Abstract
Portal cavernoma cholangiopathy (PCC) is a difficult clinical problem, where the portal cavernoma is both the cause of biliary obstruction and the obstacle to its safe surgical treatment. The available endoscopic and surgical treatment is successful in majority and further intervention is seldom required since the native liver is normal. PCC is not an accepted indication for liver transplantation as only a small proportion of patients will fail both endoscopic and surgical treatment and progressive liver failure is rarely seen. Secondary biliary cirrhosis as a result of long standing biliary obstruction is an accepted indication however establishing a portal inflow in these patients is often difficult and challenging. The deceased donor liver transplantation would always be preferable over living donor liver transplantation as PCC is usually a non-emergency transplant and the graft can have portal blood inflow through a conduit to even a small segment of patent portal venous system or even to a cavernoma vessel.
Keywords: portal biliopathy, pseudosclerosing cholangitis, extrahepatic portal venous obstruction, portal hypertension
Abbreviations: PCC, portal cavernoma cholangiopathy; DDLT, deceased donor liver transplantation; LDLT, live donor liver transplantation
Portal cavernoma cholangiopathy (PCC) is a difficult clinical problem, where the portal cavernoma is both the cause of biliary obstruction and the obstacle to its safe surgical treatment. The incidence of PCC ranges between 5% and 18%.1–5 The available endoscopic and surgical treatment is successful in majority.6–8 However a subgroup of patients including those with recurrent gastrointestinal bleeding and recurrent cholangitis may be difficult to manage if both endoscopic and surgical intervention fails or is not feasible. In such cases the available treatment options are limited. Liver transplantation is a difficult option for PCC but has the potential of treating symptomatic PCC and varices in one procedure. If there is blood flow to the intestine, it stands to reason that there will be portal outflow from the intestine through collaterals. With modern imaging it may be possible to locate one of these collaterals and plan a liver transplant using this as an inflow vessel. However if no tributary or collateral of the portal venous system is identified, then liver transplant will not be possible as caval inflow will not solve the problem of portal hypertension. For example the transplanted liver can be perfused through inflow from the bile duct varices that are often present.
Liver transplantation for PCC
The literature is scarce on transplantation as a therapeutic modality for PCC. The standalone indication of liver transplantation for PCC is secondary biliary cirrhosis occurring as a result of long standing biliary obstruction; however this is rarely seen in the present aggressive endoscopic stenting era.
Till now only few case reports are available in literature where liver transplantation has been done for PCC after failure of endoscopic and surgical measures. The first reported case of liver transplantation for PCC was in 2004 in a 40-year-male patient with progressively worsening liver functions with grade IIIb PCC.9,10 Percutaneous bile drainage did not relieve the jaundice and was complicated by a bilioportal fistula with hemobilia. Organ transplantation was judged as the best therapy because of his poor general condition, the abundant angioma-like intrahepatic tissue, and the presence of both extrahepatic and bilateral intrahepatic bile duct stenosis.
Another reported case of liver transplantation for PCC was of a young patient with a 17-years long history of non-cirrhotic portal hypertension and cavernous transformation of the portal vein (predominantly intrahepatic portal cavernoma).11 Patient developed PCC not alleviated by repeated endoscopic stent insertion, clinically mimicking primary sclerosing cholangitis and cholangiocarcinoma. The patient underwent deceased donor liver transplantation (DDLT) with a suspicion of cholangiocarcinoma on imaging studies to take care of both the primary disease and the suspected malignancy.
