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General Gynecology
Expert Reviews
Cervical pregnancy
Less than 1%, and the rarest, of ectopics
are implanted within the cervical canal
below the level of the internal cervical
os.6,9 The cause of such implantations is
unknown but predisposing factors include prior uterine curettage, induced
abortion, Ashermans syndrome, leiomyomata, presence of an IUD, in vitro
fertilization, and prior in utero exposure
to diethylstilbesterol.10-13
Raskin suggested that the diagnosis by
ultrasound examination of cervical preg-
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General Gynecology
FIGURE 1
Ovarian pregnancy
One half of 1% to almost 3% of ectopics
are implanted within the ovary.6,27,28
Ovarian pregnancy, like other nontubal
ectopic pregnancies, may occur without
the usual expected antecedent risk factors for ectopic pregnancy. The presenting signs and symptoms are similar to
other ectopic pregnancies: positive pregnancy test, abdominal pain, and vaginal
bleeding.
It is difficult to preoperatively make
the diagnosis of ovarian pregnancy. An
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ultrasound finding suggesting ovarian
implantation is a walled cystic mass
within or adjacent to an ovary, but this
does not exclude a corpus luteum and a
tubal implantation. Doppler ultrasound
cannot always distinguish between a corpus luteum and an ovarian pregnancy
implantation. This diagnosis is usually a
pathologic diagnosis (Figure 3): made by
microscopic examination of a surgically
removed adnexal mass, based on Speigelbergs criteria: the tube must be intact
and distinctly separate from the ovary,
the gestational sac must occupy the normal anatomical location of the ovary, the
gestational sac must be connected to the
uterus by the utero-ovarian ligament,
and unquestioned ovarian tissue must be
demonstrated in the wall of the gestational sac.29 Speigelbergs criteria cannot
be established with ultrasound.28
It is important for the laparoscopic
surgeon to understand that an ovarian
pregnancy may look like a hemorrhagic
corpus luteum ovarian cyst on direct inspection, and only the pathology from
cystectomy, if possible, or oophorectomy will reveal the true diagnosis. However, when an adnexal ectopic is diagnosed with a nonsurgical algorithm,
conservative medical therapy can be
successful without a true diagnosis of
location.7,30
Abdominal pregnancy
Less than 1% of ectopic pregnancies are
implanted within the abdominal cavity.6,31 The pathogenesis of abdominal
implantation is controversial. Many are
the result of secondary nidation within
the peritoneal cavity after tubal abortion,
tubal rupture, or uterine rupture.32 True
primary abdominal implantation must
satisfy the criteria of Studdiford.33 Studdiford,33 reporting a primary peritoneal
implantation in 1942, established 3 criteria for such a primary abdominal pregnancy: normal fallopian tubes with no
evidence of recent or remote trauma, the
absence of any uteroperitoneal fistula,
and the presence of a pregnancy related
exclusively to the peritoneal surface and
early enough to eliminate the possibility
of secondary implantation after a primary nidation within the tube.
General Gynecology
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The most common abdominal implantation site is the posterior culde-sac, followed by the mesosalpinx, the
omentum, the bowel and its mesentery,
and the peritoneum of the pelvic and abdominal walls, including the anterior
cul-de-sac31 (Figure 4). Treating primary abdominal pregnancies over a 12year period, Shaw et al31 found 55% in
the posterior cul-de-sac, 27% in the mesosalpinx, 9% in the omentum, and 9%
between the anterior uterine wall and the
bladder. However, pregnancy implantation can occur anywhere in the abdomen, even elsewhere in the body, including the retroperitoneal space, the liver,
the spleen, the appendix, and even the
lung.34-40 The diagnosis of these very unusually ectopic locations is frequently
made at the time of surgical intervention. The evaluation of otherwise unexplained masses or hemorrhage should
include measurement of human chorionic gonadotropin (hCG). Because there
are only individual case reports of such
unusual pregnancy implantations, no
incidence forecast nor treatment guidance can be offered.
