Preservation of the native spleen in multivisceral transplantation.

The native spleen is usually removed in patients undergoing MTV. The consequential asplenic state is associated with a high risk of sepsis, especially in immunosuppressed children. In contrast, the inclusion of an allogeneic spleen in multivisceral grafts has been associated with a high incidence of GVHD. We propose an alternative technique for patients undergoing MTV, consisting of the preservation of the native spleen. This approach avoids the additional risk of infection that characterizes the asplenic state without the detrimental side effects of the allogeneic spleen.

[Need of hepatic bipartition or split in the transplant in children]. / Necesidad de la bipartición hepática o split en el trasplante en niños.

AIM: To analyze the benefits of Split (for adult and for child) in liver transplantation. PATIENT/METHODS: 1) Analysis of the waiting list mortality estimated on 228 inclusions for transplant since January 2004 to December 2008.2) Impact of the variant techniques (living-related donor and split) on the waiting list mortality in our patients. 3) Analysis of the outcome of 33 split livers which allowed to perform 66 transplants (1994-2008). RESULTS: Estimated as number of patients by 1,000 candidates by year of exposure, the waiting list mortality was 110 in children older than 5 year old, 180 in children from 2 to 5 year-old, 90 in children between 1 and 2 year-old and 510 in younger than 1 year (p<0.05 for the last group). 36/66 split grafts were implanted by our group. Five grafts were lost, 3 due to retransplantation and 2 due to death. Overall patient/graft survival alter 10 years of follow-up was 94.5% and 85.1%, respectively. The rest of the grafts (n=30), were used in other hospitals, and 4 were lost in the early postoperative period. Since the beginning of the study, 85.4% of children between 1 and 2 years, received a living-donor or a split graft, as only 59.9% in the younger than 1 year-old group. CONCLUSION: Our results absolutely justify the ethics of split liver transplantation for an adult and a child. Despite other factors, the benefits of the variant techniques in the 1-2 year-old group are obvious. Up to 60% optimization with these techniques in children younger than 1 year would not be yet enough in order to decrease the mortality waiting list down to that of the rest of the groups.

[Intestinal transplant: in what phase are we?]. / Trasplante intestinal: ¿en qué fase estamos?

OBJECTIVE: To analyze the evolution of Small Bowel Transplantation program since the beginning of the program. MATERIAL AND METHODS: [corrected] All children who underwent intestinal transplantation between 1997 and 2009 were retrospectively reviewed: epidemiological data, status before transplant, surgical technique, immunosupression, results, survival and long.term quality of life were analysed. RESULTS: Fifty-two intestinal transplants were performed in 46 children (20 isolated bowel, 20 combined liver and intestine, and 12 multivisceral); median age was 32m (range 7m-19a); weight 12,3 kg (range 3,9-60); 31 had short gut syndrome, 8 dismotility, 5 intractable diarrhea, and two were miscellaneous. Intestinal adaptation was initially attempted in 26 patients, without success, 20 were directly listed for transplant. The modality of transplant was modified in 17 while listed. Baseline immunosupression consisted of tacrolimus and steroids, although 5 required conversion to Sirolimus later. Six died during the first month, due to sepsis/multiorganic failure (poor status at transplant); 13 died during the long-term follow-up. Acute rejection was seen in 20, chronic rejection in 3, PTLD in 8 (6 died) and GVHD in 5 patients (3 died). Overall survival after 5 years of follow-up is 65,2 % (51,7% for the graft). From 2006 to 2008, overall patient/graft survival at 6 m, 1 and 3 years after transplant is 88,7/84,1, 81,2/81,2 and 81,2/71,1%, respectively. After a median follw-up of 39 +/- 29 months, 27 patients are alive (59%), off TPN, (70% had their ostomy taken down), go to school, are scarcely hospitalized and enjoy a good quality of life. CONCLUSIONS: Intestinal transplantation has consolided itself as a good choice for irreversible intestinal failure, being feasible to achieve a normal life. Although overall survival diminishes over time, the center experience has improved the results. These patients need a very close follow-up, once transplant is over, in order to get an early diagnose of immunological complications.

