Radiological Category: Pediatrics Principal Modality: Plain film Secondary

Radiological Category: Pediatrics Principal Modality: Plain film Secondary

Radiological Category: Pediatrics Principal Modality: Plain film Secondary Modality: MRI Case Report # 826 Submitted by: J. Caleb Richards, M.D.

Faculty reviewer: Abhay Srinivasan, M.D,. University of Texas Medical School at Houston Date accepted: 15 April, 2011 Case History

Newborn child Further history temporarily withheld Chest Radiograph Test Your Diagnosis Which feature(s) is(are) present? a. Mediastinal shift b. Pneumothorax c. Cystic mass d. Situs abnormality

Chest Radiograph Answer: Mediastinal shift to the right (Hint: look at the endotracheal and nasogastric tubes) Cystic mass in the left hemithorax Additional Case History

A prenatal ultrasound and MRI were performed months earlier Prenatal Ultrasound Images in coronal plane Prenatal MRI T2WI Images in coronal plane

R L Test Your Diagnosis Which feature(s) is(are) present? a. Unilocular mass in the left hemithorax with increased signal on T2WI b. Multiloculated mass in left hemithorax with increased signal on T2WI c. Multiloculated anechoic spaces in left hemithorax with intermixed soft tissues

d. Hyperechoic lesions in right hemithorax Prenatal Ultrasound Answer: A coronal image of the chest shows multiloculated anechoic spaces with intermixed soft tissue in the left hemithorax (outlined by calipers)

Prenatal MRI T2WI Answer: Multiloculated cystic lesion with discrete walls in the left hemithorax demonstrating increased signal on T2WI R

L Test Your Diagnosis What is the diagnosis? a. Congenital Pulmonary Airway Malformation b. Pulmonary Sequestration c. Congenital Diaphragmatic Hernia d. Congenital Lobar Overinflation e. Bronchogenic Cyst Discussion Congenital Pulmonary Airway Malformation A heterogeneous group of cystic and noncystic lung lesions that largely result from early airway

maldevelopment Three types are distinguished at imaging: I. Large cyst CPAM cysts are 2-10 cm II. Small cyst CPAM cysts are 0.5-2 cm III. Microcystic or solid type CPAM cysts are smaller than 5mm, and no cystic spaces are discernable At pathologic analysis, CPAMs are classified according to cyst size and histologic resemblance to segments of the developing bronchial tree and airspaces CT showing a complex cystic mass in the left lung (arrow) all with cyst diameter less than 2 cm compatible with small cyst CPAM -Biyyam, et al

Used with permission of the copyright owner Discussion Congenital Pulmonary Airway Malformation Large cyst type Cyst diameter 2-10 cm Fetal US shows numerous variable sized anechoic spaces intermixed with echogenic soft tissue Fetal MRI shows hyperintense unilocular or multilocular lesions with discrete walls on T2-weighted images Postnatal radiography shows variable density in the region of the mass depending on the fluid contents of the cysts, and possibly mediastinal shift, depending on the size of the CPAM Gross path of a large cyst CPAM with a large multiloculated cyst (arrow)

-Biyyam, et al Used with permission of the copyright owner Discussion Congenital Pulmonary Airway Malformation Small cyst type Cyst diameter 0.5-2 cm Fetal US shows an echogenic mass with multiple small cysts T2 Weighted MRI shows a variable appearance that depends on the cystic and solid components Postnatal radiography may show a heterogeneous intrapulmonary mass with small air-filled cystic areas Gross path of a small cyst CPAM with

multiple areas of small cysts and solid areas of abnormal lung parenchyma -Biyyam, et al Used with permission of the copyright owner Discussion Congenital Pulmonary Airway Malformation Microcystic or solid type Cyst diameter less than 0.5 cm Fetal US shows a homogenously echogenic mass T2 Weighted MRI shows a homogenously hyperintense mass with normal adjacent lung parenchyma Postnatal CT may show an ill defined area of increased attenuation

Prenatal US in coronal plane showing homogenously echogenic mass in the left hemithorax without cystic component. Note the intact diaphragm (arrows) -Biyyam, et al Used with permission of the copyright owner Discussion Pulmonary Sequestration Characterized by a portion of lung which is not connected to the tracheobronchial tree and has systemic arterial supply, usually from the thoracic or abdominal aorta Postnatal radiography shows the lesions to be soft tissue density masses with smooth or lobulated contours, usually in the lung bases

Postnatal CT shows a feeding artery and possibly abnormal venous drainage Two types: Intralobar and extralobar Extralobar Has its own pleural investment and systemic drainage Most commonly diagnosed in prenatal-antenatal period Associated with other congenital anomalies like CDH, cardiac anomalies, pulmonary hypoplasia, or foregut duplication cysts May be subdiaphragmatic and mimic adrenal hemorrhage or neuroblastoma Prenatal ultrasound shows a homogenous hyperechoic mass in a paraspinal location, often left lower hemithorax Fetal T2 MRI shows a solid, well-defined, hyperintense mass Discussion Pulmonary Sequestration

