Congenital pulmonary malformations diagnosed in adulthood, how to recognize them?

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1 Congenital pulmonary malformations diagnosed in adulthood, how to recognize them? Poster No.: C-1407 Congress: ECR 2015 Type: Educational Exhibit Authors: E. De la Via, S. Isarria, M. L. Domingo, S. P. G. Alandete, M. A. Meseguer, D. Uceda, J. Vilar; Valencia/ES Keywords: Congenital, Contrast agent-intravenous, Digital radiography, CTHigh Resolution, CT-Angiography, Pulmonary vessels, Lung, Anatomy DOI: /ecr2015/C-1407 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 53

2 Learning objectives Congenital pulmonary malformations are usually diagnosed in childhood. However, sometimes the diagnosis is made in adulthood. Radiologists should recognize these types of anomalies to make a proper diagnostic approach. In this paper, congenital pulmonary anomalies are reviewed according to their relationship with the bronchial tree or with the pulmonary vasculature. Background Congenital pulmonary malformations are divided in two main groups: bronchopulmonary malformations and pulmonary vascular malformations. Bronchial anomalies: tracheal bronchus, accessory cardiac bronchus, bronchial isomerism, bronchial atresia, pulmonary aplasia / hypoplasia, bronchopulmonary sequestration, bronchogenic cysts, congenital adenomatoid malformation (CAM) and congenital lobar emphysema, are included in the first group. Pulmonary vascular malformations are classified according to the vascular abnormality (arterial, venous, or combined), and include the absence of the main pulmonary artery, pulmonary artery stenosis, pulmonary artery sling, congenital aneurysm of the pulmonary artery, congenital atresia of the pulmonary veins, single pulmonary vein, pulmonary arteriovenous malformations (PAM) and hypogenetic lung syndrome. Some of these diseases are usually diagnosed in childhood, however, others can go unnoticed and are diagnosed in adulthood. This division is arbitrary and several anomalies can coexist in the same patient. Findings and procedure details Bronchopulmonary malformations Bronchial anomalies Table 1 on page 19: 1) Tracheal bronchus. A tracheal bronchus (or a pig bronchus-because it is common in pigs) is an anatomical variant. Page 2 of 53

3 It is present in about 0,1% of the population. Clinical presentation -This is usually an incidental finding. Radiographic features - CT findings: This technique allows adequate visualization of the anomalous bronchus. In this entity, an accessory bronchus originates directly from the right tracheal wall, at or within 2 cm of the tracheal bifurcation. Fig. 1 on page 20 2) Accessory cardiac bronchus. It is a supernumerary bronchus. It may end blindly or be associated with rudimentary lung tissue. It is present in about 0,1% of the population. Clinical presentation This is usually an incidental finding. Radiographic features - CT findings: This technique allows adequate visualization of the anomalous bronchus. The anomalous bronchus arises from the inferior medial wall of the right main or intermediate bronchus. Fig. 2 on page 20 Page 3 of 53

4 3) Bronchial isomerism There is a bilateral symmetric bronchial anatomy (bilateral right of left-sided bronchial anatomy). It may be isolated or associated with congenital heart disease and/or associated changes below the diaphragm (bilaterally right sided with asplenia and bilaterally left sided with polysplenia). Clinical presentation This is usually an incidental finding. Radiographic features - CT findings: a) Left sided isomerism Fig. 3 on page 21 : - Two hyparterial bronchi and main bronchi are short and horizontal. - Two bilobed lungs - Polysplenia b) Right sided isomerism: - Two eparterial bronchi and main bronchi are long and more vertical. - Two trilobed lungs - Asplenia 4) Bronchial atresia: Bronchial atresia is a developmental defect characterised by obliteration of a lobar, segmental or subsegmental portion of the bronchus which determines the lack of continuity of the distal airways. This causes: - Bronchial obstruction # no mucous drain # distal mucocele. Page 4 of 53

