Congenital lung malformations: radiological findings and clues for differential diagnosis

Abstract

Congenital lung malformations encompass a wide spectrum of conditions with a broadly varying clinical presentation. They are often a source of morbidity in infants and children. Their management depends on the type of malformation and its clinical presentation. Usually, the diagnosis requires an imaging evaluation. Classifications of bronchopulmonary malformations have undergone significant revision in recent years and several theories have attempted to explain their confusing pathogenesis. There are considerable degrees of overlapping and hybrid conditions are common, with interrelated malformations showing various radiologic and pathologic features. Attending to the pathophysiological mechanisms and structures involved, lung malformations can be divided into three categories: bronchopulmonary anomalies, combined lung and vascular abnormalities, and vascular anomalies. The purpose of this article is to review the current imaging techniques for evaluating lung malformations in pediatric patients and their characteristic imaging findings. Moreover, this review discusses a useful classification and offers some clues to facilitate the differential diagnosis.

Pediatric thoracic mass lesions: Beyond the common

“…The most common pediatric thoracic mass lesions include lymphoma, germ cell tumors, neurogenic tumors, foregut cysts, congenital pulmonary airway malformations, vascular malformations, hemangiomas, chest wall sarcomas, pleuropulmonary blastoma, inflammatory myofibroblastic tumor, endobronchial tumors, breast tumors and cardiac tumors. These have been the subject of numerous prior publications [3,[5][6][7][8][9][10][11][12][13][14][15][16][17][18] and are beyond the scope of this review.…”

Congenital Lung Malformations: A Pictorial Review of Imaging Findings and a Practical Guide for Diagnosis

“…Furthermore, the term “hypoplasia” is related to the presence of a bronchus and rudimentary lung; also, the airways, alveoli, and pulmonary vessels are reduced in size and number [ 3 , 37 ]. This condition is typically secondary to a process (intra- or extra-thoracic), which is most commonly a congenital diaphragmatic hernia.…”

“…Other intrathoracic causes of pulmonary hypoplasia include CPAM, bronchopulmonary sequestration, a cardiac or mediastinal mass, lymphatic malformation, and the agenesis of the diaphragm [ 3 , 37 ]. The leading extrathoracic cause is often severe oligohydramnios, typically resulting from genitourinary anomalies—which include renal agenesis or severe renal dysfunction—or resulting from the prolonged preterm rupture of membranes [ 3 , 37 ]. Chest radiographs may reveal a small lung and retrosternal hyperclarity in cases of pulmonary hypoplasia, attributed to the herniation of the contralateral lung [ 39 ].…”

“…Most CPAMs are detected during prenatal US in asymptomatic patients, In the majority of cases, the blood supply originates from the pulmonary artery, with venous drainage directed toward the pulmonary veins [3,8,32]. However, hybrid lesions, which are defined as a combination of congenital pulmonary airway malformation (CPAM) and pulmonary sequestration, may exhibit a systemic blood supply [37]. These histological findings imply the hypothesis that CPAMs could result from intrauterine airway obstruction [8].…”

“…CT angiography with 3D reconstruction serves as a highly informative tool for identifying anomalous feeding arteries and drainage veins for differentiating between intraand extra-lobar sequestration (Figures 14 and 15). The CT appearance of the lesion ranges from a homogeneous soft tissue mass within the parenchyma (Figure 16) to a cystic lesion containing air or fluid, which can coexist in hybrid forms [15,37]. CT angiography with 3D reconstruction serves as a highly informative tool for identifying anomalous feeding arteries and drainage veins for differentiating between intra-and extra-lobar sequestration (Figures 14 and 15).…”