12/17/2020

Disclosures:

Speaker: Laith H. Jamil, MD, FACG Dr. Jamil has no conflicts of interest related to this talk.

Moderator: Grace H. Elta, MD, MACG Consultant: Olympus Medical

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Choledochal : Recognition and Management Laith H. Jamil, MD, FACG Section Head of Gastroenterology and Hepatology Beaumont Health, Royal Oak, MI Professor of Medicine Oakland University William Beaumont School of Medicine

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Objectives

• Identify the various types of choledochal cysts (CC) • Identify their management options

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Definition of CC

• Congenital anomalies of the that are manifested by cystic dilatation of the extrahepatic and/or intrahepatic ducts • Single or multiple • Also termed Congenital Biliary Dilation, Biliary cysts, or Cysts (BDC) • Bile duct dilation: a maximum diameter of the extrahepatic bile duct was ≥10 mm in adult patients, 5 mm in 10‐year‐old pediatric patients, and 4 mm in 5‐year‐old pediatric patients by radiographic studies

1. O'Neill JA, Jr Choledochal . Current problems in surgery 1992;29:361‐410 2. Altman RP. Choledochal cyst. Seminars in pediatric surgery 1992;1:130‐3.

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Case

• 63‐year‐old male with history of lymphoma presents with Acute • CT scan: lymphadenopathies and an infiltrating mass in the liver • Liver biopsy: moderate to poorly differentiated adenocarcinoma, likely PB or GI primary • Subsequently developed abnormal LFTs

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MRCP

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ERCP/EUS

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Pancreaticobiliary Maljunction (PBM)

• AKA Abnormal Pancreaticobiliary junction (APBJ) • Union of the BD and PD outside the duodenal wall with a long common ductal channel (≥8 mm) • Seen in 10‐58% of CC • CC are seen in 75% of PBM

Funabiki T et al Langenbecks Arch Surg. 2009;394(1):159 Misra SP, Dwivedi M. Gut 1990;31(10):1144–9. Babbitt DP Ann Radiol (Paris).1969;12(3):231‐40 Crittenden SL, McKinley MJ. Am J Gastroenterol 1985;80:643‐647 Kimura K et al Gastroenterology. 1985 Dec;89(6):1258‐65

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Komi Classification

• Type Ⅰ (BD type): A narrowed joins the at a right angle • Type Ⅱ (PD type): A pancreatic duct joins the common bile duct at an acute angle • Type Ⅲ (Complex type): It is complicated by a patent accessory pancreatic duct • A dilated common channel or accessory PD could be the cause of relapsing pancreatitis

Komi N et al Journal of Pediatric Surgery, 1992, 27, 728‐731 Kamisawa T et al J Gastroenterology 47, pages731–759(2012)

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PBM

• Japanese Study – Prospective investigation in asymptomatic individuals undergoing medical checkups – Overall incidence 0.03% (9/27,076 subjects) – 23% of cases with bile duct dilatation – 2.9% with gallbladder wall thickening

Yamao K et al Hepatogastroenterology. 1996;43(11):1238

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Pathophysiology of PBM

Kamisawa T et al J Gastroenterol. 2012 Jul;47(7):731‐59

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PBM and Benign Disorders

• Gall Stones – 17.9 % of patients with CC (more in the bile duct) – 27.3 % of patients without biliary dilatation (more in the GB) – Adults > Pediatrics – Bilirubinate gallstones most frequent • Chronic Pancreatitis: 5‐8 %

Kamisawa T et al J Gastroenterol. 2012 Jul;47(7):731‐59

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PBM and Cancer

• Carcinogenesis explained by reflux and stasis of bile mixed with pancreatic juice in the bile duct and GB • Significantly more K‐ras mutations and p53 overexpression possibly due to biliopancreatic reflux • The sequence of hyperplasia–dysplasia–carcinoma is regarded as the prevailing mechanism

