1/22. prenatal diagnosis of bladder exstrophy: what counseling?PURPOSE: bladder exstrophy has recently become one of the several congenital malformations detectable prenatally by fetal ultrasonography, which implies the necessity of a correct prognostic evaluation of quality of life on which therapeutic termination of pregnancy may depend within the temporal limits stated by the law. While maintaining a preeminently informative role, prenatal counseling has gained increasing relevance for the quality of life long-term prognosis in adults affected by bladder exstrophy, particularly in regard to urinary continence, and sexual and reproductive problems in male and female individuals. We report a case of bladder exstrophy diagnosed at 22 week of gestation, and comprehensively review the literature on prenatal diagnostic tools and procedures as well as long-term followup in such cases. MATERIALS AND methods: At week 22 of gestation a woman underwent prognostic counseling due to the diagnosis of fetal bladder exstrophy made by prenatal ultrasound. After counseling the parents elected pregnancy termination. In addition, literature data were analyzed in regard to quality of life and bladder exstrophy. RESULTS: We believe that the rarity of the prenatal diagnosis of this abnormality has resulted in the lack of a comprehensive review of necessary counseling criteria. CONCLUSIONS: We suggest what we consider to be a valid counseling approach for parents after a prenatal diagnosis of bladder exstrophy.- - - - - - - - - - ranking = 1keywords = pregnancy, gestation (Clic here for more details about this article) |
2/22. OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins.The omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a consistent and recognizable pattern of midline abdominal and pelvic defects. It is rare, affecting 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestations. This is an autopsy study of OEIS complex in monozygotic twins after pregnancy termination at 20 weeks of gestation. Unremarkable family history but concordance of monozygotic twins for the defects may support the theory that early malformation complexes, e.g., OEIS, and monozygotic twinning are manifestations of the same disturbance of early blastogenesis.- - - - - - - - - - ranking = 0.71082555793906keywords = pregnancy, gestation (Clic here for more details about this article) |
3/22. uterine prolapse associated with bladder exstrophy: surgical management and subsequent pregnancy.Congenital bladder exstrophy affects 1 in 125,000 to 250,000 females. Consisting of absence of the anterior abdominal wall with exposure of the ureteral orifices, failure of pubic symphysis fusion, and deficient anterior pelvic diaphragm musculature, bladder exstrophy is frequently associated with genital prolapse. pregnancy may be complicated by recurrent urinary tract infections, preterm labor, mild procidentia, and malpresentation. Due to the rarity of the condition, there is a corresponding scarcity of obstetric literature regarding management during pregnancy. We report the case of a young woman with surgically repaired bladder exstrophy who developed genital prolapse. The uterus was suspended using a sacral colpopexy utilizing a Gore-Tex graft. Subsequently, the patient became pregnant and delivered a healthy male infant at 35 weeks' gestation via cesarean section (without recurrence of the genital prolapse postpartum). Sacral colpopexy to correct genital prolapse associated with bladder exstrophy may preserve fertility in young patients.- - - - - - - - - - ranking = 1.6566977682438keywords = pregnancy, gestation (Clic here for more details about this article) |
4/22. bladder exstrophy: gynecological and obstetrical characteristics with reference to three cases.The authors report three cases of pregnancy in women treated for bladder exstrophy. Based on a review of the literature, and the follow-up of these cases, the aim of this study was to determine the prognosis of pregnancy, which is currently possible due to the progress in antibiotherapy and surgery. Nevertheless, these pregnancies need to be carefully followed-up, not only because of the complications that may occur to the mother and the infant, but also because of the type of delivery involved.- - - - - - - - - - ranking = 0.57834888412188keywords = pregnancy (Clic here for more details about this article) |
5/22. Antenatally diagnosed cloacal exstrophy variant with intravesical phallus in a twin pregnancy.We report a rare case of covered cloacal exstrophy variant with a hemiphallus trapped within partially closed bladder halves. The persistence of the cloacal membrane until at least 18 weeks' gestation, confirmed by antenatal ultrasound scanning, is discordant with existing theories of embryogenesis of cloacal exstrophy. The clinical presentation highlights the need for careful assessment, before and during surgery, to obtain a complete understanding of the anatomic defect before gender assignment and appropriate reconstructive efforts. A multispecialty approach and antenatal counseling are important, especially when only one fetus of twins has major birth defects.- - - - - - - - - - ranking = 1.3675233261828keywords = pregnancy, gestation (Clic here for more details about this article) |
