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FRACS Revision > Hepato Biliary > Flashcards

Hepato Biliary Flashcards

1
Q

What are the high-risk features and worrisome features for IPMN

A

HIGH RISK FEATURES

  • Jaundice
  • Main duct >10mm diameter
  • Enhancing solid component

WORRISOME FEATURES

  • Pancreatitis
  • cyst >3cm
  • wall enhancement
  • non-enhancing mural nodule
  • Duct 5-9 mm
  • abrupt calibre change in duct with distal pancreatic atrophy
  • Lymphadenopathy
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2
Q

What are EUS features concerning for malignancy in IPMN?

A

Main duct IPMN

  • duct >7 mm
  • Mural nodule >10mm

BD-IPMN

  • Cyst >3cm with thick septa
  • Mural nodule >10 mm

These patients should then be considered for surgery

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3
Q

Imaging features of solid liver lesions

A

HEMANGIOMA

  • Noncon - Hypoattenuating
  • Arterial - peripheral filling in
  • PV - centripetal fill in

FNH

  • Central scar
  • Feeding arteriole

Adenoma

  • Non-con - Hypoattenuating / isointense

HCC

  • Arterial phase - rapid enhancement
  • PV phase - washout
  • U/S duplex - peritumoral vessels

Cholangio/colorectal mets

  • non-con- hypoattenuating
  • PV - may have minor peripheral enhancement
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4
Q

What are the predictive factors for variceal bleeding?

A

Endoscopic factors

  • Location - intragastric varices > gastroesophageal varices
  • Size
    • small (straight)
    • Medium (<1/3rd of lumen)
    • Large (>1/3rd of lumen)
  • Presence of red signs (wale, cherry spot, blood blister)
  • variceal pressure (>12mm hg, exponential risk increase after 15)

Patient factors

  • Disease severity (Childs score / MELD)
  • Previous bleeding

​

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5
Q

What are the features of liver cystadenomas? How would you treat it?

A

These are rare cysts that are filled with mucin and have a thick wall with nodularity/septations/projections.

Females in 50s (similar to mucinous cystadenomas in pancreas)

Diagnosis - CT/MR/US

Differential - hydatid cyst/ cystadenocarcinoma

Treatment - complete excision due to malignant potential

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6
Q

Outline the complications of acute pancreatitis

A

Early

  1. Local
    1. Peripancreatic fluid collection -> pseudocyst
    2. Necrotic collection -> WON
    3. Gastric outlet obstruction
    4. Splanchnic venous thrombosis (treat only symptomatic patients i.e. hepatic symptoms or SB symptoms with splenic V or SMV thrombus – optimise management of panc, start anticoagulation)
    5. Colonic necrosis
  2. Systemic
    1. Exacerbation of pre-existing disease
    2. Transient (<48hrs) and persistent organ failure (>48 hrs)
    3. SIRS
    4. DVT/PE

Late

  1. Pseudocyst
  2. Chronic pancreatitis
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7
Q

Causes of portal hypertension

A

Prehepatic

  • PV thrombosis
  • Splenic V thrombosis
  • PV fibrosis
  • PV blockage by infiltrative lesions
  • Splanchnic AV fistula

Intrahepatic pre-sinusoidal

  • Schistosomiasis
  • Primary biliary cholangitis
  • Primary sclerosing cholangitis
  • Intrahepatic portal vein obstruction
  • Sarcoidosis

Sinusoidal

  • Cirrhosis
  • Amiodarone
  • NAFLD

Post Sinusoidal

  • Budd-Chiari syndrome
  • Radiation injury
  • Sarcoidosis
  • IVC obstruction
  • constrictive pericarditis
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8
Q

NAFLD - types, management

A

Types

  1. NAFL - asymptomatic and no inflammation
  2. NASH - fatty liver with inflammation

Diagnosis

  1. confirm fatty liver with imaging
  2. rule out other causes - alcohol, chronic liver disease

Treatment

  • lifestyle
    • weight loss
    • avoid alcohol
  • prophylactic
    • Immunize for hepatitis
    • DM control
  • specific
    • lipid lowering
    • Elastography to rule out fibrosis
    • liver biopsy to rule out cirrhosis, monitor for HCC if cirrhosis and transplant
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9
Q

