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Hepatopancreatobiliary (HPB) Surgery, Department of Surgery

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Presentation on theme: "Hepatopancreatobiliary (HPB) Surgery, Department of Surgery"— Presentation transcript:

1 Hepatopancreatobiliary (HPB) Surgery, Department of Surgery
GI Symposium 2015: Advances in Hepatobiliary and Pancreatic Diseases Conference February, 2015 Management of Liver Cysts Sandeep Anantha, MD HPB Surgery fellow Hepatopancreatobiliary (HPB) Surgery, Department of Surgery St. John Providence Healthcare System

2 Speaker Disclosures Nothing to disclose

3 Contents Simple Cysts Polycystic Liver Disease
Biliary Cystadenoma and Biliary IPMN Biliary Cystadenocarcinoma Diagnosis Treatment Survival Recurrence

4 Categories of Hepatobiliary Cysts
Congenital: simple cysts, polycystic disease, bile duct cysts (type IV,V), bile duct hamartomas Neoplastic: hepatobiliary cystadenoma, biliary IPMN, cystadenoca, embryonal sarcoma, cystic variants of hepatic metastases

5 Congenital Simple Cysts
No communication with the biliary tract 5% of adults, most are < 3 cm 90% asymptomatic 2/3 female, age > 50 yrs Typically solitary, but may be multifocal Cyst lining: simple cuboidal epithelium No septation, usually unilocular Larger cysts can cause compression US best modality No septation unless two cysts are adjacent

6 Congenital Simple Cysts
Complications Symptoms Intracystic bleeding Imaging: US, MRI (T1 and T2) Acute enlargement Spontaneous rupture Peritoneum, pleura Compression Bile ducts, portal vein, hepatic veins, IVC Asymptomatic cysts: no need for routine surveillance, serial US studies show no appreciable changes over years In women, typically > 50 yrs, cysts might enlarge rapidly and become symptomatic. If this occurs in men or younger female patients, consider an alternative diagnosis Symptoms of intracystic bleeding, sudden severe pain, typically few days duration T1 sequence MRI becomes hyperintense when bleeding occurs. Intracystic bleeding can change appearance of cyst. Confusion with biliary neoplasms or hydatid cysts when prior comparison studies are not available

7 Management of Symptomatic Simple Cysts
Laparoscopic deroofing (n=46)1 Hospital stay 1 day Bile leak 4% Bleeding, N.R. Recurrence, symptomatic 4% Open deroofing (n=24)1 Hospital stay 7 days Complication rate 29% Recurrence, symptomatic 5% Perc drainage and sclerotherapy (n=129)2 EtOH or minocycline Recurrence, symptomatic 5-20% Single institution (Hospital Italiano, Buenos Aires), n=78 Deroofing: laparoscopic technique of partially excising the cyst wall with endoshears or simple cautery, and allowing fluid to drain freely into the peritoneum. If the cyst is covered by a thick layer of liver parenchyma, an endovascular stapler may be employed. Complications include bile leak and bleeding. Others have added ablating the cyst epithelium with ABC or saline-linked electrocautery 1Mazza et al. JACS 2009; 209:733-9. 2Moorthy et al. Ann R Coll Surg 2001; 83:

8 Polycystic Liver Disease (PCLD)
GENETICS (autosomal dominant) Associated with ADPKD PKD1, polycystin-1, chr 16p (85%) PKD2, polycystin-2, chr 4q (15%) Isolated PCLD PRKCSH, hepatocystin, chr 19p (40%) SEC63 PATHOLOGY Simple epithelium Phenotypical and functional features of biliary epithelium Secretory capacity Retention of von Meyenburg complexes, ductal plate malformations CLINICAL Mostly asymptomatic Massive hepatomegaly with abdominal protuberance and pain Diaphragmatic, gastrointestinal compression Malnutrition, poor QOL Hepatic function preserved PKD1, more renal cysts, larger renal cysts, and rapid progression to ESRD Polycystin: alters cytosolic Ca signaling and cAMP levels Both genes in isolated PCLD involved in protein secretion from the endoplasmic reticulum

9 Evaluate size, extent, and distribution of cysts
Management of PCLD Asymptomatic Symptomatic Patient age Symptom severity Renal function Stop estrogens Stop estrogens Evaluate size, extent, and distribution of cysts Follow-up Cyst related symptoms: pain from hepatomegaly, malnutrition, diaphragmatic compression producing dyspnea, infection, bleeding, and Budd Chiari manifestations. Observation that cyst proliferation occurs under high estrogen states has led to general acceptance that estrogen therapy should be discontinued in patients with PCLD; however, little proof exists that this alters the natural course of PCLD. Management of PCLD should involve a multi-disciplinary team in order to provide optimal patient selection for various surgical and non-surgical treatment options available for patients with PCLD. Patient selection should take into consideration the age of the patient, symptomatic severity, and the size, extent, and distribution of the cysts. Renal function and the pace towards progression to ESRD. Non-surgical Surgical Somatostatin analogues mTOR inhibitors HA embolization Sclerotherapy Fenestration Hepatectomy + fenest Transplant

