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Journal of Radiology Case Reports

Meandering pulmonary vein: A case report

Case Report

Sneha Harish C1*, Rashmi Dixit1, Ayush Agarwal1, Anju Garg1

Radiology Case. 2020 Mar; 14(3):7-13 :: DOI: 10.3941/jrcr.v14i3.3799

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1. Department of Radiology, Maulana Azad Medical College & LNJP Hospital, New Delhi, India

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  ABSTRACT
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Anomalies of the pulmonary venous circulation are extremely uncommon. We report a case of an anomalous unilateral single pulmonary vein also referred to as a meandering pulmonary vein. A single large tortuous pulmonary vein was seen on the right side draining into the left atrium with associated ipsilateral absence of the middle lobe bronchus and interlobar fissures. This is considered a variant of the classical scimitar syndrome. The venous anatomy was depicted with considerable accuracy using multidetector computed tomography (MDCT) non-invasively.








  CASE REPORT
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A 28-year-old female referred from the department of obstetrics and gynecology was undergoing work up for primary infertility. She had no complaints pertaining to the respiratory system and the physical examination was unremarkable. Her chest radiograph showed an abnormal tubular radiopacity in the para cardiac region of the right lung with mild volume loss of the right lung (Fig. 1 Preview this figure

Figure 1: Conventional Radiography
28-year-old female with meandering pulmonary vein. Findings: Fig (a) Chest x-ray shows a tubular opacity (arrow) in the right lower zone in the para cardiac region with mild volume loss of the right lung. The left lung field is clear and cardiothoracic ratio is 0.5. Fig(b) is the magnified image showing the tubular opacity. Technique: Frontal chest x-ray posteroanterior view obtained using 125 kV and 1.75mAs.
). The cardiac silhouette appeared normal. Scimitar syndrome was suspected based on the chest x-ray and contrast enhanced computed tomography scan of the thorax was done. On contrast enhanced CT (CECT) there was replacement of the normal right pulmonary veins by an abnormal single tortuous pulmonary vein with a meandering course through the right lung beginning in the right upper lobe and coursing posteriorly and medially and draining into the left atrium (Fig. 2 Preview this figure
Figure 2: Computed Tomography
28-year-old female with meandering pulmonary vein. Findings: Axial (a, b & c) sections of CECT thorax of a 28-year-old female from superior to inferior levels in mediastinal window show the main pulmonary artery dividing into right (red arrow in a) and left (white arrow in a) pulmonary arteries which appear normal in course and caliber. The left superior (blue arrow in b) and left inferior pulmonary veins (blue arrow in c) are seen draining normally into the left atrium (asterisk). The tortuous meandering course of the prominent anomalous right pulmonary vein is well seen in the axial (d & g), coronal (e & h), sagittal (f & i) MIP images (yellow arrows) as it courses anteroinferiorly initially, then posteriorly through the right lung and turns medially to drain into the left atrium (asterisk) which appears normal in morphology. The inferior vena cava is also seen (red arrow in h). Technique: 128 slice CT, SIEMENS SOMATOM Definition AS+ Contrast enhanced CT scan [146mAs, 120 kV, slice thickness- 1mm (a,b,c), 10 mm(d,e,f), 41.2mm(g,h), 27.2mm (i)] acquired 60 seconds after intravenous contrast injection (60 mL of nonionic contrast medium; Omnipaque 350mgI/mL, GE Healthcare, USA). Axial CECT images(a,b,c)-Window level:40 and window width: 400. MIP images (d,e,f)-Window level:64 and window width:400; MIP image(g)- Window level: 82 and window width: 271; MIP images (h,i)- Window level:100 and window width:500.
). The main pulmonary artery, right and left pulmonary arteries were found to be normal. No systemic collateral vessels were seen. In addition, there was absence of the right middle lobe bronchus, the horizontal and oblique fissures with a single fissure seen separating the apical segment of the right upper lobe from the rest of the right lung parenchyma suggesting pulmonary hypoplasia (Fig. 3 Preview this figure
Figure 3: Computed Tomography
28-year-old female with meandering pulmonary vein. Findings: Axial (a) and sagittal(b) CT images in lung window show the single fissure (arrows) separating the apical segment of the right upper lobe anteriorly from the rest of the right lung. The anomalous pulmonary vein is seen in cross section as a nodular opacity in the right lower lobe (red arrow in b). Axial (c), coronal (d) and sagittal (e) MinIP images show the right upper lobe bronchus (yellow arrows) and right lower lobe bronchi (white arrows) with non-visualization of the right middle lobe bronchus. The rounded density seen in the sagittal image (red arrow in e) represents the anomalous right pulmonary vein. The normal branching pattern of the left lung bronchi is also seen (c,d). Technique: 128 slice CT, SIEMENS SOMATOM Definition AS+ Contrast enhanced CT scan [146mAs, 120 kV, slice thickness- 1 mm (a,b); 100mm(c,d,e)] acquired 60 seconds after intravenous contrast injection (60 mL of nonionic contrast medium; Omnipaque, GE Healthcare, USA). Window level: -600 and window width: 1200.
). Echocardiography of the patient was found to be normal. Since the patient was asymptomatic no intervention was done and the patient has been kept on follow-up.

