Liver transplantation for severe intrahepatic noncirrhotic portal hypertension.

Intrahepatic noncirrhotic portal hypertension can be idiopathic or associated with known toxic, developmental, vascular, or biliary tract diseases. Most patients are successfully managed medically or with shunting procedures. The goal of this study was to explore the reasons some patients require orthotopic liver transplantation (OLT). The clinical features, gross and microscopic liver explant pathology, and posttransplantation course in 16 patients who underwent OLT for intrahepatic noncirrhotic portal hypertension were studied. There were 11 men and 5 women with a mean age of 47 years. Clinical manifestations included gastrointestinal varices (n = 12), ascites (n = 8), encephalopathy (n = 3), and hepatopulmonary syndrome (n = 3). Cirrhosis was misdiagnosed clinically, radiographically and/or histologically in 13 patients (81%). Grossly, liver explants weighed a mean of 1,100 g, and 12 had a nodular appearance. Histologically, all 16 livers had portal tract vascular abnormalities, 15 had nodular regenerative hyperplasia (NRH), and 9 had incomplete septal cirrhosis. After OLT, mild NRH features were noted in 2 patients, and 1 of these patients developed evidence of portal hypertension. This study demonstrates that a subset of patients with intrahepatic noncirrhotic portal hypertension have severe symptoms requiring OLT. Accurate pre-OLT diagnosis is frequently difficult at advanced stages of the disease; 81% of our patients carried a diagnosis of cirrhosis. Morphologically, the explanted livers showed evidence of vascular abnormalities, NRH, and increased fibrosis, but not cirrhosis. Importantly, however, a diagnosis of cirrhosis is not required in this group of patients to qualify them for OLT, and these patients have good long-term graft function after OLT.     

Portal vein pressure and graft oxygen consumption monitoring during liver transplantation

Abnormal splanchnic circulation (ASC) is often detected too late, when hepatic circulation is already irreversibly compromised. If we could detect surgical or metabolic problems early after graft reperfusion, we might be able to correct them immediately before the damage becomes irreversible. The aim of this study was to determine if ASC can be predicted early after liver transplantation (LT) using portal vein pressure measurements and graft oxygen consumption monitoring. PATIENTS AND METHODS: Twenty-patients (13 men, 7 women of mean age 46 years) undergoing LT with the piggyback technique for hepatitis C virus (HCV)/hepatitis B virus (HBV)-related cirrhosis were retrospectively divided in two groups. Group A (16 patients), in which LT was successful, and group B (4 patients) in which LT was unsuccessful because of primary nonfunction (2 patients), infrahepatic portal vein thrombosis (1 patient), or hepatic vein kinking (1 patient). We then compared the portal blood pressure values and the prehepatic and posthepatic oxygen content difference (p-pDO(2)) before portal clamping; at the end of anhepatic phase; 5, 15, and 25 minutes after portal vein (PV) reperfusion; and 5, 20, 40, and 100 minutes after hepatic artery anastomosis. RESULTS: Early after graft reperfusion; portal pressure decreased to levels lower than that at baseline in group A, but remained high until the end of surgery in group B. At the end of surgery, p-pDO(2) increased more among group B than group A. CONCLUSION: ASC, specifically an increased PV resistance, can be predicted early after LT by portal vein pressure measurements and graft oxygen consumption monitoring.     

The Effect of Doppler Ultrasound on Early Vascular Interventions and Clinical Outcomes After Liver Transplantation

Background

During the immediate postoperative period after liver transplantation (LT), postoperative bleeding and vascular complications (stenosis, thrombosis) are the two most common complications that require therapeutic decisions. Doppler ultrasound (DUS) is the established method for screening vascular patency after LT during the immediate postoperative period. The objective of our study was to evaluate the impact of DUS performed on postoperative days (POD) 1 and 2 on early vascular interventions.