5 - Hypoplasia of the distal alveoli but with hyperinflation # airflow occurs through the pores of Kohn and the channels of Lambert. The most frequently affected are the left upper bronchi Fig. 4 on page 22 followed by the right upper and right middle lobes. Clinical presentation - Bronchial atresia is asymptomatic in most patients. Pulmonary infection may occur. Radiographic features - CT findings: This is the best technique to show the typical findings of this entity Fig. 5 on page 23 : a) Bronchocele: parahilar rounded or elongated opacity, with fluid attenuation values. It may have an air-fluid level or calcifications. b) Distal lung hyperinflation with decreased attenuation around the affected bronchus. These changes are manifested most clearly by expiratory CT Fig. 6 on page 24 or low intensity reconstructions (MinIP). - MRI findings: It shows the parahiliar bronchocele. It has high signal intensity on both T1 and T2 sequences. However, it is not useful to assess regional air trapping. Differential diagnosis: - Congenital lobar emphysema - Swyer-James-MacLeod syndrome. - Regional air trapping associated with obstruction: central nodular lesions (mainly tumors). - Bronchopulmonary Aspergillosis Page 5 of 53

6 Pulmonary agenesis/ aplasia / hypoplasia Table 2 on page 25 The arrest of lung development can be classified into three types: a) Agenesis: complete absence of one or both lungs, with noevidence of bronchial, vascular or parenchymal tissue. b) Aplasia: there is a rudimentary bronchus that ends in a cul de sac,without vessels or parenchyma # These two cases appear to be secondary to a developmental malformation of the aortic arches. c) Hypoplasia: it can be primary (no etiologic factors associated) or secondary (if present with other congenital malformations that can cause it). There are intra-thoracic causes: congenital diaphragmatic hernia, mediastinal mass, decreased of pulmonary vascular perfusion (e.g. unilateral absence of the pulmonary artery) or extra-thoracic causes: skeletal dysplasia, oligohydramnios, large abdominal mass. The decrease in the volume of the ipsilateral hemithorax is the most common finding. Clinical presentation It depends on grade of pulmonary malformation and the existence of other associated malformations. Most patients with pulmonary agenesis die in the neonatal period, but it is possible the survival to adulthood. In hypoplasia survival is higher and there is a predisposition to respiratory infections but patients may be asymptomatic. Radiographic features Agenesis, aplasia and severe hypoplasia have similar findings. There is total or near total absence of aerated lung in the hemithorax. There is a marked volume reduction identified rib lifting, raising the ipsilateral hemidiaphragm and mediastinal shift toward the affected hemithorax. Page 6 of 53

7 - CT findings: - In patients with aplasia, CT may show a rudimentary bronchus and the absence of the ipsilateral pulmonary artery. - In patients with hypoplasia, CT can show the permeable bronchi, a pulmonary artery and the hypoplasic lung Fig. 7 on page 26 Differential diagnosis: - Total atelectasis - Severe bronchiectasis with collapse - Advanced fibrothorax. Bronchopulmonary sequestration Table 3 on page 27 Bronchopulmonary lung sequestration is a malformation in which there is a portion of lung separated from the rest of parenchymal and vasculature of this field comes from a systemic artery. This malformation can be intralobar or extralobar. In the first case, it is located within the same visceral pleura surrounding parenchyma. In the second case, has its own pleural covering, it is much less common. Clinical presentation: Many cases are found incidentally at chest radiography. Patients with bronchopulmonary intralobar sequestration can have recurrent pulmonary infections and haemoptysis. In patients with bronchopulmonary extralobar sequestration recurrent infections are rare since lung is enveloped in its own pleural sac. Radiographic features Page 7 of 53

