TOG Advanced Abdominal Pregnancy 2022

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DOI: 10.1111/tog.

12808 2022;24:195–204
The Obstetrician & Gynaecologist
Review
http://onlinetog.org

Advanced abdominal pregnancy: challenges, update and


review of current management
Anna McDougall MRCOG,a* Amelie Morin MSc PhD MRCOG,
a
Tatsiana Kuzmich MD FRCR,
b

Funlayo Odejinmi MSc FRCOGc


a
Specialist Registrar in Obstetrics and Gynaecology, Whipps Cross University Hospital, Barts Health NHS Trust, Whipps Cross Road, London E11
1NR, UK
b
Consultant Radiologist, Whipps Cross University Hospital, Barts Health NHS Trust, Whipps Cross Road, London E11 1NR, UK
c
Consultant Obstetrician and Gynaecologist, Clinical Director Gynaecology, Whipps Cross University Hospital Barts Health NHS Trust, Whipps
Cross Road, London E11 1NR, UK
*Correspondence: Anna McDougall. Email: anna.mcdougall@nhs.net

Accepted on 22 April 2021.

Key content removal increases the risk of maternal morbidity, it may be


 Although extremely rare, advanced abdominal pregnancy (AAP) is left in situ.
associated with considerable maternal and fetal morbidity and
Learning objectives
mortality. However, when diagnosed early and managed  To outline the classification of AAP.
appropriately, it is possible to have successful outcomes. 
 There are no specific criteria to diagnose AAP and it may be missed
To understand the diagnostic challenges and the role of imaging in
preoperative assessment.
on ultrasound. Magnetic resonance imaging is the gold standard  To describe the different management options and associated
for evaluating placental implantation and preoperative planning.
 Management depends on the gestational age at diagnosis, with
ethical issues.
consideration of termination, preterm delivery and conservative Ethical issues
management until further fetal maturation.  There is no established guidance for the diagnosis and
 Multidisciplinary preoperative planning is paramount for optimal management of AAP.
outcome. Delivery is recommended in a tertiary centre with access  AAP requires careful evaluation when deciding whether to
to interventional radiology. terminate the pregnancy or provide expectant management.
 Management of the placenta depends on the degree of  Informed consent requires acknowledgement of the
penetration and the organ in which it embeds. Where the risk of considerable risks.

Please cite this paper as: McDougall A, Morin A, Kuzmich T, Odejinmi F. Advanced abdominal pregnancy: challenges, update and review of current management.
The Obstetrician & Gynaecologist 2022;24:195–204. https://doi.org/10.1111/tog.12808

Introduction
(1013–1106).2 In 1500, Jacob Nufer, a Swiss swinegelder,
Abdominal pregnancy is a rare form of ectopic pregnancy in successfully delivered his wife’s abdominal pregnancy. The
which implantation occurs within the abdominal cavity, resultant child lived into their seventies and his wife had a
exclusive of ovarian, tubal and intraligamentary pregnancies. further four pregnancies. Over the last century, numerous
The exact incidence remains unknown owing to the case reports and small case series have been published. In the
differences in gestational ages included in most 1960s the associated fetal survival was just 5%.3 In the 1980s,
publications; however, estimates are between 1/10 000 and despite widespread use of antenatal ultrasound, clinical
1/30 000 live births, accounting for approximately 1% of all recognition of abdominal pregnancy remained a problem,
ectopic pregnancies.1 with only one of nine women with an accurate preoperative
diagnosis of abdominal pregnancy reaching hospital alive.1
With advances in obstetric care, particularly those relating to
Historical aspects
management of massive haemorrhage and neonatal care,
Abdominal pregnancy is the oldest documented form of survival has improved. Recent estimates of maternal
ectopic pregnancy. Its first description was by Abbucasis mortality are 0–12%,4–6 and fetal survival is over 78%.7

