Seventy-six patients with biliary atresia (BA) were seen at the Kobe Children's Hospital during the period from 1971 to 1989. We reviewed the medical records of these patients to clarify the timing of orthotopic liver transplantation (OLT) and to elucidate the role of re-do portoenterostomy. Seventy-six patients were divided into three groups according to their postoperative courses; Group I (n=35): good bile flow and serum bilirubin≦1.0 mg/dl within 2 yrs after operation. There were 3 deaths. The cause of death was ruptured esophageal varices. Another three developed intrapulmonary shunt. A few patients even without jaundice in this category, cirrhosis gradually progressed, ultimately requiring OLT. Portal vein thrombosis and intrapulmonary shunt should be carefully followed. Group II (n=27): fair bile flow but serum bilirubin>1.0mg/dl 2 yrs after operation. There were 19 deaths. Growth retardation, ascites accumulation and variceal bleeding sequentially developed in these patients when serum bilirubin exceeded 10 mg/dl and chE became low (150-200 IU/l). This seems to be the proper timing of OLT because nothing is gained from awaiting OLT at this point. Group III (n=14): no bile flow. All died except for one who had OLT. Nutritional managent is mandatory in this category for a successful OLT. Re-do Kasai operation was done in 22 patients and 11 of these are now alive including one who survived OLT. Re-do portoenterostomy for failed Kasai is encouraging because it can achieve biliary drainage good enough in about half of the patients to survive long-term in a satisfactory quality of life. OLT can be deferred, allowing the patients to grow in the meantime.
Liver transplantation in children poses a variety of surgical problems which are distinct from those in adults. The topic of this presentation will be a review of the patterns of complications observed after liver transplantation in children. Although early reports of liver transplantation suggest that the great majority of candidates were older children, the epidemiology of liver disease and the experience of centers in the United States suggest that most patients who require liver transplantation do so before their second year. Therefore, the primary problem of pediatric liver tranpslantation is addressing the needs of these very small children. Common complications which are observerd in pediatric liver transplantation include hepatic artery thrombosis, which occurs between 5 and 25 percent of children in large series. Surgical management of this includes early diagnosis, thrombectomy and vascular reconstruction or liver replacement. While acute hepatic necrosis occasionally occurs, the most common presentation is due to fever and jaundice, biliary tract sepsis, common bile duct necrosis, or hepatic abscess. These lesions tend to be recognized late and are best managed with liver replacement. Primary non-function of a liver graft occurs in between 5 and 10 percent of transplants. It may be more common in small pediatric donors, since the donor selection criteria are often less rigorous because of the scarcity of small donors. While hypofunction can occasionally be managed expectantly with support of the prothrombin time, early retransplantation is usually the safest measure. Segmental liver transplants occasionally cause complications due to biliary leakage or necrosis along the cut section which requires reoperative therapy and drainage. Portal vein thrombosis is occasionally seen after liver transplantation in children. It never is a cause of graft failure and is usually managed readily by thrombectomy and release of tension on the portal vein. This occasionally requires interposition grafting. Biliary leaks occur after liver transplantation in children and are nearly always associated with hepatic antery thrombosis. If the hepatic artery is patent, revision of the hepatico jejunostomy over a stent affoeds a very high success rate. Ptients with biliary atresia may have extensive visceral adhensions and due to the extensive dissection required, have a 5 to 10 percent risk of intesitnal perforation. A readiness to re-explore children with fever and abdominal distension between seven and fourteen days after transplantation can prevent the severe complication of uncontrolled peritonitis. The prophylactic use of oral antibiotics to prevent abdominal infections has reduced the morbidity of these problems. Overall, surgical complications account for the majority of graft and patient loss in pediatric liver transplantation. Attention to detail and the use of good livers can reduce the incidence of these complications.
Several therapeutic modalities have been introduced to deal with the graft shortage in pediatric liver transplantation. We have performed thrity living related liver transplantation (LRLT) procedures over the past 19 months in 30 patients who were 8 months to 15 years of age; 23 had biliary atresia, 2 liver cirrhosis, 2 Budd-Chiari syndrome, 1 Protoporphyria and 2 progressive intrahepatic cholestasis. Sixteen lateral segmentectomies, 13 left lobectomies and one right lobectomy were performed for graft harvesting. A partial orthotopic live transplant with an intact inferior vena cava was performed. Our standard immunosuppression consists of FK 506 and low dose steroids. Postoperative management has been done on Redox theory. Arterial ketone body ratio (AKBR) was serially measured every 12 hours over the first postopertaive week and once a day later as an indicator for evaluating the viabilitiy of graft liver until the graft survival was obtained. Twenty four recipients are alive and well with the original graft and normal live function. Patient survival rate was 88% (22/25) in elective cases. 6 patients died with functioning graft due to the extrahepatic complications; one aspiration asphyxia, two cardiac insufficiency, one candida infection and one lymphoproliferative disorder. Graft complications were encountered in 5 recipients. Two patients had biliary stenosis, one portal vein thrombosis, one arterial thrombosis and two hepatic vein stenosis. Three rejection episodes were obsebed in 7 ABO compatible cases and one episode in 3 incompatible cases. In all 30 cases, AKBR increased above 1.0 within 2 days after transplantation. However, the increased AKBR above 1.0 decreased again in 5 cases. The causes of the AKBR redecrease were determined to be hepatic artery thrombosis, portal vein thrombosis, massive intraabdominal hematoma, hepatic stenosis and lymphoproliferative infiltration into the liver, respectively. The decreased AKBR recovered promptly following successful surgical interventions in 4 cases. All donors have perfectly normal hepatic function and came back to the normal social lives. Our results suggested that LRLT is a very important option toward resolving the graft shortage in pediatric liver transplantation and post operative management based on the Redox theory is crucial for outcome.
Blalock-Taussig シャント(以下 BT-S)術後,遠隔における肺動脈の発育について,手術時月齢との関連から検討した. 対象は1985年1月より1990年12月迄の6年間に行われた BT-S 手術症例中,先行する短絡手術の既往がなく,かつ吻合部の狭窄所見が認められなかった症例27例とした. 手術時及び遠隔期平均月齢は各々9.9(0.2-37)月,34.2(12-70)月であった. 対象疾患は,TOF 11,TGA 3,DORV 3,TA 3,CAVC with PS 3,PA with IVS 4例であり,手術はclassical BT-S 20例,modified BT-S(PTFE)7例であった. 術前及び遠隔期の肺動脈の評価は心血管造影にて行い,Index として PA index(以下PAI)を用い,術後PAI/術前PAI を PAI ratio として手術時月齢と比較検討した. その結果,手術時月齢と PAI ratio との間には有意な相関(r=0.57,p<0.0l)がみられ,特に2ヵ月,12ヵ月の時点で大きな変化が認められた. そこで,手術時月齢により12ヵ月未満群(12>群, n=16),12ヵ月以上群(12≦群, n=ll)とに分け,更に前者を2ヵ月未満群(2>群,n= 9), 2ヵ月以上群(2≦群,n=7)とに分け比較検討した. PAI ratio は,12>群で12≦群に比べ有意に高く(12≦群:121±28%,12>群:219±95,p<0.005),更に,2>群では2≦群に比して有意に高値であった(2>群:264±104%,2≦群:158±22,p<0.025). 肺動脈の発育は,シャント術を行う時期により大きく左右され,生後2ヵ月と12ヵ月で有意な変化がみられた. 肺動脈の良好な発育を期する為には,乳児期早期に行うのが適当と考えられた.