keyword
https://read.qxmd.com/read/37898815/course-of-an-unplanned-and-unexpected-pregnancy-in-a-39%C3%A2-year-old-patient-with-complex-bladder-extrophy-a-case-report
#1
JOURNAL ARTICLE
Susanne Schrey-Petersen, Martin Lacher, Holger Stepan
BACKGROUND: With improved operative techniques pregnancy rates have been rising in patients with anomalies of the extrophy-epispadias-complex, including also female patients with bladder extrophy. Specific risks around pregnancy need to be addressed sufficiently beforehand. CASE PRESENTATION: An unplanned pregnancy was detected at 34 weeks in a 39-year old White female patient with former complex bladder extrophy. Decades after her operation she had not received any follow-up medical care and believed to be unable to conceive due to her anomaly...
October 29, 2023: Journal of Medical Case Reports
https://read.qxmd.com/read/36091541/birth-defects-data-from-hospital-based-birth-defect-surveillance-in-guilin-china-2018-2020
#2
JOURNAL ARTICLE
Xingdi Yang, Jianjuan Zeng, Yiping Gu, Yiming Fang, Caiyun Wei, Shengkui Tan, Xiaoying Zhang
Objectives: Birth defects (BDs) are a major contributor to perinatal and infant mortality, morbidity and lifelong disability worldwide. A hospital-based study on birth defects was designed in Guilin city in the Guangxi province of Southwestern China aiming to determine the prevalence of BDs in the studied region, and the classify the BDs based on clinical presentation and causation. Methods: The study involved BDs among all pregnancy outcomes (live births, stillbirths, death within 7 days, and pregnancy terminations) born in the 42 registered hospitals of Guilin between 2018 and 2020...
2022: Frontiers in Public Health
https://read.qxmd.com/read/35594790/a-rare-presentation-of-oeis-variant-with-a-recto-bladder-neck-fistula-a-case-report-and-literature-review
#3
JOURNAL ARTICLE
Athari AlShammari, Waleed Burhamah, Amar Alnaqi
INTRODUCTION: Omphalocele, bladder extrophy, imperforate anus and spinal defect (known as OEIS) is a very rare congenital anomaly with an unknown etiology. In this report we describe a case of an OEIS variant associated with a wide pubic diastasis, bladder extrophy with a recto-bladder neck fistula and a high ano-rectal malformation. This work has been reported in line with the SCARE 2020 criteria. PRESENTATION OF THE CASE: A 30-year-old mother delivered a male baby at 39 weeks through a normal vaginal delivery...
May 4, 2022: International Journal of Surgery Case Reports
https://read.qxmd.com/read/34962003/two-new-patients-with-focal-dermal-hypoplasia-a-novel-porcn-variant-and-insights-on-the-diagnostic-considerations
#4
JOURNAL ARTICLE
Rasha Moheb Elhossini, Mohamed S Abdel-Hamid, Engy Ashaat, Ghada A Otaify, Heba Dawoud, Khalid Elshimy, Mona El Ruby, Mona Aglan
Mutations in the PORCN gene cause an X-linked dominant condition; focal dermal hypoplasia (FDH), characterized by atrophic skin, pigmented skin lesions in addition to several ocular and skeletal malformations. FDH is rare with around 275 cases reported so far from diverse ethnic groups. Herein, we provide a report of two new patients with FDH from Egypt. In addition to the typical clinical manifestations of the disease, infrequently reported clinical findings in the form of broad metaphysis, bilateral short broad femurs, and dermal sinus over the sacrum were seen in Patient 1 and partial fusion of labia majora, ventral hernia, and bladder extrophy were present in Patient 2...
March 2022: Congenital Anomalies
https://read.qxmd.com/read/34717273/adenocarcinoma-mucinosum-of-extrophy-bladder-a-rare-case-report
#5
JOURNAL ARTICLE
Agus Rizal A H Hamid, Chaidir Arif Mochtar, Lisnawati, Meilania Saraswati, Sahat Matondang, Muhammad Isa Fuad Affan
INTRODUCTION: Bladder exstrophy is a rare congenital anomaly while, bladder adenocarcinoma mucinous type is a rare type of bladder cancer, with aggressive behavior and inadequate response to radiation and chemotherapy. In extremely rare cases, untreated bladder exstrophy could transform into bladder mucinous adenocarcinoma. CASE PRESENTATION: We report a case of a 41-year-old male with untreated bladder exstrophy that transformed into mucinous adenocarcinoma. The patient also had epispadias and a right inguinal hernia...
