The Exstrophy-epispadias complex

Exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations ranging in severity from epispadias (E) to classical bladder exstrophy (CEB) and exstrophy of the cloaca (EC). Depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. Prevalence at birth for the whole spectrum is reported at 1/10,000, ranging from 1/30,000 for CEB to 1/200,000 for EC, with an overall greater proportion of affected males. EEC is characterized by a visible defect of the lower abdominal wall, either with an evaginated bladder plate (CEB), or with an open urethral plate in males or a cleft in females (E). In CE, two exstrophied hemibladders, as well as omphalocele, an imperforate anus and spinal defects, can be seen after birth. EEC results from mechanical disruption or enlargement of the cloacal membrane; the timing of the rupture determines the severity of the malformation. The underlying cause remains unknown: both genetic and environmental factors are likely to play a role in the etiology of EEC. Diagnosis at birth is made on the basis of the clinical presentation but EEC may be detected prenatally by ultrasound from repeated non-visualization of a normally filled fetal bladder. Counseling should be provided to parents but, due to a favorable outcome, termination of the pregnancy is no longer recommended. Management is primarily surgical, with the main aims of obtaining secure abdominal wall closure, achieving urinary continence with preservation of renal function, and, finally, adequate cosmetic and functional genital reconstruction. Several methods for bladder reconstruction with creation of an outlet resistance during the newborn period are favored worldwide. Removal of the bladder template with complete urinary diversion to a rectal reservoir can be an alternative. After reconstructive surgery of the bladder, continence rates of about 80% are expected during childhood. Additional surgery might be needed to optimize bladder storage and emptying function. In cases of final reconstruction failure, urinary diversion should be undertaken. In puberty, genital and reproductive function are important issues. Psychosocial and psychosexual outcome depend on long-term multidisciplinary care to facilitate an adequate quality of life

Multivariate analysis of factors affecting survival in pelvic exenteration

Of 153 patients with primary or recurrent pelvic malignancy referred for consideration of exenteration, only 40.6% (62 patients) were found to be suitable for exenteration after full assessment. Thirty percent (46 patients) were found to be inoperable on examination under anesthesia. Of the remaining 107 patients, 33% (35 patients) were found to be inoperable at laparotomy, 9% (10 patients) underwent radical hysterectomy and 58% (62 patients) had an exenterative procedure. One patient had no active disease found on final histologic review of the exenteration surgical specimen and was excluded, as the aim of this study was to look at the prognostic factors affecting survival. There remained 61 patients in the exenteration group who were analyzed. The 2-year survival rate was less than 2% for patients with inoperable disease, 48% for patients who underwent radical hysterectomy and 54.1% for patients who underwent exenteration. The 5-year survival rate for all patients undergoing exenteration for pelvic malignancies was 44% and that for cervical cancer only was 52%. Multivariate analysis of patients who had undergone exenteration showed four significantly poor prognostic factors influencing survival. They were: (a) aged older than 69 years, (b) recurrence of the tumor within 3 years, (c) persistent recurrence, and (d) positive resection margins.link_to_subscribed_fulltex