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      Repeated successful vaginal delivery in a pregnant woman with unrepaired ectopia vesicae and split pelvis: a case study

      case-report

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          Abstract

          Abstract

          Ectopia vesicae, or bladder exstrophy, is a rare malformation, more frequently found in males. Very few cases of pregnancy with unrepaired ectopia vesicae have been reported in literature. The majority of these pregnant women with ectopia vesicae have terminated their pregnancies by cesarean section due to malpresentation, preterm labor or other indications. Clemetson concluded that cesarean section was the preferable method of term delivery to avoid postpartum prolapse. We have a different opinion on this because we had an interesting case. A woman with unrepaired ectopia vesicae had two successful vaginal deliveries, in 2009 and 2019 respectively. She recovered well and did not have any symptoms or signs of pelvic organ prolapse (POP) so far.

          Case presentation

          Let us present this woman with ectopia vesicae who had four pregnancies; two spontaneous abortions and two vaginal deliveries. In 2009, she had a successful vaginal delivery at Yantai Harbor Hospital where the first author worked at that time. She met the first author again surprisingly, during her third trimester in 2019. She had a spacious pelvis and pendulous abdomen. In this fourth pregnancy, the fetus changed its presentation frequently. Still, she had the second vaginal delivery successfully. She recovered fully after delivery and did not have any symptoms or signs of POP. As far as we know, this is the first case that a patient with ectopia vesicae who has been observed for such a long time after multiple vaginal deliveries.

          Conclusions

          Doctors must evaluate the risk of vaginal delivery or cesarean section and consider maternal-neonatal health. Prior to this, women with repaired or unrepaired ectopia vesicae usually delivered their babies by cesarean section. Our practice shows that vaginal delivery is also a safe and feasible choice for some of these patients, especially for those with unrepaired, mild types of ectopia vesicae who experience no other dangerous or uncomfortable symptoms.

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          Most cited references16

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          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.
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            Bladder exstrophy-epispadias complex.

            The bladder exstrophy-epispadias complex (BEEC) represents an anterior midline defect with variable expression comprising a spectrum of anomalies involving the abdominal wall, pelvis, urinary tract, genitalia, and occasionally the spine and anus. The vast majority of BEEC cases are classified as non-syndromic and the etiology of this malformation is still unknown. This review presents the current state of knowledge on this multifactorial disorder, including historical retrospect, phenotypic and anatomical characterization, epidemiology, proposed developmental mechanisms, existing animal models, and implicated genetic and environmental components. These published lines of evidence argue strongly that BEEC occurs as a result of strong genetic predisposition that is yet to be deciphered.
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              Four cases of bladder exstrophy in two families.

              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.

                Author and article information

                Contributors
                591215843@qq.com
                Journal
                BMC Pregnancy Childbirth
                BMC Pregnancy Childbirth
                BMC Pregnancy and Childbirth
                BioMed Central (London )
                1471-2393
                12 May 2020
                12 May 2020
                2020
                : 20
                : 290
                Affiliations
                [1 ]GRID grid.452240.5, Department of Obstetrics, , Yantai Affiliated Hospital of Binzhou Medical University, ; Yantai, 264000 China
                [2 ]GRID grid.440653.0, ISNI 0000 0000 9588 091X, College of Clinical Medicine, , Binzhou Medical University, ; Yantai, 264000 China
                Article
                2931
                10.1186/s12884-020-02931-x
                7218511
                32397972
                f83bfe06-4115-413b-87d4-d72c6d93f09c
                © The Author(s) 2020

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 16 January 2020
                : 7 April 2020
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2020

                Obstetrics & Gynecology
                ectopia vesicae,vaginal delivery,split pelvis
                Obstetrics & Gynecology
                ectopia vesicae, vaginal delivery, split pelvis

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