  DISCUSSION
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Meandering pulmonary vein also called pseudo-Scimitar syndrome or Scimitar variant is an extremely rare pulmonary venous anomaly which could be confused with the classical Scimitar syndrome which is a relatively more common condition with similar appearance of a linear opacity in para cardiac location on chest x-ray. Unlike the Scimitar syndrome where the abnormal pulmonary vein drains into the inferior vena cava, in pseudo-scimitar the pulmonary vein has an abnormal course but drains normally into the left atrium. The present case is an example of this condition.


Etiology & Demographics:
Congenital anomalies of the pulmonary venous system have been divided into 3 types, namely, partial anomalous pulmonary venous drainage with or without abnormal course in the lung, anomalous venous route without abnormal connection and lastly abnormal venous diameters including varices, stenosis and atresia by Remy-Jardin et al [1]. According to this classification our case falls into the second category i.e. anomalous venous route without abnormal connection.

It has been hypothesized that these anomalies occur during the primitive splanchnic capillary stage of development. The splanchnic plexus consisting of the umbilicovitelline veins, cardinal veins and common pulmonary vein drains the immature lung. Later on, the common pulmonary vein gets incorporated into the left atrium and forms the pulmonary veins which drain into the left atrium while the umbilicovitelline veins and cardinal veins undergo regression at around 30 to 32 days [2,3]. Atresia of one of the pulmonary veins after the splanchnic circulation has separated but before pulmonary segmentation has occurred might be the cause of AUSPV formation [4].

Most of these cases are detected incidentally when imaged for some other pathology. Hence the age of detection varies significantly with cases being reported in infants and patients as old as 80 years of age. These anomalies have been reported in both males and females. In the review by Rodrigues et al a slight female predominance was seen although the authors did not mention any specific gender predilection [5].


Clinical & Imaging findings:
As the anomalous vessel drains into the left atrium, right to left shunting is not seen and hence the patients are usually asymptomatic, mostly detected incidentally. Several variants of the Scimitar syndrome have been described of which one rare variant includes a single tortuous scimitar like vein draining into the left atrium. The first of such cases was reported in 1968 by Kozuka and Nosaki [6]. The term “meandering pulmonary vein” was first used by Goodman et al to describe a single large right pulmonary vein which drained into the left atrium instead of the inferior vena cava as would be seen in a case of true scimitar vein [7]. The term anomalous unilateral single pulmonary vein (AUSPV) was used for a similar case by Goudarzi et al [2]. Unlike the true scimitar syndrome, the drainage in case of AUSPV is orthotopic.

Our case had associated right lung hypoplasia, absence of the right middle lobe bronchus and interlobar fissures with normal pulmonary arteries. Several cases have been reported with associated anomalies in patients with a meandering pulmonary vein and hence one should be on the lookout for other findings while evaluating these cases. Tortoriello et al described a case of meandering right pulmonary vein with dual drainage both to the IVC and left atrium [8]. A case of bilateral anomalous single pulmonary veins draining into the left atrium was reported by Hidvegi and Lapin [9]. In the case described by Goodman et al hypoplasia of the right main pulmonary artery and mirror image lung was noted [7]. One of the 2 cases reported by Hasuo et al had partial anomalous pulmonary venous drainage of the posterior basal vein into the IVC [10]. Right lung and pulmonary artery hypoplasia with bilateral left sided bronchial configuration were observed in association with AUSPV by Goudarzi et al [2]. Most of the reported cases of meandering pulmonary vein are of the right side with very few cases of left sided involvement being described such as the case reported by Aygun et al [11].