Salient features HC adenoma

A

HCA are benign tumours containing liver parenchymal tissue only (no biliary radicals, Kupffer cells, portal radicals)

aetiology and incidence

  • females 20-40 yrs
  • OCP use
  • anabolic steroids
  • metabolic disorder like DM, glycogen storage disorder

clinical features

  • abdo pain
  • may rupture (if >5cm)
  • malignant transformation (male, metabolic disorder, beta catenin +, >5cm)

Investigation

  • CT may show rim enhancement with centripetal enhancement. May be isodense in portal venous phase
  • AFP -ve
  • ?molecular testing for beta catenin if planning for surveillance – large beta catenin +ve may need excision

treatment

  • resect if
    • male
    • metabolic disorder
    • >5cm and beta catenin +
    • not regressing despite cessation of ocp
  • possibly resect
    • women >5cm
  • non-operative
    • women <5cm - stop OCP and look for regression
  • May present acutely with rupture and hemodynamic instability - attempt embolization followed by resection later.
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10
Q

What are the definitions of variceal bleeding in terms of time line

A

Bleeding episode is defined as atleast 2 units transufusion plus:

  • Systolic <100mm
  • tachycardic >100
  • Postural drop of 20 mm

Acute bleed - within 5 days of presentation

Treatment failure - rebleed <5days

Early rebleed - 5 days - 6 weeks

late rebleed - > 6 weeks

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11
Q

Outline the management of bleeding oesophageal varices

A

Management can be broadly classified under the following categories:

  • Resuscitative and temporising measures
  • Pharmacologic
  • Endoscopic
  • Interventional
  • Surgical

RESUSCIATION AND TEMPORISING MEASURES

  • Airway and O2
  • hemodynamic resus
  • correct coagulopathy
  • Antibiotics (ceftriaxone 1g x 7 days)
  • Balloon compression
    • Intubate before starting
    • Check position with fluoroscopy before full balloon inflation
    • can be left in situ for 24-48 hrs before necrosis
    • start with oesophageal pressure 35-40 mmhg, once bleeding stops bring down by 5 mm to 25 mm
    • Can be repeated if bleeding restarts

Pharmacology

  • Terlipressin 2mg iv q 4hrs

Endoscopy

  • Within 12 hrs of admission
  • EVL better than sclerotherapy
  • Cyanoacrylate better for gastric varices
  • If rebleeding occurs - repeat once more then go to TIPS or surgery

INTERVENTIONAL

  • TIPS if no contraindication
  • Attempt after 2 failed endoscopic attempts

SURGICAL (50% mortality due to encephalopathy, sepsis and renal failure - hence TIPS is favoured)

  • Shunt
    • Nonselective (portocaval shunt) - quickest but most encephalopathy
    • Selective (distal splenorenal)
    • Partial non-selective (portocaval interposition graft - decompresses portal system and allows hepatic flow)
  • Non-shunt
    • Oesophageal transection and reanastamosis after ligation of varices
    • Devascularization of GEJ (Sugiura procedure)
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12
Q

features of hemangioma (liver)

A

females 40 50yrs

>4cm giant haemangioma

CT - hypodense precontrast - centripetal spread of enhancement post contrast

MRI better than CT

Do not biopsy - bleeding.

Kasabach-merritt syndrome - giant haemangioma with consumptive coagulopathy

Treatment -

  • <4cm none
  • >10 cm consider excision
  • 4-10 cm weigh risk-benefit
  • Acute presentation with bleeding is very rare - consider embolization
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13
Q

What are the contraindications for TIPS?

A

TIPS is used in to treat variceal bleeding.

Absolute contraindications

  • Heart failure
  • Pulmonary HTN
  • TR
  • Severe sepsis

Relative contraindications

  • Liver cancer, particularly central
  • thrombocytopenia / coagulopathy
  • Portal vein thrombosis
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14
Q

for IPMN that are not being resected, how would you follow up?

