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Cystic Fibrosis in Children and Young Adults: Findings on Routine Abdominal Sonography

¹ Department of Pediatrics, University of Tuebingen, Hoppe-Seyler-Str. 1, D-72076 Tuebingen, Germany.

View larger version (99K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —9-year-old boy with cystic fibrosis and pancreatic exocrine insufficiency. Longitudinal (A) and transverse (B) sonograms at level of pancreatic head show small pancreas (arrows) with areas of markedly increased echogenicity, resembling echogenicity of retroperitoneal fat (star, A). Scale segment distance, 10 mm. s = superior mesenteric artery, ao = aorta.

View larger version (88K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —9-year-old boy with cystic fibrosis and pancreatic exocrine insufficiency. Longitudinal (A) and transverse (B) sonograms at level of pancreatic head show small pancreas (arrows) with areas of markedly increased echogenicity, resembling echogenicity of retroperitoneal fat (star, A). Scale segment distance, 10 mm. s = superior mesenteric artery, ao = aorta.

View larger version (102K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A —9-year-old boy in good health. Longitudinal (A) and transverse (B) sonograms show normal pancreas (arrows) for comparison with Figures 1A and 1B. Pancreatic echogenicity is equivalent to that of liver. Scale segment distance, 10 mm. Star indicates retroperitoneal fat. ao = aorta, vci = inferior vena cava.

View larger version (108K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B —9-year-old boy in good health. Longitudinal (A) and transverse (B) sonograms show normal pancreas (arrows) for comparison with Figures 1A and 1B. Pancreatic echogenicity is equivalent to that of liver. Scale segment distance, 10 mm. Star indicates retroperitoneal fat. ao = aorta, vci = inferior vena cava.

View larger version (112K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3A —37-year-old man with asymptomatic cystic fibrosis and pancreatic fibrosis. ao = aorta, s = superior mesenteric artery, vci = inferior vena cava. Transverse sonogram at level of pancreatic head (arrow) shows hypoechoic areas representing areas of fibrosis. Small anechoic areas within pancreas represent small cysts. Tiny echogenic foci with acoustic shadowing represent pancreatic calcification. Scale segment distance, 5 mm.

View larger version (124K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3B —37-year-old man with asymptomatic cystic fibrosis and pancreatic fibrosis. ao = aorta, s = superior mesenteric artery, vci = inferior vena cava. Axial contrast-enhanced T1-weighted MR image obtained at same level as A shows intermixed low signal intensity (arrow) representing pancreatic fibrosis.

View larger version (96K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4A —12-year-old boy with asymptomatic cystic fibrosis and pancreatic cystosis. Longitudinal (A) and transverse (B) sonograms at level of pancreatic head show enlarged pancreas with numerous sonolucent lesions (arrows) corresponding to cysts distributed throughout gland. Small amount of echogenic pancreatic tissue is present between cysts. Scale segment distance, 10 mm. ao = aorta.

View larger version (111K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4B —12-year-old boy with asymptomatic cystic fibrosis and pancreatic cystosis. Longitudinal (A) and transverse (B) sonograms at level of pancreatic head show enlarged pancreas with numerous sonolucent lesions (arrows) corresponding to cysts distributed throughout gland. Small amount of echogenic pancreatic tissue is present between cysts. Scale segment distance, 10 mm. ao = aorta.

View larger version (105K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5A —5-year-old girl with cystic fibrosis, hepatic steatosis, and slightly elevated liver enzyme levels. Scale segment distance, 10 mm. Longitudinal (A) and transverse (B) sonograms (3.5-MHz probe) of right lobe of liver show coarse liver parenchyma with diffusely enhanced echogenicity. In comparison, kidney (star) appears hypoechoic. Diminished through-transmission is evident.

View larger version (111K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5B —5-year-old girl with cystic fibrosis, hepatic steatosis, and slightly elevated liver enzyme levels. Scale segment distance, 10 mm. Longitudinal (A) and transverse (B) sonograms (3.5-MHz probe) of right lobe of liver show coarse liver parenchyma with diffusely enhanced echogenicity. In comparison, kidney (star) appears hypoechoic. Diminished through-transmission is evident.

