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A boy of 7 years the last 2 years was repeatedly treated for right inferior lobe bronchopneumonia. On the plain chest X-ray, there is a well defined opacity at the level of the inferior lobe of the right lung. The shadow adjoins to the mediastinum.
Suspected diagnosis???
Pulmonary hypoplasia, Congenital lobar emphysema, Pulmonary sequestration, Congenital pulmonary airway malformation, Bronchogenic cyst.
N. Testemitanu State University of Medicine and Pharmacy Department of Pediatric Surgery, Orthopedics and Anesthesiology
Background
o The aim of this lecture is to provide a concise approach to congenital lung malformations.
o Discussed issues:
o bronchogenic cyst, o pulmonary agenesis and hypoplasia, o polyalveolar lobe, o alveolocapillary dysplasia, o sequestration including arteriovenous malformation (AVM) and o scimitar syndrome, o pulmonary lymphangiectasis, o congenital lobar emphysema (CLE), and o congenital cystic adenomatoid malformation (CCAM) (sin. congenital pulmonary airway malformation (CPAM)) and other lung cysts.
Background
o Although they secrete the amniotic fluid, the lungs are unnecessary as organs of respiration in fetal life.
o However, their development must occur so that air exchange may take place at birth.
o The lungs go through o embryonic, o pseudoglandular, o canalicular, o saccular, and
Background
o Early 20th century thoracic surgery consisted of mainly thoracoplasty to collapse a tuberculoid lung or to drain an empyema.
o Only with the regular use of endotracheal intubation and mechanical ventilation in the 1950s did intrathoracic procedures become routine. o These techniques were not widely applied to newborns until the 1950s. o Although Evarts Graham performed pneumonectomy with mass ligature of the hilum, Churchill was the first to regularly perform lobectomy with hilar dissection. o Gross and Lewis successfully treated a patient with congenital lobar emphysema with lobectomy in 1943.
Agenesis
Hemangioma
Atresia, stenosis Tracheal bronchus Esophageal bronchus/lung (communicating bronchopulmonary malformation) Bronchogenic cyst Enteric duplication cyst Neuroenteric cyst Bronchial cysts (peripheral)
Agenesis
Aplasia Hypoplasia
Epidemiology
o Frequency: Congenital lung malformations represent 5-18.7% of all congenital anomalies. This range may be an underestimate because of the high frequency of undetected or asymptomatic lesions.
Generalities
o Although congenital lung malformations are rare, they are important disorders because they may lead to considerable morbidity and mortality (eg, infection, hemorrhage, respiratory failure). o Prognosis depends on the size of the lesion, and the degree of functional impairment. o Small lesions may remain asymptomatic. o Failure to recognize a malformation may lead to inappropriate intervention. o For example, placement of a chest tube to manage suspected tension pneumothorax in a patient with congenital lobar emphysema may lead to lung contusion and ventilation through the chest tube instead of into the remaining healthy lung.
Generalities
o Healthy lung is composed of an orderly system of tubes (airways) and sacs (airspaces or alveoli) in a strict relationship to pulmonary blood vessels (arterial from the right ventricle and venous return to the left atrium) (Fig.3).
Generalities
o Also present is a systemic blood supply (aorta to superior vena cava) and lymphatic drainage (Fig. 5).
o Congenital lung malformations arise whenever one or more of these structures are abnormal or when their relationships are altered.
Bronchogenic cysts
o Bronchogenic cysts are also known as foregut duplication. o They arise from an abnormal budding of the ventral foregut. o Approximately 85% are mediastinal, and 15% are intrapulmonary (Fig. 6).
Bronchogenic cysts
o The peripheral cysts are multiple and appear late in gestation. o They may be filled with air or fluid, or they may have air-fluid levels.
Bronchogenic cysts
o Many are asymptomatic, but incidental findings may be observed on chest radiography (Fig. 7, 8).
Bronchogenic cysts
o Many are asymptomatic, but incidental findings may be observed on chest radiography and (Fig. 7, 8).