A

Clinical follow-up

  • yearly review
    • abdo pain
    • jaundice
    • wt loss
    • pancreatitis

Imaging

<1cm cyst - 2-3 yrly CT/MR

1-2 cm cyst - annual CT/MR x2 and then lengthen interval

2-3cm - 3-6 EUS then lengthen interval with alternating MR–> consider surgery instead

>3cm - 3-6 monthly EUS alternating with MR -> considr surgery instead

Note: tumor markers are currently not recommeded for follow up.

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15
Q

What are the rates of invasive cancer in IPMN?

A

Main duct IPMN - 50-70%

BD-IPMN - 10-15%

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16
Q

Investigation for portal hypertension

A

If a patient has risk factors for PH (cirrhosis) and clinical manifestations (e.g ascites, varices) then investigations are not needed. Otherwise:

  1. HVPG >5mmHg
  2. US scan

Ascites

splenomegaly

nodular liver

portal vein diameter >13 mm

Portal flow velocity <12cm/sec

porto-systemic collaterals

Transient elastography

PLUS tests to determine the cause

porto-systemic collaterals

Transient elastography

PLUS tests to detremine the cause

17
Q

Indications for surgical intervention in IPMN

A

Must resect

  • all IPMN with high risk features
  • MD-IPMN 5-9mm with worrisome features
  • MD/BD IPMN with EUS-FNA suggestive of malignancy

Probabaly resect

  • BD-IPMN with worrisome features in young patient
  • >2cm cyst in young patients
  • BD_IPMN cyst enlarging by >2mm/yr

Surgical options depend on location of tumor(s), but intra-op frozen section confirming negative margin for high grade dyplasia/malignancy is needed.

options are enucleation (BD-IPMN), distal panc, pancreaticoduodenectomy, total panc (multiple tumor)

18
Q

What are simple liver cysts, differentials, when and how would you treat them?

A

No septations, contain serous fluid, do not communicate with biliary tree.

Diagnosis - CT / US

Differential

  • Cystadenoma (thick wall)
  • Hydatid
  • metastatic NET

Treatment - only if symptomatic or diagnostic uncertainty

  • non-operative - aspiration/sclerosant injection
  • Surgical - fenestration and deroofing of extrahepatic portion
19
Q

Severity assessment of pancreatitis

A

There are several ways to assess severity of pancreatitis. I use the Multiple organ dysfunction score as advised in the consensus Atlanta classification. Other methods like Glasgow score, CRP are also widely used.

Timing - On admission, and then at 24 hrs, 48 hrs and 7 days)

Mild

  1. No organ failure
  2. No local complication

Moderate

  1. Transient organ failure (<48 hrs)
  2. Local complication present

Severe

  1. Persistent organ failure (>48 hrs) (single or multiple)
20
Q

What is the pathognomonic ERCP sign of IPMN?

A

Mucin protruding from widely open papilla

21
Q

Child Pugh score

A

classifies severity of liver disease according to the

  • albumin
  • bilirubin
  • INR
  • Ascites
  • Encephalopathy

score of 5 to 6 is considered Child-Pugh class A (well-compensated disease);

7 to 9 is class B (significant functional compromise)

10 to 15 is class C (decompensated disease).

These classes correlate with one- and two-year patient survival: class A: 100 and 85%; class B: 80 and 60%; and class C: 45 and 35%

22
Q

Classify liver incidentalomas

A

Congenital

  • simple cysts
  • Polycystic liver disease
  • Haemangioma

Infective

  • Pyogenic liver abscess
  • Hydatid cyst

Neoplastic

  • Benign
    • FNH
    • Adenoma
    • Cystadenoma
    • Haemangioma
  • Malignant
    • Primary
      • HCC
      • Intrahepatic Cholangiocarcinoma
      • Cystadenocarcinoma
      • Hemangiosarcoma
    • Mets
    • Lymphoma
    • Melanoma
23
Q

Describe broadly the treatment of Gallbladder cancer

A

Staging investigations

  • CT chest abdo pelvis for nodes, vascular and peritoneal disease
  • MRI - for liver parenchyma and bile duct
  • Blood to check for jaundice (poor prognostic indicator - do not offer surgery straight up without MDM discussion)
  • Diagnostic laparoscopy
  • PET CT - controversial, use only when CT/MRI findings equivocal

Regional nodes: cystic, hepatoduodenal, hepatic artery and portal vein nodes

Non- regional nodes - para-aortic, peri-caval, SMA, coeliac nodes - involvement of these denote metastatic disease and is unresectable

Margin = 2 cm, plus clear margin on cystic duct end.

