Miscelleneous of Liver

Peliosis Hepatica

Definition:

Peliosis Hepatica is an uncommon disorder characterized by the presence of multiple, small, blood-filled sinuses within the liver parenchyma.

Risk Factors:

Commonly seen in:
- Immunocompromised patients.
- Long-term use of medications such as:
  - Steroids.
  - Androgens.
  - Azathioprine.
  - Tamoxifen.
  - Estrogen.
  - Vitamin A.

Radiographic Features:

CT and MRI:
- Shows diffuse hypodense areas spread throughout the liver.
- On imaging, enhancement may occur:
  - Early images show enhancement.
  - On delayed imaging, enhancement may progress from central to peripheral.

MCQ Discussion - Primary Sinusoidal Dilatation of Liver

Question: Primary sinusoidal dilatation of the liver is also known as:

a) Hepar lobatum

b) Peliosis hepatica

c) Von-Meyerburg complex

d) Caroli's disease

Answer: B

Peliosis Hepatica refers to primary sinusoidal dilatation of the liver, characterized by blood-filled cavities or dilated sinusoids within the liver parenchyma.

Definitions of Other Options:

a) Hepar Lobatum:
- A condition where the liver becomes lobulated due to chronic syphilis. It results in irregular fibrotic areas causing the liver to take on a lobular appearance.
c) Von-Meyerburg Complex:
- This refers to small bile duct hamartomas that are benign lesions of the liver. They are typically an incidental finding and have no malignant potential.
d) Caroli’s Disease:
- A rare congenital disorder characterized by segmental dilation of the intrahepatic bile ducts, often leading to recurrent infections, cholangitis, and stone formation.

MCQ Discussion - Peliosis Hepatica

Question: All are true about peliosis hepatica except:

a) Bile filled cyst in liver parenchyma

b) Hyperintense in T2 weighted imaging

c) Associated with TB, malignancy, and steroid intake

d) Treatment is withdrawal of causative agent

Answer: A

Peliosis hepatica is characterized by blood-filled cysts, not bile-filled cysts. Hence, A is the correct answer.

Key Points:

Peliosis hepatica involves blood-filled cysts within the liver parenchyma.
It appears hyperintense on T2-weighted MRI imaging due to the blood content (Answer B is true).
It is associated with tuberculosis (TB), malignancies, and steroid use (Answer C is true).
The primary treatment is often withdrawal of the causative agent, such as stopping steroids (Answer D is true).

Hemochromatosis

MCQ Discussion - Diagnosis of Middle-Aged Man with Weakness, Fatigue, Hyperpigmentation, Hepatomegaly, and Hypoglycemia

Question: A middle-aged man presents with complaints of weakness, fatigue, and hyperpigmentation. On examination, hepatomegaly and hypoglycemia are present. What is the most likely diagnosis?

a) Addison's disease

b) Hemochromatosis

c) IDDM (Insulin-Dependent Diabetes Mellitus)

d) Cushing's syndrome

Answer: B

Hemochromatosis is the most likely diagnosis. It is a disorder of iron overload that commonly presents with:
- Weakness, fatigue, and hyperpigmentation (due to iron deposits in the skin).
- Hepatomegaly (due to iron accumulation in the liver).
- Hypoglycemia can occur due to liver dysfunction affecting glucose metabolism.

Key Points:

Hemochromatosis:
- A genetic condition characterized by excessive iron absorption and deposition in organs like the liver, skin, and pancreas.
- Clinical features include hyperpigmentation, hepatomegaly, fatigue, and weakness.
- Can also lead to diabetes (bronze diabetes) and cardiac dysfunction.
Addison's Disease:
- Also causes hyperpigmentation, but typically presents with hypotension and electrolyte imbalances, not hepatomegaly or hypoglycemia.
IDDM:
- Primarily presents with polyuria, polydipsia, and weight loss, not hepatomegaly or hyperpigmentation.
Cushing's Syndrome:
- Causes central obesity, striae, hypertension, and muscle weakness, but hyperpigmentation and hepatomegaly are less common.

Wilson's Disease

Overview:

Autosomal recessive disorder of copper metabolism.
Caused by a defect on chromosome 13q in the ATP7B gene.
Characterized by excessive copper deposition in the liver, brain, and other tissues.

Key Features:

Hepatic copper concentration > 250 mcg/g of dry weight.
Common clinical presentations include liver damage (hepatitis, cirrhosis) and neurological symptoms (extrapyramidal signs, tremors, psychiatric disturbances).
Kayser-Fleischer rings are a hallmark finding in the cornea due to copper deposition.

Management:

Chelating Agents:
- Penicillamine: First-line therapy for enhancing urinary excretion of copper.
- Trientine: Alternative to penicillamine, also used for copper chelation.
Liver Transplantation (LTP):
- Considered in cases of severe liver damage or acute liver failure where chelation therapy is insufficient.

