SEARCH · Search PubMed

Results for “Bladder Exstrophy”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

SourceResearch connectionFrom yearThrough year

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,351 records · Page 75Linked to original sources

PubMed1995

The anatomy of the pelvis in the exstrophy complex.

We compared computerized tomography scans of the pelvis of twenty-four patients who had exstrophy of the bladder with scans of age-matched controls in order to analyze the pelvic deformity that accompanies the variably severe manifestations of this condition. The patients who had classic exstrophy of the bladder were found to have a mean of 12 degrees of external rotation of the posterior aspect of the pelvis on each side, retroversion of the acetabula, a mean additional 18 degrees of external rotation and 30 per cent shortening of the pubic rami, and progressive diastasis of the symphysis pubis. The foot-progression angle demonstrated 20 to 30 degrees of external rotation beyond the normal limits seen in early childhood, but this improved with age. The patients who had exstrophy of the cloaca and the bladder not only had all of these pelvic deformities to a greater degree but also had asymmetry of measured parameters between the right and left sides of the pelvis, malformation of the sacro-iliac joints, and occasional dislocation of the hip. An understanding of the pelvic anatomy that accompanies exstrophy is essential when corrective approaches are planned. Such an understanding will improve the rate of success of both closure of the bladder and control of urinary continence postoperatively.

Adolescent↗

PubMed1993

[Cloacal exstrophy: what therapeutic approach for what result?].

The authors report 11 cases of cloacal exstrophy and analyse the anatomical features and the results of surgery. The outcome of three patients aged 20, 18 and 9 years is related. Advances in prenatal assessment of malformations is likely an explanation for the lack of new cases in the recent years. However the surgical procedure now available provides a better quality of life to patients with such a disabling malformation.

Adolescent↗

PubMed2001

Hugh Hampton Young: a pioneer in pediatric urology.

PURPOSE: Hugh Hampton Young (1870-1945) is regarded as a major force in urology during the first half of the 20th century. While he is acknowledged for many advances in open and transurethral prostatic surgery, his contributions to pediatric urology have been less well recognized. MATERIALS AND METHODS: The manuscripts and autobiography of Hugh Hampton Young were reviewed and major contributions to pediatric urology were identified. Patient records corresponding to these areas were then obtained from the Alan Mason Chesney Medical Archives and reviewed. RESULTS: Hugh Hampton Young was the first clinically to recognize and surgically treat posterior urethral valves, the first to develop a procedure to correct incontinence in patients with the epispadias-exstrophy complex and the first to describe bilateral subtotal adrenalectomy for virilization secondary to congenital adrenal hyperplasia. He was successful in his endeavors and his techniques are still in use today or have become the building blocks of future modifications. CONCLUSIONS: The contributions of Hugh Hampton Young to pediatric urology were numerous. In addition to his accomplishments in academic and adult urology, Young should be recognized as a true pioneer in pediatric urology.

Bladder Exstrophy↗

PubMed2002

A new technique for repair of exstrophy-epispadias complex.

We report a new method of repair of exstrophy-epispadias complex (EEC) at a second stage by using flaps from the skin between the penis and scrotum (the clitoris and labia majora in females) used in seven patients. The non-pigmented skin between the penis and scrotum in males or a band of skin between the clitoris and labia majora in female is mobilized in two flaps, which are rotated superiorly. Five patients were treated primarily by this method and two patients at the time of secondary reconstruction. There were six boys and one girl. Two patients were post-pubertal, one male and one female. Follow-up (6-12 months) revealed good cosmetic and functional results. If EEC is repaired in a single stage, the entire pelvic diaphragm is visualized from inside and the sphincters can be reconstructed around the bladder neck. This procedure gives better exposure of attachments of the corporal bodies to the pubic rami, allowing easier mobilization. The bulbospongiosus muscle can be reconstructed. A normal penoscrotal relation is achieved in male patients. The root of the scrotum, which is splayed out and wide, narrows. Rotation of the flaps superiorly normalizes the symphyseal area. In female patients the appearance of the mons pubis is satisfactory. In post-pubertal children the pubic hair distribution appears normal.

Bladder Exstrophy↗

PubMed1979

The exstrophy support team: a new concept in the care of the exstrophy patient.

