The incidence of BA varies based on geography, occurring most commonly in East Asia with an incidence of in 5,000 births in Taiwan and in 10,000 to 15,000 births in the United States It is the leading cause of liver transplant and death from liver failure in children Girls are slightly more affected than boys, and it occurs in all races and ethnicities Data suggest that patients with BA have an elevated conjugated bilirubinemia shortly after birth, as early as within the first 24 to 48 hours There is no universal screening tool at present, although some countries have had success with stool color cards as a public health measure to help families identify acholic stools ( Fig 91.2 ) The cause of BA remains unknown There are several subtypes of BA, but the end result in all patients is that there is an inflammatory process that destroys both intrahepatic and extrahepatic biliary tracts and leads to complete biliary obstruction, liver damage, progressive liver cirrhosis, and liver failure Treatment is surgical, which restores biliary flow via a hepatoportoenterostomy There is strong evidence that early diagnosis and surgical correction of BA improves both long-term morbidity and mortality Patients who received surgical correction prior to 60 days of life had a 10-year survival rate of 73%, whereas patients who received surgery after 90 days of life had a 10-year survival rate of 11% Unfortunately, the mean age for surgical correction in the United States is 65.5 days of life, which has not improved in the past 20 years Up to 20% of patients have other congenital abnormalities The most common co-occurring congenital abnormalities are splenic malformations (polysplenia), situs inversus, and other vascular abnormalities Patients are also at risk for congenital cardiac defects and intestinal malrotation Clinical Considerations Clinical Recognition Initially, patients with BA are normal in appearance, but have an elevated conjugated hyperbilirubinemia Patients subsequently have persistent jaundice and develop acholic stool Without intervention, all patients will develop end-stage liver disease by year of age A clinician must have a high index of suspicion for patients with persistent jaundice There are many causes of jaundice in a newborn (see Chapters 44 Jaundice: Conjugated Hyperbilirubinemia and 45 Jaundice: Unconjugated Hyperbilirubinemia ) In general, 15% of newborns are jaundiced at weeks of age and 2% to 6% at weeks of age Persistent jaundice beyond weeks of age should be evaluated for possible BA with a measurement of serum conjugated (direct) bilirubin Triage Considerations In most patients, there are no specific triage considerations for newborns with BA, as they are typically well appearing If unrecognized, patients will have persistent jaundice and over time, develop liver failure and its related complications Clinical Assessment Patients are typically well appearing and present with persistent jaundice Acholic stools, dark brown urine, and failure to thrive may also be present, but are generally late findings and often present outside of the goal age range for early identification On examination, there may not be any physical findings other than skin discoloration and icterus, yet splenomegaly may be present if a patient has developed portal hypertension Dysmorphic features may be identified that likely suggest another disorder, such as Alagille syndrome (AS) (see section below) Initial laboratory evaluation of any patient with persistent jaundice should include a serum conjugated bilirubin level If the newborn has a conjugated (direct) hyperbilirubinemia, BA should be suspected and the infant should be referred immediately to an appropriate center Other laboratory abnormalities may demonstrate a cholestatic disease process with an elevation in liver function enzymes (AST, ALT), serum GGT, and alkaline phosphatase Synthetic liver function is generally normal early in the course of the disease, and most patients are not hypoalbuminemic or coagulopathic Ultrasound is an important initial step in evaluation of a patient with possible BA Findings may include hepatomegaly, an absent or atretic gallbladder, or the “triangular cord sign” which is a hyperechoic area that results from a fibrous hepatic duct This finding is operator dependent and has reported sensitivities of 49% to 80%, but if found, may be 98% specific Bile duct dilation is never present If inconclusive, additional diagnostic testing may be performed in consultation with Gastroenterology and Pediatric Surgery to differentiate BA from other cholestatic disease processes In patients who have already been diagnosed and undergone surgical repair, the most common complication is cholangitis, occurring in up to 55% of patients in the first years after surgery Many patients also develop portal hypertension (see section on portal hypertension), hepatic malignancy, or hepatopulmonary syndrome which results in increased pulmonary vascular blood flow and hypoxia Patients may also present with worsening cirrhosis and liver failure as a result of progression of their underlying disease Management The most important role for the emergency provider in managing a patient with BA is early identification of the disorder Once the diagnosis is made, management is primarily surgical In patients