8 - Intralobar sequestration: opacity is observed in the posterior basal segment of a lower lobe (usually left), less often looks like a cystic mass. Intralobar sequestrations can manifest as an area of increased opacity simulating pneumonia, as a mass with or without airfluid levels, or as cysts. The diagnosis is based on the demonstration of abnormal vascularization (the aorta or its branches), venous drainage takes place through the pulmonary venous system (left-right shunt) Fig. 8 on page Extralobar sequestration: well-defined triangular opacity or homogeneous soft tissue mass in the posterior costophrenic angle, usually adjacent to the left hemidiaphragm with an anomalous systemic arterial supply and drainage usually to a systemic venous vein. Differential diagnosis: - Bronchiectasis. - Lung abscess - Bochdalek hernia. # Anomalous systemic arteries without sequestration may exist Fig. 9 on page 29. Systemic arteries may supply normal basal segments in the lung (left-left shunt). Sometimes, the pulmonary artery supply to this area can be normal or absent. Bronchogenic cyst Bronchogenic cyst results from abnormal development of the lung bud (It is a type of bronchopulmonary foregut malformation). It is a cyst lined by respiratory epithelium containing cartilage, smooth muscle and bronchial glands. It is filled with fluid (serous, haemorrhagic or viscous with a high protein content). The cyst is not connected to the bronchial tree until the time it gets infected. From this moment, the content is air with or without fluid. Clinical presentation Bronchogenic cyst is asymptomatic in most patients. In some cases infections and haemoptysis may occur. Radiographic features Page 8 of 53

9 Approximately 75 to 85% are located in the mediastinum and 15 to 25% in the lung. - Radiographic and CT findings: Rounded opacity, circumscribed, fluid density (higher density if the contents are proteinaceous), smooth, thin-walled, usually located in themediastinum or in the lower pulmonary lobes. They may contain airinside and/or fluid Fig. 10 on page MRI findings: In both cases, MRI is superior to CT in the diagnosis and shows high signal intensity on T2-weighted sequences. High signal intensity in T1 may happen if the content is haemorrhagic or proteinaceous Fig. 11 on page 31. Differential diagnosis: - Congenital cystic adenomatoid malformation (CCAM) - Pulmonary sequestration - Pericardial cyst - Neurenteric cyst - Meningocele - Esophageal duplication cyst - Lung abscess. - Lung cancer Congenital cystic adenomatoid malformation (CCAM) Table 4 on page 33 Cystic adenomatoid malformation of the lung is an adenomatous proliferation of the terminal bronchioles with alveolar growth suppression. This entity is often classified into three types depending on pathologic analysis, radiographic appearance and prognosis. Type I has a good prognosis. Type II and III are associated with a poor prognosis (type II presents a frequent association with other foetal malformations). Clinical presentation Page 9 of 53

10 The clinical presentation is highly variable, and can manifest foetal hydrops, neonatal respiratory failure, recurrent lung infections in infancy or be an incidental finding in adulthood. Radiographic features Lower lobes are most commonly involved. - Type I consists of cysts measuring more than 2 cm and is the most common variety. They usually present as air-filed multicystic lesion Fig. 13 on page 34. The cysts may contain air, fluid, or both. - Type II has numerous smaller, more uniform cysts measuring cm in diameter. They usually present as an air-filled multicystic mass or a solid mass. - Type III contains microscopic cysts (less than5 mm) which gives a solid appearance. Prenatal ultrasound allows early diagnosis and monitoring of CCAM # Findings include isolated cystic or solid intrathoracic mass that may laterally displacing the mediastinum. Differential diagnosis: - Bronchopulmonary sequestration - Bronchogenic cyst - Congenital diaphragmatic herniation - Congenital lobar emphysema - Cystic bronchiectasis Congenital lobar emphysema It is characterized by marked overinflation of a lobe. It may be associated with extrinsic bronchial obstruction (hilar or mediastinal lymph nodes...) or intrinsic bronchial obstruction (bronchial mucous plugs ) or with congenital deficiency of bronchial cartilage. In over 50% of cases there is no apparent cause. It occurs as a result of a Page 10 of 53