Fig. 8. Bronchogenic cyst. CT scan demonstrates a thin-walled cyst in the right upper lobe.
Bronchogenic cysts
o Infection, hemorrhage, and, in rare cases, malignancy can occur. o Respiratory distress may result in a stridor or wheeze. o Airtrapping may lead to emphysema, atelectasis, or both. o Dysphagia, chest pain, and epigastric discomfort can occur.
o The urinary proline aids in the formation of collagen by the fetal lung.
o Thyroid transcription factors also regulate lung development. o The lung hypoplasia in congenital diaphragmatic hernia is complicated by
pulmonary hypertension.
o Pulmonary aplasia leads to respiratory distress, which may vary according to the degree of alveolar involvement. Pulmonary hypoplasia may be primary when the entire lung or when one lobe is reduced in size.
development of the distal lung tissue is incomplete (Fig. 10). o The earlier the delivery of a child, the higher the incidence of lung hypoplasia. o In babies delivered before 28
approaches 20%.
affected side, and a mediastinal shift to the side of the lesion (Fig. 11,
12). o Therefore, aplasia of the right lung can be confused with dextrocardia.
Fig. 11. Pulmonary hypoplasia. (a) Schematic illustrates pulmonary hypoplasia. (b)Anteroposterior chest radiograph of a 7-month-old infant shows opacity of the left hemithorax and small left lung (arrows) with ipsilateral displacement of the mediastinum, secondary to repaired Bochdaleck hernia.
Fig. 12. Pulmonary hypoplasia. (a)Anteroposterior radiograph shows opacity of the right hemithorax and a shift of the mediastinal structures to the right, due to pulmonary hypoplasia secondary to right diaphragmatic agenesis. (b) Angiographic MR image of a 10-year-old boy shows a hypoplastic right pulmonary artery (arrows).
Pulmonary isomerism
o Pulmonary isomerism is an anomaly of the number of lung lobes. o In the common variety of pulmonary isomerism, the right lung has 2 lobes, whereas the left has 3.
Azygous lobe
o An azygous lobe is a malformation of the right upper lobe caused by an aberrant azygous vein suspended by a pleural mesentery. o An azygous lobe is a
radiographic
Fig. 13. A. A 15-year-old boy receiving chemotherapy for osteogenic sarcoma of the left femur. The chest radiograph showed an azygous lobe of the lung with a thin fissure (arrows) separating it from the rest of the right upper lobe. Computed tomography (Panels B and C) revealed the fissure (Panel B, white arrows and Panel C, asterisk) and a small nodule in the posterior right upper lobe, 1 cm in diameter (Panel B, black arrow). Evaluation of a specimen from a thoracoscopic lung biopsy (Panel D) showed that the membranous fissure (asterisk) contained the azygos vein (arrowheads) and divided the apex of the right hemithorax. The lung nodule was found to contain metastatic osteosarcoma.
curiosity
clinical
without
Pulmonary sequestration
o If an accessory lung bud forms early enough, it leads to the formation of sequestration in the normal lung tissue. o Development late in gestation leads to extrapulmonic sequestration.
o Both types obtain their blood supply from the aorta or its branches.
o Patients may present with exercise intolerance due to these vascular shunts. o Sequestrations may also be connected to the GI tract.
Pulmonary sequestration
o Pulmonary INTRALOBAR EXTRALOBAR accounts for sequestration 6% of all
congenital lung malformations and mostly occurs in the lower lobes. o A sequestration is a bronchopulmonary mass without
normal
bronchial
or anomalous vascular supply from the systemic circulation. o Sequestered lung may be intralobar or extralobar.
Pulmonary sequestration
o Children present with recurrent respiratory problems in the same anatomic location. o Associated anomalies include diaphragmatic hernia and eventration.
Pulmonary sequestration
o About 50% of pulmonary sequestration cases are intralobar, and 60% of intralobar cases occur in the left lower lobe with equal sex distributions. o Patients with intralobar sequestration usually present late.