We reported the first case of live donor liver transplantation (LDLT) for PCC in a 26-year-old Indian man from United States who presented with growth retardation during childhood, recurrent episodes of cholangitis for 11 years, upper gastrointestinal hemorrhage, and generalized weakness for 4 years. At the age of 15-years he developed features of hypersplenism for which he underwent a splenic artery embolization. One year later, he developed obstructive jaundice. Subsequently he had recurrent episodes of obstructive jaundice with cholangitis and upper gastrointestinal haemorrhage which required multiple endoscopic retrograde cholangiopancreatography and stenting and endoscopic variceal ligation. He also had 2 episodes of life-threatening sepsis owing to cholangitis requiring immediate stent exchange and admission to intensive care units. Considering his young age, intractable symptoms, failure of endoscopic therapy, nonfeasibility of other treatment options, poor quality-of-life, and nonavailability of deceased-donor organ, the option of an LDLT was given to him. He underwent a successful right lobe graft without the middle hepatic vein, donor being his 48-year-old mother.12
LDLT as a first line therapy was also reported from China in three pediatric patients with extensive intrahepatic cavernous transformations, varices, hypersplenism and liver decompensation in one patient because of concerns regarding the expected low effectiveness of a surgical portosystemic shunt in pediatric population.13 The donor was the child's father in two cases and the patient's mother in the third case. All donor grafts were taken from the left lobe (segments 2, 3, 4, and middle hepatic vein). Graft hepatic vein was used to extend the portal vein for anastomosis in all three cases. In the first patient usual venous anastomosis of the donor portal vein to a large recipient collateral vein of portal vein was made whereas in the second case anastomosis was done to the confluence of the splenic and superior mesenteric veins. Among the three PCC patients, the tortuous, closely packed veins and venules formed a sponge like lesion that extended into the intrahepatic portal lobar branches. Hilar fibrosis and inflammation made hilar dissections around the hepatoduodenal ligament very difficult and laborious. Some technical difficulties were encountered, including the dissection of a portal vein that featured no obvious lumen, hemorrhage and the separation of the bile duct from the portal vein. However the transplantation procedure was technically feasible, and there was no procedure-related mortality in any patient. The profile of patients of PCC undergoing successful liver transplantation is given in Table 1.
Table 1.
Profile of Patients of Portal Cavernoma Biliopathy Undergoing Successful Liver Transplantation.
| Authors | Age (years) | Bilirubin (mg/dL) | Type of surgery | Portal vein reconstruction or portal inflow | Indication for liver transplantation | Outcome | Follow-up |
|---|---|---|---|---|---|---|---|
| Francoet al9 | 40 | 29 | DDLT | Iliac vein graft to achieve a complete cavoportal transposition | Progressive liver failure and intra- and extra-hepatic bile duct stenosis | Successful | 12 months |
| Hajdu et al11 | 43 | 11.8 | DDLT | – | Recurrent cholangitis and suspicion of cholangiocarcinoma | Successful; No tumor identified | 12 months |
| Zhang et al13 | 9 | 1.01 | LDLT | Venous anastomosis of donor portal vein to a large recipient collateral vein | PCC, hypersplenism | Expired | 7 days |
| 7 | 2.73 | LDLT | Short bridging vein graft to portal vein confluence of splenic and superior mesenteric vein | PCC, Hypersplenism | Successful | 26 months | |
| 10 | 12.71 | LDLT | Jump bypass graft was anastomosed end-to-side to superior mesenteric vein | PCC, hypersplenism, hepatic encephalopathy | Successful | 26 months | |
| Gupta et al12 | 26 | 1.50 | LDLT | Infracolic collateral | Life threatening sepsis, recurrent cholangitis, recurrent gastrointestinal bleed | Successful | 24 months |
DDLT, Deceased Donor Liver Transplantation; LDLT, Living Donor Liver Transplantation; PCC, portal cavernoma cholangiopathy.
Deceased versus living donor liver transplantation
DDLT is always the preferable surgery as PCC is usually a non-emergency transplant, good graft portal vein length and veins for conduits are available and recipient hepatectomy is easier. However organs from living donors offer many potential advantages over organs from brain-dead donors. The most important advantages of live donation are that it optimizes the timing of transplantation and frees patients from the waiting list. Preservation time is minimal in LDLT, so there is significantly less ischemic damage to the liver.14 LDLT is difficult, but may be an option if emergency transplant is needed and patency of some part of portal venous system is there. However the disadvantages to live donor transplantation must be considered carefully. The donor, a perfectly healthy volunteer, faces unequivocal risks of morbidity and even mortality. In LDLT there are no cadaveric venous grafts and it is a difficult surgery as extensive retroperitoneal collaterals are present in these patients with long standing portal hypertension.
Conclusion
Liver transplantation can be considered rarely in patients with PCC. However it still remains a difficult option and a last resort when all modalities fail. Secondary biliary cirrhosis is the only accepted indication, however it may not be feasible in all patients due to difficulty in establishing a portal inflow.
Conflicts of interest
All authors have none to declare.
References
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