A previously healthy 21-month-old girl was taken to the clinic for fever of unknown origin of five days duration.
The clinical diagnosis of maxillary sinusitis was made with oral amoxicillin.
After 10 days of persistent fever, jaundice, choluria, acholia, pruritus and discomfort in the right hypochondrium were observed, so the patient was hospitalized.
Laboratory results were compatible with hematocrit 16200: direct bilirubin 4 mg/L, indirect bilirubin 0.8 mg/dl, AST 201 U/L, ALT 171 U/L, ALT 5134 U/L, 36% white blood cells.
Abdominal ultrasound and magnetic resonance cholangiography (MR cholangiography) were performed, which showed dilatation of the intrahepatic and extrahepatic bile duct throughout its extension.
At the distal end of the common bile duct, small images of filling defect compatible with detritus or lithiasis were observed at the MR cholangiography.
With the suspicion of choledochal cyst, 48 hours after admission, an endoscopic retrograde cholgipancreatography (ERCP) was performed with abundant sphincterotomy.
After the procedure, the patient showed a clear clinical improvement, with disappearance of symptoms and normalization of ultrasound and laboratory parameters.
A month later, symptoms of pruritus, jaundice and fever reappeared.
In the laboratory, a new increase in the levels of direct bilirubin and alkaline phosphatase similar to those detected at the onset of symptoms was observed.
With suspicion of cholangitis, he was re-operated to repeat ERCP and then, in a deferred way, to perform the surgery of the supposed choledochal cyst.
An edematous papilla with the bile duct was found during ERCP.
A balloon and candidosis were passed and abundant whitish and mucus membranes were obtained.
The material was sent to culture (positive for Escherichia coli) and pathology.
The patient showed improvement, which lasted only 48 hours, so it was decided to perform the resection surgery of the choledochal cyst by means of a hepatoduenoanastomosis, which was performed laparoscopically without difficulties.
The symptoms disappeared, the patient was hospitalized and he fulfilled the intravenous antibiotic treatment as prophylaxis of cholangitis by Escherichia coli.
The pathology report of the sample obtained in the ERCP was received, which was: embryonal rabsarcoma of the biliary tract.
Histology and immunohistochemistry of the surgical specimen confirmed it.
Tumor staging was performed by total body scintigraphy with 99Tc, computed tomography of the chest and abdomen, and biopsy of the bone marrow, and the presence of distant metastases was ruled out.
Chemotherapy was initiated according to the protocol EpSSGRMS 2005 with vincristine, actinomycin and ifosfamide.
In the evaluation performed after the third cycle, a clear decrease in the known mass (partial remission) was observed.
The patient was evaluated by the Surgery Department, which determined that it was not feasible to perform a complete resection due to the tumor location.
Radiotherapy was indicated for local tumor control and complete chemotherapy cycles according to protocol.