Methods

We studied 200 patients who had undergone living donor or deceased donor liver transplantation between January 2011 and March 2012. Postoperative liver DUS findings of up to POD 14, including patency of hepatic artery, portal vein, and hepatic vein, were retrieved. Patients with normal DUS findings on POD 1 and POD 2 were classified as the normal early DUS group. Patients with abnormal DUS findings at POD1 or POD2 were classified as the abnormal early DUS group. Frequency of vascular interventions was compared between the two groups. Risk factors that predict vascular interventions also were assessed.

Results

On POD 1 and 2, 81.5 % (163/200) had normal DUS findings and management was not altered by subsequent DUS findings. Two patients in the normal group were found to have hepatic artery dissection and hepatic vein thrombosis on routine CT on POD 7 and received vascular intervention. DUS results in the two patients were normal until POD 6, but DUS performed after the CT on POD 7 were consistent with the CT findings. Of the 37 recipients who showed abnormal DUS findings on POD 1 or 2, the DUS findings were normalized or unchanged thereafter in 33 patients and no vascular interventions were performed. Two patients underwent hepatic artery thrombectomy on POD 2, one patient required a portal vein thrombectomy on POD 1, and one patient died on POD 3 due to bleeding. The overall incidence of vascular complication requiring vascular interventions was 2.5 %. Logistic regression identified abnormal DUS findings on POD 1 or 2 as an independent risk factor of vascular complications requiring intervention.

Conclusions

In LT recipients who demonstrate normal DUS findings in the first 2 postoperative days, additional DUS screening may have value only when clinically indicated.     

Congenital absence of the portal vein and role of liver transplantation in children.

BACKGROUND/PURPOSE: Congenital absence of the portal vein (CAPV) is a subtype of congenital portosystemic shunt, which can cause a broad spectrum of clinical manifestations. The authors report on 4 patients with CAPV including a boy with CAPV-associated encephalopathy, which was resolved effectively by liver transplantation (LT). METHODS: The case records of 4 pediatric patients with CAPV who were referred to the author's institution between 1984 and 1999 were reviewed. RESULTS: The patients (3 boys and 1 girl) ranged in age at diagnosis from 0.8 to 14 years. Two patients had growth retardation or disturbed consciousness, and the other 2 had no specific manifestations. Not only high serum levels of bile acids, ammonia, and transaminases but also low plasma levels of branched-chain amino acids were common laboratory findings. The absent portal vein was replaced by a large portosystemic shunt, which connected the splanchnic vein to the inferior vena cava or the left renal vein. Two patients survived without any symptoms, but 1 with growth retardation died of hepatic failure. The other with encephalopathy did not respond to medical therapy and underwent LT, which resolved symptoms and metabolic disorders effectively. CONCLUSIONS: Patients with CAPV do not always have a good prognosis. They should be followed up with careful observation of their symptoms, hepatic function, and metabolic abnormalities. LT might be indicated for patients with symptomatic CAPV unresponsive to medical therapy.     

Resection for hepatocellular carcinoma is a good option in Child-Turcotte-Pugh class A patients with cirrhosis who are eligible for liver transplantation.

The best treatment option for patients with single, early hepatocellular carcinoma (HCC) and cirrhosis, good liver function, and absence of portal hypertension remains to be established. The aim of this work was to compare the outcome of liver resection (LR) with that of liver transplantation (LT) for single, early HCC in Child-Turcotte-Pugh class A patients with cirrhosis younger than 70 years of age. Thirty-seven of 134 patients who underwent LR and 36 of 125 who underwent LT for HCC in our unit fulfilled the inclusion criteria. No differences were observed in mean tumor size (3 cm); HCV cirrhosis predominated in the LT group and older age in the LR group. Postoperative mortality was higher and hospital stay longer in the LT group. Patient survival was similar in both groups. Tumor recurrence was higher in the LR group (59% vs. 11%), extrahepatic recurrences predominated after LT and hepatic recurrences after LR. Disease-free survival was significantly better after LT. Eighteen patients presented hepatic recurrence after LR: 5 advanced and 13 early. Seventeen patients--13 with early HCC recurrence and 4 with liver failure--were potential candidates for salvage LT. However, 10 of 17 patients were older than 70 years at this time. Salvage LT could only be performed in 6 patients: 5 for HCC recurrence and 1 for liver failure. Results of salvage LT were similar to those of primary LT. In conclusion, only 27.6% of resected patients were eligible for LT. LR is a good option since it offers similar survival to LT. Salvage liver transplantation was performed in 16.2% of resected patients, with older age being the main contraindication. Outcome of salvage LT was similar to that of primary LT.     

