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https://www.readbyqxmd.com/read/28924534/image-of-the-month-clinical-features-in-a-newborn-with-covered-cloacal-exstrophy
#1
Alejandra Vilanova-Sánchez, Christina B Ching, Alessandra C Gasior, Karen Diefenbach, Richard J Wood, Marc Levitt
Cloacal exstrophy is the most severe type of anorectal malformations that belongs to the bladder-exstrophy-epispadias complex of genitourinary malformations. Interestingly, its variant, the covered cloacal exstrophy, is often missed. The clinical findings of this variant may include an imperforate anus, low lying umbilicus, thick pubic bone, and pubic diastasis but with an intact abdominal wall. We present an interesting case of covered cloacal exstrophy with a side-by-side duplicated bladder and discuss important considerations for the time of colostomy creation in the newborn period...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28920029/exstrophy-bladder-with-low-anorectal-malformation-a-rare-association
#2
Aditya Pratap Singh, Vinay Mathur, Ramesh Tanger, Arun Kumar Gupta
No abstract text is available yet for this article.
July 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28893435/gradual-bone-transfer-for-the-correction-of-the-pubic-diastasis-using-the-ilizarov-technique-in-closure-of-bladder-and-cloacal-exstrophy
#3
Hiroshi Kusakabe, Katsuhiko Ueoka, Shinichiro Takayama, Atsuhito Seki
BACKGROUND: In the patient of the cloacal exstrophy, cloaca with local abdominal wall is disrupted and exposed inner surface of the bladder needs early closure. Pelvic osteotomies are required for severe cases whose bladder cannot be closed by the suture of soft tissue only. We developed a technique involving the gradual positioning of bone fragments using a light, Ilizarov external fixator. The usefulness of the technique was assessed. METHODS: We enrolled 3 patients with cloacal exstrophy and 1 with bladder exstrophy as a gradual transfer group and 6 patients who were treated by other osteotomies as a control group...
September 8, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28892921/a-rare-case-of-genital-malformation-with-omphalocele-exstrophy-of-bladder-imperforate-anus-and-spinal-defect-complex-autopsy-findings
#4
K Mamatha, B R Yelikar, Varsha R Deshpande, B S Disha
Omphalocele, Exstrophy of cloaca, Imperforate anus and Spinal defects (OEIS) is a severe manifestation of exstrophy-epispadias sequence with a combination of defects including OEIS. It results from improper closure of anterior abdominal wall and defective development of cloaca and urogenital septum due to defect in blastogenesis during the 4(th) week of gestation. Identification of this complex is important through foetal autopsy as this condition can recur in siblings. Prenatal diagnosis also helps to prevent foetal death with appropriate management in the less severe cases...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28890277/-prenatal-diagnosis-of-abdominal-wall-defects
#5
H El Mhabrech, H Ben Hmida, H Charfi, A Zrig, C Hafsa
Anterior abdominal wall defects (AAWD) correspond to a wide spectrum of congenital defects affecting 6.3/10,000 pregnancies. They have in common a closure defect of the anterior abdominal wall and can be fatal or expose the fetus and the neonate (NN) to many complications. This study was based on a retrospective study of 22 cases of AAWD collected between May 2009 and December 2014. Its purpose was to specify the importance of prenatal ultrasonography in the diagnosis and prognosis assessment of these defects...
September 7, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28888708/ureteric-urethral-engraftment-as-a-new-surgical-technique-for-management-of-incontinence-in-bladder-exstrophy-complex-a-retrospective-cohort
#6
Abdol-Mohammad Kajbafzadeh, Shabnam Sabetkish, Nastaran Sabetkish
OBJECTIVES: To report the results of a novel surgical technique for achieving urinary continence in patients with bladder exstrophy complex (BEC) by ureteric-urethral engraftment (UUE) technique. PATIENTS AND METHODS: Sixteen female patients with BEC and a mean ± SD age of 3.48 ± 1.75 years were referred for primary exstrophy repair from 2009 to 2012. From these, 9 patients were operated by single-stage bladder closure (group I); while 7 patients underwent the novel technique of UUE to compare the continence achievement (group II)...