Criteria for unresectabitly:

  • unresectable hepatic mets
  • Distant mets (including non-regional nodes)
  • Peritoneal disease
  • Extensive involvement of hepatic artery, portal vein or hepatoduodenal ligament.

Treatment: surgery is the only curative treatment, but it is only offered if curative surgery can be achieved with clear margins. There is no role for palliative debulking surgery. treatment depends on the stage of disease

T1a (invades lamina propria) - cholecystectomy only

T1b (invades Muscle layer) - Controversial. Although T1b means tumour not through muscularis propria, studies have reported up to 20% LN involvement. If patient is fit then extended cholecystectomy (2cm margin on liver with non-anatomical resection of segments IVb and V) + regional lymphadenectomy

T2-3 (T2 - invades perimuscular layer T3 perforates visceral peritoneum or invades into 1 surrounding organ) - Extended chole + regional lymphadenectomy +/- bile duct resection (if negative margins can’t be achieved with chole on cystic duct stump on frozen section)

T4 (invades 2 or more surrounding organs)- palliative chemo +/- radiation

Jaundiced patient - relative contraindication to surgery due to poor prognosis - should be discussed in MDM, jaundice can be relieved with stenting post staging.

Palliative chemo -

  • gemcitabine
  • capox
  • folfox

Radio

  • EBRT + 5FU

Biliary drainage

relieve bowel obstruction - palliative bypass

24
Q

What are worrisome signs in pancreatitis secondary to hypertriglyceridemia? How does the treatment differ in presence of worrisome signs?

A

Worrisome signs

  • hypocalcemia
  • Lactic acidosis
  • organ failure
  • worsening inflammation (Temp >38.5/<35; Pulse >90, RR>20; Paco2 <32 mmHg; WCC >12 or <4)

Patients with worrisome signs need plasmapheresis.

patients without worrisome signs can be treated with insulin dextrose (.1-.3mg/kg/hr - same as DKA protocol)

25
Q

What are the differences in the clinical presentation of SCA and MCN of pancreas?

A

Serous cyst adenoma

  • Mostly women (74%)
  • age - mid 70s
  • evenly distributed through pancreas
  • commonly associated with Von-hippel-Lindau (cysts in panc and kidney –> send for genetic assessment)
  • Can be multiple
  • rarely undergo malignant transformation

Mucinous cyst neoplasm

  • Mucin containing neoplasm
  • must contain ovarian stroma (densely packed plump spindle cells)
  • Exclusively in women
  • age 50s
  • in tail/distal panc
  • very high malignant potential
26
Q

Diagnostic features of non IPMN panc cysts

A

SCA (resect only if symptomatic)

  • Can involve any part of panc
  • associated with other cystic disease e.g. VHL
  • Microcystic pattern
  • central calcification (sunburst)
  • No duct connection
  • EUS-FNA
    • Viscosity <1.6
    • Lipase >6000
    • CEA low (<5ng/ml)
  • Molecular marker - VHL +, GNAS-ve, Kras -ve

MCN (always resect, however newer studies suggest resecting >3cm like BD-IPMN)

  • mainly in body or tail
  • No connection to duct
  • Macrocystic pattern
  • Peripheral (egg cell calc) - marker of malignancy
  • Ovarian stroma present - differentiates from IPMN
  • viscosity >1.6
  • CEA high >190
  • Genetic marker - Kras +ve

Pseudocyst

  • Thick inflammatory wall
  • History of pancreatitis or pancreatic trauma
  • Connected to the duct
  • viscosity <1.6
  • Lipase >6000
  • CEA low (usually <5 but if >480 - repeat EUS in 3-6 months)

SOLID PSEUDOPAPILLARY TUMOR

  • Young females <35 yrs
  • solid and cystic components +/- calcifications
  • Body and tail
  • no definitive markers
  • Resect due to high malignant potential
27
Q

what is the definition of portal hypertension

A

PV Hypertension hepatic vein pressure gradient >5mm Hg.

