HepatoBiliary Pancreatic

Question 1

What are the variations in cystic duct anatomy?

Answer 1

A - angular (75%)
B - parrallel (20%)
C - from CHD
D- from right hepatic duct or aberrant right sectoral duct (2%)
E - Low insertion
F - short (20% <2cm)
G - Spiral (posterior) (5%)
H - Spiral (anterior)

Question 2

How is portal hypertension diagnosed? What are normal and abnormal numbers?

Answer 2

Clinically - based on the clinical manifestations of portal hypertension alone
Hepatic venous pressure gradient (HVPG) - if this diagnosis is in doubt,
- Free hepatic venous pressure (intra abdominal pressure) from the wedged hepatic venous pressure (portal venous pressure). FHVP measured by direct measurement of pressure in the hepatic vein. WHVP is by balloon occlusion of the hepatic vein.

Normal portal venous pressure is 5-10mmHg
Normal difference between portal and IVC is <5mmHg
Portal HTN present if >6mmHg
>10mmHg = clinically significant
>12mmHg = risk of variceal bleeding

Question 3

What are the causes of portal hypertension?

Answer 3

Most common cause in the western world is liver cirrhosis and world wide includes budd-chiari, schistosomiasis and portal vein thrombosis

Pre hepatic
- Portal vein thrombosis, splenic vein thrombosis

Hepatic
- Presinusoidal - schistosomiasis, congential hepatic fibrosis, and sarcoidosis
- Sinusoidal - CIRRHOTIC - EtOH, viral, NASH, primary sclerosing cholangitis, haemochromatosis, wilsons disease. NON-CIRRHOTIC - acute alcoholic hepatitis and cytotoxic drugs
- Post sinusoidal - overlap with EtOH cirrhosis and post hepatic causes.

Post hepatic
- Budd-Chiari syndrome (hepatic vein thrombosis)
- Constrictive pericarditis and right sided heart failure

Question 4

What is the pathophysiology of portal hypertension?

Answer 4

**1) Increase in resistance to portal blood flow **
- secondary to fibrosis with structural obstruction to flow and increased intrahepatic tone from endogenous vasoconstrictors (NA, endothelin, ANGII)
**2) Increase in the inflow of portal blood **
- sphanchnic arteriolar vasodilation from excessive endogenous vasodilators (NO and prostacyclin). As portal HTN gets worse, sphanchnic blood is increased with local release VEGF, NO and sphlanchnic vasodilators -> systemic hypotension, vascular underflilling -> RAS -> salt and water retention and increased cardiac output -> ascites

Question 5

What are the complications of portal hypertension?

Answer 5

1) Porto-systemic collaterals
- Coronary and short gastric veins
- Recanalisation of umbilical vein (ligamentum teres) -> caput medusae
- Inferior rectal veins
- Retroperitoneal collaterals

2) Ascites
- Splanchnic vasodilation - activation of the sodium retaining mechnism to return reperfusion pressure
- Sodium and water retention - dilutional hyponatraemia
- Spontaneous bacterial peritonitis

3) Hepatorenal syndrome
- Activation of vasoconstrictor systems (reduce blood flow)
- Progressive renal hypoperfusion (gradual decline in GFR -> renal failure)

4) Cardiopulmonary complications
- Hepatic hydrothorax with pleural effusion in cirrhosis due to movement of ascitic fluid into pleural space
- Hepatopulmonary syndrome
- Cirrhotic cardiomyopathy
- Portopulmonary hypertension

Question 6

What are the indications for a TIPS procedure?

Answer 6

Secondary prevention of recurrent variceal bleeding
Refractory cirrhotic ascites
Refractory acutely bleeding varices
Portal hypertensive gastropathy
Bleeding gastric varices

Question 7

What are the complications of TIPS?

Answer 7

1) Thrombosis, occlusion, stenosis (50% in 1st year)
2) Intra peritoneal bleeding
3) Fistula (biliary with jaundice and sepsis) and arterial with pulsitile flow)
4) Haemolysis (secondary to red blood cell damage by stent)
5) Hepatic infarction ( secondary to injury or thrombosis of hepatic artery)
6) Hepatic encephalopathy (20-30%) given contains hepatotropic hormones, nutrients, cerebral toxins.

Question 8

What are the abberations in hepatic artery anatomy?

