Fat and glycogen accumulation are wellknown causes of hepatomegaly. Jul 16, 2011 causes of hepatomegaly in neonates and children posted on july 16, 2011 by emedinews2 pediatric update. Many affected patients show antenatal signs of fetal injury. Acute liver failure in neonates differs from children with regard to aetiology and outcome. Check the full list of possible causes and conditions now. Associate professor of pediatrics and chief, joint program in gastroenterology and nutrition, department of pediatrics, ucsd school of medicine, san diego, ca. Hepatomegaly pediatrics clerkship the university of. Certain conditions such as infection, parasites, tumors, anemias, toxic states, storage diseases, heart failure, congenital heart disease, and metabolic disturbances may all cause an enlarged liver. Causes of hepatomegaly without splenomegaly are indicated by h. Common causes in neonates are biliary tract obstruction, congestive heart failure, drugs. The average liver span is 45 cm in newborns and 68 cm in children at 12 years of age.
Mcgahren iii, in pediatric surgery seventh edition, 2012. Discussion hemangioendotheliomas are benign vascular mesenchymal tumors that present in infancy usually before 6 months and may pose problems due to ensuing complications. Suprahe patic obstruction from congestive heart failure, restrictive pericardial disease, hepatic vein thrombosis. Hepatomegaly pediatrics clerkship the university of chicago. Hepatosplenomegaly in a young child can be an ominous physical finding, potentially representing a metabolic, malignant, or infectious process. Neonatal liver failure and congenital cirrhosis due to. Hepatomegaly is enlargement of the liver beyond its normal size and occurs mainly as a. Neonatal enterovirus hepatitis and coagulopathy ehc can be a severe, lifethreatening infection. Lavine, hepatomegaly in neonates and children, pediatr. Hepatomegaly, along with splenomegaly, is probably the most common abnormality found in the newborn period in infants born with a symptomatic congenital cmv infection 503. See also overview of blood vessel disorders of the liver. May 06, 2020 clinical symptoms vary according to the cause of alf and the age of the child. The treatment process depends on the specific causes of enlarged liver.
It is important to realize that the liver is easily palpated in most children at 12 cm below the right costal margin. The common causes of hepatomegaly were found to be infective hepatitis cirrhosis of the liver and tuberculer. Hepatomegaly general presentation hepatomegaly can represent intrinsic liver disease or may be the presenting physical finding of a generalized disorder. In such conditions, obstruction to pulmonary or systemic venous return reduces cardiac output. Three weeks later he complained of abdominal pain again, accompanied by a lowgrade fever, some nausea, early satiety and mild fatigue. On physical examination, there was a hepatomegaly, and the tumoral mass was palpable in the right flank and iliac region, extending to the mesogastrium and going beyond the. These forms should be returned along with the infants postpartum referral to the regional public health office of. However, not all survive, and neonates with hepatomegaly are particularly at risk. Hepatomegaly in newborns, splenomegaly in newborns, neonatal hepatomegaly, liver mass in infants, congenital liver mass. Enlarged liver hepatomegaly is a chronic health condition in which the liver is swollen beyond its normal size. Neonatal hepatitis an overview sciencedirect topics.
Neonatal hepatitis b virus infection is usually acquired during delivery. The border of the liver can be found by palpation, percussion, and auscultation. The liver edge is normally palpable in children and thin adults and some patients may have a palpable right lobe of the liver. Patient presentation a 9yearold male came to clinic with fever and stomachache. Neonatal pyogenic hepatic abscess in preterm infants is a rare entity. Hepatomegaly with or without elevated lfts may be secondary to multiple causes, which include drug side effect, active sle, infection, fatty infiltration, hepatic vein or artery thrombosis, and congestion secondary to right heart failure.
Hepatomegaly is a symptom that can be caused by a variety of conditions that affect different organ systems. Presentation hepatomegaly causes of hepatomegaly hepatitis o infections viral hepatitis acute and chronic ebv and cmv malaria abscesses amoebic or pyogenic autoimmune hepatitis alcoholic liver disease non alcoholic fatty liver disease previously known as nash tumours o metastases o hepatocellular carcinoma hcc. It is the most common inherited cause of neonatal cholestasis, affecting approximately 1 in 2,000 live births. Should be first line when cholestatic neonatal liver failure relatively rare important to detect early. This page includes the following topics and synonyms.
