Bladder exstrophy: reconstructed female patients achieving normal pregnancy and delivering normal babies

PURPOSE: Bladder exstrophy (BE) is an anterior midline defect that causes a series of genitourinary and muscular malformations, which demands surgical intervention for correction. Women with BE are fertile and able to have children without this disease. The purpose of this study is to assess the sexual function and quality of life of women treated for BE. MATERIALS AND METHODS: All patients in our institution treated for BE from 1987 to 2007 were recruited to answer a questionnaire about their quality of life and pregnancies. RESULTS: Fourteen women were submitted to surgical treatment for BE and had 22 pregnancies during the studied period. From those, 17 pregnancies (77.2 percent) resulted in healthy babies, while four patients (18.1 percent) had a spontaneous abortion due to genital prolapse, and there was one case (4.7 percent) of death due to a pneumopathy one week after delivery. There was also one case (5.8 percent) of premature birth without greater repercussions. During pregnancy, three patients (21.4 percent) had urinary tract infections and one patient (7.14 percent) presented urinary retention. After delivery, three patients (21.4 percent) presented temporary urinary incontinence; one patient (7.14 percent) had a vesicocutaneous fistula and seven patients (50 percent) had genital prolapsed. All patients confirmed to have achieved urinary continence, a regular sexual life and normal pregnancies. All patients got married and pregnant older than the general population. CONCLUSIONS: BE is a severe condition that demands medical and family assistance. Nevertheless, it is possible for the bearers of this condition to have a satisfactory and productive lifestyle.

Unusual manifestations of VACTERL association.

We report a case of VACTERL association along with unusual manifestations of pseudo-exostrophy of bladder, hemifacial microsomia and an urachal cyst communicating with the bladder.

Cas rare de syndrome polymalformatif chez un nouveau-ne

Les auteurs rapportent un cas rare de syndrome polymalformatif constitue de laparoschisis; d'extrophie vesicale; d'hermaphroditisme et de bride amniotique micromelie des jambes. La prise en charge est impossible a cause du debut tardif des consultations prenatales (C.P.N.) et une echographie pratiquee pendant le travail apres la rupture des membranes

Extrofia vesical: tratamiento de casos complicados por operaciones previas / Vesical extrophy: treatment of complicated cases by previous surgical procedures

En el tratamiento de la extrofia vesical ocurren resultados poco satisfactorios; en forma invariable, complicaciones como reproducción extrófica, prolapso de la mucosa vesical e incontinencia urinaria, que resultan al tratar de recontruir esta malformación congénita. Em la actualidad, la extrofia vesical representa un gran reto para el cirujano y para el urólogo pediátra. De marzo a septiembre de 1992 se reconstruyeron quirúrgicamente con muy buen éxito 10 casos de extrofia vesical, que ya antes habían sido operados, basados en algunos principio de osteotomía anterior para facilitar: a) cierre de la pared abdominal anterior, b) plastía de genitales; c) cierre del anillo pélvico, y d) capacidad vesical adecuada, mediante alguna forma de cistoplastía de aumento