Prenatal diagnosis of interrupted inferior vena cava as an isolated finding: a benign vascular malformation

Celentano, Claudio; Malinger, G.; Rotmensch, Siegfried; Gerboni, S; Wolman, Yoram; Glezerman, Marek

doi:10.1046/j.1469-0705.1999.14030215.x

Cited by 30 publications

(22 citation statements)

References 12 publications

(13 reference statements)

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“…Van Praagh et al [7] reported an interrupted IVC in 39 of 46 (85%) postmortem cases of visceral heterotaxy with polysplenia. But, isolated ınterrupted IVC with azygos continuation has been rarely described [8][9][10], comparable with the case we report.…”

Section: Discussionsupporting

confidence: 90%

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Isolated interrupted inferior vena cava with azygos continuation mimicking paraesophageal lymph node enlargement

Yılmaz

Halilçolar

Ünsal

et al. 2016

Monaldi Arch Chest Dis

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Azygos vein dilation has been described in patients with portal vein hypertension, obstruction of superior or inferior vena cava, right heart failure and interruption of the inferior vena cava (IVC). Interrupted IVC with azygos continuation is a rare clinical condition but in this situation, the azygos system dilates to accommodate visceral and lower the extremity of blood return to the right atrium. Although uncommon, these anomalies have been described as an isolated finding and can mimic a mediastinal mass [1,2,3,4]. If isolated interrupted IVC with azygos continuation is together with lung carcinoma, it can be misinterpreted as a lymphadenopathy in a patient who has been evaluated with unopacified CT scans.We present a case of isolated azygos continuation of the interrupted IVC mimicking a paraesophageal lymph node enlargement in a patient with a left hilar mass. Case reportA 68-year-old man was admitted to our hospital presenting a left tumour mass and lymphadenopathy on unopacified Computed Tomography scan of the chest and metastasis of the first and fourth lumbal vertebrae on Magnetic Resonance Imaging. The physical examination revealed a non-healthy man. He was febrile (37.5°C) and had a blood pressure of 150/100 mmHg, a heart rate of 100 beats/min. Breath sounds were diminished on both lungs. The heart and abdominal examinations were unremarkable. The neurological examination was significant for paralysis in both lower extremities. A posteroanterior chest x-ray revealed a left hilar mass. Contrast-enhanced spiral CT scan of the chest showed a tumour mass at the superior segment of the lower left lobe with invasion of the descending aorta and dilated azygos vein (figure 1), mild enlargement of the arch of the azygos, and enlargement of the retrocrural portions of these same veins in the absence of definable inferior vena cava (figure 2). Coronal (figure 3) and sagittal reconstructions showed that the azygos vein was as large as the descending aorta.Fiberoptic bronchoscopy showed no endobronchial lesion except of narrowing and mucosal hyperemia of the superior segment of the lower left lobe. The cytopathologic examination of the brushing and washing specimens was benign.In a routine ultrasound examination of the abdomen, the stomach, heart, liver and spleen were normally positioned and the gallbladder was present. A detailed ultrasound examination of the IVC was not carried out owing to the patient's poor health.Diagnosis of our patient was accepted to be lung carcinoma with bone metastasis and palliative radiotherapy planned. However, our patient did not accept the treatment and was discharged. DiscussionThe azygos vein usually originates below the diaphragma at the level of the first two lumbal verKeywords: Azygos vein, vascular anomaly, mimicking lymph node.

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Section: Discussionsupporting

confidence: 90%

“…This might be a second potential pitfall in the diagnosis of an azygos continuation. This problem may be resolved by late contrast-enhanced scan on CT examination or by use of magnetic resonance imaging [9].…”

Section: Discussionmentioning

confidence: 99%

Isolated interrupted inferior vena cava with azygos continuation mimicking paraesophageal lymph node enlargement

Yılmaz

Halilçolar

Ünsal

et al. 2016

Monaldi Arch Chest Dis

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“…Although the majority of cases with IVC interruption are associated with visceral heterotaxy, especially in LAI, it can occur as an isolated finding, which is very rare [22]. When IVC is absent by ultrasonography, examiners should keep in mind the possibility of associated cardiac anomalies, and if no abnormalities are detected, the prognosis is excellent unlike that associated with LAI [23].…”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of atrial isomerism in the Korean population

et al. 2014

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ObjectiveTo report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes.MethodsA total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution.ResultsOf 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSD), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outlet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%.ConclusionOur study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSD and complete heart block in LAI and a better survival rate in RAI. Although the postnatal outcomes for RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.

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“…Persistent left superior vena cava to the coronary sinus is a more common venous anomaly that has been recognized in the fetal life, both with and without cardiosplenic syndrome [10]. The occurrence of congenitally interrupted inferior vena cava without heterotaxia syndrome is extremely rare [11][12][13][14][15], and the frequency of associated additional vascular and cardiac anomalies in these patients is not well known [11]. Failure to recognize this anomaly can result in various problems during invasive medical or surgical procedures [16,17].…”

Section: Discussionmentioning

confidence: 99%

Interrupted inferior vena cava and persistent left superior vena cava without heterotaxia in a fetus

Hartas

Gupta-Malhotra

2010

Journal of Neonatal-Perinatal Medicine

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Presence of systemic venous anomalies including a persistent left superior vena cava to a dilated coronary and interrupted inferior vena cava with azygous continuation are extremely rare in absence of heterotaxia or cardiosplenic syndrome. For this reason, the diagnosis of extracardiac venous anomalies can often be missed in absence of a heterotaxia as was the case in our patient whose prior fetal echocardiogram and the first postnatal echocardiogram read by other pediatric cardiologists failed to detect these anomalies. We report these systemic venous anomalies in a fetus in the absence of heterotaxia and further describe the echocardiographic findings helpful in their detection in-utero.

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Prenatal diagnosis of interrupted inferior vena cava as an isolated finding: a benign vascular malformation

Cited by 30 publications

References 12 publications

Isolated interrupted inferior vena cava with azygos continuation mimicking paraesophageal lymph node enlargement

Isolated interrupted inferior vena cava with azygos continuation mimicking paraesophageal lymph node enlargement

Prenatal diagnosis of atrial isomerism in the Korean population

Interrupted inferior vena cava and persistent left superior vena cava without heterotaxia in a fetus

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