Pulmonary sequestration
o Approximately 95% of extrapulmonary cases are left sided. o Most extrapulmonary cases are detected in infancy, with boys affected 4 times more than girls.
Scimitar syndrome
o The constant feature of this syndrome is partial or total anomalous pulmonary venous return to the inferior vena cava. o This abnormal vein on the chest
Scimitar syndrome
o Other features of the syndrome are variable and may include dextrocardia, hypoplasia of the right lung and/or pulmonary artery, malformation of the bronchi, and systemic arterial supply to the right lung. o The clinical features vary according to age. Infants almost always present with congestive heart failure and severe pulmonary hypertension. Adults are generally asymptomatic.
Fig. 16. Chest x-ray of a five-year-old girl with Scimitar syndrome. The heart (blue outline) is shifted into the right half of the chest, and the anomalous pulmonary venous return (red) has a shape reminiscent of a Scimitar.
Scimitar syndrome
Lung hamartoma
o Hamartomas are lung nodules contain cartilage, respiratory epithelium, and collagen. o They may be in the lung tissue or the bronchial lumen.
A A
Fig. 18. Pulmonary hamartoma. (A) Chest CT scan shows a heterogeneous, sharply marginated lesion with small focal areas of calcification and fat. These findings are typical features of hamartoma. (B) The chest radiograph shows a solitary pulmonary nodule in the left lung apex. Close inspection of this nodule shows small foci of increased attenuation within the nodule, consistent with calcium. These findings are typical features of hamartoma.
Lung hamartoma
o They are presumed to be congenital because they are usually found on chest radiographs in asymptomatic adults. o They can cause airway obstruction and are usually excised for
diagnosis.
A A
Fig. 18. Pulmonary hamartoma. (A) Chest CT scan shows a heterogeneous, sharply marginated lesion with small focal areas of calcification and fat. These findings are typical features of hamartoma. (B) The chest radiograph shows a solitary pulmonary nodule in the left lung apex. Close inspection of this nodule shows small foci of increased attenuation within the nodule, consistent with calcium. These findings are typical features of hamartoma.
pulmonary
venous 19).
arterial
and
systems
without
Fig. 19. MRI angiography using MIP (Maximum Intensity Projection) reconstruction shows the presence of multiple, bilateral arteriovenous malformations of pulmonary vessels. vessels
dyspnea.
o Hemoptysis is most common in patients who also have cutaneous telangiectasis. o A continuous bruit is often heard over the lesion.
intrahilar
pulmonary
arteriovenous
Fig. 22. Alveolar capillary dysplasia: characteristic histologic features of ACD/MPV: thickened alveolar septae with scarce dilated pulmonary capillaries located away from the alveolar epithelium, with absence of the usual alveolar-capillary barrier ( ); medial hypertrophy of small pulmonary arteries and muscularization of distal arterioles (); congested pulmonary veins malpositioned, adjacent to pulmonary arteries in the same adventitial sheath (*); lymphangiectasis not present.
Pulmonary lymphangiectasis
o Pulmonary lymphangiectasis is a rare disorder in which the normal pulmonary lymphatics are dilated. o It may be associated with congenital heart disease in which the
channels occurs.
o The disease can also be part of a syndrome of lymphangiomas in many organs; it is sometimes associated with vanishing bones (Gorhams disease). o Pulmonary lymphangiectasis is congenital, but symptoms of respiratory insufficiency usually do not appear until adulthood.
respectively.
o Within one hour after birth, severe respiratory distress with profound cyanosis developed while the infant was breathing room air, and
o Congenital heart disease was ruled out, and cultures of blood and
tracheal aspirates were unrevealing. o After surfactant protein B deficiency was ruled out, an open-lung biopsy
patients.
o Lesions most commonly occur in whites, in male individuals (male-tofemale ratio, 3:1), and in young infants.