September 6, 2017: International Journal of Surgery
https://www.readbyqxmd.com/read/28868232/penile-reconstruction-with-skin-grafts-and-dermal-matrices-indications-and-management
#7
Paloma Triana Junco, Mariela Dore, Vanesa Nuñez Cerezo, Javier Jimenez Gomez, Miriam Miguel Ferrero, Mercedes Díaz González, Pedro Lopez-Pereira, Juan Carlos Lopez-Gutierrez
Introduction  The penis eventually needs specific cutaneous coverage in the context of reconstructive procedures following trauma or congenital anomalies. Local flaps are the first choice but are not always available after multiple previous procedures. In these cases, skin graft and dermal matrices should be considered. Materials and Methods  This study was a retrospective review of the past 4 years of four patients with severe loss of penile shaft skin who underwent skin reconstruction. Dermal matrices and skin grafts were utilized...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28861290/mystery-and-realities-of-phalloplasty-a-systematic-review
#8
REVIEW
Selçuk Sarıkaya, David John Ralph
The neophallus creation is still a mystery and it remains challenging even today. In this article, we performed a comprehensive review of the literature regarding phalloplasty and penile reconstructive surgery between January 2008 and May 2016. In this review, we have included 15 research articles and the results of 276 patients were examined. Studies revealed several indications and when indications were reviewed, 191 patients were female-to-male transgender, 9 patients had disorder of sex development/micropenis, 16 had penile amputation/trauma, 9 had ambiguus genitalia, 40 had exstrophy and/or epispadias, 11 had other problems...
September 2017: Turkish Journal of Urology
https://www.readbyqxmd.com/read/28833084/pregnancy-outcomes-among-patients-with-prior-bladder-exstrophy
#9
Matthieu Dap, Olivier Larmure, Olivier Morel, Jean-Louis Lemelle
Bladder exstrophy is a congenital urogenital anomaly and improved health care of children with bladder exstrophy can facilitate pregnancy during adult life. The present retrospective study included female patients who had bladder exstrophy diagnosed at birth and who presented with a pregnancy at two hospitals (university hospitals in Nancy and Strasbourg) between January 1, 2000, and December 31, 2016; there were no exclusion criteria applied. Patients were identified from hospital databases. Owing to the study design, consent and formal ethical approval were not required...
August 21, 2017: International Journal of Gynaecology and Obstetrics
https://www.readbyqxmd.com/read/28815980/sexuality-in-men-with-exstrophy
#10
EDITORIAL
Christopher R J Woodhouse
No abstract text is available yet for this article.
September 2017: BJU International
https://www.readbyqxmd.com/read/28810634/misdiagnosis-of-a-cloacal-exstrophy-variant-as-urorectal-septum-malformation-in-a-fetus-by-ultrasound-a-case-report
#11
Yang-Qing Xu, Xiao-Hong Yang, Xin-Lin Chen, Xiu-Qiin Ji, Sheng Zhao
Cloacal exstrophy variants are comprised of a wide range of characteristics, of which there are four primary features, including omphalocele, bladder exstrophy, an imperforate anus and spina bifida. The existing literature regarding the differential diagnosis from alternative urinary diseases prenatally are limited. If the bladder is present, defects in the ventral wall may not be visualized with prenatal ultrasound in certain conditions, including oligohydramnios, and differential diagnosis from urorectal septum malformation sequence is a challenge...
August 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28794909/role-of-the-lf-sine-derived-distal-isl1-enhancer-in-patients-with-classic-bladder-exstrophy
#12
Rong Zhang, Michael Knapp, Franziska Kause, Heiko Reutter, Michael Ludwig
A genome-wide association study and meta-analysis identified ISL1 as the first genome-wide significant susceptibility gene for classic bladder exstrophy (CBE). A short interspersed repetitive element (SINE), first detected in lobe-finned fishes (LF-SINE), was shown to drive Isl1 expression in embryonic mouse genital eminence. Hence, we assumed this enhancer a conclusive target for mutations associated with CBE formation and analyzed a cohort of 200 CBE patients. Although we identified two enhancer variants in five CBE patients, their clinical significance seems unlikely, implying that sequence variants in the ISL1 LF-SINE enhancer are not frequently associated with CBE...
September 2017: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/28771836/editorial-comment-to-lessons-learned-from-the-management-of-adults-who-have-undergone-augmentation-for-spina-bifida-and-bladder-exstrophy-incidence-and-management-of-the-non-lethal-complications-of-bladder-augmentation
#13
Kazuyoshi Johnin
No abstract text is available yet for this article.