11 bronchial valve mechanism with expiratory air trapping and compression of the adjacent lobes. The left upper lobe is most commonly affected, followed by the middle lobe and the right upper lobe. The involvement of the lower lobes is rare. Clinical presentation It usually manifests in the neonatal period or early infancy with respiratory distress. Radiographic features The radiographic features are increased volume, decreased attenuation and vascularity of involved lobe Fig. 14 on page 35 and Fig. 15 on page (most 36 commonly left upper lobe), and air trapping on expiratory scans. Bronchovascular markings are present in the overdistended lobe. This is a useful tip to differentiate it from pneumothorax or pulmonary cyst. If the affected lobe is markedly expanded, mediastinal shift, separation of ipsilateral ribs and hemidiaphragm depression may occur. Differential diagnosis: - Pneumothorax - Pulmonary cyst Pulmonary vascular malformations Malformations of the pulmonary arteries Table 5 on page 37 1) Absence of a main pulmonary artery Unilateral absence of a pulmonary artery is a rare malformation. This congenital abnormality is described as proximal absence of the right or left pulmonary artery. Interruption of the left pulmonary artery is much less common and may be associated Page 11 of 53

12 with congenital cardiovascular anomalies. Usually, the pulmonary circulation is through hypertrophic bronchial arteries or by abnormal artery which originates from the aorta or the innominate artery. Clinical presentation There have been two forms of presentation; isolated or associated with heart disease. When it occurs as an isolated anomaly, patients may be asymptomatic. Cases associated with congenital heart disease are diagnosed in childhood and present with hypoxia, pulmonary hypertension and/ or heart failure. Radiographic features The affected lung and the ipsilateral hilium are decreased in size. The diminute vessels are systemic and the lung is hyperlucent. However, there is no evidence of air trapping on expiration, as in the Swyer-James syndrome. Differential diagnosis: - Pulmonary hypoplasia - Congenital atresia of the pulmonary veins - Swyer-James syndrome. 2) Congenital pulmonary artery stenosis The pulmonary artery stenosis may be single or multiple, short or long, unilateral or bilateral. Generally it occurs as an isolated feature and associations are rare. Clinical presentation The diagnosis can be incidental or present with symptoms of dyspnoea and right heart failure. Page 12 of 53

13 Radiographic features Radiological findings are: poststenotic dilatation, oligohemia, signs of pulmonary hypertension and normal heart size or evidence of right ventricular hypertrophy. Differential diagnosis - Arteritis de Takayasu - Behçet syndrome 3) Congenital aneurysm of the pulmonary artery Congenital aneurysms of the pulmonary arteries are often associated with other lung (bronchopulmonary sequestration or arterivenous malformations) or cardiac malformations. Usually associated with pulmonary valve stenosis (by the hemodynamic disorder) and usually affects the left pulmonary artery. Clinical presentation Ejection systolic murmur (in pulmonary valve stenosis). Radiographic features Aneurysmal dilatation of the pulmonary artery (usually left) Fig. 16 on page 38. Differential diagnosis: - This can simulate a neoplasm. - Behçet syndrome 4) Pulmonary artery sling Page 13 of 53

14 Pulmonary artery sling is a rare congenital vascular anomaly in which the left pulmonary artery originates from the right pulmonary artery. The anomalous left pulmonary artery courses over the right main-stem bronchus and then from right to left, posterior to the trachea or carina and anterior to the oesophagus, to reach the hilum of the left lung. This causes compression of the trachea and right main-stem bronchus and it can produce obstructive emphysema, atelectasis of the right and left lungs, or both. Clinical presentation The majority of patients have symptoms (wheezing and stridor) and in children may be life threatening. Asymptomatic pulmonary artery slings are diagnosed incidentally in adulthood. Respiratory symptoms are most common and are usually characterized by wheezing and stridor with a prolonged expiratory phase. Radiographic features Imaging findings demonstrates the left pulmonary artery arising from the superior aspect of the right pulmonary artery. The left pulmonary passes over the right main-stem bronchus near its origin, and courses leftward between the trachea and oesophagus to the left pulmonary hilum Fig. 17 on page 39 and Fig. 18 on page 40. Differential diagnosis: This can simulate a neoplasm or mediastinal adenopathy. Malformations of the pulmonary veins Table 6 on page 41. 1) Congenital atresia of the pulmonary veins The congenital atresia of the pulmonary veins is a rare anomaly and probably results from a deficiency in the incorporation of the common pulmonary vein into the left atrium. It can occur in either of the two lungs, without predominance of right or left side. Clinical presentation Page 14 of 53