Normal liver size is based on normative values of liver span by percussion, degree of extension below the right costal margin, or length of the vertical axis estimated from imaging techniques. Neonatal hepatitis is often caused by a viral infection. Affected individuals have a misfolded a1at protein that fails to be secreted normally by the hepatocyte, leading to decreased a1at activity in the blood and lungs and excess retention in hepatocytes. Hepatomegaly occurs in 40% to 50% of patients, with abnormal liver function tests lfts in up to 25%. Symptomatic infection causes jaundice, lethargy, failure to thrive, abdominal distention, and claycolored stools. Fever, hepatosplenomegaly, and pancytopenia in a 5monthold.
The liver edge is smooth and nontender and usually measures 4 to 7 cm below the right costal margin. Children who have orthopedic abnormalities such as narrow chest walls or pectus excavatum can erroneously appear to have hepatomegaly. A neonate with macrosomia, cardiomyopathy and hepatomegaly. Hepatomegaly in neonates and children american academy of. In this article, we will discuss in detail about various causes, symptoms, and treatments for neonatal hepatitis. Congestive hepatomegaly liver and gallbladder disorders. A palpable spleen tip may be a normal finding in up to 30 percent of neonates. Acute liver failure in neonates, infants and children. Overall, predictors of poor prognosis include persisting severe jaundice. The objective of this study was to determine the normal range of dimensions for the liver, spleen, and kidney in healthy neonates, infants, and children. Inflammationneonatal hepatitis is a medical condition of inflammation of liver during infancy.
Presentation hepatomegaly causes of hepatomegaly hepatitis o infections viral hepatitis acute and chronic ebv and cmv malaria abscesses amoebic or pyogenic autoimmune hepatitis alcoholic liver disease non alcoholic fatty liver disease previously known as nash tumours o metastases. Congestive hepatomegaly is a backup of blood in the liver, resulting from heart failure. Frequent clinical and laboratory findings included jaundice, lethargy, anorexia, hepatomegaly, thrombocytopenia, clotting time prolongation, elevated transaminase and bilirubin values and decreased fibrinogen and albumin concentrations. Prognosis for neonates with enterovirus hepatitis and. Rare disease database nord national organization for rare. Massive hepatomegaly with respiratory distress in a. Identify the possible causes of simultaneous hepatomegaly and splenomegaly. Kupffer cell hyperplasia with tb or other generalized infections. Fever, hepatosplenomegaly, and pancytopenia in a 5month. Known viral infectionfollowing viral infection has been detected in 20% of cases causing neonatal hepatitis. Pdf a female neonate presented with progressive abdominal distension. Hepatomegaly hepatomegaly is the condition of having an enlarged liver.
Hepatomegaly is an enlargement of the liver resulting from an increase in the number or size of cells and structures within the liver. The study of hepatomegaly has been mentioned in research publications which can be found using our bioinformatics tool below. Neonatal liver cirrhosis without iron overload caused by gestational alloimmune liver disease article pdf available in pediatrics 1294. Infectious diseases of the fetus and newborn seventh edition, 2011. In neonates with tapvc or single ventricle physiology due to tricuspid atresia, cardiac output and oxygenation is dependent on right to left shunting at atrial level.
Neelam mohan, director pediatric gastroenterology, hepatology and liver transplantation, medanta the medicity. Neonatal hepatitis b virus hbv infection pediatrics. Hepatomegaly is enlargement of the liver beyond its normal size. Acute liver failure in neonates is rare, but carries a high mortality. Hepatomegaly can represent intrinsic liver disease or may be the presenting physical finding of a generalized disorder. Inflammation autoimmune disease bile acid enzyme defects canalicular bile acid transport defects infections virus, abscess idiopathic neonatal hepatitis. Although hepatomegaly usually manifests clinically as a palpable liver, not all palpable livers result from hepatomegaly. Causes for neonatal hepatomegaly doctor answers on.
Clinical symptoms vary according to the cause of alf and the age of the child. Hepatomegaly american academy of pediatrics textbook of. Neonatal and viral hepatitis, biliary atresia, inborn errors of metabolism, congenital hepatic fibrosis, buddchiari syndrome, and alpha1 antitrypsin deficiency commonly lead to cirrhosis, portal hypertension and, subsequently, ascites in children hepatocellular ascitic fluid is a transudate that. Pediatric gastroenterologist st vincent healthcare liver anatomy fetus and newborn liver function and physiology functions of the liver bilirubin metabolism breakdown product of rbc, muscle metabolized and processed in the liver.
Oct, 2008 hepatomegaly is caused by inflammation, infiltration, obstruction, storage abnormalities and vascular congestion. A neonate with macrosomia, cardiomyopathy and hepatomegaly born to an hivinfected mother. The original antenatal care guideline was published by nice in 2003. Accumulation of fluid or air in the thorax eg, pneumothorax also may displace the liver inferiorly. Neonatal liver disease idiopathic neonatal hepatitis up to 25% prognosis generally good predictors of pppg joor prognosis. Often, hepatomegaly will present as an abdominal mass.
