Diffuse biliary tract injury after orthotopic liver transplantation.

An unusual type of diffuse biliary tract injury after liver transplantation that is characterized by multiple intrahepatic biliary strictures, ductal dilatations, fluid collections, or intrahepatic abscesses has been identified. Over a 5-year period, a total of 10 patients (2%) developed diffuse intrahepatic biliary injury with established vascular patency and no obvious source for their biliary tract pathology. All patients received livers preserved in University of Wisconsin solution with a mean preservation time of 16 hours. This biliary tract injury was associated with the presence of severe preservation injury and Roux limb biliary reconstruction. Of the 10 patients, 5 were treated nonoperatively with multiple stricture dilations and stent placements, 3 underwent retransplantation, 1 was treated operatively with hepaticojejunostomy, and 1 died of sepsis. This study suggests that this complication appears to be related to preservation injury and that the etiology may be ischemic in origin.

The results of reduced-size liver transplantation, including split livers, in patients with end-stage liver disease.

We initiated a policy of using RSLT in critically ill patients in June of 1988. Since that time we have performed 30 RSLTs in 29 patients, including 28 children and 1 adult. The mean age of the children was 27 months (range 1 month to 10 years) with 14 (52%) being 1 year of age or less. The mean weight was 11.3 kg (range 2-50 kg) with 20 being 10 kg or less. A total of 22 patients were in the intensive care unit at the time of RSLT including 9 who were intubated. Of the 30 RSLTs, 23 were performed as a primary transplant while 7 were retransplants. Indications for primary transplantation included biliary atresia (n = 11), fulminant hepatic failure (n = 5), neonatal hepatitis (n = 4) and others (n = 3). The RSLT was used in retransplantation for primary nonfunction (n = 2), hepatic artery thrombosis (n = 2), chronic rejection (n = 2), and herpetic hepatitis (n = 1). The size reductions included 18 left lobes, 7 left lateral segments, and 5 right lobes. This group includes the use of the split-liver technique, which was applied to 10 patients (5 livers). The median donor/recipient weight ratio for left lobe transplants was 2:1; left lateral segments was 7.3:1; and right lobes 1.6:1. One year actuarial patient and graft survivals were 68 and 65%, respectively, with a mean follow-up of 10.6 months. The number of children dying awaiting transplantation has been significantly reduced following the introduction of RSLD (3 of 115, 2.6% vs. 12 of 95, 13%; P less than 0.02).

A selective approach to preexisting portal vein thrombosis in patients undergoing liver transplantation.

Splanchnic venous inflow is considered mandatory to ensure graft survival after liver transplantation. Over a 68-month period, we performed 570 liver transplants in 495 patients. Portal vein thrombosis was present in 16 patients. At transplant, the extent of the occlusion included portal vein alone (n = 4), portal including confluence of the splenic and superior mesenteric veins (n = 8), portal, splenic, and distal superior mesenteric veins (n = 2), and the entire portal vein, splenic vein, and superior mesenteric vein (n = 2). The operative approach included thrombectomy alone (n = 5), anastomosis at the confluence of the splenic and superior mesenteric splenic veins (n = 8), and extra-anatomic venous reconstruction (n = 3). The mean operative blood loss was 22 +/- 22 units, and the mean operative time was 9.7 +/- 4.8 hours. The 1-year actuarial survival rate was 81%, with a mean follow-up of 12.5 months. In summary, with a selective approach and the use of innovative forms of splanchnic venous inflow, portal vein thrombosis is no longer a contraindication to liver transplantation.

Syndrome of multiple bowel perforations in liver transplant recipients.

In an analysis of surgical complications following 500 consecutive orthotopic liver transplants, we identified 12 patients who developed the syndrome of multiple bowel perforations that was not due to iatrogenic injury. All cases occurred in small children (mean weight: 9.0 kg), who represented 7% of the pediatric population. Each patient had a minimum of three perforations. The typical intraoperative findings were pin-point perforations in areas of normal-appearing bowel. With only one possible exception (a patient with cytomegalovirus enteritis), no specific etiology could be determined. Management was based on multiple exploratory laparotomies and individualized operative procedures. All patients are currently alive (mean follow-up: 34.9 months). The pathogenesis of the syndrome of multiple bowel perforations remains unclear but is possibly multifactorial or related to high doses of steroids. Aggressive surgical management with semiopen treatment of peritonitis and frequent explorations has afforded excellent results.

Total gastric transposition: an alternative to esophageal replacement in children.

Total gastric transposition was performed in 21 children as an alternative procedure for esophageal replacement. The age at the operation ranged from 15 months to 11 years. Half of the children were less than 2 years old. Fifteen patients had esophageal atresia. The stomach was passed toward the neck either through the esophageal bed (6 cases, with concomitant blunt esophagectomy without thoracotomy) or the retrosternal route (15 cases). There was one death in the early postoperative period secondary to an anastomotic leak and acute mediastinitis in a case of pharyngogastric anastomosis. Three other patients developed cervical leak with spontaneous closure but this ultimately led to a late anastomotic stricture (more than 6 months) requiring endoscopic dilatation. Only one child needed more than three attempts of endoscopic dilatation. None of these patients required surgical revision. The mean follow-up was 60 months (range, 10 to 122 months). Despite bulky atonic intrathoracic stomach occurring in some children, only two patients developed regurgitation and symptoms of poor gastric emptying. There were neither early nor late respiratory problems. Excellent and good functional outcome were achieved in 85% and 15% of the patients, respectively. Two patients have not undergone a weight catch-up phase. The majority of the children have been between the 20th and 80th percentile for weight. Five children remain below the 20th and two below the 5th percentile. The remarkably low morbidity and mortality combined with satisfactory functional results indicate that the total gastric transposition is a safe and easy alternative surgical procedure for esophageal replacement in children.

