Successful vaginal delivery in a patient with reconstructed bladder exstrophy and vaginoplasty after cesarean section, a case report from Syria.

A 26-year-old woman, BMI 23, primigravida who had been married for two years, was admitted to our hospital with breech presentation at week 36 of gestation. Her past surgical history included treated bladder exstrophy (BE) by Mitrofanoff procedure (trans-appendicular continent cystostomy) at the age of five, and vaginoplasty at the age of twenty two. She also has pubic diastasis, bicornuate uterus and left-sided fallopian tube blockade at Hysterosalpingography (HSG). Her medical history revealed frequent urinary tract infections (UTIs) treated with antibiotics. These UTIs are a result for daily self- catheterization through the umbilicus. An elective cesarean section (CS) was decided to deliver the baby. During surgery, paramedian section was made, methylene blue was injected into the bladder through the umbilicus to clarify the boundaries of uterus and manufactured bladder. The uterus was opened by a diagonal paramedian high classical incision to avoid injuries to the manufactured bladder as it could not be dissected from uterus. A healthy baby boy weighing 2800 g was delivered and uterus was closed with two layers, Vicryl thread 2/0 cross stitching. Five years later, she came back to our emergency department at week 30 of gestation with mild vaginal bleeding and uterine contractions that revealed labor in its active phase. Her vital signs were within normal limits. Ultrasonography (US) showed the fetus in breech presentation, and the placenta in its normal position with no signs of abruption. Clinical vaginal examination showed an opened cervix with 4 cm dilation and 80 % effacement. Few hours later, her bleeding increased to become moderate, while her vaginal examination progresses rapidly to become 8 cm dilation*85 % effacement, and US showed signs of abruption(evolution of sub-chorionic hematoma), meanwhile fetal heart rate (FHR) which had been monitoring all the time by cardiotocography (CTG) stayed within normal limits without any decelerations. But with her active labor (rapid progression in dilation and effacement of cervix from 80*85 % to +2 descent in fetal presentation in minutes) C-section was not the best choice of delivery. So, we decided to deliver the baby vaginally as the mother's vital signs still within normal limits. As planned, vaginal birth was uneventful, and the1900g baby girl was admitted in the NICU. The mother did not suffer any complication during or after vaginal birth, and she was discharged home one day later.

Maram Balouli: gathering information, literature review, writing manuscript. Aya Ahmad: gathering information, literature review, writing manuscript. Omran Janoud: gathering information, literature review, writing manuscript. Ahmad Mustafa Ahmad: gathering information, literature review, writing manuscript. Basel Ahmad: gathering information, literature review, writing manuscript, review manuscript. Bashar AlKurdi: performing procedure, writing manuscript, review manuscript, supervise whole work.

Written informed consent was obtained from the patient for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

This is a case report, there is no need for committee ethical approval, however, in our university hospital, and patients agree and sign routinely to use their medical information in research.

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Bashar Alkurdi.

Bladder exstrophy is a challenging disease that is accompanied with other morbidities. Women with BE still have an impaired fertility due to previous surgeries and concomitant diseases, and pregnancy is high risk for both mother and baby. Balancing the pros and cones of the medical decision in order to achieve the best outcomes for both mother and child is crucial as choosing the right decision depends on the presentation and the past history of the patient not only the existence of the bladder exstrophy.

