Pediatric lung transplantation. Indications, techniques, and early results

From July 1990 to April 1993, 36 lung transplantations in 33 patients were performed in our pediatric transplant program (0.25 to 23 years, mean age 10.3 years). Eight children had been continuously supported with a ventilator for 3 days to 4.5 years before transplantation and three were supported by extracorporeal membrane oxygenation. Indications for lung transplantation in this pediatric population included the following: cystic fibrosis (n = 13), pulmonary hypertension, and associated congenital heart disease (n = 10), pulmonary atresia, ventricular septal defect and nonconfluent pulmonary arteries (n = 3), pulmonary fibrosis (n = 6), and acute respiratory distress syndrome (n = 1). Three children underwent retransplantation for acute graft failure (n = 2) or chronic rejection (n = 1). Pulmonary fibrosis was related to complications of treatment of acute of myelogenous leukemia with bone marrow transplantation in two children and to bronchiolitis obliterans, bronchopulmonary dysplasia, interstitial pneumonitis, and Langerhans cell histiocytosis in four others. Thirteen children underwent lung transplantation and concomitant cardiac repair. Bilateral lung transplantation, ventricular septal defect closure and pulmonary homograft reconstruction of the right ventricular outflow tract to the transplanted lungs was performed in three children by means of a new technique that avoids the need for combined heart-lung transplantation. Two patients had ventricular septal defect closure and single lung transplant for Eisenmenger's syndrome, two had ligation of a patent ductus arteriosus and transplantation, three additional children underwent atrial septal defect closure and lung transplantation, and two underwent lung transplantation for congenital pulmonary vein stenosis. Eight early deaths and three late deaths occurred (actuarial 1-year survival 62%). Lung transplantation in children has been associated with acceptable early results, although modification of the adult implantation technique has been necessary. Lung transplantation and repair of complex congenital heart defects is possible; heart-lung transplantation may only be required for patients with severe left heart dysfunction and associated pulmonary vascular disease. Bronchiolitis obliterans remains a major concern for long-term graft function in pediatric lung transplant recipients.     

Indications for conjunctival transplantation

Long-term follow-up (two to five years) of 12 unilateral chemical burn patients treated by conjunctival transplantation shows permanent stabilization of the ocular surface. The procedure was used in another group of five patients with unilateral recalcitrant epithelial defects. Regardless of the etiology of the epithelial defect, prompt healing of the surface occurs after conjunctival transplantation with no further stromal loss and long-term stabilization of the surface. Such results suggest that epithelial replacement may be a valuable therapeutic approach to a variety of ocular surface disorders.     

Indications for heart transplantation

Heart transplantation may have a considerable impact on the fate of the patient in the end stage of heart failure. A successful procedure requires the existence of a sophisticated programme combining the expertise of top departments of cardiology and cardiac surgery working in conjunction with the transplant centre directly responsible for removing the organ. One of the factors playing an important role in a favourable outcome is the correct indication for the procedure. The information on patient selection and criteria for the heart donor, presented in this paper, become increasingly important even for the practitioner in cardiology referring their patients to specialized departments. The paper also offers background information on the heart transplant programme in the Czech Republic suggesting the need for expanding the availability of the therapeutic method in this country.     

Indications and results of lumbar sympathectomy

According to our experience the lumbar sympathectomy is not indicated for the treatment in clinical stage II. The best results for lumbar sympathectomy are to be expected in clinical stage III. We consider the protective sympathectomy combined with reconstructive arterial surgery indicate only in individual cases.     

