Clinical, radiological, differential diagnoses of adult Congenital Pulmonary Disease

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1 Clinical, radiological, differential diagnoses of adult Congenital Pulmonary Disease Award: Certificate of Merit Poster No.: C-1163 Congress: ECR 2015 Type: Educational Exhibit Authors: D. L. Acevedo Soto, Y. M. Lopez, I. Quintana Rodríguez, C. Pascual Montero, S. L. Moyano Calvente, D. U. De la Calle Pato, D. J. C. Alcón Tejero, L. Martin; Cáceres/ES Keywords: Lung, Mediastinum, CT, Education, Congenital DOI: /ecr2015/C-1163 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 34

2 Learning objectives Review the clinical manifestations, radiological findings and differential diagnosis of various congenital anomalies adult lung. Page 2 of 34

3 Background Embryology Lung The respiratory system development begins with the evagination of cells from the foregut endoderm into the splanchnic mesenchyme at 3 weeks of gestation. The respiratory mucosa derives from the endoderm of the ventral foregut, while the supporting tissue and the vasculature have a mesodermal origin. Following the anatomic changes that occur in its architecture lung morphogenesis can be divided into five stages: embryonic, pseudoglandular, canalicular, saccular, and alveolar: 1 Embryonic period (3-7 weeks). At 26 embryonic days in the ventral wall of the foregut a laryngotraheal grove appear. Grooves lips fuse at the caudal end and form the tracheal diverticulum. From this diverticulum the two main bronchi buds bulge out. The main bronchi divide into two lobar bronchi on the left and three on the right side, defining the lobar anatomy of the human lung. All lobar airways can be detected by the sixth gestational week. Development of the pulmonary vasculature uses the primordial airways as a template. The human lungs are supplied by two vascular systems, which develop sequentially. First, the pulmonary circulatory system is established after five gestation weeks, and then the bronchial arteries arise from the aorta after a delay of approximately 3 weeks. 2 Pseudoglandular period (7-17 weeks). The conducting airways continue to branch and bud. The airway conducts expand in the periphery producing a glandular appearance. During this period mucous glands, bronchial cartilage, and epithelial airway cells develop. By the 16-th gestation week the tree of conducting airways has fully developed. 3 Canalicular period (16-26 weeks). Tubules expand to form saccules widening the airway lumina. The mesenchyme thins and the airspaces come into increasingly close apposition to capillary network to form the gas exchange region. During this period the type I cells differentiate and surfactant components start to be produced by the cuboidal type II cells. After gestation weeks despite the pulmonary immaturity preterm babies could survive with intensive care support. 4 Saccular period (22-36 weeks). During this stage the lung tissue maturate in preparation for birth. The acinar tubules continue to proliferate and expand. The surface area of Page 3 of 34

4 the gas exchange region increases and additional alveoli septae form. At 30 week of gestation surfactant is detectable in the amniotic fluid. 5 Alveolar period (36 weeks to maturity). The alveolar septation is completed in this period. Alveolar structures can be recognize histological after 32 weeks and are uniformly present at 36 gestation weeks. Most of the alveoli are formed after birth in the first two years of life. Each of this stages associate specific disorders in correspondence with the developmental processes. Pulmonary Arteries The pulmonary artery develops from the sixth aortic arch. The proximal part of the arch becomes the proximal segment of the right and left pulmonary arteries. On the right side, the distal part loses its connection with the arch, whereas on the left it maintains its connection as the ductus arteriosus. During the embryonic and pseudoglandular periods, arterial development parallels that of the airways. As the fetus matures, the vessels increase in diameter and length, and in the postnatal stage a marked increase in branching occurs, which corresponds to the proliferating alveolar development of early childhood until approximately 8 years of age. Pulmonary Veins In the embryonic stage, pulmonary venous blood drains via the splanchnic plexus into the primordium of the systemic venous system, including the cardinal and umbilicovitelline veins. Caudal and cranial outpouchings of the sinoatrial regions of the heart develop and extend toward the lung buds. With regression of the caudal portion, the cranial portion continues to develop as the common pulmonary vein, eventually connecting with that portion of the splanchnic plexus draining the lungs. In time, the common pulmonary vein is incorporated into the left atrial wall with obliteration of the majority of the splanchnic pulmonary connections, leaving four independent pulmonary veins directly entering the left atrium. Pulmonary arterial anomalies. Pulmonary agenesis, aplasia, and hypoplasia. Classification of congenital lung underdevelopment can be divided into 3 categories: (1) total absence of the lung, bronchus, and pulmonary artery); (2) presence of rudimentary Page 4 of 34

