Disease of the biliary tree

 Acute cholecystitis
 Cholelithiasis
 Cholangitis

Acute cholecystitis
Cholecystitis is the inflammation of the gallbladder, usually caused by gallstones blocking the
cystic duct or the common bile duct. It can also occur due to other factors like infections or
tumors
Clinical Features

 Abdominal pain: The most common symptom of cholecystitis is a sudden, severe pain in
the upper right or middle abdomen. The pain may also radiate to the right shoulder or
back and is often triggered or exacerbated by eating fatty or greasy foods.

 Fever and chills: Inflammation of the gallbladder can lead to fever and chills, indicating
an infectious component.

 Nausea and vomiting: Many individuals with cholecystitis experience nausea and
vomiting, especially after eating.

 Tenderness and rigidity: The abdomen may be tender to touch, and in severe cases,
there may be abdominal rigidity or guarding.
 Jaundice: If the common bile duct is obstructed by gallstones, jaundice (yellowing of the
skin and eyes) may develop.

Investigations
 Ultrasound: Ultrasonography is often the initial imaging modality of choice to diagnose
cholecystitis. It can reveal gallstones, gallbladder wall thickening, and signs of
inflammation.
 Blood tests: Blood work may show elevated levels of white blood cells (indicating
infection), liver function tests, and bilirubin levels in cases where the common bile duct
is obstructed.
 HIDA scan (cholescintigraphy): If the ultrasound is inconclusive or the diagnosis is still
uncertain, a HIDA scan may be performed. This nuclear medicine test can show the
function of the gallbladder and how well it is draining.
 CT scan or MRI: In more complicated cases or when other diagnoses are being
considered, a CT scan or MRI may be ordered to get more detailed images of the
gallbladder and surrounding structures.
Treatment

 NPO and hydration: In the acute phase of cholecystitis, patients are often advised to be
"nothing by mouth" (NPO) to rest the gallbladder. Intravenous fluids may be given to
maintain hydration.
 Pain management: Pain relief is provided using medications like acetaminophen or
nonsteroidal anti-inflammatory drugs (NSAIDs).
 Antibiotics: If there is evidence of infection, antibiotics are prescribed to combat the
infection.
 Cholecystectomy: The definitive treatment for cholecystitis is the removal of the
gallbladder. This can be done through traditional open surgery or laparoscopic surgery,
which is less invasive and has a faster recovery time.
 ERCP (Endoscopic Retrograde Cholangiopancreatography): In cases where gallstones
have obstructed the common bile duct, an ERCP procedure may be done to remove the
stones and relieve the blockage before or during the cholecystectomy.
 Percutaneous cholecystostomy: In critically ill patients who cannot undergo surgery
immediately, a tube may be inserted through the skin to drain the infected bile from the
gallbladder.

CASE 1
A 55-year-old female presents to the emergency department with complaints of sudden-onset
severe abdominal pain in the right upper quadrant that has been progressively worsening over
the past 24 hours. She describes the pain as sharp and constant, radiating to her right shoulder.
On examination, the patient appears to be in distress, and her vital signs reveal a temperature
of 38.5°C (101.3°F), heart rate of 100 beats per minute, and blood pressure of 140/90 mmHg.
Physical examination of the abdomen shows tenderness and guarding in the right upper
quadrant, and Murphy's sign is positive. Laboratory investigations demonstrate an elevated
white blood cell count and increased levels of liver enzymes and bilirubin.

a)Based on the clinical presentation and findings, what is the most likely diagnosis and why
b)Describe the important features that support your diagnosis.
c) What further investigations would you recommend, and what would be the appropriate initial
management for this patient?

a) The most likely diagnosis for this patient is acute cholecystitis.

b) Important features supporting the diagnosis of acute cholecystitis in this patient include:

Sudden-onset severe abdominal pain: The patient's complaint of sudden and severe pain in the
right upper quadrant is a typical symptom of acute cholecystitis. The constant pain that radiates
to the right shoulder is known as "referred pain" and is often seen in gallbladder inflammation.
Positive Murphy's sign: The tenderness and guarding in the right upper quadrant along with a
positive Murphy's sign (pain upon deep inspiration while the examiner's fingers are placed
below the right costal margin at the mid-clavicular line) are characteristic findings of acute
cholecystitis.

Fever and elevated vital signs: The presence of fever (temperature of 38.5°C) and an elevated
heart rate (100 beats per minute) suggests an infectious component, which can occur with
acute cholecystitis.

