AN UNUSUAL CASE OF OBSTRUCTIVE JAUNDICE- SURGICAL DILEMMA. Dr. Tejaswi Sindhiya Ragni

Transcription

1 AN UNUSUAL CASE OF OBSTRUCTIVE JAUNDICE- SURGICAL DILEMMA Dr. Tejaswi Sindhiya Ragni

2 A 65 year old male from Bangalore, farmer Presented with: Fever - 1 month Yellow discolouration of eyes and urine- 1month Pain abdomen-2 weeks Weight loss -7 kg in 2months Loss of appetite -3 weeks

3 Past history: - No h/o similar complaints - No h/o DM,HTN,COPD,CAD Personal history: -apetite reduced - normal bowel and bladder habits - known smoker stopped 10 yrs back -not known alcoholic Family history: - Not significant

4 General examination - Thin built and moderately nourished -BMI-20 kg/m2 - ICTERUS PRESENT - No pallor, clubbing, cyanosis, - No generalised lymphadenopathy - No signs of liver cell failure

5 Vitals: - Temperature -99F - pulse -80/min, regular rhythm - BP -130/80 mmhg - Respiratory rate -18/min

6 Systemic examination GIT: Inspection : - - Scaphoid shape - No scars, sinuses - No abdominal distension - No visible pulsations Palpation : - Generalised tenderness - No mass palpable - No organomegaly Percussion : -Normal Auscultation : - Normal bowel sounds - No bruit

7 CVS : S1 S2 heard RS : normal CNS : normal

8 INVESTIGATIONS Hb gm/dl TC -5900, N-69,L-20,M-7,E-2 ESR -30mm/hr Platelets-2.1 lakhs RFT BUN-18,Creatinine- 0.8mg/dl -Sodium-137meq/l,Potassium-4.5meq/l TSH : 2.5 mu/l

9 LFT: Total bilirubin-10.9mg/dl, direct-8.0mg/dl Total protein -7.3g/dl, Albumin-3.6g/dl, Globulin -3.7g/dl Alkaline phosphatase- 713u/l (0-115) SGOT- 140 U/l (0-49) SGPT -230 U/l (0-46)

10 SERUM MARKERS : HBsAg negative HCV negative Anti HAV IgM -negative Anti HEV IgM - negative HIV 1 & 2 -negative

11 OBSTRUCTIVE JAUNDICE

12 USG ABDOMEN AND PELVIS : dilated intra and extra hepatic bile duct system? calculi in terminal CBD? Stricture of terminal CBD

13 MR CHOLANGIO PANCREATICOGRAPHY WITH CT- ABDOMEN: Short segment marked circumferential mural thickening of the supra pancreatic portion of CBD Significant luminal narrowing and upstream biliary dilatation. Multiple small sub centimetric nodes anterior to the pancreatico - duodenal groove, suspicious of metastasis Mild prominence of main pancreatic duct. most probably represents malignant stricture-?cholangiocarcinoma

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15 MRI ABDOMEN

16 ERCP: Lower end CBD block?cholangiocarcinoma Spy glass cholangioscopy followed by metal stenting.

17 HP of ERCP brushings Inflammatory changes No e/o malignancy

18 MULTIPHASE CT-ABDOMEN

19 PROVISIONAL DIAGNOSIS?CHOLANGIOCARCINOMA

20 PLAN WHIPPLES PANCREATICO DUODENECTOMY

21 BIOPSY GROSS CBD - dilated with a metal stent in place. Luminal surface of the bile duct - irregular mucosa. Gall bladder - firm white circumferentially thickened wall with irregular mucosa. Pancreas patchy firm white areas.

22 HISTOPATHOLOGY

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24 HISTOPATHOLOGY(CONTD.) Marked storiform fibrosis with numerous mononuclear inflammatory cells including plasma cells, phlebitis noted. Most prominent in the gall bladder wall, pancreas and wall of CBD There is no evidence of malignancy

25 IMMUNO HISTOCHEMISTRY: IgG4 highlights in significant proportion of the plasma cells >10/HPF Pan CK highlights in surface epithelium CD 138 highlights in plasma cells

26 INVESTIGATIONS Antibody screening negative IGG g/l ( ) IGG4-5.27g/l ( )

27 FINAL DIAGNOSIS AUTOIMMUNE PANCREATITIS

28 Follow up Prednisolone -40mg for 1 month -20 mg maintainance Patient subsequently developed Diabetes mellitus and was started on insulin. At 6 months follow up, patient is asymptomatic, has fair glycaemic control.