Abdominal pregnancies frequently
progress until late diagnosis, defined as
greater than 20 weeks gestation, leading to
a high maternal mortality rate, 0.5-18%,
a rate 8 times greater than that of other
ectopic pregnancies.41,42 As pregnancy advances the placental support for the fetus
usually becomes compromised and the fetus can die. Placental separation with massive intraabdominal bleeding is unpredictable. Because of the high fetal and maternal
mortality rates, such pregnancies should
be terminated as soon as the diagnosis
in confirmed, regardless of gestational
age.31,43 There are reports of individualized management. Given the advances in
neonatology and preterm infant survival,
weighing the risk of the sudden onset of
life-threatening maternal hemorrhage vs
neonatal death must be considered when
diagnosing abdominal pregnancy at or near
neonatal survivability. Expectant management of abdominal pregnancy reaching near
term has been reported.44,45 Such expectantly management patients need to be admitted to a hospital where there is 24-hour
surgical, anesthesia, and neonatal expertise
and adequate blood bank services.
Expert Reviews
FIGURE 2
A, Midline sagittal transvaginal ultrasound appearance of a cervical pregnancy (arrow). Internal cervical os
(open arrow). B, Three-dimensional transvaginal ultrasound rendering of a cervical pregnancy (arrow).
Fylstra. Ectopics not within the fallopian tube. Am J Obstet Gynecol 2012.
acute abdominal pain and suspected ectopic pregnancy, with an abdominal implantation never suspected preoperatively. Clinically, the condition late can
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Expert Reviews
General Gynecology
FIGURE 3
be suspected when finding fetal malpresentation, oligohydramnios, malformations including compression defects and
pulmonary hypoplasia, palpation of fetal
parts just below the abdominal wall, and
an abnormally high alpha fetoprotein
level.46-49 The diagnosis has also been
made at the time of planned cesarean delivery for failure of cervical dilation.50
With normal deliveries, myometrial
contractions control blood loss from the
placental implantation site. However,
with abdominal implantation the placenta is located over tissues that cannot
contract, and partial removal of the placenta can lead to significant hemorrhage.51 In general, unless the placenta
can be delivered completely and without
difficulty, with identifying its blood supply with adequate ligation, it is preferable
to cut the umbilical cord in close proximity to the placenta and leave the placenta in situ and await spontaneous natural resorption.43,51-54 Leaving placental
tissue in situ frequently leads to a stormy
postoperative course with ileus and infection.55,56 Postoperative methotrexate
FIGURE 4
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administration plus feeding vessel embolization by interventional radiology
has been used to assist in recovery.53,57
With the universal use of early pregnancy imaging, the diagnosis can be confirmed at an early gestational age, but
this requires imaging demonstrating a
continuity of the cervix and uterus without pregnancy contents. Failure to follow basic ultrasound principles can miss
the diagnosis.58 Such early diagnosis can
spare maternal mortality at the expense
of fetal mortality, with a perinatal mortality rate of 40-95%.52 Removal of early
abdominal pregnancies has be successfully completed laparoscopically.59-63
General Gynecology
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tube. The mechanism that most probably explains scar implantation, like intramural implantation, is invasion of the
myometrium through a microscopic
tract. Like intramural pregnancy, such a
tract is believed to develop from the
trauma of previous uterine surgery, such as
curettage, cesarean delivery, myomectomy, metroplasty, hysteroscopy, and even
manual removal of the placenta.66-68 The
time interval between such trauma and a
subsequent pregnancy may impact on
implantation events. Some of the reported cases were diagnosed and treated
within a few months of a prior cesarean
delivery suggesting that incomplete healing of the uterine scar may contribute to
scar implantation.69,70
Early diagnosis with ultrasound can offer treatment options capable of avoiding
uterine rupture and hemorrhage and,
thereby, preserve the uterus. The differential diagnosis between spontaneous abortion in progress, cervicoisthmic pregnancy, and implantation within a cesarean
scar can be difficult. Strict ultrasound imaging criteria must be used to assess the diagnosis of cesarean scar pregnancy. Ultrasound should reveal an empty uterine
cavity, an empty cervical canal, development of the gestational sac in the anterior
part of the uterine isthmus, and an absence
of healthy myometrium between the bladder and the gestational sac, this last criterion allowing differentiation from cervicoisthmic implantation.71
Because of the rarity of this ectopic implantation, there are no universal treatment guidelines nor preferred treatment
concensus for cesarean scar pregnancy.