The concept of the exstrophy support team is reported. It has extended the care of children with exstrophy into a lifelong continuum of effective assistence, not just by way of medical and surgical interaction but through knowledgeable and sympathetic coordination. There has been a spectacular improvement in over-all lifetime survival and much greater patient interest and appreciation with better followup and maintenance so that we can help quickly and effectively when problems arise. Now that we have achieved an increased longevity for these patients, we must be able to assure them the best possible quality of life.

Bladder Exstrophy↗

PubMed1982

Duplication of the müllerian ducts and genitourinary malformations. Part II: analysis of malformations.

Forty-seven females with duplication of the uterus and cervix were retrospectively studied to obtain a better understanding of the reasons for failure of fusion of the müllerian ducts by analyzing the associated genitourinary malformations. Thirty-one of the 47 patients had major genitourinary malformations that corresponded to three main complexes of anomalies. Cloacal anomalies were present in 16 patients, exstrophy in two, and combined cloacal and exstrophic deformities in another two. Renal malformations ipsilateral to a unilateral occluded müllerian duct were seen in 11 patients, and in four of these an ectopic ureter opened into the occluded duct. From the analysis of these anomalies it is proposed that duplication of the female genital system, when not an isolated event, may be secondary to interference of the normal process of müllerian fusion by a persistent cloacal duct, an abnormal pelvic girdle, or an ectopic ureter.

Abdominal Muscles↗

PubMed1986

Prenatal ultrasonic diagnosis of anterior abdominal wall defects.

Nine cases of different types of anterior abdominal wall defects were diagnosed prenatally by ultrasound: gastroschisis (one case), omphalocele (five cases), exstrophy of the cloaca (one case) and the extreme form of prune belly syndrome (two cases). The ultrasonic features of gastroschisis and omphalocele are well recognized by most experienced sonographists. The ultrasonic prenatal diagnosis of exstrophy of the cloaca has not been reported previously and it is a very rare entity. The anterior abdominal wall defect is larger than in omphalocene and is located infraumbilically. Fetal ascites and a lumbosacral myelomeningocele are present as well. The extreme form of prune belly syndrome is associated with the absence of the abdominal wall musculature and marked dilatation of the urinary tract, presented ultrasonically as multiple large cysts occupying the distended fetal abdominal cavity. The differential diagnosis of these entities and guidelines for their correct prenatal ultrasonic diagnosis will be discussed.

Abdominal Muscles↗

PubMed2005

Prenatal ultrasound diagnosis of cloacal exstrophy associated with myelocystocele complex by the 'elephant trunk-like' image and review of the literature.

A case of cloacal exstrophy (CE) was detected by ultrasound as early as 22 weeks of gestation in association with myelocystocele complex, an unusual form of occult spinal dysraphism often associated with such a disease. The ultrasonographic diagnosis was made through the detection of a wavy cord-like segment of soft tissue protruding from the anterior abdominal wall, just below the umbilical cord insertion, strongly resembling the trunk of an elephant. Our article enforces the suggestion that the ultrasound elephant trunk-like image should be added to the existing major criteria for making prenatal diagnosis of CE.

Abnormalities, Multiple↗

PubMed1999

OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins.

The omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a consistent and recognizable pattern of midline abdominal and pelvic defects. It is rare, affecting 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestations. This is an autopsy study of OEIS complex in monozygotic twins after pregnancy termination at 20 weeks of gestation. Unremarkable family history but concordance of monozygotic twins for the defects may support the theory that early malformation complexes, e.g., OEIS, and monozygotic twinning are manifestations of the same disturbance of early blastogenesis.

Abnormalities, Multiple↗

PubMed1996

Penopubic flaps technique for the repair of epispadias with or without exstrophy: a preliminary report.

Six patients (average age, 2 1/2 years; range, 4 months to 8 years) with the exstrophy-epispadias complex and isolate epispadias were treated using an alternative method. An inverted U-shaped incision (with a length of approximately 3 to 5 cm and a width of approximately 0.7 to 1.0 cm) was prepared, extending from the penopubic urethral meatus toward the umbilicus. The two incisions were extended along the urethral plate toward the ventral face of the glans. The pubic flap was prepared from the skin or scar tissue on the pubic area. The neourethra was reconstructed using penopubic flaps. A fistula occurred in one patient, which healed spontaneously. The penopubic flaps enabled us to form a wide-enough urethral tube and to perform a better dissection on the pubic area.

