Liver transplantation in children weighing less than 10 kilograms

Orthotopic liver transplantation (OLT) remains a major medical and surgical challenge in small pediatric patients. From April 2003 through October 2005, 17 infants (each of whom weighed less than 10 kg) underwent the procedure. Four were girls and 13 were boys (mean age, 15.7 +/- 9.3 months [range, 2-36 months]; mean weight at the time of transplantation, 7.4 +/- 2.6 kg [range, 6-10 kg]). All transplants were obtained from living-related donors. Sixteen left lateral segments and 1 left lobe were transplanted. The median graft-to-recipient weight ratio was 3.5% +/- 1.2% (range, 1.5%-6.1%). During the early postoperative period, hepatic arterial thrombosis was identified in 2 infants, and a biliary leak in 1. Hepatic arterial thrombosis was treated by reanastomosis with polytetrafluoroethylene grafting in the first patient and by surgical embolectomy in the second. The biliary leak was treated with percutaneous drainage. In 1 infant, portal vein stenosis, which was identified during the late postoperative period, was treated by percutaneous balloon dilatation. At this time, 14 (82.3%) infants were alive, exhibiting good graft function at a median follow-up of 11 months (range, 2-36 months). Three infants died: 1 on postoperative day 47 from adult respiratory distress syndrome, 1 on postoperative day 12 from sepsis, and 1 on postoperative day 65 from sepsis associated with EBV infection. Episodes of acute rejection, which occurred in 5 patients, were treated with pulse steroid therapy. On follow-up, histologic examination revealed hepatocellular carcinoma in 2 infants and Burkitt's lymphoma in 1 infant. Our data confirm that extensive use of living-related donors in liver transplantation can result in an excellent outcome for small pediatric patients.     

Vascular reconstruction and complications in living donor liver transplantation in infants weighing less than 6 kilograms: the Kyoto experience.

Smaller-size infants undergoing living-donor liver transplantation (LDLT) are at increased risks of vascular complications because of their smaller vascular structures in addition to vascular pedicles of insufficient length for reconstruction. Out of 585 child patients transplanted between June 1990 and March 2005, 64 (10%) weighing less than 6 kg underwent 65 LDLTs. Median age and weight were 6.9 months (range: 1-16 months) and 5 kg (range: 2.8-5.9 kg), respectively. Forty-five lateral segment, 12 monosegment, and 8 reduced monosegment grafts were adopted, and median graft-to-recipient weight ratio was 4.4% (range: 2.3-9.7). Outflow obstruction occurred in only 1 patient (1.5%). Portal vein complication occurred in 9 (14%) including 5 with portal vein thrombosis. Hepatic artery thrombosis (HAT) occurred in 5 (7.7%). Patient and graft survivals were 73% and 72% at 1 yr, and 69% and 68% at 5 yr after LDLT, respectively. Thirteen of 22 grafts (58%) lost during the follow-up period occurred within the first 3 months posttransplantation. Overall graft survival in patients with and without portal vein complication was 67% and 65%, respectively (P = 0.54). Overall graft survival in patients with and without HAT was 40% and 67%, respectively. HAT significantly affected graft survival (P = 0.04). In conclusion, our surgical technique for smaller-size recipients resulted in an acceptable rate of vascular complications. Overcoming early posttransplantation complications will further improve outcomes in infantile LDLT.     