Veno-Occlusive Disease of the Liver After Lung Transplantation

Veno-occlusive disease (VOD) of the liver is mainly described after chemo-irradiation conditioning regimens during haematopoietic stem cell transplantation (SCT) and has been sporadically reported after kidney and liver transplantation. In the latter cases, it is commonly attributed to azathioprine and/or tacrolimus. One case of tacrolimus-induced hepatic VOD developing after lung transplantation (LT) has been recently reported. Here we describe another case of VOD occurring after LT, but in which the causative role was played by azathioprine.     

Long-term results of living donor liver transplantation for glycogen storage disorders in children.

Liver transplantation (LT) may be indicated in glycogen storage disorders (GSD) when medical treatment fails to control the metabolic problems or when hepatic adenomas develop. We present our institutional experience with living donor LT (LDLT) for children with GSD. A total of 244 patients underwent primary LDLT at our institution from June 1994 to December 2005. A total of 12 (5%) children (8 female and 4 male) were afflicted with GSD and were not responsive to medical treatment. Nine patients had GSD type I and 3 had GSD type III. The median age at the time of transplantation was 7.27 yr (range, 2.4-15.7). All patients presented with metabolic abnormalities, including hypoglycemia, and lactic acidosis. In addition, 4 patients presented with growth retardation. A total of 11 patients received left lobe grafts and 1 received a right lobe graft. The mean graft-to-recipient weight ratio was 1.25 (range, 0.89-1.61). Two patients had hepatic vein stenoses that were treated by balloon dilatation; 1 patient had bile leak, which settled spontaneously. The overall surgical morbidity rate was 25%. Three patients had hepatic adenomas in the explanted liver. There was a single mortality at 2 months posttransplantation due to acute pancreatitis and sepsis. The mean follow up was 47.45 months. The metabolic abnormalities were corrected and renal function remained normal. In patients with growth retardation, catch-up growth was achieved posttransplantation. In conclusion, LDLT is a viable option to restore normal metabolic balance in patients with GSD when medical treatment fails. Long-term follow-up after LT for GSD shows excellent graft and patient survival.     

Accuracy of multidetector computed tomographic angiography for detecting hepatic artery complications after liver transplantation

Aims

The aim of this study was to evaluate the accuracy of multidetector computed tomographic angiography (MDCTA) for detecting hepatic artery complications after liver transplantation.

Methods

Between July 2001 and September 2006, 212 patients underwent liver transplantation including 110 (41 female and 69 male patients); of mean age, 24 years (range = 6 months to 66 years) who were assessed with MDCTA. First, arterial phase images obtained after intravenous injection of 150 mL of contrast at a rate of 4 mL/s were acquired using the bolus triggering technique. Then portal and late-phase images were obtained. Axial and coronal maximum intensity projection (MIP) images and volume-rendered images were produced from the axial image data. Arterial vascular complications were noted. Stenosis was defined as severe (>75%), moderate (≥50%), or mild (<50%) according to its diameter. Twenty-nine of the 38 individuals with hepatic artery complications detected by MDCTA had correlative digital subtraction angiography (DSA). Seven of 110 patients with normal hepatic artery and venous pathologies in MDCTA also had DSA to investigate venous complications.