Kamisawa T et al J Gastroenterol. 2012 Jul;47(7):731‐59

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PBM and Cancer

• PBM without CC: High incidence of GB cancer (36%); Strongly consider – Prophylactic cholecystectomy – Extrahepatic bile duct resection/cholecystectomy • PBM with CC: Increased incidence of and GB cancer

Funabiki T et al Langenbecks Arch Surg. 2009;394(1):159. Hidaka E et al Cancer Res. 2000;60(3):522 Sugiyama M, Atomi Y. Br J Surg. 1998;85(7):911 Song HK et al Korean J Intern Med. 1999;14(2):1 Kimura K et al Gastroenterology. 1985 Dec;89(6):1258‐65

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PBM and Gall Bladder Cancer

• 96 patients with GB cancer and 65 patients with PBM (15 mm) were studied – PBM incidence: 16.7% in GB cancer, 2.8% among 641 consecutive patients without GB cancer – GB cancer incidence: • 1.9% among 635 patients with normal PBJ • 24.6% in patients with PBM (16 patients) – 10% (5/50) with CC – 73.3% (11/15) without CC

Kimura K et al Gastroenterology. 1985 Dec;89(6):1258‐65

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PBM and Biliary Cancers

The Japanese Study Group on PBM 1627 patients with PBM over 10 years (600 adults)

Adults and Pediatrics With CC (n=1239) Without CC (n=388)

Cancer of the biliary tract 10.6% 37.9%

GB vs Extrahepatic BD cancer 64.9 % vs 33.6 % 93.2 % vs 6.8 %

Tashiro S et al J Hepatobiliary Pancreat Surg 2003;10(5):345‐51

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PBM and Biliary Cancers

The Japanese Study Group on PBM 2561 patients with PBM over 18 years Adults 1511, pediatrics 1018 (32 excluded) One pediatric case of bile duct cancer (PBM with CC)

Adult patients (n=1511) With CC (n=977) Without CC (n=514) Bile duct cancer 6.9% 3.1% GB cancer 13.4% 37.4%

Morine Y et al J Hepatobiliary Pancreat Sci (2013) 20:472–480

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PBM and Pancreatic Cancer

• Unknown • Incidence of pancreatic cancer in Japan – General population: 0.0162 % – Patients with PBM: 0.8% (49.4‐fold increase)

Kamisawa T et al J Gastroenterol 2012 Jul;47(7):731‐59

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Recommendations for Patients with PBM

• Once the diagnosis of PBM is established, immediate prophylactic surgery is recommended • However, the surgical strategy for PBM without biliary dilatation remains controversial – Prophylactic cholecystectomy – Extrahepatic bile duct resection/cholecystectomy • To detect PBM without biliary dilatation early, MRCP is recommended for patients showing gallbladder wall thickening on screening US under suspicion of PBM

Kamisawa T et al J Gastroenterol 2012 Jul;47(7):731‐59

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Classification of Choledochal Cysts (CC) According to Todani and Colleagues

• I (IA) common type; (IB) segmental dilatation; (IC) diffuse dilatation • II Diverticulum • III Choledochocele • IV Multiple cysts; (IV‐A) intra‐ and extrahepatic, (IV‐B) extrahepatic • V Single or multiple dilatations of the intrahepatic ducts (Caroli’s disease)

Todani T et al J Hepatobiliary Pancreat Surg (2003) 10:340–344

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Epidemiology

• Incidence – Asia: 1:1000 – North America is 1:150,000 live births • F:M distribution: 3:1 to 4:1 • Age distribution: Children>>>adults (20%)

O’Neill JA Jr.. Curr Probl Surg 1992;29:361‐410 K. Wiseman et al Am J Surg, 189 (2005), pp. 527–531 Baison GN et al World J Gastroenterol 2019 Jul 14;25(26):3334‐3343

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Biliary Cyst Presentation

• Incidental: Imaging, prenatal ultrasound, or endoscopy • Neonates: cholestatic , acholic stools, vomiting, irritability, failure to thrive, , • Children and adults: triad of abdominal pain, jaundice, and a palpable mass (<20%) • Abdominal pain > adults, Jaundice >children • Older patients: >Pancreatitis and recurrent cholangitis • The male‐to‐female ratio is approximately 1:3, and it is especially predominant in young women

