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Prune belly

Prince Raj, Hirendra Birua
Congenital pouch colon (CPC) or short colon syndrome is a rare type of anorectal malformation(ARM). Type V is the rarest form of CPC. We present a 1-day-old male child with type V CPC with prune belly syndrome and congenital anterior urethrocutaneous fistula (CAUF).
April 2017: Journal of Neonatal Surgery
Luciano A Favorito, Suelen F Costa, Waldemar S Costa, Rodrigo Vieiralves, Fabio O Bernardo, Francisco J B Sampaio
PURPOSE: To compare the structure of the testis in fetuses with prune belly syndrome (PBS) to normal controls. MATERIALS AND METHODS: We studied 6 testes obtained from 3 fetuses with PBS and 14 testes from 7 male fetuses. The testicular specimens were cut into 5-μm thick sections and stained with hematoxylin and eosin (HE), to observe the seminiferous tubules; Weigert's solution to observe elastic fibers; and picrosirius red to observe collagen. The images were captured with an Olympus BX51 microscope and Olympus DP70 camera...
2017: Advances in Urology
Helce R Julio Junior, Suelen F Costa, Waldemar S Costa, Francisco J Barcellos Sampaio, Luciano A Favorito
AIMS: To study the bladder structure of fetuses with prune belly syndrome (PBS). METHODS: We studied three bladders obtained from three male fetuses with PBS and seven bladders from seven male fetuses without anomalies. Each bladder was dissected and embedded in paraffin, from which 5 μm thick sections were obtained and stained with Masson's trichrome (to quantify connective tissue and smooth muscle) and picrosirius red with polarization (to observe collagen)...
June 9, 2017: Neurourology and Urodynamics
Hemal Grover, Sanjay Sethi, Jatin Garg, Amrit Pal Ahluwalia
BACKGROUND: Prune Belly Syndrome (PBS) is a rare entity, usually found in male neonates. It comprises complex urinary tract anomalies, bilateral undescended testis and absence of anterior abdominal wall muscles. Patients with unilateral abdominal wall deficiency, unilateral undescended testis and female neonates with abdominal wall laxity are classified as Pseudo Prune Belly syndrome (PPBS). Reports on PPBS do not highlight the radiological and imaging characteristics of this syndrome and the current literature on the role of newer imaging modalities, such as Magnetic Resonance Imaging (MRI), remains relatively sparse...
2017: Polish Journal of Radiology
Marcella Pellegrino, Daniela Visconti, Vincenzo Davide Catania, Luisa D'Oria, Carlo Manzoni, Maria Giovanna Grella, Alessandro Caruso, Lucia Masini, Giuseppe Noia
INTRODUCTION: Megacystis is a condition of abnormal enlarged fetal bladder for gestational age, which is usually associated with urological malformations that may constitute a life-threatening condition for the baby. OBJECTIVE: The purpose of this study was to assess the prognostic and etiological criteria of fetal megacystis and to describe the neonatal outcome in a large series collected in a single tertiary center. STUDY DESIGN: A retrospective observational study was conducted between 2008 and 2012...
April 14, 2017: Journal of Pediatric Urology
Edwin A Smith, Arun Srinivasan, Hal C Scherz, Anthony J Tracey, Bruce Broecker, Andrew J Kirsch
INTRODUCTION: Abdominoplasty is an important component of the management of children with prune belly syndrome (PBS). While there are features of the abdominal defect in PBS which are common to all patients, there will be differences unique to each patient that should be taken into consideration in surgical planning. Specifically, we have come to realize that although the Monfort procedure assumes a symmetric pattern of abdominal wall laxity, this symmetry is rarely present. OBJECTIVE: The aim of this report is to describe our modifications and review our outcomes for the Monfort procedure which more completely address correction of the abdominal wall laxity including both common and uncommon features while positioning the umbilicus to a more anatomically correct position (Figure)...
March 22, 2017: Journal of Pediatric Urology
K Taghavi, C Sharpe, M D Stringer
Fetal megacystis is variably defined and understood. The literature on fetal megacystis was systematically reviewed, focusing on prenatal diagnosis, associations and outcomes. This yielded a total of 18 primary references and eight secondary references. Fetal megacystis has an estimated first-trimester prevalence of between 1:330 and 1:1670, with a male to female ratio of 8:1. In the first trimester, megacystis is most commonly defined as a longitudinal bladder dimension of ≥7 mm. Later in pregnancy, a sagittal dimension (in mm) greater than gestational age (in weeks) + 12 is often accepted...
February 2017: Journal of Pediatric Urology
S Castañeda, I Molina, P Jaimes, J Beltrán, J Valero, F Fierro
INTRODUCTION: The aim of the present study was to evaluate clinical presentation and management of sigmoid volvulus in children. Sigmoid volvulus is one of the three leading causes of acute obstruction of the colon and is between 50 and 90% of all large bowel volvulus. In the pediatric population only 3 to 5% of bowel obstructions are caused by volvulus and there are less than 100 cases reported in the literature. The presence of a redundant sigmoid with a narrow mesentery (dolicosigmoide) is a prerequisite for the volvulus formation...
April 15, 2015: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
Ganavi Ramagopal, Ganesh Narayana, Ashok Rathod
No abstract text is available yet for this article.
