February SFU Issue SPU 13
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World Journal of Urology
World Journal of Urology Challenges in Paediatric Urologic Practice: a Lifelong View --Manuscript Draft-- Manuscript Number: WJUR-D-19-01106R1 Full Title: Challenges in Paediatric Urologic Practice: a Lifelong View Article Type: SIU-ICUD Congenital Lifelong Urology (Dr. Wood) Keywords: diseases, urologic; abnormalities, congenital; abnormalities, genitourinary; obstructive uropathy; bladder; exstrophy, bladder; urethral valves; cloaca; Hypospadias; bladder, neurogenic Corresponding Author: John Wiener Duke University School of Medicine Durham, NC UNITED STATES Corresponding Author Secondary Information: Corresponding Author's Institution: Duke University School of Medicine Corresponding Author's Secondary Institution: First Author: John Wiener First Author Secondary Information: Order of Authors: John Wiener Nina Huck, MD Anne-Sophie Blais, MD Mandy Rickard, MN, NP Armando Lorenzo, MD, MSc Heather N. McCaffrey Di Carlo, MD Margaret G. Mueller, MD Raimund Stein, MD Order of Authors Secondary Information: Funding Information: Abstract: 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 to avoid complications. Management of most of these disorders must extend into and through adolescence before transitioning these patients to adult colleagues. The primary goal of management of all congenital uropathies is protection and/or reversal of renal insult. For posterior urethral valves, in particular, avoidance of end stage renal failure may not be possible in severe cases due to the congenital nephropathy but usually can be prolonged. Likewise, prevention or minimization of urinary tract infections is important for overall health and eventual renal function. -
Guidelines on Paediatric Urology S
Guidelines on Paediatric Urology S. Tekgül (Chair), H.S. Dogan, E. Erdem (Guidelines Associate), P. Hoebeke, R. Ko˘cvara, J.M. Nijman (Vice-chair), C. Radmayr, M.S. Silay (Guidelines Associate), R. Stein, S. Undre (Guidelines Associate) European Society for Paediatric Urology © European Association of Urology 2015 TABLE OF CONTENTS PAGE 1. INTRODUCTION 7 1.1 Aim 7 1.2 Publication history 7 2. METHODS 8 3. THE GUIDELINE 8 3A PHIMOSIS 8 3A.1 Epidemiology, aetiology and pathophysiology 8 3A.2 Classification systems 8 3A.3 Diagnostic evaluation 8 3A.4 Disease management 8 3A.5 Follow-up 9 3A.6 Conclusions and recommendations on phimosis 9 3B CRYPTORCHIDISM 9 3B.1 Epidemiology, aetiology and pathophysiology 9 3B.2 Classification systems 9 3B.3 Diagnostic evaluation 10 3B.4 Disease management 10 3B.4.1 Medical therapy 10 3B.4.2 Surgery 10 3B.5 Follow-up 11 3B.6 Recommendations for cryptorchidism 11 3C HYDROCELE 12 3C.1 Epidemiology, aetiology and pathophysiology 12 3C.2 Diagnostic evaluation 12 3C.3 Disease management 12 3C.4 Recommendations for the management of hydrocele 12 3D ACUTE SCROTUM IN CHILDREN 13 3D.1 Epidemiology, aetiology and pathophysiology 13 3D.2 Diagnostic evaluation 13 3D.3 Disease management 14 3D.3.1 Epididymitis 14 3D.3.2 Testicular torsion 14 3D.3.3 Surgical treatment 14 3D.4 Follow-up 14 3D.4.1 Fertility 14 3D.4.2 Subfertility 14 3D.4.3 Androgen levels 15 3D.4.4 Testicular cancer 15 3D.5 Recommendations for the treatment of acute scrotum in children 15 3E HYPOSPADIAS 15 3E.1 Epidemiology, aetiology and pathophysiology -
Renal Agenesis, Renal Tubular Dysgenesis, and Polycystic Renal Diseases
Developmental & Structural Anomalies of the Genitourinary Tract DR. Alao MA Bowen University Teach Hosp Ogbomoso Picture test Introduction • Congenital Anomalies of the Kidney & Urinary Tract (CAKUT) Objectives • To review the embryogenesis of UGS and dysmorphogenesis of CAKUT • To describe the common CAKUT in children • To emphasize the role of imaging in the diagnosis of CAKUT Introduction •CAKUT refers to gross structural anomalies of the kidneys and or urinary tract present at birth. •Malformation of the renal parenchyma resulting in failure of normal nephron development as seen in renal dysplasia, renal agenesis, renal tubular dysgenesis, and polycystic renal diseases. Introduction •Abnormalities of embryonic migration of the kidneys as seen in renal ectopy (eg, pelvic kidney) and fusion anomalies, such as horseshoe kidney. •Abnormalities of the developing urinary collecting system as seen