Link
http://www.casereports.in/articles/4/1/Uterine-Arterio-venous-Malformation-with-Hemoperitoneum.html
Rajshree D. Katke, Shilpa Domkundwar, Sunil C. Rathod
From the Department of Obstetrics & Gynecology1 and Department of Radiology2, Cama & Albless Hospital, Grant Government Medical College & Sir J. J. Group of Hospitals, Mumbai, Maharashtra, India.
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Introduction
Congenital uterine AVMs are thought to develop secondary to faulty arrest in the angiogenic process.
Acquired AVMs are thought to be related to uterine trauma including
curettage or cesarean delivery, retained products of conception,
chorio-carcinoma, endometrial or cervical carcinoma, gestational
trophoblastic disease and diethylstilbestrol exposure. Most commonly
they present with vaginal hemorrhage, but other presentations such as
congestive heart failure, postmenopausal bleeding, and an asymptomatic
mass have been described. Diagnosis can rapidly be made with color flow Doppler ultrasound or angiography as in our reported case.
Case Report
28
year Para-1, Living-1, Abortion-1 married since 5 years, HIV positive
female presented with abdominal pain, bleeding per vaginum with passage
of clots since last 24 hours. Her past history was insignificant except
previous history of dilation and currettage one and half year back. Her
outside ultrasound gave differential diagnosis of ruptured ectopic,
ruptured hemorrhagic cyst, vascular mass within endometrial cavity or
polyp. When patient came to our institution she was in a state of
haemorrhagic shock with pulse rate of 112 beats/min, blood pressure of
94/62 mmHg. Per abdomen examination revealed distension of lower part of
abdomen, severe tenderness and guarding in lower abdomen with
clinically no mass felt. Her speculum examination was suggestive of mild
fresh bleeding while per vaginal examination revealed bulky tender
uterus. Cervical movements were tender and forniceal tenderness was
present. Investigations showed negative urine pregnancy test,,
hemoglobin of 7.6 gm/dL, beta HCG 13 IU/mL, with normal liver and
renal function tests. We kept a provisional diagnosis of ruptured
hemorrhagic cyst. Her urgent Doppler ultrasound was suggestive of bulky
uterus with relatively well defined heterogenous predominantly
hyperechoic lesion giving whorled appearance. The 4.2x4.4x3.5 cm mass
protruding into the endometrial cavity showed cystic anechoic space
within showing mixed AV waveform with aliasing of color. The arterial
supply appeared to be directly arising from the left uterine artery with
machinery murmur and arterialization of left uterine venous waveform
with evidence of active ongoing bleed. Moderate to gross free fluid in
abdomen with internal echoes were suggestive of hemo-peritoneum [Fig.1,2].
Our
impression was uterine artery AV malformation with surrounding
heterogenous hyperechoic lesion occurring secondary to endometrial polyp
or organized hemorrhage with active bleed. However, the size of
hemo-peritoneum was disproportionate to the size of AVM and so the exact
etiology of hemo-peritoneum could not be determined.
As
patient was in state of hemorrhagic shock with hemo-peritonium and
keeping in mind the dilemma in regards to diagnosis of the patient and
decision of emergency exploratory laparotomy was taken and if required
then hysterectomy. Intraoperatively, uterus was bulky and blood was
pouring through both tubal ostia suggestive of bleeding from the
endometrial cavity. Both the fallopian tubes and ovaries were normal. As
the bleeding was from endometrial cavity which had resulted in
hemoperitoneum; the decision of hysterectomy was taken. Total abdominal
hysterectomy was performed and 1.5 liters of hemorrhagic fluid was
drained.Four pints of blood and 2 pints of fresh frozen plasma were
transfused. On cutting the specimen of uterus a vascular polyp
approximately of 4.5x4x5 cm was seen arising from uterine fundus with
100 grams blood clot filling the entire endometrial cavity just below
the polyp [Fig.3]. Patient tolerated surgical procedure well. Her
post-operative period was uneventful.