Cross-cutting view of current challenges in paediatric solid organ and haematopoietic stem cell transplantation in Europe: the European Reference Network TransplantChild.

The low prevalence of European paediatric transplanted patients and scarcity of resources and expertise led to the need for a multidisciplinary network able to improve the quality of life of paediatric patients and families requiring a solid organ or haematopoietic stem cell transplantation. The European Reference Network (ERN) TransplantChild is one of the 24 ERNs established in a European legal framework to improve the care of patients with rare diseases. ERN TransplantChild is the only ERN focused on both solid organ and haematopoietic stem cell paediatric transplantation, based on the understanding of paediatric transplantation as a complex and highly specialised process where specific complications appear regardless the organ involved, thus linking the skills and knowledge of different organ disciplines. Gathering European centres of expertise in paediatric transplantation will give access to a correct and timely diagnosis, share expertise and knowledge and collect a critical mass of patients and data that increases the speed and value of clinical research outcomes. Therefore, the ERN TransplantChild aims for a paediatric Pan-European, Pan-transplant approach.

Surgery of liver tumors in children in the last 15 years.

AIM: Aim of the study was to review our experience in the management of liver tumors in children over the last 15 years. PATIENTS AND METHODS: A cohort of 78 children with liver tumors managed in our institution between 1991 and 2006 was retrospectively reviewed. There were 45 males and 33 females with a mean age of 32 +/- 41 months at diagnosis. Most tumors were malignant (n = 57); the most frequently occurring tumor was hepatoblastoma (n = 47), followed by hepatocarcinoma (n = 5), sarcoma (n = 4), and lymphoma (n = 1). Vascular tumors (n = 12) predominated among the benign tumors followed by mesenchymal hamartoma (n = 4), focal nodular hyperplasia (n = 3), adenoma (n = 1), and inflammatory pseudotumor (n = 1). We reviewed the epidemiologic features, clinical presentation, diagnosis, treatment and outcomes. We employed MRI and angio-CT for SIOPEL PRETEXT staging and selected the management accordingly for malignant tumors. We analyzed the long-term survival using Kaplan-Meier curves. RESULTS: Benign tumors had an excellent outcome with both medical or surgical management. Of the malignant tumors 4 were PRETEXT I and were treated by left lateral segmentectomy with 100 % survival; 20 were PRETEXT II (12 left and 8 right lobe) and were treated by lobectomy of the corresponding side, except for 1 case which required OLT (90 % survival); 9 children had PRETEXT III tumors requiring trisegmentectomy or extended lobectomies with OLT in 1 case (77.7 % survival). Fourteen children had PRETEXT IV tumors: 10 received OLT and 9 of them are still alive (64.2 % survival). Overall survival was 80.8 %, and actuarial survival at 6 years was 82.2 %. Other malignant tumors had variable results. CONCLUSIONS: Outcomes have improved much in the last years. Surgical removal is necessary in most cases. Transplantation is a very useful adjunct. Treatment of these tumors should be concentrated in centers with expertise.

[Multidisciplinary approach in the management of intestinal failure]. / Tratamiento multidisciplinario del fallo intestinal.