10 Surgical Treatment Options Classification of PCLD
Type # Cysts Cyst size Total liver involvement I < 10 Large (> 10 cm) < 25% II Multiple Small, medium 25 – 50% III > 75% Type I: laparoscopic cyst fenestration (superficial cysts in anterior segments) Type II: open fenestration +/- partial hepatectomy Type III: fenestration NOT indicated, hemi-hepatectomy usually difficult A hand-full of classification systems have been developed over the years, but I will focus on two functional systems which can be applied to almost all patients with PCLD. Gigot aimed to characterize cysts based on size, number, and distribution. Patients with small number of large cysts, esp when predominantly situated in the anterior segments (III, IV, and V) are amenable to lap fenestration. Type II PCLD is typically characterized by multiple medium and small cysts deep within the liver which are not amenable to laparoscopic fenestration. These patients may be approached with partial hepatectomy + open fenestration if the distribution lends itself to preservation of an acceptable remnant liver Type III patients are very challenging and often tranplantation remains the only option. Morino et al. Ann Surg 1994; 219: Gigot et al. Surg Endoscopy 2001; 15:

11 Surgical Treatment Options Classification of Symptomatic PCLD
Type B Type C Type D Limited cyst # Multiple cysts ≥ 2 sectors preserved 1-2 sectors preserved Little liver preserved Schnelldorfer and Nagorney’s classification system considers not only the number and distribution of cysts but also considers the liver parenchyma which would be left behind after operative intervention. Type B pts with a small number of large superficial cysts can be approached with lap cyst fenestration. Type C pts can be approached with partial hepatectomy and cyst fenestration of the remnant if at least 1 relatively spareed sector can be preserved with normal inflow and outflow. For Type D patients, there is typically no relatively preserved sector and inflow or outflow vessels to that sector are often attenuated. Transplantation should be reserved for these type of patient who has severe symptoms with a declining performance status escpecially when renal transplantation is imminent. Cyst fenestration Partial Hepatectomy + Cyst fenestration Transplantation Schnelldorfer, Nagorney, et al. Ann Surg Surg 2009; 250:

12 Caroli’s Disease (Type V Bile Duct Cyst)
Segmental, cystic dilatations of the major IH ducts Ductal plate malformations Autosomal recessive disorder Linkage studies localize to chrom 3p, 8q Associations with ARPKD and medullary sponge kidney Two subtypes: simple v. complex (fibrocystic) Caroli’s syndrome: CD + hepatic fibrosis > cirrhosis Let’s now turn to Caroli’s disease which is a rare cystic disease of the liver with an incidence < 1:1M Sato et al. Int J Hep 2012.

13 Caroli’s Disease: Clinical Management
Diagnosis CT: central dots MRCP: hemi- v. total liver involvement Complications Cholestasis Hepatolithiasis Cholangitis Hepatic abscess Pancreatitis Biliary cirrhosis Hepatic fibrosis Cholangiocarcinoma, 4-13% Portal HTN

14 Percutaneous lithectomy ESWL Antibiotics Ursodeoxycholate
CD complications Medical therapy Hepatolithiasis management Cyst distribution Hemi-liver (L>>R) Partial hepatectomy Total liver Hepatic fibrosis Portal HTN Transplantation No fibrosis HJ + stone clearance Hemihepatectomy + stone clearance Medical therapy, plus ERCP lithectomy Percutaneous lithectomy ESWL Antibiotics Ursodeoxycholate CT MRCP The majority of patients with CD who present for treatment, do so because of acute or recurrent cholangitis. Pts should be started on BS abx to cover GN bacteria and ursodiol can help improve chronic cholestasis. ERCP is useful in clearing lithiasis predominantly involving the major IH ducts but is limited for extensive hepatolithiasis. Percutaneous lithectomy with or without EC shock wave lithotripsy can be used for patients with extensive hepatolithisias involving second order ducts. CT and MRCP can be used to determine the distribution of cysts and the presence of cirrhosis or portal HTN. For patients with hemiliver cysts, which more often involve the left side, partial hepatectomy can result in excellent long-term results. For pts with cysts throughout the liver, management decisions are more difficult. When HF or BC is present, esp with portal HTN, liver transplantation is really the only option. In the absence of fibrosis, HJ with choledochoscopy can provide good long-term control of cholangitis and possibly decreased incidence of cholangiocarcinoma.

