A pregnant woman with an operated bladder extrophy and a pregnancy complicated by placenta previa and preterm labor

Introduction

Bladder exstrophy (BE) is a congenital anomaly that has an incidence of 3.3–5 per 100,000 live births. This anomaly is seen 1.5–2.5 times more frequently in males than it is in females [1]. According to the most accepted theory, it occurs when an overdeveloped cloacal membrane disrupts the abdominal wall development during embryogenesis and prevents the mesenchymal tissues from migrating towards the midline [2].

Due to improvements made in neonatal care and developments made in surgery, the quality of life for these patients has improved in recent years [3]. As the number of adult patients with BE increases, some challenges have emerged for obstetricians, including fertility, pregnancy complications and appropriate mode of delivery. Subfertility, early pregnancy loss, preterm labor, malpresentation of the fetus and abnormalities of the placenta are more common in patients with BE [4]. However, the number of such pregnancy cases are limited and most centers lack experience in this field. Thus, these patients must be followed-up at multidisciplinary tertiary reference centers.

This case report aims to share our clinical experiences of managing a complicated pregnancy with an operated BE.

Case

A 29-year-old pregnant woman was referred to the Hacettepe University Hospital Perinatology clinic for an operated maternal BE, an oligohydramnios and a placenta previa during the 25^th gestational week of her pregnancy.

The patient was operated for BE and urinary diversion was provided by ureterosigmoidostomy when she was 1 year old. Through these procedures, she developed both urinary and fecal continence. When she was 28 years old, she underwent another operation for a vaginal septum; she had two pregnancies after that operation. Her first pregnancy resulted in spontaneous abortion at the 8^th week of gestation.

In this pregnancy, a combined test, a chorionic villus sampling (CVS), an ultrasonographic fetal abnormality screening and a fetal echocardiography were conducted at another healthcare institution with normal results (CVS and fetal echochardiography were performed for increased nuchal translucency) and antenatal corticosteroid therapy was administered for fetal maturation. When she was admitted to our facility, the patient was evaluated and a preterm premature rupture of the membranes (PPROM) was detected along with placenta previa totalis; thus, the patient was hospitalized. The development of the fetus was appropriate for the gestational week (25^th gestational week). As the patient had a penicillin allergy, she was given clindamicin 900 mg 3 times a day for chorioamnionitis prophylaxis. A complete blood count was done along with a biochemical analysis of blood, and the sedimentation and C-reactive protein (CRP) values were assessed. In addition, the patient had a methylenetetrahydrofolate reductase (MTHFR) A1298C and plasminogen pctivator inhibitör-1 (PAI) 4G/4G homozygous polymorphisms so that low molecular weight heparin and acetylsalicylic acid were started (enoxaparin 2000 Anti-Xa IU/0.2 mL and acetylsalicylic acid 100 mg).

The fetus was evaluated by ultrasonography daily and anhydramnios was detected on the 2^nd day of the patient’s hospitalization. During the follow-up of the patient, her CRP values rose (from 0.805 mg/dL to 7.45 mg/dL) and the maternal body temperature began to rise gradually. After 2 weeks, a multidisciplinary council decided that she should give birth; this council consisted of specialists in the fields of perinatology, neonatology, pediatric surgery and urology. A cesarean section (C/S) was performed at 27 weeks and 6 days of gestation. Four units of red-blood cells and four units of fresh-frozen plasma were prepared preoperatively for placenta previa totalis. A midline incision was preferred, as the patient had previously had BE surgery. After massive adhesiolysis, a low segment transverse incision was performed and a 1330 g male infant was delivered with APGAR scores of 4–6–7 (for 1^st, 5^th and 20^th min, respectively). Two units of red-blood cells and two units of fresh-frozen plasma were transfused intraoperatively due to excess bleeding from the placental separation site. However, bleeding control was provided, and no further replacement was necessitated after the operation. The baby was transferred to the neonatal intensive care unit and he was discharged with a full recovery after 2 months. After a 1-week follow-up at the hospital, the patient was discharged without any complications.

Discussion

BE is a complex congenital anomaly that affects the urinary, reproductive, musculoskeletal and intestinal systems [5]. Surgical interventions are essential for improving the quality of life of patients with BE. The main goals of surgery are reconstructing the deformities, providing appropriate urinary and sexual function and ensuring acceptable genital cosmetic appearance. The main surgical procedures performed by the physicians are a modern staged repair of BE, complete primary repair of BE and urinary diversions [6]. The major complications of surgery in female patients are bladder dehiscence, recurring urinary tract infections, the formation of bladder stones, bladder perforation and fistulas [7].

Patients with BE face many complications during their lives. However, these complications can be minimized and the quality of life for these patients has been improved as medicine has advanced [3]. The other long-term complications in patients with BE are vaginal and rectal prolapses and an increased risk for malignancy (bladder and colon carcinomas) [8]. Thus, managing BE is challenging and clinical follow-ups of the patients should be planned meticulously by physicians.

BE is more common in men than it is in women, and the severity of the anomaly is higher than it is in females; notably, sexual dysfunction causes decreasing fertility rates in men. Conversely, for female patients with a BE, fertility rates are lower than they are for the normal population, even though the patient in our case had two pregnancies [4].

In the pregnancies of patients with BE, antenatal complications like preterm labor, “malpresentation/placentation disorders” and miscarriages are reported to be more common [4]. Our case was complicated by the presence of MTHFR A1298C and PAI 4G/4G homozygous polymorphisms as well as placenta previa. It has been reported that MTHFR polymorphisms are associated with various obstetrical complications such as recurring miscarriages, preterm labor, preeclampsia and placentation disorders [9]. It has also been reported that DNA methylation pathway disorders (such as MTHFR polymorphisms) are associated with neural tube defects (NTDs), conotruncal heart defects and various mid-line abnormalities [10]. Our case had a history of having an abortion at the 8^th gestational week of her first pregnancy; in her second pregnancy, she had placenta previa, preterm labor (PPROM) and the fetus was in breech presentation, which was consistent with the literature.

Adult female patients with BE can have successful pregnancy outcomes if they receive appropriate antenatal care. However, the follow-up of these patients must be carried out at experienced tertiary reference centers with a multidisciplinary approach. This difficult process must be managed by obstetricians, neonatologists, urologists and pediatric surgeons working together.