Medical Care
No primary medical treatment is relevant in the management of extrahepatic biliary atresia. The pediatrician's objective is to confirm the diagnosis.
Once biliary atresia is suspected, surgical intervention is the only mechanism available for a definitive diagnosis (intraoperative cholangiogram) and therapy (Kasai portoenterostomy).
Surgical Care
Following a thorough evaluation for causes of neonatal cholestasis, intraoperative cholangiography establishes the diagnosis of extrahepatic biliary atresia. During the operation, the fibrotic biliary tract remnant is identified, and the patency of the biliary system is assessed.
In cases in which biliary patency is associated with ductal hypoplasia, further surgical intervention is not indicated, and bile may be collected to evaluate for disorders of bile acid metabolism.
In the unusual circumstance of distal patency of the common duct with acceptable proximal luminal caliber, a modified portoenterostomy may be considered in place of the traditional Kasai procedure. However, the clinician must be aware that progression of disease pathophysiology may occur. The author has observed patients undergo modified portoenterostomies (gallbladder Kasai), only to subsequently experience continued inflammation and obliteration of the extrahepatic biliary tree and to ultimately require classic portoenterostomies.
In most cases of atresia, dissection into the porta hepatis and creation of a Roux-en-Y anastomosis with a 35-cm to 40-cm retrocolic jejunal segment is the procedure of choice.
Studies have shown that extension of the portal dissection beyond the portal vein bifurcation and the umbilical point in the left hilum may improve the likelihood of achieving adequate biliary drainage.
Consultations
The evaluation of neonatal cholestasis may initially be carried out by the primary care provider, depending on the reliability of the laboratory in performing the necessary serum determinations indicated above. Obviously, further nonsurgical testing (eg, hepatobiliary imaging, liver biopsy) and surgical exploration should only be carried out in centers with considerable experience in managing this disorder.
The physician must not delay in the diagnosis of extrahepatic biliary atresia. Refer infants for appropriate subspecialty care as soon as a diagnosis of obstructive jaundice is suspected.
Diet
During the evaluation phase of biliary atresia, the infant's diet is typically not changed.
Postoperative breastfeeding is encouraged when possible because breast milk contains both lipases and bile salts to aid in lipid hydrolysis and micelle formation. Theoretically, breast milk may also protect against cholangitis, a common complication following portoenterostomy, by suppressing the growth of gram-negative and anaerobic flora. However, no data is available to support this claim.
Infants who are fed formula and who achieve adequate bile drainage should not require a special diet. Early in the postoperative course and when the status of biliary continuity may be in question, one of the medium-chain triglyceride-containing formulas (eg, Alimentum, Pregestimil) may enhance lipid digestion.
Long-Term Monitoring and Follow-up
Long-term monitoring
A study examined the medical status of children with biliary atresia with their native livers after hepatoportoenterostomy (HPE) surgery. The study concluded that over 98% of this North American cohort of subjects with biliary atresia living with native livers 5 or more years after HPE have clinical or biochemical evidence of chronic liver disease. Cholangitis and fractures in long-term survivors also underscore the importance of ongoing medical surveillance. [20]
Follow-up care
A meta-analysis reported that moderate high-dose steroid therapy improves jaundice clearance, especially for infants who undergo hepatoportoenterostomy by 70 days of age. At the present time, however, and pending more randomized controlled trials with longer follow-up, routine post-Kasai corticosteroid therapy is not recommended. [21, 22]
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Biliary atresia.
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Bile ductular proliferation in liver biopsy specimen (hematoxylin and eosin stain) from patient with biliary atresia. Also note hepatocellular bile staining as a consequence of cholestasis.
