Neonatal cholestasis is defined as prolonged elevation of serum levels of conjugated
bilirubin beyond the first 14 days of life.
Jaundice that appears after 2 wk of age, progresses after this time, or does not resolve at
this time should be evaluated and a direct bilirubin level determined.
Functional impairment of bile secretion may result from congenital defects or damage
to liver cells or to the biliary secretory apparatus.
Neonatal cholestasis may be divided into extrahepatic and intrahepatic disease.
The clinical features of any form of cholestasis are similar.
Functional impairment of bile secretion may result from congenital defects or damage
to liver cells or to the biliary secretory apparatus.
Neonatal cholestasis may be divided into extrahepatic and intrahepatic disease.
The clinical features of any form of cholestasis are similar.
In contrast to unconjugated hyperbilirubinemia, which may be physiologic, cholestasis
in the neonate is always pathologic and prompt differentiation is imperative.
The initial step in identification of cholestasis is the finding that more than 20% of the
hyperbilirubinemia is conjugated bilirubin.
Idiopathic neonatal hepatitis
which can occur in either a sporadic or a familial form, is a disease of unknown cause.
These patients presumably are afflicted with a specific yet undefined metabolic or viral
disease.
Infectious hepatitis in a neonate
may be shown to be due to a specific virus, such as herpes simplex, enteroviruses,
CMV, or, rarely, hepatitis B.
This accounts for a small percentage of cases of neonatal hepatitis syndrome.
Biliary atresia
the anatomy of abnormal bile ducts in affected patients varies markedly. A more
appropriate terminology progressive obliterative cholangiopathy. Patients may have
distal segmental bile duct obliteration with patent extrahepatic ducts up to the porta
hepatis. This is a surgically correctable lesion, but it is uncommon. However, the most
common form of biliary atresia, accounting for approximately 85% of the cases, is
obliteration of the entire extrahepatic biliary tree at or above the porta hepatis. This
presents a much more difficult problem in surgical management.
Differentiation of Idiopathic Neonatal Hepatitis from Biliary Atresia.
Idiopathic neonatal hepatitis has a familial incidence of approximately 20%, whereas
biliary atresia is unlikely to recur within the same family. A few infants with biliary
atresia have an increased incidence of other abnormalities.
Neonatal Cholestasis
Persistently acholic stools suggest biliary obstruction (biliary atresia), but patients with
severe idiopathic neonatal hepatitis may have a transient severe impairment of bile
excretion. Consistently pigmented stools rule against biliary atresia.
Abdominal ultrasound
is a helpful diagnostic tool in the evaluation of neonatal cholestasis because it will
identify choledocholithiasis, perforation of the bile duct, or other structural
abnormalities of the biliary tree such as a choledochal cyst. Hepatobiliary scintigraphy
with technetium-labeled iminodiacetic acid derivatives is used to differentiate biliary
atresia from nonobstructive causes of cholestasis.
Percutaneous liver biopsy
is a valuable procedure in the evaluation of neonatal hepatobiliary diseases and
provides the most reliable evidence.
Biliary atresia is characterized by bile ductular proliferation, the presence of bile plugs,
and portal or perilobular edema and fibrosis, with the basic hepatic lobular architecture
intact.
In neonatal hepatitis
there is severe, diffuse hepatocellular disease, with distortion of lobular architecture,
marked infiltration with inflammatory cells, and focal hepatocellular necrosis; the bile
ductules show little alteration.
Management of Patients with Suspected Biliary Atresia
All patients suspected of having biliary atresia should undergo exploratory laparotomy
and direct cholangiography to determine the presence and site of obstruction.
Direct drainage can be accomplished in patients with correctable lesion.
Malnutrition resulting from malabsorption of dietary long-chain triglycerides Replace
with dietary formula or supplements containing medium-chain Fat-soluble vitamin
malabsorption: Vitamin A deficiency (night blindness, thick skin)
Vitamin E deficiency (neuromuscular degeneration)
Vitamin D deficiency (metabolic bone disease)
Vitamin K deficiency (hypoprothrombinemia) Micronutrient
Deficiency Calcium, phosphate, or zinc supplementation
Deficiency of water-soluble vitamins Supplement with twice the recommended daily
allowance
End-stage liver disease (liver failure)Transplantation
By: Fatima Ehsan