Laboratory findings: The elevated white blood cell count indicates an inflammatory response,
while increased levels of liver enzymes and bilirubin may suggest bile duct obstruction due to
gallstones.
c) Further investigations and initial management:
stabilization first

Laboratory Investigations: Blood tests, including complete blood count (CBC), liver function
tests, and inflammatory markers (e.g., C-reactive protein), should be performed to assess the
severity of the inflammation and monitor for any complications.

Ultrasound: An abdominal ultrasound should be performed promptly to confirm the diagnosis

Blood cultures: Given the patient's fever and clinical signs of infection, blood cultures should be
obtained to identify the causative organism if there is an underlying infection.

Liver function tests: More detailed liver function tests can be conducted to assess the extent of
liver involvement and identify any other liver-related issues.

NPO and IV fluids: The patient should be kept nil per os (NPO), avoiding oral intake to rest the
gallbladder. Intravenous fluids should be administered to maintain hydration and correct any
electrolyte imbalances.

Pain management: Pain relief should be provided with appropriate medications such as
acetaminophen or NSAIDs.

Antibiotics: Empiric antibiotic therapy should be initiated to cover potential bacterial infections
associated with acute cholecystitis.

Surgical consultation: The patient should be evaluated by a surgeon to determine the need for
cholecystectomy (surgical removal of the gallbladder). In cases of severe inflammation or
complications, urgent surgery may be required.

Cholelithiasis
Clinical Features of Cholelithiasis (Gallstones)
Asymptomatic: Many individuals with gallstones may not experience any symptoms and may
only discover the presence of gallstones incidentally during imaging tests for other conditions.

Biliary Colic: The most common symptom of cholelithiasis is biliary colic, which is characterized
by sudden and severe pain in the upper right or middle abdomen. The pain can be intermittent
and may last from a few minutes to several hours. It often occurs after eating a fatty or heavy
meal and may radiate to the back or right shoulder.

Nausea and Vomiting: Some people with gallstones may experience nausea and vomiting,
especially during episodes of biliary colic.

Indigestion and Gas: Difficulty in digesting fatty foods and excessive gas or bloating may be
observed in some individuals with cholelithiasis.

Jaundice: In certain cases, gallstones may obstruct the common bile duct, leading to jaundice,
which is characterized by yellowing of the skin and eyes.

Fever and Chills: If gallstones lead to inflammation or infection of the gallbladder (cholecystitis),
patients may experience fever and chills.

Investigations for Cholelithiasis

Abdominal Ultrasound: This is the most commonly used imaging test to diagnose gallstones. It
can detect the presence of gallstones in the gallbladder and determine their size and location.
Abdominal CT Scan or MRI: In some cases, a CT scan or MRI may be required to get more
detailed images of the gallbladder and bile ducts, especially if there are complications or
suspicion of other issues.
HIDA Scan (Cholescintigraphy): This nuclear medicine test can assess the function of the
gallbladder and evaluate how well it is emptying. It is especially useful in cases where the
diagnosis is uncertain or when gallbladder dysfunction is suspected.
Endoscopic Retrograde Cholangiopancreatography (ERCP): ERCP is performed when there is a
suspicion of gallstones obstructing the common bile duct. It allows both diagnostic visualization
and therapeutic intervention to remove or break down the stones.

Treatment for Cholelithiasis

Watchful Waiting: Asymptomatic gallstones may not require immediate treatment. In such
cases, regular follow-up with a healthcare provider is recommended to monitor for any changes
or symptoms.
Pain Management: For individuals experiencing biliary colic, pain relief can be provided using
medications such as acetaminophen or NSAIDs during episodes of pain.
Lifestyle Changes: Making dietary adjustments, including reducing the intake of fatty foods, may
help reduce the frequency and severity of biliary colic attacks.
Cholecystectomy: For symptomatic or complicated gallstones, the definitive treatment is the
surgical removal of the gallbladder, known as cholecystectomy. This can be performed using
traditional open surgery or laparoscopic surgery, which is less invasive and has a faster recovery
time.

Endoscopic Stone Removal: For cases where gallstones are obstructing the common bile duct,
ERCP can be used to remove or break down the stones, often in combination with stent
placement to relieve the blockage.

