CASE REPORT
Absence of supra-renal inferior vena cava: case report
Ausência de veia cava inferior suprarrenal: relato de caso
Elton Correia Alves1, Gabriela Bóia Rocha Ferro1, Luciana Karla Lira França1, Mabel Batista Jacó1,
Guilherme Benjamin Brandão Pitta2
Abstract
The congenital absence of the inferior vena cava is a rare and occasional finding at complementary exams or abdominal surgeries. As the majority
of patients without other malformations are asymptomatic, it is hard to estimate the prevalence of these anomalies. Our goal was to report a case
of a 28-year-old patient, male, referred to thorax X-ray due to high fever state (39ºC), nonresponder to medication. The X-ray suggested azygos vein
dilatation. The interview about cardiovascular symptoms revealed thoracic pain during both physical exercises and at rest, and occasional dyspnea
during sleep related to the period of intensification of physical exercises. Computadorized tomography with and without contrast did not solve the
case, what explain the request of an angiotomography, that showed absence of thoracic inferior vena cava.
Keywords: Vena cava, inferior/abnormalities; azygos vein/abnormalities; venous thrombosis/etiology. Resumo
A ausência congênita de veia cava inferior é achado incomum e ocasional durante exames complementares ou cirurgias do abdome. Em razão
de a maioria dos pacientes sem outras malformações serem assintomáticos, há dificuldade em estimar a prevalência dessas anomalias. O objetivo
deste trabalho foi relatar o caso de um paciente de 28 anos, sexo masculino, referido para radiografia de tórax devido a quadro de febre alta (39ºC)
não-responsiva à medicação. Radiografia de tórax sugeriu dilatação de veia ázigos. Em inquérito dirigido para sintomas cardiovasculares, referiu dor
torácica (durante atividade física e em repouso) e dispneia ocasionais durante o sono associada ao período em que intensificou a prática de exercícios.
Realizada tomografia computadorizada sem contraste e, posteriormente, contrastada. As mesmas não esclareceram o caso, fazendo-se necessário
angiotomografia, a qual evidenciou ausência de veia cava inferior torácica.
Palavras-chave: Veia cava inferior/anormalidades; veia ázigos/anormalidades; trombose venosa/etiologia.
Introduction
Case report
Inferior vena cava (IVC) is a large-caliber vessel that
drains blood from the lower limbs, from most of the trunk,
besides abdominal and pelvic organs. It is formed by the
junction of the common iliac veins and lies right of the abdominal aorta, draining into the right atrium. The azygous
system connects the superior and inferior venae cavae directly or indirectly1,2. Congenital absence of IVC is an occasional finding in imaging exams. Because most patients
without other malformations are asymptomatic, it is difficult to estimate the prevalence of such anomaly.
A 28-year-old male patient was admitted to the Hospital
São Sebastião, Maceió (AL), Brazil, with high fever (39oC),
not responding to medication, being referred for radiography of the sinus cavities and chest. He had no relevant past
medical history. Chest radiography was suggestive of azygous
vein dilatation. The fever came down spontaneously after a
week, and no infection or other cause was found. After hospital discharge, he was referred to an angiologist in order to
investigate the chest radiograph finding. At the Clínica de
Especialidades (MedAngio), the patient reported chest pain
Study carried out at Clínica de Especialidades (MedAngio), Maceió (AL), Brazil.
1
Medical Students (5th period) at Universidade Federal de Alagoas (UFAL), Maceió (AL), Brazil.
2
Adjunct Professor at Universidade Estadual de Ciências de Saúde de Alagoas (Uncisal), Maceió (AL), Brazil.
No conflict of interest was declared concerning the publication of this article.
Received on: Oct 26, 2009. Accepted on: Jun 1, 2010.
J Vasc Bras. 2010;9(4):254-256.
Supra-renal inferior vena cava anomaly - Alves EC et al
at exercise and at rest, and occasional dyspnea associated
with a period of intense physical exercise. Computed tomography (CT scan), first performed without a contrast agent
and, later on, with a contrast agent, was inconclusive. Spiral
CT angiography was indicated and showed:
1) continuous IVC azygous (Figure 1);
2) no aneurysms or thrombi;
3) retroaortic renal vein.
The patient was then oriented to discontinue heavy
physical activities due to the high risk of venous thrombosis. He has been asymptomatic for six months.