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Advanced abdominal pregnancy

outcome. Perhaps a more clinically useful definition of


Literature search
abdominal pregnancy is ‘an extrauterine pregnancy in which
We searched the literature databases PubMed, BNI, CIANHL, all or most of the fetus develops within the
EMBASE, EMCARE, MEDLINE and UpToDate using abdominal cavity’.16
the keywords “advanced abdominal pregnancy” or “late
abdominal pregnancy” to find relevant publications. We
Risk factors
selected case reports from 1980 to present; key older
articles were only included in the historical section. As with other ectopic pregnancies, most women who present
Additional relevant articles referenced in our selected with AAP have no identifiable risk factors. Others, however,
articles were manually added. We reviewed 130 articles, have the same risk factors as other forms of ectopic
mostly case reports (single or series) and a few reviews. A pregnancy; for example, previous ectopic pregnancy, tubal
total of 230 cases of advanced abdominal pregnancy (AAP) pathology including surgery or infection and current use of
were described. an intrauterine contraceptive device (IUCD).17,18 Particular
risk factors for abdominal pregnancies are the presence of
uterine anomalies and a history of previous uterine surgery,
Classification and pathophysiology
especially caesarean section.11,12,19 The silent rupture theory
Abdominal pregnancies may be classified by gestation at accounts for the occurrence of reimplantation of the
diagnosis and implantation site. The term ‘advanced pregnancy in the abdominal cavity following rupture from
abdominal pregnancy’ (AAP) applies to abdominal a congenital or iatrogenic uterine defect. AAPs have been
pregnancies after 20 weeks of gestation. Early abdominal described post scar rupture,19 previous myomectomy,20,21
pregnancy (EAP), on the other hand, presents before and post uterine perforation at surgical termination of
20 weeks of gestation. Once EAP is diagnosed, immediate pregnancy.22–24 They have also been described following
termination of pregnancy is recommended, either medically in vitro fertilisation (IVF) treatment25 and in the context of a
or surgically, as the risk of intra-abdominal bleeding prolonged history of subfertility.26
outweighs the benefit of prolonging the pregnancy A higher number of AAPs are reported in developing
to viability.8 countries: 166 (72%) versus 64 (28%) from our literature
While a distinction has been made between primary and review. This may be associated with a combination of
secondary implantation, clinically, this is difficult to prove. increased reporting, increased rates of untreated pelvic
Theoretically a primary abdominal pregnancy occurs when infections and lack of access to antenatal care
implantation occurs directly in the abdominal cavity, while and ultrasound.27,28
secondary abdominal pregnancy occurs when the conception
is extruded from its primary seat of implantation and
Clinical presentation
reimplants in the abdominal cavity.2 In 1942, Studdiford9
proposed the following minimum criteria to define a primary AAP is theoretically preventable, but the absence of
abdominal pregnancy: consistent clinical features and a lack of clinician suspicion
 Normal tubes and ovaries with no evidence of recent or mean the diagnosis is frequently missed. Despite focused
remote injury antenatal care and advances in ultrasound technology, only
 Absence of any evidence of a uteroperitoneal fistula around half of all cases are diagnosed preoperatively.4,6
 Presence of a pregnancy related exclusively to the There are no specific symptoms or signs of AAP. The
peritoneal surface and early enough to eliminate the commonest presentation is abdominal pain with or without
possibility of secondary implantation following a primary vaginal bleeding.5,29–31 The pain is often persistent
nidation in the tube. throughout the pregnancy and exacerbated by fetal
Secondary abdominal pregnancies may result when the movements, potentially associated with the lack of uterine
developing fetus reimplants within the abdominal cavity, wall cushioning.32 Masukume33 describes the conservative
usually following a ruptured tubal ectopic pregnancy. More management of a patient with intermittent abdominal pain
rarely, abdominal pregnancies can occur following rupture of a from 8 weeks of gestation. The subsequent diagnosis of AAP
rudimentary horn10,11 or dehiscence of a uterine scar.12 was only made at 40 weeks of gestation, following admission
Abdominal pregnancy has been described after hysterectomy,13 with 3 days of constant periumbilical pain.
and there are several case reports of heterotopic The diagnosis of AAP is commonly made following fetal
abdominal pregnancies.14,15 demise.19,29,34–38 Numerous cases describe the diagnosis of
In practice, primary and secondary classifications are of AAP intraoperatively only after failure to induce
academic interest only and rarely influence management or labour.10,34,37,39 An oxytocin stress test (absence of uterine