A variety of uterine and fertility-sparing
treatment successes have been reported:
laparotomy hysterotomy with resection
and uterine scar dehiscence repair;72-77
laparoscopic resection and uterine scar
dehiscence repair;78,79 hysteroscopic resection alone;80,81 hysteroscopic resection after treatment with methotrexate
or uterine artery embolization;82 curettage after uterine artery embolization
and methotrexate;83 systemic methotrexate as primary treatment;84-85 direct
injection of methotrexate or hyperosmolar
glucose into the cesarean scar pregnancy;71
uterine artery embolization alone;86 and
Expert Reviews
FIGURE 5
Midline sagittal transvaginal ultrasound appearance of an early cesarean scar ectopic pregnancy
(arrow).
Fylstra. Ectopics not within the fallopian tube. Am J Obstet Gynecol 2012.
surgical treatment by laparotomy or laparoscopy, with repair, as soon as the diagnosis is confirmed may be the preferred treatment option.
One expectantly managed woman with
a cesarean scar pregnancy had severe abdominal pain develop at 35 weeks gestation necessitating an urgent laparotomy
and delivery. Although a healthy infant
was delivered, a hysterectomy was required with massive blood loss, a coagulopathy, and a 16-unit blood transfusion.
Inasmuch as maternal well being is the first
priority, despite the delivery of a healthy
newborn infant, the authors of this report
questioned the prudence of allowing such
a pregnancy to continue.92
Although cesarean scar pregnancy is an
uncommon occurrence, only with a high
index of suspicion and the use of endovaginal sonography can the diagnosis be made
early enough to prevent rupture leading to
significant maternal morbidity and loss of
future fertility. Clinical history and endovaginal ultrasound can aid in differentiating
cesarean scar pregnancy from incomplete
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Expert Reviews
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FIGURE 6
A, Transverse transvaginal ultrasound appearance of an ectopic pregnancy within the right interstitial
portion of the fallopian tube (arrow). B, Three-dimensional transvaginal ultrasound rendered coronal
appearance of a right interstitial ectopic pregnancy (arrow).
Fylstra. Ectopics not within the fallopian tube. Am J Obstet Gynecol 2012.
www.AJOG.org
Interstitial ectopic pregnancy
Two to 3% of ectopics are implanted
within the interstitial portion of the fallopian tube, that portion of the tube that
transitions from the endometrial cavity
to the isthmus through a wall of myometrium.6 This ectopic location is included
in this discussion because its diagnosis
and treatment can differ from other fallopian tube implantations. The interstitial, or cornual, portion of the fallopian
tube is tortuous, 0.7 mm in diameter and
1-2 cm in length.93 This is a relatively
thick segment of fallopian tube with a
greater capacity to expand before rupture than more distal portions of the fallopian tube.94 Because implantation
within this portion of the fallopian is still
within the tube, it is associated with the
same commonly recognized risk factors
for tubal ectopic pregnancy. No single factor clearly differentiates women with an
interstitial pregnancy from those with isthmic or ampullary ectopic pregnancies.95
Transvaginal ultrasound is the primary method for diagnosing interstitial
implantation (Figure 6). However, many
early ultrasounds show that these pregnancies are surrounded by myometrium
and can be mistaken for normally implanted pregnancies. Ultrasound findings that are highly suggestive of interstitial implantation are the identification of
an echogenic line between the gestational sac and the endometrial cavity,
the interstitial line sign, and an empty
uterine cavity with a gestational sac eccentrically located outside the endometrial cavity with a thin mantle of surrounding myometrium less than 5 mm
in thickness.96 Collectively, these ultrasound findings are 88-93% specific, but
with a sensitivity of only 40%.97,98 Coronal images generated by 3-dimensional
(3D) sonography are helpful in identifying these features97 (Figure 6, B).