15 This condition is usually diagnosed in childhood and the most frequent symptoms include recurrent pulmonary infection, mild dyspnoea, or haemoptysis due to hypertrophy of bronchial arteries. Approximately 50% of patients associate other congenital heart defects or an anomalous pulmonary venous return. Radiographic features The chest radiograph shows a small lung and septal lines with a small pulmonary artery Fig. 19 on page 42. Multidetector CT is the preferred imaging technique and completes the suspected diagnosis made in the chest X ray. CT findings : CT shows a small hemithorax with a small pulmonary artery and the absence of any draining ipsilateral pulmonary vein into the left atrium, being the atrial wall completely smooth, with no evidence of even a rudimentary pulmonary vein Fig. 20 on page 43. Mediastinal abnormal soft tissue can also be observed representing the pulmonary-tosystemic venous collateral circulation. Pulmonary artery hypoplasia explains the systemic-to-pulmonary collateral arteries observed across the bronchial and intercostal arteries. Interlobular septal thickening, peribronchovascular thickening and ground-glass opacities reflectpulmonary venous hypertension and swollen lymphatic vessels. Differential diagnosis: - Pulmonary hypoplasia secondary to unilateral absence of the pulmonary artery - Sywer-James-MacLeod syndrome 2) Single pulmonary vein A single pulmonary vein with a meandering course is an anomalous unilateral single pulmonary vein that drains to the left atrium the blood from the entire lung. The anomalous vein has a normal drainage in the left atrium, therefore there is no shunt. Page 15 of 53

16 Clinical presentation This is usually an incidental finding. Radiographic features CT shows a single pulmonary vein with a meandering course draining into the atrium Fig. 20 on page 43. Differential diagnosis: - Scimitar syndrome. - Unilateral single pulmonary arteriovenous malformation - Pulmonary nodules A single vein does not require any treatment. However, the scimitar syndrome and arteriovenous malformationse may require surgery or oclusion to correct the shunt. 3) Vein varix It is a rare lesion consisting of a focal dilatation of a pulmonary veinwithout an arterial connection. It can be congenital or acquired (in association with mitral valve disease) Clinical presentation This is usually an incidental finding. In some cases haemoptysis may occur. Radiographic features Chest radiograph shows a well-defined nodular opacity. Page 16 of 53

17 CT shows one or more veins dilated (usually near the entrance of the vein into the left atrium) Fig. 21 on page 44. Differential diagnosis: - Pulmonary nodules - Pulmonary arteriovenous malformations # Enlarged diameter of a pulmonary vein without an arterial connection Malformations of the arteries and pulmonary veins 1) Pulmonary arteriovenous malformations (PAVM) or arteriovenous fistula. Pulmonary arteriovenous malformations are caused by abnormal communications between pulmonary arteries and pulmonary veins. The findings are variable between too small to see on a radiograph to complex malformations with feeding arteries and draining veins. These are most commonly congenital in nature but PAVM may also be found in a variety of acquired conditions (hepatopulmonary syndrome, trauma, mitral stenosis ). Many patients with multiple PAVM have hereditary haemorrhagic telangiectasia (RenduOsler-Weber syndrome). Clinical presentation Symptoms in early life may vary from being totally absent to severe with cyanosis, congestive heart failure, and even fulminant respiratory failure. Usually, a single PAVM < 2 cm in diameter does not cause symptoms. The incidence of symptoms is said to be greater in patients with multiple PAVM. The classic triad in hereditary haemorrhagictelangiectasia is epistaxis, multiple telangiectasias and family history (autosomal dominant disorder). Page 17 of 53