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contraction with oxytocin administration) has previously routine, confirmation of the location of the gestational sac in
been used to aid diagnosis.40 relation to the cervix, endometrial cavity and uterus.
The nonspecific nature of the pain has led to various Standard ultrasound scanning techniques with 3.5-MHz
misdiagnoses, including fibroid degeneration,41 bowel curvilinear probe, including Doppler assessment, are used. In
obstruction, uterine rupture38 and septic peritonitis.42 In a slim women, supplementing the scan with higher resolution
review of 11 cases of AAP,43 six women presented with acute transabdominal linear probes to achieve greater spatial
abdominal pain in the third trimester, but the diagnosis of resolution, as well as 3D-enhanced techniques and
abdominal pregnancy was made only at surgery. endocavitary probes, can be employed as necessary.
A displaced cervix, usually anteriorly, has been described as Intra-abdominal gestation is usually suspected when
a reasonably reliable sign of AAP.5,23,29,44 However, it could ultrasound reveals extrauterine amniotic sac and an empty
also be mistaken for a sign of early labour, or not appreciated uterine cavity.51 With careful scanning technique and skill,
as a potential clue at the time. Bloating and persistent confident sonographic diagnosis can be achieved, which
vomiting are common;29,45,46 in one case, this necessitated relies on demonstration of an enlarged ‘empty’ uterus
delivery at 33 weeks of gestation owing to its severity.47 Not lacking intrauterine pregnancy, visualisation of fetus and
uncommonly, AAP is diagnosed on further evaluation of – placenta outside the uterus, and lack of uterine
and even at – caesarean section for a suspected myometrium around the fetus.52 The latter is recognised
placenta praevia.38,48,49 as a fetus lying unusually close to the maternal abdominal
Other signs suggestive of AAP include severe anaemia, wall (Fig. 1A, 1B). Fetal viability can be confirmed in the
abnormal fetal lie, oligohydramnios and a small-for- usual way by assessing the fetal heart and fetal movements.
gestational-age fetus. The signs and symptoms of AAP are Colour Doppler can assess umbilical cord and placental
summarised in Table 1. However, no sign is pathognomonic waveforms (Fig. 1C).
and each of these may commonly be associated with other However, even when intra-abdominal pregnancy is
conditions pre-existing or arising in pregnancy. A recognised during the scan, the role of ultrasound in the
combination of some of these should at least lead assessment of the extrauterine placenta and particularly its
obstetricians to include AAP in their differential diagnosis. points of adherence to solid organs, including bowel and
Awareness of the condition is key to preoperative diagnosis. omentum in the peritoneal cavity, is limited.51 In addition,
the referring clinician should be aware of the inherent
Ultrasound weaknesses of ultrasound related to scanning conditions,
Given the lack of specific signs to aid clinical diagnosis of artefacts, patient’s body build and operator dependence,
intra-abdominal pregnancy, early radiological diagnosis is which may limit the usefulness of this modality. Therefore, if
vital for improving the outcomes in AAP.50 Abdominal AAP is suspected, magnetic resonance imaging (MRI)
pregnancy is harder to diagnose with advancing gestation. assessment is advisable.
Any first trimester ultrasound assessment should include, as
Magnetic resonance imaging
MRI is the imaging modality of choice in the evaluation of
suspected intra-abdominal pregnancy and is the mainstay for
Table 1. Symptoms and signs of advanced abdominal pregnancy
surgical planning. The lack of ionising radiation and the
Symptoms Signs widely accepted safety of MRI techniques, combined with
superior soft tissue resolution, have increased use of this
modality during pregnancy.53 Standard MRI protocols
Abdominal pain Palpable fetal parts
performed on either 1.5 T or 3.0 T units include T1 and
Vaginal bleeding Deviated cervix T2-weighted sequence in all three planes (axial, coronal and
sagittal) supplemented by fat suppressed sequence (SPIR)
Painful fetal movements Anaemia weighted sequence and magnetic resonance angiography
Reduced fetal movements Abnormal fetal lie (MRA) with time-of-flight technique. Neither oral nor
intravenous contrast are essential.
Bloating Small for gestational age Similar to ultrasound, MRI demonstrates intra-abdominal
pregnancy as a fetus with a placenta situated outside the
Vomiting Oligohydramnios
uterus and an empty uterine cavity (Fig. 2A, B). In addition
Failed induction of labour to ultrasound, MRI can evaluate the sites of placental
attachment to surrounding visceral organs, including
bowel, liver and spleen, while angiographic MRI sequences

