1. Delayed Recognition of Bladder Exstrophy and Persistent Cloaca: an OEIS Variant Authors: Itunu O
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Labial Fusion Causing Coital and Voiding Difficulty in a Young Woman
Advances in Sexual Medicine, 2013, 3, 11-13 doi:10.4236/asm.2013.31002 Published Online January 2013 (http://www.scirp.org/journal/asm) Labial Fusion Causing Coital and Voiding Difficulty in a Young Woman Murat Ozekinci1, Selcuk Yucel2, Cem Sanhal1, Munire Erman Akar1 1Department of Obstetrics and Gynecology, Akdeniz University School of Medicine, Antalya, Turkey 2Department of Urology, Akdeniz University School of Medicine, Antalya, Turkey Email: [email protected] Received November 6, 2012; revised December 15, 2012; accepted December 29, 2012 ABSTRACT Adhesions of the labia are extremely rare in the reproductive population with only a few cases described in the literature. We report a young female patient with coital and voiding difficulty. The patient was a 22-year-old woman presented with voiding difficulty and inability to achieve sexual intercourse. Clinical examination revealed normally developed labia majora, clitoris, and vaginal opening, but fused labia minora and pinhole external urethral orifice. Intraoperatively, labial fusion was dissected with sharp dissection. Urethral dilatation was performed with dittel dilators. Local estrogen ointment was administered postoperatively for four weeks resulting with complete resolution of coital and voiding symptoms. Keywords: Labial Fusion; Labial Adhesion 1. Introduction to voiding difficulty and two episodes of urinary tract infection in the last two years. She had no history of any Labial fusion (LF) is defined as partial or complete adhe- trauma or sexual assault in any time as well. Gyneco- sion of the labia minora and estimated to occur in 0.6% - logical examination revealed partial adhesion of the labia 5% of prepubertal girls [1]. But this condition is a rare minora (Figure 1). -
Urinary Retention Due to Labial Fusion; Prof-1758 Bloodless Correction
CASE REPORT URINARY RETENTION DUE TO LABIAL FUSION; PROF-1758 BLOODLESS CORRECTION. A CASE REPORT. TARAVAT FAKHERI M.D NASRIN JALILIAN, M.D Assistant Professor – Obs & Gyn Department Assistant Professor – Obs & Gyn Department Maternity Research Center, Maternity Research center, Kermanshah University of Medical Sciences, Kermanshah University of Medical Sciences, Kermanshah, Iran Kermanshah, Iran HAMIDREZA SAEIDIBOROJENI, M.D Farahnaz keshavarzi, M.D Assistant Professor – Neurosurgery Department Assistant Professor – Obs & Gyn Department Maternity Research Center, Maternity Research Center, Kermanshah University of Medical Sciences, Kermanshah University of Medical Sciences, Kermanshah, Iran Kermanshah, Iran ABSTRACT: This report describes a 74 year old woman with urinary symptoms progressing to complete anuria with dense labial adhesions. This condition is mostly reported in pediatric age group but few reports addressed this condition in postmenopause. Key words: labial fusion, anuria INTRODUCTION prescribed and 3 months after surgery no relapse was Labial fusion which is equal to phimosis is rarely seen in noted and the patient felt comfortable about her urinary adults of postmenopause1. Different terms describing symptoms. this condition have been used in literature. The first report of labial fusion was described in1936 in American literature2.This condition is mostly discussed in childhood period and rare cases have been reported in post menopausal age group. It seems that more cases are reported so attention to etiology and treatment modalities should be paid to this age group due to paucity of information regarding etiology and the best treatment option undertaken. CASE REPORT A 74 year old woman para 5 with urinary symptoms as urinary retention was referred to a private clinic. -
Bladder & Cloacal Exstrophy