Todani T, et al. European journal of pediatric surgery 1995;5:143‐5 Shah OJ, et al. World journal of surgery 2009;33:2403‐11 Edil BH, et al. Journal of the American College of Surgeons 2008;206:1000‐5; discussion 5‐8

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Work Up

• LFTs often normal unless complication e.g. cholangitis, pancreatitis, stone, stricture, tumor, etc. • Cross‐sectional imaging (MRI/MRCP preferred) – Confirm cyst/type – Assess if cyst communicates with the biliary tree – Associated mass? – Assess PBJ • High index of suspicion: Dilated bile duct or cystic liver lesion(s)

Baison GN et al World J Gastroenterol 2019 Jul 14;25(26):3334‐3343

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MRCP vs ERCP for Choledochal Cysts

• 8‐year, 72 patients, retrospective study • All patients underwent both MRCP and ERCP • MRCP findings were compared with those of ERCP as the criterion standard • Overall detection rate of MRCP for – Choledochal cysts: 96% (69/72) – Ductal anomalies (57%): sensitivity (83%), specificity (90%), accuracy (86%) – Choledocholithiasis: 100% (8/8) – Concurrent Cholangiocarcinoma: 87% (13/15)

Park DH et al GIE 2005;62(3):360

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MRCP vs ERCP for Choledochal Cysts

Type (# of patients) Sensitivity Specificity PPV NPV

Type I (31) 81 90 86 86

Type III (11) 73 100 100 95

Type IV a (24) 83 90 80 91

Type IV b (1) 100 100 100 100

Type V (5) 100 100 100 100

Park DH et al GIE 2005;62(3):360

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Risk of Malignancy

• 20‐30‐fold ↑ risk of Cholangiocarcinoma – Type I and type IV cysts show a higher cancer incidence, even after cyst excision • Gall bladder • ??? Pancreatic Cancer • Incidence: 10–30% of adults (mean age 32) • Overall risk of cancer 10‐15% – 0.7 % < 10 years of age – 7 % 11 ‐ 20 years of age – 14 % >20 years of age – As high as 50 % in older patients

Søreide K, Søreide JA Ann Surg Oncol. 2007;14(3):1200 Voyles CR et al Arch Surg. 1983;118(8):986 Todani T et al Surg Gynecol Obstet. 1987;164(1):61 Baison GN et al World J Gastroenterol. Jul 14, 2019; 25(26): 3334‐3343

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CC And Associated Malignant Tumors In Adults

• 808 patients ≥ 18 years who underwent surgery for CC over 17 years in S Korea • Biliary tract malignant tumor was associated in 80 patients (9.9%); – bile duct cancer (40 Pts, 50.0%), – gallbladder cancer (35 Pts, 43.75%), – periampullary cancer (3 Pts, 3.75%) – synchronous gallbladder and bile duct cancer (2 Pts, 2.5%)

Lee SE et al Arch Surg. 2011 Oct;146(10):1178‐84

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Factors Predicting Malignancy

• Univariate Analysis; – > 40 years of age – absence of a gallstone – ↑CEA or CA 19‐9 serum level – presence of PBM • Mulvariate analysis; ↑CA 19‐9 level

Lee SE et al Arch Surg. 2011 Oct;146(10):1178‐84

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Type of CC and Malignancy

• Type I 68% • Type II 5% • Type III 1.6% • Type IV 21% • Type V 6%

Todani T et al Tan to Sui (Biliary Tract Pancreas). 1985;6:525‐535 Baison GN et al World J Gastroenterol. Jul 14, 2019; 25(26): 3334‐3343

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Non‐Malignant Complications

• Stone formation: Cystolithiasis, cholelithiasis, choledocholithiasis, hepatolithiasis • Cholangitis • Secondary biliary • Pancreatitis

Singham J et al Can J Surg. 2009 Dec;52(6):506‐11

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Unusual Non‐Malignant Complications

