◽

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.

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

Journal of Clinical Urology ◽

10.1177/2051415820903196 ◽

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.

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