Tumor or tumor-like lesions are uncommon in children, especially young infants. Therefore, diagnosis could be difficult based on clinical presentation. Because of tumor position, clinical features mimic other common conditions with obstructive jaundice and cholangitis, e.g., choledochal cyst, and biliary ascariasis [2,3,4,5,6]. The preoperative diagnosis in our patient was a choledochal cyst.

Sonography detected the proximal bile duct dilatation but could not detect the mass in our patient. Similar sonographic findings have been reported in children with extrahepatic bile duct tumors; even a CT scan has missed the mass in a 10-year-old boy with bile duct tumor [2, 7]. This might be due to the unfamiliarity of these masses to the radiologist, which makes the preoperative diagnosis more confusing. However, a preoperative radiological delineation of the extrahepatic bile duct should be attempted for proper surgical planning. We perform an MRCP in most children when a USG indicates the presence of a choledochal cyst. However, in this particular patient, we decided to skip this investigation due to several reasons. Firstly, our hospital lacks the facilities to conduct an MRCP; patients must bear the cost of undergoing the procedure at private facilities. Additionally, the 2-month-old girl would require anesthesia outside our hospital, which could pose a risk. Thirdly, the investigation cost, which was higher than 150 USD, was not affordable for the parents.

Though rare, cholangiocarcinoma has been reported in older children. This malignant tumor has a poor prognosis in children [1]. Therefore, preoperative diagnosis, at least per-operative confirmation of the histopathological type is of utmost importance. Per-operative frozen section biopsy of the mass helps in decision-making for pre-operatively unidentified tumors [2, 7]. At our center, facilities for a frozen section biopsy were not available. Fortunately, we could excise the tumor completely, which later turned out to be a benign fibroma. We have re-established the bile flow with a Roux-N-Y hepatico-jejunostomy. A malignant mass would have required a more extensive resection and reconstruction.

Chung et al. reported 61 cases of infantile fibromatosis; 16 were multicentric. Most cases were noted at birth or shortly after birth. Head and neck was the most common site, followed by trunk and extremities. Only four children had visceral involvement, and three died within 48 h because of extensive multi-organ fibromatosis. The alive baby had fibromatosis in the colon. None of them had bile duct involvement. Three of the solitary lesions recurred [8]. Our patient had no evidence of multi-organ involvement.

A few cases of bile duct fibroma have been reported in adults. Fibromas of various sizes and shapes led to obstructive jaundice in those patients. This presentation is similar to our patient’s. The morphology of bile duct fibromas was similar to fibromas elsewhere [9].

We are following the patient at 3-month intervals with liver function tests and sonography to detect recurrence early, if any. There is no sign of recurrence at 6 months. We advised the parents to do a half-yearly follow-up. One case has been reported to recur 1 year after bile duct teratoma excision at neonatal age [3]. Recurrence of childhood fibroma has been reported at 15 years of age [8].

The gross appearance of the liver and its histopathology showed early cirrhotic change. This might be due to cholestasis caused by the tumor. Therefore, early resection of the tumor and restoration of bile flow is recommended to avoid the progression of liver damage.