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Advanced abdominal pregnancy

Figure 1. Ultrasound appearance of a 22 week abdominal pregnancy in a 35-year-old woman. (a) Oblique ultrasound image of right flank using
3.5 MHz convex probe, showing cross-section of the fetal abdomen (arrows, callipers) seen lying freely in intra-abdominal cavity beneath maternal
anterior abdominal wall (A). No uterine wall is seen between the fetus and maternal abdominal wall. (b) Transverse ultrasound colour Doppler
image of right flank using 3.5 MHz convex probe, showing cross-section of the fetal chest (arrows, callipers) lying free in intra-abdominal cavity
beneath maternal anterior abdominal wall (A). The heart activity is seen on colour Doppler. (c) Longitudinal ultrasound image of midline lower
abdomen with 3.5 MHz convex probe, depicting placenta (asterisk) lying superior and outside to the uterus (U) seen intimately related to it.

can identify potential vascular connections, particularly the


Management
origin of major arterial feeders to the placenta (Fig. 2C, D).
This is crucial for operative planning to minimise The management of an abdominal pregnancy depends on the
intraoperative injury and avoid disastrous haemorrhage gestational age at diagnosis. Termination of pregnancy is
during placental removal.54,55 recommended for EAP. When an AAP is diagnosed at a
When intraabdominal pregnancy is discovered, MRI viable gestational age, it may be reasonable to consider
reporting protocols should include the following related delaying surgical delivery until an acceptable level of fetal
to the fetus, amniotic sac, placenta, uterus and maturity has been achieved. With advances in neonatal care,
maternal comorbidity: fetal survival rates have improved.43 Cases diagnosed at the
 Fetus: viability, lie, position, and relation to the uterus and threshold of viability present a unique management
maternal intra-abdominal organs; presence of congenital challenge. There is no evidence-based approach guided by
abnormalities; signs of fetal demise/maceration/hydrops prospective case studies or randomised trials regarding
 Amniotic sac: oligohydramnios; signs of rupture of appropriate treatment plans. Although attempts to prolong
amniotic membrane pregnancy can potentially improve chances of fetal survival,
 Placenta: position, site and extent of implantation; blood this objective must be balanced against increased risk of
supply; presence of placental adherence to the surrounding haemorrhage.4 Thus, management decisions require a
organs; presence of bleeding from placental bed or multidisciplinary team (MDT) approach, informed consent
placental infarction and careful consideration of the ethical issues.
 Uterus: assessment of uterine cavity, integrity of cervix,
uterine wall; signs of uterine rupture Pregnancy termination
 Presence of intra-abdominal fluid or haemoperitoneum When a pre-viable AAP is diagnosed, termination of
 Maternal findings and comorbidities. pregnancy should be recommended. In the UK, feticide is

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Figure 2. Magnetic resonance imaging (MRI) appearances of a 22 week abdominal pregnancy in a 35-year-old woman. (a) FIESTA-weighted
coronal and (b), T2-weighted coronal multiparametric reformatted MRI images, depicting a fetus (arrow) lying freely in the abdominal cavity
longitudinally in the right flank with the head in the right hypochondrium seen next to the liver (L). The placenta (asterisk) is seen in intra-
abdominal space, superior to the uterus (arrowheads) and adherent to the uterine fundus. The uterine cavity (C) is empty. (c) coronal and (d) axial
time of flight magnetic resonance angiography (MRA) images demonstrate placenta (asterisk) adherent to the outer side of the fundus of the
uterus (U) and feeding arteries of the placenta arising from the hypertrophied left uterine artery (arrows).