5 bronchus with absence of the pulmonary artery and lung, representing lung aplasia; (3) hypoplasia of the bronchus and pulmonary artery with a variable amount of distal lung tissue, termed lung hypoplasia. Pulmonary agenesis is a rare congenital lung abnormality comprising absence of the lung parenchyma, bronchus, and its pulmonary artery and Pulmonary aplasia is similarly characterized by a congenital absence of lung parenchyma and its associated pulmonary vasculature. However, there remains a rudimentary bronchus. At present, the causes of both pulmonary agenesis and aplasia are unknown. Pulmonary venous anomalies. Partial anomalous pulmonary venous return (PAPVR). Partial anomalous pulmonary venous return (PAPVR) occurs when a portion of pulmonary veins retain their connection to the primitive splanchnic system of cardinal veins rather than developing connections to the central pulmonary venous system. These anomalous veins may drain directly into the right atrium, the vena cavae, coronary sinus, azygous vein, and, if persistent, the left vertical vein. This anomalous drainage results in a left-to-right shunt. A sinus venosus atrial septal defect is present in up to 90% of cases. If a sinus venosus defect is present, the resulting left-to-right shunt may be hemodynamically significant, giving symptoms of increased pulmonary flow and pulmonary hypertension. Parenchymal Anomalies. Congenital bronchial atresia. Also referred to as a bronchial mucocele, bronchial atresia derives from focal obstruction of a segmental or subsegmental bronchus with normal distal airway development. At present, the cause for this obstruction is unclear but 2 theories of pathogenesis have been presented. One theory postulates that bronchial atresia results from an obliterated connection between the tip of the bronchial bud and primitive bronchial cells with normal growth distal to the focal defect. A second theory proposes that bronchial atresia is the result of a vascular insult whereby there is localized bronchial lumen disconnection resulting from interruption of bronchial arterial supply and resultant ischemia. Affected patients may present signs of respiratory distress or recurrent infections, which is largely predicated on associated parenchymal disease severity. However, if little or no parenchymal disorder is present, these lesions may be asymptomatic. Page 5 of 34

6 Congenital pulmonary airway Malformation (CPAM). Congenital pulmonary airway malformation is said to account for 25% of all congenital lung abnormalities. Is a rare pulmonary malformation with an estimated incidence between 1/25000 and 1/ It is produced by the abnormal and extensive overgrowth of the bronchiolar structures. The cause for appearance is unknown but the error occurs sometimes between the fifth to the sixth week of gestation in the pseudoglandular stage of lung development. Three types have been classified by pathologic analysis. Type I consists of cysts measuring 2-10 cm and is the most common variety. Type II has numerous smaller, more uniform cysts measuring cm in diameter, and type III are solid-appearing lesions that microscopically demonstrate tiny cysts. The disorder is usually diagnosed in the neonatal period and the first 2 years of life. It is rarely encountered in the adult. In the adult, the lower lobes are usually involved, with expansion of the involved hemithorax and compensatory shift of the mediastinum. The cysts may be single or multiple, containing air, fluid, or both. Bronchogenic cysts (BC). It is a rare congenital lung malformation resulting from an abnormal growth of the aerial conducts. The lesion occurs sometime between the third and the sixth gestation week. It is usually unilocular, has no communication with the bronchial tree and filled with clear mucous secretion. It is almost always closely attached to major airways or the esophagus by dense fibrous tissue. In 65% it is located in the mediastinum. Intrapulmonary cysts are in most of the cases located in the lower lobes. Less frequent locations are pericardium, pleural cavity, cervical, paravertebral and occasional in extra-thoracic positions. The clinical course of the cyst is strongly influenced by the presence or absence of communication with the parent bronchia. Non-communicating cysts cause symptoms by local compression but in most of the cases are asymptomatic. On the other hand a cystbronchial communication can cause two complications: tension cyst and infection. Page 6 of 34

