860 results match your criteria Prune Belly Syndrome


Dental treatment of patients with prune belly syndrome.

Spec Care Dentist 2022 May 8. Epub 2022 May 8.

Department of Veterans Affairs, California, McClellan, USA.

Background: Prune belly syndrome (PBS), also known as Eagle-Barrett syndrome (EGBRS), is a rare congenital disease characterized by deficiency or absence of abdominal wall muscles, urological abnormalities, and bilateral cryptorchidism.

Types Of Studies Reviewed: A review of literature was done using four search engines (PubMed, Google Scholar, Scopus, Science Direct) and keywords (individually and in combinations): prune belly syndrome, PBS, Eagle-Barrett syndrome, dental manifestation, clinical manifestation, and psychological aspects. The search was run with no language restrictions and covered the 1965-2021 time period. Read More

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Study of Penis Structure and Development in Fetus With Prune Belly Syndrome.

Urology 2022 May 4. Epub 2022 May 4.

Urogenital Research Unit, State University of Rio de Janeiro, Rio de Janeiro, Brazil. Electronic address:

Objective: To evaluate the influence of prune belly syndrome (PBS) on the development of penises in human fetuses.

Methods: We studied 39 human fetuses (including 4 with PBS) aged 11-22 weeks post conception. We measured the length and width of the free portion and penis root and the penis total length (PTL). Read More

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Robotic treatment of ureteropelvic junction obstruction in Eagle-Barrett Syndrome.

Can J Urol 2022 04;29(2):11116-11118

Department of Urology at University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

Eagle-Barrett Syndrome (EBS) is a rare congenital condition characterized by the triad of absent or defective abdominal wall muscles, urinary tract abnormalities, and bilateral cryptorchidism. Ureteropelvic junction obstruction (UPJO) is seldom reported in these patients, despite it being a common cause of childhood obstructive uropathy. We present the case of a patient with EBS who was subsequently identified as having symptomatic UPJO that was successfully treated with robotic pyeloplasty. Read More

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Prune Belly Syndrome Associated with Interstitial 17q12 Microdeletion.

Case Rep Urol 2022 14;2022:7364286. Epub 2022 Feb 14.

Department of Pediatrics, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.

We report a term male neonate presenting with a "prune belly," bilateral hydronephrosis, hydroureter, posterior urethral obstruction, and bilateral undescended testes. Analysis with the whole genome SNP microarray revealed an interstitial deletion of about 1.49 megabase (MB) at chromosome 17q12. Read More

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February 2022

Response to letter to the editor re "Surgical approach to Prune-Belly syndrome: A review of our series and novel surgical technique".

J Pediatr Urol 2022 Apr 5;18(2):249. Epub 2022 Feb 5.

Reina Sofia University Hospital, St/ Menendez Pidal, S/n, 14004 Córdoba, Spain. Electronic address:

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Comments on the paper Surgical approach to prune belly syndrome: A review of our series and novel surgical technique.

J Pediatr Urol 2022 Apr 5;18(2):250-251. Epub 2022 Feb 5.

Hospital Federal dos Servidores do Estado, Ministry of Health, Rio de Janeiro, Brazil. Electronic address:

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Third Renal Transplant in a Patient With Prune Belly Syndrome.

Cureus 2021 Nov 30;13(11):e20048. Epub 2021 Nov 30.

Division of Transplant Surgery, Augusta University Medical College of Georgia, Augusta, USA.

Kidneys are the most frequently transplanted organ in the United States. An infrequently encountered cause of end-stage renal disease requiring permanent dialysis is prune belly syndrome. Prune belly syndrome is mostly seen in males and over a third of patients require permanent dialysis. Read More

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November 2021

Bladder Cancer in a Long-term Survivor of the Prune Belly Syndrome.

Urology 2022 03 6;161:93-95. Epub 2021 Dec 6.

Department of Urology, Higashiosaka City Medical Center, Osaka, Japan.

Prune belly syndrome (PBS) is a rare but morbid complexity of congenital anomalies that consists of abdominal wall defect, bilateral cryptorchidism, and urinary tract dilation. With the recent advances in perinatal care, the life expectancy of patients with PBS has improved. Although renal and sexual outcomes are frequently discussed in the literature, little is known about urinary tract malignancies in adulthood. Read More

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Case Report: Novel Copy Number Variant 16p11.2 Duplication Associated With Prune Belly Syndrome.

