Primary Splenic Pregnancy- A Rare but Imperative Cause of Hemoperitoneum - Case Report and Review of Literature
Ruchi RATHORE1, .Shilpi1, Ratna CHOPRA2, Namrata NARGOTRA1
1Department of Pathology, North Delhi Municipal Corporation Medical College and Hindu Rao Hospital, NEW DELHI, INDIA
2Department of Surgery, North Delhi Municipal Corporation Medical College and Hindu Rao Hospital, NEW DELHI, INDIA
Keywords: Splenic rupture, Hemoperitoneum, Ectopic pregnancy, Spleen, Chorionic gonadotropin
Primary splenic pregnancy is an extremely rare form of extratubal ectopic pregnancy. These cases often cause splenic rupture in very early course
of their gestation thereby presenting with hemoperitoneum in emergencies. Owing to the higher risk of exsanguination and death caused by
hemoperitoneum, it is essential to diagnose these cases for proper management and better prognosis of the patients. We present the case of a
23-year-old female, gravida 2, para 1, live issue 1 presenting to the emergency outpatient department with acute abdomen and hemoperitoneum.
There was no history of trauma. The patient had a positive urine pregnancy test and raised beta HCG levels. Emergency laparotomy revealed an
otherwise unremarkable fallopian tube and ovary with a hemoperitoneum of 2.5 liters. A tiny splenic laceration was considered to be the source
of bleeding and splenectomy was performed. Microscopy was suggestive of a primary ectopic pregnancy, spleen. Since hemoperitoneum in
pregnancy is a rare but potentially fatal condition with a high risk of mortality, an accurate preoperative diagnosis is crucial in the management
of such patients. The possibility of a ruptured extratubal ectopic pregnancy must be considered as one of the differential diagnoses of acute
abdomen with hemoperitoneum in women of childbearing age.
The implantation of a fertilized ovum anywhere outside the
uterine cavity is known as ectopic pregnancy. While 95.5
% of the ectopic pregnancies are in the fallopian tube, only
1.3 % are abdominal in location1
. Such rare extratubal
pregnancies occur after the direct implantation of the
zygote onto the peritoneal surfaces. Although a variety of
extra pelvic organs such as the liver, omentum, and small
and large intestines have been described in the past, reports
from primary pregnancy in the spleen are very rare. Our
extensive search of the literature for the same yielded only
11 such cases reported in the past. Since ruptured ectopic
pregnancy is a well recognized life-threatening entity in
early pregnancy and is also one of the known causes of
hemoperitoneum in pregnancy, we present an unusual case
of primary splenic pregnancy presenting as acute abdomen
with hemoperitoneum being misinterpreted as splenic
laceration following blunt trauma to the abdomen in the
A 23-year-old, gravida 2, para 1, live issue 1, woman
presented to the medical emergency with the chief
complaints of abdominal distention along with non passage of flatus for one day and left upper quadrant pain, nausea
and vomiting since 6 hours. The pain was aggravated on
movement and deep breathing but was not radiating to
the shoulder. The patient was also feeling weak and dizzy
since that morning. There was no history of fever, cough,
weight loss, appetite loss or any recent trauma to the chest
or abdomen. The obstetric history revealed that the patient
had given birth by normal vaginal delivery one year back
followed by a medical termination of pregnancy 3 months
back in November. Her last menstrual period was due in
December at the time of presentation at the emergency and
she currently had amenorrhea for 4 weeks and 2 days.
On examination, the patient was afebrile, conscious and
oriented. Pallor was present but there was no icterus
or pedal edema. Her blood pressure was 80/60 mm Hg
and pulse was 100/min. On per abdomen examination,
the abdomen was distended and diffuse tenderness was
noted. The urine pregnancy test was positive and serum
β-human chorionic gonadotropin levels were raised to
6565mIU/ml. Peritoneal tap attempted twice yielded blood
only. Emergency transvaginal ultrasound findings were
suggestive of free fluid in the pouch of Douglas and an
empty uterine cavity. Hemogram findings were indicative of severe anemia with a hemoglobin of 3 gm/dl. The results of
all other investigations including PT/PTTK, liver function
test and kidney function test were within normal limits.
