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Staged pelvic closure of extreme pubic diastasis in the exstrophy-epispadias complex.

PURPOSE: Staged pelvic closure has been shown to be beneficial in achieving pubic approximation in children with the exstrophy-epispadias complex. We have continued to use this procedure in children with extreme pelvic diastasis and have updated our experience. MATERIALS AND METHODS: We performed closure of the pelvis in 15 patients 12 months to 14 years old presenting with a pubic diastasis of at least 8 cm (range 8 to 16). Of the patients 14 had cloacal exstrophy and 1 had classic exstrophy. Two patients with cloacal exstrophy and the patient with classic exstrophy had prior complete dehiscence at primary closure, and 1 patient with cloacal exstrophy had partial dehiscence. The technique involved bilateral innominate and vertical iliac osteotomy, and placement of a bony fixator with interfragmentary pins. The fixator was gradually closed, and soft tissue and pelvic ring closure occurred 2 to 3 weeks later. In 9 of the 15 patients an interpubic stainless steel plate was used to keep the pubis in apposition at the time of bladder closure. RESULTS: At a mean followup of 5.5 years (range 6 months to 14 years) closure was successful in all 15 patients. One patient (age 12 months) had loosening of the pin between stages, which was salvaged with pin replacement. One patient had ureteral obstruction from hematoma after pin placement without direct pressure from the pins or bone. None of the patients had dehiscence or prolapse. CONCLUSIONS: This technique of staged pelvic closure can be used in the setting of primary or secondary pelvic reconstruction in patients presenting with extreme pelvic diastasis. The gradual reduction in diastasis allows gradual stretching of the soft tissues. It converts a major reconstruction challenge into 2 well tolerated components. However, the procedure is not recommended in children younger than 1 year due to the possibility of the pins loosening during diastasis reduction.

Adolescent↗

Treatment of adult female epispadias without exstrophy in the presence of rhabdosphincter function.

Female epispadias without exstrophy is a very rare entity. Symptoms of female epispadias are primary urinary incontinence and anatomical abnormal features. A 24-year-old married women with two children presented with primary urinary incontinence. Due to previous failure of correct diagnosis and ineffective medical treatment, the patient developed psychological problems (anxiety and depression). In the presence of minimal rhabdosphincter function we could achieve socially acceptable urinary continence by bladder neck plication and a Burch colposuspension. The patient did not consent to a major reconstruction.

Adult↗

Prenatal diagnosis of exstrophy of the cloaca.

While screening for neural tub defects, three cases of exstrophy of the cloaca were found through elevated second-trimester maternal serum alphafetoprotein levels. Measurement of amniotic fluid alphafetoprotein and examination of the structure of rapidly adhering cells confirmed the abnormalities, and the pregnancies were terminated. Subsequent studies of cholinesterase isozymes by acrylamide electrophoresis showed an abnormal acetylcholinesterase band in each of the three amniotic fluids. During the same period, two more cases of exstrophy of the cloaca were identified in spontaneous abortions and another one in a stillbirth. It is suggested that the condition is more common than has been previously suspected and is often misclassified as omphalocele.

Acetylcholinesterase↗

Application of memory metallic stents to urinary tract disorders in pediatric patients.

The use of memory metallic stents for the urinary tract in pediatric patients has not been reported. The authors report on 2 patients with urinary tract disorders who were successfully treated with a memory metallic stent. A thermoexpandable, nickel-titanium alloy stent was placed at the urethroureteral junction of a 4-year-old boy with ureteral stenosis associated with cloacal exstrophy for 18 months and at the urethra of a 2-year-old girl with ischuria after a repair of cloacal anomaly for 6 months. Temporary insertion of a memory metallic stent is a safe and effective alternative for organic stricture or functional obstruction of the urinary tract in pediatric patients.

Abnormalities, Multiple↗

Genital outflow tract obstruction in an adolescent with cloacal exstrophy.