required for termination after 21+6 weeks of gestation,56  Inpatient stay because of the significant risk of life-
which is commonly performed by intracardiac potassium threatening intra-abdominal haemorrhage with
chloride injection. Women must be supported in their venous thromboprophylaxis
decision making, as termination may be unacceptable to  Regular assessment of maternal wellbeing: use of the
some women and their families, regardless of maternal and Modified Early Obstetric Warning Score; full blood count
fetal risk, for religious, cultural or personal reasons. at regular intervals, plus a blood group and screen at
intervals to ensure constant validity of sample
Expectant management  Regular assessment of fetal wellbeing: this may be
Expectant management may be entertained and result in performed by minimum fortnightly ultrasound
successful outcomes for mother and baby in the absence of assessment of fetal growth, liquor volume and Doppler
other complicating factors, and where comprehensive in addition to cardiotocography and maternal recognition
counselling of the mother has been carried out. Where of fetal movements
oligohydramnios is present, careful consideration must be  24-hour access to blood products
given to the suitability of continuing the pregnancy owing to  Access to interventional radiology
the significant risk of pulmonary hypoplasia and fetal  Continued MDT input.
compression deformities.57
Minimum requirements for expectant management of an Timing of delivery
AAP should be: Timing of delivery should be individualised. The risks of fetal
 Confirmation of diagnosis prematurity must be balanced against those of maternal
 Placenta localisation wellbeing. As the placental mass increases, so do the risks of

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Advanced abdominal pregnancy

haemorrhage and of complications associated with advanced


Box 1. Preoperative planning checklist
placental invasion, such as bowel and bladder injury. In
addition, the risk of gestational sac rupture increases beyond Pre-surgery
34 weeks of gestation.14 Ombelet (1988)35 advocates  Optimise maternal haemoglobin (iron supplementation/blood
conservative management until 34 weeks of gestation, but transfusion)
 Preoperative MRI
admits that with better neonatal intensive care this delay is  Informed consent including additional procedures as per placental
probably unnecessary. There is no clear cut-off and it is our assessment
opinion that, in the absence of complicating factors, delivery
should be considered from 30 weeks of gestation. The Setting
 Tertiary centre hospital inpatient
appropriate level of neonatal care must be organised and
may require in utero transfer, depending on gestation. Multidisciplinary team
 Senior obstetrician-gynaecologist and senior assistant
 Fetal medicine specialist
Surgical planning and management  Senior anaesthetist
 Haematologist
AAP can only be delivered surgically. Maternal  Senior Interventional radiologist
haemodynamic instability at presentation or during  Surgical team (urology, colorectal, upper gastrointestinal, vascular,
experienced gynecology surgeon)
admission would be an indication for emergency delivery
 Senior neonatologist
by laparotomy. In other circumstances, delivery is
undertaken electively or semi-electively. Once delivery has
been scheduled, thorough surgical planning is key to may be required,such as omentectomy, adnexectomy or
optimising maternal and fetal outcomes.4 The risk of hysterectomy.16,62 This review of published cases revealed
catastrophic haemorrhage at delivery is significant, and pre- rates of hysterectomy of 12.6% and unilateral or bilateral
operative measures should aim to minimise bleeding and its salpingo-oophorectomy or adnexectomy of 12.7%.
consequences. For this purpose, blood products and cell
salvage should be available. The patient should be kept in
The placenta
hospital, and transfer to a tertiary centre with 24-hour access
to interventional radiology is recommended.58 Risks must be Placental site
explained thoroughly to the mother. The site of placental implantation can be on any single or
An MDT meeting involving a senior obstetrician- multiple structure within the abdominal cavity (Table 2).
gynaecologist, fetomaternal medicine specialist, The commonest cause of morbidity and mortality in
neonatologist, anaesthetist, radiologists and surgical teams abdominal pregnancy is deep implantation of the placenta
should take place promptly following diagnosis and at regular on highly vascular intra-abdominal structures.
intervals until delivery. Information about placental The uterus and adnexae are the commonest sites of
attachments and the position of the amniotic sac obtained implantation. Evidence suggests uterine attachment is
from MRI will guide the best location of abdominal incision associated with better outcomes and less bleeding risk
to avoid the placenta.19 The operating team must include an compared with other abdominal structures. This may be
experienced obstetrician and other members depending on explained by the relatively stable blood supply.14 A series of
placental implantation; for example, a gynaecological 11 AAPs43 described five cases of complete uterine
oncologist, urologist, or a colorectal, upper gastrointestinal attachment in which no intra-abdominal haemorrhage
or vascular surgeon.19,59,60 Box 1 provides a preoperative events were noted and all five fetuses survived. Maternal
planning checklist. morbidity was certainly much less than in the six cases with
A midline or paramedian laparotomy under general partial/complete mesenteric attachment, which all had intra-
anaesthesia is the preferred approach. The incision can be abdominal bleeding events and only three fetuses survived.43
deviated sideways or adjusted in length and position
according to details about placental position.19 Unusual Management of the placenta
thickness of the peritoneum has been described and careful There is no consensus about placental management. Options
dissection may be required to access an avascular window on include removal at the time of delivery or leaving the placenta
the gestational sac and open safely. The fetus should be behind. The option chosen should weigh benefits against the
delivered without disturbing the placenta.61 Bleeding is added morbidity risk of each modality. Historically, leaving
assessed regularly and communication between the surgical the placenta in situ was the preferred approach because of the
and anaesthetic teams is essential. Anatomic derangements risk of catastrophic haemorrhage with attempted removal of
often complicate fetal delivery and increase the risk of a placenta deeply embedded on intra-abdominal vascular
intraoperative injury.16 Consequently, additional procedures structures.58 In contrast with intrauterine deliveries, the usual