October 14, 2021: International Journal of Surgery Case Reports
https://read.qxmd.com/read/33144535/-high-risk-pregnancy-in-patients-with-extrophy-epispadia-complex-uro-gynecological-characteristics-and-management
#6
JOURNAL ARTICLE
Yesica Quiroz, Erika Llorens, Rosa Novoa, Guilherme Motta, Elisa Llurba, Oriol Porta, Juan Parra, Iara Da Silva, Joan Palou, Ana Bujons
The exstrophy-epispadias complex (EEC) ranges from distal epispadias to cloacal exstrophy, with serious repercussions on the quality of life of patients. However, reconstructive surgery offers the opportunity to reach adulthood and consider motherhood.The objective of this work is to assess the uro-gynecological characteristics and the risks that pregnant women have with EEC. MATERIALS AND METHOD: Retrospective study of 50 patients diagnosed with EEC and treated in a reference center for this pathology, born between 1968 and 2000...
November 2020: Archivos Españoles de Urología
https://read.qxmd.com/read/32513370/isolated-female-epispadias-with-urinary-incontinence
#7
Shahzad Ali, Hira Moosa, Saeed Ahmed Khan
Isolated female epispadias without bladder extrophy is a rare variant of female urethral anomaly reported rarely in the literature. For optimal and early management, early clinical diagnosis is important. Female with isolated epispadias usually presents with primary urinary incontinence with abnormal looking external genitalia on examination. We present a case of a 23-year female with urinary incontinence with bifid clitoris and widely separated labia minora on genital examination. Reconstructive surgery of urethra, labia minora and bifid clitoris was performed in one stage...
April 2020: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://read.qxmd.com/read/31214313/spontaneous-rupture-of-continent-urinary-reservoir-with-extrophy-epispadias-complex
#8
Richard B Knight, Benjamin Thomas, Alexios Tsiotras, Matthew Kasprenski
Spontaneous rupture of a continent urinary reservoir is a rare, but clinically important event. The diagnosis of reservoir rupture should be considered in any patient with peritonitis and a history of continent urinary diversion. A pouchogram may confirm the diagnosis, but ultimately a laparotomy is mandatory in the setting of peritonitis and sepsis. Catheter drainage alone has been reportedly successful for patients who meet certain criteria. This case highlights the key steps in evaluation and management of a ruptured continent urinary reservoir...
June 2019: Journal of Surgical Case Reports
https://read.qxmd.com/read/30534833/multidisciplinary-approach-of-assessing-malformed-fetuses-exemplified-in-a-rare-case-of-pentalogy-of-cantrell-associated-with-craniorachischisis-pulmonary-extrophy-and-right-sided-aortic-arch-with-aberrant-brachiocephalic-artery
#9
JOURNAL ARTICLE
Camelia Albu, Adelina Staicu, Roxana Tania Popa-Stănilă, Alina Camelia Milaş, Liviu Bogdan Chiriac, Tünde Elisabeta Kovács, Vasile Florin Stamatian
We present the imagistic and pathological assessment of a unique case of complete pentalogy of Cantrell associated with craniorachischisis, pulmonary extrophy and right-sided aortic arch with aberrant brachiocephalic artery. For this particular case, the complete and correct diagnosis required a post-mortem imagistic high-resolution magnetic resonance imaging (MRI) at 7 T and detailed stereomicroscopic autopsy. Also, we discussed the pathogenesis and possible etiology of pentalogy of Cantrell and the associated malformations of the case presented...
2018: Romanian Journal of Morphology and Embryology
https://read.qxmd.com/read/30458829/massive-localized-lymphedema-in-an-unreported-location-retroperitoneum
#10
JOURNAL ARTICLE
Dilek Ertoy Baydar
BACKGROUND: Massive localized lymphedema (MLL) is a non-neoplastic benign soft tissue lesion that may be confused with sarcomas or other neoplastic proliferations both clinically and morphologically. Most occur in morbidly obese adults on the lower extremities. The objective of this article is to document a case of MLL in the retroperitoneal cavity which is a previously unreported site for this lesion, and to highlight its unusual clinical features. CASE PRESENTATION: The patient was a non-obese male who had undergone major abdominal surgery due to bladder extrophy 17 years ago...
