Jaundice in children - Imaging features

Transcription

1 Jaundice in children - Imaging features Poster No.: C-2340 Congress: ECR 2011 Type: Educational Exhibit Authors: M. D. R. Matos, A. T. Ferreira, A. V. Nunes ; Lisboa/PT, Oeiras/PT, /PT Keywords: Ultrasound, Nuclear medicine conventional, MR, Abdomen, Pediatric, Pathology DOI: /ecr2011/C-2340 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 25

2 Learning objectives To present imaging features and algorithms for the most common causes of jaundice in infants and children Background The persistence or new onset of jaundice in infants two weeks or older is no longer physiologic and requires investigation. Causes for cholestasis vary with patient age. In the neonate, bilary atresia, neonatal hepatites syndrome and choledochal cyst are predominant, and in older children hepatocelullar disease is more common. Biliary tract obstruction is less frequently implicated. Radiological investigation helps differentiate extrahepatic from intrahepatic causes. Ultrasound should be performed promptly in the neonate so that biliary atresia can be excluded (fig.1 on page 3), because surgery in that case is performed before 8 weeks of age. Extrahepatic causes, like choledochal cysts, inspissated bile or lithiasis, if excluded, direct the diagnosis towards differentiating biliary atresia from neonatal hepatitis. An HIDA scan is performed when ultrasound directs the diagnosis to biliary atresia (e.g: triangular chord sign, irregular, small gallbladder, dilatation of the hepatic artery); biopsy and operative cholangiography can be performed. The finding of a choledochal cyst may also require cyntigraphy and MR cholangiography to clarify biliary anatomy. Hepatic tumours, though rare, are important causes of jaundice and require CT or MRI. In cirrhosis, imaging reflects the signs of chronic disease Page 2 of 25

3 Fig.: Algorithm for the diagnostic approach of neonatal jaundice References: M. D. R. Matos; Lisboa, PORTUGAL Images for this section: Page 3 of 25

4 Fig. 1: Algorithm for the diagnostic approach of neonatal jaundice Page 4 of 25

5 Imaging findings OR Procedure details NEONATAL JAUNDICE Biliary atresia Rare disease. Incidence of 1:8.000 Single most common cause of neonatal cholestasis Congenital obliteration of the extrahepatic bile duct; the intrahepatic bile ducts are often abnormal and irregular. Three main types: type 3 (occlusion at the level of the porta hepatis with atresia of the whole extrahepatic duct system) accounts for more than 90% of cases, type 2 (atresia of the hepatic duct with residual patency of the right and left hepatic ducts) and type 1 (atresia of the common bile duct -CBD with proximal patent ducts) Gallbladder may be small with clear content No single pre-operative investigation can diagnose biliary atresia with certainty Ultrasound features that point towards the diagnosis: 1- The triangular cord sign (fig.1 on page 10), which represents the fibrotic remnant of the hepatic duct junction and is seen as an echogenic triangular or tubular structure cranial to the bifurcation of the portal vein at the porta hepatis; 2 - An absent or small gallbladder with irregular walls, although a small gallbladder may also be seen in neonatal hepatitis. Change of gallbladder size after a full feed has been used to exclude biliary atresia Perform HIDA (hepatobiliary scintigraphy with technetium iminodiacetic acid 99Tcmc) scan if ultrasound features are suggestive of biliary atresia or the diagnosis cannot be excluded. Nonexcretion of bile into the bowel by 24 h (fig.2 on page 10) MRCP (magnetic resonance cholangiopancreatography) has been used in a few centres to demonstrate the biliary structures and diagnose biliary atresia and to demonstrate the triangular cord sign If a HIDA scan does not exclude biliary atresia percutaneous liver biopsy is necessary Alagille syndrome (arteriohepatic dysplasia) may also present in the neonatal period with jaundice, with simmilar echopattern. histology reveals paucity and dysplasia of intelobular ducts. The presence of congenital anomalies (butterfly vertebra, abnormal facies, e.g.) help the diagnosis Treatment: Kasai portoenterostomy, usually preceded by an operative cholangiogram to confirm the diagnosis. Resolution of jaundice after surgery is more likely if surgery is performed before 8 weeks of age, when larger ductules are present at the porta hepatis Page 5 of 25

