Robotic-Assisted Surgery Approach in a Biliary Rhabdomyosarcoma Misdiagnosed as Choledochal Cyst
A 2 years-old girl was admitted for loss of appetite and slight fever. Laboratory studies showed: AST 537 mU/mL, ALT 589 mU/mL, GGT 738 mU/mL, alkaline phosphatase 718 mU/L, total bilirubin 1.8 mg/dL. Hepatic infection profile and tumor markers were negative. On physical examination there was no tenderness, hepato-spleenomegaly or abdominal mass. Abdominal ultrasound showed hypoechoic dilatation of the common hepatic duct (CHD) and common bile duct (CBD). Thickening of the CHD walls with dilatation of the intrahepatic bile ducts were suggestive of CC. Abdominal CT scan showed an ovoid, heterogeneously non enhancing mass localized at the liver hilum of 18×30 mm with hyperdense contents. Magnetic resonance imaging (MRI) confirmed the presence of marked dilatation with thickened wall of CHD and CBD consistent with congenital CC type IV (). Laparoscopic intraoperative cholangiography, prior to choledochal cyst excision, is usually valuable for detection of biliary variants. Liver biopsies are routinely done for grading of liver fibrosis in obstructive cholangiopathy (). Unfortunately cholangiography in our case was not conclusive and CHD and intrahepatic biliary ducts were not visualized. The biopsies showed fibrosis of the septum at the portal space associated with signs of chronic inflammation.\nThe child underwent mini-invasive robotic surgery for CC resection and Roux-en-Y choledochojejunostomy. Open umbilical insertion of the 8.5 mm stereoscope was used.\nTwo 5 mm working ports were placed at the left and right hypocondrium on either side of the umbilicus under direct vision, at 3 cm from the costal margins along the hemi-clavicular line bilaterally. A 3 mm servicing trocar was inserted in the right inferior iliac space. Transabdominal stitches were fixed on the gallbladder and on the falciform ligament for liver retraction. Pressure insufflations were around 4 mmHg. Circumferential dissection of the CC was achieved to the duodenum and ligated. Proximally dissection extended beyond the CHD to the left and right hepatic biliary ducts that were also involved in a grapelike tumor. The high degree of magnification allowed the resection to be carried out in the parenchyma to free the right posterior and anterior ducts and evidenced another supernumerary duct. On the left side, the lesion was freed from the grapelike cysts and healthy tissue was found at the two segmental tributaries. Although feasible by robotic approach, but still controversial in the literature, the anastomosis at the porta hepatis was carried out in open surgery. A retrocolic Roux-en-Y portoenterostomy was performed using 5/0 PDS. Histologic examination revealed a botryoidembryonal rhabdomyosarcoma of the bile ducts affecting both the CBD and CHD. The patient underwent chemotherapy after diagnosis. Twelve months later, the patient is relapse free, with normal bilirubin and liver profile.