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cases in which methotrexate was administered to developing
Table 2. Sites of placental implantation (from 182 cases in which
placental site was specified) placental abscess and ultimately leading to two
maternal deaths.29,59
Literature Uterine artery embolisation has been used as an alternative
Site of placental implantation review (182 Hymel, 2015
(single and multiple) frequency cases) (23 cases)58
strategy to minimise blood loss. It is usually performed
encountered n n (%) immediately after delivery of the baby to minimise bleeding at
surgery and accelerate placental avascularisation in the
postpartum period. This approach has been used successfully
Uterus and adnexa 116 11 (47.8) with gradual decrease in placental volume; low vascularisation
Bowel 75 7 (30.4) on Doppler and MRI and complete resorption at 15 months.61
In a case of fetal demise, Cardosi34 reports successful pre-
Omentum 53 (4.3) delivery selective artery embolisation of bilateral uterine
arteries and a branch of the ileocolic artery to minimise
Broad ligament 28 Not specified
bleeding. The placenta was left in situ owing to multiple
Pelvic wall 19 Not specified attachments, including the bladder dome and parametrium.
Blood loss was 500 mL and bhCG was undetectable after
Abdominal wall 19 (4.3)
67 days. However, embolisation has been linked to the
Pouches surrounding uterus 13 (8.7) development of ileus, sepsis, intestinal perforation and the
need for subsequent placental removal.34,68,58.
Bladder 11 Not specified In our literature review, the placenta was removed at
Peritoneum 11 Not specified
surgery in 137 of 226 cases (61%). Surgical removal was
successful in 55% of cases by Sunday-Adeoye (2011)4 and
Upper abdomen 8 Liver (4.3) 69% of cases by Rohilla (2018).5 Most cases reported are
from developing countries, where there is possibly less access
Major vessels 8 Not specified
to MRI and/or methotrexate, and where arranging regular
follow-up may be difficult.69–71
Single site of insertion encountered in 55 cases (30.2%). In order of The current consensus is to establish the safest management
frequency these were: uterus (24%); bowel (16%); tubes (15%);
broad ligament (7%); omentum (7%); pouch of Douglas (5%); liver based on MRI data: if safe to do so, removal at surgery is the
(4%) and other (12%). preferred method. If the placenta is invading major vascular
Multiple sites of insertion encountered in 125 cases (69%). structures, artery ligation or embolisation can be performed
before surgical removal. If the placenta cannot be safely
removed, the cord should be clamped and cut as close as
haemostatic mechanisms of uterine contraction and possible to the placental mass. The placenta is left in situ with
subsequent spiral artery constriction in the third stage are further monitoring to ensure adequate resorption.
absent in extrauterine pregnancies.
Leaving the placenta behind increases the risk of maternal
Complications
morbidity related to complications such as placental mass
abscess,29,61 leading to sepsis and necrosis.19,28,63 Other Fetal complications
reported sequalae include paralytic ileus, secondary Oligohydramnios is common in AAP and although there are
haemorrhage, disseminated intravascular coagulation,5 cases of successful outcomes associated with this, there is a
return to theatre,19,63,64 fistulae formation,65 persistent significant risk of pulmonary hypoplasia and compressive
hydronephrosis66 and protracted hospital stay. This approach deformities. In our search, significant fetal deformities were
also requires follow up with regular ultrasound scans and/or documented in 26 cases, but not universally reported. The
serum beta-human chorionic gonadotrophin (bhCG) levels to largest review of fetal outcomes in 1161 AAPs since 1809
assess placental involution.36 Although structural involution revealed a combined rate of fetal deformation and
can be very slow (up to 5.5 years), the hormonal function malformation of 21%, compared with a background risk of 2
declines more rapidly (10 days to 7 weeks).30,41,67 and 4%, respectively.7 In order of frequency, these included
To accelerate resorption when the placenta is left behind, facial/cranial asymmetry, joint abnormalities/webbing, Talipes
methotrexate has been administered, but cannot be routinely equinovarus, scoliosis/kyphosis, pulmonary hypoplasia and
recommended because of a significant risk of infection. torticollis. In addition, fetal growth restriction was
Hymel29 describes the development of intra-abdominal documented in 24%.7 These may be attributed to both
abscess in 80% of the six cases in which methotrexate was extrinsic compression within the abdominal cavity and
given with placenta in situ. Rahman59 reports all of the five vascular disruption with extrauterine placentation.