Results of kidney transplantation in children weighing less than 11 kilograms

Weigh and age are risk factors of graft failure. The aim of the study is to review the characteristics and the outcome of cadaver renal transplant in children weighing less than 11 Kg. From 1985 to 1999 10 cadaver renal transplant were performed in 10 children (7 boys and 3 girls). Primary renal disease were renal dysplasia(3), posterior urethral valves(5) and congenital nephrotic syndrome(2). All except two suffered end stage disease from birth. The cadaver donor age ranged from 4 to 45 years (mean 12.3). Cold ischaemia time was 14 to 30 hours (median 22.8 h). Grafts were placed extraperitoneally in the iliac fossa in all patients and special care was taken in aggressive intravascular volume expansion. In the first 5 children initial immunosuppression consisted of CyA, Pd and Aza. After 1991, the other five received sequential induction therapy with polyclonal antibodies and triple therapy (CyA, Pd, Aza). Renal function was evaluated as GFR yearly by Swartz formula and the actuarial and graft survival rates were obtained by Kaplan-Meier analysis. Patient survival was 90% at 1 and 10 years. Graft survival was 80% at 1 and 5 years; it decreased to 64% at 7 years. Seven continue with their first graft and the mean follow-up time is 6.6 years. Their renal function measured by the mean of GFR yearly decreased lightly from 102 ml/min/1.73 m2 at 1 year to 87.6 ml/min/1.73 m2 at 5 years. A successful patient and graft survival can be achieved in young receiving kidneys and small reciepients can improve their physical and mental development after transplantation.     

Surgical aspects of dialysis in newborns and infants weighing less than ten kilograms

PURPOSE: Renal failure occurs in children with moderate frequency. Surgical aspects of establishing and maintaining dialysis access in small infants are exceptionally challenging. The purpose of this review is to evaluate the authors' experience with dialysis access for infants less than 10 kg, particularly with respect to the surgical care required. METHODS: A retrospective review was conducted between 1991 and 1999 of all pediatric dialysis patients weighing 10 kg or less (n = 29). Age at start of dialysis, duration of dialysis, modes of dialysis, and complications specific to peritoneal (PD) and hemodialysis (HD) were examined. RESULTS: The mean age at start of dialysis was 10.4 months and continued for an average duration of 16.3 months. Seventy-two percent of all patients required both modes of dialysis. HD and PD duration averaged 7.8 and 10.5 months, respectively. Catheter durability was 3.1 and 4.5 months per catheter for HD and PD, respectively. There was no significant difference in complications when comparing HD and PD. Patients who weighed 5 to 10 kg had significantly longer PD catheter durability than patients 0 to 5 kg (P = .001). Forty-one percent of patients terminated dialysis after transplantation, whereas 24% died awaiting transplantation. CONCLUSION: Despite a large number of operations required, infants less than 10 kg can be bridged successfully, by surgical intervention and subsequent dialysis, to transplantation.     

Living donor liver transplantation with reduced monosegments for neonates and small infants

BACKGROUND: In pediatric living donor liver transplantation, left lateral segment or monosegmental graft is used to overcome size discrepancies between adult donors and pediatric recipients. For neonates and extremely small infants, however, problems related to large-for-size graft are sometimes encountered even when using such grafts. The reduced monosegmental graft, in which the caudal part of the monosegmental graft is resected, has been introduced to address this problem. METHODS: Of 566 children who underwent transplant between June 1990 and September 2004, reduced monosegment living donor liver transplants were used for nine patients (median age, 144 days; median weight, 4.1 kg). This technique was used for infants with estimated graft-to-recipient weight ratio (GRWR) > or =4.0% when using the left lateral segment. RESULTS: Graft and patient survival was 66.7%. GRWR was reduced from 7.45+/-2.70% to 3.39+/-0.89% using this modification. Transaminase levels at days 1 and 2 after transplantation were significantly higher in reduced monosegmental transplantation than in left lateral segmental transplantation. Hepatic artery thrombosis and portal vein thrombosis were observed in one case each. CONCLUSION: Reduced monosegmental living donor liver transplantation represents a feasible option for neonates and extremely small infants with liver failure.     

The effects of cardiopulmonary bypass on thyroid function in infants weighing less than five kilograms.

