Could you send the MRCP images?

Ok, if you have involved the left ducts too It's a type IV Klatskin tumor. If the patient has a big S6-7, then you could performe Left PVE and S8 or S5 or both and after extended left hepatectomy. Yet you did not say us anything about hepatic arteries or portal vein branch involvement, therefore our ideas could be no useful. Big hug Marcel

Dear Marcel,

I would just go ahead with right hepatectomy + biliary confluence resection + segment I resection, maybe after PVE and left biliary drainage for FLR < 35%.

Warmest regards.

Luca

Dear Marcel,

agree with Luca, would do a right resection with Sg 1 and put a Neuhausdrain in the right side, but only if the patient is not cholestatic. If the patient is cholestatic (i.e. the left lobe cannot adequately drain the bile, resection is not an option. That is a palliative situation. 

Dear Marcel,

I generally agree with the above mentioned considerations, but in alternative You could try the so-called "Tay-Mahal resection" proposed by Kawarada et al on 1999 (J Gastroint.Surg). If You do not have an extension of the tumor on S6-S7 (bifurcation of the right posterior branch), You can perform it, obtaining radicality (Caudate Lobe specimen inclusion), living left liver in situ.

Warmest Regards

Marcello

Thank you guys for the tips to this challenging case. MRI show no vascular invasion in either side. Bilirubin level was controlled with TPBD. I will operate him next week and try one of the above ideas depending on the intraoperative findings. I will come back and tell you what I did.

Boa sorte, Marcel. Espero que corra bem.

I agree whit surgeons for a right hepatectomy. Good luck!!  

What'about the colon....any IBD?

Dear Marcel

Challenging case indeed!

You don´t tell us the age of the patient, anyway, I fully agree with Luca´s comments.

In the future, If the patient is lucky with no recurrence of the tumor, she/he could benefit from a transplant in case of PSC evolution provided an adequate period of time, maybe 5 years, has passed since tumor resection.  

Good luck and best regards

Good Luck Marcel, you're going to give the patient the best chance!

Warmest regards.

Luca

Some further informations. Patient is a male, 41-year-old with no liver symptons before the onset of jaundice. He has ulcerative colitis with good relief of symptons with adequate treatment. He was on vacation in Germany when he became jaundiced. His extensive workup showed a segmental sclerosing cholangitis. All biopsies were negative (including spyglass examination of the common bile duct). He received double stenting (PTBD). He came back to Brazil. He was operated on in Brazil with local resection followed by extended lymphadenectomy and Roux-en-Y hepaticojejunostomy. Frozen sections came negative for cholangiocarcinoma and distal and proximal margins were free. Portal vein and hepatic artery were easily skeletonized, given the impression that it was indeed a benign stricture. Intraoperative cholangiogram showed left liver more sick by PSC than the right liver. Definitive pathology showed no lymphnodes metastasis and a definitive cholangiocarcinoma within the thick common bile duct. Proximal margins were positive and distal were negative. He is now with normal liver function and no jaudice. He was then referred to me. This is the complete case. Any change in the treatment?

 Now the patient has an anastomosis to the common hepatic duct (with positive margin);  How has you made the diagnosis of klaskin IIIA? Intraop cholangiogram or other studies? How can you be sure that the disease affecting the left lobe is only PSC and not cholangio? Remember that  diagnosis has been made by definitive pathology examination and not prior to surgery even after an extensive workup. If the patient has any or multiple intrahepatic cholangios, any further surgery may not be of any benefit. Any data about tumor markers?

The diagnosis of IIIa was done by the preoperative cholangiogram (ERCP). The disease affecting left lobe is clearly PSC. We can see more ductopenia in the left lobe, i.e. biliary tree from the left liver is more scarce than the right. Liver biopsies from right and left show same degree of PSC. CA19-9 of 342.

So, although the patient was supposed to suffer a Klatskin IIIA tumor he received a local resection and now you are considering the right hepatectomy plus caudate lobe as the adequate oncological procedure. In this case, considering all circumstances you have presented, I would go on with the intervention.

Good luck and best wishes 

Totally agree!

These are some of the images of MRI before operation.

These are the intraoperative view of right and left liver of diagnostic laparoscopy performed in Germany before patient returned to Brazil.

Intraoperative views don´t have good quality but both lobes look the same to me. The MRI is difficult to evaluate as the left lobe is not completely drawn; however it would be better if you could have a new MRI  to evaluate the situation after the operation. Moreover, the coronal view shows a small left lobe with relatively big segments 6 and 7. Do the patient have sufficient remnant liver for a right hepatectomy plus caudate lobe taking into account that the parenchyma is not a normal one?

These are the ERCP images performed in Germany before patient returned to Brazil. Note that a tumor is inside the bile duct. It was biopsied and returned negative for tumor.

I decided to review the pathology taken at the surgical exploration where lymphadenectomy was performed alon with resection of the common bile duct until the bifurcation. The "tumor" inside the bile duct was inflammatory that is the reason why both pre-operative biopsy and frozen section was negative. However, definitive analysis came back cholangiocarcinoma. In the review, my pathologist said that there was cholangiocarcinoma around the bile duct and not inside, given the idea that the tumor began inside the liver and cause obstruction of the main bile duct. With this new information, our thinking was that a cholangiocarcinoma arised in the intrahepatic  bile duct, on the left or the right liver. I was not convinced that it was a IIIa because left liver was much smaller than expected. My feeling was that the tumor could in the left side with invasion of the right bile duct. Therefore I decided not to perform PVE and re-explore the patient. If the tumor was from the right side, I would go for a right trisectionectomy after PVE.

I finally operated on the patient and found a tumor coming from the left side of the caudate lobe with invasion of the bifurcation. Left liver was really small and with signs of chronic obstruction (this was not possible to see before because in the first MRI the patient had stents in both sides and after the first operation, hepaticojejunostomy was obstructing the view of the hilar plate.

We decided to perform a left hepatectomy including caudate lobe and to redo the hepaticojejunostomy with the same jejunal loop (adequate lenght). Frozen section showed tumor on the left liver and left hepatic duct. Right bile duct was free of tumor. Anyway, we dissected the right duct until bifurcation (anterior and posterior section) and we resect the right bile duct to be sure.

Final pathology came negative for right duct with free margins. Patient developed bile leak, limited. The operation was only 3 weeks after first operation and was really difficult due to hard adhesions. It would be better to wait a little longer but the family did not want to risk stay with positive margins.

Here are some photos taken from the operation.

Case 1 shows the small left liver

Case 2 shows the different coloration between left and right liver

Case 3 shows final view after left hepatectomy and redo HJ

Thank you everybody for the help and great inputs in this challenging case.

Marcel

Nice solution, Marcel. Fortunately, the tumor arose from the left lobe and not from the right as the left lobe seemed to be small as I told you in my previous comment.

Thank you for this challenging case and the nice discussion that it has generated.

I will use it in next case-session at my unit.

Best regards,

Mikel

Congrats Marcel, in the end you didn't need to remove S5-8, great!!! Left hepatectomy permits to leave more parenchima. See you

Thank you guys for the immediate support and great suggestions.