18 PAVM may give rise to complications, especially: bleeding, paradoxical embolism (Sroke), septic emboli (brain abscess), hypoxemia, and high-output cardiac failure. Radiographic features PAVM is a dilated vascular sac with draining artery and vein. It is most frequent seen as a peripheral, well-defined round or oval nodule of uniform density more commonly in the lower lobes. It can be single Fig. 22 on page 45 or multiple Fig. 23 on page 46 Fig. 24 on page 47 (usually 2 to 8 lesions) and simple (single feeding artery) Fig. 24 on page 47 or complex (two or more feeding vessels) Fig. 25 on page 48. PAVM on plain chest radiographs may be obscured by haemorrhage into contiguous parenchyma. Microvascular telangiectases may have normal chest radiographs, or only a vague increase in pulmonary vascular markings at the bases. Differential diagnosis: - Abnormal systemic vessels or other congenital or acquired vascular malformation. - Highly vascular parenchymal mass - Bronchial atresia 2) Hypogenetic lung syndrome or Scimitar syndrome Hypogenetic lung syndrome is characterized by pulmonary hypoplasia and anomalous venous drainage of the right lung. An anomalous pulmonary vein drains most of the time to the inferior vena cava below the right hemidiaphragm. The lung is small and associated with a small or absent pulmonary artery and systemic arterial supply. Clinical presentation It may be asymtomatic. Dyspnea (left-right shunt) Bronchopulmonary infections or haemoptysis Page 18 of 53

19 Radiographic features The radiograph shows a small right pulmonary hilium and a slight decrease in vascularization, deviation of the heart and mediastinum to the right and anomalous drainage vein that follows a parallel path to the right atrium (similar in appearance to a curved scimitar) Fig. 24. The findings can be confirmed by CT Fig. 26 on page 49 and Fig. 27 on page 50. Differential diagnosis: - Single pulmonary vein - Pulmonary sequestration. Images for this section: Table 1: Summary table of bronchial anomalies Page 19 of 53

20 Fig. 1: Tracheal bronchus. A. Coronal CT reconstruction (minimal intensity projection) shows the entire right upper lobe bronchus arising from the right tracheal wall above the carina. B. Axial CT shows the right upper lobe bronchus. Page 20 of 53

21 Fig. 2: Accessory cardiac bronchus. Coronal CT reconstruction (A) and axial CT (B) show the accessory cardiac bronchus arising from the inferior medial wall of the intermediate bronchus. Page 21 of 53

22 Fig. 3: A. Left side isomerism without congenital heart disease neither polysplenia. Coronal CT reconstruction (minimal intensity projection) shows bilateral symmetry of the bronchi left isomerism (right lung with the same organization as the left lung, with two hyparterial bronchi, short and horizontal main bronchi and two bilobed lungs). B. Left side isomerism with polysplenia. Coronal CT angiography: Female patient, 31 years with incidental diagnosis of left pulmonary isomerism in pulmonary CT angiography for suspected pulmonary thromboembolism. Page 22 of 53

23 Fig. 4: Chest radiograph of bronchial atresia. There is a bronchial atresia involving the left upper lobe. The upper lobe is increased in volume, radiolucent and vascularity is decreased and there is a parahiliar rounded opacity. Page 23 of 53

24 Fig. 5: Coronal and axial CT reconstructions. The upper left lobe is increased in volume, radiolucent and vascularity is decreased. A rounded mucous plug is visible distal to the site of bronchial obstruction. Page 24 of 53

25 Fig. 6 Page 25 of 53

26 Table 2: Summary table of pulmonary agenesis/ aplasia / hypoplasia Page 26 of 53

27 Fig. 7: Axial and coronal CT sections (A. Parenchymal window. B and C. Mediastinal window) show compensatory hyperinflation of the right lung, with heart and mediastinum occupying the back of the left hemithorax. Hypoplastic left lung with hypoplasia of the main pulmonary artery and left main bronchus that ends in blind pouch are seen. Page 27 of 53