Answer 8

1) Accessory or replaced LHA
- Left gastric artery (4-10%) (courses in lesser omentum with vagal branches to liver)
- Splenic artery (2%)
- Coeliac trunk

2) Accessory or replaced RHA
- SMA (10-20%) - runs from left to right behind lower end of CBD to emerge on its right posterior border
- Gastroduodenal artery (6%)
- Right gastric artery (2%)

Question 9

What are the varation types in hepatic duct confluence?

Answer 9

A -> normal bifurcation (57%)
B -> trifurcation (12%)
C -> aberrant right sectoral duct into CHD (20%) with C1 being right anterior sectoral duct to CHD (16%) and C2 being right posterior sectoral duct CHD (4%)
D -> aberrant right sectoral duct into **LHD ** (5%). Right posterior sectoral duct -> LHD (5%) and right anterior sectoral duct -> LHD (1%)
E -> absence of hepatic duct confluence (3%)
F -> drainage of right posterior sectoral duct into cystic duct (2%)

Question 10

What is the pathophysiology of gall stone formation?

Answer 10

There are 2 main types of gall stones, cholesterol and pigment stones

1) Cholesterol stones (90%) formed by:
- supersaturation of bile with cholesterol (imbalance of bile salts, phospholipids and cholesterol) - RFs age, sex, genetics, obesity, drugs, diet and liver disease.
- gall bladder hypomotility/stasis - emptying, absorption and secretion
- accelerated crystal nucleation by pronucleating agents (mucous, glycoproteins, immunoglobulins, calcium, infection)
- enterohepatic circulation (ileal resection, bowel transit time, cholestyramine)

2) Pigment stones (10%) results from supersaturation of bile with unconjugated bilirubin -> forms complex calcium salts that are insoluble
- Chronic haemolytic syndromes (bilirubin breakdown of haem -> conjugated in liver -> hydrolysis of conjugated bilirubin -> increased unconjugated bilirubin in bile
- Bacterial infection of bile (E cloi, bacteroides -> B-glucuronidase hydrolyzes conjugated bilirubin)
- Ileal resection/bypass disease -> increased bile salts reach caecum, increase solubility of unconjugated bilirubin -> reabosrption -> increased concentration in bile
3) normaly cholesterol is rendered soluble in bile by the balance between bile salts, cholesterol, and phospholipids -> this imbalance leads to cholesterol nucleation into crystals
-

Question 11

What parasitic infections are associated with benign biliary strictures?

Answer 11

1) Liver fluke (flat worm)
- eating inadequately infected cooked fish
- fluke passes into biliary tree, grows into maturity and sheds ova into GIT
- acute obstruction with chronic infection associated with hepatolithiasis

2) Ascaris
- Roundworm migration into the biliary tree with worm traversing the ampulla -> recurrent pyogenic cholangitis or papillary stenosis -> treated with albendazole

Question 12

What is primary sclerosing cholangitis and what are the known conditions it is associated with?

Answer 12

Progressive obliterative fibrosis of intrahepatic and extrahepatic biliary tree
Thought to be immunological with associations with ulcerative colitis, crohn’s, riedel’s thyroiditis, retroperitoneal fibrosis and IgG4 pancreatits

Question 13

What is the management of primary sclerosing cholangitis?

Answer 13

1) Antibiotics for cholangitis
2) Ursodeoxycholic acid (pruritis, biochemical function and histological appearance)
3) Endoscopic or percutaneous dilation of short/dominant strictures (stents high risk of complications)
4) Liver transplantation - persistent jaundice, intractable pruritis, recurrent cholangitis, malnutrition
5) Exclusion of cholangiocarcinoma (must be excluded, as a majority have recurrence if transplanted) - suspect if sudden rapid deterioration or dominant stricture

Question 14

What are the differentials for a benign liver tumour?

Answer 14

**Epithelial **
- Hepatocellular (Nodular transformation, focal nodular hyperplasia, hepatic adenoma)
- Cholangiocellular (Bile duct adenoma and biliary cystadenoma)

Mesenchymal
- Blood vessels (Haemangioma, infantile haemangioendothelioma, hereditary haemorrhagic telangiectasia)
- Adipose tissue (lipoma, myelolipoma, angiomyolipoma)
- Muscle tissue (leiomyoma)
- Mesothelial (mesothelioma)

Mixed
- Hamartoma
- Teratoma

Question 15

What is the clinical presentation of cavernous haemangioma?