• Intraperitoneal cyst rupture • Bleeding • Gastric outlet obstruction • Intussusception

Tani C et al Pediatr Radiol. 2009 Jun;39(6):629‐31 Can MF et al Turk J Gastroenterol. 2006 Mar;17(1):70‐3 Kirimlioglu V et al Dig Dis Sci. 2000 Mar;45(3):544‐8 Lakhtakia S et al Gastrointest Endosc. 2008 Dec;68(6):1194‐5

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Case

• 23 year old female • Intermittent RUQ, epigastric and lower quadrant pain, plus nausea of 4 months duration • MRCP CBD

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Case

• 61 year old female presents to an OSH with AP • Imaging showed distal biliary stricture with upstream dilation of 3.5 cm. LFTs elevated • Working Dx: distal CBD stricture • ERCP performed with stent placement • 1 week later worsening pain • EUS: No pancreatic mass, normal distal CBD

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MRCP

Courtesy of Kapil Gupta, M.D

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Case

• 12 year old female presents with abdominal pain • Imaging: acute appendicitis and focal dilation of the bile duct (17 mm) • MRCP

Courtesy of Philip K. Frykman, MD

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Type 1 Choledochal Cysts

• Most common type – 65%‐84% in Eastern cohorts – 67%‐73% in Western cohorts • Has no intrahepatic component • The common hepatic duct proximal to the cyst is usually normal

Baison GN et al World J Gastroenterol. Jul 14, 2019; 25(26): 3334‐3343 Todani T et al J Hepatobiliary Pancreat Surg. 2003;10(5):340

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Type Ia

.Cystic dilation involves the CBD, part or all of the CHD and extrahepatic portions of the L&R HD .Associated with a PBM

Todani T et al J Hepatobiliary Pancreat Surg. 2003;10(5):340

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Type Ib

.Focal, segmental dilation of an extrahepatic bile duct (often the distal CBD) .Not associated with a PBM

Todani T et al J Hepatobiliary Pancreat Surg. 2003;10(5):340

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Type Ic

.Smooth, fusiform, diffuse, or cylindrical dilation, usually from the PBM to the extrahepatic portions of the L&R HD .Associated with a PBM

Todani T et al J Hepatobiliary Pancreat Surg. 2003;10(5):340

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Cancer in Type 1

• European study, 350 patients with CC, Pancreaticobiliary junction (PBJ) was analyzable in 263 – 190 (72.2 %) had PBM. Similar rate in Types I and IVa • 131 patients with Type 1 CC, 73% with PBM • Incidence of synchronous cancer 9.1% (12/131) – With PBM 10.5% (10/96) – Without PBM 5.7% (2/35)

Ragot E et al World J Surg. 2017;41(2):538

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Management

• Obstrucve lesion ?→ EUS &/or ERCP • Distal Margin: – Preop: MRCP, EUS, &/or ERCP – Intraop: Cholangiography, choledochoscopy

Jabłońska B World J Gastroenterol. 2012 Sep;18(35):4801‐10

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Surgical Management

• Complete excision of the cyst: from the bifurcation of lobar HDs into the parenchyma of the pancreas near the junction of the PD • Coupled with cholecystectomy • Biliary tract continuity – Roux‐en‐Y hepaticojejunostomy (HJ) – Hepaticoduodenostomy (HD) – Jejunal interposition HD

Baison GN et al World J Gastroenterol 2019 Jul 14;25(26):3334‐3343

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Type II

• True choledochal diverticula arising from the extrahepatic duct • Communicate with the bile duct through a narrow stalk • 2% of biliary cysts

Jabłońska B World J Gastroenterol. 2012 Sep;18(35):4801‐10

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Management

• DDX: pancreatic, mesenteric, and hepatic cysts • If in doubt about Dx → hepatobiliary scintigraphy or ERCP • Treatment: Surgical excision of the cyst

Lie DC et al J Pediatr Surg 2000;35:1117‐9 Lipsett PA et al J Hepatobiliary Pancreat Surg 2003;10(5):352‐9

