Liver Abscess & Liver Disease Systemic Illness In Children 

Liver Abscess

Liver abscesses are endemic to Southeast Asia, including India. Males are disproportionately more affected by liver abscesses than females for reasons unknown.

Types of Liver Abscesses in Children

 There are two types of liver abscesses in children.

Pyogenic Liver Abscess: 

The common cause for this type of liver abscess is Staphylococcus aureus (>50% of cases) in children. Anaerobes are found in 30% of the cases. Gram-negative bacteria such as E.coli, Streptococcus pyogenes, and Klebsiella are also found in many cases. Salmonella typhi is also one of India's most common causes of pyogenic liver abscesses. A mixed infection is often seen.

Amoebic Liver Abscess: 

Entamoeba histolytica is the only cause of the amoebic liver abscess.

Origin of Pyogenic Liver Abscess

• Portal vein: Bacteria can reach the liver through the portal vein. It can reach the portal vein via umbilical venous catheterization, omphalitis, and intra-abdominal infections.
• Hepatic Artery: Bacteria can also reach the liver through the hepatic artery. The dissemination occurs whenever there is systemic sepsis or septicemia: Pyelonephritis, endocarditis, or any other abscess in the body.
• Biliary tract: via the biliary tract, infections can arrive as ascending cholangitis. E.coli is the predominant pathogen.
• Direct/Contiguous: This transmission results from a procedure or trauma.

Also read: Types Of Liver Transplantation In Children

Origin of Amoebic Liver Abscess 

There's an amoebic infection involving the colon. The infection travels through the portal vein, and the trophozoites reach the liver.

Clinical Features

• For pyogenic liver abscesses, clinical features are non-specific and may include fever, right upper quadrant pain, chills, malaise, vomiting, and irritability in the child.
• In the case of an amoebic liver abscess, there may be a history of amoebic dysentery.
• Fontan's triad for liver abscess in children includes fever, right hypochondriac pain, and hepatomegaly. This triad is only seen in 50% of the cases in children.
• In 2/3rds of the cases, abscesses are single and in 2/3rds of the cases, the right lobe is more involved than the left lobe.
• The portal vein gets divided into right and left branches. The right hepatic portal vein is the continuation of the common portal vein. The left branch is angled. So, the bacteria have a higher tendency to go into the right lobe of the liver.

Investigations

Mild anemia and leukocytosis are present in most children. ESR and CRP are significantly elevated in patients with pyogenic liver abscesses. Mild elevation is seen in patients with amoebic liver abscesses. Mild elevation in the alkaline phosphatase is seen. The rise in AST, ALT, and bilirubin levels is not significantly seen. A blood culture is needed for a pyogenic liver abscess, and it will be found positive. Serological tests like IHA and ELISA can be done for an amoebic liver abscess. Stool examination can also be done for amoebic cases via PCR or ELISA. PCR is considered superior. Live trophozoites are seldom seen. Imaging is always recommended whenever an abscess is suspected. A plain X-ray will show the elevation of the right hemidiaphragm +/- Right pleural effusion. The initial investigation of choice is USG. It is very sensitive in the picking of abscesses. Contrast Enhanced CT-SCAN is more effective in the detection of smaller, left-sided, or deeply located abscesses. A double target sign with an inner ring of abscess and an outer ring of edema is found in the CECT scan as a sign of liver abscess in adults and children.

Also read: Mitochondrial Hepatopathies

Treatment of Liver Abscess

Irrespective of the nature, medical management in the form of antibiotics is always the initial therapy. In the case of amoebic liver abscess, anti-parasitics are to be given. Vancomycin, metronidazole, and ceftriaxone are given for pyogenic liver abscesses.

• Vancomycin covers Staphylococcus aureus.
• Ceftriaxone covers gram-positive and gram-negative bacteria.
• Metronidazole covers anaerobes.

This combination of drugs is given for 4-6 weeks. Out of these, the first two weeks need parenteral administration. The mode of administration can be decided for the rest of the duration. The drug of choice for an amoebic liver abscess is metronidazole, given for 7-10 days. An anti-luminal agent called paramomycin helps eradicate the infection from the gut.

USG-Guided Percutaneous Aspiration

Percutaneous aspiration can be USG-guided, catheter-guided, or wire-based. USG-guided percutaneous aspiration is only recommended only in specific settings:

• If there's a large abscess measuring 5-7 cm that fails to resolve with antibiotic therapy
• If there's a large abscess in the left lobe
• For an impending rupture with an abscess ring of less than 1 cm on imaging

Left lobe abscess can be life-threatening because it can rupture into the pericardium, pleural space, and peritoneum.