Fig. 27. Chest X-ray. (front and lateral views) Congenital lobar emphysema on the right side of the chest in a neonate. Film shows marked lucency and hyperexpansion in the middle lobe of the right lung; this finding is consistent with lobar emphysema. The possibility of tension pneumothorax is unlikely because lung markings are seen in this region, with splaying of the pulmonary vessels. Compressive atelectasis is present in the left upper and right lower areas of the lungs. The mediastinum and heart are shifted to the left. The osseous structures are intact.
Fig. 28. Same patient as in the previous 2 images. After surgery, the left lung is expanded. A thoracotomy tube is on the right, with a small right-sided pneumothorax.
Fig. 29. CCAM on chest radiograph in a newborn. Large cystic changes in the left lung, leading to a mediastinal shift to the right due to their mass effect.
Fig. 30. Contrast enhanced computerized tomography of the chest of the patient with congenital cystic adenomatoid malformation (CCAM)
o (3) solid cystic adenomatoid malformation (13%), which has the worst prognosis and is an airless tissue mass composed of cuboidal epithelium-lined bronchioles.
o The difference in prognosis may be because the solid and microcystic lesions involve a relatively large amount of lung tissue. Macrocystic lesions are comprised of large, air filled, nonfunctioning spaces involving smaller areas of lungs.
o Malignancy can occur in the cystic adenomatoid malformation (pulmonary blastoma, rhabdomyosarcoma, and bronchoalveolar carcinoma). See the images below.
Fig. 32. Initial radiograph in a patient with congenital cystic adenomatoid malformation on the first day of life with opaque lungs and a suggestion that the right lung is slightly more voluminous than the left lung.
Fig. 33. Radiograph obtained in the same patient as in the previous image on the second day of life shows that the physiologic fluid is resorbed and replaced with an air-containing cystic area occupying the right upper lung.
Polyalveolar lobe
o In a polyalveolar lobe, the number of alveoli increased to more than 3 times normal. o The alveoli are counted microscopically in random lung sections.
o When extra lung fluid is retained, respiratory distress may occur in the
first days of life. o This generally benign anomaly may be associated with some cases of
INDICATIONS
Bronchogenic cyst
o Resection is recommended because of the potential for infection, hemorrhage, and respiratory compromise. o Resection is especially important in the peripheral lesions, which are
usually multiple.
o These can frequently be excised thoracoscopically because they seldom have a major blood supply.
Pulmonary sequestration
o Resection is recommended, even in asymptomatic patients, to prevent infection, hemorrhage, shunting from arteriovenous anastomoses, or compression of normal lung mass leading to respiratory distress.
Pulmonary sequestration
o Resection is recommended, even in asymptomatic patients, to prevent infection, hemorrhage, shunting from arteriovenous anastomoses, or compression of normal lung mass leading to respiratory distress.
Scimitar syndrome
o When symptoms of scimitar syndrome are related to anomalous pulmonary venous return, this return can be redirected surgically. o Symptoms are often related to the bronchial abnormalities and chronic
infection.
o In these cases, pneumonectomy is indicated.
Fig. 37. Congenital lobar emphysema of the upper lobe of the left lung.
Fig. 38. Congenital lobar emphysema of the upper lobe of the left lung.
WORKUP
Laboratory Studies
o Hemoglobin testing is always valuable in respiratory illness because the result is an important factor in oxygen delivery and in planning surgery, which often involves major vessels.
Imaging Studies
(Chest radiography)
Bronchogenic cysts: Bronchogenic cysts are usually fluid-filled lesions and are well circumscribed in the mediastinum.
Fig. 40. Mediastinal bronchogenic cyst.
Solid masses may be difficult to differentiate from fluid. Intrapulmonic cysts appear as solitary nodules unless they contain air.
Imaging Studies
(Chest radiography)
Pulmonary hypoplasia: In pulmonary hypoplasia, a mediastinal shift to the side of a homogenous density may be depicted, with compensatory herniation of the uninvolved lung.
Fig. 41. Chest radiograph shows diffuse haziness of the right hemithorax (arrow), slight deviation of the trachea and heart to the right, and poor differentiation of the right heart border.