View larger version (120K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5C —5-year-old girl with cystic fibrosis, hepatic steatosis, and slightly elevated liver enzyme levels. Scale segment distance, 10 mm. High-resolution sonogram through right lobe of liver shows poor visualization of portal triads (arrows).

View larger version (101K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6A —15-year-old girl with asymptomatic cystic fibrosis and focal biliary fibrosis. Longitudinal sonogram of left lobe shows coarse heterogeneous hepatic parenchyma and diminished visibility of intrahepatic vessels. Scale segment distance, 10 mm.

View larger version (196K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6B —15-year-old girl with asymptomatic cystic fibrosis and focal biliary fibrosis. High-resolution sonogram of liver shows irregular echotexture and hyperechoic periportal thickening (arrows) due to presence of focal fat.

View larger version (116K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7A —21-year-old man with cystic fibrosis, multilobular biliary cirrhosis, and slightly elevated liver enzyme levels. Longitudinal sonogram of left lobe of liver shows small heterogeneous liver with numerous large, irregular regenerative nodules (stars). Scale segment distance, 10 mm.

View larger version (124K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7B —21-year-old man with cystic fibrosis, multilobular biliary cirrhosis, and slightly elevated liver enzyme levels. High-resolution sonogram of liver surface shows irregular margin with lobulation (arrow) due to scarring. Scale segment distance, 5 mm.

View larger version (116K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8A —23-year-old man with cystic fibrosis, multilobular biliary cirrhosis, and portal hypertension. Longitudinal sonogram of left lobe of liver shows small liver with irregular margin. Ascites (star) next to liver is evident. Scale segment distance, 10 mm.

View larger version (76K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8B —23-year-old man with cystic fibrosis, multilobular biliary cirrhosis, and portal hypertension. Color Doppler sonogram obtained at same level as A shows numerous vessels (arrow) within minor omentum, representing portosystemic collateral vessels. Star indicates ascites.

View larger version (69K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8C —23-year-old man with cystic fibrosis, multilobular biliary cirrhosis, and portal hypertension. Pulsed Doppler image shows hepatofugal flow within varices.

View larger version (106K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8D —23-year-old man with cystic fibrosis, multilobular biliary cirrhosis, and portal hypertension. Longitudinal sonogram shows marked enlargement of spleen, measuring 19 cm in length, indicating portal hypertension.

View larger version (84K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9 —6-year-old boy with asymptomatic microgallbladder. Longitudinal sonogram shows example of biliary abnormality in fasting patient with cystic fibrosis. Small, empty gallbladder (GB) (arrow) is 25 mm long. Scale segment distance, 10 mm.

View larger version (127K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10 —9-year-old boy with sludge (arrow) within small gallbladder. Sonogram shows example of biliary abnormality in fasting patient with cystic fibrosis. Echogenic fat tissue surrounds gallbladder.

View larger version (104K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 11 —16-year-old girl with asymptomatic cholelithiasis. Longitudinal sonogram of gallbladder shows example of biliary abnormality in fasting patient with cystic fibrosis. Microgallbladder containing multiple shadowing echogenic calculi (arrowhead) and mild diffuse thickening of gallbladder wall (arrow) are evident.

View larger version (140K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12A —11-year-old boy with asymptomatic cystic fibrosis and dilatation of intrahepatic bile duct. Transverse high-resolution B-mode (A) and color Doppler (B) sonograms of left lobe of liver show dilated bile duct (arrowhead) beside hepatic artery (star) and portal vein (arrow). Scale segment distance, 5 mm. VP = portal vein, BD = bile duct, AH = hepatic artery.

View larger version (88K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12B —11-year-old boy with asymptomatic cystic fibrosis and dilatation of intrahepatic bile duct. Transverse high-resolution B-mode (A) and color Doppler (B) sonograms of left lobe of liver show dilated bile duct (arrowhead) beside hepatic artery (star) and portal vein (arrow). Scale segment distance, 5 mm. VP = portal vein, BD = bile duct, AH = hepatic artery.