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Advanced abdominal pregnancy

Intra-abdominal fetal demise occurs frequently, as shown Wide variation exists in reported maternal mortality rates.
in 80/224 (36%) published cases we reviewed. Neonatal Several case series and literature reviews report rates of 0–
deaths secondary to prematurity, pulmonary hypoplasia, 5%,4,5,30,35,40,43 while in a review of 163 cases in 13 countries
severe deformities and chorioamnionitis were reported in 25 between 1946 and 2008, the rate was 12%.6 The overall risk of
cases. The perinatal mortality rates reported in the literature death has been estimated at 7.7 times that of tubal ectopic
range from 72–83%.6,30,35 The review of fetal outcomes by pregnancy and 90 times that of an intrauterine pregnancy.1
Stevens (1993)7 suggests that advances in maternal and There were eight recorded maternal deaths found in our
neonatal care have led to better survival, with 78% survival literature search; seven of these at gestations beyond
rate of live born infants born beyond 30 weeks of gestation in 26 weeks and secondary to haemorrhage, sepsis and
1971–1990 compared with 55% before 1933. pulmonary embolism.
There is a paucity of data on long-term outcomes. A Recurrence of AAP appears to be exceptionally rare,
review of 40 surviving neonates reported high rates of presumably owing to the intense scrutiny under which
chronic pulmonary disease, spasticity and abnormal further pregnancies are held. The only case we are aware of
development.7 Sequalae of prematurity may be describes surgical management of an early ectopic pregnancy
encountered. Unfortunately, the full effect on the child of followed by two AAPs, both presenting in the third trimester
an intra-abdominal gestation cannot be fully estimated until with placental abruption and fetal demise.77 Long-term
more robust data is available. follow up and subsequent pregnancy data are inadequate for
meaningful analysis, but successful pregnancies after AAP
Maternal complications have been reported.5 Any further pregnancy should ensure
The greatest risk in an AAP is that of life-threatening intra- thorough evaluation of pregnancy location at 6–8 weeks of
abdominal haemorrhage. As previously described, the risk gestation and increased obstetric surveillance throughout.
appears to be less when the placenta is attached to the uterus
compared with highly vascular structures such as liver or
Ethical considerations
spleen.43 Massive intra-abdominal haemorrhage was the
cause of death in a previously undiagnosed 26-week AAP,72 Given its rarity, a clinician may never encounter an AAP in
and in 10 AAP cases described by Worley et al.,16 30% their practice, thus an awareness of this diagnosis is essential.
required emergency laparotomy for intra-abdominal Meticulous first-trimester ultrasound assessment of
haemorrhage. This risk underpins the necessity for pregnancy location can allow timely diagnosis, treatment
inpatient surveillance with 24-hour access to blood products. and prevention of the continuation into advanced gestations.
The presence of an intra-abdominally growing fetus and AAPs are commonest in developing countries where access
placenta are frequently associated with persistent and or to antenatal care and ultrasound may be limited. However,
worsening abdominal pain. AAPs have been described in the we should also face the sad reality that even in developed
context of acute intestinal obstruction,73 bilateral ureteral counties with well-established antenatal services, vulnerable