Magnetic resonance imaging (MRI)
may be helpful if ultrasound imaging is
inconclusive. MRI criteria for diagnosis are identical to those of transvaginal
ultrasound: eccentricity of the gestational sac, presence of myometrial tissue that surrounds the entire gestational sac with a thickness of less than 5
mm, and detection of an interstitial
General Gynecology
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line that connects the gestational sac to
the endometrial cavity.99
Interstitial ectopic pregnancies are frequently mislabeled as cornual ectopics. Cornual pregnancy refers to a pregnancy within the horn of a bicornuate
uterus, communicating or noncommunicating, and the clinical outcome of this
implantation varies greatly and depends
on the size and expansile capacity of the
affected horn.96
Angular pregnancies are implanted in
one of the lateral angles of the uterine
cavity, medial to the uterotubal junction,
and lead to asymmetric enlargement of
the uterus.100 What distinguishes an interstitial ectopic pregnancy from an angular pregnancy is that the laparoscopic
appearance of the bulge of an interstitial
pregnancy is lateral to the round ligament,
whereas the bulge of an angular pregnancy
is medial to the round ligament, displacing
the round ligament laterally. Over one
third of angular pregnancies end in early
abortion, but for those that continue pelvic pain, persistent vaginal bleeding, placental retention during the third stage of
labor, and rarely uterine rupture can be
expected complications.100
Although interstitial ectopic pregnancies can rupture later in gestation, in the
early second trimester, many rupture
less than 12 weeks and many as early as 7
to 9 weeks gestation.93,101-103 Interstitial
ectopics are associated with a higher rate
of hemoperitoneum, hypovolemia, and
catastrophic hemorrhage and a 2-2.5%
maternal mortality rate.104
Diagnosis after rupture most commonly requires laparotomy and frequently hysterectomy. However, with a
high index of suspicion, such ectopics
can be diagnosed early and successfully
treated conservatively. Minimally invasive laparoscopic surgery has revolutionized the treatment options.
Transcervical hysteroscopic suction
evacuation with laparoscopic or ultrasonographic guidance as been reported
with success.105,106 Laparoscopic cornuotomy with salpingostomy and laparoscopic cornual excision or cornual
wedge resection for small ectopics can
also be successful but with the required
laparoscopic skills to do so.107-115 Success with selective uterine artery emboli-
Expert Reviews
FIGURE 7
Laparoscopic appearance of an unruptured ectopic pregnancy within the isthmic portion of the left
fallopian tube.
Fylstra. Ectopics not within the fallopian tube. Am J Obstet Gynecol 2012.
tube, it is associated with the same commonly recognized risk factors for tubal
ectopic pregnancy. No single factor
clearly differentiates women with an
isthmic pregnancy from those with interstitial or ampullary ectopic pregnancies. Implantation in this portion of the
fallopian tube is included in this discussion, because the surgical management
of isthmic implantations differs from
distal fallopian tube implantations. The
isthmic portion of the tube is narrow
with a compact, well-defined muscularis
layer.122 Ectopics implanted within this
portion of the fallopian tube quickly invade the muscularis layer and usually
rupture early.122 Although linear salpingostomy has been reported to be successful, because of this muscularis invasion
such ectopics treated with surgical salpingostomy are at higher risk of leaving
chorionic villi behind, and, therefore, a
persistent ectopic pregnancy, requiring
additional rescue therapy. Optimal surgical success requires resection of that
portion of the fallopian tube, with or
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Expert Reviews
General Gynecology
FIGURE 8
Posthysterectomy ovarian
ectopic pregnancy
Laparoscopic appearance of an ovarian pregnancy (arrow) after prior subtotal cesarean hysterectomy. Posthysterectomy vaginal cuff (open
arrow).