28 Table 3: Summary table of bronchopulmonary sequestration. Page 28 of 53

29 Fig. 8: Axial and coronal CT sections (A. Parenchymal window. B. Mediastinal window C. MIP reconstruction) show soft tissue density image in the posterior basal segment of left lower lobe with abnormal vascularization (systemic arterial supply from a branch originating from the descending thoracic aorta (B) and venous drainage through the pulmonary venous system (C). Page 29 of 53

30 Fig. 9: Axial sections, MIP reconstruction (A. Mediastinal window B. Parenchymal window): Anomalous systemic arteries without sequestration Page 30 of 53

31 Fig. 10: Axial and coronal CT sections (A. Mediastinal window B. Parenchymal window) show subcarinal mass with fluid level corresponding to a bronchogenic cyst. Page 31 of 53

32 Fig. 11: Bronchogenic cyst with a high protein content. CT and MR (T1)images. Page 32 of 53

33 Fig. 12: Axial and coronal CT sections (A. Mediastinal window B. Parenchymal window) show subcarinal mass with fluid attenuation corresponding to a mediastinal bronchogenic cyst. Page 33 of 53

34 Table 4: Summary table of congenital cystic adenomatoid malformation Page 34 of 53

35 Fig. 13: Axial CT section (Parenchymal window): Cystic adenomatoid malformation type I.There is a large multiloculated cystic lesion (with air content) centered in the left lower lobe. Page 35 of 53

36 Fig. 14: Congenital lobar emphysema diagnosed in adulthood. Chest radiograph and CT show increased volume, decreased attenuation and vascularity of the left middle and upper lobes. There is a slight shift of the mediastinum to the left. Courtesy of Dr. Cáceres, H.G.U. Vall d'hebron, Barcelona, Spain. Page 36 of 53

37 Fig. 15 Page 37 of 53

38 Table 5: Summary table of malformations of the pulmonary arteries Page 38 of 53

39 Fig. 16: Congenital aneurysm of the left pulmonary artery associated with pulmonary valve stenosis. A) CT imaging shows the aneurysm of the left pulmonary artery b) MRI cardiac imaging shows severe valvular stenosis, calculating a rate of outflow of 410 cm / sec. Page 39 of 53

40 Fig. 17: Chest radiograph: pulmonary artery sling can simulate a neoplasm. Page 40 of 53

41 Fig. 18: CT angiography shows left pulmonary artery arising from the right pulmonary artery. The left pulmonary passes over the right main-stem bronchus near its origin, and courses leftward between the trachea and esophagus to the left pulmonary hilum. Page 41 of 53

42 Table 6: Summary table of malformations of the pulmonary veins. Page 42 of 53

43 Fig. 19: Chest radiograph. In the right lung, anomaly venous in the medial lobe is note. In left lung, loss of lung volume and hypoplasia of the main pulmonary artery are seen. Page 43 of 53

44 Fig. 20: A. CT (axial view) shows congenital atresia of the left pulmonary veins (arrowheads) B. CT (axial view) Left lung hypoplasia and a small left pulmonary artery are noted. C. CT (axial view) Smooth thickened interlobular septa are observed. D. CECT (MIP reconstruction, oblique view) shows unilateral single pulmonary vein with a meandering course. Page 44 of 53

45 Fig. 21: CT shows one vein dilated in the left lung(near the entrance of the vein into the left atrium). Page 45 of 53

46 Fig. 22: Chest radiograph. Retrocardiac tubular images suggesting pulmonary arteriovenous malformation. TC images confirm presence of arteriovenous malformation in the posterior segment of the left lower lobe with the afferent artery and draining Page 46 of 53

47 Fig. 23: Chest radiograph. Two high-density nodular lesions in right lower lobe, which is come a pulmonary vessel are identified. These findings correspond to arteriovenous malformation in a patient with Rendu-Osler-Weber syndrome. Page 47 of 53