Bladder & Cloacal Exstrophy: A 30 Year Journey of Innovation Rosemary H. Grant, RN BSN Boston Childrens Hospital Department of Urology/ Surgical Programs 27th Annual APSNA Scientific Conference Palm Desert , California Bladder & Cloacal Exstrophy There are no disclosures Bladder & Cloacal Extrophy Objectives Identify 3 systems involved in the Exstrophy Complex Diagnosis Define the procedure for management of the exstrophied bladder State 2 components of psychosocial support for the Exstrophy Population 1 Exstrophy Complex Exstrophy – Epispadias (EEC): Classic Bladder Exstrophy Epispadias Cloacal Exstrophy (OEIS): Omphalocele Exstrophy Imperforate anus Spinal anomaly Exstrophy/Epispadias Complex (EEC) Incidence- 1:10,000- 1:50,000 live births 5:1 ratio of male- female births Embryology Typically occurs between 9 and 12 weeks gestation Cloacal membrane ruptures prematurely AFTER separation of the GI and GU tracts Presentation: Eversion of the bladder through an abdominal wall defect Exposure of the inner bladder mucosa Exposure of the dorsal urethra Lack of musculature in the anterior abdominal wall over the bladder Bladder is exposed and drains onto the abdomen Bladder Exstrophy Prenatal Diagnosis (Fetal US) Courtesy of Carol Barnewolt, MD 2 Bladder Exstrophy (Boy) Boy: Frontal view Umbilicus Bladder Low-lying umbilicus Urethral Exposed (inside-out) bladder Plate Urethra open on dorsum (top) Glans of the penis Penis Scrotum Bladder Exstrophy (Girl) Girl: Frontal view Umbilicus Bladder open on Bladder abdominal wall Urethral Urethra open -
Labial Fusion Causing Upper Urinary Tract Obstruction
CASE REPORT LABIAL FUSION CAUSING UPPER URINARY TRACT OBSTRUCTION JOHN C. NORBECK, M.D. MICHAEL R. RITCHEY, M.D. DAVID A. BLOOM. M.D. From the Section of Urology, University of Michigan Medical Center, Ann Arbor, Michigan ABSTRACT-An infant with dense and nearly complete labial fusion had marked hy- droureteronephrosis which resolved after incision of the adherent labia. Although this is an unusual consequence of labial fusion, it argues for upper urinary tract screening in these children. Under the title “Atresia of the Vulva in Chil- ureteral reflux. On diuretic renography, the t% dren,” Nowlin and Adams’ presented the first de- times were prolonged bilaterally but both sides re- tailed report of labial adhesions in the American sponded to furosemide. Differential function was literature in 1936. Campbell* used the term vulvar 51 percent on the right and 49 percent on the left. fusion and provided a comprehensive review of 9 The patient was placed on a regimen of suppres- cases in 1940. Only 29 cases were reported by sive antibiotics and given estrogen cream for topi- 1949.’ The urinary tract is rarely investigated in cal labial application. children with labial adhesions. We report a case Renal ultrasonography three weeks later re- of labial fusion that resulted in marked hy- vealed complete resolution of the hydronephrosis, dronephrosis. normal renal echotexture, and a normal bladder (Fig. 3). An IVU showed prompt symmetric func- CASE REPORT tion and a normal collecting system. The t% nor- A six-month-old female baby was hospitalized malized bilaterally on diuretic renogram. A fol- for a febrile urinary tract infection. -
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. -
An Unusual Clinical Presentation of Labial Fusion in Post Pubertal Period
International Journal of Reproduction, Contraception, Obstetrics and Gynecology Pandey K et al. Int J Reprod Contracept Obstet Gynecol. 2018 Mar;7(3):1233-1235 www.ijrcog.org pISSN 2320-1770 | eISSN 2320-1789 DOI: http://dx.doi.org/10.18203/2320-1770.ijrcog20180925 Case Report An unusual clinical presentation of labial fusion in post pubertal period Kiran Pandey, Kaustubh Srivastava, Snehlata Singh*, Pavika Lal Department of Obstetrics and Gynecology, GSVM Medical College, Kanpur, Uttar Pradesh, India Received: 01 November 2017 Accepted: 20 January 2018 *Correspondence: Dr. Snehlata Singh, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Labial fusion is sealing of labia minora in midline, also known as labial adhesion or labial agglutination or synechia vulvae. This condition is common in pre-pubertal females usually asymptomatic when oestrogen levels are low and commonly resolves spontaneously post-puberty if unresolved medical treatment includes use of estrogen cream or betamethasone cream application, very rarely surgical treatment required, if not responding to medical treatment due to dense adhesions. This case report is unusual as it has presented in a post-pubertal female requiring surgical management. Keywords: Labial adhesion, Labial agglutination, Labial fusion, Synechia vulvae, Surgical excision INTRODUCTION normal. On local examination of genitals, labia majora was normal and labia minora was densely and firmly Labial fusion occurs when both the lips of labia minora fused in midline. -
