A previously healthy female patient with a history of controlled pregnancy, born at term 38 weeks, small for gestational age (2,670 kg).
She was admitted at 47 days of life with a 20-day history of progressive jaundice and choluria, with good weight gain, and was referred to the regional hospital for study.
Symptoms with cholestatic pattern, in which there was an increase in total bilirubin (BT), allergic contact tracings of direct bilirubin (BD) (12.75 mg/dl-10.18 mg/dl), and gamma-gluteptate
Viral hepatitis was diagnosed at the Regional Hospital and serology for hepatitis A, B and C was negative.
Acolic stools were found favorably.
She was referred to gastroenterology for previous control tests that maintained a cholestatic pattern with elevated BD and transaminase levels 204 BT 10.58, BD 7.88, GOT 109.
At 52 days of life, an abdominal ultrasound was performed, which showed intrahepatic biliary tree dilatation and a gallbladder present but collapsed despite fasting.
Due to the presence of the gallbladder and the large dilatation of the intrahepatic bile duct, a liver biopsy was performed and a magnetic resonance cholangiography was performed.
It showed a normal liver with conserved parenchyma, slight dilatation of intrahepatic bile ducts to the level of its confluence, distally it was not possible to visualize extrahepatic bile ducts.
Biliary atresia was diagnosed with cholangioresonance and biliary bypass surgery (Kasai) was decided.
Attention was paid to the decrease in bilirubin levels in serial examinations, as well as to seizures.
Due to the presence of acolic stools, surgical exploration was decided.
During surgery the hepatic surface was visualized as congestive liver (confirmed by biopsy), gallbladder present, lumen and bile inside.
Intraoperative cholangiography showed a critical stenotic zone at the common hepatic duct, achieving contrast passage towards the distal (intra- and extra-hepatic) bile duct with intrahepatic proximal bile duct.
Cholecystectomy and common hepatic duct dissection were performed, demonstrating stenosis at the level of the duct caused by a vascular ring originating from the right hepatic artery.
The biliary tract was resected proximally to the stenosis by performing a hepatic-duene anastomosis.
Abdominal drainage and nasogastric tube were installed.
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The patient recovered uneventfully, with debit decreasing due to the drainage removed at 6 days and feeding at 4 days.
She was discharged 8 days after surgery with decreased liver tests (BT 3.98, BD 3.46, GOT 266, GPT 134, FA 431, GGT 586).
In outpatient controls the patient was asymptomatic, with good oral tolerance, stained stools and normal liver and general tests.
