extrophy

A 58-year-old Nigerian male had open prostatectomy undertaken in a private hospital 3 months before presenting at our out-patient clinic with leakage of urine and a widening supra pubic defect of 10 weeks duration. Examination showed the posterior bladder wall and visible trigone. Subsequently the defect was closed and he remained continent at follow up.

Prenatal ultrasonographic features of patent urachus consist in anechoic proximal cord cyst communicating with the bladder associated with large umbilicus. Distinction should be made with omphalocele and bladder extrophy. Spontaneous evolution leads to rupture during mid-trimester and bladder protrusion. Karyotyping is not mandatory in isolated typical cases. Early postnatal surgery is usually required.

BACKGROUND: Role of pelvic osteotomy in surgical management of bladder exstrophy is controversial But pelvic rim closure importantly. Bulking agents have been used for perineal and pelvic dysfunction in adults. In this study, bladder extrophy repair was performed without pubic closure And bulking agent injections were used as the strut of anterior pelvis for prevention of Organ prolapse and other functions in this series. PATIENTS AND METHODS: During the period 2008-2012, twenty-five exstrophy-epispadias complex patients with a mean age of 14 months underwent surgical reconstruction...

We report the case of a 24-year old man with a past history of vesical extrophy and ureterosigmoidostomy in childhood, admitted with acute left flank pain and acute renal failure. The complaints started the day following the endoscopic resection of a sigmoid polyp. The stricture of the ureterosigmoid junction was diagnosed by intravenous urography. Conservative treatment with endo-ureteral dilatation was successfully performed. At 2 years of follow-up, the patient is still asymptomatic, without any residual hydronephrosis...

Surgical glues have been used in pediatric surgery because of the fragility of tissue, and to prevent major surgeries. The present report describes our experience with using a new cyanoacrylate Glubran 2 (Viareggio, Italy) in the treatment of five cases of tracheoesophageal atresia with fistula (one fistula protection, three recurrent fistula, and one unstable patients), two cases of hypospadias, one case of vesicutanouse fistula after bladder extrophy, and one case of cloacal extrophy from January-December 2008...

BACKGROUND: Subspecialization defined pediatric surgery using Alder Hey innovations in neonatal surgical units (Rickham) and anesthesia (Jackson-Rees). In neonatal surgery, United Kingdom subspecialization for cloacal extrophy and biliary atresia acknowledges their dependence on multidisciplinary management and the desirability of caseload for training. We phased in regional subspecialization for esophageal atresia (EA) repair and replacement surgery while trainee numbers increased nationally to reduce hours...

Authors present two cases of pregnancy and delivery of patients with bladder extrophy. Both had undergone various and different reconstructive surgical operations in childhood, which gave them the opportunity to lead a normal life, to become pregnant and deliver. A total of three deliveries are observed--one with Caesarian, one--normal vaginal delivery and one--normal delivery with breech presentation. Bladder extrophy is a rare and severe innate anomaly of the genital-urinary tract, associated with genital, urologic and orthopedic malformations...

Bladder exstrophy is rare and associated with an increased incidence of bladder cancer. Unreconstructed bladder extrophy presenting in an adult is very rare as most of the patients undergo repair in childhood. Most of the cancers are adenocarcinomas. We report a rare case of squamous cell carcinoma occurring in exstrophic unreconstructed bladder in a 58-year-old male patient.

Variant presentations of cloacal exstrophy are exceedingly rare. Historically, genetic males with cloacal extrophy were re-assigned to the female gender due to phallic inadequacy. Early recognition of intravesical phallic structures in cloacal exstrophy cases may impact gender reassignment discussions and long-term gender outcomes. We report the case of a male infant with cloacal exstrophy presenting with an intravesical phallus, review and compare the presenting anatomical features of the three previously reported cases, and discuss the potential impact of these findings on gender reassignment in these complex children...

A new less traumatic method of pubic bone approximation in extrophy of the urinary bladder in children is proposed. To ascertain efficacy of the method, results of 3-stage reconstructive plastic operations with pubic bone approximation without osteotomy (group 1, n=27), one-stage reconstructive plastic operations with transversal suturing of the anterior abdominal wall (group 2, n=19), and one-stage reconstructive plastic operations according to the new techniques of pubic bone approximation (group 3, n=9) were compared...

A case of cranioraschischisis including incomplete pentalogy of Cantrell (PC) is described. The female fetus had a large omphalocele with evisceration of the heart, left lung, liver, stomach, and intestines accompanying anencephaly, cervical, thoracal lumbar, spina bifida. The fetus had ectopia cordis and diaphragmatic agenesia with an intact sternum. We present a case of a neonate with the stigmata for PC with the exception of a sternal defect. A literature review is also included. Sonographers should check for ventral and dorsal anomalies with PC because they may occur simultaneously...

OBJECTIVE: The aim of the German Network for Congenital Uro-REctal Malformations is to collect data of affected patients with anorectal malformation (ARM) or extrophy-epispadias complex, and to investigate molecular causes, clinical implications, and psychosocial outcome. The current issue was to assess the postoperative sequelae related to lower urinary tract dysfunction in patients with ARM. MATERIALS AND METHODS: Two hundred and sixty-seven patients with ARM (112 females, 155 males, median age 6 years, range 0-56 years) were investigated via standardized case report forms comprising interview, analysis of medical data, and personal questionnaires...

BACKGROUND: Access to pediatric surgical care in many sub-Saharan African countries is strongly limited by lack of medical facilities, adequate transport system, and trained medical and nursing manpower. The mortality rate for major congenital abnormalities remains extremely elevated in this area of the world. Strong efforts have been spent during the past decades to elevate the level of pediatric surgery standards in these countries through cooperation programs acting through periodical medical missions or supporting local medical teaching institutions...

BACKGROUND: Patients who have undergone reconstruction of bladder extrophy can have an extremely short penis, which can be functionally and psychologically debilitating. Penile reconstruction with the radial forearm free flap has the potential to provide these patients with improved genital appearance and sexual function. Quality of life after penile reconstruction is an important outcome measure in evaluation of the procedure. METHODS: We describe five patients who underwent total penile reconstruction with radial forearm free flaps and interview them with a quality of life questionnaire...

The isolated bladder extrophy is a major congenital malformation which prenatal diagnosis is essential. It is based on the non-visualization of the bladder during the first trimester ultrasound but in most cases, it is confirmed by the morphological ultrasound of the second trimester. For the baby, the prognosis of life quality depends on the accurate identification of the malformation, the foetal sex and the associated pathologies. Thus, a detailed ultrasound evaluation is necessary and requires an operator experienced in the search of malformations...

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No abstract text is available yet for this article.

No abstract text is available yet for this article.

BACKGROUND: Bladder extrophy malformation includes outward rotation of the innominate bones, pubic symphysis diastasis and acetabular retrovertion. We have corrected the pelvic deformation during the reconstruction of the bladder neck and the urethra in older children. METHODS: From 1/01/2002 to 1/01/2006 in the Department of Pediatric Urology and the Department of Pediatric Orthopedics in Katowice 4 children were operated on for bladder extrophy. The age of the patients ranged from 2...

OBJECTIVE: To review the outcome of patients who underwent Mainz II pouch continent urinary diversion and assess the suitability of the technique for the treatment of bladder extrophy and irreparable VVF for use in our region of Ethiopia. PATIENTS AND METHODS: Between Jan, 2004 to Nov. 30, 2008, 13 patients had continent urinary diversion for bladder extrophy epispadia complex and irreparable VVF in Mekelle hospital. All underwent Mainz II pouch urinary diversion...