845 results match your criteria Prune Belly Syndrome

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 Apr 24. 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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Gonadal Function and Reproductive System Anatomy in Postpubertal Prune-Belly Syndrome Patients.

Urology 2020 Nov 13;145:292-296. Epub 2020 Jun 13.

Human Reproduction Unit, Division of Urology, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.

Objective: To gain insight into the causes of infertility in Prune Belly Syndrome (PBS) by evaluating reproductive system anatomy and gonadal function in a cohort of postpubertal PBS patients.

Methods: We contacted all PBS patients 14 years old or older treated and followed at our institution. Age at orchiopexy, type of orchiopexy (with or without ligation of gonadal vessels), testicular volumes and positions were evaluated. Read More

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November 2020

Challenges in pediatric urologic practice: a lifelong view.

World J Urol 2021 Apr 23;39(4):981-991. Epub 2020 Apr 23.

Department of Pediatric, Adolescent and Reconstructive Urology, Medical Faculty Mannheim, University Medical Center Mannheim, Heidelberg University, Mannheim, Germany.

The role of the pediatric urologic surgeon does not end with initial reconstructive surgery. Many of the congenital anomalies encountered require multiple staged operations while others may not involve further surgery but require a life-long follow-up and often revisions. Management of most of these disorders must extend into and through adolescence before transitioning these patients to adult colleagues. Read More

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Prune belly syndrome in surviving males can be caused by Hemizygous missense mutations in the X-linked Filamin A gene.

BMC Med Genet 2020 02 21;21(1):38. Epub 2020 Feb 21.

Department of Urology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA.

Background: Prune belly syndrome (PBS) is a rare, multi-system congenital myopathy primarily affecting males that is poorly described genetically. Phenotypically, its morbidity spans from mild to lethal, however, all isolated PBS cases manifest three cardinal pathological features: 1) wrinkled flaccid ventral abdominal wall with skeletal muscle deficiency, 2) urinary tract dilation with poorly contractile smooth muscle, and 3) intra-abdominal undescended testes. Despite evidence for a genetic basis, previously reported PBS autosomal candidate genes only account for one consanguineous family and single cases. Read More

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February 2020

Anterior and posterior rectus abdominis sheath stiffness in relation to diastasis recti: Abdominal wall training or not?

J Bodyw Mov Ther 2020 Jan 28;24(1):147-153. Epub 2019 Oct 28.

Department of Neuroscience-Anatomy, Erasmus University Medical Center, Rotterdam, the Netherlands.

Introduction: This study explores the anatomical relation of the rectus abdominis muscles with the anterior and posterior rectus sheaths. The elastic behavior of these fascial sheets is also assessed. Both of these analyses form an anatomic-biomechanical basis for diagnosis and treatment, especially in relation to diastasis recti abdominis (DRA). Read More

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January 2020

Prune Belly Syndrome in the Pig (Sus scrofa domesticus).

J Comp Pathol 2020 Jan 13;174:81-85. Epub 2019 Dec 13.

Private Practitioner, Clínica Veterinaria Salud Animal, Griñón, Madrid, Spain.

Prune belly syndrome (PBS) is a rare multisystemic disease characterized by a deficiency of abdominal wall musculature, urinary tract anomalies and bilateral intra-abdominal testes. PBS in females is called pseudo prune belly syndrome (PPBS) and is characterized by abdominal wall laxity and urinary tract defects. We report three male and one female stillborn piglets with abdominal distension, hypoplastic abdominal wall musculature and urinary tract anomalies. Read More

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January 2020

Prune belly syndrome.

Med Clin (Barc) 2020 12 25;155(12):568. Epub 2019 Nov 25.

Sección de Gastroenterología y Nutrición, Hospital Infantil Universitario Niño Jesús, Madrid, España.

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December 2020

Study of prostate growth in prune belly syndrome and anencephalic fetuses.

J Pediatr Surg 2020 Oct 5;55(10):2221-2225. Epub 2019 Nov 5.

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

Background: To compare the growth of the prostate in anencephalic, prune belly syndrome (PBS) and control fetuses.

Methods: We studied 35 prostates from normal human fetuses aged 11-22 weeks postconception (WPC); 15 from anencephalic fetuses aged 13-19 WPC; and 6 from PBS fetuses aged 13-31WPC. After prostate dissection, we evaluated the prostate length, width and thickness with the aid of a computer program (Image Pro and Image J). Read More

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October 2020

Renal Arterial Doppler Resistive Index in Differentiating Obstructive and Non-Obstructive Hydronephrosis in Children at Tikur Anbessa Specialized Hospital, Ethiopia, October, 2018.

Ethiop J Health Sci 2019 Sep;29(5):591-596

Department of Radiology, School of Medicine, College of Health Sciences, Addis Ababa University, Ethiopia.

Background: Most congenital anomalies of the urinary tract present with hydronephrosis. Some are physiological while others pathological. Ultrasound, micturating cyst urethrography, dynamic renal scintigraphy, intravenous urography, dynamic and static magnetic resonance urography are used for examination. Read More

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September 2019

Prune belly syndrome: current perspectives.

Pediatric Health Med Ther 2019 6;10:75-81. Epub 2019 Aug 6.

Emory University, Children's Healthcare of Atlanta, Atlanta, GA 30328, USA.

Prune belly syndrome (PBS) is a rare but morbid congenital disease, classically defined by a triad of cardinal features that includes cryptorchidism, urinary tract dilation and laxity of the abdominal wall musculature. Children often require numerous surgical interventions including bilateral orchidopexy as well as individually tailored urinary tract and abdominal wall reconstruction. Along with the classic features, patients with PBS often experience gastrointestinal, orthopedic, and cardiopulmonary comorbidities. Read More

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A homozygous missense variant in CHRM3 associated with familial urinary bladder disease.

Clin Genet 2019 12 11;96(6):515-520. Epub 2019 Sep 11.

Manchester Centre for Genomic Medicine, Manchester University NHS Foundation Trust, Health Innovation Manchester, Manchester, UK.

CHRM3 codes for the M3 muscarinic acetylcholine receptor that is located on the surface of smooth muscle cells of the detrusor, the muscle that effects urinary voiding. Previously, we reported brothers in a family affected by a congenital prune belly-like syndrome with mydriasis due to homozygous CHRM3 frameshift variants. In this study, we describe two sisters with bladders that failed to empty completely and pupils that failed to constrict fully in response to light, who are homozygous for the missense CHRM3 variant c. Read More

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December 2019

Long term follow-up in a patient with prune-belly syndrome - a care compliant case report.

Medicine (Baltimore) 2019 Aug;98(33):e16745

4th Pediatric Clinic, Department of Nephrology and Dialysis Children, Emergency Hospital for Children Iaşi, Romania.

Rationale: Malformative uropathies represent a major cause of Chronic Kidney Disease (CKD) in children. Genitourinary system is the most frequent and sever affected in Prune-Belly syndrome cases. That is why the findings of early diagnosis and vigilant monitoring for these situations remain a major challenge for the medical team. Read More

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Do prune-belly syndrome and neural tube defects change testicular growth? A study on human fetuses.

J Pediatr Urol 2019 Oct 6;15(5):557.e1-557.e8. Epub 2019 Jul 6.

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

Background: There are no reports comparing testicular volume between normal fetuses, fetuses with prune-belly syndrome (PBS), and fetuses with anencephaly. The study hypothesis was that PBS and especially anencephaly alter the testicular volume during the human fetal period.

Aim: The objective of the study was to compare the testicular growth in fetuses with anencephaly, with PBS, and without anomalies. Read More

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October 2019