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Variceal bleeding remains a common cause of morbidity for children with both intrahepatic and extrahepatic portal hypertension. Occasionally, patients referred for liver transplant evaluation have significant variceal bleeding, despite adequate synthetic liver function. During a 7-year period, 322 children were referred for liver transplant evaluation. Six underwent distal splenorenal shunt surgery after evaluation. There were four boys and two girls. The average age was 11 +/- 4 years, and the average weight was 39 +/- 15 kg. The etiology of variceal bleeding was intrahepatic portal hypertension in five (1 biliary atresia, 2 chronic hepatitis, 2 congenital hepatic fibrosis) and extrahepatic portal vein thrombosis in one. Two patients had no previous attempts at sclerotherapy (one because of an abnormality in platelet function, the other because of extensive gastric varices), and four had multiple previous sclerotherapy treatments. No patient had preoperative encephalopathy. Three cases were Child's class A, and three were Child's class B. Preoperative evaluation of the portasystemic system was performed with magnetic resonance (MR) imaging or splenoportography. All patients underwent a distal splenorenal shunt procedure, four of whom also had splenopancreatic disconnection. One patient required 100 mL of blood replacement, and five required no blood. The average length of hospital stay was 9.8 +/- 2.2 days. Postoperative complications were minimal. All patients are alive, without recurrent gastrointestinal bleeding or encephalopathy, and they have patent shunts, which was confirmed by MR or Doppler ultrasound at a mean of 25 +/- 20 months after shunt surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

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Orthotopic liver transplantation has become the accepted treatment for end-stage liver disease in children. To evaluate the efficacy of the University of Wisconsin (UW) preservation solution in pediatric liver transplantation, a group of 34 livers preserved with UW solution was compared in a nonrandomized fashion with a historical control group of 34 livers preserved with Euro Collins (EC) solution. Primary graft nonfunction did not occur in either group. Both groups were similar with respect to age, sex, weight, diagnosis, severity of the recipient's condition, donor condition at harvest, donor/recipient blood type match, and immunosuppressive management. The UW group had a significantly higher bilirubin, AST, ALT, and GGT during the first week after transplantation when compared with the EC group but no significant differences were noted after the ninth post-transplant day. No differences were noted when the groups were compared as to surgery time (9.1 v 8.4 hours), blood volumes replaced (1.8 v 2.0), number of ICU days (5.0 v 6.5), total number of infections per graft (1.0 v 0.8), total hospital days (31 v 30), and hospital cost ($134,000 v $126,000). The total preservation time was improved from 7 hours (range, 3.2 to 9.9) in the EC group to 13.9 hours (range, 6.9 to 22.3) in the UW group (P < .001). UW solution allows a significant increase in cold ischemic time in liver transplantation when compared with EC. This increase in preservation time resulted in no detrimental effect when compared with EC and potentially led to milder episodes of rejection in the postoperative period.

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Primary lymphoma of the rectum is rare. Its usual grouping with primary lymphoma of the gastrointestinal tract leaves little in the literature that deals specifically with a primary rectal lymphoma. Various treatments have been reported ranging from abdominoperineal resection with adjuvant chemotherapy and radiation therapy to chemotherapy or radiation therapy alone. A case of primary rectal lymphoma is presented and the literature is reviewed. In the few cases reported in the literature, patients treated surgically had a better prognosis than those treated nonsurgically with an overall 5-year survival of 50 per cent. Recommended treatment includes varying forms of surgical therapy ranging from local resection to abdominoperineal resection. Adjuvant chemotherapy, radiation therapy, or both should be considered.

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Survival in ABO-incompatible (ABO-I) liver transplantation has been reported to be between 40% and 60%. Management techniques have included routine immunosuppression as well as prophylactic antilymphoblast globulin, pre- and posttransplant plasmapheresis (PLPH), and splenectomy. Over a 6-year period, 155 orthotopic liver transplants were performed in 139 pediatric patients. Seven children received an ABO-I allograft. In the latter transplants, immunosuppression consisted of triple-drug therapy (cyclosporine A, prednisone, and azathioprine) along with prospective double-volume PLPH for ABO titers (IgA and IgM) greater than or equal to 1:8. Splenectomy was not performed on any patient. One patient was refractory to PLPH and was treated with a hemofiltration system using an immunoadsorption cartridge with synthetic A group antigen. The overall survival for patients receiving ABO-I allografts was 57% (4/7), with a 67% (4/6) graft survival in those patients treated with PLPH. The graft survival for patients treated with prospective PLPH and MALG was 60% (3/5). There was a 60% incidence of rejection in those patients treated with prospective PLPH and these episodes were all mild (steroid bolus only). While ABO-I transplantation is a reasonable option in the emergency setting, further study is necessary before it should be routinely used to increase the general donor organ pool in pediatric liver transplantation.

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A review of 39 instances of excision of a cyst of the thyroglossal duct performed at St. Paul Medical Center, Dallas, Texas, revealed two patients with carcinoma of the thyroglossal duct. A review of the English literature yielded 146 instances of this uncommon tumor. Eighty-four per cent were papillary adenocarcinoma of thyroid type and 5 per cent were squamous cell carcinoma. The patients were from six to 81 years old with a 2:1 female to male ratio. Metastatic disease to lymph nodes was noted in 11 per cent and invasion of overlying strap muscles was found in 4 per cent. Carcinoma of the thyroid gland occurred in 14 per cent. Preoperative diagnosis was rare. The Sistrunk procedure is recommended for initial surgical therapy with further surgical or adjuvant therapy dependent on associated clinical findings. Prognosis for carcinoma of the thyroglossal duct of thyroid type parallels that of carcinoma of the thyroid gland.

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