choledochalcyst introduction, diagnosis and management.pptx

Choledochal cyst introduction, diagnosis and management

1. Choledochal Cyst Dr. Rohan kumar

2. Introduction Cyst dilatation thoughout the billiary tree B iliary cyst is more appropriate word Approximately 1 in 100,000 - 1 in 150,000 More common in some asian countries(1:1000) More common in women (3-4 times) Frequently diagnosed in infancy or childhood Adult patients more acute presentation Associated with billary cancer 10-30%[ ~ 16%] (particularly cholangioCA-varies on age and type)

3. A natomy

4. classification Todani classification refined in 2003 to incorporate the presence of APBJ

5. Classification Type I - Cystic or fusiform dilatation of the Extra hepatic bile ducts A - Cystic dilatation with ABP B - Focal or segmental dilatation without ABPJ C - Smooth , fusiform, diffuse or cylindrical dilatation associated with an APBJ D - Dilatation of cystic duct +CBD +CHD

6. Type II - Diverticulum of the CBD 60% between CHD and biliary bifurcation 20% suprapancreatic CBD 20% intrapancreatic CBD Type III - Cystic dilatation limited in intraduodenal portion of distal CBD More likely encountered incidentally often seen with pancreatitis managed primary with endoscop y

7. Type IV - Multiple cystic dilatation of extra (IV B) + intrahepatic bile ducts(IVA)( Left lobe predominantly involved) Type V - Cystic dilatation of the intrahepatic bile ducts with normal extrahepatic bile duct (referred to as Caroli disease) Type V +congenital hepatic fibrosis= Caroli syndrome Type VI - Isolated dilation of cystic duct (rare)

8. TYPE PREVALANCE I 50-90% (IA M/C) II 2-3% III 1-6% IV 15-35% V 20% VI Rare

9. PATHOGENESIS may be congenital or acquired Congenital cysts may result from an unequal proliferation of embryologic biliary epithelial cells before bile duct cannulation is complete. Fetal viral infection may also have a role cyst formation may be the result of ductal obstruction or distension during the prenatal or neonatal period. Associated with various deve lopmental anomalies

10. Acquired cysts may be the result of an abnormal pancreaticobiliary junction (APBJ). APBJ is a rare congenital anomaly, with a prevalence of 0.03% Pancreatic and bile duct junction lies outs ide of the duodenal wall and sphincter of oddi reflux of pancreatic juices into bilia ry system activation of pancreatic enzymes inflammatory response deteri o ratio n of ductal wall ductal wall dilation and cyst formation

11. It is present in about 70 (50-80) percent of patients with biliary cysts and may be a significant risk factor for malignancy with the cyst. In addition, patients with APBJ without biliary cysts appear to be at a markedly increased risk for gallbladder cancer Diagnostic criteria length of common channel>8mm intraductal amylase level >8000IU/L Associate with type IA,IC AND IV

12. Abnormal pancreaticobiliary junction

13. Clinical Presentation Jaundice Pain in RUQ Abdominal mass Classical triad + only in 25% At least 2 features present in 85%

14. CLINICAL MANIFESTATIONS   The majority of patients with biliary cysts will present before the age of 10 years. 25% detected by 1 year 35-55% by 10 year 2 5 % present in adulthood

15. Infants with biliary cysts commonly present with conjugated hyperbilirubinemia (80 percent), failure to thrive an abdominal mass (30 to 60 percent). The triad of pain, jaundice, and an abdominal mass is found in 11 to 63 percent of infants.

16. In contrast, in patients older than two,the most common present ation is Chronic abdominal pain(50 to 96 %) recurrent cholangitis (34 to 55 %). Abdominal mass (10 to 20 %) Pancreatitis (20 %) Biliary lithiasis (8 %)

17. Rarely, biliary cysts present with intraperitoneal rupture bleeding due to erosion into adjacent vessels portal hypertension secondary biliary cirrhosis due to prolonged biliary obstruction and recurrent cholangitis. In addition, type III cysts can case gastric outlet obstruction due to the obstruction of the duodenal lumen or intussusception.

18. Associated hepatobilliary pathology Cystolithiasis (most common) Hepatolithiasis C alculous cholecystitis Pancreatitis Cholangiocarcinoma Intrahepatic abscess Cirrhosis with or without portal hypertension

19. Investigation Transabdominal ultrasound Computed tomography CT cholangiogarphy Endoscopic ultrasound Intraductal ultrasound Endoscopic retrograde cholangiopancreatography Magnetic resonance cholangiopancreatography

20. Transabdominal ultrasound First imaging modality used for the evaluation Not detect type III and type V cysts. sensitivity of 71 to 97 % Factors that may limit the usefulness of an ultrasound include the patient's body habitus, the presence of bowel gas, and limited visualization due to overlying structures.

21. Computed tomography CT can detect all types of biliary cysts. C T lead to significant increase in incidental detection Can evaluate for the presence of malignancy. It is also useful for determining the extent of intrahepatic disease in patients with type IVA or V cysts. Significant limitations for detailed biliary anatomy Utility repaced by MRCP

22. CT cholangiography has high sensitivities for visualizing the biliary tree (93%), biliary cysts (90%), intraductal stones (93%) However, its sensitivity is lower for imaging the pancreatic duct (64 %)

23. CT cholangiography

24. Endoscopic ultrasound (EUS) EUS can demonstrate extrahepatic biliary cysts and provide detailed images of the cyst wall and pancreaticobiliary junction. unlike transabdominal U/S, it is not limited by body habitus, bowel gas, or overlying structures.

