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PubMed1993

Complications of paraexstrophy skin flaps in the reconstruction of classical bladder exstrophy.

We reviewed the cases of the exstrophy/epispadias complex treated at our institution between July 1976 and April 1992. A total of 78 patients was identified who had paraexstrophy skin flaps used in the bladder closure, of whom 31 (40%) had a complication as a result of the flaps. The main complication encountered was a urethral stricture where the paraexstrophy skin flaps joined the urethral plate area. Multiple maneuvers were undertaken to correct these problems, including direct vision internal urethrotomy (12 cases), multiple urethral dilations (4), open revision (3) and full thickness skin grafts (5). Seven patients had such a complex stricture situation that they required either continent urinary diversion (5), colon conduit diversion (1) or cutaneous ureterostomy (1), the latter 2 patients having undergone vesicostomy elsewhere before referral. Of the remaining 24 patients who did not undergo a diversionary procedure 12 have undergone an epispadias repair and bladder neck reconstruction, 7 underwent an epispadias repair and 5 await further treatment. Freedom from complications in the initial closure of exstrophy significantly improves the chances of successful reconstruction. The avoidance of problems leading to obstruction, infection, hydronephrosis and reflux nephropathy will provide better kidneys regardless of bladder suitability for function or augmentation. Our use of paraexstrophy flaps has decreased but when they are required, special care in design, placement and followup is advised to avoid complicating strictures and their sequelae.

Bladder Exstrophy↗
PubMed1999

Exstrophy of the bladder.

Exstrophy of the bladder is a rare congenital defect that occurs when the abdominal wall and underlying structures, including the ventral wall of the bladder, fail to fuse in utero. As a result, the lower urinary tract is exposed, and the everted bladder appears through the abdominal opening. Various surgical interventions have been employed with variable success in the hope of achieving complete dryness, full control over delivery of urine, freedom from catheters and external appliances, and a protected upper urinary tract. The most popular surgical approach is the primary bladder closure with secondary bladder neck reconstruction. Comprehensive nursing, medical, and surgical care are necessary to preserve renal and sexual function. The many complex problems experienced by these infants and their families call for a multidisciplinary approach. This article reviews occurrence, clinical presentation, and management of exstrophy of the bladder.

Bladder Exstrophy↗
PubMed1977

Exstrophy of the bladder.

Exstrophy of the bladder is the most common of a spectrum of related anomalies. The pelvic skeletal changes in all of these disorders vary in degree but are similar in type. The kidneys are urinary collecting systems are usually normal in patients with untreated exstrophy, and the "hurley" appearance of the lower ureters should be regarded as a physiologically normal variant.

Bladder Exstrophy↗
PubMed1985

Exstrophy of bladder: evolution of management.

Eighty-nine patients with bladder exstrophy were seen at our institution over the last fifty years. There were 63 males and 26 females. Cloacal exstrophy constituted 9 per cent of our experience. Twenty-seven patients underwent primary urinary diversion with subsequent genital reconstruction early on in our series. Of the 57 children operated on since 1951, 50 were judged eligible for and underwent a planned multistaged reconstruction. We realized a 50 per cent success rate. The majority of failures were diverted into an ileal conduit for persistent incontinence.

Asia↗
PubMed1974

Exstrophy of the bladder and associated anomalies.

Bladder exstrophy is a rare malformation of the urogenital tract. Its incidence is about one per 25,000-40,000 births, with a two to one ratio of males over females. No hereditary factors have been established, and it has been stated in the literature repeatedly that this defect does not occur with an increased frequency in the same family. However, Higgins reports bladder exstrophy in twins on two occasions, and twice in brothers and sisters.

Adult↗
PubMed1978

Exstrophy of the bladder: treatment by trigonosigmoidostomy--long-term results.

Twenty-five patients with bladder exstrophy were treated by implanting the vesical trigone into the sigmoid colon. Twenty-three have been followed for 3 to 19 years, with an average of 10 years. Taking into account the clinical status, renal function, the intravenous urogram, continence and social life, 18 patients were considered to have had an excellent result. There were 2 failures; 1 post-operative death occurred due to ruptured oesophageal varices and 1 patient developed bilateral hydronephrosis which necessitated cutaneous urinary diversion. In 4 cases the results, while satisfactory, were only fair in that 2 developed stones and 2 showed symptomatic electrolyte disturbances requiring treatment. Trigonosigmoidostomy preserves intact the ureterovesical junction which prevents colo-ureteric reflux and this probably accounts for the generally excellent long-term results. We believe the operation is preferable to ureterosigmoidostomy in the management of bladder exstrophy.