Front Pediatr 2021 23;9:729932. Epub 2021 Sep 23.

Department of Urology, University of Louisville, Louisville, KY, United States.

Prune belly syndrome (PBS) is a rare congenital disease that predominantly occurs in males and is identified by its classic triad of abdominal wall musculature deficiencies, cryptorchidism, and urinary tract abnormalities. However, numerous anomalies involving the kidneys, heart, lungs, and muscles have also been reported. A multitude of chromosomal abnormalities have been implicated in its pathogenesis. Read More

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September 2021

Surgical approach to prune-belly syndrome: A review of our series and novel surgical technique.

J Pediatr Urol 2021 Oct 27;17(5):704.e1-704.e6. Epub 2021 Jul 27.

Pediatric Surgeon, Reina Sofia University Hospital, St/ Menendez Pidal, S/n, 14004, Córdoba, Spain. Electronic address:

Introduction: Surgical management of children with Prune-belly syndrome (PBS) can be divided into three categories: urinary tract reconstruction, abdominal wall reconstruction, and orchidopexy. Adequate repair of the abdominal wall by abdominoplasty at an early age, allows an adequate aesthetic appearance, but also allows a correct development of walking, breathing, defecation and urination.

Objective: To present a novel surgical technique for abdominal wall reconstruction, which combines plication with complete overlap of the fascia and neoumbilicoplasty with an island flap rotated on itself. Read More

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October 2021

MP44-09 UNDERSTANDING PRUNE BELLY SYNDROME AT SINGLE CELL RESOLUTION.

J Urol 2021 09 4;206(Suppl 3):e796. Epub 2021 Aug 4.

Introduction And Objective: Prune Belly Syndrome (PBS) is characterized by bladder dysmyogenesis, yielding a dysfunctional compliant thick wall with excess collagen deposition. To dissect the cellular heterogeneity and gene expression networks altered in PBS, we report the cell type composition and transcriptional activity of PBS human bladder by using single cell RNA sequencing (scRNA-seq).

Methods: Using IRB-approved methods, bladder dome from 2 PBS and 6 non-PBS control (CO) males underwent fresh single-cell digestion. Read More

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September 2021

Robotic abdominoplasty for abdominal wall lateral bulging repair in an adult prune belly syndrome patient: a novel approach.

Hernia 2021 12 17;25(6):1727-1730. Epub 2021 Jul 17.

Division of Urology, Hospital das Clinicas da Universidade de Sao Paulo, University of São Paulo School of Medicine, Avenida Dr. Eneas de Carvalho Aguiar, 255-Sala 710F, 7° Andar, São Paulo, SP, 05403-000, Brazil.

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December 2021

Prune belly syndrome in Finland - A population-based study on current epidemiology and hospital admissions.

J Pediatr Urol 2021 Oct 26;17(5):702.e1-702.e6. Epub 2021 Jun 26.

Department of Pediatric Surgery, Turku University Hospital and University of Turku, Kiinamyllynkatu 4-8, 20521, Turku, Finland.

Background: Prune belly syndrome (PBS) is a multisystem disease characterized by absent or deficient abdominal musculature with accompanying lax skin, urinary tract abnormalities, and cryptorchidism. Previous studies have estimated a birth prevalence of 1 in 35,000-50,000 live births.

Objective: We set out to clarify the epidemiology and early hospital admissions of PBS in Finland through a population-based register study. Read More

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October 2021

Hypoplasia of abdominal wall muscles following massive fetal persistent chylous ascites without anemia.

Oxf Med Case Reports 2021 Jun 18;2021(6):omab039. Epub 2021 Jun 18.

Department of Obstetrics and Gynecology, Faculty of Medicine, Saitama Medical University, Saitama, Japan.

Abdominal wall hypoplasia is a widely known clinical finding of genetic disorders such as the prune belly syndrome. On the other hand, there are few cases of abdominal wall muscle hypoplasia associated with fetal ascites due to fetal hydrops caused by fetal anemia have been reported. We report a case of fetal chylous ascites without anemia, resulting in abdominal wall muscle hypoplasia and flabby skin. Read More

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Modern management of and update on prune belly syndrome.

J Pediatr Urol 2021 08 24;17(4):548-554. Epub 2021 Apr 24.