Based on the above findings, a presumptive diagnosis
of ruptured ectopic pregnancy was made and the
patient was referred to the gynecology department. She
underwent emergency laparotomy for the same. However,
intraoperatively it was noted that there was 2.5 liters of
blood and blood clots in the peritoneal cavity. The uterus
and bilateral adnexa were unremarkable and the source
of bleeding was from a laceration in the spleen. Since the
spleen was lacerated and not enlarged in size, rupture due
to blunt trauma was suspected and medico legal concerns
were raised. A splenectomy due to uncontrolled bleeding for
a splenic laceration and a dilatation and curettage (D&C)
for an incomplete abortion were performed for the patient
under general anesthesia. She was given 3 units of blood
transfusion postoperatively and was thereby stabilized
We received the splenectomy specimen along with some
blood clots in one container and an endometrial biopsy (EB)
in another. On gross examination, the specimen of spleen
measured 10x5.5x3.5 cm and weighed 100 gms. The outer
surface was predominantly unremarkable and the capsule
was smooth and glistening except at the inferior pole where
a small capsular rupture measuring 1 cm in maximum dimensions was seen. The capsule over the inferior pole
was also congested. On cut section, a small 1x1 cm welldemarcated
nodular hemorrhagic lesion suggesting a blood
clot was noted in the subscapular region at the inferior pole.
A single focus in this lesion also showed tiny grey-white
areas attached to its capsule (Figure 1). The rest of the spleen
seemed to be unremarkable grossly. Multiple sections were
taken from the spleen and examined histopathologically.
The endometrial biopsy and blood clots sent together were
also examined microscopically.
Click Here to Zoom
|Figure 1: Gross image of spleen showing gestational sac at inferior
pole. The rest of the spleen appears unremarkable grossly.
On histopathology, the endometrial biopsy revealed the
presence of secretory endometrium with extensive decidual
change in the stroma. Multiple sections examined did
not show any chorionic villi. Section from the nodular
lesion at the inferior pole of the spleen showed blood
predominantly in the subscapular regions of the inferior
pole. Numerous chorionic villi and trophoblastic tissue
amongst these hemorrhagic areas were seen invading
the splenic parenchyma (Figure 2). The rest of the spleen
showed congestion in the splenic sinuses. Based on the
above findings, a diagnosis of splenic ectopic pregnancy
was made on histopathology.
Click Here to Zoom
|Figure 2: Low power image of spleen showing products of
conception in the parenchyma of the spleen. These products
are subcapsular in location and are surrounded by the lymphoid
follicles of spleen (H&E; x100).
Pregnancies occurring within the peritoneal cavity
excluding the fallopian tube, ovaries and ligaments of the
uterus are named abdominal pregnancies. These account
for 1.3 % of all the ectopic pregnancies with a reported incidence of 10.9 per 100000 live births in the United States2,3
. The maternal mortality rate of these potentially lifethreatening
cases is very high, accounting up to 5.1/1000
cases in the United States and as high as 7% in the Indian
. The higher incidence in developing
countries is attributed to the higher frequency of pelvic
inflammatory disease, as in our country5
Abdominal pregnancies are classified as primary or
secondary depending on the site of fertilization of the ovum.
When the fertilization takes place within the peritoneal
cavity, the pregnancy is named a primary abdominal
pregnancy and when it is associated with a tubal rupture
followed by implantation at a secondary site it is named
a secondary abdominal pregnancy. Primary abdominal
pregnancies are extremely rare when compared to secondary
abdominal pregnancies. To accurately diagnose a case with
primary abdominal pregnancy, the Studdiford criteria are
(1) grossly normal fallopian tubes and ovaries with no
evidence of recent injury; (2) no evidence of uteroplacental
fistula; and (3) a pregnancy of no more than 12 weeks’
gestation with trophoblastic elements related exclusively
to a peritoneal surface6. The third criterion makes it
possible to exclude secondary abdominal pregnancies.
