Pelvic arteriovenous malformation presenting as bloating and abdominal
distension: a case report and literature review
Takayoshi Iijima1, Aya
Tokinaga-Uchiyama1, Natsumi
Fuchimukai1, Fumi Yanagisawa1, Yuko
Sonan1, Ayaka Nakashima1, Mayu
Shimomukai1, Kazunori Mukaida1,
Hironori Murakami2, Yuta Kume2,
Yukihiro Bonkohara2, Mika Okuda1
1. Department of Obstetrics and Gynecology, National Hospital
Organization Yokohama Medical Center
2. Department of Vascular Surgery, National Hospital Organization
Yokohama Medical Center
1 INTRODUCTION
Arteriovenous malformation (AVM) is an abnormal structure in which
arteries and veins are directly connected without interposition of a
capillary bed, forming a vascular nidus. 1 Although AVMs can occur
anywhere in the body, pelvic AVMs (pAVMs) are rare and may pose a
diagnostic and therapeutic challenge. While uterine arteriovenous
malformations (uAVMs), located in the uterus, usually present with
bleeding, pAVMs occurring outside the uterus show non-specific symptoms
and are, thus, difficult to diagnose. 2, 3
We report a case of pAVM presenting with abdominal distension and
evaluated as a pelvic mass using color Doppler transvaginal ultrasound.
Surgical resection of the AVM was performed by vascular surgeons.
Post-surgery, the patient was symptom-free and had no subsequent
recurrence.
2 CASE
A 51-year-old woman, gravida 3, para 2, presented to our hospital with
progressive and generalized abdominal distention since one month. She
had not yet reached menopause. Her obstetric history included artificial
abortion by surgical curettage 21 years prior, normal vaginal delivery
14 years prior, and an emergency cesarean section due to placental
abruption 12 years prior. No other significant complaints were reported,
except abdominal bloating. Her symptoms were exacerbated after meals and
exertion, and alleviated on sitting. At admission, her vital signs were
unremarkable and physical examination revealed a soft and non-tender
abdomen. The pulsating mass was palpable on the left side of the pelvis
but non-tender.
Transvaginal ultrasonography showed an anechoic mass measuring 6 cm×3 cm
in diameter on the left side of the pelvis, and color Doppler showed a
mosaic pattern in the mass, representing turbulent blood flow (Fig. 1-1,
1-2).
A diagnosis of pAVM was made using contrast-enhanced computed tomography
(CT) and angiography (Fig. 2-1 and 2-2, 3). The inflow vessels were the
left internal iliac and inferior gluteal arteries. An abnormal vein
around the AVM and a dilated left ovarian vein were also seen in the 3D
CT angiography (Fig 2-2).
As complete resection was feasible due to the size and location of the
AVM and fertility preservation was not required, surgical resection was
planned. Surgery was performed by both gynecologists and vascular
surgeons.
Intraoperative findings included an AVM measuring approximately 6 cm× 3
cm in the retroperitoneal cavity. The uterus and ovaries were normal in
size, and no other mass/cystic lesions were observed in the abdominal
cavity. Initially, the left internal iliac artery was ligated to reduce
the blood flow to the AVM. Hysterectomy and adnexectomy were then
performed to achieve an optimal view of the surgical field. As the
ligation of the internal iliac artery did not completely interrupt the
blood flow to the AVM, multiple inflowing blood vessels from the lower
gluteal artery were also ligated. The AVM was then successfully
resected.
Histopathology of AVM showed evidence of congenital pAVM. Grossly, the
specimen showed dilated vein-like blood vessels and arteries.
Microscopically, some regions of the vascular wall were thinned, and
some sections of the intima layer of the vascular wall were thickened
via hyperplasia of smooth muscle fibers. This indicates abnormal
differentiation of the vascular structures.
The symptoms of abdominal distension were resolved within two days of
the surgery, and the abnormal blood flow in AVM and abnormal vasculature
disappeared, as evidenced by the contrast-enhanced CT performed on
post-operative day 6 (Fig. 4). Following this, the patient was
discharged on post-operative day 7. A follow-up ultrasound after six
months showed no evidence of a recurrent lesion.
3 DISCUSSION
The following two important points are highlighted in this case study:
Abdominal bloating may potentially be the only symptom of pAVM, and
transvaginal ultrasound and color Doppler are valuable tools for the
detection of pAVMs.
The symptoms of pAVMs are usually non-specific, and patients may be
asymptomatic and accidentally diagnosed.4 When symptomatic, they may
present with abdominal or pelvic pain, lower urinary tract symptoms,
such as hematuria and dysuria. 5, 6, 7 Uterine arteriovenous
malformations (uAVMs) often present with bleeding.8 In addition, the
presence of abnormal shunts may also cause secondary heart failure and
pelvic congestion syndrome (PCS). 9, 10 PCS is a clinical syndrome
presenting as chronic pelvic pain that lasts for more than 3–6 months.