7 Congenital lobar emphysema (CLE). Congenital lobar emphysema is a rare condition characterized by the overinflation and distension of one or more pulmonary lobes. The basic defect is the inability of the affected lobe to deflate normally. The over expanded portion compresses the rest of the lung affecting the normal respiration. By mediastinal shift can also compress the contralateral lung. The cause of the disease is represented by an intrinsic or extrinsic bronchial narrowing. Intrinsic narrowing can be produced by the weakness or absence of bronchial cartilage so that there is air entry but collapse of the narrow bronchial lumen during expiration. A large pulmonary artery, a prehilar bronchogenic cyst, an enlarged mediastinal node, an aneurismal ductus arteriosus could compress the bronchial tree and affect the cartilage rings. The upper left lobe is the most frequent affected (42%) followed by right middle (35%) and right upper lobe (21%). Multiple lobar involvements are possible but usually there is only one lobe affected. Male to female ratio is about 3 to 1. Symptoms are the result of the compression of the normal lung tissue and vary according to the size of the affected lobe and the degrees of compression. Combined pulmonary arterial and venous anomaly. Pulmonary arteriovenous malformations (AVMs). Pulmonary arteriovenous malformations (AVMs) result from a focal defective development of the pulmonary capillary network. This defect causes a direct communication between a pulmonary arterial branch and its adjacent pulmonary vein. Pulmonary AVMs are also known as pulmonary arteriovenous fistulae, a more accurate term given the underlying defect.most pulmonary AVMs are congenital, although a small subset may be acquired in patients with prior cardiac surgery for congenital heart disease as well as patients with a history of atypical lung infection (eg, tuberculosis) and patients with chronic liver disease and Congenital pulmonary AVMs are characteristically associated with the autosomal dominant disorder Osler-Weber-Rendu, also known as hereditary hemorrhagic telangiectasis (HHT).The presence or absence of symptoms as well as the decision for intervention is largely predicated on the amount of shunting. Large or complex lesions are generally associated with substantial shunts and are more likely to evoke patient symptoms. Treatment is generally offered when feeding arteries measure greater than 3 mm in transverse dimension. Often endovascular coil embolization or Page 7 of 34

8 balloon occlusion is the mainstay of therapy. However, surgical excision may also be used. Combination of Vascular and Parenchymal Anomalies. Pulmonary sequestration (PS). Pulmonary sequestration is a rare congenital lung malformation characterized by a mass of nonfunctioning lung tissue with no connection with the normal tracheobronchial tree ( ). The lesion has distinguished blood supply originating from the systemic artery system. It represents % of all pulmonary malformation. The origin of the lesion is uncertain but the most widely accepted hypothesis is that it results from an accessory lung bud developing inferior to the normal lung bud. There are 2 distinctive forms of pulmonary sequestration: intralobar and extralobar. Intralobar sequestration is rarely symptomatic and when present symptoms are nonspecific chest pain, pleuritic pain, shortness of breath, and wheezing, recurrent infection. The recurrent localization of the pathology in the lower lobe suggests the diagnostic. Extralobar sequestration usually it is asymptomatic and discovered incidentally on a routine X-ray. Extralobar sequestration may cause severe respiratory distress, congestive heart failure, mitral regurgitation. In some particular uncommon cases the sequestration can produce even franc hemoptysis. Differential diagnostic refer to other congenital lung malformations (CCAM type 1 and 3, bronchogenic cyst, diaphragmatic hernia), lung infections (pneumonia, tuberculosis), malignant or nonmalignant mediastinal masses. Hypogenetic lung syndrome (scimitar síndrome). Many interesting terms are used to describe the Scimitar Syndrome (SS) as hypogenetic lung syndrome or pulmonary venolobar syndrome. Its main elements are: 1. Hypoplasia/ lobar aplasia/agenesis involving the right lung. Abnormal lobation including bronchial isomerism with hyparterial bronchus and unilobar lung may be present. Lobar agenesia Page 8 of 34

