GANGGUAN PANKREATOBILIER - PowerPoint Presentation

1. GANGGUAN PANKREATOBILIERDr Willy B Uwan, MARS, SpPD, K-GEH, FINASIMSMF Penyakit DalamKlinik Penyakit Hati dan Saluran CernaUnit Endoskopi Diagnostik dan TerapeutikRSU St Antonius Pontianak

2. Pankreatitis AkutDefinisi Peradangan akut, non-bakterial pada organ pankreas.PatofisiologisTerjadinya pankreatitis akut diawali karena adanya jejas di sel asini pankreas akibat:Obstruksi duktus pankreatikus (terutama oleh migrasi batu empedu)Stimulasi hormon cholecystokinin (CCK) sehingga akan mengaktifasi enzim pankreas (misalnya karena pengaruh hipertrigliseridemia dan alkohol)Iskemia (misalnya pada pankreatitis akut paska prosedur endoscopic retrograde cholangiopancreatography / ERCP atau atherosklerosis)

3. EtiologiBeberapa penyebab pankreatitis akut adalah:Obstruksi batu di duktus koledokus (38%)Alkohol (36%)Pankreas divisium (7%)Komplikasi paska tindakan ERCP (5,4%)Hipertrigliseridemia (1-4 %)Obat-obatan (1-4 %)HiperkalsemiaPankreatitis akut idiopatik (10-15 %)

4. ETIOLOGYGallstones, microlithiasis, biliary sludgeEthanol (5 years, > 50g/day), ERCPToxins, tumors, traumaSteroids and ulcersMumps and other infectionsAutoimmuneStenosis (sphincter of Oddi dysfunction, papillary stenosis)Hypertriglyceridemia, hypercalcemia, hypothermiaGenetic (<30 years old, family history)Drugs (Azathioprine, estrogen, HIV drugs, tetracycline, sulfa

5. Fase Pankreatitis AkutPada umumnya perjalanan klinis pankreatitis akut dapat dibagi:Fase awalFase awal terjadi pada minggu pertama, ditandai dengan adanya systemic inflammatory response syndrome / SIRSFase lambatFase lambat berlangsung beberapa minggu sampai bulan dan ditandai dengan adanya SIRS yang persisten atau oleh karena komplikasi lokal dari pankreatitis akut.

6. DiagnosisDiagnosis pankreatitis akut ditegakkan berdasarkan:AnamnesisPemeriksaan fisikLaboratoriumPemeriksaan imagingMenurut klasifikasi Atlanta (2012), diagnosa pankreatitis akut tegak bila memenuhi 2 dari 3 kriteria berikut:Nyeri perut bagian atasPeningkatan amilase atau lipase > 3x nilai batas atas normalPemeriksaan imaging (USG / CT scan atau MRI)

7. Sistem skor Marshall untuk menilai gagal disfungsi organSistem organSkor 01234Respirasi(Pa02/Fi02)>400301 – 400201 – 300101 – 200≤101Ginjal(serum kreatinin; mg/dl)<1,41,4 – 1,81,9 – 3,63,6 – 4,9>4,9Kardiovaskular(mm Hg)>90<90, respon(+) dengan cairan<90, respon(-) dengan cairan<90, pH<7,3<90, pH<7,2

8. Definisi pankreatitis akut berat: perbandingan kriteria Atlanta 1992 dan 2012Kriteria Atlanta (1992)Kriteria Atlanta (2012)Pankreatitis akut ringanTidak adanya gagal organTidak adanya komplikasi lokalPankreatitis akut ringanTidak adanya gagal organTidak adanya komplikasi lokalPankreatitis akut beratKomplikasi lokal dan atauGagal organPerdarahan gastrointestinal >500 cc /24 jamSyok – tekanan darah sistolik ≤90 mmHgPa02 ≤ 60%Kreatinin ≥ 2 mg/dlPankreatitis akut sedang – beratKomplikasi lokal atau sistemik tanpa gagal organ persistenGagal organ sementara (<48 jam)Pankreatitis akut beratGagal organ persisten (>48 jam) (memakai kriteria skor Marshall)Gagal organ tunggalGagal organ multiple