Imaging studies may show dilation and regurgitation of the ovarian veins
and dilation of the pelvic plexus.9
In the present case study, we report abdominal bloating as the only
presenting symptom for pAVM. As abdominal bloating was completely
relieved after surgery, AVM was considered to be the cause. Since the
AVM was present in the retroperitoneum and was not large enough to
obstruct the intestinal tract, it is highly unlikely that bloating was
the result of compression of the intestinal tract. The symptoms may have
been caused by the secondary PCS. The imaging findings were typical of
PCS. This suggests that pelvic venous overload, congestion, and changes
in hemodynamics may have contributed to the symptoms.
We conducted a literature review to explore the clinical scenarios
associated with pAVMs using the MEDLINE database. The search was done
using the strategic keyword “pelvic AVM.” The case reports that did
not mention symptomatology and those that revealed uterine AVM, and
those that were written in languages other than English were excluded
from the review (Table 1). A total of 31 articles(28 case reports and 3
review articles) were retrieved based on the inclusion and exclusion
criteria. There were 43 cases, including the case reviewed here. Some of
the articles did not provide patient age and sex. Approximately
one-third of the cases reported (14 out of 37) were of female patients.
The most common presenting complaint was pelvic and abdominal pain
(46%, 20 out of 43). Seven patients (16%) were asymptomatic and
diagnosed accidentally. Heart failure symptoms were reported in six
cases (14%) and were considered to be due to secondary PCS. None of the
cases presented with abdominal distension as the chief complaint exept
the case reviewed here.
Among the diagnostic strategies available for pAVMs, color Doppler
transvaginal ultrasonography is a convenient and useful technique. It is
less invasive and suitable for the screening of pAVMs. However, for a
definitive diagnosis, dynamic CT imaging and angiography are required.
11
In the present case, transvaginal ultrasound revealed a cystic lesion
with a mosaic pattern, suggesting turbulent blood flow. The internal
iliac veins were visualized via the arterial phase of dynamic CT, and
the inflow and outflow vessels were identified using angiography. Thus,
these modalities serve as a valuable tool for the diagnosis and
follow-up of vascular lesions such as AVM.
Abdominal bloating is a common and non-specific symptom that can be a
manifestation of wide variety of ailments. Gastrointestinal causes are
usually considered when associated symptoms such as constipation and
nausea are present. Gynecological causes should also be ruled out as the
gastrointestinal tract may be mechanically compressed by ovarian tumors,
uterine fibroids, etc., giving rise to such symptoms. 12, 13 Thus, even
if abdominal symptoms are the only chief complaints, a detailed
investigation including the pelvic cavity aids in ruling out rare
diseases, as in our present case.
In uAVM cases, color Doppler ultrasound shows a colored mosaic pattern
in the non-echoic region of the myometrium, representing turbulent blood
flow. 14, 15 Similarly, an anechoic lesion in the pelvis, as in the
present case, can be diagnosed as AVM with similar findings on color
Doppler imaging.
Although the natural history of pAVM is not well established, it is
known to progress slowly and become symptomatic over time. 16 A delay in
diagnosis may make the management more challenging. Additionally, if the
secondary PCS progresses over time, heart failure may supervene. One
study observed a large pAVM associated with cardiac failure, which was
difficult to treat and recurred many times despite repeated treatments.
17 Therefore, for the early detection and efficient treatment of the
disease, even unrelated and non-specific symptoms must be thoroughly
investigated.
4 CONCLUSION
We diagnosed a patient, who presented with abdominal bloating and
distention as chief complaints, with pAVM. Although pAVM is a rare
disease, it may sometimes be associated with common symptoms such as
abdominal distension. Non-invasive imaging studies, including color
Doppler ultrasound, may play a key role in diagnostic screening for
pAVMs. As early detection and treatment are crucial for pAVM,
recognizing all the possible symptoms and the use of non-invasive rapid
screening will prevent a missed diagnosis. This case report recognizes a
rare manifestation of a known disease and aims to broaden the outlook of
clinicians and surgeons.
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5 AUTHOR CONTRIBUTION
TI: drafted the manuscript contributed and performed the surgery. AU:
performed the surgery and revised the manuscript. AN: revised the
manuscript.NF, FY, MS, YS, MO: contributed to the clinical management of
the patient. KM, HM, YK, YB: contributed to the clinical management of
the patient and performed the surgery.
ACKNOWLEDGMENT
Published with written consent of the patient. We would like to thank
Editage (www.editage.jp) for English language editing.
CONFLICT OF INTEREST
The authors have no conflicts of interest to declare.