Answer 15

Asymptomatic - incidental on imaging

Symptomatic (with increasing size)
- Abdominal pain or fullness (tumour thrombosis/bleeding)
- Early satiety
- Nausea/vomiting
- Fever

Rare
- obstructive jaundice/biliary colic
- Gastric outlet obstruction
- Spontaneous rupture
- Kasabach-Merrit syndrome - thrombocytopaenia + hypofibrinogenaemia (consumption of clotting factors)

Question 16

What is seen on multiphase CT scan for haemangioma?

Answer 16

Non contrast -> well demarcated hypodense mass, calcifications 10%

Contrast -> early peripheral nodular or globular enhancement
- Delayed centripetal filling
- Isodense or hyperdense on delayed
- Lesions > 4cm , centre may not opacify
- Lesions < 2cm - early homogenous enhancement

Question 17

What are the management options for haemangioma?

Answer 17

Observation
- lesions < 5cm and asymptomatic
- asymptomatic giant haemangiomas with imaging 6 monthly

Surgical excision
- large symptomatic lesions
- Complications (rupture/bleeding)
- Failure to exclude malignancy radiologically or diagnostic uncertainty

Question 18

What is focal nodular hyperplasia?

Answer 18

Hyperplastic regenerative response to hyperperfusion by an anomalous artery characteristically found at the centre of the lesion.
- congenital vascular anomaly associated with cavernous haemangioma and hereditary haemorrhagic telangiectasia
- may be responsive to OCP, being larger, vascular and more symptoms

Question 19

What are the imaging findings of focal nodular hyperplasia on CT multiphase?

Answer 19

Non contrast -> hypoor isodense, central scar in 30%
Arterial phase -> Hyperdense
Portal venous phase -> isodense central scar may become hyperdense as contrast diffuses in

Question 20

What are the management options for focal nodular hyperplasia?

Answer 20

Non operative
- lesions are stable and complications are rare
- repeat imaging 3-6 months to document stabilty
- OCP can be continued and reasonable to extend follow up imaging 6-12 mo on woman who continue to take OCP

Surgical resection
- diagnostic uncertainty
- rare symptomatic lesions
- women with large FNH (>8cm) planning pregnancy

Question 21

What are the 3 main mutations associated with hepatic adenoma?

Answer 21

1) Telangiectatic
- exclusive to woman , small risk of bleeding, no malignant potential, lesions have marked steatosis

2) Beta catenin
- risk of malignancy and associated with OCP

3) HNF1 alpha
- small risk of bleeding, highest risk of malignant transformation

Question 22

What are the CT multiphase imaging findings for hepatic adenoma?

Answer 22

Non contrast -> well demarcated, hypo or isodense
Contrast -> patchy enhancement with variegated appearance with filling during portal venous phase, heterogenous secondary to haemorrhage, necrosis and firbosis

NOTE - MRI primovist is useful for FNH v adenoma when adenoma fails to light up

Question 23

What are the management options for hepatic adenomas?

Answer 23

Men - all hepatic adenomas should be considered for resection. Consider location as well as comorbidity.

Woman - <5cm, observation with cessation of OCP. > 5cm considered for resection but this may vary if the patient is on OCP, with cessation of OCP and repeat imaging to see if there has been regression. Waiting period of longer than 6 months between imaging.

Pregnancy - adenomas tend to grow during pregnancy and need observation. Pregnancy should not be discouraged in the presence of a small HCA.

Question 24

What are the risk factors associated with pancreatic cancer?

Answer 24

Lifestyle
- smoking
- EtOH
- Obesity and lack of physical

Systemic
- Type 2 DM

Pancreatic conditions
- Chronic pancreatitis
- Pancreatic lesions (IPMN and mucinous cystic neoplasm) - pancreatic intraepithelial neoplasia with sequential progression dysplasia -> invasive malignancy)

Genetics
- Familial pancreatic cancer (RR 18) - 2 x 1st degree relatives
- Lynch, FAP, Li Fraumani, Cystic fibrosis, Peutz jaghers, BRCA 1 and 2, familial atypical multiple mole syndrome (FAMMM with CDKN2A mutation)

Question 25

What is the gastrinoma triangle?

Answer 25

Bordered by:
1) Junction of the body and neck of the pancreas
2) Junction of the 2nd and 3rd parts of duodenum
3) Junction of common bile duct and cystic duct

Question 26

What are the hereditary associations with PNETs?

