Hepatomegaly

Hepatomegaly

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Hepatomegaly Microchapters
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Overview
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Differential Diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Seyedmahdi Pahlavani, M.D. [2],Iqra Qamar M.D.[3]
Synonyms and keywords: Enlarged liver; liver enlargement

Overview

The liver is an organ present in vertebrates and some other animals. The adult human liver normally weighs between 1.4 to 1.6 kilograms (3.1 to 3.5 pounds), and it is a soft, pinkish-brown "boomerang shaped" organ. It is located on the right side of the upper abdomen below the diaphragm. The size of the liver increases with age and ranges between 5 cm to 15 cm in adulthood. A normal liver is less than 16 cm on ultrasound evaluation. Hepatomegaly may be found during physical examination or on imaging studies. Imaging is more accurate in determining liver size. In some conditions normal liver may be palpated as enlarged liver including thin people, during deep inspiration, right pleural effusion, and emphysema resulting in hyperinflation of the chest with diaphragmatic descent and downward displacement of the liver. Common pathological causes that may result in hepatomegaly are hepatitis, storage disorders, impaired venous outflow, infiltrative disorders, and biliary obstruction.

Causes

Various causes of hepatomegaly may be classified on the basis of etiology into hepatitis, storage disorders, impaired venous outflow, infiltrative, and obstructive causes.

Etiology		Disease
Hepatitis	Infections	Acute and chronic viral hepatitis
		Bacterial liver abscess
		Parasitic infections
		Granulomatous hepatitis
	Ischemia	Ischemic hepatitis ("shock liver")
	Toxins	Alcoholic hepatitis
	Steatosis	Alcoholic fatty liver disease
		Nonalcoholic steatohepatitis
	Medications	Drug induced liver injury (DILI)
	Immune mediated hepatitis	Autoimmune hepatitis
	Copper deposition	Wilson disease
Storage disorders	Glycogen	Glycogen storage disorders
		Diabetes mellitus
	Lipid	Gaucher disease
		Nonalcoholic steatohepatitis
	Protein	Alpha-1 antitrypsin deficiency
	Iron	Hemochromatosis
Impaired venous outflow	Cardiac	Right heart failure
		Constrictive pericarditis
	Hepatic vein	Hepatic vein thrombosis
		Inferior vena cava web
	Intrahepatic	Sinusoidal obstruction syndrome
		Peliosis hepatis
Infiltrative diseases	Benign primary liver tumors	Hemangiomas
		Adenomas
		Focal nodular hyperplasia
	Malignant primary liver tumors	Hepatocellular carcinoma
		Cholangiocarcinoma
		Fibrolamellar carcinoma
		Hemangioendothelioma
	Metastatic or disseminated tumors	Myeloma
		Lymphoma
		Leukemia
		Metastatic solid tumors
Biliary obstruction	Primary biliary cirrhosis
	Primary sclerosing cholangitis
	Biliary atresia
Other	Anatomic variations	Riedel's lobe
	Cystic liver disease	Polycystic liver disease
		Caroli's disease

Differential Diagnosis

Patients with hepatomegaly need to be differentiated from other patients presenting with similar complaints such as abdominal pain.

Abbreviations: RUQ= Right upper quadrant of the abdomen, LUQ= Left upper quadrant, LLQ= Left lower quadrant, RLQ= Right lower quadrant, LFT= Liver function test, SIRS= Systemic inflammatory response syndrome, ERCP= Endoscopic retrograde cholangiopancreatography, IV= Intravenous, N= Normal, AMA= Anti mitochondrial antibodies, LDH= Lactate dehydrogenase, GI= Gastrointestinal, CXR= Chest X ray, IgA= Immunoglobulin A, IgG= Immunoglobulin G, IgM= Immunoglobulin M, CT= Computed tomography, PMN= Polymorphonuclear cells, ESR= Erythrocyte sedimentation rate, CRP= C-reactive protein, TS= Transferrin saturation, SF= Serum Ferritin, SMA= Superior mesenteric artery, SMV= Superior mesenteric vein, ECG= Electrocardiogram, US = Ultrasound