9 (absence of parenchyma with absent corresponding bronchus) was found in our patient. Errors of segmentation may occur. 2. An anomalous right pulmonary vein connection to the systemic venous circulation either below or above the diaphragm most commonly to the inferior vena cava, occasionally into the hepatic vein, portal vein, azygos vein, coronary sinus or the right atrium. The vein may drain the whole lung or part of it. 3. Partial or complete anomalous systemic arterial supply from the aorta or one of its branches to the right lung with absent or hypoplastic right pulmonary artery. Accessory diaphragm, horseshoe lung and absent inferior vena cava should also be looked for. The scimitar syndrome is more common in women (1.4: 1). Familial occurrences have been reported. The incidence is estimated to be one to three per births. Manifestations of clinical symptoms in SS is dependent on the size of the resultant left to right shunt magnitude and ranges from asymptomatic to heart failure. Delineation of vascular supply is helpful to prevent torrential bleeding at surgery. Aortography is the more traditional investigation to document arterial supply. Differential diagnosis of systemic arterial supply to the lung includes SS, pulmonary sequestration, pulmonary arteriovenous malformation, interruption of pulmonary artery, rarely cystic adenomatoid malformation and sole or accessory arterial supply to the normal lung. Page 9 of 34

10 Images for this section: Fig. 2: Embriology - From: Jeffrey A. Whitsett, Susan E. Wert and Bruce C. Trapnell. Genetic disorders influencing lung formation and function at birth. Human Molecular Genetics, 2004, Vol. 13, Review Issue 2 R207-R215. Human Molecular Genetics, 2004, Vol. 13, Review Issue 2 R207-R215. Page 10 of 34

11 Findings and procedure details Pulmonary arterial anomalies. Pulmonary agenesis, aplasia, and hypoplasia. Radiographic findings in lung aplasia and agenesis are similar. The affected hemithorax is typically small and increased in opacity with evidence of ipsilateral volume loss including mediastinal shift to the ipsilateral side and elevation of the hemidiaphragm. There is often compensatory overinflation and herniation of the contralateral lung, which can be appreciated as focal lucency extending across the mediastinum on the frontal radiograph and increased lucency within the retrosternal clear space on the lateral radiograph. Cross-sectional imaging, particularly CT, is helpful in delineating pulmonary agenesis from pulmonary aplasia by showing the presence or absence of a rudimentary bronchu. Furthermore, CT shows the absence of the affected lung and its pulmonary artery. MR imaging is less used in pulmonary agenesis and aplasia compared with CT given its lack of lung parenchymal definition. However, the absence of a pulmonary artery and, to some extent, the presence or absence of a rudimentary bronchus may be seen. In contrast with pulmonary agenesis and pulmonary aplasia, the pulmonary artery and bronchus are both present but hypoplastic in pulmonary hypoplasia. There is a variable degree of associated lung parenchyma. Pulmonary hypoplasia can have a variety of chest radiographic findings depending on severity, with severely hypoplastic lungs simulating lung aplasia and milder forms showing only subtle mediastinal shift, increased opacity of the affected lung, and elevation of the ipsilateral hemidiaphragm CT is currently the imaging modality of choice because it can show residual/hypoplastic lung parenchymal tissue with more detail than other cross-sectional modalities, such as US and MR. Depending on severity, the residual lung parenchyma may be near normal in volume or markedly hypoplastic. However, unlike both lung aplasia and agenesis, a small pulmonary artery can be shown on CT, particularly with the aid of high-quality 3D reconstructions and MPR. Furthermore, the presence of an intact, although hypoplastic, bronchus can readily be shown. Pulmonary venous anomalies. Page 11 of 34

