A 27-Year-Old Brazilian Woman with a History of Left Salpingectomy and Late Diagnosis of an Extra-Uterine Intra-Abdominal Pregnancy and Live Birth at 26 Weeks’ Gestation

Background

Abdominal pregnancy is a special type of ectopic pregnancy, characterized by implantation of the embryo in the peritoneal cavity, with tubal, ovarian, and intraligamentary pregnancies excluded, accounting for approximately 1% of all cases [1]. It was first reported in 1708 after an autopsy and since then numerous cases have been reported, with a current incidence of 1: 10 000 to 1: 30 000 pregnancies [2]. Therefore, we present this case report of late-diagnosed abdominal pregnancy with a live fetus.

Case Report

A 27-year-old woman underwent the first ultrasound of the pregnancy in an external service to the General Hospital and was referred to the same due to suspicion of extra-uterine pregnancy. Ultrasound imaging showed a fetus, apparently located in the abdominal cavity to the right of the uterus, placenta inserted behind the uterus, absent amniotic fluid, fetus with heartbeat, estimated weight of 670 g, and gestational age 25 weeks and 1 day.

The patient had a history of pregnancy in the left uterine horn, with resection and raffia of the same in 2014, with the tube and ovary preserved. She had no previous comorbidities, except a diagnosis of gestational diabetes with lifestyle change treatment; other prenatal exams within the normal range. She arrived at the service without concerns, with good fetal movement, and without adverse events during this pregnancy. On physical examination, it was possible to identify a uterus of increased volume, approximately at the umbilical scar line, and easy palpation of fetal parts and auscultation of fetal heartbeat. On vaginal touch, bulging of the vaginal sac was observed with anteriorization of the cervix. The patient was admitted to an obstetric inpatient unit to clarify the case and schedule the interruption of pregnancy.

Magnetic resonance imaging of the total abdomen was performed on the day of admission. No topical pregnancy was identified. There was placental formation in the topography of the left adnexal region, surrounded by the ipsilateral fallopian tube. The fetus was inside the abdominal cavity, predominantly located on the right flank and iliac fossa, with the cephalic portion between the bladder and the rectum, and the lower limbs interlocking on the right flank. We found marked pyelocaliceal dilatation with transition in caliber of the ureters at the level of the described alterations (Figures 1–3).

Aiming at lung maturation, 2 ampoules, each with 1 mL containing 3 mg betamethasone acetate and 3.945 mg betamethasone disodium phosphate were injected intramuscularly and a new obstetric ultrasound was performed for a better fetal evaluation, showing an irregular cranial contour. There was a cystic image with thin septa adjacent to the fetal neck measuring 3.5×2.7 cm. The heart had 4 cavities and was apparently balanced. The thorax was apparently reduced in size, and it was not possible to evaluate the integrity of the diaphragmatic dome.The feet were in plantar flexion and the bladder had increased in size.

After a multidisciplinary discussion with the oncologic surgery team (which was invited to participate in the case due to its complexity) in which maternal and fetal risks and benefits were extensively evaluated, and also after explanation and discussion with the patient, aiming at a better evaluation of placental implantation in large vessels, it was decided to perform an angiotomography with abdominal contrast to ensure a better surgical planning to assure the patient’s safety (Figure 4). The examination demonstrated increased arterial impregnation of the intrauterine vessels; signs of volumetric increase of the tubal region to the left, with increased vascularization by contrast; placenta located in the hypogastric/iliac fossa to the right with extension to the mesogastric region/left flank, without cleavage plane with posterior body wall of the uterus and left uterine attachment; calibrous vessels from the uterine and left ovarian arteries apparently supplying the placenta were visualized, as well as signs of venous drainage from the placenta through the left gonadal vein, which presented with increased caliber; and there were no signs of involvement of the intestinal loops by the placenta. After performing an angiotomography, the team talked to the patient about interrupting the pregnancy due to the increased risk to the mother’s life as the pregnancy progressed. Vascular surgery, urology, oncology surgery, and obstetrics teams met.

For preoperative preparation, magnesium sulfate (8 mL at 50% diluted in 12 mL of distilled water in an attack dose with maintenance with 10 mL at 50% in 240 mL of saline solution in an infusion pump at 50 mL/h) for fetal neuroprotection and ampicillin due to prematurity were performed, as well as an 8-h fast and a supply of blood components (4 units of packed red blood cells, 8 units of plasma, and 4 units of cryoprecipitate).

Surgery was performed in 3 stages. Before starting the surgery, spinal anesthesia was performed and a conversion to general anesthesia was planned after delivery. In the first step, the urological surgery team implanted a double-J catheter bilaterally to facilitate identification of the ureters in case more extensive surgery involving hysterectomy was needed during the transoperative period. In the second step, the vascular surgery team performed catheterization of the right femoral artery with balloon placement in the left hypogastric artery to reduce major bleeding.

In a third and last step, exploratory laparotomy was performed. A fetus was visualized (Figure 5), without amniotic sac, between intestinal loops in the abdominal cavity, removed in pelvic presentation with immediate clamping of the umbilical cord (Figure 6), with the placenta implanted in the left uterine artery and left gonadal artery and posterior uterine wall with invasion of the sigmoid rectum (Figure 7). Due to massive bleeding after fetal removal and the patient’s evolution to hemodynamic instability, uterine preservation and conservative management with placenta maintenance and clinical treatment for absorption were not possible and a monoblock extended total hysterectomy with sigmoid (Figures 8, 9) and colostomy was performed. In the transoperative period, the patient presented hypovolemic shock, requiring replacement of blood products, with good recovery. After being moved to the ICU for postoperative recovery, she was discharged to the ward in less than 24 h. The female neonate was immediately attended to by the neonatal ICU team. She presented an Apgar score of 2 in the first minute and 6 in the fifth minute, weighing 960 g, and with a physical examination compatible with 26 weeks and 3 days of gestational age. She required orotracheal intubation soon after birth. General inspection identified skin remnants on the head and neck and 3 arteries and 1 vein in the umbilical cord stump. On physical examination on pulmonary auscultation, she presented ruddy vesicular murmurs, with air intake, but no chest expansion; furthermore, she remained cyanotic even under mechanical ventilation, with no response to surfactant use (200 mg per kilogram). A chest X-ray identified hypoextended lungs. On the day after birth, with less than 24 h of life, she died after cardiorespiratory arrest, with no response to management.

