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Abdominal pregnancy

Abdominal pregnancy
Literature review current through: Jan 2024.
This topic last updated: Oct 24, 2022.

INTRODUCTION — Abdominal pregnancy is a rare form of ectopic pregnancy in which the pregnancy implants within the peritoneal cavity, exclusive of the fallopian tubes, ovaries, broad ligament, and cervix. It is associated with high maternal and fetal morbidity (eg, maternal hemorrhage, fistula formation, fetal malformation) and mortality.

Early recognition and treatment of abdominal pregnancy may improve outcomes. However, despite advances in diagnostic modalities, the diagnosis of abdominal pregnancy is often a late diagnosis (eg, at the time of intended cesarean birth or emergency surgery for intraabdominal hemorrhage).

This topic will focus on issues related to abdominal pregnancy. The diagnosis and management of other ectopic pregnancies (eg, tubal, ovarian, cervical, heterotopic) and abnormally implanted intrauterine pregnancies (eg, cesarean scar, placenta accreta spectrum) are discussed in detail separately.

(See "Ectopic pregnancy: Epidemiology, risk factors, and anatomic sites" and "Ectopic pregnancy: Clinical manifestations and diagnosis" and "Ectopic pregnancy: Choosing a treatment".)

(See "Cervical pregnancy: Diagnosis and management".)

(See "Cesarean scar pregnancy".)

(See "Placenta accreta spectrum: Clinical features, diagnosis, and potential consequences" and "Placenta accreta spectrum: Management".)

TERMINOLOGY

There are two types of abdominal pregnancy [1]:

Primary abdominal pregnancy results from implantation of a blastocyst in the abdominal cavity.

Secondary abdominal pregnancy results from the implantation of a pregnancy into the abdominal cavity that was previously located elsewhere (eg, ruptured tubal pregnancy).

While of interest physiologically, the type of abdominal pregnancy is not clinically relevant; in this topic, we use the term abdominal pregnancy to refer to a pregnancy implanted in the abdominal cavity, irrespective of its initial implantation site.

Abdominal pregnancy is further classified as:

Early, when diagnosed at ≤20 weeks of gestation, or

Advanced, when diagnosed >20 weeks of gestation

The gestational age at which the abdominal pregnancy is diagnosed may affect management decisions. (See 'Hemodynamically stable patients with live gestations' below.)

INCIDENCE — It is difficult to estimate the frequency of abdominal pregnancy because it is rare; some studies report incidence rates of one per 10,000 to 30,000 live births or 1 percent of all ectopic pregnancies [1]. Secondary abdominal pregnancy is thought to be more common than primary abdominal pregnancy [2,3], but most studies do not specify the type of abdominal pregnancy.

Heterotopic intrauterine and abdominal pregnancies [4] and twin abdominal pregnancies [5] have also been described.

RISK FACTORS — Risk factors for abdominal pregnancy are extrapolated from data on tubal ectopic pregnancy (table 1). (See "Ectopic pregnancy: Epidemiology, risk factors, and anatomic sites", section on 'Risk factors'.)

Risk factors specific to abdominal pregnancy are less well understood; one such factor is tubal factor infertility and higher number of embryos transferred during in-vitro fertilization (IVF). In one systematic review including 28 case reports of abdominal pregnancy after IVF, a history of tubal surgery and ectopic pregnancy were reported in 50 and 39 percent of patients, respectively [6]. Transfer of >2 embryos and fresh compared with frozen embryos were also associated with increased risk.

An association between abdominal pregnancy and cocaine use has also been reported (one study, 55 cases) [7]; the mechanism by which this occurs is unclear.

NATURAL HISTORY — Abdominal pregnancy is associated with high maternal and fetal morbidity and mortality. The prognosis depends on several factors, one of which is the gestational age (ie, ≤20 or >20 weeks) at diagnosis.

The natural history of patients with early (ie, ≤20 weeks of gestation) abdominal pregnancy is similar to that of tubal ectopic pregnancy and is described separately. (See "Ectopic pregnancy: Clinical manifestations and diagnosis", section on 'Natural history'.)

The natural history of patients with advanced (ie, >20 weeks of gestation) abdominal pregnancy is described here. In a review of 22 reports from 13 countries including 163 cases of advanced abdominal pregnancy, the following outcomes were reported [8]:

Maternal Mortality – 12 percent. Other studies report a maternal mortality between 0.5 and 18 percent [9,10]. This rate is higher than that of tubal ectopic pregnancies [7]. (See "Ectopic pregnancy: Epidemiology, risk factors, and anatomic sites", section on 'Mortality'.)

