HEPATOMEGALY

Liver is normally palpable up to 2 cm in first two years of life, though Riedel lobe may be palpable even in older children. A palpable liver does not necessarily mean hepatomegaly and may be pushed-down by thoracic pathology, e.g.

Normal liver span is ~5 cm in newborns and ~7 cm in older children. A liver span of gt;8 cm in childhood is abnormal.

Etiology: Hepatomegaly may be-(a) infective, (b) meta­bolic, (c) hematologic, (d) infiltrative, or (e) congestive in origin, apart from rare congenital malformations (Table 15.2).

TABLE 15.2: Cause of hepatomegaly

• Infective Hepatomegaly

- Viral: Hepatitis A-E, HIV, EBV

- Bacteria: Typhoid, sepsis, abscess, tuberculosis

- Spirochetal: Leptospirosis

- Protozoa: Amoebic hepatitis

- Parasitic: Malaria, kala-azar

- Intrauterine infections

• Autoimmune hepatitis

• Congestive hepatomegaly

- Cardiac: CCF, pericarditis

- Pre-cardiac: Budd-Chiari syndrome

• Compensatory hepatomegaly (#8593; erythropoiesis)

- Hemolytic anemia

- Nutritional anemia

• Metabolic/storage disorders

- Carbohydrates: Glycogen storage disorders

- Fat: Gaucher/Niemann-Pick disease

- Mineral: Indian childhood cirrhosis, Wilson disease

- Protein: Tyrosinemia, Reye syndrome

• Cholestatic syndromes

• Inhltrative hepatomegaly

- Leukemia, lymphoma

- Hepatic tumors (primary/metastatic)

- Histiocytosis

• Congenital cysts, malformations and CHF

• Miscellaneous

- Physiological (lt;2 years)

- Displaced liver: Visceroptosis, pneumothorax

- Drug-induced Hepatitis

CHF: Congenital hepatic fibrosis

D/D between various causes of hepatomegaly (Table 15.3) depends on:

• Characteristics, e.g.

• Age: Hepatomegaly in newborns and young infants usually indicate intrauterine infections, septicemia, cholestatic syndromes and metabolic disorders, while hemolytic anemia and congestive hepatomegaly is more common in older infants and toddlers. Viral hepatitis and other infective causes predominate in school children.

• Co-existing splenomegaly usually indicates portal hypertension, hemolytic anemia, chronic infections, metabolic disorders or infiltrative disorders, e.g. leukemia. Isolated hepatomegaly is mainly infective (viral hepatitis, abscess) or congestive in origin (cardiac failure).

• Associatedfindings, e.g. jaundice (viral hepatitis), pallor (hemolytic anemia, leukemia), lymphadenopathy (malignancy), ascites (chronic liver disease), etc. provide vital diagnostic clues in hepatomegaly.

TABLE 15.3: D/D Broad causes of hepatomegaly

*finding may change over time

15.3 JAUNDICE

TABLE 15.4: Causes of jaundice (Post-neonatal)

Jaundice (Icterus) is a clinical diagnosis with yellowish discoloration of sclera, mucus membranes and skin, due to elevated serum bilirubin levels. Normal serum bilirubin levels are 0.3 -0.8 mg/dl with predominant unconjugated fraction (unconjugated:conjugated ratio gt; 8:1). However, icterus is clinically visible, only when serum bilirubin levels exceeds 2.0 mg/dl.

Normal bilirubin metabolism and neonatal jaundice is discussed in Chapter 12.13. Present section mainly pertains to post-neonatal jaundice.

Etiology: Common causes of post-neonatal jaundice may be divided into—(a) hemolytic jaundice due to increased bilirubin production, (b) hepatic jaundice due to impaired bilirubin metabolism, and (c) obstructive or cholestatic jaundice due to inadequate bilirubin excretion (Table 15.4).

Diagnostic evaluation: Clinical differentiation between three broad groups—hemolytic, hepatic and obstructive jaundice, is the first step in evaluation of jaundice (Table 15.5), followed by relevant laboratory studies for specific etiology.

• Course: Hepatitis A is the commonest cause of short duration, self-limiting icterus (lt;2-3 weeks) in Indian children, while prolonged icterus indicates chronic liver disease or congenital hemolytic anemia. Recurrent jaundice is common in chronic liver diseases or choledochal cysts.

• Relevant history related to adverse environmental factors (infective hepatitis), similar family history (hemolytic anemia, metabolic disorders) or previous treatment (drug-induced).

Hemolytic jaundice

• Congenital: Hereditary spherocytosis, thalassemia

• Drug/toxins: Quinine (in G6PD deficiency)

• Acquired: Malaria, polycythemia, transfusions

• Micro-angiopathic: Hemolytic-uremic syndrome

Hepatic jaundice

• Infective: Viral hepatitis, leptospirosis, malaria,

• Drug/toxins: Isoniazid, rifampicin, valproate

• Chronic liver disease: Chronic active hepatitis, cirrhosis

• Metabolic: Wilson disease

• Familial: Crigler-Najjar syndrome, Gilbert syndrome

Obstructive jaundice

• Neonatal hepatitis

• Intrahepatic biliary atresia

• Extrahepatic causes:

- Biliary tree-extrahepatic biliary atresia

- Gallbladder-gallstones, choledochal cyst

- Porta hepatis-tubercular nodes, portal fibrosis

- Ampulla of Vater-worms, pyloric stenosis

• Inherited: Dubin-Johnson/Rotor syndrome*

^Conjugated jaundice, but not due to obstructive etiology

• Itching and clay-colored stools strongly indicate obs­tructive jaundice, though these features are not uncommon in recover phase of infective hepatitis.

• Coexisting splenomegaly with pallor indicates hemolysis—congenital or acquired (malaria, sepsis).

• Signs of metabolic liver dysfunction, e.g. edema, ascites, steatorrhea, etc. indicates hepatic jaundice.

• Liver function tests differentiate between hemolytic, hepatic and obstructive jaundice, though further investigations are needed to pinpoint exact etiology.

TABLE 15.5: D/D Broad types of jaundice

¹ except G6PD deficiency, ² or recurrent (G6PD def./sickle cell disease), ³ in G6PD deficiency, ⁴ HAV after 3-5 years, Wilson disease after 5 years,

⁵ persistent in chronic liver disease, ⁶ present in Wilson disease and other metabolic causes, ⁷ or in late childhood (gallstones), ⁸ recurrent in choledochal cyst, gallstones, ⁹ except in hemolytic gallstones, ¹⁰ due to gallstones

15.4