Discussion:
Regardless
of the etiology, uterine AVMs are a potential source of significant
morbidity and, rarely, mortality. The true incidence is unknown, but the
majority of cases are found in women of reproductive age. Most
commonly, they present with menorrhagia or meno-metrorrhagia requiring
blood transfusions in 30% of reported cases [3].
In our case, the patient presented with hemo-peritoneum and state of
hemorrhagic shock as unusual presentation. The differentiation between a
uterine AVM and other causes of uterine bleeding is necessary because
instrumentation can lead to massive hemorrhage.
Historically,
uterine AVMs were diagnosed by laparotomy or pathologically after
hysterectomy. Angiography is the gold standard for diagnosis,
classically showing complex vascular connections supplied by
hypertrophied feeding arteries and early drainage through enlarged and
hypertrophic veins. Although angiography has the ability to assess the
size of AVMs and vessel involvement, it is invasive and cannot
accurately detect the degree of pelvic extension. Beside it needs
expertise and the cath lab is not available everywhere. Ultrasound and
MRI accurately delineate the extent of adjacent organ involvement and
are now considered the modalities of choice when evaluating a patient
with suspected uterine AVM. Again the cost of MRI and its availability
are prohibitive. Ultrasound with Doppler study remains the modality of
choice in day to day practical life. As this patient presented with
hemo-peritoneum leading to hemorrhagic shock, exploratory laparotomy
was done as a life-saving procedure.
Gray-scale
ultrasound features of uterine AVMs include multiple anechoic
structures with a serpentine contour within the myometrium [4]. However, these features are often confusing and nonspecific [3,4].
The addition of color and spectral analysis can lead to more accurate
diagnoses. On color Doppler, these hypoechoic structures exhibit
vascular flow. Huang et al suggested two distinct color patterns
consistently associated with uterine AVMs. The first is the
juxtaposition of blue and red components, suggesting blood flow
reversals caused by overlapping vessels of varying orientations and flow
directions. The second is color aliasing and the separation of red and
blue components by yellow and white, suggesting focal high velocities [3].
Spectral Doppler demonstrates low-resistance and high-peak systolic
velocities with continuous high flow throughout systole and diastole [2,3].
MRI
provides accurate definition of uterine AVMs and effectively delineates
invasion of adjacent organs. Characteristic features include a bulky
uterus with a focal mass, disruption of the junctional zones, multiple
serpiginous flow-related signal voids within the lesion, and prominent
parametrial vessels [2,3]. Gadolinium-enhanced MRI demonstrates hypervascular arterial dominant flow [5-7]. But it can be used only in stable patients and only a few affluent patients can be advised MRI.
Prior
to embolotherapy, hysterectomy or uni/bilateral uterine artery ligation
were the therapies of choice. Since the first reported case of
transcatheter uterine artery embolization for a uterine AV fistula in
1982, embolo-therapy has become a well-recognized alternative to
surgical intervention for uterine AVMs, with the major advantage of
maintaining childbearing capacity. Ghai et al recently reported a
retrospective review of all patients who underwent pelvic arterial
embolization for traumatic uterine AVMs at their institution during a
10-year span. The authors concluded that selective uterine artery
embolization is a safe and effective treatment for traumatic uterine
AVMs while preserving the possibility of future pregnancy [8].
Gelfoam offers 3 to 5 weeks of occlusion that is sufficient to occlude
the AVM while still permitting slow development of collateral vessels,
allowing for the preservation of fertility and resumption of
menstruation. Furthermore, an increasing number of pregnancies following
transcatheter embolization of uterine AVMs are being reported [8,9].
The
current literature, suggests that transcatheter uterine artery
embolization of traumatic uterine AVMs is a safe and effective
alternative to surgical therapy while offering the major advantage of
preserving childbearing capacity. But in our case, patient had abnormal
presentation i.e. hemo-peritoneum so emergency exploratory laparotomy
with total abdominal hysterectomywas performed.
Conclusion:
Uterine
A-V malformation are rare cause of menorrhagia, and ultrasound with
Doppler study remains the modality of choice in day to day practical
life.
References:
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