29 AUTO IMMUNE PANCREATITIS Pancreatic manifestation of a systemic fibroinflammatory disease. Affects not only the pancreas but also various other organs including bile duct, salivary glands, retroperitoneum and lymphnodes. Lympho plasmacytic infiltrate rich in IgG4+ve cell. Responds to steroid therapy.

30 CLINICAL FEATURES PANCREATIC: Obstructive jaundice Diabetes Steatorrhea Upper abdominal discomfort Weight loss EXTRA PANCREATIC: Biliary stricture Sclerosing cholangitis Sialoadenitis Retro peritoneal fibrosis Chronic thyroiditis Interstitial nephritis

31 Clinical Features (Contd.) Most common obstructive jaundice with biliary stricture, focal mass or enlargement of pancreas. Major differential diagnosis pancreatic or biliary carcinoma Abdominal pain and attacks of acute pancreatitis are uncommon.

32 Histopathology Gross : pancreas is indurated, firm, gray to white and normal lobular architecture is lost. Involvement of main pancreatic duct results in proximal stenosis or obstruction and distal dilatation CBD thickened and stenotic with proximal dilatation

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34 Microscopy Infiltration of peri ductal space with plasma cells,t lymphocytes Acinar destruction, obliterative phlebitis, storiform or whirling fibrosis of parenchyma A dense infiltration of IgG4+ plasma cells in the pancreas is characteristic of AIP.

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36 IMAGING USG findings : Diffusely or focally enlarged pancreas with stricture of main pancreatic duct dilatation of upstream pancreatic duct or CBD Typically absent : calcifications, stones, pseudo cysts CT: Diffuse pancreatic enlargement,uniform enhancement, minimal pancreatic infiltration.

37 MRI: Global pancreatic enlargement decreased T1 signal,increased T2 signal, peri pancreatic decreased signal intensity ERCP: segmental or diffuse irregular narrowing of the main pancreatic duct.

38 MRCP : skipped,non visualised main pancreatic duct lesions. Segmental or diffuse irregular narrowing of the main pancreatic duct may not be seen Increased numbers of circulating immunoglobulin, specifically IgG4 are hallmark of disease

39 Diagnostic criteria Histology : (one of following) Periductal lymphoplasmacytic infiltrate with obliterative phlebitis and storiform fibrosis Lymphoplamacytic infiltrate with storiform fibrosis with abundant (>10 igg4) IMAGING: Diffusely enlarged gland with delayed rim enhancement, diffusely irregular and attenuated main pancreatic duct. Focal pancreatic mass/enlargement, focal pancreatic ductal stricture, calcification and pancreatitis.

40 Diagnostic criteria (Contd.) SEROLOGY: elevated serum igg4 level Other organ involvement : hilar /intra hepatic biliary strictures, persistant distal biliary stricture, parotid/lacrimal gland involvement, mediastinal lymphadenopathy, retroperitoneal fibrosis Response to steroids : resolution /marked improvement of pancreatic/extra pancreatic manifestations

41 Treatment : Cornerstone - corticosteroids therapy If diagnosis remains in doubt and malignancy has been excluded, response to cortico steroids can be reasonable method to diagnosis.

42 AIM OF PRESENTATION Auto immune pancreatitis is unique subtype of recently identified chronic pancreatitis that is immune mediated and represents one manifestation of a systemic IgG4 related disease process mimicking malignancy. It is important to recognize because it responds often dramatically to steroids and reduce unnecessary surgical procedures.

43 REFFERENCES 1. Chari ST, Smyrk TC, Levy MJ, et al. Diagnosis of autoimmune pancreatitis : the mayo clinic experience.clin Gastroentero Hepatol 2006; 4(8): Kamisawa T. IgG4-Positive plasma cells specifically infiltrative various organs in autoimmune pancreatitis.pancreas 2004;29(2): Kamisawa T, Egawa N, Nakajima H. Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol 2003;98(12): Yoshida K, Toki F, Takeuchi T, et al. Chronic pancreatitis caused by an autoimmune abnormality.dig Dis Sci 1995;40(7): Sarles S, Sarles JC, Muratore R, et al. Chronic inflammatory sclerosis of pancreas. Am J Dig Dis 1961; Pearson RK, Longneker DS, Chari ST, et al. Controverse in clinical pancreatology. Pancreas 2003;27(1) Sood S, Fossard DP, Sharrock K, Chronic sclerosing pancreatitis in sjogrens syndrome ; case report. Pancreas 1995;10(4): Weber SM, Cubukcu O, Palesty JA,et al. Lymphoplasmacytic sclerosing pancreatitis ;inflammatory mimic of pancreatic carcinoma. J Gastrotest Surg 2003;7(1):

44 THANK YOU