Management Conference Young girl with RUQ mass and pain

1. Management ConferenceYoung girl with RUQ massand pain RaikaJamali MD Digestive Disease Research Center Tehran University of Medical Sciences

2. A 11 years girl with abdominal pain, pruritis and icterus from 3 months ago. • A mass in RUQ with continuous RUQ pain but no obvious radiation or association with eating. • Abdominal pain from 1 year ago without icterus or fever. • Detection of cyst in RUQ not obviously localized by sonography from one month before this admission, suggested as hydatid cyst. • Treatment with Albendazole one month before admission.

3. Physical Exam • A well being girl with no distress. Vital signs were stable. No fever. • Sclera was icteric with mild anemia. • Chest & heart were normal. • There was a mass in RUQ in inspection. • Tenderness in RUQ without morphy sign. • An ill defined mass was detected in RUQ in palpation which does not move with respiration. • It was palpated about 5 cm below right costal margin. • Spleen was normal .No ascitis was detected.

4. LAB DATA • WBC=4500 ( 2% Eos) • Hb=11.1 Ferritin=60 • MCV=75 Serum Iron=115 • MCH=24 TIBC=208 • MCHC=33 • Plt=249000

5. BUN=9 AST=252 • Cr=0.7 ALT=228 • Ca=7.5 ALP=1214 • P=4 Bili direct =3.5 • Na=142 Bili direct =5.5 • K=4 Total protein=6.5 • FBS=108 Albumin=4 • ESR=6PT=13 • TG=62 LDH=505 • Cholesterol=94 Amylase= NL

6. Anti HBs Ag=neg • Anti HBs Ab=neg • Anti HBc Ab=neg • Anti HCV Ab=neg • Anti Ecinococcus granolosus Ab=neg

8. Abdominal Sonography • A SUBHEPATIC MASS MEASURING 140 X100 MM WHICH CAUSED MARKED DILATATION OF INTRA & EXTRA BILIARY DUCTS.

9. Abdominal CT Scan • A regular lobulated cystic mass in right lobe of liver (120 x 120) without calcification causing dilatation of intra & extra biliary ducts.

18. MRCP & MRI • Huge thicked wall cystic mass (140 x120 mm), at the portahepatis that seems connected to biliary tree and gall bladder, resulted in severe dilatation of intra hepatic bile ducts and displaced right kidney posteriorly. • Spleen, kidneys, bowel loops and abdominal wall are normal. • Finding could be due to congenital anomalies like choledocal cyst, Duplication cyst, mesenteric cyst and hydatid cyst are in DDx.

26. Surgery report • There was a mass(10 x 20 cm) in hilum of liver which had compressed the duodenum anteroinferiorly. It was not resectable because of extension and adhesion to the mesocolon & stomach. • Gall bladder was bilobulated. • Stomach ,intestine & uterus were normal. • Cyst was draineged which was full of bile. • Cystodoudenostomy to D2 was done.

30. Pathology • Microscopic Description: Sections show fibrotic cyst wall with columnar epithelial lining, inflammation and ulceration area. • Diagnosis: Choledocal cyst wall, resection: - Cyst wall with fibrosis, hemorrhage, acute & chronic inflammation and ulceration.

31. Biliary cysts • Biliary cysts are cystic dilatations, which may occur singly or in multiples throughout the bile ducts. • They were originally termed choledochal cysts (involving the extrahepatic bile duct) but the clinical classification was revised in 1977 to include intrahepatic cysts.

32. The incidence of biliary cysts has been estimated to be 1:100,000 to 150,000. • The incidence is higher in some Asian countries (Japan) . • The female to male ratio is about 3:1. • In the past, the majority of cases were reported in children, although more recent series report equal numbers in adults and children .

33. Pathologic features of choledochal cysts are variable, ranging from normal bile duct mucosa to carcinoma . • More commonly in children there is a densely fibrotic cyst wall with evidence of chronic and acute inflammation . • In adults, there are frequently inflammatory changes, erosions, sparse distribution of mucin glands, and not infrequently metaplasia .

34. In the case of type III cysts (see below), the cyst is most often lined by duodenal mucosa, and less commonly by bile duct epithelium. • Cysts may be congenital or acquired and have been associated with a variety of anatomic abnormalities. • Familial occurrence of cysts has been described .

35. Congenital biliary cysts may be diagnosed prenatally and can be associated with biliary atresia . • Fetal viral infection may also have a role; reovirus RNA has been isolated from biliary tissue of neonates with infantile biliary obstruction and choledochal cysts. • Cyst formation may be the result of ductal obstruction or distension during the prenatal or neonatal period.

