AN UNUSUAL CASE OF OBSTRUCTIVE JAUNDICE- SURGICAL

DILEMMA

Dr. Tejaswi Sindhiya Ragni

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

• 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

• 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

• Vitals:

- Temperature -99F

- pulse -80/min, regular rhythm

- BP -130/80 mmHg

- Respiratory rate -18/min

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

• CVS : S1 S2 heard

• RS : normal

• CNS : normal

INVESTIGATIONS

• Hb -11.5 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

• 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)

• SERUM MARKERS : HBsAg –negative

HCV – negative

Anti HAV IgM -negative

Anti HEV IgM - negative

HIV 1 & 2 -negative

OBSTRUCTIVE JAUNDICE

• USG ABDOMEN AND PELVIS : dilated intra and extra hepatic bile duct system

? calculi in terminal CBD

? Stricture of terminal CBD

MR CHOLANGIO PANCREATICOGRAPHY WITH CT-ABDOMEN:

• Short segment marked circumferential mural thickening of the supra pancreatic portion of CBD

• Significant luminal narrowing and upstream biliarydilatation.

• 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

MRI ABDOMEN

❑ ERCP:

• Lower end CBD block ?Cholangiocarcinoma

• Spy glass cholangioscopy followed by metal stenting .

HP of ERCP brushings

• Inflammatory changes

• No e/o malignancy

MULTIPHASE CT-ABDOMEN

PROVISIONAL DIAGNOSIS

?CHOLANGIOCARCINOMA

PLAN

WHIPPLES PANCREATICO DUODENECTOMY

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.

HISTOPATHOLOGY

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

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

INVESTIGATIONS

• Antibody screening –negative

• IGG -18.89 g/l (0.03-2.0)

• IGG4 -5.27g/l (0.03-2.0)

FINAL DIAGNOSIS

AUTOIMMUNE PANCREATITIS

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 .

AUTO IMMUNE PANCREATITIS

• Pancreatic manifestation of a systemic fibro-inflammatory 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.

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

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 .

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

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.

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.

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 .

• 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

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.

Diagnostic criteria (Contd.)

❑SEROLOGY: elevated serum igG4 level

❑Other organ involvement : hilar /intra hepatic biliary strictures, persistant distal biliarystricture, parotid/lacrimal gland involvement, mediastinal lymphadenopathy, retroperitoneal fibrosis

❑Response to steroids : resolution /marked improvement of pancreatic/extra pancreatic manifestations

• Treatment :

• Cornerstone - corticosteroids therapy

• If diagnosis remains in doubt and malignancy has been excluded, response to corticosteroids can be reasonable method to diagnosis.

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.

REFFERENCES

1. Chari ST, Smyrk TC, Levy MJ, et al. Diagnosis of autoimmune pancreatitis : the mayo clinic experience.Clin Gastroentero Hepatol 2006; 4(8): 1010-1016.

2. Kamisawa T. IgG4-Positive plasma cells specifically infiltrative various organs in autoimmune pancreatitis.pancreas 2004;29(2):167-168.

3. Kamisawa T, Egawa N, Nakajima H. Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol 2003;98(12):2811-12.

4. Yoshida K, Toki F, Takeuchi T, et al. Chronic pancreatitis caused by an autoimmune abnormality.Dig Dis Sci 1995;40(7):1561-68.

5. Sarles S, Sarles JC, Muratore R, et al. Chronic inflammatory sclerosis of pancreas . Am J Dig Dis 1961;6.688-698

6. Pearson RK, Longneker DS, Chari ST, et al. Controverse in clinical pancreatology . Pancreas 2003;27(1)1-13.

7. Sood S, Fossard DP, Sharrock K, Chronic sclerosing pancreatitis in sjogrenssyndrome ; case report . Pancreas 1995;10(4):419-421.

8. Weber SM, Cubukcu O, Palesty JA,et al. Lymphoplasmacytic sclerosingpancreatitis ;inflammatory mimic of pancreatic carcinoma . J Gastrotest Surg2003;7(1):129-137.

THANK YOU