MCQ Discussion - Diagnosis of 20-Year-Old Male with Extrapyramidal Symptoms and Liver Damage

Question: A 20-year-old male presents with extrapyramidal symptoms and liver damage. What is the most likely diagnosis?

a) Wilson's disease

b) Huntington's disease

c) Parkinson's disease

d) Hemochromatosis

Answer: A

Wilson's disease is the most likely diagnosis, as it commonly presents with a combination of neurological symptoms (such as extrapyramidal symptoms) and liver damage in young adults.

Key Points:

Wilson's Disease:
- A genetic disorder of copper metabolism, leading to copper accumulation in the liver, brain (particularly the basal ganglia, causing extrapyramidal symptoms), corneas, and other organs.
- Common symptoms include liver dysfunction (hepatitis, cirrhosis), neurological symptoms (tremors, dystonia, and psychiatric disturbances), and Kayser-Fleischer rings in the eyes.
- Typically presents in adolescence or early adulthood.
Huntington's Disease:
- A neurodegenerative disorder that presents with chorea (not extrapyramidal symptoms) and cognitive decline, usually starting in the 30s-40s.
Parkinson's Disease:
- Causes extrapyramidal symptoms like tremors, rigidity, and bradykinesia, but generally occurs in older adults and is not associated with liver damage.
Hemochromatosis:
- Causes iron overload and liver damage but is associated with diabetes, hyperpigmentation, and cardiac issues, rather than extrapyramidal symptoms.

Liver Biopsy

MCQ Discussion 1 - Liver Biopsy

Question: Liver biopsy is done through the 8th intercostal space (ICS) in the midaxillary line to avoid:

a) Lung

b) Pleural cavity

c) Subdiaphragmatic space

d) Gall bladder

Answer: A

The lung is the primary structure to avoid by performing the liver biopsy in the 8th ICS midaxillary line. This location minimizes the risk of puncturing the lung and pleural cavity.

Key Points:

The 8th ICS location helps avoid the lung and ensures a safe approach to the liver without entering the pleural space or subdiaphragmatic regions.

MCQ Discussion 2 - Percutaneous Liver Biopsy

Question: Not true regarding percutaneous liver biopsy:

a) Needle tract metastasis for HCC - 2-3%

b) Bleeding risk - 5%

c) Transjugular biopsy not suitable for peripherally placed lesions

d) Biliary peritonitis can occur in patients with obstructive jaundice

Answer: B

The bleeding risk for percutaneous liver biopsy is not as high as 5%. It is generally much lower, around 0.5-2%.

Key Points:

Needle tract metastasis occurs in 2-3% of cases when biopsying hepatocellular carcinoma (HCC) (Answer A is true).
Transjugular biopsy is less suitable for peripheral lesions, as it accesses the liver via major hepatic veins (Answer C is true).
Biliary peritonitis is a possible complication in patients with obstructive jaundice (Answer D is true).

Peritoneovenous Shunt (PVS) - Key Points for Revision

Overview:

The Le Veen shunt is designed to relieve ascites due to chronic liver disease (CLD).
It involves a silastic tube connecting the peritoneal cavity to the internal jugular vein, leading into the superior vena cava (SVC).
A one-way valve within the tube ensures peritoneal fluid is drained to the circulation due to pressure differences during the respiratory cycle.
Complications include occlusion, displacement, and infection.

Denver Shunt:

A modified version, the Denver shunt, has a chamber over the costal margin for clearing debris and reducing occlusion rates.

Denver Shunt

Key Features of PVS:

Palliative procedure that does not prolong life.
PVS reinfuses ascites fluid, reducing the prerenal stimulus to sodium retention and improving diuretic responsiveness.
TIPS (Transjugular Intrahepatic Portosystemic Shunt) reduces portal pressure and ascites formation, unlike PVS, which simply reinfuses ascitic fluid into the circulation.

MCQ Discussion 1 - Peritoneovenous Shunt (PVS)

Question: Which of the following statements about the peritoneovenous shunt (PVS) is/are correct?

a) For cirrhotic patients with intractable ascites, the LeVeen shunt is an effective bridge to liver transplantation.

b) Replacement of ascites with saline or lactated Ringer's solution reduces the coagulopathy following PVS.

c) Oliguria in the immediate postoperative period following PVS is common and self-limiting.

d) The transjugular intrahepatic portacaval shunt with stent (TIPSS) works on the same principle as the PVS.

Answer: C

Oliguria in the immediate postoperative period following PVS is common and self-limiting (Answer C is true).
The LeVeen shunt is not typically a bridge to liver transplantation, and TIPS works on a different principle (Answer D is false).

MCQ Discussion 2 - Indications for PVS

Question: Which of the following clinical situations are considered good indications for PVS?

a) A 50-year-old cirrhotic man had an emergency portacaval shunt for bleeding varices and postoperatively had an ascites leak and mild superficial wound infection.

b) A 57-year-old woman with primary biliary cirrhosis (PBC) has difficult to control ascites and diuretic-induced encephalopathy.

c) A 46-year-old resistant alcoholic has chronic ascites uncontrolled by diuretics combined with repeat paracentesis.

d) A 34-year-old woman taking BCPs had rapid onset of ascites and is found to have hepatic vein thrombosis causing Budd-Chiari syndrome.