Pediatric Urology Unit, Division of Urology, Hospital Das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. Electronic address:

Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, a variable degree of urinary tract involvement and cryptorchidism. Most cases of PBS are sporadic and have a normal karyotype, with 95% patients being male. In the last decade, mutations in known genes that regulate embryonic genitourinary myogenesis have been identified and with increasing knowledge of these critical genes involved in bladder maldevelopment, advances can be made in genetic counseling. Read More

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A rare case report of prune belly syndrome with malnutrition.

Oxf Med Case Reports 2021 Apr 28;2021(4):omab017. Epub 2021 Apr 28.

Department of Pediatric Internal Disease, Kabul University of Medical Science, Maiwand University Hospital, Kabul, Afghanistan.

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. Read More

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Respiratory support in the absence of abdominal muscles: A case study of ventilatory management in prune belly syndrome.

Paediatr Respir Rev 2021 Mar 25;37:44-47. Epub 2020 Jul 25.

Departement of Critical Care, Universitair Ziekenhuis Antwerpen, Wilrijkstraat 10, 2650 Edegem, Belgium.

Prune belly syndrome (PBS) results in a total lack of abdominal musculature. Abdominal muscles have an important function during inspiration and expiration. This puts the patient at risk for respiratory complications since they have a very limited ability to cough up secretions. Read More

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A novel technique of abdominoplasty for prune belly syndrome.

Afr J Paediatr Surg 2020 Jul-Dec;17(3 & 4):108-110

Department of Paediatric Surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

Prune belly syndrome is an extremely rare congenital condition occurring predominantly in males. This triad syndrome comprises of partial or complete deficient abdominal wall muscles, undescended testes and dilated urinary collecting system. We present the case of a 2-year-old male patient, who presented with classic prune belly syndrome, operated with modification in classical Monfort technique of abdominoplasty. Read More

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Megacystis-microcolon-intestinal hypoperistalsis syndrome associated with cystic fibrosis and meconium peritonitis in a female neonate 4 days of age - case report and review of the literature.

Med Pharm Rep 2020 Oct 25;93(4):422-427. Epub 2020 Oct 25.

Pediatric Surgery Department, Emergency Children's Hospital, Cluj-Napoca, Romania.

We present a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) in a four days old female infant who presented with abdominal distension, bilious vomiting, massive hematuria and feeding intolerance which was first interpreted as Prune Belly Syndrome (PBS), referred to our department after iatrogenic gastric and colonic perforation. Berdon syndrome or MMIHS is a rare congenital anomaly characterized by a massive enlarged bladder, distended abdomen, microcolon, functional obstruction of the gastrointestinal tract, and malrotation. Read More

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October 2020

Unusual variant of pseudo prune belly syndrome.

BMJ Case Rep 2020 Oct 30;13(10). Epub 2020 Oct 30.

Paediatric Surgery, Christian Medical College and Hospital Vellore, Chennai, Tamil Nadu, India

Unilateral pseudo prune belly syndrome (PPBS) is a rare variant with only two other cases found in the main literature until. We present a 9-month old boy with left-sided lax abdominal wall, undescended testes and major vesicoureteric reflux involving only the left side. He underwent left orchidopexy and left end ureterostomy followed by left nephrectomy. Read More

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October 2020

Impact of the COVID-19 pandemic on the surgical activity of Pediatric Urology: analysis of postoperative complications according to the Clavien-Dindo classification.

Actas Urol Esp (Engl Ed) 2020 Dec 17;44(10):659-664. Epub 2020 Sep 17.

Servicio de Cirugía Pediátrica. Hospital Universitario 12 de Octubre, Madrid, España.

Introduction And Objective: The coronavirus disease 2019 (COVID-19) has caused a pandemic of global impact that forced social-political measures to be taken, such as the declaration of the state of alarm in Spain. At the same time, the reorganization of the pediatric medical-surgical activities and infrastructures was carried out, with the consequent suspension of the non-urgent surgical activity of Pediatric Urology. We analyzed the impact of the COVID-19 pandemic on surgical activity in a Pediatric Urology division, as well as surgical complications according to the Clavien-Dindo classification. Read More

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December 2020

Successful sperm retrieval in prune belly syndrome.

Asian J Urol 2020 Oct 20;7(4):376-378. Epub 2019 Jul 20.

Department of Urology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

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October 2020

Fetal bladder outflow obstruction: Interventions, outcomes and management uncertainties.

Early Hum Dev 2020 11 10;150:105189. Epub 2020 Sep 10.