Bladder Exstrophy↗

PubMed1987

Exstrophy of the cloacal membrane. A pathologic study of four cases.

Exstrophy of the cloaca is a rare congenital anomaly. The authors add the pathologic findings of four distinct cases reported in this article to those reported in the literature. In all cases, common anomalies were recognized. In addition, there were rare anomalies, such as single umbilical artery, vestige of the left superior vena cava, common mesenterium, calcification of the cerebellum, incomplete segmentation of the left lung, abnormal shape of the liver, and knock-knee. The embryology of this complex anomaly is difficult. It is considered that this anomalous condition results from breakdown and mesodermal invasion of the cloacal membrane.

Abnormalities, Multiple↗

PubMed1977

Closure of a postexstrophy prostatic fistula.

A technique for closure of difficult prostatic fistulas has been used in two patients with exstrophy and epispadias. In this technique, a rotated, twisted full thickness skin flap is used. The tip of the flap forms the roof of the closed fistula and is made out of the epidermis. The pedical of the flap is buried, and therefore, the epidermis is removed, leaving only the dermal layer to be buried. Consequently, there is no tension on the fistula closure, and the chance of breakdown is vastly reduced. The long term results is these two patients has been excellent. In the older child or adult, this technique may provide the only consistent means for achieving such a closure. The technique is obviously adaptable to other situations in which a simple closure is plagued with secondary breakdown.

Adolescent↗

PubMed1989

Phallic reconstruction in cloacal exstrophy.

During the last 25 years 8 genotypic male subjects with cloacal exstrophy have undergone phallic reconstruction. All 8 patients had phallic inadequacy after completion of the reconstruction with penile lengths at or below 2 standard deviations below the mean. Of the patients 4 have reached puberty and 2 have documented physiological impotence by nocturnal penile tumescence testing. Three patients have required intensive psychiatric counseling, ranging from 18 months to 3 years because of feelings of sexual inadequacy. Of 2 patients who have attempted sexual relations 1 is unable to penetrate the vagina because of phallic size and 1 is presently engaged in successful vaginal intercourse. As a consequence of phallic inadequacy and its associated psychological trauma in these individuals we strongly recommend sexual conversion as part of the initial management plan.

Adult↗

PubMed2004

Psychosexual development in genetic males assigned female: the cloacal exstrophy experience.

Genetic males who have cloacal exstrophy, a rare, severe pelvic field defect that leads to aphallia, traditionally have been socially, legally, and surgically sex-assigned female at birth and reared female, despite a male karyotype and a typical male prenatal hormonal milieu. Psychosexual development in such children previously has been unknown. Studies of 29 children revealed that despite the absence of the typical postnatal and pubertal androgen surges and the presence of female genitalia, all female-assigned subjects displayed a marked male-typical shift in psychosocial and psychosexual development. Nearly half of them have declared themselves male. Psychosexual development, including gender identity, in genetic and hormonal male neonates seems to be influenced heavily by prenatal androgen exposure. The clinical practice of surgical sex assignment at birth requires re-evaluation.

Bladder Exstrophy↗

PubMed1984

Anatomy of the penis and its deformities in exstrophy and epispadias.

The anatomy and erectile deformities of the penis in patients with exstrophy and epispadias are described. The information was derived from cavernosograms in 17 patients, an operation in 12, a pelvic computerized tomography scan in 1 and a cystectomy for carcinoma in 1. The inferior pubic ramus and, therefore, the corpora cavernosa are parallel to the floor when the patient is standing. Although the penis loses some of its length through the separation of pubic bones the corpora are shorter than in normal men. Ten corpora were rudimentary in 6 patients, perhaps because of vascular damage during a neonatal operation. The most common erectile deformity was tight dorsal chordee owing to intrinsic curvature of the corpora. The deformity is not caused by scar tissue or shortness of the reconstructed urethra. Patients with 1 rudimentary corpus have lateral deviation on erection.

Adolescent↗

PubMed2003

Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) in triplet pregnancy after IVF and CVS.