Answer: C

Chronic ascites that is uncontrolled by diuretics and requires repeat paracentesis (as in Answer C) is a good indication for PVS.

Key Points:

PVS is considered when ascites is difficult to control with diuretics and paracentesis becomes frequent.
Patients with diuretic-resistant ascites and good liver function are suitable candidates.

Image Diagnosis

Question:

Identify the CT pathology:

a) Budd-Chiari Syndrome (BCS)

b) Cirrhosis

c) Tuberculosis (TB)

d) Pseudomyxoma peritonei

Answer: D

Pseudomyxoma peritonei is characterized on CT by mucinous ascites, which appears as low-density, gelatinous fluid in the peritoneal cavity. It often results from mucin-producing tumors, such as those originating in the appendix or ovaries. [Scallooping over the Liver = Pseudomyxoma Peritonnei]

Key Features of the Other Conditions:

Budd-Chiari Syndrome (BCS):
- CT may show hepatic vein obstruction, caudate lobe hypertrophy, and ascites.
Cirrhosis:
- CT findings include a shrunken, nodular liver, splenomegaly, and signs of portal hypertension.
Tuberculosis (TB):
- Abdominal TB can present with ascites, lymphadenopathy, and peritoneal thickening, but lacks the characteristic mucinous appearance seen in pseudomyxoma peritonei.

IPMN & MCN - Biliary Vs Pancreas

Institute Data - IPNB/MCN-L Ratio:

Samsung: 5.7:1
Washington: 1:3
London: 1:6.3

IPNB (Intraductal Papillary Neoplasm of the Bile Duct) vs. IPMN (Pancreatic Intraductal Papillary Mucinous Neoplasm):

IPNB shows:
- Higher histology.
- More associated invasive cancer compared to IPMN.

MCN-L (Mucinous Cystic Neoplasm - Liver) vs. MCN-P (Mucinous Cystic Neoplasm - Pancreas):

MCN-L has:
- Lower histology.
- Less malignant transformation compared to MCN-P.

MCQ Discussion - IPMN-Biliary

Question: Which of the following is not a feature of IPMN-Biliary (IPMN-B)?

a) More common in the East

b) Presents later than cystadenoma

c) More jaundice than cystadenoma

d) Oncocytic type has more invasive malignancy incidence

Answer: D

The oncocytic type of IPMN-B does not have the highest incidence of invasive malignancy. The intestinal type of IPMN-B is generally associated with a higher risk of invasive malignancy, making D the correct answer.

Key Points:

IPMN-B is indeed more common in the East (Answer A is true).
IPMN-B tends to present later than cystadenomas (Answer B is true).
Jaundice is more frequent in IPMN-B than in cystadenomas due to its impact on the bile ducts (Answer C is true).
Oncocytic types do not have the highest malignancy risk; rather, the intestinal type carries this distinction (Answer D is false).

Syncrhonous CRLM

Synchronous [along with or <6months] CRLM Management Pathways:

Stage IV Rectal Cancer:
- The first step is to assess whether the metastasis is resectable or not/borderline resectable.
If Not/Borderline Resectable:
- If symptomatic:
  - Colostomy is performed.
- If not symptomatic:
  - Aggressive neoadjuvant chemotherapy is initiated.
  - Restaging follows to reassess the resectability.
- If after restaging, the metastasis is resectable:
  - Consider a liver-first approach, which includes the sequence of liver resection followed by primary tumor resection and neoadjuvant/adjuvant radiochemotherapy.
- If not resectable after restaging:
  - Proceed to second-line chemotherapy and restaging.
  - If still not resectable, palliative care is considered.
If Resectable:
- Minor or major resection options are considered, which depend on the tumor burden and liver involvement.
- Options include:
  - Simultaneous resection (of both liver and primary tumor).
  - Neoadjuvant chemotherapy followed by resection.
Resection Approaches:
- L+/R- (Liver resection first, rectal surgery after): This is called the liver-first approach, with neoadjuvant therapy before liver resection.
- L+/R+ (Simultaneous resection of liver and rectum): This hybrid approach includes adjuvant radiochemotherapy.
- L-/R+ (Rectal resection first, liver resection after): Known as the classical approach, where rectal surgery is followed by liver surgery and adjuvant radiochemotherapy.

Key Management Points:

The liver-first approach is preferred when the liver disease is more extensive than the rectal disease.
Simultaneous resection is favored when both the primary rectal tumor and liver metastasis are resectable.
Restaging after initial chemotherapy helps determine resectability and the appropriate surgical approach.
Palliative care is considered when the disease remains unresectable after multiple interventions.