in duplicate collecting systems, posterior urethral valves, and ureteropelvic junction obstruction. Introduction •Prevalence is about 3-6 per 1000 births •CAKUT is one of the commonest anomalies found in human. •It constitute approximately 20 to 30 percent of all anomalies identified in the prenatal period •The presence of CAKUT in a child raises the chances of finding congenital anomalies of other organ-systems Why the interest in CAKUT? •Worldwide, CAKUT plays a causative role in 30 to 50 percent of cases of end-stage renal disease (ESRD), •The presence of CAKUT, especially ones affecting the bladder and lower tract adversely affects outcome of kidney graft after transplantation Why the interest in CAKUT? •They significantly predispose the children to UTI and urinary calculi •They may be the underlying basis for urinary incontinence Genes & Environment Interact to cause CAKUT? • Tens of different genes with role in nephrogenesis have been identified. -
Bladder Augmentation and Continent Urinary Diversion in Boys with Posterior Urethral Valves
peDIATRIC urology bladder augmentation and continent urinary diversion in boys with posterior urethral valves Małgorzata baka-ostrowska Pediatric Urology Department Children’s Memorial Health Institute, Warsaw, Poland key worDs posterior urethral valves. Valve ablation in a neonate with sig- urinary bladder » valve bladder » bladder nificant reflux and a markedly trabeculated bladder can remodel itself remarkably within the first year of life. The persistence of augmentation hydronephrosis, bladder wall thickening, and trabeculation, as well as persistent elevation of serum creatinine can all be the manifes- abstraCt tation of persistent bladder outlet obstruction (BOO), so urethros- copy with repeated valve ablation is necessary. But what do you do Posterior urethral valve (PUV) is a condition that leads to if the obstruction is not anatomic? Carr and Snyder consider the characteristic changes in the bladder and upper urinary point at which a functional obstruction occurs and which manage- tract. Dysfunction of the bladder such as a hyperreflec- ment is reasonable [1]. They concluded that dysfunctions of the tive, hypertonic, and small capacity bladder as well as bladder such as a hyper-reflective, hypertonic, and small capacity sphincter incompetence and/or myogenic failure should bladder, as well as sphincter incompetence and/or myogenic failure be adequately treated. Poor compliance/small blad- should be adequately treated. der could be treated with anticholinergics, but bladder Myogenic failure with overflow incontinence and incomplete augmentation will probably be indicated. Although bladder emptying should be treated with time voiding, double bladder reconstruction with gastrointestinal segments voiding, α-blockers, and intermittent catheterization. can be associated with multiple complications, includ- Detrusor hyperreflexia with urinary frequency and urge urinary ing metabolic disorders, calculus formation, mucus incontinence (UUI) are usually managed with anticholinergics. -
Long Term Follow up Result of Posterior Urethral Valve Management
Research Article JOJ uro & nephron Volume 5 Issue 1 - February 2018 Copyright © All rights are reserved by Punit Srivastava DOI: 10.19080/JOJUN.2018.05.555654 Long term Follow up Result of Posterior Urethral Valve Management Richa Jaiman and Punit Srivastava* Department of Pediatric Surgery, S N Medical College Agra, India Submission: December 01, 2017; Published: February 06, 2018 *Corresponding author: Puneet Srivastava, Associate Professor Surgery, S N Medical College Agra, UP, India, Tel: 919319966783; Email: Abstract Introduction: study is to compare Posteriorthe long term urethral result valve posterior (PUV) urethral is a commonest valves that cause are managed of urinary by differentoutflow obstructiontechniques atleading our institute. to childhood renal failure, bladder dysfunction and somatic growth retardation. The incidence of PUV is 1 in 5000 to 8000 male birth. The objective and scope of present Material and Methods: Study was carried out in S N Medical college Agra India. It is a retrospective study of the patients who were managed fromResults: 2007-17 and followed up in our department. 76% patients presented with urinary symptoms, 16.7% presented with septicemia and 6.3% presented with failure to thrive. Valve patientsablation inwas each the grade primary II, III mode and IV.of treatment4 patients developedin 23 patients, chronic vesicostomy renal failure 5 patients and 3 patients and high had diversion stage renal in 2 disease. patients. Vesicoureteric reflux was present in 26 patients. According to IAP classification of growth and development 17 patients were normal 4 patients had PEM grade - I and 3 Conclusion: care to monitor and treat the effects of altered bladder compliance. -
