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38 C. M. Leeper et al.
Fig. 4.14 a and b Ultrasound is utilized for image-guided percutaneous drain placement
bris will produce color signal whereas nonmobile solid material does not [27].
In addition to establishing the diagnosis of pleural effusion as well as the extent and posi-
tion of the fluid, ultrasound may help to assess the exact position for fluid aspiration or drainage (Fig. 4.14a and 4.14b). Management of pleural effusion may range from drainage to fibrinoly-
394 The Thorax
sis to operative decortication depending on the stage.
Solid Pleural Masses
Malignant disease involving the pleural space is much less common in children than in adults. It can occur for instance with Wilms tumor, neuro­blastoma, leukemia, and sarcomas. Primary chest wall neoplasms and pleural metastases are often accompanied by hemorrhagic pleural effusions, which appear as echogenic debris-filled fluid in sonography. The presence of pleural fluid aids in detection of solid masses adherent to the parietal or visceral pleura.
Diaphragm
The diaphragm is a thin muscle that separates the thoracic cavity from the abdominal cavity. The diaphragm presents best next to the cardia (ster­nal section) and one may also visualize the dia­phragm with high-resolution transducers cranial to the liver and spleen. Ultrasound is a valuable tool in assessing the diaphragm allowing delin­eation of juxta-diaphragmatic masses, contour abnormalities and hernias, and evaluation of dia­phragmatic motion.
Diaphragmatic Hernia
A diaphragmatic hernia is a defect of the dia­phragm that permits displacement of abdominal contents into the chest (Fig. 4.15a, 4.15b, 4.15c,
4.15d, 4.15e). Diaphragmatic hernias in infants
are generally congenital, representing the most common intrathoracic anomaly seen in the fetus. In older children, they may also be acquired as a result of trauma.
Congenital diaphragmatic hernias (CDHs) (incidence 2.4:10,000) are located on the left in 80 % of patients, are more common in males, and are often associated with other congenital anomalies. Survival ranges from 50 to 90 % with major morbidity resulting from pulmonary hypo­plasia [26].
Approximately 50–70 % of CDH are diag­nosed antenatally by obstetric ultrasound. Fetal ultrasound features include polyhydramnios, intrathoracic bowel loops or stomach, an echo­genic chest mass or mediastinal shift. The diag­nosis can be made as early as 11 weeks, but most may not be evident until 16 weeks or later [28]. Postnatally, the diagnosis of CDH is often evi­dent on chest radiograph with intestine visualized in the chest cavity. In cases that are equivocal, especially with right-sided hernias, ultrasound (sagittal and coronal scanning) becomes a useful modality for confirmation and further character­ization. Ultrasound will demonstrate abdominal tissue extending into the thoracic cavity. While a herniation of liver or spleen is easily identifiable, the reliable detection of air-filled bowel or stom­ach might be more challenging.
Diaphragmatic Eventration/ Diaphragmatic Paresis
Eventration is an abnormal elevation of the dia­phragm resulting from the incomplete develop­ment of the central tendon; this results in para­doxical motion during respiration which inhibits normal pulmonary function. This defect may be a congenital lesion or may be a complication of a birth trauma, mediastinal tumor, or cardiotho­racic surgery [26].
The movement of the diaphragm has tradi­tionally been examined by fluoroscopic screen­ing, however, the use of ultrasound for this pur­pose in children is increasing due to the many advantages it affords [29]. The ultrasound must be performed with the child in quiet respiration. Difficulties arise for the sonographer when the child is on a ventilator as the ventilator fills the lungs with air causing the diaphragms to move down with inspiration even when paralyzed. In this case, it is best to assess the diaphragms for a short period of spontaneous breathing.
Sagittal or coronal imaging of the upper ab­domen provides information about that particu­lar hemidiaphragm, whereas transverse imaging allows comparison of both hemidiaphragms and evaluation for paradoxic motion with unilateral
40 C. M. Leeper et al.
Fig. 4.15 Congenital diaphragmatic hernia, prenatal and neonatal ultrasound. a Sagittal ultrasound image of the right fetal chest; dome of the liver (arrow) was adjacent to the thoracic apex, with a small crescent of pleural fluid in the intervening space (arrowhead). b Sagittal ultrasound image of the left fetal chest; left lobe of the liver (arrow) was herniated into the thoracic cavity, although not as severely as the right lobe. Pleural effusion was present. c. X-ray of the chest and abdomen reveals a large cen-
paralysis. With M-mode recording, ultrasound can provide quantitative information about dia­phragmatic excursion as diaphragmatic move-
tral intrathoracic mass (arrows). d Transverse ultrasound image of the neonatal chest demonstrated bilateral pleural fluid which extended around the heart (curved arrow) and communicated with ascitic fluid. The liver (arrow) and gallbladder fundus (arrowhead) were identified at this level. e Transverse neonatal ultrasound of the right upper quadrant of the abdomen; a small rim of diaphragm was seen posterolateral to the liver, with an abrupt termination (arrows). [43]
ment appears as a sinusoidal curve (Fig. 4.16). Diaphragmatic excursion is normal when greater than 4 mm and symmetric between the leaflets of
414 The Thorax
Fig. 4.16 Right eventration. Excursion of the diaphragm is reduced on dynamic imaging, and amplitude is greatly decreased in M-mode
the diaphragm (less than a 50 % difference). Nor­mal excursion will be toward the transducer with a subxiphoid approach [30]. Intraindividual com­parisons in the course of a disease can be very useful. Typical sonographic signs of diaphrag­matic paresis are: (1) limited or lack of mobil­ity in the longitudinal and cross-sectional view, (2) reduced or zero amplitude in M-mode, and (3) in cross section, greater distance between the transducer and the diaphragm line on the affected side [28].