Triiodothyronine is an important regulator of cellular metabolism and may have potential use as an inotropic agent. The aim of this study was to determine the effects of cardiopulmonary bypass on thyroid function in infants weighing less than 5 kg. Serial measurements of triiodothyronine, thyroxine, and thyroid-stimulating hormone were made in 10 infants and corrected for the effects of hemodilution. We demonstrated a fall in triiodothyronine and thyroxine levels, with some recovery after 3 to 6 hours. An additional decrease then occurred, reaching a trough at 48 hours (representing a fall of 78% for triiodothyronine and 57% for thyroxine) before hormone levels returned to normal at 5 to 7 days. Thyroid-stimulating hormone concentrations increased and decreased, predating and complementing exactly the changes in triiodothyronine and thyroxine. These results are quantitatively and, for thyroid-stimulating hormone, qualitatively different from those previously reported in adults. In two patients who died, however, and in one who had a particularly difficult postoperative course, no increase in triiodothyronine, thyroxine, or thyroid-stimulating hormone concentrations was found after a trough had been reached at 48 to 72 hours, which suggests abnormal function at the hypothalamopituitary level.     

Internal vascular access for hemodialysis in children weighing less than fifteen kilograms.

An arteroarterial femoral graft using expanded polytetrafluoroethylene is described which has been successfully for vascular access in young children having small peripheral vessels. This graft allows high flow and favorable patency for dialysis without the complications of arteriovenous shunting or the risks associated with an external hemodialysis device. This graft has been used successfully for outpatient dialysis in children weighing as little as 9 kg and may be a useful adjunct in long-term dialysis of patients for whom more conventional means of vascular access are not acceptable.     

肝癌肝移植的适应证及疗效评价

肝切除是肝癌最常用的治疗手段。但是由于肝功能不全等因素,很多患者在诊断肝癌时已丧失了手术机会。肝移植不仅清除了肿瘤而且去除了硬化的肝脏,因此对早期肝癌伴有肝功能不全的患者,是理想的治疗方式。对肝癌肝移植来说,适应证的选择是影响术后肝癌复发及远期疗效的最关键因素。米兰标准则是最常用的纳入标准,按此标准进行肝癌肝移植5年生存率大于60．0％,效果良好。但米兰标准也存在局限性,更多的研究主张扩大米兰标准,建立新的标准。在我国,一些学者结合国人肝癌的特点提出了自己的标准,这些标准扩大了肝癌肝移植的适应证,且并未降低术后生存率,但有待进一步验证并形成相对统一的中国标准。另外,采用合理的综合治疗手段,预防肝癌的复发亦有待进一步的研究和探讨。     

Long-term outcome of liver transplantation for autoimmune hepatitis

BACKGROUND: Liver transplantation is the final therapeutic option for about 10% of patients with autoimmune hepatitis (AIH) who do not respond to medical therapy. The aim of this study was to evaluate the long-term outcome in serologically defined subgroups of AIH after transplantation. METHODS: Pre- and post-transplantation data of 28 patients with AIH transplanted between 1987 and 1999 were retrospectively analyzed and compared with 24 patients, who underwent liver transplantation because of Wilson's disease and glycogen storage disease type 1. RESULTS: Serological analyses identified patients with AIH type 1 (n = 13), type 2 (n = 5), and type 3 (n = 10). The 5-yr patient survival rate after liver transplantation was 78.2%, which was not significantly different from the control group. Six AIH patients and four control patients required re-transplantation because of initial non-function, chronic rejection or AIH recurrence. Patients transplanted for AIH (88%) had more episodes of acute rejection when compared with patients transplanted for genetic liver diseases (50%). Clinical and histological features of chronic rejection were present in four patients, which did not differ significantly from the controls. Recurrence of AIH was diagnosed in nine patients (32%) based upon the presence of autoantibodies, increased gamma-globulins, steroid dependency, and histological evidence of chronic hepatitis. These combined features were not found in any of the controls. CONCLUSIONS: Our data do not suggest that AIH subtypes influence prognosis after liver transplantation. Despite a high frequency of acute cellular rejection episodes and disease recurrence, transplantation for AIH has a 5-yr survival rate, which does not differ from that observed in patients transplanted for genetic liver diseases.     