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Case

• 87‐year‐old female presents with epigastric pain • Abdominal US: 14mm CBD, GB sludge/stones • LFT: ALP 436, AST 218, ALT 287, T bili 3.4. WBC 12.2

• MRCP GB

CBD PBJ

PD

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ERCP

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Type III (AKA Choledochocele)

• Cystic dilations are limited to the intra‐duodenal portion of the distal CBD • 1‐5% of all CC • Average age at presentation: 51 years • Lining: duodenal epithelium or biliary epithelium (increased risk of malignancy) • Several subtypes • Incidence of malignancy: 2.5% in symptomatic choledochocele

Todani T et al J Hepatobiliary Pancreat Surg. 2003;10(5):340 Law R, Topazian M CGH 2014;12:196‐203

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Most Common Types

• Type A: The intramural bile duct opens into a cystically dilated segment, which communicates to the duodenal lumen via a separate orifice • Type B: The bile duct that opens normally into the duodenal lumen, with the choledochocele arising as a diverticulum of the intra‐ampullary common channel

Law R, Topazian M CGH 2014;12:196‐203 O’Neill JA Jr. Curr Probl Surg 1992;29(6):374

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Duodenal Duplication Cysts

• Cystic structures typically found in D2 and D3 • Intimately attached to the duodenal wall • They have a muscle coat • Almost 50% of duodenal duplications communicate with the periampullary ducts, often to the pancreatic duct, and at times via an aberrant duct

Law R, Topazian M CGH 2014;12:196‐203

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Management

• Symptomatic: Treat • Asymptomatic: – Type IIIA: Endoscopic sphincterotomy (followed by biopsy of the cyst epithelium), and/or endoscopic snare resection – Type IIIB: Surgical or endoscopic resection via polypectomy snare • Follow up: Endoscopic biopsies of the cyst mucosa a year later (assess for dysplasia)

Law R, Topazian M CGH 2014;12:196‐203

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Case

• 65‐year‐old Korean male post cholecystectomy presented with RUQ pain and fever • LFTs normal except – AP 713 U/L (n≤120) • WBC 15.5 • MRCP: Liver abscess

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2 Years Later

• Progression of intrahepatic and extrahepatic bile duct dilation • LFTs Normal except – AP 183 U/L (n ≤120) – ALT 50 U/L (n≤47)

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Type IV

• Multiple cysts • 15‐35% of CC • Type A: Both intrahepatic and extrahepatic cystic dilations Associated with PBM • Type B: Multiple extrahepatic cysts only • Malignancy Risk: 21%

Todani T, et al Journal of hepato‐biliary‐pancreatic surgery 2003;10:340‐4 Baison GN et al WJG 2019; 25(26): 3334‐3343

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Type IV and PBM

• European prevalence: IVa 36/52 (69.2%), IVb 4/5 (80%) • Incidence of biliary cancer among patients with CC IV with PBM was 25 % (1 out of 4 patients)

Ragot E et al World Journal of Surgery volume 41, pages538–545(2017)

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Management

• Surgical excision and creating a Roux‐en‐Y hepaticojejunostomy • If previously managed by a cystenterostomy for symptomac relieve →surgery (30% post cystenterostomy risk of malignancy)

Jabłońska B. World J Gastroenterol. 2012 Sep 21;18(35):4801‐10 Singham J et al Can J Surg. 2010 Feb;53(1):51‐6

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Symptomatic Residual Intrahepatic Cysts Type IV A

• If patient becomes symptomatic from residual intrahepatic cysts – Segmental hepatectomy – Surgical un‐roofing – Liver transplantation in some cases

Yamada T et al J Nippon Med Sch. 2009 Apr;76(2):103‐8

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Timing in Neonates

• Unclear • Early surgical intervention if – Progressive intrahepatic ductal dilation – Cyst enlargement – Deterioration of liver function • Harbingers of obstruction and/or cholangitis • External drainage procedures (as a bridge) – Nutritional compromise – Acute infection