Maternal hemorrhage requiring blood transfusion – 80 percent.

Fetal mortality – 72 percent. Others report a fetal mortality rate between 45 and 90 percent [10].

Fetal morbidity – 20 to 40 percent [11]. Fetal morbidity includes fetal deformation (eg, facial or cranial asymmetry, limb deficiencies) which results from fetal compression and a lack, or absence, of amniotic fluid. Fetal growth restriction may also occur as blood supply from the colonized placenta is poor compared with intrauterine pregnancy [4].

Case reports detailing poor maternal and/or fetal outcomes include, but are not limited to: massive hemoperitoneum with maternal and fetal death [12]; maternal hemorrhage, neonatal death, and postpartum sepsis requiring relaparotomy [5]; maternal hemorrhage requiring intraabdominal packing and relaparotomy [1]; bowel obstruction requiring decompression with a nasal gastric tube followed by delivery of an infant with fetal malformation (eg, unilateral clubfoot, fascial asymmetry) [13]; fetal death followed by maternal intraabdominal abscess formation requiring relaparotomy [3].

Although uncommon, successful outcomes have also been reported [2,3,11,14,15]. In one case report, a patient was diagnosed with an abdominal pregnancy during a routine ultrasound at 22 weeks of gestation [14]. Magnetic resonance imaging (MRI) revealed an intraabdominal pregnancy with the placenta inserted along the abdominopelvic peritoneum. The patient declined pregnancy termination and was thus managed expectantly. She delivered a live born infant at 32 weeks without fetal malformation via planned cesarean birth; blood transfusion, while anticipated, was not required. The placenta was left in situ and treated postpartum with selective uterine artery embolization (UAE). A 15-month postpartum pelvic ultrasound demonstrated almost complete resolution of the placental structures.

CLINICAL PRESENTATION — Patients with an abdominal pregnancy may be asymptomatic, present with nonspecific pregnancy symptoms (eg, nausea and/or vomiting [particularly in patients with placental invasion of the bowel or omentum], abdominal pain, painful fetal movements, vaginal bleeding [from a decidual reaction in the uterus]), or life-threatening intraabdominal hemorrhage (particularly in patients with placental invasion of the liver or spleen) [4,11].

A high index of suspicion is needed, particularly in patients with risk factors for ectopic pregnancy. (See 'Risk factors' above.)

CLINICAL FINDINGS

Physical examination — At early gestational ages (<20 weeks), physical examination findings are typically absent. At more advanced gestations (≥20 weeks), physical examination findings may include:

Abnormal fetal lie [11]

Easily palpable fetal parts [11]

Fetal growth restriction [7]

Failed induction of labor or absence of labor at term [4]

Absence of fetal heart tones (ie, fetal demise)

Marked oligohydramnios [3]

Imaging findings

Ultrasound — Sonographic findings vary and include [16]:

An empty uterus with a fetus in a gestational sac outside the uterus. Abdominal pregnancy may be erroneously reported as a normal intrauterine pregnancy if the relationship between the pregnancy and myometrium is not evaluated at the time of ultrasound. (See 'Differential diagnosis' below.)

Close approximation to, and absence of myometrium between, the fetus and maternal abdominal wall.

Absence of myometrium between the fetus and urinary bladder.

Placenta outside the confines of the uterine cavity. However, localization of the placenta in the peritoneal cavity and the detection of placental adherence to solid organs, bowel, and omentum can be difficult with ultrasound.

However, these findings are missed in approximately 50 percent of patients and are more commonly missed in the second and third compared with the first trimester [3].

Training in obstetrics ultrasound should emphasize features suggestive of abdominal pregnancy (which may improve recognition of these findings, when present), and the importance of documenting that a pregnancy is within the uterus during each ultrasound examination [3].

Magnetic resonance imaging — For patients with sonographic features suspicious for abdominal pregnancy or in whom ultrasound images are suboptimal, magnetic resonance imaging (MRI) may be a useful adjunct to ultrasound to accurately localize the pregnancy, the placenta, its blood supply, and adherence to surrounding organs.

In a retrospective study including nine patients with abdominal pregnancy in which MRI images were reviewed by two radiologists who were blinded to the clinical and operative findings, MRI accurately located the placenta in all nine patients [17].

MRI may also be used for patients undergoing expectant management (to evaluate fetal growth, amniotic fluid levels, placental invasion) and for preoperative planning. The latter is particularly important as severe hemorrhage is likely if there is an attempt to remove the placenta during surgery [17]. (See '>20 weeks and choosing expectant management' below and 'Managing the placenta' below.)