OEIS Complex, a Case Report
Case Report iMedPub Journals Journal of Intensive and Critical Care 2016 http://www.imedpub.com ISSN 2471-8505 Vol. 2 No. 1:4 DOI: 10.21767/2471-8505.100013 OEIS Complex, A Case Report Manassero-Morales G, Franco-Bustamante K, Matos-Rojas I Abstract National Institute of Child Health, San Borja, Introduction: The OEIS complex includes: Omphalocele, Exstrophy of the cloaca, Lima, Perú Imperforate anus, and Spinal defects. The OEIS complex affects 1 in 200,000 to 400,000 pregnancies and its etiology is uncertain. The purpose is to present our experience in this unusual coexistence of malformations never reported in Latin Corresponding author: America. Manassero-Morales G Clinical Case: A male infant, three months of age, diagnosed at birth with omphalocele, cloacal exstrophy, double hemi bladder, rectum and anal atresia, [email protected] bilateral clubfeet and lumbosacral bifid spine. Abdominal MRI confirmed clinical findings and also showed horseshoe kidney, bilateral enlarged renal pelvis. Instituto Nacional de Salud del Niño-San Borja, Lumbosacral spine MRI revealed lipomeningocele, hypoplastic sacrum and pubic Lima, Perú agenesia. In the first two days of life, this patient underwent several surgical procedures. Currently (3 months old) is waiting for further surgical reconstruction. Conclusion: The case described meets the clinical criteria for OIES complex; in the Citation: Manassero-Morales G, Franco- absence of a family history that could suggest a pattern of Mendelian inheritance Bustamante K, Matos-Rojas I .OEIS Complex, and normal cytogenetic study. We conclude that this is an isolated case of probable A Case Report. J Intensive & Crit Care 2016, multifactorial inheritance. -
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 -
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). -
Labial Adhesion: New Classification and Treatment Protocol
Original Study Labial Adhesion: New Classification and Treatment Protocol Mirzaman Huseynov MD 1,*, Ali Ekber Hakalmaz MD 2 1 Department of Pediatric Surgery, Private Safa Hospital, Istanbul, Turkey 2 Department of Pediatric Surgery, Gaziosmanpasa Public Hospital, Istanbul, Turkey abstract Study Objective: To determine the subtypes of labial adhesion (LA) and arrange treatment options accordingly. Design and Setting: Patients who presented to our clinic with LA between July 2016 and February 2018 were divided into 4 groups. Location of the adhesion area, thickness of the adhesive tissue, and response to topical steroid (betamethasone valerate 0.1% ointment) therapy were identified as common features. Participants: Seventy-five prepubertal girls. Interventions and Main Outcome Measures: To determine the subtypes of the LA and evaluate the treatment response of patients in each subtype group. Results: LA was divided into 4 subtypes according to their common characteristics. For patients with type I, 2 weeks of topical steroid treatment resulted in complete recovery (100%). For those with type II, 12 (80%) patients had complete response to topical steroid treatment for an average of 3 weeks. Type III and IV patients were completely unresponsive to topical steroid treatment. Conclusion: Classification of LA patients into subtypes and determination of treatment on the basis of this classification make a major contribution in planning the treatment of patients, not by trial-and-error, but using a predetermined strategy. Key Words: Labial adhesions, Labial agglutination, Labial adhesion management, Prepubertal Introduction to the characteristics of subtype. In this study we aimed to identify the subtypes of LA and plan treatment options Labial adhesion (LA) can be defined as fusion of the labia accordingly. -
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.............................................................................................................................................................