Chelsea and Westminster and Imperial College Hospitals, United Kingdom of Great Britain and Northern Ireland; Imperial College London, United Kingdom of Great Britain and Northern Ireland. Electronic address:

Fetal lower urinary tract obstruction (LUTO) is classically based on prenatal ultrasound identification of a dilated/ thick-walled bladder, bilateral hydronephrosis, dilated ureters and a dilated posterior urethra (also known as the "keyhole sign") in a male fetus. Although the most common underlying diagnosis is posterior urethral valves, the prenatal appearance may be similar with urethral atresia or stenosis, the Prune-Belly Syndrome, or even a cloacal anomaly in a female. These conditions form part of the Congenital Anomalies of Kidney and Urinary Tract (CAKUT) spectrum, which is the commonest cause of end-stage renal disease in children. Read More

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November 2020

Prune perineum surgical correction - Treatment of a rare syndrome.

J Pediatr Urol 2020 10 19;16(5):723-724. Epub 2020 Aug 19.

Urology Unit, Hospital Das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil.

Introduction: Prune Perineum Syndrome (PPS) is rare, with only three cases described in the literature. Treatment requires abdominal and pelvic surgeries. Our goal is to provide a video with steps of the surgical corrections of this syndrome. Read More

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October 2020

Prune-Belly syndrome, a rare case presentation in neonatology: about one case in Yaounde, Cameroon.

Pan Afr Med J 2020 17;36:102. Epub 2020 Jun 17.

Faculty of Medicine and Biomedical Sciences, University of Yaounde 1, Yaounde, Cameroon.

The Prune-Belly syndrome (PBS) is a rare pathology predominating in male infants, classically manifesting with the triad including aplasia of the abdominal wall muscles, dilatation of the urinary tract, and testicular abnormalities. We report and discuss the case of a full-term male newborn, in whom clinical examination at birth revealed abdominal wall muscle hypoplasia, cryptorchidism, urinary tract dilatation and renal failure. The diagnosis was made based on physical assessment, abdominal ultra-sonographic imaging, and blood sampling of urea and creatinine. Read More

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December 2020

Challenging cases in urology: Hematuria in a man with prune belly syndrome.

Urol Times 2019 Nov;8(5):9-10

Department of Urology, University of Texas Southwestern Medical Center, Dallas, TX.

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November 2019

The role of intra-abdominal pressure in human testicular migration.

Int Braz J Urol 2021 Jan-Feb;47(1):36-44

Unidade de Pesquisa Urogenital, Universidade do Estado do Rio de Janeiro - Uerj, Rio de Janeiro, RJ, Brasil.

Objectives: This review aims to study the role of the abdominal wall in testicular migration process during the human fetal period.

Materials And Methods: We performed a descriptive review of the literature about the role of the abdominal wall in testicular migration during the human fetal period.

Results: The rise in intra-abdominal pressure is a supporting factor for testicular migration. Read More

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Are pressure pop-offs beneficial to the bladder in boys with posterior urethral valves?

J Pediatr Urol 2020 Aug 3;16(4):488.e1-488.e8. Epub 2020 Jun 3.

Division of Urology, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 E Chicago Ave, Chicago, IL, 60611, USA. Electronic address:

Background: Pressure pop-offs, such as high-grade vesicoureteral reflux with renal dysplasia, have historically been considered beneficial for renal and bladder outcomes in boys with posterior urethral valves (PUV). Recent longer-term studies have called into question the beneficial effects of pop-offs on renal function.

Objective: To evaluate how pop-offs affect bladder outcomes in boys with PUV. Read More

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Demographics and baseline care among newly transitioning adult congenital urology patients.

J Pediatr Urol 2020 Aug 1;16(4):476.e1-476.e6. Epub 2020 Jun 1.

University of California, San Francisco, Mission Hall Global Health Sci Bldg, 550 16th Street, 6th Floor, Box 1695, San Francisco, CA, 94143, USA. Electronic address:

Introduction: In 2017, UCSF established a formal Transitional Urology (TU) clinic co-run by pediatric and adult urology aimed at providing comprehensive urologic care for people progressing into adulthood with complex urologic histories.

Objective: We aim to describe baseline demographic and disease characteristics of this population, understand gaps in care, and gauge follow-through.

Study Design: We performed a retrospective chart review of all new patients in the TU clinic at UCSF from February 2017 through January 2019. Read More

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