obstruction74 and bilateral hydronephrosis.75 Pre-eclampsia women such as asylum seekers and those from poor
is not uncommon in AAPs. It is likely in such cases that the socioeconomic backgrounds may have reduced access to care.
ectopic placentation results in defective endovascular Unlike EAP, where termination of the pregnancy is the
trophoblastic invasion.16 accepted recommendation, there is no guidance for the
Intra-operative haemorrhage with handling of the placenta diagnosis and management of AAP. Pre-delivery diagnosis
is frequently encountered and subsequent blood transfusion best serves the patient by allowing comprehensive assessment.
is required in 70–90% of patients.16 Infective complications An informed decision can only be made once the potential
encompassing minor wound infection, placental abscess, maternal and fetal consequences of each management option
fistulae and peritonitis were documented in 15% of the cases have been explained.
we reviewed. Re-laparotomy may be necessary either in the Such decision making may be easier at early and late
immediate postpartum period or several months later when pregnancy gestations, but controversy arises in mid-
associated with placental mass.25 gestations, especially pre-viability, when fetal survival must
A truly remarkable phenomenon associated with be balanced against maternal morbidity. Neonatal care has
undiagnosed, undelivered AAP is that of lithopedion improved significantly in recent years. Nevertheless, survival
(‘stone child’), in which the retained fetus becomes versus neurological damage, disability and quality of life must
calcified within the abdominal cavity. Typically, this is an be clearly explained to parents.
asymptomatic incidental finding, which may be discovered Women and their families must be supported in their
decades after a pregnancy event, as illustrated by the case of decision making. The provision of continuing language
an 80-year-old woman investigated for abdominal pain in interpretation may be required to engage effectively in the
whom a 34-week lithopedion was discovered.76 care process. This should include written consent stating

202 ª 2022 Royal College of Obstetricians and Gynaecologists.


17444667, 2022, 3, Downloaded from https://obgyn.onlinelibrary.wiley.com/doi/10.1111/tog.12808 by Cochrane Malta, Wiley Online Library on [04/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
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J Med Genet 1993;47:1189–95.
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requires a high index of clinical suspicion, but where made 1942;44:487–91.
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13 Yesßilyurt H, Ozyer € Mollamahmuto
Sß, Uzunlar O, glu L. Abdominal pregnancy
are best managed. Given the rarity of this condition, a
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lessons from three cases from Zimbabwe and a literature appraisal of
diagnostic and management challenges. Womens Health (Lond)
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21 Pieh-Holder KL, Scardo JA, Costello DH. Lactogenesis failure following
AMD and AM designed the article and contributed equally to successful delivery of advanced abdominal pregnancy. Breastfeed Med
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22 Zhang J, Li F, Sheng Q. Full-term abdominal pregnancy: a case report and
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approved the final version. management of second trimester abdominal pregnancy. BMJ Case Rep
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