Results

MDCTA showed hepatic artery complications in 38 of the 110 patients who were assessed with this modality. DSA confirmed the MDCTA findings in all but 1 of the 29 patients assessed with catheter angiography. Fourteen of the 38 individuals also underwent percutaneous interventions and treatment. Fifteen patients had early hepatic artery complications, and 23 late hepatic artery complications. The most common early complications were thrombosis (66.6%) and stenosis (26.6%). The most common late complications were stenosis (56.5%) and thrombosis (26%). If we evaluate the early and late complications, the incidence of late complications was greater than that of the early complications (61% vs 39%). There was no statistically significant difference in cadaveric and living donor liver transplants for early versus late or for type of complications.

Conclusions

MDCTA is noninvasive imaging modality that accurately shows a variety of vascular complications after liver transplantation. We suggest that if we suspect any vascular complication with Doppler ultrasound, we must perform MDCTA for diagnosis. If we detect severe/moderate stenosis, the patient must undergo DSA.     

Hepatic Vascular Disease After Kidney Transplantation: Report of Two Cases and Review of the Literature

Severe hepatic vascular disease developed in two patients 4and 8 years after kidney transplantation, while receiving combinedimmunosuppressive therapy with prednisone and azathioprine.Portal hypertension and marked liver failure were observed inboth cases. The diagnosis was established by histological examinationof liver biopsies showing typical veno-occiusive disease ofthe liver associated with peliosis in both cases. Azathioprine was discontinued. Two years later one patient wasasymptomatic and liver function tests were normal. The secondpatient died 3 years later from liver failure. Early recognitionof hepatic vascular disease arising in kidney transplant recipientswould be of utmost importance, as substitution of another immunosuppressiveagent for azathioprine could halt the process leading to portalhypertension.     

Development of pulmonary hypertension in 5 patients after pediatric living-donor liver transplantation: de novo or secondary?

The development of portopulmonary hypertension (PH) in a patient with end-stage liver disease is related to high cardiac output and hyperdynamic circulation. However, PH following liver transplantation is not fully understood. Of 617 pediatric patients receiving transplants between June 1990 and March 2004, 5 (median age 12 yr, median weight 24.5 kg) were revealed to have portopulmonary hypertension (PH) after living-donor liver transplantation (LDLT), as confirmed by echocardiography and/or right heart catheterization. All children underwent LDLT for post-Kasai biliary atresia. In 2 patients with refractory biliary complications, PH developed following portal thrombosis; 2 with stable graft function, who had had intrapulmonary shunting (IPS) before LDLT, were found to have PH in spite of overcoming liver dysfunction due to hepatitis. PH developed shortly after distal splenorenal shunting in 1 patient, who suffered liver cirrhosis due to an intractable outflow blockage. The onset of PH ranged from 2.8 to 11 yr after LDLT, and mean pulmonary artery pressure (mPAP) estimated by echocardiography at the time of presentation ranged from 43 to 120 mmHg. Three of the 5 patients are alive under prostaglandin I2 (PGI2) treatment. Of these, 1 is prepared for retransplantation for an intractable complications of liver allograft, while the other 2 with satisfactory grafts are being considered for lung transplantation. Even after LDLT, PH can develop with portal hypertension. Periodic echocardiography is essential for early detection and treatment of PH especially in the recipients with portal hypertension not only preoperatively but also postoperatively.     

Liver transplantation after severe hepatic trauma: a sustainable practice. A single‐center experience and review of the literature

Severe hepatic trauma is a rare indication for liver transplantation (LT). We report our single‐center experience of LT for hepatic trauma. Four new cases are discussed in light of a literature review in order to depict the pathways leading from hepatic trauma to LT and to assess the outcomes of this practice. LT is generally indicated in case of uncontrollable hemorrhage, acute liver failure, or post‐traumatic late sequelae. Hepatic vessels thrombosis, sepsis, major hepatic resections, and a late referral are factors associated with the progression toward irreversible liver failure. Considering all reported cases, early patient and graft survival reached 68% and 62%, respectively, but in the last decade both have improved to 84%. LT after severe hepatic trauma is a sustainable practice considering the current good outcomes and the ineluctable death of these patients without LT.     