25. Endoscopic ultrasound

26. Intraductal ultrasound (IDUS) used for the diagnosis of early malignant changes in a biliary cyst . more sensitive than direct cholangiography for detecting early malignancy in the cyst wall.

27. Intraductal ultrasound (IDUS)

28. Hepatobiliary scintigraphy using radio-labeled dyes : technetium-99m-labeled hepatic iminodiacetic acid (HIDA), which is selectively taken-up by hepatocytes and excreted into the bile. HIDA scanning is useful for extrahepatic cysts, with a sensitivity up to 100% for type I cysts. I t is inadequate at visualizing the intrahepatic bile ducts HIDA scanning may also be useful in cases of cyst rupture

29. HIDA SCAN

30. Cholangiography   Direct cholangiography (whether intraoperative, percutaneous, or endoscopic) has a sensitivity of up to 100 percent for diagnosing biliary cysts previously was a commonly obtained test. PTC most advantageous for Prior roux-en y HJ, cystojejunostomy P atients with type IV cyst with stricture or tumour PBD may be performed after PTC can identify abnormal pancreatobiliary junction, and filling defects due to stones or malignancy. Increase risk of cholangitis and pancreatitis. [ Patients with cystic disease are greater risk for these complications ]

31. Radiology

32. Magnetic resonance cholangiopancreatography [MRCP] Does not have the risks of cholangitis and pancreatitis as direct cholangiography Sensitivity 73 - 100 %. less sensitive than direct cholangiography for excluding obstruction. The data are variable with regard to its ability to diagnose an abnormal pancreatobiliary junction. [46-75%]

33. MRCP

34. Management Based on cyst type and associated hepatobiliary pathology  Aims of preoperative management Complete cholangiographic definition of extent of cystic process and duct pathology Control of biliary infections Careful review of preoperative imaging to plan for aberrant biliovascular anatomy

35. In the past, some patients were treated with internal drainage via a cystenterostomy Risk of future malignancy remains high in this group (18.4% v/s 1.9% with cyst excision) development of complications including cholangitis, hepatolithiasis, pancraetitis can be as high as 70%

36. Because of these complications, patients requiring treatment now generally undergo cyst excision with hepaticoenterostomy . In patients with ascending cholangitis require treatment with antibiotics and drainage. Drainage can often be obtained via ERCP or percutaneous transhepatic cholangiography.

37. Reduction in malignancy is based on the potential carcinogenic effect of pancreatic secretions is eliminated because of total diversion from the biliary tract the production of mutagenic secondary bile acids is reduced because bacterial overgrowth in the bile is less frequent abnormal cyst epithelium is excised.

38. Treatment For types I, II, and IV – Excision of the extrahepatic biliary tree - including cholecystectomy, with a Roux-en-Y hepaticojejunostomy are ideal. In some difficult case, some surgeons advocate leaving posterior cyst wall intact with mucosectomy Resection of intrapancreatic portion is important can predispose to malig nacy or stones In type IV, additional segmental resection of the liver may be appropriate

39. For type III Preoperative deleniation of distal CBD and pancreatic duct in relation to cyst is critical Principal g oal is maintaining normal outflow of biliary and pancreatic ducts Minimizing future risk of malignanc y Choice of procedure based on age, comorb idity profile, presence of symptoms, Cyst subtype, size of lesion  

40. Type IIIA >> Endoscopic sphincherotomy Type IIIB>> Endoscopic or surgical resection (can offen be managed with endoscopic sphincterotomy or endoscopic resection) Lobeck and colleagues developed more aggressive algorithm based on cyst size Lesions <3cm Intestinal epithelium- Endoscopic sphincterotomy Biliary epithi lium- Local resection(open/endoscopic) and surveillance Cyst>3cm Intestinal epithelium- Cyst excision Biliary epithi lium- Cyst excision with surveillance v/s Pancreaticoduoenectomy

41. For type V — Depend on Distribution of intrahepatic cysts Presence of hepatic fibrosis S econdary biliary cirrhosis Carcinoma Supportive non operat ive mangement medical tretment of recurrent cholangitis and sepsis Stone extra ction when possible

42. Approx 80% have unilobar disease Partial hepatectomy procedure of choice As with type IVA cysts, some patients with type V cysts will eventually require liver transplantation.

43. Alternatives to surgery   In patients who refuse surgical resection or who are poor surgical candidates, lesser interventions (such as LC or ERCP) may treat symptoms caused by gallstones or sludge. No proven effective method of screening biliary cysts for dysplasia or intramucosal cancer. If screening is attempted, an intraductal ultrasound is probably the most sensitive test for detecting early malignancy in the cyst wall.

44. Hepaticojejunostomy Roux-en-Y

45. Complications Cholangitis Biliary stone formation Anastomotic stricture Residual debris in the intrahepatic bile ducts Intrahepatic bile duct dilatation Malignancy

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