Lung
Consolidation—Atelectasis, Pneumonia, Abscess
Airless lung can appear sonographically similar to liver (hepatization) (Fig. 4.17) [31]. Despite the lack of air in the consolidated lung, the un­derlying internal architecture of the lung is pre­served, allowing differentiation from masses or other processes.
In pneumonia, branching linear echogenici­ties representing air bronchograms are often seen [32] (Fig. 4.17). Entrapped fluid or mucoid mate-
rial within bronchi in necrotizing or postobstruc­tive pneumonias produces hypoechoic branching structures— the sonographic fluid bronchogram [33]. Pulmonary vascular flow is preserved in simple pneumonic consolidation and is readily demonstrated with color Doppler. If there is an effusion present, the diagnosis of consolidation can be aided by the presence of an effusion; an aerated lung lobe will float over the effusion, while a consolidated lobe will swim within the effusion (the jellyfish sign) [34].
In atelectatic lung, air bronchograms are also present. Blood vessels become crowded togeth­er and display an orderly linear and branching structure that distinguishes this entity from the more irregular vasculature found in neoplasms.
Distinguishing pneumonic consolidation from simple atelectasis can be difficult radiographi­cally. It has been suggested that ultrasound can be more specific than CT scan in this situation. If there is movement of the air within bronchi, this usually indicates pneumonia, whereas the air bronchograms in atelectasis are most often static. In one study, this dynamic air bronchogram had a sensitivity of 61 % and a positive predictive value of 97 % in distinguishing pneumonia from atelec­tasis [35].
42 C. M. Leeper et al.
Fig. 4.17 Images in a 4-year-old boy with pneumonia. a Frontal radiograph of the chest shows opacity at the right lung base (star). b CT and c US images show hepatization
Abscesses form as a complication of lung in­fection. Parenchymal necrosis, indicated by de­creased echogenicity with lack of color Doppler flow within a region of pulmonary consolidation, is a characteristic finding of early abscess. Devel­oped abscesses display a thick wall and air fluid levels. If abutting the pleura, lung abscesses are sonographically visible, and ultrasound-guided aspiration and drainage can play an important role in diagnosis and treatment [26].
Pneumothorax
Ultrasound is popular for the diagnosis of pneu­mothorax in trauma patients, ventilated patients, and those undergoing lung procedures amongst others [36]. Characteristic signs such as lung sliding [37], comet tail artifacts (Fig. 4.18a and
4.18b) [38], the A-line sign [38], and lung point
[39] have been described for the sonographic di­agnosis of pneumothorax. Sensitivity and speci­ficity of this diagnosis is operator dependent, with some reports as high as 100 % in one study of 285 patients.
of the right lower lobe (star) with air bronchograms (long arrow) and surrounding pleural effusion (short arrows). [2]
Tumors
Primary lung neoplasms are very rare in chil­dren. Pulmonary blastoma is the most common and usually starts as a peripheral lesion, often at­taining large size before becoming clinically ap­parent. Other less common tumors include mu­coepidermoid carcinoma, hemangiopericytoma, leiomyosarcoma, rhabdomyosarcoma, and bron­chogenic tumors. If the tumor is fully surrounded by aerated lung tissues, it might be sonographi­cally invisible; a reliable sonographic evaluation of intrathoracic tumors is only possible if they are next to the chest wall or diaphragm or if they are surrounded by a pleural effusion. As with pleural lesions, if a lung mass is sufficiently pe­ripheral and abuts the lung surface, percutaneous ultrasound-guided biopsy is a safe and effective method for obtaining a tissue diagnosis.
Bronchopulmonary Malformations (BPM)
Congenital parenchymal masses include con­genital pulmonary airway malformation (CPAM)
434 The Thorax
Fig. 4.18 a and b US images of the right and left lung bases demonstrate absence ( arrowheads) and presence ( arrows) of comet tail sign, indicating right-sided pneumothorax. [2]
and sequestration. Although often regarded as separate entities, these malformations are part of a spectrum of CPAMs and may have overlapping imaging and histologic features. These masses may be detected prenatally by ultrasound or MR imaging, appearing as variably solid or cystic structures. Postnatally, plain radiographs usually show the lesion as incidental finding or on im­ages taken for respiratory symptoms.