Laboratory findings — Abnormal laboratory findings are typically absent. In patients in whom prenatal screening for aneuploidy or fetal anomalies was performed, the following may be abnormal; however, these are nonspecific and cannot be used to make a diagnosis of abdominal pregnancy:

Increased maternal serum alpha-fetoprotein (AFP) and/or increased human chorionic gonadotropin (hCG) – Direct abdominal implantation of the placenta may enhance fetal-maternal transfer of AFP and hCG [2,18].

Decreased maternal serum unconjugated estriol (uE3) – Smaller placental size and altered vascularity may disrupt placental sulphatase activity, resulting in decreased estriol production and decreased levels of maternal serum uE3 [18].

These markers are discussed in detail separately. (See "Neural tube defects: Overview of prenatal screening, evaluation, and pregnancy management", section on 'Alpha-fetoprotein' and "Maternal serum marker screening for Down syndrome: Levels and laboratory issues", section on 'Types and timing of serum marker screening tests'.)

DIAGNOSIS — The diagnosis of abdominal pregnancy is made by visualization (with fetal imaging [ie, transvaginal ultrasound, MRI] or intraoperatively during surgical exploration) of an extrauterine pregnancy and an intact uterus (ie, no evidence of uterine rupture).

The majority of patients will be diagnosed at the time of surgery [1]. In the review of 163 cases described above (see 'Natural history' above), preoperative diagnosis was made in only 45 percent of cases [8].

Other diagnostic criteria (ie, intact bilateral fallopian tubes and ovaries, absence of uteroperitoneal fistula, attachment of the pregnancy exclusively to the peritoneal surface), known as the "Studdiford criteria," have been described and subsequently modified [19], but are primarily of historical interest only.

DIFFERENTIAL DIAGNOSIS

Tubal pregnancy – Early abdominal pregnancy can be easily misinterpreted as a tubal ectopic if the pregnancy is implanted close to the adnexa. However, the diagnosis of abdominal pregnancy can be excluded when a gestational sac with a yolk sac or embryo is identified in the adnexa at the time imaging, surgery, or during pathology review of the surgical specimen. (See "Ectopic pregnancy: Clinical manifestations and diagnosis", section on 'Diagnosis'.)

Intrauterine pregnancy (IUP) – An abdominal pregnancy can erroneously be reported as an IUP if the relationship between the pregnancy and myometrium is not fully evaluated (see 'Ultrasound' above). The diagnosis of abdominal pregnancy can almost always be excluded when a gestational sac with a yolk sac or embryo is identified in the uterus (the exception is a heterotopic pregnancy including both an intrauterine and abdominal pregnancy).

Similarly, pregnancy in a rudimentary horn of a unicornuate uterus can be misinterpreted as an abdominal pregnancy. However, a rudimentary horn pregnancy, and an associated uterine horn rupture, is not an abdominal pregnancy because the gestational sac and placenta originate inside the uterus. (See "Congenital uterine anomalies: Clinical manifestations and diagnosis", section on 'Unicornuate uterus'.)

Uterine rupture – Uterine rupture, resulting in extrusion of the pregnancy from the uterus into the abdominal cavity, can be misinterpreted as an abdominal pregnancy. However, such patients often have risk factors for uterine rupture (eg, prior cesarean birth, myomectomy). The diagnosis of abdominal pregnancy can be excluded when a complete disruption of the myometrium is visualized intraoperatively. (See "Uterine rupture: Unscarred uterus", section on 'Diagnosis' and "Uterine rupture: After previous cesarean birth", section on 'Diagnosis'.)

MANAGEMENT — As with other high-risk pregnancies, patients with abdominal pregnancy should transferred, whenever possible, to a tertiary care hospital in which a multidisciplinary care team (eg, maternal-fetal medicine, neonatology, gynecology oncology specialists), a variety of treatment options (eg, uterine artery embolization [UAE]), and blood bank services are available. Management depends on many factors (eg, hemodynamic stability, fetal status [ie, live versus demised], gestational age [ie, ≤20 or >20 weeks] at which the abdominal pregnancy was diagnosed).

Patients with hemodynamic instability or embryonic/fetal demise — A patient with hemorrhage and existing or impending hemodynamic instability or with embryonic/fetal demise require surgical removal of the pregnancy. This is often performed with laparotomy, but laparoscopic removal has been described. (See 'All other patients' below.)

Selective arterial embolization may be performed in some patients prior to surgical management to reduce blood loss. This technique is similar to that of UAE; however, in the case of abdominal pregnancy, thrombotic agents are placed into the branches of the internal iliac arteries that are feeding the area of bleeding. (See "Uterine fibroids (leiomyomas): Treatment with uterine artery embolization", section on 'Embolization'.)