Open surgery is performed when antibiotics and percutaneous aspiration fail or an abscess ruptures into a secondary space. There is a multiloculated abscess that is not amenable to percutaneous aspiration

Prognosis

The prognosis is excellent in children for both types of liver abscesses. The abscess cavity resolves in 3 to 6 months.

Alos read: Neonatal Cholestasis: Causes, Diagnosis, and Treatment in Infants

Liver Disease in Inflammatory Bowel Disease

Liver diseases are more common in ulcerative colitis than in Crohn's disease. Manifestations can be in the form of sclerosing cholangitis. It can also be in the form of overlap syndrome, with features of autoimmune hepatitis and sclerosing cholangitis. Fatty liver disease. Drug toxicities, mainly 5-amino salicylic acid, used in the management of IBD

Liver Disease in Celiac Disease

It can be without any autoimmune diseases or with autoimmune liver disorders. Certain children with celiac disease are found to have mild elevations in AST, ALT, and ALP. The liver shows changes related to fatty liver and mild periportal inflammation. Most of these conditions reverse with a gluten-free diet. Autoimmune hepatitis and autoimmune cholangitis may be associated with celiac disease. These require immunosuppressant management.

Liver Disease in Cardiac Disease

Hepatic injury can occur as a complication of severe, acute, or chronic CCF, cyanotic congenital heart disease, or acute hypovolemic shock. The hallmark of injury is the centrilobular area. Two types of manifestations are possible here. Due to congestive cardiac failure, there'll be increased right ventricular pressure. It gets transferred to the right atrium and from there to the inferior vena cava. There'll be increased pressure in the hepatic vein as well as the portal vein. From the portal veins there will be back pressure changes into the hepatic ductules, which undergo congestion. There's mild fibrosis. So, the damage occurs in the centrilobular area.

Liver Disease Due to TPN (Total Parenteral Nutrition)

TPN-associated cholestasis is the most common manifestation of this disease. It usually develops after the 2nd week of TPN. If a child is on complete TPN, it produces prolonged fasting in the child. There is a decrease in the baseline secretion of the CCK (cholecystokinin) enzyme. This enzyme is involved in maintaining the biliary flow. The reduced biliary flow will cause an increase in the sludge formation, promoting gallstones and local inflammatory changes and cholestasis. It causes damage to the mucosal integrity of the gut. The risk factors include the following:

• Prolonged TPN using soy-based formulas
• Prematurity and LBW (Low birth weight)
• Gram-negative sepsis
• NEC (necrotising enterocolitis)
• Short bowel syndrome
• TPN composition-based injury: It can be high calories, protein content, or amino acid-induced injury.

Also read: Nelson 22nd Edition Updates Neonatal Jaundice

Liver Disease in Cystic Fibrosis

The onset of liver disease occurs at a median age of IO years, and >90% occurs by 20 years.

The pathognomonic lesion is focal biliary cirrhosis. The liver disease tends to occur mainly in males with pancreatic insufficiency and requires 2 CFTR mutations without residual function. Treatment with oral ursodeoxycholic acid (10-15 mg/kg/day) may be beneficial in improving liver function, presumably by improving bile flow. The SERPINA-1 gene has been implicated in many patients with cystic fibrosis who are at an increased risk of developing liver disease.

Liver Disease in Bone Marrow Transplant

Liver disease in BMT patients can arise due to opportunistic infections. Vaso-occlusive disease: Occlusion due to fibrosis of small hepatic venules but without thrombosis. Onset is in the first 3 weeks. GVHD (graft-versus-host disease (GvHD): Liver disease shows between 14 to 21 days after a successful bone marrow transplantation. Its immune response is directed against bile duct epithelium. Hemosiderosis can occur secondary to frequent blood transfusions.

Liver Disease in Sickle Cell Anemia

Hepatic sickle cell crisis or “sickle hepatopathy.” It occurs in 10% of patients with sickle cell disease. It manifests with intense right upper quadrant pain and tenderness, fever, leukocytosis, and jaundice. Bilirubin levels may be markedly elevated. Serum ALP levels may be only moderately elevated. It tends to self-resolve within one to three weeks. It doesn't require any specific therapy. It can also have features related to multiple blood transfusions, like hemosiderosis.

Liver Disease in Histiocytic Disorders can occur in

In Langerhans cell histiocytosis, periportal inflammation and sclerosing cholangitis can occur. Hemophagocytic lymphohistiocytosis (HLH) syndrome manifests as an acute hepatic failure.

Also read: Gastrointestinal Foreign Bodies in Children: Bezoars & Ingestion

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