A 12-year-old girl is brought to the emergency department due to severe pain in the right upper quadrant, which started suddenly 12 hours ago. The patient has also had nausea, vomiting, fever, and rigors. Medical and family histories are both unremarkable. Temperature is 39.3 C (102.7 F), blood pressure is 110/60 mm Hg, pulse is 110/min, and respirations are 18/min. Scleral icterus is present. The abdomen is tender to palpation in the right upper quadrant without rebound or involuntary guarding. Laboratory results are as follows: Abdominal imaging reveals mild gallbladder distension without stones or mucosal thickening; a cyst is present in the common bile duct with dilation of the intrahepatic ducts. Which of the following is the best next step in management?

Question

A 12-year-old girl is brought to the emergency department due to severe pain in the right upper quadrant, which started suddenly 12 hours ago.  The patient has also had nausea, vomiting, fever, and rigors.  Medical and family histories are both unremarkable.  Temperature is 39.3 C (102.7 F), blood pressure is 110/60 mm Hg, pulse is 110/min, and respirations are 18/min.  Scleral icterus is present.  The abdomen is tender to palpation in the right upper quadrant without rebound or involuntary guarding.  Laboratory results are as follows:  Abdominal imaging reveals mild gallbladder distension without stones or mucosal thickening; a cyst is present in the common bile duct with dilation of the intrahepatic ducts.  Which of the following is the best next step in management?

Quizplus · Accepted Answer

11ec25c8_d8dc_f9da_bc81_15e03f19f54d_MD0003_00 This patient's fever, jaundice, and right upper quadrant (RUQ) pain are concerning for acute cholangitis, a potentially life-threatening infection. In the setting of a cyst within the common bile duct, the most likely underlying etiology is a congenital biliary cyst. biliary cyst Although biliary cysts classically cause chronic or subacute jaundice, RUQ pain, and a RUQ mass, some patients (like this one) initially present with complications. Proximal narrowing of the cystic lesion can cause mild obstruction, allowing for translocation of bacteria from the portal circulation, as well as stasis within the bile ducts, promoting the development of acute cholangitis. Classic findings of obstructive cholestasis include direct hyperbilirubinemia and elevated alkaline phosphatase; the addition of severe pain, vomiting, fever, and leukocytosis is indicative of acute cholangitis. Transaminases may be mildly elevated. Regardless of the underlying etiology, acute cholangitis is treated with intravenous fluid, antibiotics, and urgent biliary drainage. Drainage is best achieved using endoscopic retrograde cholangiopancreatography (ERCP), with a sphincterotomy and/or a biliary stent is used to facilitate the flow of bile. Once the patient has recovered from acute cholangitis, complete surgical excision of the biliary cyst should be performed to reduce the risk of developing cholangiocarcinoma. endoscopic retrograde cholangiopancreatography (Choice A) After biliary drainage with ERCP, acute cholangitis due to choledocholithiasis, a diagnosis more common in adults, warrants cholecystectomy to prevent recurrent episodes. This patient has no stones; therefore, cholecystectomy would not prevent the recurrence of acute cholangitis in this patient. (Choices B and D) Cystoenterostomy (ie, connecting the biliary cyst to the small bowel) and percutaneous drainage both facilitate biliary flow in patients with cholestasis. However, both are more invasive than ERCP and therefore are not first-line management. Moreover, cystoenterostomy does not prevent the risk of cholangiocarcinoma associated with biliary cysts and would not be performed in lieu of complete cyst excision. Educational objective: The initial presentation of a biliary cyst may be acute cholangitis and should be suspected in a patient with fever, right upper quadrant pain, and obstructive jaundice, as well as a cystic bile duct mass. Initial management of acute cholangitis is urgent biliary drainage (ie, endoscopic sphincterotomy); complete surgical excision of the cyst should be performed later to reduce the risk of cholangiocarcinoma. References: The role of endoscopic retrograde cholangiopancreatography in choledochal cysts and/or abnormal pancreatobiliary junction in children. (http://www.ncbi.nlm.nih.gov/pubmed/29359001) Impact of previous cyst-enterostomy on patients' outcome following resection of bile duct cysts. (http://www.ncbi.nlm.nih.gov/pubmed/27358675)

A 12-Year-Old Girl Is Brought to the Emergency Department Due