9. Klasifikasi Pankreatitis AkutBerdasarkan Klasifikasi Atlanta 2012, tingkat keparahan pankreatitis akut dibagi:Pankreatitis akut ringanPankreatitis akut sedangPankreatitis akut berat

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12. TatalaksanaTerapi supportif: resusitasi cairan, koreksi gangguan elektrolit dan koagulasi, pemberian oksigenAntibiotik: diberikan pada infeksi ekstra pankreas seperti kolangitisAnalgetik: pilihan narkotik injeksiTerapi nutrisiTerapi pembedahan

13. Acute PANCREATITIS

14. EPIDEMIOLOGYAP was responsible for approximately 300,000 hospital admissions in the United States in 2012.Recent studies show the incidence of AP varies between 4.9 and 73.4 cases per 100,000 worldwide. Although the case fatality rate for AP has decreased over time, the overall population mortality rate for AP has remained unchanged.

15. PATHOPHYSIOLOGYDUCT OBSTRUCTIONACINAR CELL INJURY

16. DIAGNOSIS (REVISED ATLANTA CONSENSUS 2012)2 of the 3 criteria:Abdominal pain consistent with the diseaseSerum amylase and or lipase greater than three times the upper limit of normal, and or Characteristic findings from abdominal imagingContrast-enhanced computed tomography (CECT) and or magnetic resonance imaging (MRI) should be reserved for patients in whom:the diagnosis is unclear orwho fail to improve clinically within the first 48 – 72h after hospital admission or to evaluate complications

17. CLINICAL PRESENTATIONSudden-onset abdominal pain and persists for hours to days Nausea and vomitingDelirium, hemodynamic instability, extreme respiratory distress

18. PHYSICAL EXAMINATIONAbdominal tenderness with guarding especially in the epigastric regionBowel sounds are diminished

19. LABORATORY STUDIESElevated serum amylase and or lipase ≥ 3xULN > 5xULN and lipase is more specific

20. IMAGING STUDIESUltrasound:Most sensitive noninvasive for detecting gallstone and biliary tract dilationCT scanning:Superior to ultrasound for detecting the changes associated with pancreatitis and its complications

21. IMAGING STUDIESERCP:Primarily a therapeutic tool in acute biliary pancreatitis (no role in diagnosing)EUS:A sensitive test for detecting persistent biliary stones for further ERCP procedure

22. SYSTEMIC COMPLICATIONSPulmonary processes (hypoxemia, pleural effusions, ARDS)Renal failureCoagulopathyDeliriumShock

23. LOCAL COMPLICATIONINTERSTITIAL PANCREATITIS WITH ACUTE PERIPANCREATIC FLUID COLLECTIONRESOLVING INTERSTITIAL PANCREATITIS WITH PSEUDOCYST

24. LOCAL COMPLICATIONPANCREATIC AND PERIPANCREATIC NECROSISWALLED-OFF PANCREATIC NECROSIS (ENCAPSULATED IN 4-6 WEEKS)

25. INITIAL ASSESSMENT AND RISK STRATIFICATIONHemodynamic status and resuscitative measuresStratify patients into higher and lower risk categories (admission setting)Evaluate organs failureTenner et al, American College of Gastroenterology Guideline: Management of Acute Pancreatitis, Am J Gastroenterol

26. DEFINITIONS OF SEVERITYThe onset of acute pancreatitis is defined as the time of onset of abdominal pain Early dischargeNo need imagingVery rare mortality SIRSMortality 36-50%Infected necrosis