6 Neonatal hepatitis It is the main differential to biliary atresia Often there are no specific ultrasound features, although the liver may be enlarged with increased echogenicity Usually the biliary system is unremarkable Severe neonatal hepatitis may also show non-excretion of bile on the HIDA scan and these cases may progress to liver biopsy to exclude biliary atresia. MRCP may play a role in this group in the future Choledochal cyst Cystic malformations of the biliary tree Can be classified into five types (fig.3 on page 11) There is an increased incidence in females and in oriental races. 30% present before 1 year of age. The classic triad of episodic abdominal pain, jaundice and a palpable mass occurs in only 25% of cases The diagnosis is usually made by ultrasound where the cystic dilatation of the choledochus is recognized with or without intrahepatic duct dilatation (fig. 4 on page 12) HIDA may be useful in confirming the biliary origin of the cyst and excluding other diagnoses such as simple liver cyst or duplication cyst If a choledochal cyst is suspected, MRCP is useful to demonstrate the anomalous pancreatobiliary union and clarify anatomy pre-operatively (fig.4 on page 12) Treatment: radical cyst excision and hepatojejunostomy Inspissated bile syndrome It is a rare cause of jaundice in neonates There is extrahepatic obstruction due to sludge or inspissated bile Predisposing factors include prematurity, infection, dehydration, total parenteral nutrition, Frusemide, gastrointestinal dysfunction, cystic fibrosis and haemolysis Ultrasound demonstrates dilatation of the biliary tree with sludge in the bile duct and gallbladder Further imaging is not usually required. Bile plug syndrome may reduce spontaneously or following treatment with ursodeoxycholic acid Persistence or complications such as cholangitis and liver abscess can be treated by percutaneous transhepatic cholangiography (PTC) or cholecystography with irrigation of the biliary tree with contrast and saline Page 6 of 25

7 Spontaneous perforation of the bile duct Occurs at the junction of the cystic and common hepatic duct with no obvious cause Ultrasound shows a complex mass around the bile duct and duodenum, with free intraperitoneal fluid HIDA shows isotope within the peritoneal cavity Treatment is surgical Cholelithiasis Gallstones in infancy are usually asymptomatic and reduce spontaneously. Rarely can impact in the CBD Consist of pigment stones due to immaturity of bile acid production and secretion in association with delayed oral feed, total parenteral nutition (PTN), dehydration, prematurity and diuretics Persistent obstruction and jaundice require PTC and flushing or surgery JAUNDICE IN THE OLDER CHILD Hepatocellular diseases Intrahepatic causes of jaundice have non-specific ultrasound findings, and appearances depend on the severity and stage of the disease Acute hepatitis: normal or enlarged liver with diffuse decrease in parenchymal echogenicity and bright walled portal tracts (due to oedema in the hepatocytes). The gallbladder wall may be diffusely thickened Chronic hepatitis: ultrasound generally shows increased echogenicity of liver parenchyma with a coarsened echotexture. Diagnosis requires a clinical assessment, biochemistry, haematology, radiology, histopathology and microbiology. Liver biopsy may be necessary to confirm the diagnosis Metabolic causes of jaundice include Wilson disease, cystic fibrosis, glycogen storage disease and alfa1-antitripsin disease. US findings are nonspecific, the liver may show increased echogenicity. Glicogenosis may be occur with hepatic adenomas (fig.5 on page 13) Cholelithiasis Cholelithiasis and choledocholithiasis are uncommon causes of jaundice, gallstones have been increasingly recognized in paediatric patients Page 7 of 25