Diagnosis and Management in Most Frequent Congenital Defects Of
Prof. dr hab. Anna Wasilewska ~ 10% born with potentially significant malformation of urinary tract, but congenital renal disease much less common 1. Anomalies of the number a. Renal agenesis b. Supernumerary kidney 2. Anomalies of the size a. Renal hypoplasia 3. Anomalies of kidney structure a. Polcystic kidney b. Medullary sponge kidney 4. Anomalies of position • Ectopic pelvic kidney • Ectopic thoracic kidney • Crossed ectopic kidney with and without fusion 5. Anomalies of fusion • Horseshoe kidney • Crossed ectopic kidney with fusion 6. Anomalies of the renal collecting system a. Calcyeal diverticulum b. Ureterpelvic junction stenosis 7. Anomalies of the renal vasculature a. Arteriovenous malformations and fistulae b. Aberrant and accessory vessels. c. Renal artery stenosis The distinction between severe unilateral hydronephrosis and a multicystic dysplastic kidney may be unclear bilaterally enlarged echogenic kidneys, associated with hepatobiliary dilatation and oligohydroamnios suggests autosomal recessive polycystic kidney disease. Simple cysts Autosomal Dominant Polycystic Kidney Disease Autosomal Recessive Polycystic Kidney Disease Multicystic Dysplastic Kidney Disease cysts may be › solitary or multiple › unilateral or bilateral › congenital (hereditary or not) or acquired common increasing incidence with age single or multiple few mms to several cms smooth lining, clear fluid no effect on renal function occasionally haemorrhage, causing pain only real issue is distinction from tumour Characterized by cystic -
Irish Rare Kidney Disease Network (IRKDN)
Irish Rare kidney Disease Network (IRKDN) Others Cork University Mater, Waterford University Dr Liam Plant Hospital Galway Dr Abernathy University Hospital Renal imaging Dr M Morrin Prof Griffin Temple St and Crumlin Beaumont Hospital CHILDRENS Hospital Tallaght St Vincents Dr Atiff Awann Rare Kidney Disease Clinic Hospital University Hospital Prof Peter Conlon Dr Lavin Prof Dr Holian Little Renal pathology Lab Limerick University Dr Dorman and Hospital Dr Doyle Dr Casserly Patient Renal Council Genetics St James Laboratory Hospital RCSI Dr Griffin Prof Cavaller MISION Provision of care to patients with Rare Kidney Disease based on best available medical evidence through collaboration within Ireland and Europe Making available clinical trials for rare kidney disease to Irish patients where available Collaboration with other centres in Europe treating rare kidney disease Education of Irish nephrologists on rare Kidney Disease. Ensuring a seamless transition of children from children’s hospital with rare kidney disease to adult centres with sharing of knowledge of rare paediatric kidney disease with adult centres The provision of precise molecular diagnosis of patients with rare kidney disease The provision of therapeutic plan based on understanding of molecular diagnosis where available Development of rare disease specific registries within national renal It platform ( Emed) Structure Beaumont Hospital will act as National rare Kidney Disease Coordinating centre working in conjunction with a network of Renal unit across the country -
Case Report Jun 2013; Vol 23 (No 3), Pp: 360-362