The ballon in the left hypogastric artery was removed soon after the procedure. The patient remained in a ward bed for 4 days, received cabergoline 1 g, ampicillin 2 g in association with Sulbactam 1 g, and diet progression. She was discharged from the hospital for follow-up at the gynecological and oncologic surgery outpatient clinic, with a plan for intestinal transit reconstruction which had not yet been reestablished to date. The double-J catheter was removed at an outpatient visit 2 months after surgery.

Discussion

Although the etiology of abdominal pregnancy still remains unknown, it is known that embryo implantation in the abdominal cavity can be either primary or secondary. Secondary cases are more common and occur after rupture of a tubal pregnancy, tubal abortion, or even after rupture of a hysterotomy scar, a rudimentary horn, or uterine perforation [3], as in the case of our patient who had a history of pregnancy in the left uterine horn, with resection and raffia of the same.

It is a life-threatening situation due to high maternal and fetal morbidity and mortality, with a maternal mortality rate around 0.5% to 18% and a perinatal mortality rate between 40% and 95% [4]. In addition, it is estimated that approximately 21% of newborns from an abdominal pregnancy have some malformation, probably due to compression of the fetus in the absence of amniotic fluid. Typical deformities include limb defects, facial and cranial asymmetry, joint abnormalities, and central nervous system malformations [5]. However, its early detection is difficult, with only 45% of diagnoses being made prenatally [6], with most cases diagnosed after complications or only intraoperatively. When symptomatic, the clinical signs are not very specific, but some authors believe that some signs can alert to the possibility of the diagnosis: abdominal pain with disordered bowel movements, abdominal pain during active fetal movements, spread of the abdomen due to an irregular presentation, palpation of fetal parts below the maternal abdominal wall, and failure to trigger labor. Unfortunately, these signs only appear during advanced abdominal pregnancy [7].

Ultrasonography remains the main test for diagnosis, and The Royal College of Obstetricians and Gynecologists has recommended the use of the sonographic criteria described by Gerli et al for diagnosis: absence of an intrauterine gestational sac, absence of dilatation of both uterine tubes and of complex ovarian masses, a gestational sac surrounded by intestinal loops and separated from them by the peritoneum, and a wide mobility similar to flotation of the sac, particularly evident with pressure from the transvaginal transducer toward the posterior fundus. [8]. However, due to the complexity and rarity of the condition, these criteria are not always present and clear even to an experienced echographer, and it is essential to perform an additional imaging exam to elucidate the case. MRI has proven to be a great help, as it provides an easier diagnosis and helps to localize and identify the relationship between the placenta and adjacent organ and tissues. The location of the placenta can help to decide whether to continue with the pregnancy and to develop a relatively safe and reasonable treatment option and surgical planning [9].

Open surgery has been the main means of treatment for advanced abdominal pregnancies due to better control of the risk of bleeding related to placental extraction, justified only if the placenta is easily removed with low risk of bleeding. It is believed that for placental delivery to regions rich in vessels and with low mobility (pelvic ligaments, the region of the iliac cases, liver, and spleen), surgery should be meticulous to avoid separation of the placenta, which should be left in its implantation site to be resorbed spontaneously postoperatively whenever possible [10]. The use of medications to assist the absorption process has been studied, the most prominent being methotrexate, but its use remains controversial because it involves a high risk of infection due to accelerated necrosis. Moreover, in cases where the placenta remains, we should be alert for the appearance of postoperative complications such as intestinal obstruction, infection, hemorrhage, anemia, and fistula, among others. These complications can worsen the maternal prognosis, with a mortality rate above 18% [11].

Conclusions

Abdominal pregnancy is a rare type of ectopic pregnancy and is a life-threatening situation. Although this is not a frequent situation in obstetrics, it requires attention and care by specialists so that everyone is prepared to act in the best possible way. Its biggest challenge is to make an early diagnosis, since most cases go unnoticed through the ultrasound performed in the first trimester, and when symptomatic, they do not present themselves in a specific way. In our case, we were lucky because, although our patient had her first ultrasound done in an external service and late, she did it with an experienced physician and member of our clinical staff, which ensured, besides the diagnosis, an immediate referral to the obstetric center for a better evaluation and investigation of the case. It was a great challenge because, although it is a reference hospital for high-risk pregnancy, most of the obstetrics team had never had experience with a similar case, which required many team members to seek information and ensure the best care for this patient. For this, a multi-specialized team, involving, besides obstetrics, the radiology sector, surgical oncology, vascular surgery, urological surgery, anesthesiologists, transfusion agency, adult ICU, and neonatologist, was involved, since the fetus was alive and viable according to the gestational age. Unfortunately, from the fetal point of view, we did not have the best desired outcome; however, from the maternal point of view, although our first plan was for conservative treatment with metrotexate, since the patient did not have any living child, in view of the severity of the condition, the surgical complexity, and the unforeseen events during the transopetarean section, we consider that we had the best possible outcome.