Lugo‐Vicente HL J Pediatr Surg.1995;30 :1288– 1290 Marchildon MB Pediatr Surg Int.1988;3 :431– 436 Clifton MS et al Pediatrics 2006; 117: e596‐e600

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Timing of Surgery

• Three patient groups that had been operated: – < 1 year old (group I, n = 26) significantly fewer surgical complications and less severe liver fibrosis – 1‐16 years old (group II, n = 48) – > 16 years old (group III, n = 33)

Tsai MS, et al J Gastrointest Surg. 2008;12:2191‐2195

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Surgical Outcome

• Postoperative morbidity (2.5%‐27%) • Mortality (0%‐6%) • Late AEs: – Most common: Biliary‐enteric anastomosis stricture – Less common: peptic ulcer disease, cholangitis, biliary and intrahepatic stones, pancreatitis, liver failure and biliary cancer

Jabłońska B World J Gastroenterol. Sep 21, 2012; 18(35): 4801‐4810

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Patient with Previous Cystenterostomy

• If previously managed by a cystenterostomy →surgery (30‐50% post cystenterostomy risk of malignancy) • Japanese study: 3/8 patients developed 2nd carcinoma – mean age at detection of carcinoma 35 years, 15 years less than that of primary carcinoma – The mean interval between drainage and detection of carcinoma was 10 years • European Study: 8% (27/350) CC patients developed cancer – Previous enterocystic derivation was present in 6/27 (22%) patients with cancer – The median age of patients with cancer after cystenterostomy was 43.1 years (range 25– 60 years)

Todano T et al Surg Gynecol Obstet 1987 Jan;164(1):61‐4. Watanabe Y et al J Hepatobiliary Pancreat Surg 1999;6(3):207‐12 Ragot E et al World Journal of Surgery volume 41, pages538–545(2017)

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Bile Duct Cancer Developed After Cyst Excision For Choledochal Cyst

• 23 patients with cholangiocarcinoma after excision of CC • Incidence: 0. 7% • Nearly half of the 23 patients in the literature had undergone inadequate cyst excision • Intervals between cyst excision and cancer detection: 1‐19 years (average, 9.0 +/‐ 5.5 years) • Sites of cancer development: intrahepatic (6); anastomotic (8); hepatic side residual cyst (3); and the intrapancreatic duct (6) • All patients with CC also require life‐long follow‐up for cancer, usually via serial ultrasonography and monitoring of liver enzymes

Watanabe Y et al J Hepatobiliary Pancreat Surg 1999;6(3):207‐12 Singham J et al Can J Surg. 2010;53:51‐56 61

Follow Up

• No clear guidelines • Post‐excisional malignant disease: 0.7%‐6 % • Type III cysts treated with endoscopic sphincterotomy: Endoscopic biopsies of the cyst mucosa a year later • Frequency of post total cyst excision (6‐12 months?): – LFTs to screen for anastomotic biliary stenosis (25%) – CEA and CA 19‐9 ? – Abd US? • 33.3% risk of malignancy in patients with incomplete cyst resection compared with 6% after complete cyst resection

Watanabe Y et al J Hepatobiliary Pancreat Surg. 1999;6(3):207 Zheng X et al J Pediatr Surg. 2013;48(10):2061 Liu YB et al Chin Med J (Engl). 2007;120:1404‐1407 Lee SE et al Arch Surg. 2011 Oct;146(10):1178‐84 62

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Non‐Surgical Management Type I, II, or IV

• Periodic imaging: Unproven value • Can consider yearly imaging studies (MRI/MRCP, CT with contrast, or IDUS), particularly if findings will alter patient management • Patient who refuse surgical resection/poor surgical candidates – Laparoscopic cholecystectomy – Endoscopic sphincterotomy – Endoscopic stent placement

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Case

• 84‐year‐old male • Presents with flank pain and hematuria • Imaging showed kidney cysts and liver cyst • MRI/MRCP

Courtesy of Ashley Wachsman, M.D.