8 Most common in adolescent girls, associated with obesity, pregnancy or the oral contraceptive pill. Other risk factors include TPN, drugs, biliary tract obstruction, cholangitis, haemolytic disease (sickle cell disease, thalasaemia), cystic fibrosis, Crohn's disease or ileal resection, and family history Diagnosis is made by ultrasound (figs.6 on page 13 and 7 on page 14). Usually associated with ductal dilatation when lying within the CBD Spontaneous resolution of gallstones may occur in children, particularly infants, but surgical treatment is recommended in symptomatic children with complicated gallstone disease Sclerosing cholangitis It is a rare cause of chronic progressive liver disease, due to an inflammatory obliterative fibrosis affecting the intrahepatic and extrahepatic biliary tree Typical findings are strictures in multiple segments of the biliary tree with intervening dilated segments The disease may involve both the intrahepatic and extrahepatic ducts Ultrasound and CT (fig.8 on page 14) show irregular dilatation of the bile ducts or thickening of the bile duct walls. MRCP has high specificity but in negative or inconclusive cases endoscopic retrograde cholangiopancreatography (ERCP) or PTC are required Neoplasms Hepatic malignancies account for less than 2% of all paediatric malignancy, yet liver neoplasms are the most common gastrointestinal malignancy in childhood Two thirds of liver masses are malignant Children present with an abdominal mass, but can present with jaundice The most common liver masses in a child under 3 years of age is a hepatoblastoma (fig.9 on page 16) or infantile haemangioendothelioma - figs.10 on page 17, 11 on page 18 and 12 on page 19 (85% occur in infants less than 6 months). In children older than 3 years the most common tumours are hepatocellular carcinoma - figs.13 on page 20, 14 on page 21 and 15 on page 21 (HCC) and undifferentiated embryonal sarcoma Infantile haemangioendothelioma may present clinically as a liver mass in association with congestive cardiac failure or consumptive coagulopathy (Kasabach-Merrit syndrome) and cutaneous haemangioma. Precocious puberty is occasionally associated with hepatoblastoma Alpha feto protein levels are raised in 80-90% hepatoblastomas and 50% of HCC Page 8 of 25

9 Evaluation of a hepatic mass by ultrasound should assess location, extent of disease (hepatoblastoma, HCC and IHE may be solitary or multiple whilst metastatic disease (fig.16 on page 22) is usually multiple) and character of the lesion (cystic or mixed, vascularity, presence of calcification). A cystic lesion is more likely benign and the main diagnosis is cystic mesenchymal hamartoma. Calcification is often present in hepatoblastoma, undifferentiated sarcoma and IHE and less commonly in HCC. A highly vascular lesion with increased flow in the hepatic artery and hepatic veins with tapering of the distal aorta is typical of IHE Vascular invasion or thrombosis points towards malignancy Biliary duct dilatation is suggestive of rhabdomyosarcoma of the biliary tree (fig.17 on page 23) or cholangiocarcinoma Extrahepatic sites of disease suggest metastatic disease If a mass cannot be fully characterized by ultrasound, CT or MR are required On CT pre-contrast hepatoblastoma is hypointense with early enhancement following contrast. HCC may have a central scar Malignant lesions are hypointense relative to normal liver on T1 although occasionally isointense or hyperintense, and hyperintense on T2. They may be heterogeneous due to haemorrhage, necrosis or focal fat Post-contrast images demonstrate diffuse heterogeneous enhancement with early washout IHE on CT and MRI following contrast show early peripheral nodular enhancement and progressive centripeta enhancement on delayed scans IHE generally involute by months of age and can be followed up by ultrasound Pancreatic masses Rare in children Divided in exocrine tumours, endocrine tumours, cystic tumours or mesenchymal tumours. Metastatic disease may also involve this organ Pancreatic masses identified on ultrasound require further evaluation by CT or MRI Cirrhosis Causes include: chronic hepatitis, congenital hepatic fibrosis, biliary atresia, cystic fibrosis, metabolic disease (Wilson's disease, glycogen storage disease, tyrosinaemia, galactosaemia, alpha-1 antitrypsin deficiency), BuddChiari syndrome and TPN. Ultrasound: coarse and nodular liver often with mild right hepatic lobe and medial segment of the left lobe and compensatory hypertrophy of the caudate and left lateral lobe. Secondary signs are ascites and portal hypertension with splenomegaly and colateral circulation. Colour Doppler is essential Page 9 of 25