12 Partial anomalous pulmonary venous return (PAPVR). Imaging of PAPVR depends on the exact pulmonary vein/veins that are anomalous, where these anomalous vessels drain, and whether the associated shunt is hemodynamically significant. Chest radiography is limited in the evaluation of PAPVR. If a substantial shunt is present, chest radiography may show signs of pulmonary overcirculation with pulmonary arterial enlargement and right heart enlargement. CT and MR depict in excellent detail the course and drainage of the anomalous pulmonary veins. However, MR is the primary imaging modality of choice given its ability to quantitate shunting by determining the pulmonary flow (Qp)/systemic flow (Qs) ratio. A Qp/Qs ratio of greater than 1.5 is considered hemodynamically significant and is tabulated on cine phase contrast images. The most common PAPVR involves the left upper lobe whereby a vertically oriented anomalous pulmonary vein courses lateral to the aortic arch and drains into the left innominate vein and this type of PAPVR is also the most common PAPVR to be unassociated with congenital heart disease. In addition to the anomalous pulmonary venous drainage, CT and MR are able to show an associated sinus venosus defect, which often occurs with PAPVR of the superior right pulmonary vein. A sinus venosus defect appears as a focal defect in the posterior-superior aspect of the atrial septum. On MR, a jet flow void may be observed to pass across this defect. On CT, intermixing of contrasted and uncontrasted blood or a focal defect in the superior-posterior and, less commonly, the posteroinferior wall may be shown. Parenchymal Anomalies. Congenital bronchial atresia. On chest radiography, impacted mucus distal to the atretic segment of bronchus appears as a round or oval opacity in the apical or apicoposterior segments of the upper lobes. Distal air trapping may appear as focal hyperlucency of the lung distal to the area of mucus plugging. If there is an associated congenital pulmonary mass lesion, the focal mucus impaction and hyperinflation may be obscured. In these cases, the radiographic findings consist of those of the mass lesion rather than the associated bronchial atresia. Page 12 of 34

13 CT accurately shows the findings of bronchial atresia and helps confirm the diagnosis if suspected on chest radiographs. The mucus-filled bronchus distal to the area of atresia appears as a dilated, tubular, soft tissue opacity involving the apical or apicoposterior segments of the upper lobes. Distal to the mucus-filled bronchus, the lung is hyperinflated, which is postulated to result from collateral air drift through the interstitium and pores of Kohn with the collateralized air subsequently becoming entrapped within the affected segment. This hyperinflation appears on CT as a segmental area of hypoattenuated lung parenchyma distal to the mucus-filled bronchus. Congenital Cystic Adenomatoid Malformation. Diagnostic is made on X-ray showing multiple air filled spaces (macrocystic) or a solid area (microcystic). Computed tomography (CT) is the most utile technique for diagnostic and pre-therapeutic evaluation. It show large air filled, fluid filled cysts or containing air fluid levels (type 1); solid mass with multiple small cysts (type3). Other diagnostic means include magnetic resonance (MRI) and bronchoscopy. One of the most recent developments in the area is virtual bronchoscopy (VB). VB is a non-invasive threedimensional (3D) technique that uses multidetector CT generated image. It can evaluate the airways down to the sixth- to seventh-order bronchial subdivisions. Bronchogenic cysts. Diagnostic is made on plain chest X-ray or with more accuracy using thoracic computed tomography. Intrapulmonary bronchogenic cyst appears as a solitary, sharply defined round mass with water density. An air-fluid image indicates a communication between the cyst and the bronchial tree. Congenital lobar emphysema. The diagnostic is made on X-ray of the chest showing a hyperlucent overexpanded area with attenuated but defined vascularity and compression of the remaining lung on that side. It can show also widening of the ribs spaces, depression of the diaphragm, mediastinal shift and compression of the contralateral lung. A lateral Xray will show anterior herniation of the expended lobe and the posterior displacement of the heart. Computed tomography scanning can provide more accurate information of the overdistended lobe and its vascularity, as well as information about the remaining lung. It shows a hyperlucent, overexpanded lobe with compression of the remaining lung and mediastinal shift. Other diagnostic tools are: pulmonary scintigraphy, MRI and ultrasound. Page 13 of 34

