Congenital bronchopulmonary vascular malformations, "sequestration" and beyond

Abstract

Congenital bronchopulmonary vascular malformations (BPVMs) include a broad spectrum of disorders that involve abnormalities in the form of disruptions of normal communication and/or presence of abnormal communication between one or more of the three main systems of the lung, namely, the airways, arteries, and veins. The establishment of abnormal communications by means of small openings or anastomoses is termed as malinosculation. The aim of this pictorial essay is to illustrate the imaging appearances of the various types of pulmonary malinosculation.

Keywords: Congenital bronchopulmonary vascular malformations; malinosculation; systematic classification.

Figure 1(A-G)

Graphical representation of various types of bronchopulmonary vascular malinosculation. Type A, B, and C represent isolated bronchial, arterial, and venous malinosculations, respectively. Various combinations of Type A, B, and C result in bronchoarterial (D), bronchovenous (E), arteriovenous (F), and bronchoarteriovenous (G) malinosculations. (Image adapted from Lee et al.[5] and used after modification with authors permission)

Figure 2(A and B)

Isolated bronchial malinosculation - Tracheal bronchus. Axial (A) and coronal (B) chest sections in lung window show the origin of tracheal bronchus (white arrow) supplying the apical segment of the right upper lobe above the level of carina

Figure 3

Isolated bronchial malinosculation - Bronchial atresia. Axial CT chest section in lung window shows a mucoid impacted bronchus (black arrow) supplying the apicoposterior segment of the left upper lobe with hyperinflation (white arrow) of the involved segment

Figure 4

Isolated bronchial malinosculation - Bronchogenic cyst. Axial CT chest section in mediastinal window shows a well-defined non-enhancing cystic lesion (white arrow) of fluid attenuation in the anterior mediastinum

Figure 5(A and B)

Isolated bronchial malinosculation - Congenital pulmonary airway malformation. Axial (A) and coronal (B) CT chest sections in lung window show a large multiseptated cystic lesion (arrows) with a single dominant cyst in the left lower lobe with associated mediastinal shift

Figure 6(A and B)

Isolated bronchial malinosculation - Congenital lobar emphysema. Axial (A) and coronal (B) CT chest sections in lung window show hyperinflated left upper lobe (arrows) with attenuated lung markings and herniation across the midline

Figure 7(A-C)

Isolated arterial malinosculation - Congenital interruption of pulmonary artery. Axial CT chest mediastinal (A, B) and lung window (C) sections show absent right pulmonary artery with normal left pulmonary artery (thin white arrow) and prominent bronchial arteries (thick white arrow) at the right hilum supplying the right lung. Inflammatory changes are noted in the right lung with volume loss

Figure 8(A-D)

Isolated arterial malinosculation - Isolated systemic arterial supply to normal lung. Axial chest CT sections mediastinal (A, B) and lung window (C) show prominent artery (black arrow) arising from the thoracic aorta supplying the left lower lobe. Note the absence of normal pulmonary blood vessels (black arrow) accompanying the left lower lobe bronchi. Angiogram (D) shows prominent systemic artery supplying the left lower lobe. Patient underwent coiling of the systemic artery for complaint of hemoptysis

Figure 9(A-C)

Isolated arterial malinosculation - Dual arterial supply to normal lung. Axial chest CT sections mediastinal (A) and lung window (B) show prominent artery (white arrow) arising from aorta supplying the left lower lobe. Note the presence of normal pulmonary artery branches (black arrow) accompanying the left lower lobe bronchi indicating dual arterial supply. Coronal chest section (C) depicts systemic arterial supply to left lower lobe

Figure 10(A-E)

Isolated venous malinosculation - Partial anomalous pulmonary venous drainage (PAPVC). Axial CT sections in mediastinal window (A-E) show anomalous drainage of the left superior pulmonary vein (thin white arrow) into the left brachiocephalic vein (thick white arrow)

Figure 11(A-D)

Isolated venous malinosculation – Total anomalous pulmonary venous drainage (TAPVC), supracardiac variety. Coronal CT sections (A-D) in lung window show anomalous drainage of common pulmonary venous trunk (black arrows) into the left brachiocephalic vein (white arrow at the junction)

Figure 12(A and B)

Combined bronchoarterial malinosculation – Intralobar sequestration. Axial CT chest sections in mediastinal (A) and lung window (B) show a prominent systemic artery (white arrow) arising from the thoracic aorta supplying a cystic lesion (black arrow) in the posterior basal segment of the right lower lobe. This segment of lung did not show bronchial communication and had normal pulmonary venous drainage (not shown)

Figure 13(A-C)

Combined bronchovenous malinosculation – Congenital pulmonary airway malformation (CPAM) with partial anomalous pulmonary venous drainage. Axial CT chest sections mediastinal (A, B) and lung window (C) show partial anomalous pulmonary venous drainage of the right superior pulmonary vein into the superior vena cava (white arrow) associated with a multicystic lesion lesion (CPAM) in the right upper lobe (black arrow)

Figure 14(A and B)

Combined bronchovenous malformation – Pulmonary hypoplasia and scimitar vein. Chest radiograph (A) shows mediastinal shift to the right side with prominent scimitar vein (arrow) in the right lung base. CT axial section (B) shows scimitar vein draining the right lung into the IVC (arrow) with mediastinal shift to the right due to lung hypoplasia. No anomalous systemic artery was seen to diagnose scimitar syndrome

Figure 15(A-C)

Combined arteriovenous malinosculation – Arteriovenous malformation (AVM). Axial CT chest mediastinal window (A, B) shows a nidus (thin white arrow) of prominent tortuous tangle of vessels in the right upper lobe supplied by a prominent pulmonary artery and drained by prominent pulmonary vein (thick white arrows). 3D volume-rendered image (C) confirms the vascular nature of the lesion with prominent supplying pulmonary artery and vein

Figure 16(A-F)

Combined bronchoarteriovenous malinosculation – Meandering pulmonary vein with right lung hypoplasia and systemic arterial supply to lung. Chest CT axial sections (A-E) in mediastinal window show anomalous course of the right inferior pulmonary vein (white arrow) taking a circuitous course through the right lung before draining into the left atrium (meandering pulmonary vein) and a prominent systemic artery (black arrow) arising from the aorta supplying the right lower lobe. 3D volume-rendered image (F) confirms systemic supply to the right lower lobe (black arrow) and meandering right inferior pulmonary vein (white arrow)