CASE
A 45-year-old female presents to the emergency department with severe right upper quadrant
abdominal pain that radiates to her back. The pain started approximately 6 hours ago and has
been progressively worsening. She describes the pain as colicky and states that it is exacerbated
by fatty meals. Upon examination, her temperature is 38.5°C (101.3°F), and there is tenderness
and guarding in the right upper quadrant of the abdomen. Laboratory tests reveal elevated
white blood cell count and increased levels of alkaline phosphatase and bilirubin. An abdominal
ultrasound is performed, which shows multiple gallstones in the gallbladder with associated
gallbladder wall thickening.
a)What is the most likely diagnosis,
b) what are the appropriate next steps in the management of this patient?

a) The most likely diagnosis for this patient is cholelithiasis, which refers to the presence of
gallstones in the gallbladder.

b) Investigation:
The abdominal ultrasound revealed multiple gallstones in the gallbladder, supporting the
diagnosis of cholelithiasis.

c) Management:
The appropriate management for cholelithiasis, especially when it presents with acute
symptoms, would include:

Pain Management: Provide pain relief to alleviate the severe abdominal pain. Medications like
acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs) can be used.

NPO and Hydration: Advise the patient to be "nothing by mouth" (NPO) to rest the gallbladder
and prevent further stimulation of bile secretion. Intravenous fluids should be given to maintain
hydration.

Surgical Consultation: While not all cases of cholelithiasis require surgery, if the patient has
persistent or severe symptoms, a surgical consultation may be necessary. In cases of acute
cholecystitis (inflammation of the gallbladder due to gallstones), cholecystectomy (surgical
removal of the gallbladder) may be recommended to prevent complications.

Monitoring: Keep the patient under close observation to assess the response to treatment and
ensure the condition is not worsening.
CASE 3
A 42-year-old male presents with a history of recurrent episodes of jaundice, itching, and
fatigue over the past several months. On physical examination, there is no abdominal
tenderness or hepatomegaly. Laboratory investigations reveal elevated liver enzymes (AST and
ALT) and markedly elevated alkaline phosphatase and bilirubin levels. Serological tests for viral
hepatitis and autoimmune liver diseases are negative. Imaging studies demonstrate multifocal
strictures and dilations of the intrahepatic and extrahepatic bile ducts. A liver biopsy shows
evidence of onion skin fibrosis and ductular proliferation.

Based on the clinical presentation, laboratory findings, and imaging results, what is the most
likely diagnosis and why What further investigations may be required to confirm the diagnosis,
and what are the treatment options for this condition?

Based on the clinical presentation, laboratory findings, and imaging results, the most likely
diagnosis for this patient is Primary Sclerosing Cholangitis (PSC).

Reasoning for the Diagnosis

Recurrent episodes of jaundice, itching, and fatigue: These symptoms are characteristic of
cholestasis, which is a common feature of PSC due to the inflammation and strictures of the bile
ducts.

Elevated liver enzymes (AST and ALT): This is indicative of liver cell damage or inflammation,
which can occur in PSC due to the immune-mediated destruction of the bile ducts.

Markedly elevated alkaline phosphatase and bilirubin levels: Elevated alkaline phosphatase
and bilirubin are typical markers of cholestasis, which is a prominent feature of PSC.

Negative serological tests for viral hepatitis and autoimmune liver diseases: The absence of
viral hepatitis and autoimmune markers helps to rule out other potential causes of liver disease,
pointing towards a diagnosis of PSC.
Imaging studies demonstrating multifocal strictures and dilations of bile ducts: This "beading"
appearance on imaging (e.g., MRCP or ERCP) is characteristic of PSC and represents the
irregular narrowing and dilation of the intrahepatic and extrahepatic bile ducts.

Liver biopsy showing onion skin fibrosis and ductular proliferation: These histological findings
are consistent with PSC and reflect the chronic inflammation, fibrosis, and bile duct injury seen
in the disease.
Further Investigations to Confirm the Diagnosis:
Endoscopic Retrograde Cholangiopancreatography (ERCP): This invasive procedure can provide
a detailed assessment of the bile ducts' anatomy and confirm the presence of multifocal
strictures and dilations, characteristic of PSC.
Magnetic Resonance Cholangiopancreatography (MRCP): A non-invasive imaging modality that
can also visualize the bile ducts and demonstrate the typical "beading" appearance.

Liver Function Tests (LFTs): Regular monitoring of liver enzymes, alkaline phosphatase, and
bilirubin levels to assess disease progression and response to treatment.
IgG4 levels: Although the serological tests for autoimmune liver diseases were negative, it might
be relevant to measure IgG4 levels to exclude IgG4-related sclerosing cholangitis, a condition
with some overlapping features.
Treatment Options:

Ursodeoxycholic Acid (UDCA): It is the first-line therapy for PSC and can help improve liver
function tests and delay disease progression, although it does not cure the condition.
Endoscopic Therapy: Endoscopic dilation or stenting of strictures can be performed to alleviate
symptoms and improve bile flow.

Liver Transplantation: In advanced stages or when complications like liver failure or