36. Abnormal pancreaticobiliary junction • An abnormal pancreaticobiliary junction (APBJ) is present in about 70 percent of patients with biliary cysts and may be a significant risk factor for the development of malignancy in the biliary cyst . • APBJ is characterized by a long common channel (usually over 2 cm in length) and may represent failure of the embryological ducts to migrate fully into the duodenum. In support of this hypothesis is the observation that the ampulla of Vater is diminutive or flat in patients with APBJ.

37. ERCP demonstrating an anomalous pancreaticobiliary junction in a child with recurrent abdominal pain and pancreatitis. Note the long, dilated common channel (thick arrow) containing a stone (thin arrow). The patient also has pancreas divisum.

38. ERCP in an adult with obstructive jaundice demonstrates an anomalous pancreaticobiliary junction with a malignant biliary stricture replacing the cystic duct insertion. There is no evidence of a biliary cyst.

39. A long common channel may predispose to reflux of pancreatic juice into the biliary tree, since the ductal junction lies outside of the sphincter of Oddi. • This can result in increased amylase levels in bile , intraductal activation of proteolytic enzymes, alterations in bile composition, and theoretically biliary epithelial damage, inflammation, ductal distension, and cyst formation. • Elevated sphincter of Oddi pressures have been documented in APBJ and could promote pancreaticobiliary reflux.

40. APBJ may be usefully subdivided based upon: • the presence of an acute or right angle at the bile duct/pancreatic duct junction, • the presence or absence of a dilated common channel, • and the presence or absence of a dominant dorsal pancreatic duct . • This classification system reflects anatomic issues of importance to the surgeon during cyst resection.

41. Proteinaceous plugs or stones within a dilated common channel ( figure 1) may cause ductal obstruction and pancreatitis, and cyst resection alone may not resolve the patient's symptoms. • Surgical sphincteroplasty or endoscopic sphincterotomy may be required in the treatment of such patients.

42. (IA) common type; (IB) segmental dilatation; (IC) diffuse dilatation; (II) diverticulum; (III) choledochocoele; (IVA) multiple cysts (intra- and extrahepatic); (IVB) multiple cysts (extrahepatic); (V) single or multiple dilatations of the intrahepatic ducts.

43. CLINICAL MANIFESTATIONS Infants: • conjugated hyperbilirubinemia (80 %), • failure to thrive, or an abdominal mass (30 to 60 %). • The triad of pain, jaundice, and abdominal mass is found in (11 to 63 %) .

44. patients older than two: • chronic and intermittent abdominal pain (50 to 96 %). • Intermittent jaundice and recurrent cholangitis (34 to 55%). • Abdominal mass is less common(10 to 20%). • Pancreatitis (20%) • Biliary lithiasis (8%) • Rarely, intraperitoneal rupture, bleeding due to erosion into adjacent vessels, or portal hypertension and cirrhosis .

45. DIAGNOSIS • A diagnosis of biliary cyst should be considered in adults when a dilated portion of the bile ducts or ampulla is identified, especially in the absence of overt obstruction. • A high level of suspicion is required for diagnosis, particularly for Type I cysts, which may go undiagnosed unless considered in the differential diagnosis of patients found to have ductal dilation.

46. Acute or chronic biliary obstruction may cause marked biliary dilation that mimics a Type I cyst. • Such patients usually present with jaundice or markedly elevated serum alkaline phosphatase, have a readily identifiable obstructing lesion such as a stone or stricture, and their biliary dilation often improves after appropriate treatment. • Careful evaluation for APBJ may help with diagnosis in indeterminate cases.

47. Utrasound or CT may suggest the presence of a biliary cyst. • Direct cholangiography (whether intraoperative, percutaneous, or endoscopic) has long been considered the best test for diagnosis and evaluation. • Cholangiography demonstrates areas of cystic dilation, excludes overt obstruction of the bile duct, and delineates the presence of an APBJ. It may also demonstrate stones or malignancy in the cyst.

48. MRCP also appears to be useful for diagnosis. It accurately demonstrates cystically dilated segments of the biliary tree, and identifies APBJ in over 75 percent of cases . • However, MR is less sensitive than direct cholangiography for excluding obstruction.

49. Endoscopic ultrasound (EUS) can also demonstrate extrahepatic biliary cysts and provide detailed images of the cyst wall and pancreaticobiliary junction. • Intraductal ultrasound (IDUS) has been used for diagnosis of early malignant changes in a biliary cyst . • This technique is likely to be more sensitive than direct cholangiography for detection of early malignancy in the cyst wall.

50. CANCER RISK • Biliary cysts are associated with an increased risk of cancer, particularly cholangiocarcinoma. • The incidence of malignancy increases with age. • 0.7 percent in patients under 10 years of age, • 6.8 percent in patients 11 to 20 years of age, • 14.3 percent in patients over 20 years of age • 50 percent has been reported in older patients.