Intralobar Shares the pleural investment with the normal lung and usually drains into pulmonary venous system Commonly diagnosed in older children or adulthood, usually presenting as recurrent lower lobe pneumonia CT shows a homogeneous soft tissue mass, cysts containing air or fluid, focal emphysema, or a hypervascular focus of lung parenchyma Homogenous mass in the left lower lobe with a feeding vessel arising from the aorta, a finding diagnostic of sequestration -Biyyam, et al Used with permission of the copyright

owner Discussion Hybrid Pulmonary Sequestration and Pulmonary Airway Malformation Hybrid lesions can occur that display both imaging features of both sequestration and CPAM The macrocystic CPAMs, especially the small cyst type, are more likely to have an abnormal systemic arterial supply and thus be classified as hybrid Transverse view of the chest shows a hyperechoic mass (calipers) with a cystic component (arrow) suggesting a CPAM. However, color doppler reveals a feeding artery (arrowhead) arising from the aorta, a finding compatible with sequestration. -Biyyam, et al Used with permission of the copyright owner

Discussion Congenital Diaphragmatic Hernia Herniation of bowel contents into the thorax typically through a posterior defect in the diaphragm More common on the left than right (5:1) Large CDH causes compression of the pulmonary tissue with resultant pulmonary hypoplasia Right sided CDH with the liver (Li) and hepatic flexure (arrows) herniated into the right hemithorax -Biyyam, et al Used with permission of the copyright owner

Discussion Congenital Diaphragmatic Hernia Imaging clues: Plain film: Bubble like lucencies in the chest that appear like bowel, although the hernia may initially appear radiodense prior to air introduced into bowel Absent bowel gas in the abdomen Herniated liver in right sided CDH is soft tissue density Ultrasound: Fluid filled stomach and bowel in thorax Color doppler is useful in identifying portal and hepatic veins in herniated liver

MRI Meconium filled bowel is hyperintense on T1 and hypointense on T2 Transverse view of the chest with color doppler showing portal and hepatic veins of the herniated liver -Biyyam, et al Used with permission of the copyright owner Discussion Congenital Lobar Overinflation Characterized by progressive lobar expansion with compression of the ipsilateral lung Underlying cause can be secondary to an intrinsic cartilaginous abnormality with resultant weak or absent bronchial cartilage or extrinsic compression of an airway

The collapsed airway acts as a one way valve causing air trapping Although the alveoli expand, the walls remain intact; therefore the term emphysema is inaccurate The left upper lobe (42%), right middle lobe (35%), and right upper lobe (20%) are much more commonly involved than the lower lobes (each less than 1%) Plain film showing hyperlucent left hemithorax with mediastinal shift -Biyyam, et al Used with permission of the copyright owner Discussion Congenital Lobar Overinflation On radiography, the affected lung is initially radiodense, since the affected section

still contains fluid As the fluid is resolved, the affected lung overexpands and becomes lucent on radiography and CT and eventually causes mass effect Fetal ultrasound shows a homogenously hyperechoic mass Fetal MRI may show a homogenously hyperintense mass on T2 weighted imaging CT shows an overinflated left upper lobe -Biyyam, et al Used with permission of the copyright owner Discussion Bronchogenic Cyst Part of the spectrum of foregut duplication cysts

Are developmental lesions resulting from abnormal ventral budding of the tracheobronchial tree Mostly located in the mediastinum near the carina Less commonly located in the lung parenchyma, pleura, or mediastinum Most are found incidentally In infants, symptoms are related to compression of trachea or bronchi and esophagus leading to wheezing, stridor, dyspnea, and/or dysphagia Intraparenchymal cysts may present with recurrent infection Gross specimen of a bronchogenic cyst which is translucent -Biyyam, et al Used with permission of the copyright owner Discussion

Bronchogenic Cyst Plain film findings are nonspecific, showing soft tissue density mass in the middle mediastinum CT is helpful showing the cysts to be well-defined lesions with smooth walls and water attenuation Occasionally the cysts show attenuation slightly greater than water due to proteinaceous mucoid material and/or milk of calcium content Infected cysts will often contain air and thick enhancing walls On T2 MRI the cysts are high in signal intensity Well circumscribed, unilocular, nonenhancing hypoattenuating cyst in the middle mediastinum which was pathologically proven to be

bronchogenic cyst -Biyyam, et al Used with permission of the copyright owner Diagnosis Congenital Pulmonary Airway Malformation Type I References Biyyam, D et al. Congenital Lung Abnormalities: Embryologic Features, Prenatal Diagnosis, and Postnatal Radiologic-Pathologic Correlation. RadioGraphics 2010; 30:17211738 Rosado-de-Christenson, M et al. From the Archives of the AFIP: Congenital Cystic Adenomatoid Malformation. RadioGraphics 1991; 11:865-886 Lee, E et al. Multidetector CT Evaluation of Congenital Lung Anomalies. Radiology

2008; 247:632-648 Lee, S et al. Fetal MRI in the Evaluation of Chest Anomalies: A Pictorial Essay. University of Alberta Health Sciences Journal 2006; 3:27-30

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