10 Images for this section: Fig. 1: Biliary atresia - transverse ultrasound images of the liver show the typical "cord sign" Page 10 of 25

11 Fig. 2: 24 hour HIDA scan performed in a neonate with jaundice: radiotracer has not reached the gastro-intestinal system Page 11 of 25

12 Fig. 3: Diagram of the common types of choledochal cyst Page 12 of 25

13 Fig. 4: A type 1 choledochal cyst in a 2-month-old girl. Transverse ultrasound (A) demonstrates a cystic structure in the right upper quadrant. Magnetic resonance cholangiopancreatography (B)demonstrates this abnormality Fig. 5: Glycogen storage disease with hepatocelular adenoma in a teenager with jaundice. Transverse hepatic sonogram shows coarse echostructure of liver with icoechoic and hyperechoic lesions Page 13 of 25

14 Fig. 6: Transverse ultrasound image of the gallbladder shows a large stone with acoustic shadowing Page 14 of 25

15 Fig. 7: Longitudinal ultrasound image shows choledocholithiasis in an 8 year old boy with jaundice Page 15 of 25

16 Fig. 8: Sclerosing cholangitis - CT shows irregular dilatation of the bile ducts Page 16 of 25

17 Fig. 9: Hepatoblastoma - ultrasound transverse and longitudinal images (A) show a large heterogeneous mass with lobulated borders in the dependence low and posterior aspects of the liver. CT scan (B) revealed a soft tissue attenuating mass with coarse calcifications Page 17 of 25

18 Fig. 10: A 7-month-old girl with a liver haemangioendothelioma. B-mode ultrasound image (A) shows a large heterogeneous liver lesion, with increase vascularity on powerdoppler image (B) Page 18 of 25

19 Fig. 11: CT shows typical enhancement pattern with low attenuation lesion pre-contrast (A) with calcifications, and peripheral nodular enhancement and centripetal in-filling on delayed images (B) Page 19 of 25

20 Fig. 12: A 5-month-old girl with multiple haemangioendothelioma. Ultrasound shows multiple hypoechoic lesions (A)and differential in caliper of the abdominal Aorta (B, consistent with this diagnosis Page 20 of 25

21 Fig. 13: Hepatocellular carcinoma in a teenager patient - B-mode (A) ultrasound showed a well demarcated solid mass in the posterior aspect of the liver, which showed significant vascularity on Doppler (B) Fig. 14: Hepatocellular carcinoma in a teenager patient - non-enhanced CT scan (A) shwed an hypodense lesion, with precocious contrast enhancement (B) and early washout (C) Page 21 of 25

22 Fig. 15: Hepatocellular carcinoma in a teenager patient - MRI: axial T1-weihghted image shows a lesion with intermediate signal, wich had high signal in axial (B) and coronal (C) T2-weighted images Page 22 of 25

23 Fig. 16: Lymphomatous involvement of the liver - transverse ultrasound scan shows multiple hypoechoic lesions Page 23 of 25

24 Fig. 17: Rhabdomyosarcoma of the common bili duct in a 8 year-old boy. Transverse ultrasound scan (A) shows a round hypoechoic solid lesion in the region of the pancreatic head. Axial images of helical CT show (A) a soft tissue attenuation mass in the porta hepatis and (B) marked dilatation of the common bile duct Page 24 of 25

25 Conclusion Evaluating the jaundiced patient requires a multidisciplinary approach, in which imaging studies play a determinant role Personal Information References Woodley et al, Imaging of the jaundiced child. Imaging, 16 (2004), Farrant P, Meire HB, Mieli-Vergani G. Improved diagnosis of extrahepatic biliary atresia by high frequency ultrasound of the gallbladder. Br J Radiol 2001;74: Siegel MD, Jaundice in infants and children. Ultrasound clinics Farrant P,et al, Improved diagnosis of extrahepatic biliary atresia by high frequency ultrasound of the gallbladder. Br J Radiol 2001;74:952-4 Helmberger et al, Pediatric liver neoplasms: a radiologic-pathologic correlation. Eur Radiol 1999;9: Page 25 of 25