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Abdominopelvic venous distension with venous varices and iliac vein aneurysm: rare imaging manifestation of chronic rheumatic heart disease
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  1. Muniraju Maralakunte1,
  2. Arun Sharma1,
  3. Neelam Dahiya2 and
  4. Manphool Singhal1
  1. 1Radiodiagnosis, Post Graduate Institute of Medical Education and Research, Chandigarh, India
  2. 2Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
  1. Correspondence to Dr Arun Sharma; drarungautam{at}gmail.com

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Description

A middle-aged postmenopausal woman presented with abdominal distension and dull aching non-localising abdominal pain for a month, and sonography of the abdomen was advised for the same. A moderate amount of ascites was seen on sonography with congestive hepatomegaly and dilated inferior vena cava (IVC) and hepatic veins. More strikingly, dilated and tortuous anechoic vascular structures are seen in the pelvis, which could be traceable along the course of gonadal veins on either side. On review of her medical records, there was a significant history of rheumatic heart disease and its treatment course, including mitral balloon valvuloplasty done at separate time intervals in 2004 and 2006. Metallic mitral valve replacement was considered in 2016 due to the recurrence of symptoms. She improved clinically and was kept on medical treatment, viz. digoxin 0.25 mg, torsemide 20 mg, metoprolol tartrate 50 mg, aspirin 325 mg, nicoumalone 2 mg. Her recent echocardiography report showed dilated atria, severe tricuspid regurgitation ('tricuspid regurgitation velocity max (TRV max) 3.3 m/s, peak gradient (PG) 42.70 mm Hg), pulmonary hypertension, global left ventricular hypokinesia and severe left ventricular systolic dysfunction (ejection fraction 20%–25%). Aortic valve was mildly thickened with presence of mild aortic regurgitation and normally functioning mechanical prosthetic mitral valve. Chest radiograph showed cardiomegaly with prominent pulmonary conus. Right sided cardiac chambers were dilated. Contrast-enhanced CT revealed abdominopelvic venous varices involving the bilateral gonadal veins, bilateral iliac veins (common, internal and external), IVC and hepatic veins with the presence of right internal iliac venous aneurysm measuring 4.4×4.3×3.6 (anteroposterior×craniocaudal×transverse) cm (figure 1). Mild bilateral lower extremity pitting oedema was seen. However, no varicose veins or chronic cutaneous changes to suggest deep venous thrombosis were seen.

Figure 1

Postmenopausal woman with rheumatic heart disease status postmetallic mitral valve replacement, and presenting with non-localising abdominal pain and abdominal distension. Chest radiograph (A) showing cardiomegaly with prominent pulmonary conus. Contrast-enhanced CT (B–E) axial images showing disproportionately dilated right sided cardiac chambers (B), serpentine right adnexal varices (C) (black arrow), globular internal iliac venous aneurysm (~4.4×4.3×3.6 cm anteroposterior×craniocaudal×transverse) (D) (black arrow), and periuterine veins (white arrow). Coronal maximum intensity projection reformatted image (E) showing dilated inferior vena cava (IVC) and hepatic veins (HV), dilated renal and gonadal veins (right gonadal vein) and ascites. RA, right atrium; RV in B, right ventricle; LV, left ventricle; rGV, right gonadal vein; RV in E, renal vein; AP×CC×TR, anteroposterior×craniocaudal×transverse.

The reported incidence of the internal iliac venous (IIV) aneurysm is extremely rare. Only four cases with IIV aneurysms are reported to date in the literature; the index case is the first female subject with an IIV aneurysm. Congestion of the IVC and hepatic veins are commonly observed in patients with underlying cardiac disease. However, there is a single case report association of pelvic venous insufficiency with the inferior vena caval reflux (due to tricuspid regurgitation).1 Long-standing rheumatic heart disease and its complications such as tricuspid regurgitation could have caused increased venous pressure with the development of IIV aneurysm and pelvic and gonadal varices in the index case. Iliac venous aneurysms are rare clinical conditions, and the external iliac vein (60.4%) is the most frequent site, followed by the common iliac vein (25%).2 Iliac venous aneurysms could be primary or secondary, and traumatic arteriovenous fistula (AFV) is the most common (32%) secondary cause.2 May–Thurner syndrome is the most common cause of primary iliac venous aneurysms in women.2 Currently, knowledge regarding clinical presentation and management of the iliac venous aneurysms is elusive. A recent systemic review reported that most patients with iliac venous aneurysms are asymptomatic; however, patients may present with lower limb swelling and pain.2 Thromboembolic events, aneurysmal rupture and mass effects are potential complications related to the iliac venous aneurysm. There is a theoretical possibility of torrential haemorrhage secondary to aneurysmal rupture due to the spacious loose pelvic compartment. Duplex ultrasound, CT or magnetic resonance venography are the diagnostic imaging modalities that could be used to diagnose and assess the aneurysmal size and its complications if any. Catheter venography is generally reserved for endovascular treatment of the condition. It is pertinent to highlight the fact that the first therapeutic goal should be to optimally manage the heart valve problems and the poor heart performance to prevent such complications. Anticoagulation, open surgery (including aneurysmal resection, AFV ligation), endovascular (coil embolisation) and hybrid techniques are the different management strategies reported in the literature. Administration of continuous anticoagulants to the index case could have prevented the thromboembolic phenomenon. Optimal treatment of rheumatic heart disease-related tricuspid regurgitation may even halt the further progression of iliac venous aneurysm.

Learning points

  • Internal iliac venous (IIV) aneurysm is an extremely rare condition.

  • Current knowledge on aetiology, clinical presentation and management of the IIV aneurysm is elusive.

  • Long-standing rheumatic heart disease and its complications such as tricuspid regurgitation could have caused increased venous pressure with the development of IIV aneurysm.

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Footnotes

  • Contributors All authors (MM, AS, ND, MS) contributed equally in planning, conception and design, writing, interpretation, revision and final approval of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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