Imaging Studies
(Chest radiography)
o Pulmonary sequestration: In pulmonary sequestration, an opaque or cystic lesion is seen, depending on the presence of infection.
Fig. 42. Chest radiograph. The PA view demonstrates a left sided triangular density of the medial left lung base. On the lateral view, the triangular density is seen posteriorly over the left lung base.
Imaging Studies
(Chest radiography)
o Congenital lobar emphysema (CLE): In congenital lobar emphysema, the involved lobe crossing the midline and the compressed normal lung can be seen.
Imaging Studies
(Chest radiography)
o This appearance does not change during expiration or in the decubitus position. o Vascularity of the involved site is attenuated.
Imaging Studies
(Chest radiography)
o The intercostal spaces in the involved site appear widened, and the hemidiaphragm is flattened. Lucent, anteriorly herniated lung pushes the lung posteriorly, as seen on the lateral view.
Imaging Studies
(Chest radiography)
o The lesion must be differentiated from contralateral lung hypoplasia and ipsilateral pneumothorax.
Imaging Studies
(Chest radiography)
o The lesion must be differentiated from contralateral lung hypoplasia and ipsilateral pneumothorax.
Imaging Studies
(Chest radiography)
o Cystic adenomatoid malformation (CAM) or congenital pulmonary airway malformation (CCAM): Cystic adenomatoid malformation is seen as a mass of air-filled cystic spaces with mediastinal shift.
Imaging Studies
(Chest radiography)
o In a newborn, the lung fluid may not have been absorbed, and the mass may appear fluid filled and homogenous.
Imaging Studies
(Chest radiography)
o In an older child, pneumatoceles may be confused with cystic adenomatoid malformation. o Pneumatoceles are postinfectious pulmonary cysts.
Fig. 47. Chest X-ray with large, expanding, multicystic pneumatocele associated with Respiratory Syncytial Virus infection.
Imaging Studies
(Chest radiography)
o They occur after Staphylococcus aureus infection. o However, they are unlikely to recur in the same area of the lung as where cystic adenomatoid malformation occurs.
Fig. 47. Chest X-ray with large, expanding, multicystic pneumatocele associated with Respiratory Syncytial Virus infection.
Imaging Studies
(Chest radiography)
o Bronchogenic cysts are central or mediastinal (relatively early embryologic origin) and peripheral or intrapulmonary (late origin). o The latter are rare (15%) and tend to be multiple.
Fig. 48. Chest X-ray revealing bronchogenic cyst in the posterior right middle lobe.
Imaging Studies
(Chest radiography)
o Congenital lobar emphysema is exceedingly rare in the lower lobes (< 5% of patients). o It is associated with respiratory distress and mediastinal shift.
Imaging Studies
(Chest CT scanning)
o Bronchogenic cysts: In patients with bronchogenic cysts, CT findings are characteristic. o The lesions are sharply marginated and nonenhancing. If the lesions are seen as soft-tissue attenuation instead of water attenuation, differentiating from lymph nodes may be difficult.
Imaging Studies
(Chest CT scanning)
o Pulmonary hypoplasia: In lung hypoplasia, loss of lung volume and associated anomalies can be seen.
Fig. 51. Right lung hypoplasia. Chest CT scan shows herniation of left lung to right side with narrow right main bronchus and shift of heart to right side.
Imaging Studies
(Chest CT scanning)
Pulmonary sequestration: In pulmonary sequestration, the findings may be only an unusual solid attenuation. Therefore, CT may have little to add to
sonographic
and
plain
be
Fig. 52. CT with IV contrast of the thorax showing an Intralobar Bronchopulmonary Sequestration. The yellow arrow in frames A and B point to a hyperdense region in the left lower lobe of the lung with small cystic lesions containing air within it. The red arrows in frames C and D show a contrast enhanced vessel arising from the aorta and supplying the area of hyperdensity in the lung.
visualized
with
vascular
contrast enhancement.