Bladder exstrophy, also known as Ectopia vesicae, is a rare malformation often associated with other deformities such as varying degrees of split pelvis (typically seen with eversion of the bladder, abnormal appearance of external genitalia, and divergence of the pubis), weakness and loss of the inferior abdominal wall, and abnormal development of other organs like the vaginal septum, uterus, umbilicus, etc. [ 6 , 7 ]. There is no known embryological mechanism behind the development of this malformation; however, it is believed that an error in ventral fusion due to persistent weakness of the cloacal membrane by the underlying mesoderm may be the most important cause [ 8 ]. The incidence rate of Bladder exstrophy ranges from 1:30,000 to 1:50,000 live births and is two or three times more common in males. The highest rates of successful treatment are achieved through performing treatment shortly after birth [ 1 ]. The initial management of such cases is complex and should focus on the entire pelvis. Some studies suggest that surgical repair should be performed as soon as possible after delivery while others argue that surgery is not urgent regarding the reproductive system. There are different types of vaginoplasty available for treatment options [ 9 ]. Regarding pregnancy in this population, approximately, 90 % of women report sexual activity. However, Dyspareunia is considered so frequent, even after vaginoplasty [ 9 ]. Low fertility rate in this group is usually attributed to coexisting diagnosis which includes endometriosis, tubal obstruction and polycystic ovarian syndrome [ 9 ]. Pregnancies in patients with bladder exstrophy carry specific risks range from prolapse of the internal reproductive organs due to pelvic instability and urinary complications (hydronephrosis, pyelonephritis) to operative risks at the time of the delivery [ 9 , 10 ]. For some studies, elective cesarean should be performed in majority of cases at 37 weeks under a cover of urological specialists in the operation room. Furthermore, Vaginal delivery should only be considered in uncomplicated pregnancies when a senior obstetrician and urologist is available for dealing emergencies [ 11 ]. Our 26-year-old patient, who had a bladder exstrophy treated with Mitrofanoff procedure, and vaginoplasty, presented 5 years ago with pregnancy. As per recommendations of many studies, an elective cesarean section (CS) by a paramedian section was performed [ 11 ]. This paramedian section was selected to avoid injury to the reconstructed bladder. In the literature, to the best of our knowledge, we found that one case study reported a successful delivery of a baby in a woman with untreated bladder exstrophy who underwent an elective cesarean delivery at term due to breech presentation [ 12 ]. However another case study reported a woman with two vaginal deliveries regardless having untreated bladder exstrophy [ 6 ]. In her first pregnancy, our patient underwent elective cesarian delivery, whereas for her second pregnancy, after five years, a decision was made to perform a vaginal delivery due to the active labor which made cesarean delivery not the most suitable choice. Placental abruption is one of the most important differential diagnosis for third trimester bleeding and occurs in about 1 % of all pregnancies. Hypertension, preeclampsia, gestational diabetes, PROM, chorioamnionitis and oligohydraminos are the most common risk factors [ 13 ]. In our case, the patient did not have any specific risk factor for abruption, nevertheless, signs and ultrasound features of abruption developed late in our patient. The fast progression of effacement and dilation and the major previous surgical history made us go with the vaginal delivery rather than c section. Balancing the pros and cones of the medical decision in order to achieve the best outcomes for both mother and child is crucial as choosing the right decision depends, to a great extent, on the presentation and the past history of the patient not only the existence of the bladder exstrophy. And this concept is clear through the published studies where no specific guideline to treat these cases was mentioned; even though a systematic review recommended considering the vaginal delivery as the treatment of choice in all cases with few exceptions [ 10 ].

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Bladder exstrophy (BE) or Ectopia Vesicae is a rare congenital anomaly in which the anterior wall of the bladder is absent. It affects 1 of 50,000 live births, and is three times more frequent in males [ 1 ]. It begins in the fourth week of gestation due to ventral fusion error and presents as protrusion of the dorsal mucosal surface of the bladder through a defect in the abdominal wall. Other clinical presentations in females that may negatively affect sexuality and fertility include pubic diastasis, dysplasia of the pelvic floor muscles, clitoris bifurcated, short vagina and narrow introitus [ [1] , [2] , [3] ]. Advances in reconstruction surgery led to higher survival rates, and alternative catheterization methods like Mitrofanoff conduit (trans-appendicular continent cystostomy) allowed clean self- catheterization [ 4 ]. However, women with BE still have an impaired fertility due to previous surgeries and concomitant diseases, thus pregnancy is high risk for both mother and baby [ 2 ]. Delivery should be at a tertiary referral obstetric hospital with urology cover, and planned cesarean section is the most appropriate mode of delivery for majority of patients [ 2 ]. We present a case of 26-year-old woman with treated BE, vaginoplasty, and bicornuate uterus that came at week 36 of gestation. An elective cesarean section was made by a paramedian section. Five years later, she came back with placenta abruption at week 30 of gestation, and a successful vaginal delivery was made. The case has been reported in line with the SCARE 2023 criteria [ 5 ].

There is no conflict of interest to disclose.