The combination of congenital anomalies in patients with cloacal exstrophy presents a difficult management problem. Experience with these problems is limited because of the infrequent occurrence of the disorder and because few patients survive to adulthood. The present paper describes a 14-year-old female who presented with hyperchloremic metabolic acidosis, secondary to retained urine in a redundant ileal conduit, and symptoms of müllerian outflow tract obstruction. The literature is reviewed on genetic females with cloacal exstrophy, revealing a high incidence of müllerian fusion defects. Data are presented showing that women with cloacal exstrophy have a high potential incidence of genital outflow tract obstruction. These patients must be followed closely during adolescence so that diagnosis and treatment may be initiated early.

Abnormalities, Multiple↗

[The effect of enterocystoplasty in childhood on linear growth].

OBJECTIVE: The assumption that enterocystoplasty in children has a detrimental effect on linear growth has been based almost exclusively upon a chance finding in a retrospective study 10 years ago. We re-evaluated the same research question in a larger cohort and with a longer follow-up. PATIENTS AND METHODS: Between 1982 and 1997, 242 children and adolescents underwent enterocystoplasty. Patients with conditions involving organ systems apart from the urinary tract, and those with myelomeningocele, malignant diseases, reduced glomerular filtration rate and incomplete notes were excluded. In the definitive study cohort (123; mean age at operation 8.6 years; mean age at investigation 16.8 years), enterocystoplasty had been undertaken using colon in 70, ileum in 37, a combination of both in 11, ileocaecal segments in three and stomach in two patients. RESULTS: In all, 1215 height and weight measurements had been recorded. The distribution of percentile positions before and after enterocystoplasty showed a normal configuration, with 83 % and 80 % of patients growing within two standard deviations of the 50th percentile. After surgery, 85 % either remained the same or reached a higher percentile. Nineteen (15.5 %) were in a lower position, with a similar tendency in the weight percentile. A clinically relevant growth disorder was recognized in four patients with a complete endocrinological evaluation; in none of these was enterocystoplasty thought to be a causal factor. CONCLUSIONS: It is very unlikely that loss of the preoperative percentile position on the growth curve in 15 % of children after enterocystoplasty is a consequence of that particular surgery. Rather, it is a non-specific phenomenon that has to be considered in any clinical population of the same size and age distribution after the same length of time.

Adolescent↗

[Prenatal diagnosis of pelvic floor malformations].

Pelvic floor malformations are congenital defects in the pelvic and perineal area, which are derived from a defective development of the cloaca membrane, the uro-rectal septum, the mesonephric and para-mesonephric ducts and the urogenital sinus. These are complex anomalies with a profound emotional impact on parents. The prenatal diagnosis is possible favoring the prenatal counseling for the parents and expediting the post-natal evaluation and management. Aim of the present paper is to review the current criteria for the prenatal diagnosis of the following malformations: anorectal anomalies, cloacal anomalies, persistence of the urogenital sinus, bladder and cloacal exstrophy and ambiguous genitalia.

Anal Canal↗

Double discontinuous lipomyelomeningocele: CT findings.

A 3-year-old girl with repaired cloacal exstrophy and two skin-covered dorsal lumbosacral masses was documented to have two lipomyelomeningoceles at noncontiguous levels of a tethered spinal cord. The clinical, radiographic, myelographic, CT, and surgical appearances are presented to illustrate the features of this extremely rare anomaly.

Bladder Exstrophy↗

Complete phalloplasty using the free radial forearm flap for correcting micropenis associated with vesical exstrophy.

PURPOSE: We present a new surgical technique for reconstructing the penis in a man with micropenis associated with vesical exstrophy. MATERIALS AND METHODS: A free radial forearm flap was used to create a penis of normal length and diameter. The flap was wrapped around the native micropenis. A penile prosthesis was then inserted in the flap to provide erection. RESULTS: The flap was well vascularized and no skin damage was observed 6 years after reconstruction. The patient achieved sexual intercourse on a regular basis. He is satisfied with the result. CONCLUSIONS: Free transfer of the radial forearm flap may be done in select men with micropenis associated with vesical exstrophy for penile reconstruction. An inflatable prosthesis may be inserted in the flap to provide erection. The results of this technique have remained stable in the long term. This method provides a new tool for phalloplasty in these difficult cases.