14 Combined pulmonary arterial and venous anomaly. Pulmonary arteriovenous malformations (AVMs). On chest radiography, pulmonary AVMs appear as well-circumscribed nodular or serpiginous soft tissue density occurring in the lower lobes in 50% to 70% of cases. Despite this lower lobe predominance, pulmonary AVMs may be seen in any lobe. Single or multiple lesions may be seen. In some instances, a curvilinear opacity directed toward the mediastinum may appear to arise from the lesion. This opacity represents the associated feeding artery, the enlarged draining vein, or both. Although these are intraparenchymal lesions, on frontal and lateral radiographs they may project over the mediastinum, making exact localization difficult.ct is the imaging modality of choice for the location, number, and size of the lesion and its associated arterial supply and venous drainage. On noncontrast CT, pulmonary AVMs appear as focal lobulated or serpiginous, soft tissue density, intraparenchymal masses. With the administration of intravenous contrast, pulmonary AVMs markedly enhance. The number, course, and size of the feeding arteries and draining veins are well shown and important for treatment planning. Two-dimensional MPR and 3D reconstructions are especially useful in evaluating the angioarchitecture even in cases of complex AVMs. Combination of Vascular and Parenchymal Anomalies. Pulmonary sequestration. Diagnostic is made on plain X-ray: ill-defined consolidation inside the lung or a soft tissue mass with wellor ill-defined borders. A cystic area or air-fluid level can also be seen. There are certain difficulties in diagnosing from chest plain X-ray extralobar sequestration. The image could be obscured by cardiac or vertebral shadow. Angiography is a really useful mean for diagnostic and pretherapeutic evaluation. A higher value has CT with contrast medium when the abnormal blood supply can be identified. CT can also give useful information about regional anatomy, the relations between the sequestration and the surrounding structures, about other concomitant anomalies. Bronchoscopy and bronchography are not useful. The key of pulmonary sequestration diagnostic is to identify the aberrant systemic blood supply. Page 14 of 34

15 Hypogenetic lung syndrome (scimitar síndrome). On plain radiograph varying degree of right lung hypoplasia and dextroposed cardiac silhouette are seen. The scimitar sign consists of a broad curved vertically oriented band along the right heart border and disappearing at the right hemidiaphragm due to the abnormal vein. It may be straight, thin or multiple. This telltale sign may or may not be present on chest radiograph. The absence of the scimitar sign is attributable to the very small lung, marked mediastinal shift and unfavorable orientation or small caliber of the vein. Absence of the scimitar sign may be mistaken for dextrocardia or Swyer-James syndrome. Occasionally a wandering right pulmonary vein may create confusion before entering normally into the left atrium. CT establishes degree of lung underdevelopment in patients with SS and differentiates it from atelectasis, brochopulmonary sequestration and true dextrocardia. Contrast enhanced CT can of the thorax may demonstrate feeder vessels and large draining vein. Vascular engorgement as a result of perfusion of lower resistance lung circulation at systemic pressure can be reflected as increased CT attenuation. Multisectional helical CT scan generate angiogram-like images to show pulmonary artery hypoplasia / absence and bronchographic images to visualized airway abnormalities. Page 15 of 34