大鼠原位肝移植模型的手术操作技巧探讨

目的 建立一个稳定的大鼠原位肝移植模型,探讨其手术技巧。方法在Kamada“二袖套法”的基础上进行改良。供体改经腹主动脉进行肝脏冷灌注;肝上下腔静脉用连续缝合法吻合,门静脉和肝下下腔静脉用袖套法吻合．胆总管采用单管内支架胆管端端吻合法。结果共施行大鼠原位肝移植140例,无肝期平均11min．手术成功率为97％,大鼠1周存活率为95％。结论改良的两袖套法具有操作简便、无肝期短、手术成功率高、大鼠术后存活时间长的优点,是大鼠原位肝移植的理想术式。娴熟细致的外科操作、受体无肝期的长短是决定动物存活的关键。     

Use of cuffed endotracheal tubes in infants less than 5 kilograms: A retrospective cohort study

BackgroundImproved understanding of airway anatomy and refinement of equipment have led to the increased use of cuffed endotracheal tubes (ETTs) in infants and children. Despite expanded evidence on the potential advantages of cuffed ETTs in pediatric patients, there remains limited data on their use in infants less than 5 kilograms (kg). The current study retrospectively evaluates the perioperative use of cuffed ETTs in infants weighing 2–5 kg.MethodsThis is a retrospective study from a tertiary care children's hospital involving a 3-year period. Data regarding anesthetic care, airway management, and postoperative course were retrospectively retrieved from the electronic medical record.ResultsThe study cohort included 1162 patients, 1086 of whom had their tracheas intubated with a cuffed ETT and 76 with an uncuffed ETT. Patients were divided into two groups for analysis: 2 to <3 kg and 3 to 5 kg. In both weight groups, cuffed ETTs resulted in a decreased need for more than one laryngoscopy and a change in ETT size with no increase in postoperative airway effects including stridor.ConclusionsThese data provide additional information regarding the efficacy and safety of cuffed ETTs in neonates and infants.     

A contemporary evaluation of surgical outcome in neonates and infants undergoing lung resection

Background/Purpose

The timing and need of resection of asymptomatic congenital lung lesions are controversial. The morbidity of such surgery needs to be considered in the decision analysis. We analyzed the contemporary outcome of infants and neonates undergoing lung resection.

Methods

With institutional review board approval, all patients 12 months or younger undergoing lung resection between 1995 and 2004 in 2 hospitals were reviewed. Demographic data, indications for surgery, operative procedure, complications, use of regional anesthesia, length of stay (LOS), and follow-up were assessed.

Results

Forty-five patients (28 male, 17 female) with a median age of 4 months (2 days-12 months) were evaluated. Congenital lesions (42) were the most frequent indication for surgery. Twenty-two (48.9%) patients had cardiorespiratory symptoms or infection preoperatively. Lobectomy was the most common operation (40/45). Three patients had intraoperative difficulty (bleeding, hypotension, desaturation). Significant postoperative complications occurred in 7 patients: prolonged air leak or chest tube drainage (4), anemia or bleeding (2), respiratory distress requiring reintubation (1). Fewer complications occurred in asymptomatic vs symptomatic patients (1/23 vs 6/22). Of 12 patients, 7 (58%) requiring 24 hours of ventilation or longer were 3 months or younger. Increasing age did significantly influence the chance of successful extubation (P = .01; odds ratio, 1.5; 95% confidence interval, 1.0-2.0), as did the use of epidural anesthesia (P < .001). Median LOS was 6 days (2-89 days). Asymptomatic patients had shorter LOS (median, 4 days; range, 2-20 days; P = .024) vs symptomatic patients (median, 8 days; range, 4-89 days). The only death occurred from underlying heart disease. Mean follow-up at 35 months (12-132 months) revealed no subjective reduction in cardiopulmonary function.

Conclusions

Lung resection is safe and well tolerated in infancy. Surgery should be scheduled before the development of symptoms but likely after 3 months of age to improve the chances of postoperative extubation. The use of regional anesthesia may facilitate this.