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Case

• 30‐year‐old male with presumed cirrhosis secondary to congenital hepatic fibrosis • Presents with abdominal pain, fever and mild abnormalities in LFTs (TB 1.7, AP 155)

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Type V

• Single or multiple intrahepatic cystic dilations alone (no associated strictures) • 20% of these cysts • – Less common – Without other apparent hepatic abnormalities – Usually presents with recurrent cholangitis • Caroli syndrome – More common – Associated with congenital hepatic fibrosis

Todani T, et al Journal of hepato‐biliary‐pancreatic surgery 2003;10:340‐4

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Caroli’s

• Autosomal recessive (reports of autosomal dominant inheritance) • Associated with autosomal recessive polycystic kidney disease (ARPKD) • Non‐obstrucve Saccular or fusiform dilataon of bile ducts → stagnaon of bile → biliary sludge and intraductal lithiasis • Bacterial cholangis →sepcemia and hepac abscess formaon • Secondary biliary cirrhosis can occur due to biliary obstruction

Summerfield JA et al J Hepatol. 1986;2(2):141. Desmet VJ Histopathology. 1992;20(6):465 Ward CJ et al Nat Genet. 2002;30(3):259

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Caroli’s

• Incidence: 1 in 1 000 000 population • Males and females are equally affected • > 80% of patients present before age 30 • Caroli’s syndrome patients can present with and its sequelae

Giovanardi RO et al Hepatogastroenterology. 2003;50:2185–2187 Lu SC et al Textbook of Gastroenterology. Philadelphia: Lippincott Williams and Wilkins; 2003. pp. 2225–2233

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Diagnosis

• Imaging (MRCP) • Rarely need liver biopsy • Central dot sign: The fibrovascular bundles containing portal vein radical and a branch of hepatic artery bridging the saccule

Dağli U et al Eur J Gastroenterol Hepatol. 1998 Feb;10(2):109‐12. CE Clinical Endoscopy Clin Endosc 2234‐2400 2234‐2443 Korean Society of Gastrointestinal

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DDX

• PSC: ductal dilation rarely saccular, tends to be more isolated and fusiform, IBD association • Recurrent pyogenic cholangitis: Saccular dilation unusual • Polycystic : cysts rarely communicate with the bile ducts

Yonem O, Bayraktar Y World J Gastroenterol 2007 Apr 7; 13(13): 1930–1933

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Management

• Largely supportive (antibiotics, ERCP, cholangioscopy, EHL, ESWL) • Hepatic resection: Disease limited to a single lobe • Liver transplantation: Extensive disease and frequent complications

Caroli‐Bosc FX et al Endoscopy. 1998 Aug;30(6):559‐63 Okugawa T et al Gastrointest Endosc. 2002 Sep;56(3):366‐71 Mabrut JY et al Ann Surg. 2013 Nov;258(5):713‐21

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Ursodeoxycholic acid in Caroli’s Syndrome

• 12 patients with Caroli's syndrome and intrahepatic stones • The duodenal bile of these patients contained cholesterol crystals • Ursodeoxycholic acid 10‐20 mg/kg daily • Results after 48 (range 12‐114) months – sustained clinical remission – return to normal liver function – dissolution of intrahepatic stones on ultrasound in all patients (9 partial, 3 complete)

Ros E et al Lancet. 1993 Aug 14;342(8868):404‐6

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Take Home Points

• High index of suspicious in unexplained cystic dilations of the biliary tree • High risk for malignancy • Paents with PBM are at increased risk for GB cancer → strongly consider Cholecystectomy • Cancer screening: Not proven

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Take Home Points

• Type I → Surgery

• Type II → Surgery

• Type III → Endoscopic therapy

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Take Home Points

• Type IV → Surgery

• Type V → Supporve/symptomac treatment. May require surgery/liver transplantation

• Consider post surgical FU

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Thank You

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Questions?

Speaker: Laith H. Jamil, MD, FACG

Moderator: Grace H. Elta, MD, MACG

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