48 Fig. 24: In CT images of this patient, the presence of arterivenosas malformations (3) is confirmed by identifying the afferent and efferent vessel. Page 48 of 53

49 Fig. 25: Axial CT section (Mediastinal view) shows a complex arteriovenous malformation in posterior segment of right upper lobe with multiple vascular connections in a patient with Rendu-Osler-Weber syndrome. Angiography images show the arteriovenous malformation (selective catheterization of the afferent artery) and the result after the proximal embolization with several hidrocoils of sizes ranging from 4-6 mm. Chest radiography after the process in which employees coils are identified. Courtesy of Dr. Martínez. Department of Radiology. La Fe University and Polytechnic hospital, Valencia, Spain. Page 49 of 53

50 Fig. 26: Fig 26 and 27. Chest radiograph, CT and volume rendering reconstruction(vr) show a small right pulmonary hilium and a slight decrease in vascularization, deviation of the heart and mediastinum to the right and anomalous drainage vein that follows a parallel path to the right atrium. Courtesy of Dr. Cáceres, H.G.U. Vall d'hebron, Barcelona, Spain. Page 50 of 53

51 Fig. 27 Page 51 of 53

52 Conclusion Congenital pulmonary malformations are sometimes diagnosed incidentally or rarely due to little symptomatology in adulthood. It is important to be aware of these entities, recognizing their imaging findings to make an appropriate diagnosis. Personal information Esperanza de la Vía Oraá Internal medical resident in Dr. Peset University Hospital, Valencia. Spain. References - Couceiro Naveira E, López Ramón y Cajal C (2002). Congenital cystic adenomatoid malformation of the lung: prenatal diagnosis. Progresos de Obstetricia y Ginecología 45(6): DillmanJR, YarramSG, Hernandez R (2009). Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol 192(5): Gossage James R, Kanj G (1998). Pulmonary arteriovenous malformations. American Journal of Respiratory and Critical Care Medicine 158(2): Heyneman LE, Nolan RL, Harrison JK, McAdams HP (2001). Congenital unilateral pulmonary vein atresia: radiologic findings in three adult patients. AJR Am J Roentgenol 177(3): Himesh VV. Greenberg SB, Krishnamurthy R (2012). MR imaging and CT evaluation of congenital pulmonary vein abnormalities in neonates and infants. Radiographics 32(1): Mata JM, Cáceres J, Lucaya J, García-Conesa JA (1990). CT of congenital malformations of the lung. Radiographics. 10 (4): Page 52 of 53

53 - Porres DV, Morenza OP, Pallisa E, Roque A, Andreu J, Martínez M (2013). Learning from the pulmonary veins. Radiographics 33(4): Vanherreweghe E, Rigauts H, Bogaerts Y, Meeus L (2000). Pulmonary vein varix: diagnosis with multi-slice helical CT. European 10 (8): Fraser R. Colman N. Müller N. Paré P.D. Fundamentos de las enfermedades del tórax, 3º ed. Barcelona, Masson, 2006; p Webb W, Higgins C. Thoracic imaging: pulmonary and cardiovascular radiology. 1ª ed.philadelphia, Lippincott Williams & Wilkins; p Cura JL, Pedraza S, Gayete A. Radiología esencial. 1ª ed. Madrid, Panamericana, p Rodrigues, M. A., Ritchie, G., & Murchison, J. T. (2013). Incidental meandering right pulmonary vein, literature review and proposed nomenclature revision.world Journal of Radiology, 5(5), doi: /wjr.v5.i Vanherreweghe E, Rigauts H, Bogaerts Y, Meeus L (2000). Pulmonary vein varix: diagnosis with milti-slice helical CT. European 10 (8): Mata JM, Cáceres J, Lucaya J, García-Comensa JA (1990). CT of congenital malformations of the lung. Radiographics 10 (4): Page 53 of 53