Adolescent↗
PubMed1999

Bilateral posterior pelvic resection osteotomies in patients with exstrophy of the bladder.

This paper describes a modification of bilateral posterior iliac osteotomies for bladder exstrophy, in which a strip of ilium is resected subperiosteally lateral to the sacroiliac joints, allowing easier anterior closure with less breakdown compared with traditional osteotomies. Thirty-one children underwent repair of bladder exstrophy between 1974 and 1994. Orthopaedic procedures included: closed reduction and cast application in the newborn period (four patients), classic bilateral posterior iliac osteotomies (12 patients), and bilateral posterior resection osteotomies (15 patients). Dehiscence occurred after one closed reduction, five classic osteotomies, and one resection osteotomy. Urinary continence was obtained in four patients who underwent closed reduction, nine patients who underwent classic posterior osteotomies, and nine patients who underwent posterior resection osteotomies.

Bladder Exstrophy↗
PubMed1976

Exstrophy of the bladder--an alternative method of management.

Sixteen bladder exstrophy patients were treated by diverting the urine into a non-refluxing colon conduit. There were 4 new, previously unoperated cases and 12 had been diverted earlier by ileal conduit. Subsequently, 11 conduits were detached from the abdominal wall and joined end-to-side to the rectosigmoid colon. This method has proved satisfactory to date but long-term assessment will be needed. This staged method of ureterosigmoid urinary diversion appears to be a satisfactory way to manage new patients with bladder exstrophy and patients who have been diverted previously by an ileal conduit. From our experience with non-refluxing colon conduits we conclude also that it is: 1) a better method than ileal loop for permanent diversion, 2) indicated for patients with an ileal loop who are not doing well and 3) useful in patients undergoing anterior exenteration, providing the option of joining it to the rectosigmoid if there is no recurrent disease or radiation proctitis.

Adult↗
PubMed1985

Cloacal exstrophy and related abdominal wall defects in Manitoba: incidence and demographic factors.

A retrospective descriptive study of omphalocele and exstrophic defects of the abdominal wall was carried out in Manitoba to determine the prevalence of these defects, changes in birth incidence with time and their etiologic heterogeneity. The incidence of isolated omphalocele was similar to that reported in other series but the overall incidence of other types of abdominal wall defects was high. Cloacal exstrophy, bladder exstrophy and amnion disruption defects contributed significantly to the number of cases ascertained and emphasized the heterogeneity of abdominal wall defects. There were fluctuations in birth incidence with time with cloacal exstrophy especially apparently being more common in 1980-81. No specific demographic or environmental factors were documented in the cases seen in 1980-81 which could explain the high incidence in these years. Cloacal exstrophy is, however, now appreciated to be much more common than first realized. Studied directed at elucidation of the causes of apparent changes in incidence of malformations such as omphalocele or exstrophic defects will have to take into account both the fluctuations in background incidence of these defects and the considerable diversity in their etiology.

Abdominal Muscles↗
PubMed1996

Embryonic development of the ventral body wall and its congenital malformations.

Congenital abdominal wall defects, frequently associated with other anomalies, are found in many forms. Consequently, there is still controversy in the literature concerning nomenclature, classification, and pathogenesis. Recently, we proposed a new nomenclature and classification of abdominal wall defects based on the early development of the umbilical cord and of the ventral body wall. According to this classification the complete spectrum of abdominal wall defects, including cloacal exstrophy, bladder exstrophy, and epispadias, can be subdivided into four types: primary (thoraco-)abdominoschisis, omphalocele, body wall dysplasia, and secondary (thoraco-)abdominoschisis. Each type is characterized by its specific configuration of the placenta, the membranes, the umbilical cord, and the fetus. Anomalies such as urachal remnants and omphalomesenteric duct malformations can be explained by disturbances during later stages of umbilical cord development.

Abdominal Muscles↗
PubMed2004

Another case of autosomal dominant exstrophy of the bladder.

OBJECTIVE: Exstrophy of the bladder is a rare malformation due to an anterior midline defect. Most cases of this condition with variable expression occur sporadically, but there are some cases indicative of a strong genetic component apart from environmental factors. This is a report about another rare mother-child pair with bladder exstrophy. METHODS: We present the clinical data of a familial case of bladder exstrophy with an affected mother and her equally affected male fetus. RESULTS: Prenatal diagnosis of bladder exstrophy in the fetus was assessed by ultrasound at the 19th gestational week and was confirmed after termination of pregnancy at the 21st gestational week. CONCLUSION: The present case may be additional evidence for an autosomal dominant inherited variant of this malformation complex with implication for counselling of affected patients.

Abortion, Induced↗