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An Approach to Obstructive Jaundice

Doctor, my eyes are yellow ! An Approach to Obstructive Jaundice

Introduction
Clinical Approach
Clinical Examination
Causes of Jaundice
Evaluation Priorities
Differential Diagnosis


Context : Casualty / OPD

Introduction

Patients with obstructive jaundice are usually admitted to the hospital. They are vulnerable to sepsis, dehydration, and pose complex diagnostic dilemmas that need to be untangled. They are best admitted and fast-tracked for investigation.

Management depends on the cause of jaundice. In practice, pre-hepatic and hepatic causes of jaundice are managed by a medical team, and the surgical teams take responsibility for the patients with “post hepatic”, cholestatic jaundice. Biliary obstruction is the aetiology in approximately 30% of all patients presenting with jaundice.

Clinical Approach

Try to decide if jaundice is a brand new symptom, or if it reflects a progression of long-standing disease.

ASK ABOUT JAUNDICE THOROUGHLY : define the presenting complaint

Temporal elements
The onset and duration of jaundice are essential
Is this a gradual and progressive symptom ? = Neoplastic cause
Does jaundice fluctuate, and is of short duration = ? biliary stones
Associated symptoms
Is there pain?
Severe intermittent pain & rigors = cholangitis, stones
Painless = early neoplasm
Severe persistent pain (often felt in the lower back) and LOW = malignancy
Pruritus - this has little relation to the cause of jaundice, but it is a symptom that needs intervention for palliation
Is the gut function normal ?
Pale stool and dark urine are characteristic of cholestasis,
Steatorrhoea may suggest chronic pancreatitis
Nausea and vomiting are associated with either hepatitis or early duodenal / gastric outlet obstruction
Precipitants or aetiological factors
Ask about the use of medications, herbal medications, dietary
supplements, and recreational drugs
? any hepatitis risk factors (e.g., travel, possible IV drugs exposure)
Exposure to hepatotoxic substances, especially alcohol
History of inherited disorders, including liver diseases & haemolytic disorders
HIV status
NB - history of abdominal operations, including gallbladder surgery

Clinical Examination

General examination
Jaundice is the dominant sign - it is typically noted in the sclerae and
confirmed by a yellow tinge in the frenulum of the tongue

Pyrexia = cholangitis, sepsis, hepatitis
Anaemia = malignancy , malnutrition
Dehydration/malnutrition = malignancy, sepsis
Peripheral adenopathy = malignancy
Looks for signs of pruritus, and features of chronic liver failure such as spider naevi, bruising, gynecomastia, testicular atrophy, oedema, palmar erythema, Dupuytren’s contracture, hepatic fetor, flap tremor, hepatic enchephalopathy 
Nutritional deficiencies are common in chronic alcoholics (Pellagra/ Wernicke’s/ Peripheral Neuropathy)
Abdominal examination
Courvoisier sign (a palpable non-tender gallbladder, caused by obstruction distal to the take-off of the cystic duct by malignancy)
Signs of chronic liver failure/portal hypertension such as ascites, distended abdominal wall veins, splenomegaly
A palpable mass and ascites may be a sign of advanced malignancy 
look for scars of previous operations. A laparoscopic incision can be easily missed! 
Tenderness in the right upper quadrant
Rectal
Blood PR = malena may be due to bleeding oesophageal varices, bright red blood may be due to haemorrhoids associated with portal hypertension

Causes of Jaundice


Unconjugated
hyperbilirubinaemia

Mixed
hyperbilirubinaemia

Conjugated
hyperbilirubinaemia

Common causes
Haemolysis
Extravasation of blood into the tissue
Stress situations (e.g. sepsis)
Viral/ alcoholic hepatitis Non-alcoholic Steatohepatitis 
Drugs and toxins
Cirrhosis
Biliary obstruction
(gallstones, pancreatic or biliary malignancy, chronic pancreatitis) typically managed by surgical teams
Rare causes
Dyserythropoiesis
Impaired hepatic bilirubin
uptake
Impaired bilirubin conjugation (Gilbert syndrome)
Ischemic hepatitis, Liver infiltration, Inherited disorders (e.g. Dubin-
Johnson syndrome, Rotor syndrome), TPN, Postoperative jaundice
Biliary obstruction Iatrogenic stricture, sclerosing cholangitis, hilar adenopathy, HIV cholangiopathy, choledochal cyst 
Cholestasis
Severe sepsis, Primary biliary cirrhosis, Viral, Drugs, progressive familial Intrahepatic cholestasis, Intrahepatic cholestasis of pregnancy