View larger version (116K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13A —1-day-old boy with cystic fibrosis and meconium ileus. Transverse (A) and longitudinal (B) high-resolution sonograms show small descending colon with diminished caliber (microcolon) (arrows) and dilated, meconium-filled small bowel (star). Scale segment distance, 5 mm. s = spleen, k = kidney, sp = spine.

View larger version (119K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13B —1-day-old boy with cystic fibrosis and meconium ileus. Transverse (A) and longitudinal (B) high-resolution sonograms show small descending colon with diminished caliber (microcolon) (arrows) and dilated, meconium-filled small bowel (star). Scale segment distance, 5 mm. s = spleen, k = kidney, sp = spine.

View larger version (124K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 14A —10-month-old girl with cystic fibrosis and distal intestinal obstruction syndrome. Abdominal radiograph obtained with patient in left recumbent position shows right colon filled with fecal mass (star) and multiple air-filled levels within dilated small bowel.

View larger version (92K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 14B —10-month-old girl with cystic fibrosis and distal intestinal obstruction syndrome. Transverse sonogram of right lower quadrant shows dilated bowel loaded with fecal material (star). Scale segment distance, 5 mm

View larger version (122K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 15A —3-year-old girl with asymptomatic cystic fibrosis and small-bowel intussusception. Transverse high-resolution sonogram of left upper quadrant shows multiple concentric ring sign of jejunal intussusception caused by entering and returning limbs of infolded intussusceptum. Star-shaped center (star) represents transverse section of infolded jejunal loop of intussusceptum. Scale segment distance, 5 mm.

View larger version (118K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 15B —3-year-old girl with asymptomatic cystic fibrosis and small-bowel intussusception. Longitudinal sonogram shows short-segment intussusception (length, 10 mm). Entering and returning wall of intussusceptum (arrows) is known as sandwich sign. Spontaneous reduction was found during real-time sonographic examination.

View larger version (121K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 16A —9-year-old girl with asymptomatic cystic fibrosis and mucoid appendix. Transverse (A) and longitudinal (B) high-resolution sonograms at level of right psoas muscle show swollen appendix (diameter, 9 mm) with hypoechoic luminal contents (star) corresponding to inspissated mucoid secretions. Small amount of free fluid is present. Linear echogenic appearance of preserved submucosal layer (arrows, B) and lack of periappendiceal inflammatory changes are evident. Scale segment distance, 5 mm.

View larger version (133K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 16B —9-year-old girl with asymptomatic cystic fibrosis and mucoid appendix. Transverse (A) and longitudinal (B) high-resolution sonograms at level of right psoas muscle show swollen appendix (diameter, 9 mm) with hypoechoic luminal contents (star) corresponding to inspissated mucoid secretions. Small amount of free fluid is present. Linear echogenic appearance of preserved submucosal layer (arrows, B) and lack of periappendiceal inflammatory changes are evident. Scale segment distance, 5 mm.

View larger version (126K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 17A —15-year-old boy with asymptomatic cystic fibrosis and colonic wall thickening. Transverse (A) and longitudinal (B) high-resolution sonograms of ascending colon show fecal content (star) within lumen and pronounced (4.5 mm) nodular thickening (double arrow) of wall with increased thickness of echogenic submucosal layer (arrow). Pericolonic fat proliferation (arrowhead, B) is evident. Scale segment distance, 5 mm.

View larger version (142K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 17B —15-year-old boy with asymptomatic cystic fibrosis and colonic wall thickening. Transverse (A) and longitudinal (B) high-resolution sonograms of ascending colon show fecal content (star) within lumen and pronounced (4.5 mm) nodular thickening (double arrow) of wall with increased thickness of echogenic submucosal layer (arrow). Pericolonic fat proliferation (arrowhead, B) is evident. Scale segment distance, 5 mm.

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