Radiology Case Reports 14 (2019) 36–40

Available online at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/radcr

Case Report

Pelvic congestion syndrome due to agenesis of the

infrarenal inferior vena cava

Terence Menezesa, Ehsan A. Haidera,b,∗, Faten Al-Douria,b, Mohamed El-Khodarya,b,

Ishaq Al-Salmia
a Department of Radiology, St. Joseph’s Healthcare Hamilton, Hamilton, Canada
b Department of Radiology, Faculty of Health Sciences, McMaster University, Hamilton, Canada

a r t i c l e i n f o a b s t r a c t

Article history: The inferior vena cava (IVC) is the main conduit of venous return to the right atrium from
Received 4 March 2018 the lower extremities and abdominal organs. Agenesis of the IVC has an incidence of <1%
Accepted 2 April 2018 in the general population [1], although it has been reported in the literature as occurring in
Available online 6 October 2018 up to 8.7% of the population [2]. Patients with absent IVC may present with symptoms of
lower extremity venous insufficiency [6], idiopathic deep venous thrombosis [7], or pelvic
Keywords: congestion syndrome. To our knowledge there have only been a few cases reported in the
Absence of IVC literature of agenesis of the IVC associated with pelvic congestion syndrome [3,10,11]. We
IVC anomalies present another interesting case of pelvic congestion syndrome due to absent IVC.
Pelvic congestion syndrome © 2018 The Authors. Published by Elsevier Inc. on behalf of University of Washington.
DVT This is an open access article under the CC BY-NC-ND license.
Varices (http://creativecommons.org/licenses/by-nc-nd/4.0/)

how absence of the infrarenal IVC can present as pelvic

1. Introduction congestion syndrome.

Our patient is a 34-year-old female who presented with

gradually worsening pelvic pain. She had a medical history
significant only for varicose veins in the lower extremities.
No other significant previous medical history was elicited. No 2. Case report
prior imaging was available. No prior ultrasound had been
performed. She had never been pregnant and there was no A 34-year-old female presented with gradually worsening
significant obstetrical history, Gravidity Term Preterm Abor- chronic, dull, and aching pelvic pain and menorrhagia over a
tion Living (GTPAL). A contrast-enhanced magnetic resonance few years. No back pain or radiculopathy was present. There
imaging (MRI) of the abdomen and pelvis was performed, and was no history of thrombolysis, intervention, or surgery in
showed complete absence of the infrarenal inferior vena cava the past. Gynecological examinations in the past were unre-
(IVC) with significantly tortuous and dilated vessels in the markable. Her only relevant medical history was some lower
pelvis. Pelvic veins were dilated up to 2 cm. This case shows extremity varicose veins that were treated conservatively. A

∗
Corresponding author at: Department of Diagnostic Imaging, St. Joseph’s Healthcare Hamilton, 50 Charlton Avenue E, Hamilton, ON
L8N 4A6, Canada.
E-mail addresses: tmenezes1@gmail.com (T. Menezes), ehaider@stjosham.on.ca (E.A. Haider), faldouri@stjosham.on.ca (F. Al-Douri),
melkhoda@stjosham.on.ca (M. El-Khodary), dr.ish@hotmail.com (I. Al-Salmi).
https://doi.org/10.1016/j.radcr.2018.04.004
1930-0433/© 2018 The Authors. Published by Elsevier Inc. on behalf of University of Washington. This is an open access article under the
CC BY-NC-ND license. (http://creativecommons.org/licenses/by-nc-nd/4.0/)
 Radiology Case Reports 14 (2019) 36–40 37

Fig. 1 – Axial 2D FIESTA image at the level of the infrarenal abdominal aorta shows no adjacent IVC to the right (thin arrow).
Instead there is some T2 hypointense soft tissue that may represent atretic IVC or collaterals. A normal positioned and
normal caliber aorta is seen to the left (solid arrow).