Evaluation Priorities

How sick is the patient ?
Do I need to start a resuscitation protocol?
(Needed in cases of cholangitis, or severe pancreatitis)
Hb, blood gas, lactate, U&E, WBC, blood culture
Start IV fluid rehydration, IV antibiotics, Vit K
What is the diagnosis ?
exclude “medical causes” of jaundice ?
Check urine
Serum U&E, LFT’s, lipase, hepatitis serology,
AFP
CXR
Abdominal US

Differential Diagnosis 

The three key steps are:


Diagnostic steps
Typical findings of biliary obstruction
1
Is jaundice cholestatic?
(Or is it mixed or unconjugated?)
Total bilirubin elevated >70% Conjugated transaminases mildly raised or normal ALP and GGT markedly elevated
2
Differentiate medical causes of Cholestatic Jaundice” (typically Hepatic Diseases) 
from 
Biliary Obstruction
US shows biliary dilatation (Intra/extrahepatic)
US is very accurate at making this distinction
Normal CBD < 7mm
3
Differentiate “benign” obstructive jaundice (typically caused by biliary calculi)
from
“malignant” obstructive Jaundice (most commonly caused by carcinoma of the head of the pancreas)
Visible gallstones, shrunken gall bladder, dilated CBD = choledocholithiasis



Distended gall bladder, dilated CBD, no gallstones = malignant biliary obstruction

The patient has unequivocal biliary obstruction - what next ?

Common causes
Clinical clues
Investigations
Choledocholithiasis
Short history, may be relapsing,
associated with rigors of cholangitis No loss of weight, often obese Intermittent severe pain common

Bilirubin moderate to high
WBC often raised
US – galls stones visible in CBD & in
gall bladder

Periampullary Carcinomas (HOP
carcinoma, distal cholangio, ampullary carcinoma
)
Loss of weight, progressive jaundice
Painless unless advanced disease Courvoisier’s law- palpable gall bladder

Bilirubin high to very high
WBC normal may be anaemic
US – NO galls stones visible, Intra
and extrahepatic biliary dilatation &
gall bladder
May have liver mets and ascites
HOP mass difficult to see on US
Chronic pancreatitis
Loss of weight, progressive jaundice
Recurrent pain due to chronic
pancreatitis may have steatorrhea
Courvoisier’s law - palpable gall bladder +

Bilirubin high to very high
WBC normal, may be anaemic
US – NO galls stones visible, Intra
and extrahepatic biliary dilatation &
gall bladder, pseudocyst in HOP
visible
HOP mass may be difficult to
differentiate from malignancy

Uncommon causes
Clinical clues
Investigations


In the lumen ?
Ascaris infestation blocked stent
In the wall ?
HIV cholangiopathy/ Iatrogenic stricture/ Klatskin’s/ adenopathy/sclerosing cholangitis/ choldeochal cyst/ Mirizzi syndrome
Outside the wall ?
Portal adenopathy
Benign biliary stricture
The commonest cause is Iatrogenic Stricture post biliary surgery
Gall bladder absent
Intrahepatic dilatation

Klatskin tumours
(”hilar” cholangiocarcinoma)
Loss of weight, progressive jaundice
Painless unless advanced disease
No palpable gall bladder

Bilirubin high to very high
WBC normal, may be anaemic
US – NO galls stones visible, dilated
intrahepatic ducts, but CBD & gall
bladder normal
May have liver mets and ascites
Portal adenopathy
(metastases, lymphoma, rarely TB)
Loss of weight, progressive jaundice
Primary carcinoma usually obvious
No palpable gall bladder

Intrahepatic biliary dilatation
Nodal mass in porta hepatis

Mirizzi syndrome
Cholangitis, tender gall bladder, recurrent bouts of right UQ pain
Bilirubin moderate
WBC elevated
US – gall stones visible, gall bladder
thick-walled and edematous,
Intrahepatic biliary dilatation

HIV cholangiopathy
RUQ pain, chronic diarrhoea, not
always jaundiced, HIV with severe immunosuppression

Multifocal strictures, segmental
dilations of the biliary tract
Evidence of cytomegalovirus (CMV),
cryptosporidium, microsporidium
infections
Sclerosing cholangitis
Intermittent sepsis and jaundice,
pruritus, fatigue
Associated with ulcerative colitis
(75% of cases)

Bile duct wall thickening and focal
bile duct dilations may be normal
early on

Ascaris infestation
Children, poor socio-economic background
Intra and extrahepatic biliary
dilatation & gall bladder, visible
Ascaris in CBD

Choledochal (Biliary)
cyst

Children and young adults,
jaundice, pruritus, cholangitis

Intra and / or extrahepatic cystic
dilatations of the biliary system

Does the patient have cholangitis ? 

Any of the above causes of biliary obstruction can be complicated by sepsis, typically with gram –'ve organisms.