27. DEFINITIONS OF SEVERITYA score of ≥ 2 in any system define the presence of organ failure

28. PROGNOSTIC SCORING SYSTEM (SEVERITY OF AP)Ranson’s criteria; Glasgow prognostic criteria; APACHE II; Balthazar CT severity index; BISAP:is applicable within the first 24h presentation≥ 3 had a mortality rate of approx. 15%CRITERIAPOINTSBUN > 25mg/dL1Impaired mental status1Presence of SIRS (≥ 2 criteria)1Age > 60yo1Presence of pleural effusion1

29. TREATMENTSupportive (first 24h is golden hours):Massive volume repletionParenteral/enteral feedingNGT?No rule for the use prophylactic antibioticsNo evidence to support routine use of somatostatinERCP

30. INITIAL MANAGEMENTObtain vital signs at frequent intervals (such as every 4-6 h)Supplemental oxygen be administered during the first 24–48 h, especially if narcotic agents are used to control pain.BGA should be performed when oxygen saturation is ≤95%, hypoxemia or hypotension refractory to a bolus of IV fluids.

31. INITIAL MANAGEMENTAggressive hydration using isotonic crystalloid solution should be provided to all patients, unless cardiovascular, renal or other related comorbid factors exist (most beneficial during the first 12 – 24 h).Lactate Ringer solution may be the preferred. Large volume normo-saline may lead to a non-anion gap, hyperchloremic metabolic acidosis.

32. AGGRESSIVE HYDRATIONBolus 1 to 2 L of crystalloids (approx. 20 mL/kg)Continuous infusion of 150 to 300 cc/hour (approx. 3 mL/kg/h), first 24 hoursFluid requirements assessment (intervals 6 h of admission and for 24-48 h)Decrease hematocrit and BUN Maintain a normal creatinine

33. ICU? SevereNeed aggressive fluid resuscitation (elderly and cardiovascular disease)Deteriorating respiration (no hypoxemia)

34. NUTRITION IN APMild acute pancreatitis:Oral feedings can be started immediately if there is no nausea and vomiting and the abdominal pain has resolved.Initiation of feeding with a low-fat solid diet appears as safe as a clear liquid diet. Severe acute pancreatitis:Enteral nutrition is recommended (nasogastric and nasojejunal).Parenteral nutrition should be avoided, unless the enteral route is not available/not tolerated/not meeting caloric requirements.

35. NUTRITION IN APOral intake of limited amounts of calories is usually initiated when:Abdominal pain has subsidedParenteral narcotics are no longer required Nausea and vomiting have ceasedAbdominal tenderness has markedly decreasedBowel sounds are present Overall assessment of the patient has improved

36. ANTIBIOTICS IN APShould be given for:Infection included extra-pancreaticNecrosis who deteriorate or fail to improve after 7-10 days of hospitalization → infectedIs not recommended:Prophylactic antibiotics No source of infection is identified

37. ERCP IN AP Concurrent acute cholangitis should undergo ERCP within 24 hProgressive bilirubin increasing (CBD obstruction/biliary pancreatitis)In the absence of cholangitis and or jaundice, MRCP or EUS rather than diagnostic ERCP should be used to screen for choledocholithiasis if highly suspected.

38. Autoimmune Pancreatitis (Ig G4– Associated Cholangitis) Stricturing of the pancreatic duct, focal or generalized pancreatic enlargement, IgG4 > 140 mg/dL Lymphoplasmacytic infiltrate on biopsy Response to corticosteroid therapy

39. Primary Sclerosing Cholangitis

40. EpidemiologyPrevalence: 6-16 cases/100.000Incidence: 1 case/100.000Geographical variation Men > WomenMedian age: 40 yearsConcomitant (60-80%) with UC

41. PathophysiologyImmune-mediated processGenes: HLA-DRB1*1501-DQB1*602, HLA-DRB1*1301-DQB1*0603, HLA-A1-B8-DRB1*0301-DQB1*0201‘Second Hit’ – environmental trigger, toxin or infectious exposureInnate and adaptive immune systemLymphocyte migration, cholangiocyte damage, fibrosis

