Early Diagnosis of Extrahepatic Biliary Atresia in an Open-Access Medical System

Abstract

IntroductionBiliary atresia (BA) is the most common cause of cholestatic jaundice in infancy. Early diagnosis and surgical management, ideally before 60 days of age, result in improved outcomes. We aimed to determine the age at diagnosis of BA in the Military Health System (MHS) and to compare the age at diagnosis by access to care models. We hypothesized that children with BA receiving primary care in military facilities have an earlier age at diagnosis due to decreased economic and access barriers.MethodsData for all Tricare enrollees born in fiscal years 2004–2008 with a diagnosis of BA were extracted from MHS databases. Non-parametric tests, Kaplan-Meier curves and log rank tests compared differences in age at diagnosis by type of primary care facility, gender, prematurity and presence of additional anomalies.Results64 subjects were identified within the five year period. Median age at diagnosis was 40 days [range 1–189], with 67% diagnosed by 60 days and 80% by 90 days. 45 (70%) received civilian primary care within the MHS. There was no difference in the median age at diagnosis between subjects in the MHS with civilian primary care vs. military primary care (37 days [1–188] vs. 46 days [1–189]; p = 0.58).ConclusionIn the MHS, two-thirds of infants with biliary atresia are diagnosed prior to 60 days of life. Gender, prematurity or presence of additional anomalies do not affect the timing of diagnosis. Civilian and military primary care models within the MHS make timely diagnoses of biliary atresia at equivalent rates.

Biliary Atresia – An Easily Missed Cause of Jaundice amongst Children in Uganda

“…A study in Seattle Washington reported a median age of referral for biliary atresia to be 61 days with a mean of 51 days which was rather late attributed to delays in investigating neonatal jaundice and acholic stools and this declines the success of portoenterostomy 8,9 . This sharply contrasts the situation in the industrial world where the median age at diagnosis is 40 days and median age at Kasai operation is between 54 and 69 days of age 10 .…”

“…21/46 (45%) presented with one of the following complications at admission; failure to thrive, sepsis, ascites, liver cirrhosis, and portal hypertension. This reflects complete lack of screening strategy for biliary atresia as reported in some come countries by using colored (chromatic) cards with a stool color scale distributed to parents 6,7,10 .…”

“…Efforts must continue to be made to eliminate delayed diagnosis in order to improve success of Kasai and reduce the necessity of liver transplantation 10 .…”

Preterm Infants With Biliary Atresia

“…Moreover, preterms with multifactorial cholestasis may have grey stools, and biopsy results with bile duct proliferation may also resemble BA (21,22). Despite the foregoing, prematurity itself seems not to affect the timing of BA detection (23), but merely delays the timing of the KPE (4). Our results emphasize that one of the main challenges in BA, that is, early detection of the disease, is also present in preterm infants.…”

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“…Kasai portoenterostomy, a surgical procedure that restores bile flow within 30 days of life, could slow disease progression and prevent the need for liver transplants (4,5). However, BA is typically diagnosed at an average age of 40-50 days, making it challenging to confirm it in its early stages (6,7).…”