November 20, 2018: Diagnostic Pathology
https://read.qxmd.com/read/29941348/the-intravesical-phallus-in-patients-with-cloacal-exstrophy-an-embryologic-conundrum
#11
JOURNAL ARTICLE
Karl Benz, Mahir Maruf, Clark Hatheway, Matthew Kasprenski, John Jayman, Steven Docimo, Francis Schneck, John Gearhart
BACKGROUND: Phalluses present inside the extrophied bladder of cloacal exstrophy (CE) newborns have been sporadically reported in the literature; this clinical entity has largely unknown origins and may represent an extremely rare anomaly of CE. OBJECTIVE: Along with nearly doubling the number of reported intravesical phalluses in the literature, this study aims to outline the common anatomic features and discuss the implications for theories of CE embryogenesis...
June 8, 2018: Journal of Pediatric Urology
https://read.qxmd.com/read/29704585/the-role-of-human-acellular-dermis-in-preventing-fistulas-after-bladder-neck-transection-in-the-exstrophy-epispadias-complex
#12
JOURNAL ARTICLE
Karl S Benz, John Jayman, Mahir Maruf, Gregory Joice, Matthew Kasprenski, Nikolai Sopko, Heather Di Carlo, John P Gearhart
OBJECTIVE: To evaluate human acellular dermis (HAD) as an adjunct during bladder neck transection (BNT) by comparing surgical outcomes with other types of tissue interposition. METHODS: A prospectively maintained institutional database of exstrophy-epispadias complex (EEC) patients was reviewed for those who underwent a BNT with at least 6 months follow-up. The primary outcome was the occurrence of BNT-related fistulas. RESULTS: In total, 147 EEC patients underwent a BNT with a mean follow-up time of 6...
July 2018: Urology
https://read.qxmd.com/read/27138190/cnv-analysis-in-169-patients-with-bladder-exstrophy-epispadias-complex
#13
JOURNAL ARTICLE
Catharina von Lowtzow, Andrea Hofmann, Rong Zhang, Florian Marsch, Anne-Karoline Ebert, Wolfgang Rösch, Raimund Stein, Thomas M Boemers, Karin Hirsch, Carlo Marcelis, Wouter F J Feitz, Alfredo Brusco, Nicola Migone, Massimo Di Grazia, Susanne Moebus, Markus M Nöthen, Heiko Reutter, Michael Ludwig, Markus Draaken
BACKGROUND: The bladder exstrophy-epispadias complex (BEEC) represents the severe end of the congenital uro-rectal malformation spectrum. Initial studies have implicated rare copy number variations (CNVs), including recurrent duplications of chromosomal region 22q11.21, in BEEC etiology. METHODS: To detect further CNVs, array analysis was performed in 169 BEEC patients. Prior to inclusion, 22q11.21 duplications were excluded using multiplex ligation-dependent probe amplification...
April 30, 2016: BMC Medical Genetics
https://read.qxmd.com/read/26419040/-the-method-of-colpopoiesis-in-an-urinary-bladder-extrophy
#14
JOURNAL ARTICLE
V L Dronova
Complex of extrophy--epispadias--a rare inborn malformation of genitourinary system, abdominal wall muscles and pelvic bones. The observation was adduced, concerning an urinary bladder extrophy in a girl in combination with vaginal agenesia after noneffective primarily--postponed closure of urinary bladder, osteotomy (in the age of 4 yrs old), with consequent ureterosygmostomy. In the patient there was conducted a reconstructive operation for vagina creation, using nonfunctioning bladder, right-sided nephrectomy for severe ureterohydronephrosis, reconstruction of anterior abdominal wall and external genitalia...
May 2015: Klinichna Khirurhiia
https://read.qxmd.com/read/25914851/intermittent-hyperammonemic-encephalopathy-after-ureterosigmoidostomy-spontaneous-onset-in-the-absence-of-hepatic-failure
#15
Wolfgang Jäger, Anne-Odette Viertmann, Claudia Janßen, Frank Birklein, Joachim W Thüroff, Raimund Stein
Intermittent hyperammonemic encephalopathy after ureterosigmoidostomy is a rare, but if unrecognized, potentially lethal condition. Ureterosigmoidostomy was performed in a male patient with bladder extrophy. After 35 years, he developed hyperammonemic encephalopathy. Diagnostic procedures did not reveal hepatic nor metabolic disorders. Despite administration of preventive medical treatment, several episodes recurred. A durable prevention was finally achieved by conversion into an ileal conduit. Intermittent hyperammonemic encephalopathy can occur decades after ureterosigmoidostomy...
