Four cases of bladder exstrophy in two families.
نویسندگان
چکیده
Bladder exstrophy is a rare congenital anomaly, caused by abnormal development of the cloacal membrane. To our knowledge, 18 familial patients with this malformation have been described. Two sets of familial cases with bladder exstrophy are reported here: two cousins and a mother and son and the published reports of the 18 familial cases among 682 index patients with bladder exstrophy are reviewed. Ultrasonography is advocated as the investigation of choice for early prenatal diagnosis.
منابع مشابه
P-12: Long-Term Follow-Up (18–35 Years) of Male Patients with History of Bladder Exstrophy (BE) Repair in Childhood: Erectile Function and Fertility Potential Outcome
Background Bladder exstrophy is a rare condition that may lead to severe psychosexual malformation and require a lifelong follow-up. Aim. We describe the long-term sexual outcome of patients with bladder exstrophy treated at our institution at early stage. MaterialsAndMethods Thirty patients with mean age of 26 years (range 18–35 years) were included in the study. Fifteen patients underwent sta...
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OBJECTIVE To identify genetic and nongenetic factors contributing to the risk of bladder exstrophy-epispadias complex (BEEC). PATIENTS AND METHODS In all, 285 families with BEEC were invited to participate in the study, and 232 of them were recruited. Epidemiological information was obtained from 151 of the consenting families, with a detailed clinical genetic examination of 94 probands. In a...
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Introduction: Bladder Exstrophy (BE) is a congenital anomaly in which the bladder and abdominal wall and external genitalia are pathologically open. Incidence of this anomaly is one in 50000. Treatment of this anomaly is complicated and, the old method of treatment was pelvic osteotomy. The aim of this study was offering a new surgical approach, which is closure of the bladder in bladder ex...
متن کاملStaged Management of Bladder Exstrophy
Bladder exstrophy or as an erroneously known as ectopia vesica is a rare congenital malformation of the lower part of the abdomen and the genital area. It involves the urinary bladder, abdominal wall, pubic bones and the external genitalia. It requires functional reconstruction, which is often done as a single or in multiple stages; the aim of management is to preserve kidney functions, maintai...
متن کاملOur initial experience with the technique of complete primary repair for bladder exstrophy.
OBJECTIVE We reviewed our initial results with complete primary repair of exstrophy in regard to continence status and the need for subsequent continence procedures. PATIENTS AND METHODS We performed a retrospective review of our surgical records from 1996 to 2008 to identify all patients with bladder exstrophy managed at our center. RESULTS Sixteen children were closed successfully. Six pa...
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عنوان ژورنال:
- Journal of medical genetics
دوره 31 6 شماره
صفحات -
تاریخ انتشار 1994