Extrophy anomalies

INTRODUCTION: Patients with spina bifida and other spinal dysraphisms commonly suffer from fecal incontinence and constipation, which can be treated with antegrade continence enemas. Currently, information regarding outcomes and satisfaction in children who have Chait cecostomy tubes is lacking. The aim of our study was to evaluate the effectiveness of Chait cecostomy tubes in management of constipation in children with spinal dysraphisms. MATERIALS AND METHODS: A questionnaire was completed by patients and/or their families during office visits at the University of Iowa or Nationwide Children's Hospital during follow-up pediatric urology office visits...

BACKGROUND/PURPOSE: The aims of surgical management in cloacal exstrophy (CE) have shifted to optimizing outcomes and quality of life while minimizing morbidity. This report reviews the single-institution experience of complications of bladder closure in CE. METHODS: Patients with CE were identified from a prospectively-maintained bladder exstrophy-epispadias complex database. Operative and follow-up data were analyzed to compare complications and failure rates of bladder closure between closures performed with and without osteotomy and primary versus reoperative closures...

During the period of 1961-1975 in the Child Surgery Clinic of Leningrad Medical Institute of Pediatrics reparative-plastic operations for the urinary bladder extrophy were performed in 39 patients. Most of children were operated upon at the age under 1 year. In 36 patients plastic reconstruction of the urinary bladder, sphincter and urethra was performed with local tissues after the Young technic in the G. A. Bairov modification. Late results of surgical treatment were studied in 26 patients within the terms from 1 to 14 years...

UNLABELLED: Epispadias associated or not to bladder extrophy is a congenital malformation with a difficult surgical correction. Mitchell published his technique in 1996, and it's based in the complete dissection of the uretheral band, ventral peneal skin and both corporas including their proximal insertion, that in next steps will be separated in all the length. These manoeuvres permit to: 1) Correct the dorsal incurvation; 2) Locate the urethra in a ventral position; 3) Locate the meatus at the tip of the penis with a vertical orientation...

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...

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...

OBJECTIVES: To assess the role of correct anatomical reconfiguration of the anterior perineal musculature in exstrophy-epispadias (E-E) patients. To stress the use of a bipolar stimulator to detect the perineal muscular complex intraoperatively, and to increase the functional results of reconstruction in E-E patients. METHODS: A total of 22 patients with E-E complex were treated in a 7-year period: 17 patients presenting classic bladder extrophy (aged 3 days to 6 years) and 5 incontinent male epispadias (aged 9 months to 16 years)...

Bilateral posterior iliac osteotomy as a preliminary stage in the surgical treatment of congenital extrophy of the bladder allows easier approximation of the soft tissues in front of the urethra and facilitates closure of the rectal muscles and fasciae, reducing or abolishing the need for using fascial strips and carrying out plastic procedures to close the skin.

Although vesical extrophy is a well-known anomaly, the variants of vesical extrophy are exceptional. We present the case of a newborn child, who upon exploration, revealed a defect of closure in the infra-umbilical abdominal wall through which vesical mucous membrane protruded. A probe introduced into the urethra appeared externally at the place of the infraumbilical defect. Following the diagnosis of superior vesical fissure, resection of the protruding vesical mucous membrane and closure were carried out...

A study of the development of hereditary anorectal malformations in pig embryos resulted in major corrections of all current theories concerning normal and abnormal anorectal development. The principal event in anorectal development proved to be a shift of the dorsal part of the cloaca and the adjacent gut to the body surface of the tail groove. Regression of the dorsal part of the cloacal membrane forms an essential part in this process. Agenesis of this part to the membrane blocks the normal shift of the anorectum of the body surface with anorectal malformation as the result...

Congenital anomalies of structures originating from Mullerian ducts are important gynecological problem. They often coexist with malformations of urinary tract. In this report we describe the case of 31 year old women with didelphus and bladder extrophy. The surgical treatment consisted on transabdominal total hysterectomy due to endometriosis IV (rAFS) narrowing ureteroenterostomy.

Bladder exstrophy-epispadias complex is a rare spectrum of congenital anomalies that includes a midline abdominal wall defect and a widened pelvis with an anterior diastasis. Our patient was involved in a motorcycle accident with severe multiple injuries and concomitant bladder extrophy. In a unique and urgent clinical setting, his congenital pubic diastasis was initially misdiagnosed as a traumatic finding. A 21-year-old man presented with pelvic and extremity injuries following a motorcycle accident. Multiple fractures in the lower and upper extremities were diagnosed...

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...

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...

Salvage surgical procedures after failed reconstruction for an extrophy-epispadias complex are extremely challenging. The goals are to restore continence and improve aesthetic appearance in order to provide quality of life and an improved body image to the patient. We describe the surgical steps in an adult patient who presented anal urinary incontinence and a poor body image due to the absence of an umbilicus and the presence of hypertrophic scars. He underwent a modified Mainz II reconstruction of the lower urinary tract at childhood for an extrophy-epispadias complex...

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...

A personal series of 42 consecutive unselected bladder extrophies over a 20-year period is critically analyzed. The incidence is calculated as 1 in 25,000 live births. Four died unoperated of multiple anomalies and two died following surgery. Of the remaining 36, cosmetic closure was possible in 34. Most closures were done at about 1 year, though 1 month of age is now preferred. Iliac osteotomy has been abandoned. Further staged surgery using Young-Dees bladderneck reconstruction and ureteric reimplant high in the bladder has been used in 11 selected cases...

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...

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A 4 year old boy, who underwent 2 times of unsuccessful primary closure of the extrophied bladder at 7 days and 5 months after birth, was treated with construction of a rectal bladder with sigmoid-pull through by Duhamel procedure. Vas deference, seminal vesicles and prostate were not found at the cystectomy operation. Epispadias urethral mucosa was removed and phalloplasty was performed using the ventral hooded foreskin. The results were cosmetically and functionally excellent. He could keep continence and his upper urinary tract was normal at 6 months after the operation...