B. Koneru

Liver Transplantation for Tyrosinemia: A Review of 10 Cases from the University of Pittsburgh

SummaryResults of liver transplantation in 10 patients with tyrosinemia are reviewed. The indications for transplantation were: hepatoma in three, acute liver failure in two, and progressive chronic liver disease in five. One patient died during surgery. Of the remaining nine who survived the operation, one died at six months as a result of bronchial aspiration and aspiration pneumonia, and a second transplanted for hepatoma died five months later with metastases. Seven patients are alive 6 months to 6 1/2 years following transplantation. Of these seven patients, six have normal liver function and a good performance status. One is awaiting retransplantation for chronic rejection. Hepatocellular carcinoma (HCC) was found either preoperatively or incidentally in five patients, all older than 2 years at the time of their transplant. Four of these are alive and well without evidence of tumor with follow-ups between 3 1/2 and 6 1/2. Four of the five patients less than 2 years of age had hepatocellular dysplasia without evidence of carcinoma on histologic examination of the resected liver. This experience suggests that liver transplantation should be considered seriously for children with hereditary tyrosinemia who are more than 2 years of age because beyond that age the incidence of hepatocellular carcinoma (HCC) increases substantially.

TRANSMISSION OF FATAL HERPES SIMPLEX INFECTION THROUGH RENAL TRANSPLANTATION

Herpes simplex virus (HSV)* infections cause significant morbidity and occasional mortality following organ transplantation. The majority of these infections are believed to be reactivation of a latent and remote infection in the recipient (1–3). Rare cases of primary herpes simplex infection following renal transplantation have been reported but the source of infection in these is unknown (4, 5). Donor kidneys have been shown to be a source of cytomegalovirus infections (2) but evidence has been minimal regarding their role in HSV infections (6,7). We recently documented one instance of transmission of primary HSV-2 infection by renal transplantation but thought this was a rare occurrence (7), but 21 months after that we had a tragic experience in which two renal recipients died of disseminated herpes infection with fulminant hepatitis after receiving organs from the same donor. Liver and heart from that donor were also transplanted into different recipients. It is our intent in this report to present evidence that donor kidneys in this case also acted as a source of the herpes simplex infection.

Biliary Tract Complications in Orthotopic Adult Liver Transplantation

In a series of orthotopic liver transplantations performed between April and August 1987 at the University of Pittsburgh, the monofilament absorbable suture polyglyconate was compared with a braided absorbable suture, polyglactin 910, for its biliary complication rate over a 6-month postoperative period. Complications that were suture-related (obstruction or leak from the anastomotic site) occurred in 1 of 21 transplantations in the polyglyconate group compared with 8 of 26 in the polyglactin 910 group (p = 0.02). Even though the patient sample was relatively small, it appears that the type of suture used for the biliary anastomosis directly correlates with the outcome. A larger patient trial could confirm these initial results.

The use of pulmonary artery sequestration as an hepatic arterial conduit. A case of unusual hepatic arterial supply.

J. P. is a 20-month-old Portuguese male patient who presented with a diagnosis of biliary atresia. Approximately 10 months previously the patient had undergone a laparotomy and cholecystectomy for unexplained jaundice. At the time of surgery the diagnosis of biliary atresia was established, and the patient was later transferred for liver transplantation evaluation. The physical examination showed a deeply jaundiced infant male patient in no apparent distress. Systolic blood pressure was 94, heart rate 102, respiratory rate 28, temperature 35.9°C, and the weight was 8.9 kg. The sclera were icteric, neck supple, lung fields were clear bilaterally, heart was regular and without murmurs. Abdominal girth was 52 cm with obvious superficial venous collaterals. The liver and spleen were enlarged and easily palpable. Laboratory studies included: Hct 32%, WBC 9.0, Plt 249K, total bilirubin 16.2, GGTP 194, SGOT 268, SGPT 93, and AP 856. Blood urea nitrogen, creatinine, amylase, and albumin were all within normal limits. Ultrasonography revealed a cirrhotic liver with patent hepatic vessels and no bile-duct dilatation. The patient was transplanted on 7/15/87 without complications. The arterial anastomosis was performed between the donor abdominal aorta and the celiac axis of the recipient (after ligation of the donor left gastric and splenic arteries). On the 1st postoperative day the patient had an ultrasound that showed no pulsation in the hepatic artery. Arterial thrombosis was confirmed by angiography. The patient was retransplanted the following day (7/17/87) with a liver that was slightly smaller than ideal. During the harvesting of the liver, it was noted that the donor (a 12-month-old child) had significant pericardial and right pleural adhesions. During the final stages of harvesting the liver, a large artery was found entering the right lower lobe of the lung from the diaphragm, an obvious pulmonary sequestration. The artery was preserved and was later found to be emanating from the celiac axis. In addition, the liver had a triple arterial supply: an hepatic artery from the celiac, a left hepatic branch from the left gastric artery, and a right hepatic branch from the superior mesenteric artery. A modification of the fold-over technique (1) was done, but because of the size of the liver there was not adequate length to comfortably anastomose this complex to the recipient celiac axis. For this reason, the pulmonary artery originating from the donor celiac axis was used to make the anastomosis (Fig. 1). Figure 1 Donor anatomy (above) with final reconstruction (below). RHA: right hepatic artery; LHA: left hepatic artery; and SMA: superior mesenteric artery. The patient recovered from this 2nd transplantation uneventfully and was discharged from the hospital on 8/23/87 with normal liver function. The patency of his arterial complex was established 4 times postoperatively by ultrasound. On his last out-patient visit, he was doing well with a bilirubin of 0.4, PT 11.7 sec, SGOT 19, SGPT 10, and a GTP 16. The celiac axis as the origin of a pulmonary arterial sequestration is present 1% of the time (2). As symptoms of pulmonary sequestration almost always present early in life with complications leading to corrective surgery, this anomaly as a harvesting enigma will probably be found only in children. As was shown here arterial anomalies do not preclude the use of the hepatic graft and can often be used advantageously. Donor organs should not be discarded secondary to vascular anomalies. A thorough understanding of reconstructing the hepatic arterial supply can be both organ- and life-saving.