16 Images for this section: Fig. 3: Pulmonary aplasia. (a) Frontal chest radiograph depicts the trachea (white arrow) and the left main bronchus ( red arrow); however, the right main bronchus is not seen. There is rightward mediastinal shift. Compensatory hyperinflation of the left lung extending into the left hemithorax is also noted. (b) Coronal CT scan with absence of the right lung parenchyma. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 16 of 34

17 Fig. 4: Pulmonary aplasia. (same case as in fig. 3). (a) Axial with lung window CT. There is rightward mediastinal shift. Compensatory hyperinflation of the left lung extending into the left hemithorax (black arrow) is also noted. (b) Coronal lung window CT scan shows absence of the right lung parenchyma. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 17 of 34

18 Fig. 5: Anomalous pulmonary venous return. (a-d) Axial CT chest revealed an anomalous pulmonary venous return with a pulmonary vein draining to the brachiocephalic vein. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Fig. 6: Congenital bronchial atresia. (a-d) Axial lung window CT demonstrate hyperinflation and relative oligemia of the right lower lobe, mild bronchial wall thickening and mild bronchiectasis with associated air trapping and subtle cystic formation. Background of mild paraseptal emphysema. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 18 of 34

19 Fig. 7: Congenital bronchial atresia. (same case as in fig. 6). (a-b) Coronal lung window CT show the right lower lobe lateral basal segmental bronchus abruptly. Scattered foci of mucoid impaction within the distally reconstituted segmental and subsegmental bronchi. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 19 of 34

20 Fig. 8: Congenital pulmonary airway malformation. (a) Frontal chest radiograph showing multiple air filled spaces cystic (white arrow) in right upper lobe and an area macrocystic (red arrow) in right lower lobe. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES Fig. 9: Congenital pulmonary airway malformation. (same case as in fig. 8). (a-b) Axial and Coronal CT Image cyst (white arrow) measuring more than 4 cm with hypodense contained therein is located in lower lobe. Mediastinal shift to the right side (red arrow) is also seen. Service of Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 20 of 34

21 Fig. 10: Congenital pulmonary airway malformation. (same case as in figs. 8-9). (a-b) Axial and Coronal lung window CT images demonstrate multiple cystic lesions (white arrow) in the left upper lobe, with a dominant cyst (red arrow) measuring more than 4 cm with hypodense contained therein is located in lower lobe. Mediastinal shift to the right side is also seen. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 21 of 34

22 Fig. 11: Bronchogenic cyst. (a-b) Frontal and lateral chest radiograph shows a soft-tissue mass (white arrow) in the right hemithorax. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Fig. 12: Bronchogenic cyst. (same case as in fig. 11). (a-b) Axial and coronal CT scan shows a well-circumscribed unilocular water-attenuation cyst (white arrow) in the middle mediastinum. The cyst has smooth, imperceptible walls with no enhancement and was pathologically confirmed to be a bronchogenic cyst. Radiology, San Pedro de Alcánta Hoapital, Cáceres/ES. Page 22 of 34

23 Fig. 13: Congenital lobar emphysema (a-b) Frontal and lateral chest radiograph shows hyperinflation (white arrow) of the middle lobe right. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 23 of 34

24 Fig. 14: Congenital lobar emphysema. (same case as in fig. 13). (a-b) Coronal lung window CT scan shows organized pulmonary vessels coursing through an overdistended middle lobe of right lung (white arrow). Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Fig. 15: Congenital lobar emphysema. (same case as in figs ). (a) Axial lung window CT images show hyperinflation of the middle lobe (white arrow). Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 24 of 34

25 Fig. 16: Pulmonary arterioveous malformations. (a-b) Frontal and lateral chest radiography shows a well-circumscribed nodular soft tissue density (white arrow) in the left lower lobe. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 25 of 34