Varices develop at >10 mmHg

Varices bleed >12mmHg

Decompensation >15 mm Hg

28
Q

how are pancreatic neuroendocrine tumors graded?

A

Several systems exist. I use the WHO system which uses degree of differentiation, Ki67 and mitotic index.

Well differentiated

  • Pan NEN Grade 1 (low) - Ki 67 <3%, Mitotic index (per 50 hpf) <2
  • Pan NEN Grade 2 (intermediate) - Ki 67 3- 20%, Mitotic index 2-20
  • Pan NEN Grade 3 (high) - Ki 67 >20%, mitotic index >20

Poorly differentiated

  • Pan NEC Grade 3 (high) Ki 67 >20%, mitotic count >20
29
Q

Features of FNH

A

Lobulated lesion around a central scar

females 20-40 yrs.

etiology - injury to portal tract >> av fistula >> hyperperfusion of local arterioles >> oxidative stress>> stellate cells in liver react and form scar

Investigation

  • CT - hyperintense in arterial phase with hypointense central scar
  • Biopsy if concern

Treatment

  • no malignant potential
  • ensure diagnosis correct - may rarely need resection for diagnosis
  • asymptomatic patients - no treatment
  • symptomatic patients - look for other causes
30
Q

Differentials for cystic liver lesions

A

Congenital

  • single simple cyst
  • polycystic liver disease
  • Type IVa and type V choledochal cysts

Infective

  • Pyogenic liver abscess
  • Hydatid cyst

Neoplastic

  • Benign - cystadenoma
  • Malignant - cystadenocarcinomna, metastatic NET
31
Q

Pathophysiology of hepatorenal syndrome

A

It is not well understood but endotoxemia resulting in renal vasoconstriction has been implicated.

Endotoxins in the gut are usually prevented from being absorbed by detergent effect of bile salts. In obstructive jaundice, this protective affect is lost leading to endotoxemia which causes renal vasoconstriction through pathways that are not fully understood (IL have been implicated)

32
Q

What are the clinical symptoms of IPMN

A

They can be asymptomatic and incidentally picked up on un related scans.

  • Abdominal pain
  • weight loss
  • Jaundice in 16% (c.f. panc cancer - painless jaundice)
  • History of smoking, pancreatitis
33
Q

Molecular markers of pancreatic cysts

A

GNAS - IPMN

KRAS - IPMN and MCN

VHL - Serous cyst (if KRAS and GNAS negative)

34
Q

MELD score

A

Statistical score that was originally used to predict mortality after TIPS but studies have validated it use as a reliable scoring system for selecting patients for liver transplant and risk prediction for other surgical interventions in patients with cirrhosis.

Components

  • Bilirubin
  • INR
  • Creatinine
  • more recently Na has been added

It has been suggested that patients with a MELD score below 10 can undergo elective surgery, those with a MELD score of 10 to 15 may undergo elective surgery with caution, and those with a MELD score >15 should not undergo elective surgery

35
Q

What are the indications for EUS -FNA in IPMN?

A

There is some controversy between accepted guidelines for investigation and management of IPMNs. EUS is used for surveillance and initial work up of IPMN (where surgery is not yet indicated)

Initial evaluation: (to look for features of malignancy)

  • BD-IPMN with worrisome features
  • MD IPMN with duct size 5-9 mm

Surveillance

  • Cysts >2cm

FNA of cyst content has sensitivity of 65% and specificity if 91% for detecting malignant IPMN. It can sometimes help in detecting the type of cyst

+ve mucin stain = IPMN or MCN (mucinous cystic neo)

+ve inflammatory cells = pseudocyst

+ve Amylase = Pseudocyst (very high), IPMN (high)

+ve CEA = IPMN or MCN

FNA of cyst content has sensitivity of 65% and specificity if 91% for detecting malignant IPMN. It can sometimes help in detecting the type of cyst

+ve mucin stain = IPMN or MCN (mucinous cystic neo)

+ve inflammatory cells = pseudocyst

+ve Amylase = Pseudocyst (very high), IPMN (high)

+ve CEA = IPMN or MCN

FRACS Revision (12 decks)

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