Although pulmonary angiography is the gold standard for diagnosis of pulmonary venous anomalies non-invasive techniques like MDCT and magnetic resonance angiography (MRA) can satisfactorily delineate the vascular anatomy with minimal patient discomfort, MRA having the added advantage of using non-ionizing radiation. Angiography should be reserved for doubtful cases or when intervention is required.


Treatment & Prognosis:
It is important to distinguish meandering pulmonary vein from other pathologies and make the correct diagnosis as management varies significantly. As was seen in our patient isolated AUSPVs are usually asymptomatic and they do not require any surgical intervention [9]. These patients are kept on follow up and they have a good prognosis.


Differential Diagnoses:
AUSPV or meandering pulmonary vein can mimic several other pathologies such as the true scimitar syndrome, pulmonary nodules, pulmonary arteriovenous malformations, pulmonary varices etc [12,2,3,10].
• Classical Scimitar syndrome: It consists of hypoplasia of the right lung, dextroposition of the heart, hypoplasia of the right pulmonary artery, an abnormal pulmonary vein draining into the inferior vena cava (IVC) and a systemic collateral supply to the lung [12].
• Pulmonary varix: It refers to localized congenital dilatation of the pulmonary vein. On chest x-ray it is seen as a smooth lobulated soft tissue lesion or a mass or a tubular peripheral opacity in the lung. In addition to the above findings transfissural collaterals can also be seen on CT. The angiographic criteria for the diagnosis include presence of a normal pulmonary arterial tree, drainage of the varix by the pulmonary vein, filling of the varix at the same rate as the normal pulmonary vein and drainage into the left atrium with no shunt formation between systemic and pulmonary circulations. Also, there is delayed emptying of the varix in comparison to the normal pulmonary veins [13].
• Pulmonary arteriovenous malformations: They occur as a result of abnormal communications between pulmonary arteries and veins without the intervening capillary bed. On chest x-ray they are seen as homogenous round or oval opacities along with curvilinear opacities coursing towards the hilum. CT angiography is the gold standard in which it is seen as a well-defined lesion with feeding artery and draining vein [14].
• Pulmonary nodules: The anomalous pulmonary vein can sometimes be confused with nodules due to various pathologies. These can be delineated on computed tomography which shows better depiction of the actual pathology.

  TEACHING POINT
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Abstract
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Discussion
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Meandering pulmonary vein is a variant of the classical scimitar syndrome in which the pulmonary vein has an abnormal course but normal drainage into the left atrium. Since this condition generally does not require any intervention it is important to distinguish this entity from the classical scimitar syndrome in which the abnormal vessel drains into the inferior vena cava.








  FIGURES
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Display figure 1 in original size

Figure 1: 28-year-old female with meandering pulmonary vein.

Findings:
Fig (a) Chest x-ray shows a tubular opacity (arrow) in the right lower zone in the para cardiac region with mild volume loss of the right lung. The left lung field is clear and cardiothoracic ratio is 0.5. Fig(b) is the magnified image showing the tubular opacity.

Technique:
Frontal chest x-ray posteroanterior view obtained using 125 kV and 1.75mAs.


Figure 1: Conventional Radiography (Open in original size)
28-year-old female with meandering pulmonary vein.

Findings:
Fig (a) Chest x-ray shows a tubular opacity (arrow) in the right lower zone in the para cardiac region with mild volume loss of the right lung. The left lung field is clear and cardiothoracic ratio is 0.5. Fig(b) is the magnified image showing the tubular opacity.

Technique:
Frontal chest x-ray posteroanterior view obtained using 125 kV and 1.75mAs.


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Display figure 2 in original size
Figure 2: 28-year-old female with meandering pulmonary vein.

Findings:
Axial (a, b & c) sections of CECT thorax of a 28-year-old female from superior to inferior levels in mediastinal window show the main pulmonary artery dividing into right (red arrow in a) and left (white arrow in a) pulmonary arteries which appear normal in course and caliber. The left superior (blue arrow in b) and left inferior pulmonary veins (blue arrow in c) are seen draining normally into the left atrium (asterisk). The tortuous meandering course of the prominent anomalous right pulmonary vein is well seen in the axial (d & g), coronal (e & h), sagittal (f & i) MIP images (yellow arrows) as it courses anteroinferiorly initially, then posteriorly through the right lung and turns medially to drain into the left atrium (asterisk) which appears normal in morphology. The inferior vena cava is also seen (red arrow in h).