Prospective evaluation of the role of quantitative Doppler ultrasound surveillance in liver transplantation.

Doppler ultrasound (DUS) is able to measure parameters of blood flow within vessels of transplanted organs, and vascular complications are associated with abnormal values. We analyzed the findings of 51 consecutive patients who underwent DUS on 2 occasions in the first postoperative week following liver transplantation for cirrhosis to determine the range of values in patients following liver transplantation. Three patients developed early vascular thromboses that were detected by the absence of a Doppler signal. In patients making an uneventful recovery, the arterial velocity tended to increase and the resistive index (RI) to decrease during the first postoperative week. All recipients were shown to have high-velocity segments within the hepatic artery, without an increase in flow resistance. Assessment of the portal vein revealed narrowing at the anastomosis, associated with a segmental doubling of flow velocity, and the mean portal venous flow decreased by approximately 20% in the first postoperative week. In conclusion, a wide range of abnormalities occurs in the vessels of liver transplant recipients, which were not associated with the development of vascular complications or affect patient management.     

Hepatic venous pressure gradient to assess fibrosis and its progression after liver transplantation for HCV cirrhosis.

Progression of fibrosis following recurrent hepatitis C virus (HCV) infection is frequent after liver transplantation (LT). Histology remains the gold standard to assess fibrosis, but the value of hepatic venous pressure gradient (HVPG) is being explored. We evaluated patients with recurrent HCV infection after LT to assess whether HVPG correlates with liver histology, particularly fibrosis. A total of 90 consecutive patients underwent 170 HVPG measurements concomitant with transjugular liver biopsy (TJB), with 31.5 (range, 6-156) months of follow up. Median biopsy length was 22 mm and total portal tract count was 12 (complete 6, partial 6). Median HVPG was 4 mmHg: 38% of patients > or =6 mmHg (portal hypertension, PHT), 13% > or =10 mmHg. HVPG correlated with Ishak stage (r = 0.73, P < 0.001) for mild (0-3) and severe fibrosis (4-6), and grade score (r = 0.47, P < 0.001), but neither correlated with interval from LT nor biopsy length. HVPG was > or =10 mmHg in 15 patients: 12 had stage 5 or 6, and 3 severe portal expansion. HVPG was repeated in 49, between 7 and 60 months with weak correlation to fibrosis score (r = 0.30, P = 0.045). A total of 12 patients with HVPG > or =6 mmHg had fibrosis score < or =3, while 8 patients had normal HVPG but fibrosis stage > or =4. These discrepancies were mostly associated with specific histological features such as perisinusoidal fibrosis rather than errors in measuring HVPG. In 29 with HVPG <6 mmHg at 1 yr, none decompensated compared to 4 of 13 (31%) with PHT. In conclusion, HVPG correlates with fibrosis and its progression, due to recurrent HCV infection, assessed in TJB.     

Serious intestinal bleeding from vascular ectasia secondary to portal thrombosis after living-related liver transplantation in a child

Serious intestinal bleeding from vascular ectasia secondary to extrahepatic portal thrombosis is much less frequent than variceal bleeding, and its treatment is not clearly defined. We describe a 4-year-old girl with repeated intestinal bleeding from vascular ectasia, without any varix, with late extrahepatic portal vein thrombosis (PVT) and late hepatic artery thrombosis (HAT) after living-related liver transplantation. The bleeding stopped after simple splenectomy. She has presented neither bleeding nor any serious complications related to splenectomy for 1 year to date. We think uncontrollable hemorrhage from gastrointestinal vascular ectasia secondary to extrahepatic portal thrombosis in a pediatric patient can and should be treated by simple splenectomy, because patients with this complication usually have a normally functioning liver. However, it is not clear whether this procedure is effective for variceal bleeding.     