BACKGROUND: Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare sporadic condition. CASE: We identified an infant with major malformations resembling OEIS. He was the product of a 30-week triplet pregnancy conceived by in vitro fertilization (IVF) and evaluated by chorionic villi sampling (CVS). In this article, we review the possible pathogenetic mechanisms in this case, including IVF, multiple gestation, trauma to the uterus or uterine vessels following CVS, and placenta accreta. CONCLUSIONS: We conclude that the cumulative effects of all or some of these factors may have resulted in uteroplacental insufficiency adequate to produce this phenotype. This case provides additional evidence for the uterine vascular pathogenesis of OEIS complex in humans.

Abnormalities, Multiple↗

PubMed1995

Omphalocele-exstrophy-imperforate-anus-spina bifida (OEIS) complex in a male prenatally exposed to diazepam.

A male clinically affected by the OEIS complex was studied. His mother, aged 30 years, has an affective disorder and ingested 30 mg of Diazepam daily, from 3 months previous to the gestation and during the entire pregnancy. At birth, a closure during the entire pregnancy. At birth, a closure defect of the anterior abdominal wall, exstrophy of hemibladders, exposure of intestinal epithelium, abnormal pelvis, imperforate anus, and bifid penis were noted. Birth weight was 3600 g and other measurements were not recorded. Colostomy was performed in the postnatal period followed by partial closure of the abdominal wall defect, and iliac osteotomies. At six years, 6 months of age, physical examination showed somatometric measurements around the third percentile (height 109 cm, weight 17 kg, cephalic circumference 48.5 cm). Clinically he presented mild mental retardation, functional colostomy, incomplete closure of the vesical exstrophy, imperforate anus, bifid penis and scrotum, descended testes, diastasis of pubis, lumbosacral scoliosis and shortening of the left leg (clinical photograph of the external features is not included as we were not able to obtain authorization to do so). Radiological studies (Figure 1) revealed wide separation of the ischiopubic bones; lumbosacral region with rotoscoliosis, platyspondyly and dysraphism; left coxa valga, and right coxa vara. The abdominal ultrasonographic studies showed unilateral renal agenesis (left). Chromosomal analysis (GTG bands) in peripheral blood lymphocyte cultures demonstrated a normal 46,XY constitution. Exposure to other substances, particularly alcohol, were excluded with the study of the mother's medical history and through information obtained from relatives.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Drug-Induced↗

PubMed2005

A novel approach to skin coverage for epispadias repair.

PURPOSE: Current techniques for epispadias repair have resulted in significant improvement in the reconstruction of the urethra, corpora and glans. The final challenging step is to enhance the cosmetic result by accomplishing skin coverage for the penis and subpubic area, creating a penopubic and penoscrotal angle without dorsal suture lines, and avoiding future dorsal tethering of the penis as a result of scar contraction. We report a novel technique for penile skin coverage in the patient with epispadias that results in a superior cosmetic outcome. MATERIALS AND METHODS: The technique involves creation of 2 flaps-a ventral preputial transverse island flap rotated dorsally to cover the dorsal aspect of the penile shaft, and an advancement flap from the patch of skin present between the penis and scrotum in epispadias, which is advanced distally to cover the ventral aspect of the penis. The 2 flaps are sewn to each other with 2 lateral suture lines. Thus, the dorsal and ventral aspects of the penis are covered with intact skin devoid of suture lines. RESULTS: This technique was used in 8 males 2 days to 15 years old. The epispadias was part of exstrophy in 5 patients and an isolated defect in 3. Both flaps healed well in 7 of 8 patients. In 1 exstrophy case a segment of the transverse island flap became ischemic and was discarded intraoperatively. Dorsal skin coverage in this patient was achieved using a laterally based flap from the inguinal area, which healed without problem. Followup was 6 to 33 months. No patient had development of skin tethering, curvature or recurrence of the dorsal chordee. The cosmetic appearance of the penis was subjectively superior to that of boys who underwent skin closure using reverse Byars flaps. CONCLUSIONS: The cosmetic appearance of the penis using this novel technique is superior because of the absence of the dorsal scar that may cause chordee, the development of penopubic and penoscrotal angles, which gives the penis a more normal appearance, and the absence of the redundant patch of skin between the shaft of the penis and the scrotum.

Adolescent↗