26 Fig. 17: Pulmonary arterioveous malformations. (same case as in fig. 16). (a) Axial TC shows two hypervascular masses paracardiac location in the lower left lobe, closely related to one another. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Fig. 18: Pulmonary arterioveous malformations. (same case as in figs ). (ab) Coronal TC maximum-intensity projection shows two hypervascular masses (white arrow) paracardiac location in the lower left lobe, dependent mass vascularization of a branch of the inferior lobar artery, with separate drainage of both lesions through venous branches that converge towards the lower venous confluent. increased caliber of the left main pulmonary artery (red arrow). Radiology, San Pedro de Alcánta Hospital, Cáceres/ ES. Page 26 of 34

27 Fig. 19: Pulmonary arterioveous malformations. (same case as in figs ). (a-b) Lung arteriography shows dependent mass vascularization of a branch of the inferior lobar artery, with separate drainage of both lesions through venous branches that converge towards the lower venous confluent. Radiology, San Pedro de Alcánta Hospital, Cáceres/ ES. Page 27 of 34

28 Fig. 20: Pulmonary arterioveous malformations. (same case as in Figs ). (a-b) 3D reconstruction shows shows dependent mass vascularization of a branch of the inferior lobar artery. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Fig. 21: Intralobar sequestration. (a-b) Frontal and lateral chest radiograph shows a cystic area with air-fluid level in left lower lobe can also be seen. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 28 of 34

29 Fig. 22: Intralobar sequestration. (same case as in fig. 21). (a-b) Axial lung window TC shows a cystic area with air-fluid level in left lower lobe. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 29 of 34

30 Fig. 23: Intralobar sequestration. (same case as in figs ). (a-b) Coronal lung window TC shows a cystic area with air-fluid level in left lower lobe. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Fig. 24: Intralobar sequestration. (same case as in figs ). (a-b) Coronal maximumintensity projection and 3D reconstruction TC images show the feeding artery (red arrow) of the mass arising from the descending aorta and venous drainage (white arrow) into the left inferior pulmonary vein. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 30 of 34

31 Fig. 25: Partial hypogenetic lung syndrome (scimitar syndrome). (a) Frontal chest radiograph shows a vertically oriented curvilinear opacity projecting over (white arrow) the right lower hemithorax. Also noted is hypoplastic right lung. Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 31 of 34

32 Fig. 26: Partial hypogenetic lung syndrome (scimitar syndrome). (same case as in fig. 25). (a-c) Axial, coronal and sagittal reconstruction contrast-enhanced CT images show the anomalous vein draining into the inferior vena cava (red arrow). Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Fig. 27: Partial hypogenetic lung syndrome (scimitar syndrome). (same case as in figs ). (a-b) 3D reconstruction CT images show the anomalous vein draining into the inferior vena cava (white arrow). Radiology, San Pedro de Alcánta Hospital, Cáceres/ES. Page 32 of 34

33 Conclusion It is important to know the origin, pathology and radiological manifestations of congenital lung malformations different adult because diagnosis is difficult and identification allows proper management of these patients. Page 33 of 34

34 References 1. Thacker P, Rao A, Hill J, Lee E. Congenital lung anomalies in children and adults current concepts and imaging findings. Radiol Clin N Am 2014, 52: Lee E, Dorkin H, Vargas S. Congenital pulmonary malformations in pediatric patients: review and update on etiology, classification and imaging findings. Radiol Clin N Am 2011, 49: Biyyam R, Chapman T, Ferguson M, et al. Congenital lung abnormalities: embryologic features, prenatal diagnosis and postnatal radiologic-pathologic correlation. Radiographics 2010, 30: Lee E, Boiselle P, Cleveland R. Multidetector CT evaluation of congenital lung anomalies. Radiology 2008, 247: Berrocal T, Madrid C, Novo S et al. Congenital anomalies of the tracheobronchial tree, lung and mediastinum: embryology, radiology and pathology. Radiographics 2004, 30: Epelman M, Kreiger P, Servaes S, et al. Current imaging of prenatally diagnosed congenital lung lesions. Semin Ultrasound CT MRI 2010, 31: Page 34 of 34