Management of the placenta is described below. (See 'Managing the placenta' below.)

Hemodynamically stable patients with live gestations — There is no evidence-based management strategy for hemodynamically stable patients with a live abdominal pregnancy; patients may choose expectant management or termination and the decision is generally determined by gestational age. In this topic we use 20 weeks as the threshold at which to base management decisions; this is consistent with published studies [1]. As the lower limit of fetal viability is approximately 22 weeks of gestation, pregnancies advancing beyond this threshold (>20 weeks) may attain fetal viability and thus have a chance (although not a high likelihood) of newborn survival; by contrast, pregnancies well below this threshold (≤20 weeks) do not (see "Periviable birth (limit of viability)"). However, while allowing a gestation to advance beyond 20 weeks may improve fetal survival, it also confers an increased risk to the mother (ie, maternal morbidity and mortality).

Other factors (eg, patient preference, experience of the physician caring for the patient, presence of a fetal anomaly or severe oligohydramnios, access to a tertiary care hospital), will also guide management decisions.

>20 weeks and choosing expectant management — Patients >20 weeks and choosing expectant management are typically monitored in the inpatient setting and managed similarly to patients with placenta accreta spectrum disorders (see "Placenta accreta spectrum: Management"). The optimal timing of inpatient hospitalization is unclear and there are no prospective studies or randomized trials regarding the gestational age at which admission should occur. Case reports including patients with advanced abdominal pregnancy describe inpatient management beginning at the time of diagnosis [14].

Periodic fetal assessment with both ultrasound (eg, every two to four weeks) and magnetic resonance imaging (MRI; eg, every four weeks) are used to evaluate fetal growth, amniotic fluid levels, the integrity of the gestational sac, placental invasion into surrounding structures, and for preoperative planning. (See 'Imaging findings' above.)

In hemodynamically stable patients, delivery is typically planned between 32+0 and 35+6 weeks of gestation; key features of delivery include the following:

Preoperative placement of catheters for selective arterial embolization may be performed and used prophylactically after delivery of the fetus or in the event of intraoperative hemorrhage [1]. (See 'Patients with hemodynamic instability or embryonic/fetal demise' above.)

Spinal anesthesia may be used for delivery and subsequently converted to general anesthesia. (See "Anesthesia for cesarean delivery".)

A vertical skin incision is preferred to provide maximal abdominal exposure. (See "Incisions for open abdominal surgery", section on 'Choice of incision'.)

Amniotomy of the gestational sac is followed by delivery of the infant and ligation of the umbilical cord at its placental insertion site.

Management of the placenta is described below. (See 'Managing the placenta' below.)

All other patients — For hemodynamically stable patients with gestations ≤20 weeks or those >20 weeks choosing termination, we perform surgical management. While this is usually performed with laparotomy, laparoscopic removal has been described [20,21]. Management of the placenta is described below. (See 'Managing the placenta' below.)

Adjuvant therapies (eg, intraabdominal sac injection of potassium chloride [22], methotrexate [23], selective arterial embolization [20,24]) have limited evidence for efficacy, but may be used in selected patients (eg, patients in whom the abdominal pregnancy has colonized vessel-rich areas [such as the liver or spleen], patients with pregnancies >20 weeks of gestation) to help prevent intraoperative hemorrhage.

Managing the placenta — The optimal management of the placenta is unclear and various methods (eg, removal of the placenta at the time of surgery, leaving the placenta in situ, pre- or postoperative methotrexate, selective embolization of the placental bed) have been described [1]. The choice of procedure is often individualized and determined by the site of placental localization, and perceived risks of hemorrhage and damage to involved structures (eg, bowel). Preoperative imaging may aid in this decision. (See 'Magnetic resonance imaging' above.)

If removal of the placenta is attempted, the placental blood supply is identified and ligated, and is similar to the removal in patients with placenta accrete spectrum disorder (see "Placenta accreta spectrum: Management"). Meticulous dissection is required when placental invasion involves vessel-rich (eg, iliac vessels, hepatic portal, spleen) or mobility-poor (eg, pelvic ligaments) regions [4]. Residual placenta at these sites may result in late post-operative bleeding, even in the absence of placental blood flow (as documented by Doppler sonography) or normalization of human chorionic gonadotropin (hCG) levels.