with a conjugated hyperbilirubinemia, ED providers should further evaluate for cholestasis (total and fractionated bilirubin, GGT, alkaline phosphatase), aminotransferase elevation (AST, ALT), and liver function (albumin, PT/INR, PTT) (see Fig 44.1 for further information on the workup of Conjugated Hyperbilirubinemia) Patients should also receive an abdominal ultrasound with Doppler to assess for other anatomic causes as well as obstruction and chest x-ray to assess for possible vertebral body changes or rib cage changes suggestive of Alagille’s The patient should be urgently referred to Gastroenterology and Pediatric Surgery CHOLELITHIASIS/CHOLECYSTITIS Gallstones or cholelithiasis occasionally occur in adolescents Often the condition remains asymptomatic until a complication develops, such as cholecystitis It is primarily seen in patients with hemolytic anemias (pigment stones) such as sickle cell disease and hereditary spherocytosis, however it becoming increasingly common in otherwise healthy children, likely as a result of childhood obesity and diabetes Other risk factors for developing gallstones includes the use of total parenteral nutrition (TPN), systemic infection, antibiotic use, biliary anatomic abnormalities, cystic fibrosis, increased estrogen, and family history While not nearly as common in adults, children account for 4% of all cholecystectomies, and two-thirds are female Biliary colic results from acute transient obstruction of the cystic duct or common bile duct by gallstone(s) Cholecystitis is an aseptic inflammatory process that develops as a reaction to chemical injury triggered by obstruction to the cystic duct by a gallstone Cholangitis results from secondary bacterial infection by enteric organisms in the face of biliary tract obstruction or after surgical manipulation of the biliary tract Acute cholangitis may be mild and superficial, producing only short-lived symptoms, or it may be severe, causing suppurative cholangitis with septic shock and formation of hepatic abscesses The pain of biliary colic is acute in onset, often follows a meal, and is usually localized to the epigastrium or right upper quadrant Some children localize the pain to the periumbilical area In contrast to the colicky pain of intestinal or ureteral origin, biliary colic does not worsen in relatively short cyclic paroxysms or bursts but instead is characterized by its sustained, intense quality Unlike pancreatitis, the patient tends to move about restlessly and the pain is not improved by changes in position In addition, referred pain is common, particularly to the dorsal lumbar back near the tip of the right scapula Nausea and vomiting are commonly associated with biliary colic but are not severe and protracted as seen with pancreatitis Mild jaundice occurs in 25% of patients An attack of acute cholecystitis begins with biliary colic, which increases progressively in severity or duration Pain lasting longer than hours suggests cholecystitis and the temperature is usually mildly elevated As the inflammation worsens, the pain changes character, becoming more generalized in the upper abdomen and increased by deep respiration and jarring motions In contrast, acute cholangitis should be suspected in the patient who has right upper quadrant abdominal pain, shaking chills with spiking fever, and jaundice (Charcot triad) These patients usually have a history of abdominal surgery A dangerous aspect of this disorder is that overwhelming sepsis can develop rapidly Listlessness and shock are characteristic of advanced or severe cholangitis and usually reflect gram-negative septicemia Cholangitis can evolve rapidly before the development of significant jaundice Clinically apparent jaundice may be absent even in postsurgical BA patients Laboratory tests are typically nonspecific in cholecystitis A CBC and blood smear may show evidence of hemolysis The leukocyte count averages 12,000 to 15,000 per mm3 with a neutrophilic leukocytosis Elevated leukocyte counts raise concern for cholangitis The level of serum bilirubin may be elevated but rarely exceeds mg/dL Higher values are more compatible with either complete common bile duct obstruction or cholangitis The levels of serum transaminases (ALT and AST) and alkaline phosphatase may be mildly elevated but are often normal Marked elevation in the levels of transaminases may occur with acute, complete common duct obstruction Serum amylase levels may be mildly elevated without other evidence of pancreatitis The general management guidelines for the ED provider include discontinuation of oral intake, support with IV fluids, pain control, and surgical consultation If acute cholangitis is suspected, antibiotics should be ... inconclusive, additional diagnostic testing may be performed in consultation with Gastroenterology and Pediatric Surgery to differentiate BA from other cholestatic disease processes In patients who have... a result of progression of their underlying disease Management The most important role for the emergency provider in managing a patient with BA is early identification of the disorder Once the... changes suggestive of Alagille’s The patient should be urgently referred to Gastroenterology and Pediatric Surgery CHOLELITHIASIS/CHOLECYSTITIS Gallstones or cholelithiasis occasionally occur in