CPAM
CPAM is the most common BPM (incidence
0.66:10,000) [26]. CPAM is a congenital hamar­tomatous lesion of the lung resulting in a mass of disorganized lung tissue that is localized to a sin­gle bronchial tree segment. CPAMs can be classi­fied into two categories: (1) macrocystic lesions containing single or multiple cysts that are at least 5.0 mm in diameter and (2) microcystic le­sions presenting as a solid echogenic mass. Most CPAMs derive their blood supply from the pul­monary artery and drain via the pulmonary veins, with the exception of hybrid lesions, which can have a systemic blood supply. Importantly, they normally communicate with bronchial tree [26].
CPAMs are typically diagnosed antenatally on fetal ultrasound or in the early postnatal period with chest radiograph. Ultrasound will usually demonstrate an area of hyperechogenic tissue with or without hypoechoic cysts that vary in number and size (Fig. 4.19a, 4.19b, 4.19c). Signs of mass effect may be seen, such as mediastinal
shift, diaphragmatic eversion, and polyhydram­nios. With very large lesions, heart failure (hy­drops) due to mediastinal shift and cardiac com­pression can occur [26]. Ultrasound can also con­tribute to the evaluation of these patients when there is a suspected sequestrated segment in as­sociation with the CPAM.
Pulmonary Sequestration
Pulmonary sequestration is a segment of lung which does not function, has an anomalous arte­rial blood supply from the systemic circulation, and has no communication with the tracheobron­chial tree. It is due to a developmental abnormal­ity in which there is an accessory tracheobronchi­al foregut bud. There are two types of pulmonary sequestration: intralobar, which shares visceral pleural investment with normal lung and drains into the pulmonary venous system, and extralo­bar, which has a separate pleural investment and may have either systemic or pulmonary venous drainage [26].
Many pulmonary sequestrations are diagnosed prenatally but some are diagnosed in children with the typical clinical presentation of a lower lobe consolidation that never clears completely. In those patients, color Doppler sonography is diagnostically reliable. Due to the typical loca­tion, one will be able to visualize the sequestra­tion best from subxiphoidal or subcostal in most patients. It has an echogenicity similar to liver tissue with occasional central hypoechoic areas.
44 C. M. Leeper et al.
Fig. 4.19 Congenital pulmonary airway malformation (CPAM). a CT scan and b chest radiograph demonstrate intrathoracic mass. c Ultrasound shows hyperechogenic tissue with cysts
454 The Thorax
Fig. 4.20 Extralobar sequestration. Longitudinal, oblique ultrasound scan of the mid-abdomen. Aberrant vessel ( arrows) anterior to the abdominal aorta a for bet- ter comparison, the scan is presented in the same position
Since the sequestration has no connection to the bronchial system, it does not show reflexes with high echogenicity. The key diagnostic feature of sequestration is demonstrating systemic arte­rial supply, usually from the descending aorta. In cases of blood supply originating from the abdominal aorta, the visualization of the feeding vessel is often possible (Fig. 4.20a and 4.20b). Contrast-enhanced CT and MR imaging are often required in unclear cases or in older patients with limited acoustic windows.
Intralobar sequestrations have pulmonary venous drainage and variable degrees of hyper­echogenicity by ultrasound. They are always as­sociated with the lower lobes and are distinguish­able by the absence of pulmonary arterial inflow. Extralobar sequestrations (Fig. 4.21) occur more commonly in males and are four times more common on the left. Over 60 % of patients have associated anomalies (e.g., diaphragmatic de-
as the lateral angiogram ( SM—superior mesenteric artery, C—celiac axis, H—head, F—feet), b Abdominal aorto- gram, lateral view demonstrating the aberrant artery ( ar­rows) supplying the sequestration (compare with a). [44]
Fig. 4.21 Extralobar sequestration. Longitudinal view of solid triangular mass (S) in the chest, separated from the liver (L) (K—right kidney). [44]
fect, CPAM). They appear as homogeneous hyperechoic masses at any level in the pleural space and can be found within or beneath the diaphragm [26] (Fig. 4.22). They are found in a
46 C. M. Leeper et al.
Fig. 4.22 Inptrapericardial extralobar sequestration. Sagittal right parasternal ultrasound scan showing the thymus (T) and the mass (M) in the superior mediastinum. The acoustic shadows are caused by rib cartilage (r). The mass abuts the aorta (AO), and vessels arising from the right pulmonary artery (RPA) could be demonstrated. [45]
paraspinal location with systemic blood supply and systemic venous drainage identified by color Doppler.
Cysts
Other cystic masses are displayed as hypoechoic areas. They can be visualized by ultrasound if they are located peripherally, but reliable identi­fication may require additional imaging modali­ties in many cases. Differential diagnoses include abscesses in the final stage, bronchogenic cysts, echinococcal cysts, pericardial cysts, and arterio­venous fistulas. The latter can be distinguished using color Doppler sonography.
Summary
Ultrasound is the most important imaging modal­ity in children and can play an important role in evaluation of the pediatric chest. It can be used as an adjunct or the sole diagnostic modality for a variety of congenital and acquired conditions. The mediastinum, lungs, chest wall, diaphragm, and the major intrathoracic vessels can all be
evaluated with ultrasound imaging. Ultrasound permits dynamic, real-time assessment of these structures without risk or radiation exposure.
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