Imaging Studies
(Chest CT scanning)
Congenital lobar emphysema: In congenital lobar emphysema, the involved lobe and its vascularity can be easily outlined as compared to normal lung parenchyma.
Fig. 53. Axial CT of an infant with mediastinal shift shown on a chest radiograph showing hyperinflation of the left upper lobe without destruction of the alveolar walls, consistent with congenital lobar emphysema
Imaging Studies
(Chest CT scanning)
o Cystic of cystic adenomatoid adenomatoid can be malformation: Different types malformation
with
Fig. 54. Chest CT scan shows an abnormal left lung with extensive, thin walled, well-defined cystic lesions. Some of the lesions appear to contain small amounts of fluid (short arrow). There is a mediastinal shift to the left and hyperexpansion of the right lung (long arrow).
CT
than
with
chest
radiography
can
still
be
o The cysts appear bright on T2weighted images and do not enhance after the administration of
gadolinium-based
Fig. 55. MRI in the supine (A, B) and right lateral decubitus (C, D) positions. Bronchogenic cyst masquerading as pericarditis.
contrast
material.
as of
the
diagnostic for
choice
hydrops fetalis in a fetus with a prenatal, MRI may be beneficial for planning surgery. Cysts larger
Fig. 57. Congenital cystic adenomatoid malformation. Fetus at 22 weeks gestation. a, b and c Fetal sagittal, coronal, and axial single-shot fast spin-echo MR images of the fetal chest show a complex hyperintense lesion with cysts inside in the right lung (arrows). CT after birth d shows the lesion, which is proportionally smaller than in the MR images
Imaging Studies
(Prenatal ultrasonography)
o Lesions on prenatal sonograms may shrink or disappear with advancing gestational age. o Most pulmonary parenchymal lesions appear as echogenic fetal chest masses. o The masses may be unilateral (eg, in cystic adenomatoid malformation, congenital diaphragmatic hernia, pulmonary sequestration) or bilateral (eg, in tracheal atresia). o A cystic component is seen in cystic adenomatoid malformation and congenital diaphragmatic hernia (CDH) and can make differentiation of these lesions difficult.
Imaging Studies
(Prenatal ultrasonography)
o Pulmonary hypoplasia: In lung hypoplasia, renal malformations, oligohydramnios, decreased fetal movements in neuromuscular disease, dysmorphisms in trisomies, and skeletal dysplasias may be identified. The thoracic-to-abdomen ratio and lung area are useful parameters. Pulmonary arterial flow can be measured by using Doppler studies.
Fig. 58. Diaphragmatic Hernia. A. Transverse image through the chest shows the stomach (S) and a large volume of small bowel (straight arrows) occupying the left chest and displacing the heart (curved arrow) into the right thorax. No lung is visible, indicating that severe pulmonary hypoplasia is very likely. B. In another fetus, a moderate volume of lung (L) is visible in the right thorax even though the heart (long arrow) is displaced rightward by the herniation of bowel (short arrows) and stomach (S) into the left thorax.
Imaging Studies
(Prenatal ultrasonography)
o Congenital lobar emphysema: In congenital lobar emphysema, a large fluidfilled lobe may be seen.
Fig. 59. Sagittal section of the thorax showing enlargement of the left lung. The right lung has normal echogenicity.
Imaging Studies
(Prenatal ultrasonography)
o Cystic adenomatoid malformation: In cystic adenomatoid malformation, a unilateral cystic mass is seen. However, the patient's postnatal clinical course and chest radiographic findings may be normal, and CT, MRI, and/or MRA may be indicated.
Fig. 60. 27th week of gestation. Image shows an axial view through the area of cystic adenomatoid malformation of the left fetal lung, type I. Large anechogenic, left-sided lesion (arrow) displaces the heart to the right.
Imaging Studies
o Sequestration does not fill up at all during the early pulmonary phase, but it
does during the systemic (late) phase. o The value of radionuclide imaging is limited because of the lack of anatomic
details.