Pulmonary Sequestration: A 29 Patient Case Series and Review

Abstract

Introduction: Pulmonary sequestration also known as bronchopulmonary sequestration is a rare disease, with very few case series reviewed in literature. In this study, we review the demographics, presentation, imaging and treatment of pulmonary sequestration in 29 patients from our institution, and provide comparison data from previously published series with an overview of the disease history.

Materials and methods: Records reviewed for all patients evaluated and treated in our institution with a pathological proven diagnosis of pulmonary sequestration from January 2004 through December 2013. Collected data included demographics, clinical presentation, diagnostic imaging, location of the lesion, type of sequestration, and subsequent treatment.

Results: Of the 29 patients reviewed 8 (28%) were children 0-2 years, 1 adolescent age 17, and 20 (69%) adults 21-70 years with a mean age of 42 among adults. Systemic arterial supply to the sequestered segment was demonstrated with computed tomographic angiography (CTA) in 25 patients (86%). In 19 patients (66%), the sequestered segment was located in the left lower lobe, and 16 (55%) were intralobar.

Conclusion: Diagnostic delays of pulmonary sequestration were common among the adult population as the presenting symptoms often mimicked other common pulmonary diseases, such as pneumonia and asthma. These findings were consistent among previously published series. CTA was the preferred imaging modality for preoperative planning with high sensitivity and specificity in identifying the lesion.

Keywords: Bronchopulmonary malformations; Computed tomographic angiography; Congenital lung diseases; Intralobar and extralobar sequestration.

[Table/Fig-1]:

Graphic representation of the bronchopulmonary malformations spectrum (CLH: congenital lobar hyperinflation, BC: bronchogenic cyst; CPAM: congenital pulmonary airway malformation; BA: bronchial atresia; PS: pulmonary sequestration; AVM: arteriovenosus malformations [5].

[Table/Fig-3]:

(a) Chest X-Ray, PA and lateral views demonstrating a right lower lobe round mass-like density (arrows), this is better visualized on the lateral view as the mass is located posterior to the heart. (b) Axial CTA chest revealing an aberrant artery branching off the descending thoracic aorta to supply a wedge shaped mass-like vascular lesion in the medial-basal segment of the right lower lobe. (c) 3D reconstructed MRA- left posterior oblique view showing the aberrant artery branching off the descending thoracic aorta, just above the diaphragm.

[Table/Fig-4]:

Axial CTA chest. A large aberrant arterial supply originating from the abdominal aorta traversing the cruciate ligament of the diaphragm and supplying a sequestered right lower lobe segment. Right image demonstrate the lung window, where an intralobar sequestration appears as a wedge shaped heterogeneous mass of cystic and nodular densities.

[Table/Fig-5]:

(a) The celiac axis gives rise to the splenic artery, left gastric and common hepatic artery. An anomalous vessel (arrow) branches off superior and medial to the proper hepatic artery. (b) Selective catheterization of the aberrant artery shows a more medial branch of this vessel extending to the diaphragmatic surface and supplies the diaphragm. (c) The artery travels laterally to supply an area of abnormal vascularity in the right costophrenic sulcus (arrow), representing the area of pulmonary sequestration, which drains into a pulmonary vein. (d) The aberrant artery was embolized using 3 coils (bottom arrow), the major supply to the pulmonary vascularity was eliminated, and the phrenic branch (top arrow) was preserved.

[Table/Fig-6]:

Principal malformations of the respiratory system at different stages during embryological development