Current results and evolving indications for liver transplantation in children

Liver transplantation continues to be successful and effective treatment for acute and chronic liver failure, and many important lessons have been learned. The development of innovative operative techniques has much reduced the waiting list mortality rate and has extended transplantation to younger and sicker children and to those with functionally normal livers who may benefit from auxiliary liver transplantation. The incidence and range of postoperative complications have improved with increased medical and surgical expertise. As information on long-term outcome for liver transplantation is gained, it is clear that many children will benefit from early elective liver transplantation before the development of significant growth or psychosocial retardation. Early transplantation is also indicated in children with cirrhosis and intrapulmonary shunting or cystic fibrosis with moderate lung disease. During the same period, evolving medical therapy has altered the natural history, patient selection, and timing of transplantation in children with tyrosinaemia type I, primary bile acid disorders, neonatal haemochromatosis, and potentially, cystic fibrosis. It is now clear that children with significant multisystem disease, such as mitochondrial disorders or severe systemic oxalosis, are no longer suitable candidates for liver transplantation. The successful development of liver transplantation has brought good quality life to many children and their families. There are still many lessons to learn and there are future challenges such as the ever-increasing problems of donor scarcity and the search for potent but less toxic immunosuppressive agents.     

Surgical hepatology: liver transplantation and extensive resections]

The study revealed the existence of differences between various representatives of the genus Neisseria gonorrhoeae, as well as intraspecific variations of N.gonorrhoeae by the spectrum of biological characteristics, including antilusozyme and anticomplement activity and resistance to the bactericidal action of blood serum and to the preparation of human leukocyte interferon. In contrast to other Neisseria species, all N.gonorrhoeae were characterized by a high level of anticomplement activity and resistance to the host's bactericidal systems, while a high level of antilysozyme activity, significantly exceeding that of gonococci, was found to be characteristics of other species of the genus Neisseriaceae. Within the species N.gonorrhoeae, the properties under study were associated into a single factor, closely correlated to the duration of the infectious process, which served as the basis for the development of a mathematical algorithm for the identification of persisting gonococcal strains.     

Long-term results after liver transplantation for primary hepatic epithelioid hemangioendothelioma

BACKGROUND: Hepatic epithelioid hemangioendothelioma (PHEHE) is a multifocal, low-grade malignant neoplasia characterized by its epithelial-like appearance and vascular endothelial histogenesis. The outcome of 16 patients treated with orthotopic liver transplantation (OLT) is the subject of this report. METHODS: A retrospective study of 16 patients with HEHE (7 men, 9 women) with ages ranging from 24 to 58 years (mean 37 +/- 10.6 years). Follow-up intervals ranged from 1 to 15 years (median of 4.5 years). RESULTS: Actual patient survival at 1, 3, and 5 years was 100, 87.5, and 71.3%, respectively. Disease-free survival at 1, 3, and 5 years was 81.3, 68.8, and 60.2%, respectively. The 90-day operative mortality was 0. Involvement of the hilar lymph nodes or vascular invasion did not affect survival. The 5-year survival of HEHE compares favorably with that of hepatocellular carcinoma at the same stage (stage 4A): 71.3 versus 9.8% (p = 0.001) CONCLUSIONS: The long-term survival obtained in this series justifies OLT for these tumors even in the presence of limited extrahepatic disease.     

Improved results of liver transplantation in patients with portal vein thrombosis

OBJECTIVE: To analyze the impact of preexisting portal vein thrombosis (PVT) on the operative management and outcome of liver transplantation. DESIGN: Retrospective review of 1423 patients who received transplants over 11 years. SETTING: Tertiary referral center. PATIENTS OR OTHER PARTICIPANTS: Seventy patients who underwent liver transplantation who had preexisting PVT. INTERVENTIONS: Portal vein thromboendovenectomy, vein grafting, or use of portal collateral veins for inflow during liver transplantation. MAIN OUTCOME MEASURES: Postoperative PVT, intraoperative transfusion, retransplantation rate, 30-day and 1-year actuarial survival rates. RESULTS: Operative management consisted of thromboendovenectomy in 61 cases, vein graft to the superior mesenteric vein in 6 cases, and vein graft to other mesenteric veins in 3 cases. The incidence of posttransplant PVT was 3% (n = 2). The mean +/- SD transfusion requirement was 23 +/- 18 U. The 1-year actuarial survival rate was 74% but improved from 66% in the first 35 cases to 82% in the latter 35 cases. CONCLUSIONS: Thromboendovenectomy is the procedure of choice for PVT. Results of liver transplantation in patients with PVT improve significantly with experience gained and are equivalent to results in patients without PVT.