6/22. Twin pregnancy achieved through TESE in an adult male exstrophy.bladder exstrophy is a rare anomaly, it compromises bladder functions, and in males it occurs with an impairment of reproductive functions, because of erectile and ejaculatory deficit. Advancements in the surgical treatment of bladder exstrophy have allowed an improvement of the bladder functions while spontaneous conception is still impaired. This is a case report of a pregnancy and subsequent birth of twins following testicular sperm extraction, on a man born with classical bladder exstrophy with infertility due to an ejaculation.- - - - - - - - - - ranking = 1.4458722103047keywords = pregnancy (Clic here for more details about this article) |
7/22. Spontaneous twin gestation after vaginal dilation in a woman with uterus didelphys and bladder exstrophy.BACKGROUND: bladder exstrophy is an uncommon anomaly rarely associated with uterus didelphys. Fertility is limited by associated vaginal malformations, which have traditionally required surgical reconstruction. CASE: A woman with a history of bladder exstrophy and hypoplastic vagina presented complaining of painful intercourse. The patient underwent vaginal dilator treatment after corrective surgery for the bladder defect. Without any further fertility therapy she subsequently conceived twins, with one implantation in each horn of a didelphic uterus. CONCLUSION: bladder exstrophy, uterus didelphys, and vaginal hypoplasia share a common embryological devel-opment, and the finding of one anomaly can alert to the presence of another. Vaginal dilators may be used in place of surgical reconstruction to allow conception.- - - - - - - - - - ranking = 0.84330223175624keywords = gestation (Clic here for more details about this article) |
8/22. Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) in triplet pregnancy after IVF and CVS.BACKGROUND: Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare sporadic condition. CASE: We identified an infant with major malformations resembling OEIS. He was the product of a 30-week triplet pregnancy conceived by in vitro fertilization (IVF) and evaluated by chorionic villi sampling (CVS). In this article, we review the possible pathogenetic mechanisms in this case, including IVF, multiple gestation, trauma to the uterus or uterine vessels following CVS, and placenta accreta. CONCLUSIONS: We conclude that the cumulative effects of all or some of these factors may have resulted in uteroplacental insufficiency adequate to produce this phenotype. This case provides additional evidence for the uterine vascular pathogenesis of OEIS complex in humans.- - - - - - - - - - ranking = 1.6566977682438keywords = pregnancy, gestation (Clic here for more details about this article) |
9/22. Cloacal exstrophy: prenatal diagnosis before rupture of the cloacal membrane.Embryologically, cloacal exstrophy is thought to result from persistence and subsequent rupture of the infraumbilical cloacal membrane during the fifth embryonic week. We report a case of cloacal exstrophy in which a prenatal diagnosis was made prior to rupture of the cloacal membrane. A routine ultrasound at 17 weeks' gestation demonstrated monoamniotic twins. One twin was normal, but the other was found to have a sacral myelomeningocele, "rocker-bottom" feet, splaying of the pubic rami, and a large cystic mass protruding from the infraumbilical anterior abdominal wall. A repeat ultrasound was performed at 22 weeks, with the same findings. At 26 weeks, further examination showed disappearance of the abdominal cyst, a small omphalocele, no demonstrable bladder, and the suggestion of prolapsed bowel inferior to the umbilical cord insertion. After delivery at 34 weeks, the abnormal twin was found to have the typical findings of cloacal exstrophy, myelomeningocele, bilateral lower limb anomalies, and extremely foreshortened small bowel. rupture of the presumed cloacal membrane after 22 weeks in this case is inconsistent with our current understanding of the embryology of this anomaly, and should stimulate a reexamination of the current concepts. If the characteristic features are recognized, cloacal exstrophy can be diagnosed by prenatal ultrasound, permitting prenatal counseling and appropriate perinatal management.- - - - - - - - - - ranking = 0.21082555793906keywords = gestation (Clic here for more details about this article) |
10/22. pregnancy in a couple with a male partner born with severe bladder exstrophy.bladder exstrophy is a rare congenital condition, with variable degrees of reproductive dysfunction, from mild exstrophy allowing regular intercourse and reproduction, to severe forms with significant cosmetic deformity and failure to reproduce spontaneously. This is a case report of a patient with severe congenital bladder exstrophy and failure to reproduce spontaneously due to microphallism and anejaculation, treated with testicular sperm aspiration and intracytoplasmic sperm injection (ICSI), resulting in the conception and birth of a healthy unaffected singleton male infant. This the first case report of bladder exstrophy of such a severe degree with a successful reproductive outcome. It illustrates that artificial reproductive techniques can be successful in achieving conception and ongoing pregnancy, including birth of a healthy infant, in patients where intercourse is impossible due to specific genital defects.- - - - - - - - - - ranking = 0.28917444206094keywords = pregnancy (Clic here for more details about this article) |
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