risk for the disease.8,9 Splenic malformation and other syndromic features are present in about 10% of cases. Reports of familial biliary atresia are exceptionally rare10 and studies of twin infants showed discordance of presentation.11,12
can progress to bridging and nodularity. The loss of native bile ducts also occurs in the later stages of disease.24 Occasionally, bile duct plate malformations may be present and have been reported to predict a worse clinical outcome.25
Figure 14.2 Biliary atresia. Portal tracts are expanded by fibrosis and ductular proliferation. Note multinucleated giant hepatocytes (upper center; lower center).
Figure 14.3 Biliary atresia. Masson-trichrome stain demonstrates extensive bridging fibrosis in a 16-week-old patient.
Table 14.1 Clinical and histopathologic features of biliary atresia and neonatal hepatitis syndrome
scant small bile ducts in the portal plate sections are associated with a worse prognosis and lower survival (Fig. 14.7).26,27,28
Figure 14.4 Biliary atresia. At earlier stages, cholestasis and minimal portal changes may be the only findings. This biopsy was obtained at 9 weeks of age.
Figure 14.5 Kasai specimen. The components of a Kasai specimen that should be sampled for microscopic examination.
Figure 14.6 Kasai specimen. Cross-section of partially occluded bile duct with fibrosis, chronic inflammation and inspissated luminal bile.
cause.34 Other considerations include sepsis (Escherichia coli) and other viral processes (Coxsackievirus, Echovirus, Herpesvirus, and Adenovirus). The syndrome is more commonly described in male infants and is associated with prematurity and low-birth weight.35 Clinically, not all patients will have noticeable jaundice, but conjugated hyperbilirubinemia is consistently present.
Table 14.2 Etiology of neonatal hepatitis
Chapter 17), viral hepatitis B and certain metabolic disorders. A diligent search for viral cytopathic effect is important in identifying infectious hepatitis, and immunohistochemical studies may be useful in this setting.
Figure 14.8 Neonatal hepatitis. Prominent giant cell transformation of hepatocytes and extramedullary erythropoiesis.
Figure 14.9 Neonatal hepatitis. Multinucleated giant hepatocytes predominate in this centrilobular region. Note the lack of significant ductular proliferation and portal fibrosis.