Hepatic allograft rescue following arterial thrombosis. Role of urgent revascularization.

Hepatic artery thrombosis is a continuing source of morbidity and mortality following orthotopic liver transplantation. The cornerstone of therapy has been urgent retransplantation that is limited by organ availability. For this reason we developed a policy of urgent revascularization for allograft rescue. Hepatic artery thrombosis developed following 15 transplants of which 11 underwent urgent rearterialization. The diagnosis was made a mean of 4.8 days (range 1-10) following transplantation. Duplex ultrasonography was diagnostic in all patients and confirmed by angiography in 4 (36%). Three patients with hepatic artery thrombosis were identified following screening ultrasonography and were clinically unsuspected. Upon reexploration, a specific technical reason for hepatic artery was found in 4 patients (36%). Twelve arterial revascularization procedures were performed in 11 patients including: thrombectomy alone (n = 4); revision of anastomosis with thrombectomy (n = 5); and thrombectomy with placement of vascular conduit (n = 3). Following revascularization, 8 patients maintained hepatic artery patency. Three patients eventually required retransplantation secondary to biliary sepsis. Biliary tract complications developed in 6 patients, at a mean of 23 days following revascularization and included: breakdown of the biliary anastomosis (n = 4); stricture (n = 1); and sludge formation (n = 1). The overall graft and patient survival are 74% and 82% respectively, with a mean follow-up of 6.8 months. Hepatic allograft rescue with the use of urgent revascularization following hepatic artery thrombosis appears to be an effective means of either avoiding retransplantation or providing a bridge until a suitable donor becomes available.

Vascular complications after orthotopic liver transplantation.

Over a 57-month period, we performed 430 orthotopic liver transplants in 372 patients. A total of 38 vascular complications were identified including hepatic artery thrombosis (n = 24), portal vein thrombosis (n = 6), combined hepatic artery thrombosis/portal vein thrombosis (n = 3), and hepatic artery rupture (n = 5). A number of potential risk factors for the development of vascular thrombosis were evaluated with only children, weight less than 10 kg, and cold ischemia time found to be significant. The clinical presentation included fulminant hepatic failure, allograft dysfunction, biliary sepsis, and screening ultrasound. Duplex ultrasonography was diagnostic in nearly all cases. Therapeutic modalities included revascularization, revascularization followed by retransplantation, retransplantation alone, and observation. Five cases of hepatic artery rupture occurred in four patients. Infectious arteritis was present in four patients. The 6-month actuarial survival in patients with vascular complications was 70%. Early diagnosis is critical for graft salvage, with surgical intervention the mainstay of therapy.

The impact of extended preservation on clinical liver transplantation.

The introduction of UW solution into clinical transplantation has permitted extended cold storage preservation of the liver. Over a 46-month period, we have performed 308 orthotopic liver transplants (266 primary, 42 retransplants) in 266 recipients. Our experience is divided into cold-storage preservation in Eurocollins (163 transplants in 140 recipients) and UW (145 transplants in 131 recipients) solutions. Donor and recipient factors were comparable between the two groups. The use of UW solution has permitted an increase in the mean preservation time from 5.2 +/- 1.0 [EC] to 12.8 +/- 4.3 [UW] hr (P less than 0.001). The mean total operating time was reduced but intraoperative blood loss was unchanged with UW preservation. The number of transplants performed during the daytime hours has increased dramatically (21.5% [EC] vs. 71% [UW], P less than 0.001). The incidence of primary nonfunction, hepatic artery thrombosis, 1-month graft survival, and early retransplantation were similar in the 2 groups. Initial allograft function as determined by bile production, histology, and clinical assessment were likewise similar. Mean serum bilirubin, transaminase, and prothrombin levels were virtually identical by 5 days posttransplant. The enhanced margin of safety afforded by extended preservation has increased the capability for distant organ procurement and sharing, minimized organ wastage, and improved the efficiency of organ retrieval. With the relaxation of logistical constraints, our rate of liver import has nearly doubled (20.9% [EC] vs. 39.3% [UW], P less than 0.001). Extended preservation has permitted the development of reduced-size liver grafting (n = 12), resulting in a significant reduction in the number of deaths occurring while awaiting transplantation. Therefore, we advocate the use of UW solution with selective extension of preservation based not only on donor and recipient factors but also on manpower, resource, and logistical considerations.

Primary sclerosing cholangitis: the emerging role for liver transplantation.

Primary sclerosing cholangitis is a progressive liver disease for which orthotopic liver transplantation is the only curative procedure. Questions exist regarding the role of temporizing procedures and the timing of transplantation. During the past 4 yr, we have performed liver transplants in 177 adult recipients. Twenty-six patients (14.6%) with primary sclerosing cholangitis received 30 transplants including 12 men and 14 women. The recipients were examined for a number of preoperative and postoperative variables. The 4-yr actuarial survival in patients with primary sclerosing cholangitis after transplantation was 88%. Patients were segregated according to preoperative risk variables. Twenty patients were low and medium risk, with one death (95% survival). Three patients were high risk, with two deaths (33% survival). In conclusion, orthotopic liver transplantation is safe and effective therapy for primary sclerosing cholangitis. Early referral for transplantation is recommended to reduce the mortality associated with this procedure in those with advanced hepatic failure.

Adenocarcinoma of the gallbladder in two siblings.

The authors present two siblings with adenocarcinoma of the gallbladder. This is the third report on familial occurrence of such disease. The cancer was diagnosed at age earlier than usual. One of the patients did not have gallstones. The literature is reviewed. Remarks are made about the relationship between heredity and cancer.