Discussion
The IVC is formed between the sixth and eighth weeks of embryonic life in a complex process involving three
primitive vein pairs (posterior cardinal, subcardinal and
supracardinal veins), which are extensively anastomosed.
Such anastomosis recede during the embryologic process,
thus originating the IVC3,4. When abnormalities occur during this development, anomalies of IVC such as partial or
total agenesis, may occur3-5. The azygous system tends to
dilate as a compensatory mechanism.
As these anomalies may be asymptomatic, their frequency is hard to measure. However, their prevalence in the
J Vasc Bras 2010, Vol. 9, Nº 4
255
general population is estimated to be 0.5%6. About 90% of
the cases are related to partial absence of the supra-hepatic
segment of the IVC4. Total absence of the renal and suprarenal IVC segments is very rare.
IVC anomalies may be accompanied by vascular, cardiac, bowel, portal and liver malformations, and polyesplenia4. Most cases are asymptomatic and occasionally
found by routine exams not related to the malformation.
However, in case of insufficiency of collateral veins,
blood drainage from the lower limbs and pelvis becomes
inadequate, causing venous stasis and a predisposition
to venous thrombosis7. Bilateral and recurrent deep vein
thrombosis (DVT) in the proximal vein of young patients without history of coagulopathies may imply IVC
anomaly, so imaging methods are required. About 5 to
7% of the patients with DVT present some IVC anomaly;
the incidence is higher in young patients and those with
bilateral DVT6.
There is no indication for interventional treatment
in case of partial IVC absence. However, knowing this
entity is important in order to avoid unnecessary surgical procedures. Since this condition may predispose the
patient to DVT, some authors recommend anticoagulant
therapy, but this is no consensus4. In the present case,
oral anticoagulation therapy and thrombophilia investigation were not necessary because the patient had an
Figure 1 – (A) Abdominal inferior vena cava; (B) IVC with azygous continuation; (C) azygous vein; (D) cava-azygous transition; (E) azygous vein; (F)
hemiazygous vein; (G) azygous vein draining into the right atrium.
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J Vasc Bras 2010, Vol. 9, Nº 4
anatomical congenital alteration, not an acquired functional one; the only recommendation was to restrict
heavy physical activities. Anticoagulant therapy is quite expensive for the patients and, when the disease can
be controlled with behavioral measures, patients accept
the treatment more easily, besides having their quality of
life improved. The patient has been followed for one year
and has been asymptomatic, under the cardiovascular
point of view.
References
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Gardner E, Gray DJ, O’rahilly R. Anatomia. 4. ed. Rio de Janeiro:
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Moore K, Dalley AS. Anatomia orientada para a clínica. 4. ed. Rio
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cava inferior: relato de caso e revisão da literatura. Revista Imagem.
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4.
Felicio ML, Martins AS, Andrade RR, et al. Ausência parcial de
veia cava inferior associada à malformação intestinal. Revista
Brasileira de Cirurgia Cardiovascular. 2007;22:362-4.
Supra-renal inferior vena cava anomaly - Alves EC et al
5.
Lichtenfels E, Becker AS, Pires VC, et al. Diagnóstico etiológico da
trombose venosa profunda de repetição dos membros inferiores.
Jornal Vascular Brasileiro. 2006;5:157-9.
6.
Onzi RR, Costa LF, Angnes RF, et al. Malformação de veia cava inferior e trombose venosa profunda: fator de risco de trombose
venosa em jovens. Jornal Vascular Brasileiro. 2007;6:186-9.
7.
Obernosterer A, Aschauer M, Schnedl W, et al. Anomalies of the
inferior vena cava in patients with iliac venous thrombosis. Ann
Intern Med. 2002;136:37-41.
Correspondence:
Guilherme Benjamin Brandão Pitta
Rua Desportista Humberto Guimarães, 1.081/702 – Ponta Verde
CEP 57035-030 – Maceió (AL), Brazil
E-mail: [email protected]
Authors’ contributions
Study conception and design: ECA
Data analysis and interpretation: ECA, LKLF and GBBP
Data collection: GBRF, ECA and MBJ
Writing: LKLF, MBJ, ECA and GBRF
Critical analysis: GBRF and GBBP
Final text approval*: ECA, GBRF, LKLF, MBJ, GBBP
Statistical analysis: MBJ
Overall responsibility: GBBP
Financing information: N/I
*All authors have read and approved the final version of the paper submitted
to the J Vasc Bras.