Iran J Pediatr Case Report Jun 2013; Vol 23 (No 3), Pp: 360-362 Spontaneous Rupture of Kidney Due to Posterior Urethral Valve– Diagnostic Difficulties Katarzyna Kiliś-Pstrusińska1 ,MD,PhD; Agnieszka Pukajło-Marczyk1,MD; Dariusz Patkowski2 ,MD,PhD; Urszula Zalewska-Dorobisz3 ,MD,PhD; Danuta Zwolińska1 ,MD,PhD 1. Department of Paediatric Nephrology, Wroclaw Medical University, Wrocław, Poland 2. Department of Paediatric Surgery and Urology, Wroclaw Medical University, Wrocław, Poland 3. Department of Radiology, Wroclaw Medical University, Wrocław, Poland Received: Jan 18, 2012; Accepted: Aug 04, 2012; First Online Available: Nov 22, 2012 Abstract Background: Spontaneous kidney rupture could develop in the course of posterior urethral valve (PUV), the most common cause of outflow urinary tract obstruction in male infants. However, urinary extravasation is a rare complication among this group of children. Case Presentation: Our case report presents diagnostic difficulties connected with spontaneous kidney rupture due to PUV in a 6 week-old infant. Due to not equivocal images, thundery course of disease and rapid deterioration in the infant`s condition, the patient required an urgent laparatomy. Conclusion: This case showed that the investigation of renal abnormalities during early neonatal period, is very important specifically in PUV that can lead to kidney rupture. Iranian Journal of Pediatrics, Volume 23 (Number 3), June 2013, Pages: 360-362 Key Words: Urinoma; Kidney Rupture; Urinary Extravasation; Posterior Urethral Valve Introduction rare complication among this group of children. The clinical manifestation is often nonspecific. Kidney rupture in developmental age most often Our report presents diagnostic difficulties takes place in the aftermath of multiorgan trauma, connected with spontaneous kidney rupture due especially when urinary abnormalities or to PUV in a 6-week old male. -
Fetal Megacystis: a New Morphologic, Immunohistological and Embriogenetic Approach
applied sciences Article Fetal Megacystis: A New Morphologic, Immunohistological and Embriogenetic Approach Lidia Puzzo 1,*, Giuliana Giunta 2, Rosario Caltabiano 1 , Antonio Cianci 2 and Lucia Salvatorelli 1 1 Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, Azienda Ospedaliero-Universitaria “Policlinico-Vittorio Emanuele”, Anatomic Pathology Section, School of Medicine, University of Catania, 95123 Catania, Italy; [email protected] (R.C.); [email protected] (L.S.) 2 Department of General Surgery and Medical Surgical Specialties, Department of Obstetrics and Gynecology-Policlinico Universitario G. Rodolico, University of Catania, 95123 Catania, Italy; [email protected] (G.G.); [email protected] (A.C.) * Correspondence: [email protected]; Tel.: +39-095-3782026; Fax: +39-095-3782023 Received: 23 September 2019; Accepted: 26 October 2019; Published: 28 November 2019 Abstract: Congenital anomalies of the kidney and urinary tract (CAKUT) include isolated kidney malformations and urinary tract malformations. They have also been reported in Prune-Belly syndrome (PBS) and associated genetic syndromes, mainly 13, 18 and 21 trisomy. The AA focuses on bladder and urethral malformations, evaluating the structural and histological differences between two different cases of megacystis. Both bladders were examined by routine prenatal ultrasound screening and immunohistochemistry, comparing the different expression of smooth muscular actin (SMA), S100 protein and WT1c in megacystis and bladders of normal control from fetuses of XXI gestational age. Considering the relationship between the enteric nervous system and urinary tract development, the AA evaluated S100 and WT1c expression both in bladder and bowel muscular layers. Both markers were not expressed in the bladder and bowel of PBS associated with anencephaly. -
Epispadias and Exstrophy
Epispadias and Exstrophy How is bladder exstrophy/epispadias treated? Bladder exstrophy is managed surgically. One technique is to close it in stages. First the bladder and bladder neck are closed, the bones of the pelvis are brought together, and the urethra is made. Secondary surgeries are required in the first year of life to reconstruct the penis and to manage inguinal hernias. In some instances the entire reconstruction including bladder, bladder neck, pelvic bones, and urethra are closed all in one surgery. Both these techniques require an experienced surgeon. Both techniques will require the child to go to the intensive care unit (ICU) immediately after surgery for a few days. The child is then followed for a few more days in the regular (non ICU) part of the pediatric hospital before the child is ready for discharge. Hernia repairs will need to be performed later and often the child has vesicoureteral reflux, which may need to be corrected. Depending on the initial size of the bladder continence can be achieved up to 70% of the time. However, often these children require further surgeries to manage the bladder neck and treat incontinence. Often these children need to catheterize. Epispadias occurs in males and females. It occurs in bladder exstrophy and without bladder exstrophy. In males it occurs when the penis does not properly form a tube out to the tip of the penis and the opening is along the shaft of the penis. It can occur from the tip of the penis all the way to the bladder and depending on the position of the opening it can be associated with urinary incontinence (urine leakage) and retrograde ejaculation (ejaculate going backward toward the bladder instead out the urethra). -