Answer 26

MEN1, Von hippel lindau, NF1 and tubular sclerosis

Question 27

What do all functional PNET (except one) require for symptomatic control?

Answer 27

Somatostatin analogue
(exception is gastrinoma)

Question 28

What are the syndromes, symptoms, diagnosis tests and medications needed for each of the PNETs?

Answer 28

**Insulinoma **
- Whipples triad
- Neurogenic symptoms relieved with eating
- Insulin/glucose ratio > 0.3 in presence of hypoglycaemia AND C peptide suppresion test
- Overnight feeding, diazoxide titrated to symptom solution, somatostatin analogue

**Gastrinoma **
- Zollinger-ellison
- Complicated peptic ulceration or GORD, diarrhoea, abdominal pain
- Serum fasting Gastrin > 1000 (gastric pH <2.5) AND Secretin stimulation test
- High dose PPI (may be required)

Glucagonoma
- Glucagonoma syndrome
- Necrolytic migratory erythema, weight loss, diabetes mellitus, stomatitis, diarrhoea, thromboembolism
- Glocagon > 1000
- Somatostatin analogue, thrombosis prophylaxis

**VIPoma **
- Verne-Morrison syndrome
- Profuse watery diarrhoea, hypokalaemia
- VIP plasma > 1000
- Somatostatin analogue

Somatostatinoma
- Gall stones, steatorrhoea, hypochlorryhdria, glucose intolerance
- Raised plasma somatostatin

Question 29

What are the absolute indications for surgical resection of a IPMN?

Answer 29

Main duct IPMN
Mixed duct IPMN

Branch duct IPMN with the following:
1) Pancreatic duct > 10mm
2) Mural nodules > 5mm
3) Obstructive jaundice

Question 30

What are worrisome features of IPMN?

Answer 30

Cystic lesion > 3cm
Narrowing of pancreatic duct
Mural nodules < 5mm
Growth > 5mm over 2 years
Pancreatitis
Thickening of IPMN wall
Lymphadenopathy
Pancreatic duct 5-9mm
Ca 199 elevated

Question 31

What is the basis for pancreatitc resectability?

Answer 31

Resectable - no arterial involvement and < 180 SMV/PV

Borderline resectable - < 180 arterial involvement and > 180 SMV/PV

Locally advanced - occluded, not reconstructrable SMV/PV and > 180 arterial involvement

Question 32

What are the indications and benefits for neoadjuvant chemotherapy for pancreatic adenocarcinoma?

Answer 32

Indications
- Borderline resectable
- Locally advanced
- Consider in resectable cases (esp if very high Ca 19/9, large tumours, large regional LNs - also pre opo imaging is not always accurate)

Benefits
- Test biology (improve selection of patients that may benefit)

• Improves outcomes (increase R0 resection rates, overall survival, less operative morbidity, smaller tumours, lower rates of LVI and PNI and better overall survival)
• Avoid systemic treatment delay (early treatment of micromets)
• Downstage

Question 33

What are the characteristic features of serous cystic tumours?

Answer 33

F>M
Evenly distributed throught pancreas

Microcystic lesion (Multiple cysts < 2cm) with central calcification (Sunburst calcification). Microcystic honeycomb with central scar.

Associated with Von Hippel Lindau

Management is usually observant given malignant transformation is rare. But if symptomatic then consider resection.

Question 34

What are the characteristic features of mucinous cystic neoplasm?

Answer 34

F > M
More commonly located in the tail of the pancreas

Septated cystic lesions that can be unilocular
Signs of malignant transformation - large size (>5cm), thickened or irregular cyst wall, peripheral calcification, solid component within cyst

Usually lined by OVARIAN LIKE STROMA

ALl MCNs should be resected given their potential for malignant transformation

Question 35

For cyst aspiration, what are the differences between pseudocyst, MCNs, SCA?

Answer 35

Question 36

What are the characteristic features of solid pseudopapillary cystic neoplasm?

Answer 36

Occurs in young woman 20-30’s
Frequently large
Rare cystic tumour

Question 37

What is the pathophysiology of chronic pancreatitis?

Answer 37

Repeated injury to acinar, ductal and islet cells -> healing by fibrosis -> calcification, ductal strictures, parenchymal atrophy -> loss of endocrine and exocrine function -> dysplasia and malignancy

Question 37

What is chronic pancreatitis?

Answer 37

This is chronic progressive inflammatory changes within the pancreas resulting in permanent structural damage and impairment of exocrine and endocrine function.