Oct, 2008 patient presentation a 9yearold male came to clinic with fever and stomachache. Hepatomegaly is caused by inflammation, infiltration, obstruction, storage abnormalities and vascular congestion. Neonatal liver disease beyond newborn jaundice 2014 montana perinatal association conference tom flass md, ms. Symptoms after birthsymptoms are often observed during 1 to 3 months after birth. The presence of a palpable liver does not always indicate hepatomegaly. References nelsons text book of pediatrics, 19th edition. Massive hepatomegaly with respiratory distress in a newborn.
Approach to a child with hepatosplenomegaly summary of pathophysiological mechanisms 1. The disorder can range from a mild, temporary transient disease that improves without treatment to more serious forms that cause additional. A newborn with distended abdomen rajeevan selvaratnam. Since then a number of important pieces of evidence have become available, particularly concerning gestational diabetes, haemoglobinopathy and ultrasound, so that the update was initiated. Hepatomegaly general presentation hepatomegaly can represent intrinsic liver disease or. When evaluating a child with hepatomegaly it is very important to identify conditions in which immediate medication intervention is needed and provide appropriate treatment options. William britt, in infectious diseases of the fetus and newborn seventh edition, 2011. A retroperitoneal mass, choledochal cyst, or perihepatic abscess also may be mistaken for hepatomegaly. Started in 1995, this collection now contains 6769 interlinked topic pages divided into a tree of 31 specialty books and 732 chapters. There are several causes of nlf and early diagnosis is mandatory to elucidate the etiology and determine a specific treatment or the best management strategy. The differential diagnosis of hepatomegaly includes. Hepatomegaly in neonates and children american academy.
Nov 14, 2012 approach in neonates with hepatosplenomegaly 34. Neonatal liver failure nlf is a major cause of neonatal morbidity and mortality, presenting as acute liver failure andor congenital cirrhosis. After completing this article, readers should be able to. Neonatal hepatitis b virus hbv infection pediatrics msd. Common causes of neonatal liver failure are neonatal. The infant is infected during pregnancy in uterus or after delivery. His rapid strep was positive and he was treated with penicillin. He was seizure at the age of 8 month 3 weeks and weight was 1. Pdf neonatal liver cirrhosis without iron overload caused. A normal liver should feel soft and is easily moveable upon inspiration. It is smooth, uniform, nontender and descends to meet the palpating fingers on inspiration. Intrauterine infectionstudies show that about 20% of infants are infected before the birth in uterus. What is the differential diagnosis of hepatomegaly.
Balistreri, evaluation of liver disease in the pediatric patient, pediatr. Additional signs that may occur include unusually dark urine and pale or claycolored acholic stools. He had no sore throat, diarrhea, changes in bowel or urinary. Disease bioinformatics research of hepatomegaly has been linked to liver diseases, liver neoplasms, icterus, anemia, liver cirrhosis. Severe heart failure causes blood to back up from the heart into the inferior vena cava the large vein that carries blood from the lower parts of the body to the heart. Prognosis for neonates with enterovirus hepatitis and coagulopathy. Last usg report of his mother shows fatal left kidney is mild hydronefrotic, two vessel umbilical cord single artery. Normal liver, spleen, and kidney dimensions in neonates. Venoocclusive disease causes hepatomegaly by obstructing intrahepatic blood flow. We present the case of a 5monthold girl who had fever, hepatosplenomegaly, and hematologic abnormalities at the initial examination. It is a non specific medical sign having many causes, which can broadly be broken down into infection, direct toxicity, hepatic tumours, or metabolic disorder.
Hepatomegaly in infancy and childhood springerlink. Idiopathic neonatal hepatitis occurs with direct hyperbilirubinemia and may be. The prognosis for this socalled idiopathic neonatal hepatitis is generally good, with mortality of 25%. Pdf neonatal liver cirrhosis without iron overload. Some infants with idiopathic neonatal hepatitis may also have an enlarged liver hepatomegaly. Hepatomegaly in newborns, splenomegaly in newborns, neonatal hepatomegaly, liver mass in infants, congenital liver. There can be many inner abnormal conditions that can trigger this health problem however excess alcohol intake is mostly correlated with this issue.
65 1284 802 692 307 536 654 799 251 470 384 39 1105 597 1500 46 1236 1164 1058 1171 1131 1085 235 1204 298 1158 229 1078 821 225 1101 187 146 485 1019 580 361 1431 786 1307 711 558 651 198 735 1239 84 1228