Hepatomegaly

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Hepatomegaly

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Overview

Causes

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 - 1.6 kilograms (3.1 - 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 liver size increases with age and it ranges between 5 cm to 15 cm in adulthood. A normal liver is less than 16 cm in ultrasound evaluation. Hepatomegaly may be found in physical examination or during imaging studies. Imaging is more accurate in determining liver size.[1][2] In some certain conditions normal liver may be palpated as enlarged liver including thin people, during deep inspiration, right pleural effusion, and when emphysema results in hyperinflation of the chest with diaphragmatic descent and downward displacement of the liver. Common pathologic causes that may result in hepatomegaly are hepatitis, storage disorders, impaired venous outflow, infiltrative disorders, and biliary obstruction.

Causes

Causes of hepatomegaly may be classified on the basis of etiology into hepatitis, storage disorders, impaired venous outflow, infiltrative and obstructive causes.[3][4][5][6][7][8][9][10][11][12][13][14]

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
Drugs/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/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.[3][4][5][6][7][8][9][10][11][12][13][14]

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 − + + + − −

Skin manifestations may include:

Primary biliary cirrhosis RUQ/Epigastric − − + − − −
  • Increased AMA level, abnormal LFTs
  • ERCP
  • Pruritis
Primary sclerosing cholangitis RUQ + − + − − − 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 ± − ± + ± − Endoscopy is used to confirm diagnosis.

Images used to find complications

Extra intestinal findings:
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 + + + + + ±
  • US
  • CT
Hepatocellular carcinoma/Metastasis RUQ + − + + − −
  • US
  • CT
  • Liver biopsy

Other symptoms:

Budd-Chiari syndrome RUQ ± − ± − − − CT scan findings: Ascitic fluid examination shows:

References

  1. ↑ Sapira JD, Williamson DL (1979). "How big is the normal liver?". Arch Intern Med. 139 (9): 971–3. PMID 475535.
  2. ↑ Niederau C, Sonnenberg A, Müller JE, Erckenbrecht JF, Scholten T, Fritsch WP (1983). "Sonographic measurements of the normal liver, spleen, pancreas, and portal vein". Radiology. 149 (2): 537–40. doi:10.1148/radiology.149.2.6622701. PMID 6622701.
  3. ↑ 3.0 3.1 Wolf AD, Lavine JE (2000). "Hepatomegaly in neonates and children". Pediatr Rev. 21 (9): 303–10. PMID 10970452.
  4. ↑ 4.0 4.1 Chau TN, Lai ST, Tse C, Ng TK, Leung VK, Lim W, Ng MH (2006). "Epidemiology and clinical features of sporadic hepatitis E as compared with hepatitis A". Am. J. Gastroenterol. 101 (2): 292–6. doi:10.1111/j.1572-0241.2006.00416.x. PMID 16454833.
  5. ↑ 5.0 5.1 Bernstein DL, Hülkova H, Bialer MG, Desnick RJ (2013). "Cholesteryl ester storage disease: review of the findings in 135 reported patients with an underdiagnosed disease". J. Hepatol. 58 (6): 1230–43. doi:10.1016/j.jhep.2013.02.014. PMID 23485521.
  6. ↑ 6.0 6.1 Torbenson M, Chen YY, Brunt E, Cummings OW, Gottfried M, Jakate S, Liu YC, Yeh MM, Ferrell L (2006). "Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus". Am. J. Surg. Pathol. 30 (4): 508–13. PMID 16625098.
  7. ↑ 7.0 7.1 Chatila R, West AB (1996). "Hepatomegaly and abnormal liver tests due to glycogenosis in adults with diabetes". Medicine (Baltimore). 75 (6): 327–33. PMID 8982149.
  8. ↑ 8.0 8.1 Mukewar S, Sharma A, Lackore KA, Enders FT, Torbenson MS, Kamath PS, Roberts LR, Kudva YC (2017). "Clinical, Biochemical, and Histopathology Features of Patients With Glycogenic Hepatopathy". Clin. Gastroenterol. Hepatol. 15 (6): 927–933. doi:10.1016/j.cgh.2016.11.038. PMID 28043933.
  9. ↑ 9.0 9.1 Charrow J, Andersson HC, Kaplan P, Kolodny EH, Mistry P, Pastores G, Rosenbloom BE, Scott CR, Wappner RS, Weinreb NJ, Zimran A (2000). "The Gaucher registry: demographics and disease characteristics of 1698 patients with Gaucher disease". Arch. Intern. Med. 160 (18): 2835–43. PMID 11025794.
  10. ↑ 10.0 10.1 Drebber U, Kasper HU, Ratering J, Wedemeyer I, Schirmacher P, Dienes HP, Odenthal M (2008). "Hepatic granulomas: histological and molecular pathological approach to differential diagnosis--a study of 442 cases". Liver Int. 28 (6): 828–34. doi:10.1111/j.1478-3231.2008.01695.x. PMID 18312287.
  11. ↑ 11.0 11.1 Park MA, Mueller PS, Kyle RA, Larson DR, Plevak MF, Gertz MA (2003). "Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients". Medicine (Baltimore). 82 (5): 291–8. doi:10.1097/01.md.0000091183.93122.c7. PMID 14530778.
  12. ↑ 12.0 12.1 Cooke CB, Krenacs L, Stetler-Stevenson M, Greiner TC, Raffeld M, Kingma DW, Abruzzo L, Frantz C, Kaviani M, Jaffe ES (1996). "Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin". Blood. 88 (11): 4265–74. PMID 8943863.
  13. ↑ 13.0 13.1 Long RG, Scheuer PJ, Sherlock S (1977). "Presentation and course of asymptomatic primary biliary cirrhosis". Gastroenterology. 72 (6): 1204–7. PMID 870368.
  14. ↑ 14.0 14.1 "EASL Clinical Practice Guidelines: management of cholestatic liver diseases". J. Hepatol. 51 (2): 237–67. 2009. doi:10.1016/j.jhep.2009.04.009. PMID 19501929.


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