July 2016: Journal of Neonatal Surgery
Karim Awad, Anupam Lall
Prune belly syndrome is a rare abnormality; its association with VACTERL is even rarer. This association has been reported in literature a few times since first reported in 1993 and so far the majority have either been stillbirths or died shortly after birth. We present a case of Prune belly syndrome associated with VACTERL who is now one year old.
July 2016: Journal of Neonatal Surgery
Romain Boissier, Eugenie Di Crocco, Alice Faure, Geraldine Hery, Véronique Delaporte, Eric Lechevallier, Pierre D E Mouriquand, Jean-Michel Guys, Gilles Karsenty
OBJECTIVE: To document the long-term outcomes of paediatric augmentation gastrocystoplasty (AGC) in terms of preservation of renal function and maintenance of dryness, and to analyse the rate of complications. PATIENTS AND METHODS: The medical records of children who had undergone AGC between 1992 and 2000 (minimum time interval of 15 years) were reviewed retrospectively. The following data were collected: age at surgery, the cause of bladder dysfunction, functioning of the AGC, any complications, and the long-term outcome of the patients...
December 2016: BJU International
R Chhabra, A Awan, C Stapleton, G Cavalleri, P Conlon
No abstract text is available yet for this article.
June 1, 2016: Clinical Medicine: Journal of the Royal College of Physicians of London
Paulo Cesar Koch Nogueira, Isabel de Pádua Paz
OBJECTIVE: The abnormalities of the genitourinary tract development are the leading cause of chronic kidney disease (CKD) in children. The diagnosis of this disease in Brazil is late and incomplete, which results in increased morbidity and mortality in this age group. Early diagnosis of this condition is the prerogative of generalist pediatricians, and the aim of this study was to review the clinical signs and symptoms associated with developmental abnormalities of the genitourinary tract...
May 2016: Jornal de Pediatria
Herman S Bagga, Songhua Lin, Alun Williams, Jesse Schold, Nathan Chertack, David Goldfarb, Hadley Wood
PURPOSE: Improved bladder and renal management benefit patients with congenital uropathy and congenital pediatric kidney disease. This may translate to delayed initial renal transplantation in these patients, and improved graft and patient survival. Our primary study purpose was to determine whether patients with congenital uropathy and congenital pediatric kidney disease have demonstrated later time to first transplantation and/or graft survival. MATERIALS AND METHODS: SRTR (Scientific Registry of Transplant Recipients) was analyzed for first renal transplant and survival data in patients with congenital uropathy and congenital pediatric kidney disease from 1996 to 2012...
April 2016: Journal of Urology
Michael P Leonard
No abstract text is available yet for this article.
April 2016: Journal of Pediatric Urology
M L Garcia-Roig, J D Grattan-Smith, A M Arlen, E A Smith, A J Kirsch
INTRODUCTION: Magnetic resonance urography (MRU) has proven to be useful in the setting of complex urologic anatomy. Prune belly syndrome (PBS) patients are known to have malformed and highly variable urinary tract anatomy due to significant dilation and renal dysplasia. OBJECTIVE: To further characterize the renal and ureteral anatomy and renal function in patients with PBS via MRU. STUDY DESIGN: Children with PBS undergoing MRU (2006-2011) were identified...
April 2016: Journal of Pediatric Urology
Laura Travan, Samuele Naviglio, Gabriele Cont, Pierpaolo Brovedani, Riccardo Davanzo, Sergio Demarini
We report the case of an infant born after parvovirus B19-induced fetal hydrops, who presented at birth with bilateral abdominal wall laxity, which was more evident on the flanks. Imaging exams revealed congenital hypoplasia of oblique abdominal muscles not associated with other anatomical abnormalities except for small liver calcifications. We review the medical literature and identify similar cases associated with fetal ascites. We propose that isolated hypoplasia of abdominal wall muscles can be associated with fetal ascites from various causes, and represents a separate condition from prune belly syndrome...
July 2016: Congenital Anomalies
Malik Mahmood Alam
No abstract text is available yet for this article.
July 2015: Journal of Ayub Medical College, Abbottabad: JAMC
Angela M Arlen, Susan S Kirsch, Natan E Seidel, Michael Garcia-Roig, Edwin A Smith, Andrew J Kirsch
OBJECTIVE: To compare health-related quality of life (HRQoL) in children with prune-belly syndrome (PBS) and their caregivers to healthy controls, as children and adolescents with PBS face numerous potential physical and psychosocial challenges. MATERIALS AND METHODS: Study participants completed the Pediatric Quality of Life Inventory Generic Core Scales (PedsQL) 4.0 generic core scales (children) or Quality of Life Enjoyment and Satisfaction Questionnaire Short Form (Q-LES-Q-SF) (caregivers) in an online, anonymous format...
January 2016: Urology
Bin Liu, Summer L Kaplan, Hongming Zhuang
Patients with prune belly syndrome usually have tortuous ureters, which can cause difficulty in the interpretation of renal scan used to evaluate possible urine leak after renal transplant. We reported a renal scan finding in a pediatric renal transplant patient with prune belly syndrome. The radioactivity in the dilated ureter, which was lateral to the renal transplant, appears to be urine leak.
March 2016: Clinical Nuclear Medicine
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