Technique:
128 slice CT, SIEMENS SOMATOM Definition AS+
Contrast enhanced CT scan [146mAs, 120 kV, slice thickness- 1mm (a,b,c), 10 mm(d,e,f), 41.2mm(g,h), 27.2mm (i)] acquired 60 seconds after intravenous contrast injection (60 mL of nonionic contrast medium; Omnipaque 350mgI/mL, GE Healthcare, USA). Axial CECT images(a,b,c)-Window level:40 and window width: 400. MIP images (d,e,f)-Window level:64 and window width:400; MIP image(g)- Window level: 82 and window width: 271; MIP images (h,i)- Window level:100 and window width:500.


Figure 2: Computed Tomography (Open in original size)
28-year-old female with meandering pulmonary vein.

Findings:
Axial (a, b & c) sections of CECT thorax of a 28-year-old female from superior to inferior levels in mediastinal window show the main pulmonary artery dividing into right (red arrow in a) and left (white arrow in a) pulmonary arteries which appear normal in course and caliber. The left superior (blue arrow in b) and left inferior pulmonary veins (blue arrow in c) are seen draining normally into the left atrium (asterisk). The tortuous meandering course of the prominent anomalous right pulmonary vein is well seen in the axial (d & g), coronal (e & h), sagittal (f & i) MIP images (yellow arrows) as it courses anteroinferiorly initially, then posteriorly through the right lung and turns medially to drain into the left atrium (asterisk) which appears normal in morphology. The inferior vena cava is also seen (red arrow in h).

Technique:
128 slice CT, SIEMENS SOMATOM Definition AS+
Contrast enhanced CT scan [146mAs, 120 kV, slice thickness- 1mm (a,b,c), 10 mm(d,e,f), 41.2mm(g,h), 27.2mm (i)] acquired 60 seconds after intravenous contrast injection (60 mL of nonionic contrast medium; Omnipaque 350mgI/mL, GE Healthcare, USA). Axial CECT images(a,b,c)-Window level:40 and window width: 400. MIP images (d,e,f)-Window level:64 and window width:400; MIP image(g)- Window level: 82 and window width: 271; MIP images (h,i)- Window level:100 and window width:500.


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Display figure 3 in original size
Figure 3: 28-year-old female with meandering pulmonary vein.

Findings:
Axial (a) and sagittal(b) CT images in lung window show the single fissure (arrows) separating the apical segment of the right upper lobe anteriorly from the rest of the right lung. The anomalous pulmonary vein is seen in cross section as a nodular opacity in the right lower lobe (red arrow in b). Axial (c), coronal (d) and sagittal (e) MinIP images show the right upper lobe bronchus (yellow arrows) and right lower lobe bronchi (white arrows) with non-visualization of the right middle lobe bronchus. The rounded density seen in the sagittal image (red arrow in e) represents the anomalous right pulmonary vein. The normal branching pattern of the left lung bronchi is also seen (c,d).

Technique:
128 slice CT, SIEMENS SOMATOM Definition AS+
Contrast enhanced CT scan [146mAs, 120 kV, slice thickness- 1 mm (a,b); 100mm(c,d,e)] acquired 60 seconds after intravenous contrast injection (60 mL of nonionic contrast medium; Omnipaque, GE Healthcare, USA). Window level: -600 and window width: 1200.


Figure 3: Computed Tomography (Open in original size)
28-year-old female with meandering pulmonary vein.

Findings:
Axial (a) and sagittal(b) CT images in lung window show the single fissure (arrows) separating the apical segment of the right upper lobe anteriorly from the rest of the right lung. The anomalous pulmonary vein is seen in cross section as a nodular opacity in the right lower lobe (red arrow in b). Axial (c), coronal (d) and sagittal (e) MinIP images show the right upper lobe bronchus (yellow arrows) and right lower lobe bronchi (white arrows) with non-visualization of the right middle lobe bronchus. The rounded density seen in the sagittal image (red arrow in e) represents the anomalous right pulmonary vein. The normal branching pattern of the left lung bronchi is also seen (c,d).

