Introduction
Spontaneous hemoperitoneum in pregnancy (SHiP) - defined as
blood within the peritoneal cavity of non-traumatic
aetiology [1,2] is a rare, life-threatening event,
particularly relevant to women with endometriosis if they
are undergoing in vitro fertilization (IVF) treatment. This
dramatic complication has been associated with high
perinatal morbidity and mortality, but the trigger of the
spontaneous bleeding has not been established [3,4]. Given
the rarity of SHiP, its diagnosis is almost always
unsuspected until the time of imaging, which is undertaken
in patients who present with acute abdominal pain and/or
distension and anaemia. Although the aetiology of SHiP
remains unknown and may be multifactorial, several theories
have been proposed to explain the rare complication.
Case Presentation
An elderly primigravida, at 27weeks of gestation (ICSI
pregnancy with Donor egg) known case of hypothyroidism
was referred to our tertiary care hospital
complaining of a sudden onset of moderate lower abdominal
pain since 2 hours with reduced perception of fetal
movements in a state of shock. She denied trauma. The
patient's past medical history was unremarkable. The course
of the recent pregnancy had also been uneventful except with
history of anaemia since second trimester on irregular iron
supplements and on Ecospirin 75mg OD. Physical
examination revealed tachycardia (148 bpm), BP of 80/40 mm
Hg on adrenaline supports with severe pallor. An abdominal
examination revealed that her abdomen was tense, distended
and tender. The uterus was tense and tender, and fetal heart
sound could not be localised with hand held Doppler. A
gentle per vaginum examination after ruling out placenta
previa was performed which revealed cervix soft, internal os
closed, no bleeding p/v.
Counselling and Preparation
After obtaining high risk consent following an emergency USG
scan suggestive of IUD and moderate hemoperitoneum with Hb
6.4 gms, she was shifted to OR for exploratory laparotomy
with wide bore canula inserted, necessary blood
investigation done and multiple blood components issued with
multidisciplinary approach alerting Senior Surgeons,
Anaesthetists and blood bank. Patient's bystanders were
counselled regarding the possibility of placental abruption
or rupture uterus and need of laparotomy to rule out the
same and the most likely complications like DIC, Sepsis,
MICU admission, Massive blood transfusions, hysterectomy as
well.
Revelation in OR
On opening the abdomen, she was found to have massive
hemoperitoneum. Approximately 3 litres of dark colored
altered blood and organised clots and the same was evacuated
from the abdominal cavity, with distorted anatomy of uterus
buried in adhesions. There were diffuse endometriotic
implants all over the uterus which bled profusely. Bladder
was pulled up and adherent to the anterior uterine
wall, sharp dissection was done and adhesions were released,
bladder was pushed down.A transverse incision was put in the
lower segment, a fresh stillborn female baby as breech
weighing 1080 gm was delivered. There was no evidence of
abruption since the liquor was clear and no retroplacental
clots seen. Uterus was closed in layers. Bleeding continued,
Search for the source of bleeding continued and to our
surprise, there were multiple rents, largest measuring 5 x 5
cm on the fundo-posterior wall and two lateral rents of 2 x
3 cm and 2 x 2 cm on the either sides with diffuse oozing.
Repair of the rent was performed in two layers using vicyrl
and hemostasis attained to some extent. Since the patient
was on Ecospirin there was diffuse oozing. There were dense
adhesions between sigmoid colon to the posterior surface of
the uterus, and to the lateral pelvic wall, exteriorsation
of the uterus could not be done. Senior Gastro-surgeon was
called in for opinion and decided not to do the adhesiolysis
since the patient was on Ecospirin and was in shock.
With the support of uterotonics, transxemic acid and massive
blood transfusion, abdomen was closed after keeping a drain
in situ with catheter draining clear urine at the end of the
surgery.
Critical Care and Supports
Post op period was uneventful managed in critical care unit
with IV fluids, antibiotics, drain care (removed after 5
days) and serial monitoring was done to look for excessive
bleeding She had total blood transfusion (7 units of PRBC, 4
units of FFP, 4 units of Platelet Concentrate). The patient
was discharged from the hospital in good general condition
after 11 days.
Counselling and education given to the patient and her
family regarding avoiding future crisis pregnancy due to
high probability of rupture uterus.
Discussion and Review of literature
Endometriosis and Deciduosis Are Associated with
Decidual Bleeding
SHiP in patients with endometriosis raises the possibility
of multiple bleeding sites in the pelvis. All of these need
to be identified in order to achieve haemostasis. This can
be very challenging, as the large uterus obstructs access to
the posterior pelvic cavity during pregnancy. The posterior
pouch of Douglas can also be obstructed by the presence of
endometriosis-related adhesions and multiple endometriotic
implants.
Aziz et al. [5] described a focus of endometriosis and
decidualization in a woman who was 30 weeks into her first
pregnancy and demonstrated the presence of very thin-walled
blood vessels in the decidualized stroma. Mizumoto et al.
[6] documented the presence of ruptured vessels on the
serosal surface of the uterine fundus at 20 weeks in a woman
during her first pregnancy. Histology demonstrated an
intense decidual reaction. The blood vessels were distended
and exhibited disintegration of the vessel wall. Lier et al.
described a case of late postpartum SHiP in a patient with
ovarian endometriosis.
Mechanisms of Ectopic Decidual Bleeding
Emerging evidence suggests that endometriosis is associated
with progesterone resistance characterized by the suboptimal
expression of target genes [7]. Therefore, it is tempting to
speculate that "functional" progesterone withdrawal triggers
the involution of the decidual phenotype of the ectopic
endometrium surrounding distended parametrial arterioles,
leading to peritoneal bleeding of unpredictable severity.
In this case, there was evidence of endometriosis stage IV
found during the laparotomy. A rupture of unscarred uterus
is rare [8]. The ultrasound exam was beneficial in this case
because it revealed the presence of free fluid inside the
abdominal cavity and Intra uterine fetal demise,
which led us initially to include uterine rupture in the
differential diagnosis and minimised the delay in the
decision to perform an emergency laparotomy to determine the
site of bleeding, which could have been obstetric or
non-obstetric. Rupture of the uterus should be considered in
pregnant women with hemoperitoneum, even when caesarean
section is absent from the obstetric history especially with
menstrual diary suggestive of endometriosis.
Conclusion
Available evidence suggests that during pregnancy a link
exists between ectopic decidualization, particularly that
occurring in endometriotic foci, and the occurrence of SHiP.
Alterations in vessels' walls have been demonstrated in the
few cases where relevant biopsies were obtained and
examined. Indeed, it seems that arterioles can become
modified in the absence of trophoblast. There is a need to
carefully examine vascular alterations at the site of
bleeding leading to SHiP to gain information on the
pathophysiology of this serious complication. Finally, there
are indications that subclinical decidual bleeding may be a
potential risk factor for preterm labour, further clinical,
pathological and molecular investigations are required.
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