2015: Central European Journal of Urology
https://read.qxmd.com/read/25698887/cyclopia-a-rare-condition-with-unusual-presentation-a-case-report
#16
Ghassan Sa Salama, Mahmoud Af Kaabneh, Mohamed K Al-Raqad, Ibrahim Mh Al-Abdallah, Ayoub Ga Shakkoury, Ruba Aa Halaseh
INTRODUCTION: Cyclopia (alobar holoprosencephaly) (OMIM% 236100) is a rare and lethal complex human malformation, resulting from incomplete cleavage of prosencephalon into right and left hemispheres occurring between the 18th and the 28th day of gestation. Holoprosencephaly occurs in 1/16,000 live births, and 1/250 during embryogenesis. Approximately 1.05 in 100,000 births are identified as infants with cyclopia, including stillbirths. Cyclopia typically presents with a median single eye or a partially divided eye in a single orbit, absent nose, and a proboscis above the eye...
2015: Clinical Medicine Insights. Pediatrics
https://read.qxmd.com/read/25408817/surgical-management-of-complete-penile-duplication-accompanied-by-multiple-anomalies
#17
JOURNAL ARTICLE
Irfan Karaca, Erdal Turk, A Basak Ucan, Derya Yayla, Gulcin Itirli, Derya Ercal
Diphallus (penile duplication) is very rare and seen once every 5.5 million births. It can be isolated, but is usually accompanied by other congenital anomalies. Previous studies have reported many concurrent anomalies, such as bladder extrophy, cloacal extrophy, duplicated bladder, scrotal abnormalities, hypospadias, separated symphysis pubis, intestinal anomalies and imperforate anus; no penile duplication case accompanied by omphalocele has been reported. We present the surgical management of a patient with multiple anomalies, including complete penile duplication, hypo-gastric omphalocele and extrophic rectal duplication...
September 2014: Canadian Urological Association Journal
https://read.qxmd.com/read/25372631/embryonic-origin-and-compartmental-organization-of-the-external-genitalia
#18
JOURNAL ARTICLE
Ana M Herrera, Martin J Cohn
Genital malformations occur at a high frequency in humans, affecting ~1:250 live births. The molecular mechanisms of external genital development are beginning to be identified; however, the origin of cells that give rise to external genitalia is unknown. Here we use cell lineage analysis to show that the genital tubercle, the precursor of the penis and clitoris, arises from two populations of progenitor cells that originate at the lateral edges of the embryo, at the level of the posterior hindlimb buds and anterior tail...
November 5, 2014: Scientific Reports
https://read.qxmd.com/read/24917746/bilateral-ureteral-tapering-and-secondary-ureteroneocystostomy-for-late-stenosis-in-a-patient-with-bladder-extrophy
#19
Emanuela Altobelli, Alfredo M Bove, Federico Sergi, Maurizio Buscarini
OBJECTIVE: To report a case of severe hydronephrosis 20 years after bladder exstrophy (BE) repair, managed by bilateral ureteral tapering and secondary ureteroneocystostomy. CASE PRESENTATION: A 21-year-old woman with a history of BE and ureteral reimplantation, presented with hematuria-dysuria syndrome and recurrent febrile urinary tract infections. After counselling, she elected to undergo bilateral ureteral tapering and second ureteroneocystostomy. CONCLUSION: Hydronephrosis secondary to ureteralvesical stricture in BE patients can be successfully managed with ureteral reimplantation associated to ureteral tapering even after a prior reimplantation...
February 2013: Current Urology
https://read.qxmd.com/read/24669676/bladder-exstrophy-management-at-tikur-anbesa-university-hospital-addis-ababa-ethiopia
#20
JOURNAL ARTICLE
Belachew Dejene
BACKGROUND: Bladder exstrophy is a rare congenital malformation which presents a defect of anterior lower mid abdominal wall, with anteriorly deficient bladder wall, widening of symphysis pubis, and epispadias. Management includes single or multiple stage repairs and urinary diversion procedures aimed at preservation of renal function, maintenance of continence to urine, normal appearing external genitalia and satisfactory sexual function. OBJECTIVE: To review the experience of Bladder extrophy management at Tikur Anbessa University Hospital, Addis Ababa, Ethiopia, in five years...
July 2013: Ethiopian Medical Journal
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