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 ConferenceFebruary, 2015Management of Liver CystsSandeep Anantha, MDHPB Surgery fellowHepatopancreatobiliary (HPB) Surgery, Department of SurgerySt. John Providence Healthcare System
4 Categories of Hepatobiliary Cysts Congenital: simple cysts, polycystic disease, bile duct cysts (type IV,V), bile duct hamartomasNeoplastic: hepatobiliary cystadenoma, biliary IPMN, cystadenoca, embryonal sarcoma, cystic variants of hepatic metastases
5 Congenital Simple Cysts No communication with the biliary tract5% of adults, most are < 3 cm90% asymptomatic2/3 female, age > 50 yrsTypically solitary, but may be multifocalCyst lining: simple cuboidal epitheliumNo septation, usually unilocularLarger cysts can cause compressionUS best modalityNo septation unless two cysts are adjacent
6 Congenital Simple Cysts Complications SymptomsIntracystic bleedingImaging: US, MRI (T1 and T2)Acute enlargementSpontaneous rupturePeritoneum, pleuraCompressionBile ducts, portal vein, hepatic veins, IVCAsymptomatic cysts: no need for routine surveillance, serial US studies show no appreciable changes over yearsIn women, typically > 50 yrs, cysts might enlarge rapidly and become symptomatic. If this occurs in men or younger female patients, consider an alternative diagnosisSymptoms of intracystic bleeding, sudden severe pain, typically few days durationT1 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)1Hospital stay 1 dayBile leak 4%Bleeding, N.R.Recurrence, symptomatic 4%Open deroofing (n=24)1Hospital stay 7 daysComplication rate 29%Recurrence, symptomatic 5%Perc drainage and sclerotherapy (n=129)2EtOH or minocyclineRecurrence, symptomatic 5-20%Single institution (Hospital Italiano, Buenos Aires), n=78Deroofing: 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 electrocautery1Mazza 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 ADPKDPKD1, polycystin-1, chr 16p (85%)PKD2, polycystin-2, chr 4q (15%)Isolated PCLDPRKCSH, hepatocystin, chr 19p (40%)SEC63PATHOLOGYSimple epitheliumPhenotypical and functional features of biliary epitheliumSecretory capacityRetention of von Meyenburg complexes, ductal plate malformationsCLINICALMostly asymptomaticMassive hepatomegaly with abdominal protuberance and painDiaphragmatic, gastrointestinal compressionMalnutrition, poor QOLHepatic function preservedPKD1, more renal cysts, larger renal cysts, and rapid progression to ESRDPolycystin: alters cytosolic Ca signaling and cAMP levelsBoth genes in isolated PCLD involved in protein secretion from the endoplasmic reticulum
9 Evaluate size, extent, and distribution of cysts Management of PCLDAsymptomaticSymptomaticPatient ageSymptom severityRenal functionStop estrogensStop estrogensEvaluate size, extent, and distribution of cystsFollow-upCyst 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-surgicalSurgicalSomatostatin analoguesmTOR inhibitorsHA embolizationSclerotherapyFenestrationHepatectomy + fenestTransplant
10 Surgical Treatment Options Classification of PCLD Type# CystsCyst sizeTotal liver involvementI< 10Large (> 10 cm)< 25%IIMultipleSmall, medium25 – 50%III> 75%Type I: laparoscopic cyst fenestration (superficial cysts in anterior segments)Type II: open fenestration +/- partial hepatectomyType III: fenestration NOT indicated, hemi-hepatectomy usually difficultA 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 liverType 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 BType CType DLimited cyst #Multiple cysts≥ 2 sectors preserved1-2 sectors preservedLittle liver preservedSchnelldorfer 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 fenestrationPartial Hepatectomy+Cyst fenestrationTransplantationSchnelldorfer, Nagorney, et al. Ann Surg Surg 2009; 250:
12 Caroli’s Disease (Type V Bile Duct Cyst) Segmental, cystic dilatations of the major IH ductsDuctal plate malformationsAutosomal recessive disorderLinkage studies localize to chrom 3p, 8qAssociations with ARPKD and medullary sponge kidneyTwo subtypes: simple v. complex (fibrocystic)Caroli’s syndrome: CD + hepatic fibrosis > cirrhosisLet’s now turn to Caroli’s disease which is a rare cystic disease of the liver with an incidence < 1:1MSato et al. Int J Hep 2012.
13 Caroli’s Disease: Clinical Management DiagnosisCT: central dotsMRCP: hemi- v. total liver involvementComplicationsCholestasisHepatolithiasisCholangitisHepatic abscessPancreatitisBiliary cirrhosisHepatic fibrosisCholangiocarcinoma, 4-13%Portal HTN
14 Percutaneous lithectomy ESWL Antibiotics Ursodeoxycholate CD complicationsMedical therapyHepatolithiasis managementCyst distributionHemi-liver (L>>R)Partial hepatectomyTotal liverHepatic fibrosisPortal HTNTransplantationNo fibrosisHJ + stone clearanceHemihepatectomy + stone clearanceMedical therapy, plusERCP lithectomyPercutaneous lithectomyESWLAntibioticsUrsodeoxycholateCTMRCPThe 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 cystadenomaBiliary IPMNBiliary cystadenocarcinomaCystic intrahepatic cholangiocarcinomaCystic variants of secondary liver tumorsNETs, mucinous adenocaLastly, 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 multiloculationThickened septaeMural nodularityIntracystic debrisPapillary projectionsCyst aspiration is not helpfulCEACA 19-9Fluid cytologyThe 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 SCCa BCA SCSeo et al. Eur J Gastro/Hep Int J Hep 2010; 22:
18 Biliary cystadenoma Bile duct IPMN Ovarian stroma supporting columnar epitheliumFemale prevalenceMucin productionNo bile duct communication unless fistulaLarge cyst, often segment IVConcomitant cystadenocarcinoma (0-15%)1-3Bile duct IPMNPancreatic IPMN resemblanceNo ovarian stromaMale prevalenceBile duct communicationCystadenocarcinoma (30%)1-3Describe 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 characteristicsMural nodularity, enhancement, hemorrhage, thick septaeFormal resection/hepatectomy requiredEH 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
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 SurvivalAfter excision, long-term outcomes were better among patients with BCA versus BCAC.*Arnaoutakis et al. Ann Surg 2015;261:361–367
24 RecurrenceIncidence 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 MRISimple hepatic cysts warrant treatment ifLargeSymptomatic- pain, pressureCompressive effects leading to biliary dilatationSimple cysts can be treated by fenestration (lap or open), enucleation.Hepatobiliary cystic neoplasms should be resectedBenign: enucleationMalignant: partial hepatectomy
26 THANK YOU Acknowledgements Dr.Michael Jacobs MD, FACS Dr.Janak Parikh MD, MSHSLauri Bolo ACNP-C