Organ system	Disease	Clinical manifestations							Diagnosis		Comments
		Symptoms					Signs
		Abdominal Pain	Fever	Nausea or vomiting	Jaundice	Weight loss	Hypo- tension	Rebound Tenderness	Lab Findings	Imaging
Pancreato-biliary disorders	Pancreatic carcinoma	Epigastric	−	+	+	+	−	−	↑ Alkaline phosphatase ↑ serum bilirubin ↑ gamma-glutamyl transpeptidase ↑ CA 19-9	MDCT with  PET/CT MRI	Skin manifestations may include: Bullous pemphigoid Cicatricial pemphigoid Migratory superficial thrombophlebitis (classic Trousseau's syndrome) Pancreatic panniculitis
	Primary biliary cirrhosis	RUQ/Epigastric	−	−	+	−	−	−	Increased AMA level Abnormal LFTs	ERCP	Pruritis
	Primary sclerosing cholangitis	RUQ	+	−	+	−	−	−	Increased liver enzymes Increased IgM, IgG4 Anti-neutrophil cytoplasmic antibody (p-ANCA) Anti-nuclear antibody (ANA) Anti-smooth muscle antibody (Anti-Sm) Anti-endothelial antibody Anti-cardiolipin antibody	ERCP and MRCP shows Multiple segmental strictures Mural irregularities Biliary dilatation and diverticula Distortion of biliary tree	The risk of cholangiocarcinoma in patients with primary sclerosing cholangitis is 400 times higher than the risk in the general population.
Systemic disease	Whipple's disease	Diffuse	±	−	±	+	±	−	Thrombocytopenia Hypoalbuminemia Small intestinal biopsy for Tropheryma whipplei	Endoscopy is used to confirm diagnosis. Images used to find complications Chest and joint x-ray CT MRI Echocardiography	Extra intestinal findings: Uveitis Endocarditis Encephalitis Dementia Hepatosplenomegaly Arthritis Ascites
	Hemochromatosis	RUQ	−	−	−	−	−	−	>60% TS >240 μg/L SF Raised LFT Hyperglycemia	Ultrasound shows evidence of cirrhosis	Extra intestinal findings: Hyperpigmentation Diabetes mellitus Arthralgia Impotence in males Cardiomyopathy Atherosclerosis Hypopituitarism Hypothyroidism Extrahepatic cancer Prone to specific infections
Hematologic causes	Myeloma	Diffuse	±	−	−	+	±	−	Anemia Elevated ESR and CRP Proteinuria	−	Mainly in old age Back pain Kidney involvement
	Lymphoma	Diffuse	+	−	−	+	±	−	Anemia Elevated ESR and CRP	−
	Leukemia	Diffuse	+	±	−	+	±	−	Anemia Elevated ESR and CRP	−
	Disease	Abdominal Pain	Fever	Nausea or vomiting	Jaundice	Weight loss	Hypo- tension	Rebound Tenderness	Lab Findings	Imaging	Comments
Cardiac causes	Heart failure	Diffuse/ RLQ/LLQ	−	−	−	+	±	−	CBC Increased BNP and Pro BNP	Echocardiography	Exertional dyspnea Chest pain Edema
	Constrictive pericarditis	Diffuse/ RLQ/LLQ	−	−	−	+	±	−	-	Echocardiography	Exertional dyspnea Chest pain Edema
Hepatic causes	Viral hepatitis	RUQ	+	+	+	+	Positive in fulminant hepatitis	+	Abnormal LFTs Viral serology	US	Hep A and E have fecal-oral route of transmission Hep B and C transmits via blood transfusion and sexual contact.
	Liver abscess	RUQ	+	+	+	+	+	±	CBC Blood cultures Abnormal liver function tests	US CT
	Hepatocellular carcinoma/Metastasis	RUQ	+	−	+	+	−	−	High levels of AFP in serum Abnormal liver function tests	US CT Liver biopsy	Other symptoms: Splenomegaly Variceal bleeding Ascites Spider nevi Asterixis
	Budd-Chiari syndrome	RUQ	±	−	±	−	−	−	Elevated serum aspartate aminotransferase and alanine aminotransferase levels may be more than five times the upper limit of the normal range. Elevated serum alkaline phosphatase and bilirubin levels, decreased serum albumin level.	CT scan findings: Early enhancement of the caudate lobe and central liver around the inferior vena cava Delayed enhancement of the peripheral liver with accompanying central low density (flip-flop appearance)	Ascitic fluid examination shows: Total protein more than 2.5 g per deciliter White blood cells are usually less than 500/μL.