August 3, 2017: International Journal of Urology: Official Journal of the Japanese Urological Association
https://www.readbyqxmd.com/read/28771821/lessons-learned-from-the-management-of-adults-who-have-undergone-augmentation-for-spina-bifida-and-bladder-exstrophy-incidence-and-management-of-the-non-lethal-complications-of-bladder-augmentation
#14
REVIEW
Douglas A Husmann
The present review provides clinical insights and makes recommendations regarding patient management garnered by the long-term follow up of patients undergoing enteric bladder augmentation for the management of congenital anomalies. A prospectively maintained database on 385 patients that have experienced an enteric bladder augmentation, using either the ileum or colon, was reviewed. Evaluations included methods used to prevent bladder calculi formation and recurrence, the incidence and etiology of renal calculi development, the incidence and treatment of vitamin B12 deficiency, and the complications and need for surgical revisions for continent catheterizable stomas...
August 3, 2017: International Journal of Urology: Official Journal of the Japanese Urological Association
https://www.readbyqxmd.com/read/28768624/pseudoexstrophy-a-forme-fruste-of-bladder-exstrophy
#15
Edmond Wright, Edwin Harmon, Michael Steiner, Michael Nowicki
No abstract text is available yet for this article.
August 2, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28721463/exstrophy-bladder-reconstruction-or-diversion-for-the-underprivileged
#16
REVIEW
Yogesh Kumar Sarin, Virender Sekhon
The surgical techniques for management of bladder exstrophy epispadias complex have evolved from staged reconstruction, complete primary repair to radical mobilization. Post-operative complications add to the multiplicity of surgical procedures at each step. The end results are variable with many achieving continence rates of 85-89% only after bladder augmentation and clean intermittent catheterization. The situation is further complicated in resource-poor settings, where illiteracy and poverty are the driving factors for choosing a single operative procedure for creation of low pressure reservoir aiming at upper tract preservation and good primary continence...
July 18, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28694586/duplicate-bladder-exstrophy-with-hypospadias-a-rare-exstrophy-variant-with-unusual-association
#17
Bibekanand Jindal, K Lalatendu Kumar, Sadasivan Jagdish
No abstract text is available yet for this article.
July 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28687949/controversies-of-sex-re-assignment-in-genetic-males-with-congenital-inadequacy-of-the-penis
#18
Venkatachalam Raveenthiran
Sex assignment in 46XY genetic male children with congenital inadequacy of the penis (CIP) is controversial. Traditionally, children with penile length less than 2 cm at birth are considered unsuitable to be raised as males. They are typically re-assigned to female-sex and feminizing genitoplasty is usually done in infancy. However, the concept of cerebral androgen imprinting has caused paradigm shift in the philosophy of sex re-assignment. Masculinization of the brain, rather than length of the penis, is the modern criterion of sex re-assignment in CIP...
July 8, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28666504/pregnancy-in-a-treated-case-of-ectopia-vesicae
#19
Fazeela Shahzad, Shazia Khalid Khan, Madiha Siraj
The patient was born with bladder exstrophy and underwent multiple surgeries for its correction. Finally, she had ureterosigmoidostomy at the age of 9 years, which ultimately led her to live till reproductive age. The children born with this defect are capable of surviving till adult life. She was received by us with preterm labour, referred from Hafizabad. She also had hyperchloremic metabolic acidosis and mild hydronephrosis. She was managed with multidisciplinary, modified care but the pregnancy ended up in an emergency cesarean section due to non-reactive cardiotocograph and persistently reduced fetal movements...
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28652161/the-surgical-management-of-bladder-polyps-in-the-setting-of-exstrophy-epispadias-complex
#20
John Jayman, Ali Tourchi, Mahsa Shabaninia, Mahir Maruf, Heather DiCarlo, John P Gearhart
OBJECTIVE: To investigate the surgical course for treating bladder polyps in patients with exstrophy epispadias complex (EEC). EEC bladder polyps are unique pathologic entities, with a distinct difference between polyps discovered at birth and polyps developed after failed exstrophy closure. METHODS: A prospectively maintained database of 1300 patients with EEC was reviewed for bladder exstrophy patients with bladder polyps. The following data were obtained: patient demographics, polyp type, past medical and surgical history, and continence outcomes...
June 23, 2017: Urology
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