Technique:
128 slice CT, SIEMENS SOMATOM Definition AS+
Contrast enhanced CT scan [146mAs, 120 kV, slice thickness- 1 mm (a,b); 100mm(c,d,e)] acquired 60 seconds after intravenous contrast injection (60 mL of nonionic contrast medium; Omnipaque, GE Healthcare, USA). Window level: -600 and window width: 1200.


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Display figure 4 in original size
Figure 4: Summary table for meandering pulmonary vein

Figure 4: Table (Open in original size)
Summary table for meandering pulmonary vein

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Display figure 5 in original size
Figure 5: Differential diagnosis table for meandering pulmonary vein

Figure 5: Table (Open in original size)
Differential diagnosis table for meandering pulmonary vein

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  REFERENCES
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1. Remy-Jardin M, Remy J. Spiral CT angiography of the pulmonary circulation. Radiology 1999 Sep;212(3):615-36 Get full text
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2. Goudarzi M, Sabouri S, Fayazi N, Shabestari AA, Karam MB, Kahkouee S. Anomalous unilateral single pulmonary vein mimicking pulmonary nodules on HRCT of the lungs and scimitar syndrome on chest radiograph: multidetector CT findings. Journal of thoracic imaging 2009 May 1;24(2):142-6 Get full text
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3. Hyun D, Do YS, Lim SJ, Park HS, Park KB. Anomalous unilateral single pulmonary vein mimicking pulmonary arteriovenous malformation. Cardiovascular and interventional radiology 2014 Jun 1;37(3):835-8 Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine

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4. Hanson JM, Wood AM, Seymour R, Petheram IS. Anomalous unilateral single pulmonary vein: two cases mimicking arteriovenous malformations and a review of the literature. Australasian radiology 2005 Jun;49(3):246-51 Get full text
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5. Rodrigues MA, Ritchie G, Murchison JT. Incidental meandering right pulmonary vein, literature review and proposed nomenclature revision. World journal of radiology 2013 May 28;5(5):215 Get full text
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6. Kozuka T, Nosaki T. A pulmonary vein anomaly: unusual connection and tortuosity of the right lower lobe vein. The British journal of radiology 1968 Mar;41(483):232-4 Get full text
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7. Goodman LR, Jamshidi A, Hipona FA. Meandering right pulmonary vein simulating the scimitar syndrome. Chest 1972 Oct 1;62(4):510-2 Get full text
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8. Tortoriello TA, Vick III GW, Chung T, Bezold LI, Vincent JA. Meandering right pulmonary vein to the left atrium and inferior vena cava: the first case with associated anomalies. Texas Heart Institute Journal 2002;29(4):319 Get full text
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9. Hidvegi RS, Lapin J. Anomalous bilateral single pulmonary vein demonstrated by a 3-dimensional reconstruction of helical computed tomographic angiography: case report. Canadian Association of Radiologists Journal 1998 Aug 1;49(4):262 Get full text
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10. Hasuo K, Numaguchi Y, Kishikawa T, Ikeda J, Matsuura K. Anomalous unilateral single pulmonary vein mimicking pulmonary varices. Chest 1981 May 1;79(5):602-4 Get full text
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11. Aygun MS, Calik M, Calik SG. Meandering pulmonary vein: Very rare incidental finding. Eurasian Journal of Pulmonology 2018 Jan 1;20(1):43 Get full text
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12. Gao YA, Burrows PE, Benson LN, Rabinovitch M, Freedom RM. Scimitar syndrome in infancy.J Am Coll Cardiol.1993;22:873-882. Get full text
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13. Shostak E, Ameer R, Gruden J, Jessurun J. A Diagnostic Conundrum: Progressive Tubular Lung Mass in Asymptomatic Young Woman. Chest 2019 May 1;155(5):e131-5 Get full text
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14. Tellapuri S, Park HS, Kalva SP. Pulmonary arteriovenous malformations. The international journal of cardiovascular imaging 2018 Nov 1:1-8 Get full text
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  ABBREVIATIONS
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AUSPV = Anomalous unilateral single pulmonary vein
CECT = Contrast enhanced computed tomography
IVC = Inferior vena cava
MDCT = Multidetector computed tomography
MRA = Magnetic resonance angiography









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