Significance of C4d deposition in the diagnosis of rejection after liver transplantation

C4d immunohistochemical staining of liver allograft biopsies was performed to assess its relationship to other pathological changes in the liver. C4d deposition was detected in 69.2% of liver graft biopsies from patients under going rejection, 33.3% of liver graft biopsies from patients with hepatitis B relapse after transplantation, and 28.6% of liver biopsies from patients with hepatitis B. When rejection occurred C4d deposition was located in the vascular walls of portal areas and hepatic sinusoidal walls. Examination of biopsies from patients with hepatitis B relapse after transplantation or hepatitis B infection showed C4d deposition only in the vascular walls of the portal area. C4d deposition in both vascular walls of portal area and hepatic sinusoidal walls was detected in only one of 12 ischemia-reperfusion damage cases. Repeated biopsy of the same patient 1 month later revealed acute cellular rejection. No C4d deposition was found in biopsies from a patient with bile duct occlusion after liver transplantation. C4d might serve as a sensitive marker for the diagnosis of liver rejection.     

Use of Preserved Vascular Homografts in Liver Transplantation: Hepatic Artery Aneurysms and Other Complications

Hepatic artery aneurysms/pseudoaneurysms (HAAs) are rare but serious complications after orthotopic liver transplantation (OLT). Revascularization should accompany aneurysmectomy if possible and is more feasible if the aneurysm presents late after transplantation. The optimal conduits for revascularization in this situation are not known. Two patients with hepatic artery aneurysms/pseudoaneurysms who had aneurysmectomy and revascularization with third-party cadaveric iliac arterial grafts 1 and 4 years after OLT are presented in detail, with an emphasis on the preservation method used for the grafts. Both livers were successfully revascularized with arterial grafts preserved for 21 and 26 days after procurement. Hepatic patency was documented in both 5 and 6 months after repair; graft function has remained normal 13 and 32 months after repair. Third-party vessels preserved for shorter periods have been used successfully in four other situations, including living-donor liver transplantation, and are briefly discussed. In conclusion, properly preserved vascular homografts are useful in LT for purposes other than initial vascular reconstruction. They also provide an excellent vascular conduit in recipients of livers from other (possibly living) donors.     

De novo hepatitis with autoimmune antibodies and atypical histology: a rare cause of late graft dysfunction after pediatric liver transplantation

BACKGROUND: Late graft dysfunction after orthotopic liver transplantation is commonly due to chronic rejection, recurrence of primary disease, sepsis, lympho-proliferative disease, or vascular or biliary complications. Herein we describe a subset of pediatric liver transplant patients in whom late graft dysfunction was associated with autoimmune markers, bile ductular proliferation, and portal infiltrates, which progress to fibrosis. This subset of patients has not been previously described. METHODS: Six of the 115 children followed for greater than 5 years after transplantation developed this unusual form of graft dysfunction. All children were on a low-dose single immunosuppressive therapy (mean trough cyclosporine concentration 89 microg/L) and had been tapered off steroids for a median duration of 1.5 year. Liver biopsies were performed in all children to evaluate the graft dysfunction, and the histologic findings were interpreted by an experienced hepato-pathologist. All patients were tested for antibodies to hepatitis C virus, hepatitis B surface antigen, and IgM antibodies to hepatitis A. Smooth muscle antibody, antinuclear antibody, and antibody to liver/kidney microsome type 1 were sought by indirect immunofluorescence. International Autoimmune Hepatitis Group scores were calculated. All patients underwent ultrasonography with doppler studies at the onset of graft dysfunction. Three patients with marked bile duct proliferation on histology had cholangiograms. RESULTS: Histology in all patients showed mononuclear cell infiltrates in the portal area with interface hepatitis, portal fibrosis, and ductular proliferation without duct damage or loss. All six patients had positive antinuclear antibody or smooth muscle antibody titers. Viral studies for hepatitis A, B, and C were negative in all patients. On the International Autoimmune Hepatitis Group scoring system, five patients had probable autoimmune hepatitis (score of 10-15) and one had definite autoimmune hepatitis (score > 15) at the onset of graft dysfunction. All were treated with azathioprine and prednisone similar to treatment for autoimmune hepatitis. However, despite aggressive treatment, four patients developed bridging portal fibrosis resulting in graft loss in two patients. CONCLUSION: This clinical constellation is associated with worse outcome then that previously described for pediatric patients with posttransplantation de novo autoimmune hepatitis. Further studies are needed to find an optimal treatment regimen for these patients.     