15 Hepatobiliary Cystic Neoplasia
Biliary cystadenoma Biliary IPMN Biliary cystadenocarcinoma Cystic intrahepatic cholangiocarcinoma Cystic variants of secondary liver tumors NETs, mucinous adenoca Lastly, I would like to review intrahepatic biliary cystic neoplasms. This entity represents only 5% of liver cysts but often generates the most respect and interest. One of the major challenges in fully understanding and studying these lesions is that we do not have a concensus vocabulary for properly describing these neoplasms. Many of these tumors are unveiled incidentally during cross sectional imaging of the abdomen for unrelated indications. When they present with symptoms, e.g. abdominal protuberance, RUQ fullness, dyspnea, or rarely, with cholangitis due to biliary mucinosis, these tumors are typically quite large with a median diameter > 8 cm.

16 Making the diagnosis US is most useful Cyst aspiration is not helpful
Assess for multiloculation Thickened septae Mural nodularity Intracystic debris Papillary projections Cyst aspiration is not helpful CEA CA 19-9 Fluid cytology The most common of the neoplastic cysts are biliary cystadenoma and IPMN of the IH bile ducts. Discriminating either one of these tumors from a simple cyst of the liver can be challenging. US is probably the most important diagnostic test and having access to a skilled sonographer is crucial for characterizing these cysts. Unlike simple cysts, cystic neoplasms are typically unifocal and have a predilection for the central liver, namely segment IV. They contain internal septae and if thickened, and coinciding with mural nodularity, may indicate transformation into a biliary cystadenocaricnoma. Percutaneous cyst aspiration for cytology and fluid marker levels is not helpful. As shown in this scatterplot, cyst fluid levels for CEA and CA 19-9 can not discriminate a simple cyst from biliary cystadenoma or from cystadenocarcinoma. Ca BCA SC Ca BCA SC Seo et al. Eur J Gastro/Hep Int J Hep 2010; 22:

17 *Arnaoutakis et al. Ann Surg 2015;261:361–367

18 Biliary cystadenoma Bile duct IPMN
Ovarian stroma supporting columnar epithelium Female prevalence Mucin production No bile duct communication unless fistula Large cyst, often segment IV Concomitant cystadenocarcinoma (0-15%)1-3 Bile duct IPMN Pancreatic IPMN resemblance No ovarian stroma Male prevalence Bile duct communication Cystadenocarcinoma (30%)1-3 Describe each cyst separately. While fenestration with cyst lining ablation has been applied to biliary cystic neoplasms with acceptable recurrence rates, This should be avoided. Open technique cyst enucleation is usually the treatment of choice for non-invasive cystic neoplasms and provides excellent recurrence-free survival. But, keep in mind that biliary IPMN, like its pancreatic counterpart, may involve bile ducts adjacent to the cyst and place the patient at risk for local recurrence after enucleation. 1DeVaney et al. Am J Surg Path 1994; 18: 2Koffron et al. Surgery 2004; 136: 3Seo et al. Eur J Gastro/Hep Int J Hep 2010; 22:

19 Biliary cystadenocarcinoma
Arises in both cystadenoma and IPMN-B* Incidence- rare ~10% US and MRI detect subtle characteristics Mural nodularity, enhancement, hemorrhage, thick septae Formal resection/hepatectomy required EH bile duct resection may be required if central ducts involved *IPMN of the bile duct may progress to invasive cancer more rapidly than cystadenoma. US and MR better than CT at detecting subtle signs of invasive cancer

20 Treatment Resection Hepatic Lobectomy Liver transplant

21 *Arnaoutakis et al. Ann Surg 2015;261:361–367

22 Survival Median RFS - 12.1 years (95% CI 6.5– 18.1)
1-year, 3-year, and 5-year RFS was 89.1%, 72.6%, and 61.4%, respectively. 1-year, 3-year, and 5-year OS was 95.0%, 86.8%, and 84.2%, respectively. *Arnaoutakis et al. Ann Surg 2015;261:361–367

23 Survival After excision, long-term outcomes were better among patients with BCA versus BCAC. *Arnaoutakis et al. Ann Surg 2015;261:361–367

24 Recurrence Incidence of recurrence is higher among patients who underwent an unroofing/fenestration(48.6%) versus for patients who had a partial or major hepatectomy (15.7% and 10%, respectively P < 0.001) *Arnaoutakis et al. Ann Surg 2015;261:361–367

25 Summary Hepatic cysts are common.
Diagnostic imaging includes USG, CT or MRI Simple hepatic cysts warrant treatment if Large Symptomatic- pain, pressure Compressive effects leading to biliary dilatation Simple cysts can be treated by fenestration (lap or open), enucleation. Hepatobiliary cystic neoplasms should be resected Benign: enucleation Malignant: partial hepatectomy

26 THANK YOU Acknowledgements Dr.Michael Jacobs MD, FACS
Dr.Janak Parikh MD, MSHS Lauri Bolo ACNP-C

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