Appendix 3.1 Birth Defects Descriptions for NBDPN Core, Recommended, and Extended Conditions Updated March 2017
Appendix 3.1 Birth Defects Descriptions for NBDPN Core, Recommended, and Extended Conditions Updated March 2017 Participating members of the Birth Defects Definitions Group: Lorenzo Botto (UT) John Carey (UT) Cynthia Cassell (CDC) Tiffany Colarusso (CDC) Janet Cragan (CDC) Marcia Feldkamp (UT) Jamie Frias (CDC) Angela Lin (MA) Cara Mai (CDC) Richard Olney (CDC) Carol Stanton (CO) Csaba Siffel (GA) Table of Contents LIST OF BIRTH DEFECTS ................................................................................................................................................. I DETAILED DESCRIPTIONS OF BIRTH DEFECTS ...................................................................................................... 1 FORMAT FOR BIRTH DEFECT DESCRIPTIONS ................................................................................................................................. 1 CENTRAL NERVOUS SYSTEM ....................................................................................................................................... 2 ANENCEPHALY ........................................................................................................................................................................ 2 ENCEPHALOCELE ..................................................................................................................................................................... 3 HOLOPROSENCEPHALY............................................................................................................................................................. -
OEIS Complex (Omphalocele, Exstrophy of Bladder, Imperforate Anus and Spine Defects)
Journal of Human Anatomy OEIS Complex (Omphalocele, Exstrophy of Bladder, Imperforate Anus and Spine Defects) Kandavadivelu M* Mini Review PSG Institute of Medical Sciences & Research, India Volume 2 Issue 1 Received Date: January 22, 2018 *Corresponding author: Manickavasuki Kandavadivelu, PSG Institute of Medical Published Date: February 16, 2018 Sciences & Research, India, Tel: 9842766782; E-mail: [email protected] Abstract OEIS complex comprises of a combination of defects including Omphalocele, Exstrophy of bladder, Imperforate anus and Spinal defects. The prenatal ultrasound of the fetus may show grossly malformed fetus. It may be provisionally diagnosed as OEIS complex by Non - visualization of urinary bladder, presence of Omphalocele, limb and spine defects in the ultrasound. During dissection of the fetus short umbilical cord and adherent amniotic membrane may be observed. On opening the sac, liver, intestines and cloaca were found to be contents. External genitalia and anal opening may not be seen. There may be an imperforate anus. Fetal spine may shows scoliosis, hemivertebrae or any other vertebral anomalies. Prognosis of the fetus depends on the associated anomalies and its severity. Serial ultrasonography may be used to accurately diagnose abdominal wall defect in utero and to diagnose the associated anomalies. The possibility of correction of the defects after birth of the fetus or termination of the fetus may be determined by the Ultrasound. Keywords: Exstrophy of bladder; Omphalocele; Scoliosis; Umbilical cord Introduction of the herniation of the abdominal contents into the proximal part of the umbilical cord. Herniation of the OEIS complex comprises of a combination of defects intestines into the cord occurs in approximately 1 in 5000 including Omphalocele, Exstrophy of bladder, Imperforate births and herniation of liver and intestines occur in 1 in anus and Spine defects.