Question 38

What are the potential causes of chronic pancreatitis?

Answer 38

TIGAR-O

Toxic-metabolic
- EtOH, smoking, hypercalcaemia, hyperlipidaemia, chronic renal failure, medications and toxins

Idiopathic
- Associated with early onset CP as well as late onset CP

Genetic mutations
- PRSS1, CFTR, SPINK1, others

Autoimmune
- IgG4 (can get associated biliary stricture, scllerosing adenitis, retroperitoneal fibrosis, interstitial nephritis)

Recurrent and severe AP associated CP
- post severe necrotic pancreatitis, vascular disease/ischaemia

Obstructive
Pancreatic divisim, SOD, duct obstruction, post traumatic pancreatitc duct scars

Question 39

What are three differentials to consider for chronic pancreatitis?

Answer 39

1) Pancreatic cancer
- patients in which there is suspicion for cancer, surgical resection should be considered as difficult differentiate with imaging and EUS+FNA

2) IPMN
- MD IPMN can have main duct dilatation
- difference is ductal stricture and calcification

3) Autoimmune pancreatitis
- associated with extrapancreatitc stricture and does not have calcification
- sausage shpaed and iGG4 elevated

Question 40

What are the principles of medical management of chronic pancreatitis?

Answer 40

Analgesia - WHO pain ladder used. Co anlgesics used such as gabapentin. Discuss in conjunction with pain specialist.

EtOH avoidance - professional EtOH consumption avoidance

Smoking cessation - smoking cessation clinics and help/guidance

Exocrine replacement - considered in all patients with chronic pancreaitis and can be based on pragmatic clinical assessment (faecal elastase)

Question 41

What are the endoscopic/surgical options for chronic pancreatitis?

Answer 41

Surgical superior to endoscopic overall
If endotherapy does not work rapdily after procedure, then is unilkely to be of beneift long term

1) Endoscopic
a) Drainage/stenting of main pancreatic duct - assess duct, remove calcifications, leave a single stent or multiple to facilitate drainage
b) Coeliac plexus block - LA injection under EUS
c) Complications of chronic pancreatitis - stricture, pseudocyst, short term duodenal stenting

2) Surgical
a) Panc head mass - often a concern about underlying malignancy and therefore require pancreatico-duodenectomy (cancer a concern) or duodenum preserving pancreatitic head resection (similar outcomes for pain but DPPHR less morbid)
b) Dilated main pancreatic duct w/o head mass - decompression of the main pancreatic duct with resection of the pancreatic tail, splenectomy and longitudinal lateral pancreatico-jejunostomy.
c) Total pancreatectomy can be considered in younger patients with low risk of cancer. Require islet autotransplantation
d) Gastrojej bypass for duodenal stenosis

Question 42

What are the causes of portal vein thrombosis?

Answer 42

Inherited
- factor V leiden, protein C and S def, antithrombin III def

Acquired
- Cirrhosis
- Cancer -> CRC, pancreatic ca, HCC
- Inflammatory conditions, abdominal (pancreatitis, IBD), systemic (antiphospholipid syndrome), infection , trauma, surgery, myeloproliferative disorders/malignancy

Question 43

What measures are taken to medically optmise a patient prior to taking them to theatre for liver cirrhosis?

Answer 43

Diuretics with spironolactone 100mg and frusemide 40mg

Na and fluid restriction

Stop ACEIs, ARBs, beta blockers

NSAIDS avoided as they can reduce urinary sodium excretion

Question 44

What is the UCSF criteria for liver transplant in the context of HCC?

Answer 44

• Single tumour < 6.5cm
• Maximum of 3 tumours, none > 4.5cm
• Cumulatuve size < 8cm

5 year survival of 60-75%

Question 45

List the different causes of a gall bladder polyp

Answer 45

Benign
- cholesterol polyp 60%
- adenomyomas (25%)
- inflammatory polyps (10%)
- adenomas (1%)
- others (leiomyomas, fibromas, lipomas)

Malignant
- adenocarcinoma (80%)
- mucinous cystadenomas, squamous cell carcinoma, adenocanthomas

Malignant

Question 46

What risk factors are associated with gall bladder polyps?

Answer 46

Age > 50
Indian ethnicity
PSC
Sessile polyp
Size

Question 47

What is the Bismuth Corlette Classification?

Answer 47

Classification system used for cholangiocarcinoma to identify anatomical location