Late hepaticartery thrombosis after liver transplantation: which strategy? A single‐center retrospective study

The management of late hepatic artery thrombosis (LHAT) after liver transplantation (LT) is not codified. The objective of this study was to retrospectively evaluate outcomes after LHAT. All patients with HAT diagnosed 3 months or later after LT on computed tomography between 1993 and 2017 were included. Our policy was to apply a conservative management for asymptomatic or mild symptomatic patients and reserve retransplantation to symptomatic patients with diffuse cholangitis or liver abscess. A total of 56 patients were analyzed. LHAT diagnosis was made after a median interval of 48 months from LT (ranging from 3 to 368.3). At diagnosis, 28 (50%) patients were asymptomatic, 10 (17.8%) had mild symptoms (transient acute cholangitis), and 18 (32.1%) had severe complications. Asymptomatic patients experienced a 5‐year graft survival of 57% vs. 40% in those with mild symptoms and 11% in those with severe complications (P < 0.001). However, there was no difference in overall patient survival between groups. Our results suggest that conservative management of LHAT for asymptomatic patients or patients with mild complications is safe. Retransplantation should be reserved to patients with severe biliary complications.     

Living donor liver transplantation for multiple intrahepatic portosystemic shunts after involution of infantile hepatic hemangiomas

We describe a 6-year-old girl presenting with multiple intrahepatic portosystemic shunts after the involution of infantile hepatic hemangiomas (IHHs), who successfully underwent living donor liver transplantation. The chronological changes of radiologic findings indicated that remnant portovenous shunts at the time of IHHs involution developed gradually on the background of atrophic intrahepatic portal veins. This suggests that patients should be carefully followed up for the late onset of intrahepatic portosystemic shunts after the involution of IHHs.     

Vascular complications after orthotopic liver transplantation after neoadjuvant therapy for hilar cholangiocarcinoma.

Liver transplantation after neoadjuvant chemoradiotherapy has emerged as an effective treatment for patients with localized, node-negative, unresectable hilar cholangiocarcinoma (CCA) or CCA arising in the setting of primary sclerosing cholangitis (PSC). However, concern has arisen regarding the potential for vascular complications due to high-dose neoadjuvant therapy before transplantation. We reviewed our experience with specific aims to determine the incidences of arterial, portal, and hepatic venous complications in patients transplanted for CCA compared with patients who undergo transplantation for other indications, and to describe patient outcome as a result of these vascular complications. We reviewed data for all patients who underwent liver transplantation for CCA between January 1993 and April 2006 and compared the incidences of vascular complications to whole organ and living donor recipient control groups. Sixty-eight patients underwent neoadjuvant therapy and subsequent liver transplantation. Arterial complications arose in 21%; portal venous complications arose in 22%; and overall, 40% developed vascular complications. Late hepatic artery complications occurred more often in living donor recipients transplanted for CCA compared with the living donor control group (P=0.047). Late portal vein complications occurred more often in both whole organ and living donor recipients transplanted for CCA compared with the control groups (P=0.01 and P=0.009). Hepatic venous complications were rare. Patient and graft survival were not different between CCA and control patients. Liver transplantation with neoadjuvant therapy is associated with far higher rates of late arterial and portal venous complications, but these complications do not adversely affect patient and graft survival.