42. Clinical PresentationsAsymptomaticNon-specific: fatigue, pruritus, jaundice, weight lossLess common: fever, chill, night sweat, abdominal painJaundice, Hepato-splenomegaly, ExcoriationsCirrhosis

43. Clinical PresentationsElevated alkaline phosphatase and bilirubinAminotransferase normal or mildly elevatedSynthetic function altered in advanced disease

44. Clinical PresentationsUS: ductal wall thickening and focal bile duct dilations, gallbladder wall thickening, distention, gallstones and massCT: thickened and inflamed bile ducts, saccular dilation intra-hepatic ducts and mass in gallbladderMRCP: “beaded” appearance – multifocal short annular strictures alternate between normal and dilated

45. DiagnosisGold standard: MRCP with sensitivity and specificity of 86% and 94%, and ERCPMultifocal annular strictures alternating with segments of normal or dilated bile ducts of intrahepatic and/or extrahepaticbile ducts – ‘bead on a string apperance’Rule out IgG-4Liver biopsy rarely helpful – “onion skin” (25%), small duct PSC, PSC-AIH overlap syndrome

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47. Onion Skin ApperanceNEJM 1995

48. DiagnosisClassic PSC: biliary strictures with normal intervening segments or diffusely involved long segments. Radio-occult: strictures not present, shallow ulcerations bile ductSmall duct PSC more difficult to diagnosisAntibodies are non-specific

49. Serum Autoantibodies in PSC

50. Differential DiagnosisClinical Liver Disease, Vol 3, No 3, March 2014

51. TreatmentsNo proven medical therapy Ursodeoxycholic acid (UDCA): 15 mg/kg/day – improve biochemical markers and inflammationEndoscopic treatment - dominant bile-duct stenosis Liver transplantation (LTx) – end stage liver disease, portal hypertension refractory therapy, intractable pruritus, reccurent cholangitisLTx: 5 and 10 year survival rates of 87.4% and 83.2 %

52. Major RCT of UDCA in the Treatment of PSC PSC Management and Surveillance Clinical Liver Disease, Vol 3, No 3, March 2014

53. ComplicationsObstructiveColorectal Cancer, Cholangiocarcinoma, HCC, Gallbladder CancerCirrhosisCholelithiasis, Fat-soluble vitamin deficiencies, Osteoporosis

54. The risk of developing PSC-associated cancers Dtsch Arztebl Int 2013

55. Clinical surveillance of PSCDtsch Arztebl Int 2013

56. Cholangiocarcinoma surveillance in PSC Clinical Liver Disease, Vol 3, No 3, March 2014

57. PrognosisMedian time to death or transplantation: 12-18 years for asymptomatic and no IBD 9 years for symptomaticMayo Risk Score: R = 0.03 (age [years]) + 0.54 loge (bilirubin [mg/ dL]) + 0.54 loge (AST [UIL]) + 1.24 (variceal bleeding [0/1]) − 0.84 (albumin [g/dL])

58. Gallbladder cancer surveillance in PSC Clinical Liver Disease, Vol 3, No 3, March 2014

59. Subclassification of PSC

60. Relationships between subphenotypes of PSC

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62. Diagnostic Algorithm for the Overlap Syndromes

63. Diagnostic Features of the Overlap Syndromes

64. Treatment Options for the Overlap Syndromes

65. Simplified Criteria for the Diagnosis of AIH

66. KolangiokarsinomaTumor primer dari epitel duktus biliarisKanker hepatobilier terbanyak kedua setelah karsinoma hepatoseluler95% adalah adenokasinomaLokasi 20% intra hepatik /perifer, 80% ekstra hepatik (70-80% perihilar dan 20-30% distal duktus biliaris)Faktor risiko: kolangitis sklerosing primer, kista duktus biliaris, sirosis hepatis, hepatitis B dan C, infeksi clonorchis sinensis

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