Fylstra. Ectopics not within the fallopian tube. Am J Obstet
Gynecol 2012.
without immediate tubal reanastomosis.123 When diagnosed early and unruptured, medical therapy can be successful,
although consideration should be given
to gestational age, hCG levels, and the
presence of cardiac activity.7
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ectomy, now a more commonly performed operation, has also failed to prevent a patent cervical canal and an
ectopic pregnancy after hysterectomy.131
With the current popularity of laparoscopic supracervical hysterectomy, many
investigators, including this author, are
concerned about a potential increase in the
incidence of ectopic pregnancy after
hysterectomy.127,130,134
Two cases of late presentation ectopic pregnancy have followed supracervical cesarean hysterectomy.127,133 Leaving
a remnant of cervix or the epithialization
of a much larger vaginal cuff closure area
because of cervical dilation at the time of
cesarean hysterectomy may increase fistulous tract formation.
Because the symptoms of ectopic pregnancy can be mimicked by common immediate complications after hysterectomy,
such as protracted abdominal pain, pelvic
hematoma formation, vaginal cuff infection, and vaginal bleeding, ectopic pregnancy is rarely expected in most posthysterectomy cases until additional imaging or
repeat operation confirms the diagnosis.
The prevention of early presentation
ectopic pregnancy after hysterectomy is
the prevention of pregnancy before hysterectomy. Hysterectomy, like tubal sterilization, if possible, should be avoided in the
luteal phase of the menstrual cycle in those
women not previously sterilized or not using reliable contraception, unless no vaginal intercourse has occurred during the
preoperative period. Women should be
preoperatively counseled as such. Any
woman who has undergone hysterectomy
and had not previously undergone tubal
sterilization or had a partner vasectomy, or
was not using reliable contemporaneous
contraception, should be considered at
risk for this diagnosis should otherwise unexplained postoperative pain or bleeding
occur.
It may not be possible to prevent all
late presentation ectopic pregnancies
after hysterectomy, but its prevention is
the prevention of vaginal-to-peritoneal
cavity communication. Vaginal cuff closure, regardless of operative technique,
should be sure not to incorporate the fallopian tube into the vaginal cuff, and
postoperative vaginal cuff granulation
tissue, a very common finding, must be
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differentiated from a portion of prolapsed fallopian tube, with biopsy, if necessary. When the cervix is left in situ,
techniques should be used to obliterate
or isolate the residual cervical canal, thus
preventing a patent cervical canal allowing sperm access to the peritoneal cavity.
The incidence of ectopic pregnancy after
hysterectomy is infinitesimal, despite an
estimated 600,000 hysterectomies each
year in the United States, and that onethird of all US women have had a hysterectomy by age 60 years.135 However, it would
be prudent for any woman, even after
hysterectomy with ovaries in situ, who
presents with an acute abdomen or abdominal-pelvic pain to be screened for
pregnancy, and only with a high index of
suspicion will the diagnosis be made.
Summary
Ectopic pregnancy occurs in 1 of every 50
pregnancies. Early transvaginal ultrasound
can locate most, if not all early pregnancies.
The late diagnosis of an ectopic pregnancy
increases the risk for loss of fertility and of
maternal mortality. Many nontubal ectopic locations are diagnosed in the operating room or are never known because of a
successful response to medical management for a pregnancy of unknown location. Although medical management with
methotrexate has been successfully used,
nontubal ectopic pregnancies frequently
may require surgical intervention.
f
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