Cholangitis
Commonest precipitant :
  • gallstones
  • blocked biliary stent
  • ERCP without
    adequate drainage
Requires biliary obstruction
and infected bile
Charcot’s triad- rigors, pain,
jaundice

It is a clinical diagnosis
Need to have a high index of suspicion (do not delay starting treatment while waiting for investigations)
Can develop septic shock

Bilirubin moderate to high
WBC very high
US – depends on the primary
cause of biliary obstruction, but
may have cholangitic liver
abscesses

I am not quite sure what is going on, and I need more information

HPB conditions are heavily reliant on high-quality imaging, and through multidisciplinary review. A lot of conditions are inaccessible and do not allow histological confirmation of the diagnosis, so therapeutic decisions are frequently based on a clinical and radiological evaluation only.

Different units adopt protocols to the availability of equipment, local skill and expertise.

Most diagnoses, other than obvious CBD stones, need more imaging than the US alone.

It is important to recognize and treat cholangitis early. Avoid dehydration and correct coagulopathy while awaiting further investigations.

Imaging investigations in obstructive jaundice

Test
Ideal context
Issues to consider
Abdominal US
Best in thin patients, excellent to confirm calculi, biliary dilatation, gall bladder inflammation
The most widely used FIRST imaging test 
Operator dependent, but easily available and relatively cheap. Not good to image pancreas or retroperitoneum.
Abdominal CT
Best for obese patients, or suspected retroperitoneal or pancreatic pathology
Essential investigation in cases of
suspected malignancy; moderately expensive, uses IV contrast
MRCP
A non-invasive way of imaging biliary
system
No risk to the patient
Not therapeutic (diagnostic information only)
Increasingly available
Expensive, often unnecessary if a
PTC or ERCP is clearly indicated
ERCP
Excellent imaging of biliary and pancreatic system& allows therapeutic intervention.
Ideal to extract CBD stones or to palliate jaundice in advanced peri-ampullary malignancy
Invasive, potential complications
include pancreatitis, bleeding,
perforation and cholangitis.
Requires advanced endoscopic
expertise & hospital admission
PTC
Excellent imaging of intrahepatic
biliary system & allows therapeutic
intervention
Best to palliate jaundice in advanced
Klatskin tumours
Invasive, potential complications
include cholangitis and bleeding
Requires advanced radiological expertise & hospital admission.
Endoscopic US
Excellent visualization of pancreas and distal CBD
Best tool to guide directed biopsy of
suspicious lesions in the pancreas
Limited availability and skill
Invasive potential complications include pancreatitis, bleeding, perforation
Requires advanced endoscopic expertise
Laparoscopy
Useful to look for early peritoneal
metastases in suspected malignant
disease
Commits patient to surgery, uses
theatre resources, requires surgical
expertise

The role of the junior surgeon on this context is the ward care of the patients (highly prone to intermittent sepsis, renal failure and nutritional deficit), and to manage sensibly all the complex permutations of investigations available. Always discuss with the senior consultant prior to booking investigations, particularly invasive procedures like ERCP and PTC.

SHOULD I OPERATE OR NOT ?

The decision to intervene surgically, or endoscopically is complex and always requires senior supervision.
You have to know when to intervene and when not to. There is no substitute for clinical experience, and the heterogeneity of clinical conditions makes a one-size-fits-all approach impossible.
All cases need thorough review, and great harm comes from inappropriate surgery, especially in advanced malignancy.

These are some thoughts to guide your decision making

Common diagnosis
Early intervention
Definitive treatment
Choledocholithiasis
ERCP & stone extraction
Laparoscopic cholecystectomy, once CBD is cleared
Cholangitis
Initial resuscitation with IV antibitiotics and IV fluids. 
Correct coagulopathy.
If not improving may require organ support and ICU referral.
Adequate biliary drainage (e.g. stone extraction or stenting, usually via ERCP)
Periampullary carcinomas (HOP carcinoma, distal cholangio, ampullary carcinoma)
ERCP & stent
...but inappropriate deployment of CBD stent can trigger sepsis and delay curative surgery
A proportion of cases with early disease may be cured by surgical resection (pancreaticoduodenectomy or Whipple’s procedure.
Incurable cases are well palliated by ERCP stent
Chronic pancreatitis
ERCP only if cholangitis present
Persistent jaundice requires
intervention
Inflammatory mass or stricture
  • Surgical management
    - Resection e.g Whipple’s
    - Drainage procedure e.g
    Frey procedure

    - Biliary bypass e.g
    hepaticojejenostomy)
  • Endoscopic management
    - Biliary stent
Pseudocyst
  • Surgical management
    - (internal drainage e.g.
    cystgastrostomy)
  • Endoscopic management
    - EUS guided drainage of
    the cyst

    - Biliary stent
NeoHealth® ID 19213 · updated 9/15/2021