Since our case fulfilled all these criteria, we labeled it as a
primary ectopic pregnancy of the spleen.
Abdominal pregnancy is associated with a very wide
range of signs and symptoms due to its variable location.
The clinical suspicion index is also low due to the rarity
of this condition and the absence of the classical triad of
abdominal pain, amenorrhea and vaginal bleeding in
these cases. Various locations reported in the past include
the small and large intestine, omentum, liver, diaphragm,
pancreas, retroperitoneum and spleen3,7,8. Risk factors
associated with these are however similar to other ectopic
pregnancies and include a history of PID, ectopic gestation,
endometriosis, in vitro fertilization and previous surgeries
on the tube such as tubal reconstructive surgeries or tubal
recanalization surgeries9. While there were no such risk
factors in our case, 3 of 12 reviewed cases in our study had
an intrauterine contraceptive device at presentation (Table
In accordance with the previous studies, our patient
presented with chief complaints of sudden onset left upper
quadrant pain and dizziness. On reviewing the previously
published reports it was noted the mean age of these
patients was 25.3 years, ranging from 23 to 37 years, and in
10 of 12 previously published cases a presumptive diagnosis
of ruptured ectopic pregnancy was made preoperatively
(Table I). There was only one case where the diagnosis was made only after histologic examination. Although
the first diagnosis was ruptured tubo-ovarian ectopic
pregnancy in our case, the possibility of missed abortion
with splenic rupture due to blunt trauma was considered
after exploratory laparotomy. Despite the fact that there was
no history of blunt trauma to the abdomen, medico legal
concerns were raised in our case. The final diagnosis of
splenic ectopic pregnancy in our case was made only after
Due to the rarity of such cases and the complexity of
the diagnosis, abdominal pregnancies are often missed
preoperatively. In a study conducted by Costa et al. it was
seen that even ultrasound coupled with clinical evaluation
had a success rate of only 50% in the diagnosis20. The
guidelines for the use of USG to diagnose abdominal
pregnancy as provided by Allibone et al.21 still have
reported diagnostic errors of 50-90% in various case series.
In recent years, only one study quotes the preoperative
diagnosis of splenic pregnancy made with transvaginal
USG and its successful management by laparoscopy19.
In our case unfortunately the diagnosis of splenic ectopic
pregnancy was missed on USG.
Splenic implants have been reported at a variety of sites
ranging from the superior pole to the lower pole and
hilum. In some cases, the ectopic gestation manifested as
capsular projections but in all the cases the subcapsular
location was a phenomenon. In our case also, no capsular
projection could be identified on gross examination though
the gestational sac was seen in the subcapsular region at the
lower pole of spleen.
According to the previously reported literature, splenic
gestations range in size from 2 to 3.5 cm and present
with hemoperitoneum at 6-8 weeks of gestational
age. It is hypothesized that the earlier presentation of
splenic pregnancies as compared to the other abdominal
pregnancies may be because of the rupture of the splenic
capsule at a much smaller size of gestational sac than other
abdominal pregnancies3. In accordance with the previous
studies our patient presented with hemoperitoneum even
earlier at only 4 weeks of gestation with a gestational sac
of 1x1 cm only. This could be due to its very superficial
location just beneath the capsule in the lower pole of spleen.
In conclusion, since hemoperitoneum in pregnancy is
a rare but potentially fatal condition with a high risk of
mortality, an accurate preoperative diagnosis is crucial
in the management of such patients. The possibility of a
ruptured extratubal ectopic pregnancy must be considered
as one of the differential diagnoses of acute abdomen with
hemoperitoneum in women of childbearing age.
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