Adult↗

Covered exstrophy and visceral sequestration: a rare exstrophic variant.

We report a case of covered exstrophy and sequestration of a segment of bowel on the surface of the abdomen, which is a rare variant of the exstrophic complex. The external genitalia and urinary continence were normal. Simple excision of the ectopic bowel was curative.

Abnormalities, Multiple↗

Ileocecal ureterosigmoidostomy: an alternative to conventional ureterosigmoidostomy.

We describe a 1-stage procedure that involves use of the ileocecal segment as an intervening urine conduit to the large bowel to achieve a continent diversion. The ureters are anastomosed end to end to the terminal ileum that is intussuscepted into the cecum. The cecum then is joined to the lower sigmoid by an end-to-side anastomosis. Mixed urine and feces are eliminated through the rectum. The results in 5 patients with exstrophy and 1 with epispadias between 5 months and 13 years old are reported. Ureteral reflux was not observed. Urinary tract infection developed in 2 patients. Ileocecal ureterosigmoidostomy is a reasonable alternative to intact ureterosigmoidostomy that may reduce the risk of development of cancer.

Adolescent↗

Cloacal exstrophy.

Although each patient with cloacal exstrophy must be considered as a unique individual and much controversy still remains about the treatment of this complex syndrome, our review of the literature suggests that the previously detailed series of stages in the approach to this patient represents a conservative, individualized, yet successful approach.

Bladder Exstrophy↗

Duplicated exstrophy: report of three cases.

Three cases of duplicate exstrophy are described and the theoretical mechanisms of embryogenesis discussed. In all 3 cases there was classic pelvic skeletal deformity. Urinary control was normal in 2 of the 3 patients. The surgical approach consisted of plastic repair of the abdominal wall defect in all patients and correction of urinary incontinence in 1 patient.

Bladder Exstrophy↗

Pseudoexstrophy associated with megalourethra.

Pseudoexstrophy is a rare exstrophy variant that has musculoskeletal stigmata of the exstrophy complex without any associated defect in the urinary system. The authors report a case of pseudoexstrophy with scaphoid megalourethra, and anorectal malformation. Kidneys and ureters were normal. Preliminary surgery consisted of a divided high sigmoid colostomy. The rare combination of pseudoexstrophy and megalourethra is discussed.

Abnormalities, Multiple↗

The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): recurrence in sibs.

The OEIS complex comprises a combination of defects including omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects. It may represent the most severe manifestation of a spectrum of birth defects, the exstrophy-epispadias sequence. The OEIS complex affects 1 in 200,000 to 400,000 pregnancies and is of unknown cause. The purpose of the current report is to document the occurrence of OEIS in sibs from separate pregnancies and suggest that some cases may have a genetic basis.

Abnormalities, Multiple↗

Delayed development of the scrotum in exstrophy.

Early attempts at orchiopexy should be avoided in children with exstrophy, since the testes will often descend spontaneously by puberty. In many cases the scrotum will grow much larger and will become pendulous by puberty. In a patient with a rudimentary scrotum orchiopexy will only result in fixing the testes in an abnormally high position owing to the lack of a sac into which to place them. If the scrotum develops later the testicles will probably be unable to descend further. Scrotal skin should not be used for urethroplasty operations in children with exstrophy and special techniques should be applied if it is necessary to repair hernias in these patients. In the child with partially descended testes but with only a small patch of wrinkled scrotum efforts may be undertaken to increase scrotal size by topical hormone treatment (or by injections) before the onset of puberty to help fertility and emotional development.

Adult↗

Surgery to the external genitalia.

Surgery to the external genitalia is one of the most challenging chapters of reconstructive urology, due to the need to correct complex problems such as hypospadias or epispadias (isolated or associated to exstrophies). Although this specialty has shown a continuous technical advancement there is no consensus as to the most efficient surgical procedure or to the best strategy to treat these pathologies. The papers reviewed here reflect these statements and reinforce the idea that surgeons' good sense, allied with their experience in the area, are the factors that define the choice of the ideal surgical option for each case.

Adolescent↗