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Biliary atresia, cytomegalovirus, and age at referral
P I Tarr1, J E Haas, D L Christie
1Department of Pediatrics, University of Washington School of Medicine, Seattle, USA.
Insights
Cytomegalovirus (CMV) infection is common in infants with extrahepatic biliary atresia (EHBA). Early referral for EHBA is crucial, as infected infants are often referred later than non-infected infants.
Area of Science:
- Pediatric Surgery
- Infectious Diseases
- Hepatology
Background:
- Extrahepatic biliary atresia (EHBA) is a neonatal cholestatic condition requiring timely surgical intervention.
- Cytomegalovirus (CMV) infection is a potential co-morbidity in infants with cholestasis.
Purpose of the Study:
- To determine the incidence of cytomegalovirus (CMV) infection in patients diagnosed with extrahepatic biliary atresia (EHBA).
- To assess the age at which EHBA patients are referred for surgical correction.
Main Methods:
- Retrospective chart review of EHBA patients diagnosed between 1989 and 1993.
- Analysis of patient demographics, age at referral, and diagnostic methods for CMV infection (serology, culture, histopathology).
Main Results:
- Twenty-four percent (5/21) of evaluated EHBA patients had documented CMV infection.
- Patients with CMV infection were referred significantly later (median 82.4 days) than non-infected patients (median 48.8 days).
- No significant difference in bile duct size was observed between infected and non-infected groups.
Conclusions:
- CMV infection is frequently associated with EHBA at the time of presentation.
- The presence of CMV infection should not impede the diagnostic workup for EHBA.
- Reducing the age of referral for infants with persistent jaundice is critical for optimal EHBA management.
Objective:
To determine the frequency with which patients with extrahepatic biliary atresia (EHBA) are infected with cytomegalovirus (CMV) and to ascertain the age at referral to a specialty center for surgical correction of EHBA.
Methods:
The charts of all patients discharged from the Children's Hospital and Medical Center between July 1, 1989 and December 31, 1993 with a new diagnosis of EHBA were reviewed to determine the frequency with which EHBA was accompanied by CMV infection. Data analyzed included age at referral and sex of patients, histopathologic evidence of CMV infection and size of bile ducts in the resected liver, and serologic (IgM) or culture diagnosis of CMV infection.
Results:
Twenty-three patients with EHBA were evaluated at Children's Hospital and Medical Center in the study period. Twenty-one of the patients with EHBA were appropriately evaluated for infection with CMV and infection was documented in 5 (24%) patients. The median age of referral for all patients was 61 days (range 10 to 124 days). Infected patients were referred later (82.4 +/- 28.7 days) than noninfected patients (48.8 +/- 21.8 days) (P = .01) and were more likely to be girls, bu the medians of the diameters of the bile ducts in the resected porta hepatis were similar. Viral inclusions were not identified in any of the liver specimens.
Conclusions:
CMV infection is present in an unexpectedly large proportion of patients with EHBA at the time of referral. The establishment of CMV infection in infants with cholestasis should not deter the search for EHBA. Physicians should strive to reduce the age of referral of patients with EHBA to pediatric surgical centers by evaluating infants who remained jaundiced at 4 weeks of age.