contrast-enhanced MRI of the abdomen and pelvis was per- veins: the postcardinal, subcardinal, and supracardinal veins
formed, and showed a complete absence of the infrarenal IVC [3]. The IVC is composed of four segments: hepatic, suprarenal,
(Fig. 1). Tortuous dilated vessels were seen in the myometrium renal, and infrarenal. The hepatic segment is derived from
and pelvis (Figs. 2 and 3). Pelvic veins were dilated up to 2 cm. the vitelline vein. The suprarenal segment develops from the
The right external and internal iliac veins joined and then im- right subcardinal vein by formation of the subcardinal-hepatic
mediately drained into a large right lumbar collateral (Fig. 4). anastomosis. The renal segment derives from the right supra-
The left external and internal iliac veins appeared to drain subcardinal and postsubcardinal anastomoses. The infrarenal
into small paravertebral venous channels. The left gonadal segment develops from the right supracardinal vein. In the
vein was dilated measuring 12 mm (Figs. 4 and 5). It drained thoracic region, the supracardinal veins give rise to the azygos
into the left renal vein. The right gonadal vein was replaced and hemiazygos veins. In the abdomen, the postcardinal veins
by multiple tortuous vascular channels, which appeared to are progressively replaced by the subcardinal and supracardi-
drain into the right renal vein. Both renal veins drained into nal veins but persist in the pelvis as the common iliac veins [4].
the infrahepatic IVC. The infrarenal IVC was absent and re- Absence of the entire posthepatic IVC implies that all three-
placed by multiple tortuous vascular channels, which com- paired venous systems failed to develop properly. Absence of
municated with the paravertebral and ascending lumbar ve- the infrarenal IVC suggests failure of development of the pos-
nous plexuses. No filling defects or thrombus was identi- terior cardinal and supracardinal veins. It is difficult to iden-
fied. The remaining portions of the visualized abdomen and tify a single embryonic event that causes either of these sce-
pelvis were unremarkable. The intrahepatic IVC was incom- narios, which leads to controversy as to whether these condi-
pletely imaged and the suprahepatic IVC was out of the field of tions are true embryonic anomalies or the result of perinatal
view. IVC thrombosis [5].
Patients with absent IVC may present with symptoms of
lower extremity venous insufficiency [6], idiopathic deep ve-
nous thrombosis [7], or pelvic congestion syndrome. Although
3. Discussion patients with the absence of infrarenal IVC are generally
asymptomatic, the most common clinical symptom is Deep
Agenesis of the IVC has an incidence of <1% in the general Vein Thrombosis (DVT) [8], which is typically treated with an-
population [1], although it has been reported in the literature ticoagulation, though in our patient no signs or symptoms
occurring in up to 8.7% of the population [2]. IVC develop- of DVT were elicited. Reduced venous flow, venous hyperten-
mental abnormalities occur at 6–10 weeks of gestation when sion, and thrombophilia are felt to play a role in the develop-
the infrahepatic IVC develops from three pairs of embryonic ment of DVT in these cases. Our patient did, however, have
38 Radiology Case Reports 14 (2019) 36–40

Fig. 2 – Axial 2D FIESTA shows dilated parametrial veins up to 2 cm (arrow). On T2-weighted magnetic resonance images,
pelvic varices appear as multiple hyperintense dilated tubular structures around the uterus, ovaries, and pelvic sidewall.

Fig. 3 – Contrast-enhanced, fat-saturated T1 image showing dilated tortuous vessels in the pelvis. The bladder is seen
anteriorly (x).
 Radiology Case Reports 14 (2019) 36–40 39

Fig. 4 – Axial 2D FIESTA image at the expected confluence of the right internal and external iliac veins shows no right
common iliac vein (yellow arrow). Instead the right internal and external iliac veins drain into a large right lumbar vein (red
arrow). A significantly dilated left gonadal vein measuring 12 mm in diameter is also noted (blue arrow). (For interpretation
of the references to color in this figure legend, the reader is referred to the web version of this article.)

Fig. 5 – Coronal LAVA FLEX sequence shows enhancing dilated pelvic vessels (red arrow) with a dilated left ovarian vein
(yellow arrow). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of
this article.)
40 Radiology Case Reports 14 (2019) 36–40

lower extremity varices, which were attributable to an absent

IVC.
4. Conclusion
Pelvic venous congestion is a relatively common and over-
looked condition that can be painful and debilitating for many Complete absence of IVC is rare entity and can be a cause of
women. It was first described in 1857 by Louis Alfred Richet pelvic congestion syndrome. Our patient presented with fea-
to describe a chronic, dull pelvic pain, pressure, and heavi- tures of pelvic congestion syndrome. Cross-sectional MRI can
ness that persisted for >6 months [9]. These symptoms are play a role in diagnosis of absent IVC and associated pathol-
attributable to dilated, tortuous, and congested pelvic veins. ogy. The mainstay of treatment is surgical bypass therapy. Our
Symptoms are exacerbated with prolonged standing, menses, case is one of the few reported in the literature.
and increased abdominal pressure. The symptoms are usually
worse at the end of the day.
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