Intralobar pulmonary sequestration supplied by vessel from the inferior vena cava: Literature overview and case report

Abstract

Sequestration is a congenital abnormality that can occur in both children and adults. The clinical presentation often manifests as recurrent pneumonia throughout the lifetime of the patient. Pathologically, sequestration is a disorganized region of lung parenchyma without a normal pulmonary artery and with no interconnecting air passage (ie, it is isolated from the bronchi and pulmonary arteries). Sequestration can be either intralobar or extralobar and is usually supplied with blood from an anomalous vessel originating from the thoracic aorta or abdominal aorta (big circulation/systemic circulation), which is one of the mandatory criteria for diagnosing sequestration. CT angiography or catheter angiography can assist in identifying the anomalous vessel both for diagnosis and surgical resection. We present a rare case of intralobar sequestration with the distinction of being supplied with blood from the abdominal vena cava, as confirmed by CT angiography, surgery, and postoperative pathology. It is hoped that this study will contribute to the literature by introducing a rare case of congenital lung abnormality and pulmonary vascular malformation.

Keywords: Congenital pulmonary airway malformation; Extra lobar pulmonary sequestration; Interlobar pulmonary sequestration; MRR, Multiplanar reconstruction; Pulmonary sequestration; VAS, Video-assisted surgery; VRT, Volume rendering technique.

Fig. 1

Chest radiograph on admission (high-kV film). Suspected abnormal opacity located close to the spine, medial to the right radial-diaphragmatic angle (arrow).

Fig. 2

CT scan of the lung window and mediastinum before intravenous contrast injection. A: A soft tissue mass is seen, with a size of 36 × 26 mm (measured on lung window), a clear borderline, located close to but still separate from the spine (separated by a fat layer denoted by the red arrow). B, C, D: Anteriorly, there is a characteristic image of the pulmonary vein branching into the soft tissue space in front of the spine and D: connecting to an azygos vein (blue arrow).

Fig. 3

Images of chest CT scan in lung and mediastinal windows after contrast administration (early phase). Axial and MPR images. A: The lung window shows an abnormal vessel emerging from the mass, branching in the normal lung tissue (yellow arrow). B: The vascular branch is separated from the abdominal vena cava (just above the point of origin of the hepatic vein); C, D, E: Branches via the diaphragm, ascends outwards, penetrates the mass, and branches in the parenchymal region to the right border of the mass (red arrow). F: The abdominal aorta (which is not associated with any other abnormal vessel) passes through the diaphragm and branches off the intercostal vessels (white arrow).

Fig. 4

Late-phase chest CT scan after intravenous contrast. A, B: Axial and coronal mediastinal windows show a mass with many cyst-like hypodense foci inside (red arrow). B: Abnormal branching to sequestration arising from the abdominal vena cava (yellow arrow).

Fig. 5

VRT anterior chest image view. A: Abnormal branch of blood supply to sequestration mass (red arrow). B: Abdominal vena cava (yellow arrow).

Fig. 6

Magnetic resonance imaging (MRI) of the thoracic spine with intravenous Gadolinium injection. A: T2 Stir; B: T2 Stir after injection (Axial) and C: T2 Stir after injection (Coronal) both show a dissected right lower lobe parenchymal mass, unrelated to the spine (arrow).

Fig. 7

Observational images during laparoscopic surgery and gross specimens taken after surgery. A: Abnormal vascular branch ascends from the superior surface of the right diaphragm (yellow arrow). B: Vasectomy with cardiac staple. C: Posterior vascular truncation process cut above the diaphragm (blue arrow). D, E: Postoperative isolated lung mass and abnormal branch lumen which was smooth and showed no venous valve (white arrow). Conclusion: The macroscopic image is consistent with the sequestration structure and the blood supply artery.

Fig. 8

Microscopic anatomy of the sequestration specimen after surgery.

Fig. 9

Microscopic anatomy of the patient's blood supply to the sequestration mass after surgery.

Fig. 10

Chest X-ray 40 days after surgery. A blurred band is visible at the base of the right lung (arrow).

Fig. 11

Unenhanced chest CT 10 months after surgery. A strip of the staple suture is still visible in the right lower lobe.