In cases in which the placenta cannot be removed, it may be left in situ. However, adverse events (eg, secondary hemorrhage, sepsis, failure of lactation, disseminated coagulation, fistulae formation, bowel obstruction) have been reported with this approach. In such cases, postoperative adjuvant therapies (eg, selective arterial embolization, methotrexate, mifepristone) may be used to induce degradation of the placenta and decrease risk of intraabdominal hemorrhage [4]. However, use of postoperative methotrexate can lead to rapid placental lobular necrosis, resulting in intraabdominal abscesses, hemorrhage, and even death [3].

No cases of postoperative placenta growth or malignancy have been reported.

FOLLOW-UP — Long-term follow-up is needed; however, the duration and type of follow-up depend on the treatment modalities used.

Measurements of serum human chorionic gonadotropin (hCG) levels are typically obtained weekly in all patients until the level remains undetectable.

Ultrasound with Doppler is also used to document resolution of the placenta and its blood supply, which often occurs after normalization of hCG levels. Resorption is also typically incomplete and ossified placenta has been described at follow-up intervals of at least two years [4].

FUTURE PREGNANCIES — Successful pregnancy outcomes after abdominal pregnancy have been reported. In one case, spontaneous conception resulted in an intrauterine pregnancy and a full-term, liveborn infant was delivered by cesarean birth 17 months after the preceding abdominal pregnancy; the residual placenta from the abdominal pregnancy did not appear responsive to hormone stimulation [20].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Obstetric hemorrhage" and "Society guideline links: Ectopic pregnancy".)

SUMMARY AND RECOMMENDATIONS

Terminology – Abdominal pregnancy is a rare form of ectopic pregnancy in which the pregnancy implants within the peritoneal cavity, exclusive of the fallopian tubes, ovaries, broad ligament, and cervix. It is further classified as early (≤20 weeks of gestation) or advanced (>20 weeks of gestation). (See 'Introduction' above and 'Terminology' above.)

Risk factors – Risk factors for abdominal pregnancy are extrapolated from data on tubal ectopic pregnancy (table 1) and include tubal factor infertility and higher number of embryos transferred during in-vitro fertilization (IVF). (See 'Risk factors' above.)

Natural history – Abdominal pregnancy is associated with high maternal and fetal morbidity (eg, maternal hemorrhage, fistula formation, fetal malformation) and mortality. However, some abdominal pregnancies may continue to a viable gestation and successful pregnancy outcomes have been reported. (See 'Natural history' above.)

Diagnosis – The diagnosis is made by visualization (with fetal imaging [ie, transvaginal ultrasound, magnetic resonance imaging [MRI]] or intraoperatively during surgical exploration) of an extrauterine pregnancy and an intact uterus (ie, no evidence of uterine rupture). The majority of patients are diagnosed at the time of surgery. (See 'Diagnosis' above.)

Management – Patients with abdominal pregnancy should be transferred, whenever possible, to a tertiary care hospital in which a multidisciplinary care team (eg, maternal-fetal medicine, neonatology, gynecology oncology specialists), a variety of treatment options (eg, uterine artery embolization [UAE]), and blood bank services are available. (See 'Management' above.)

Patients with hemorrhage and existing or impending hemodynamic instability or with embryonic/fetal demise require surgical removal of the pregnancy. (See 'Patients with hemodynamic instability or embryonic/fetal demise' above.)

Hemodynamically stable patients may choose expectant management or termination; the decision is generally determined by gestational age, but other factors (eg, patient preference, experience of the physician caring for the patient, presence of a fetal anomaly or severe oligohydramnios, access to a tertiary care hospital) also guide management decisions. (See 'Hemodynamically stable patients with live gestations' above.)

-Patients >20 weeks and choosing expectant management are typically monitored in the inpatient setting with periodic fetal assessment and planned delivery between 32+0 and 35+6 weeks of gestation. (See '>20 weeks and choosing expectant management' above.)

-For patients with gestations ≤20 weeks or those >20 weeks choosing termination, surgical management is typically performed; adjuvant therapies (eg, intraabdominal sac injection of potassium chloride, methotrexate, selective arterial embolization) may be used to help prevent intraoperative hemorrhage. (See 'All other patients' above.)

The optimal management of the placenta is unclear and various methods (eg, removal of the placenta at the time of surgery, leaving the placenta in situ, pre- or postoperative methotrexate, selective embolization of the placental bed) may be used. Preoperative imaging may aid in this decision. (See 'Managing the placenta' above.)

Follow-up – Long-term follow-up is needed and includes measurements of serum human chorionic gonadotropin (hCG) levels until the level remains undetectable and ultrasound with Doppler to document resolution of the placenta and its blood supply. (See 'Follow-up' above.)

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Topic 132045 Version 4.0

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