OBJECTIVE: [corrected] Our aim was to analyze our results in the management of intestinal failure with a multidisciplinary approach including optimized parenteral nutrition, reconstructive surgery and intestinal transplantation (ITx). MATERIAL AND METHODS: We included all patients evaluated by our team with the diagnosis of IF. We assessed outcome, mortality and complications in children that achieved adaptation and those listed for ITx. RESULTS: Seventy one children (40 boys, 31 girls) were evaluated between 1997 and 2006 because of IF. Forty eight (76%) were referred from other institutions. In 56 cases (80%) IF began in the newborn period. Causes of IF were: short bowel syndrome (52) intestinal motility disorders (16) and intestinal epithelial disorders (3). Median birth weight in the group of SBS was 2.2 Kg and prematurity was an associated condition in 15% of them. Overall, fourteen patients (20%) achieved intestinal adaptation with progressive weaning from PN, the management of these children consisted of optimized parenteral and enteral nutrition and autologous intestinal reconstructive surgery. Nine (13%) are stable under home parenteral nutrition regimen. Eight children (11%), all of them listed for liver and small bowel transplantation, died in the waiting list after a mean waiting time of more than 300 days, with a median of 4 laparotomies and 4 episodes of catheter related sepsis. Four children (5.6%) died in the adaptation process or before their inclusion on the waiting list. Finally, twenty five (35,2%) children underwent 28 intestinal transplantation: 9 isolated small bowel transplantation (SBTx), 16 combined liver and small bowel (CLSB) and 3 multivisceral (MVTx). Among transplanted patients, 9 (36%) died, (3 MVTx, 1 SBTx and 8 CLSB) and four were retransplanted. CONCLUSIONS: Intestinal Transplantation is an established alternative to parenteral nutrition in the treatment of IF, although complications and mortality rates are still considerable, especially MVTx and CLSBTx. Mortality in children listed for intestinal transplantation remains also high. Intestinal adaptation can be achieved with adequate rehabilitation therapy even in some cases with apparently irreversible intestinal transplantation. Early referral before liver failure or other complications arise is crucial is crucial in order to improve the outcome of these patients.

[Orthotopic liver transplantation in children younger than one year]. / Trasplante hepático en menores de un año.

BACKGROUND: Orthotopic liver transplantation (OLT) in children younger than one year is associated to higher waiting list mortality and alternative graft sources are required. We present our experience with this particular group of age. METHODS: Infants younger than one year who received an OLT between 1986 and 2005 were reviewed focused on graft and children survival depending on period and type of graft. Periods were 1:1986-1995; 2:1996-2000 and 3:2001-2005. We also evaluate cold ischemia time (CIT), graft lost causes and differences between CIT and anhepatic time (AT) depending on graft type. RESULTS: Eighty-three children received 103 OLT. Liver transplant indications were 59 (72%) biliary atresia, 8 (10%) metabolic causes, 6 (8%) liver failure, 3 (4%) cirrhosis and 7 (6%) miscelaneous. Patient and graft survival after 5 years was increased depending on period: 45% and 65% on period 1, 70% and 80% on period 2, 94% y 97% on period 3 (p < 0.0198). Thirty-seven grafts were reduced lobes (42%); 8 (21%), 17 (45%) and 12 (35%) during periods 1, 2 and 3 respectively and their 5 years survival rate was 68%. Twenty-four were whole grafts (31%); 11 (45%), 10 (45%) and 3 (14%) during periods 1, 2 and 3 and their 5 years survival rate was 63%. Fourteen grafts were living-related donor (16%); 1 (7%), 2 (14%) and 11 (79%) during periods 1, 2 and 3 and their 5 years survival rate was 93%. Eight (11%) were split; 0, 1 (12%) and 7 (90%) during periods 1, 2 and 3 and their 5 years survival rate was 100%. Average CIT depending on graft was: living donor 5,5 hours (IQR: 4-7), split 6,1 hours (IQR: 5-8), whole 9.2 hours (IQR: 6-11) and reduced 8.5 hours (IQR: 6-11) (p < 0.05). Average AT depending on graft was: living donor 1 hour (IQR: 0.5-1.5), split 1 hour (IQR: 0.5-1.4), whole 1,1 hours (IQR: 0.5-1.5) (p > 0.1). Twenty-four grafts were lost (28%): 10 (41%) were surgical related causes and 6/10 (60%) of them were whole grafts. CONCLUSIONS: Survival rates in children younger than one year are similar to another groups of age. There was a significant increase on graft survival according to transplantation group experience. A higher rate of graft lost is associated to whole grafts. Most frequent reasons of graft lose were related to sepsis and immunosuppresion. A significant shortening of CIT is observed in related living donor and split grafts.

Management of cytomegalovirus infection in solid organ transplant recipients: SET/GESITRA-SEIMC/REIPI recommendations.

Cytomegalovirus (CMV) infection remains a major complication of solid organ transplantation. Because of management of CMV is variable among transplant centers, in 2011 the Spanish Transplantation Infection Study Group (GESITRA) of the Spanish Society of Infectious Diseases and Clinical Microbiology (SEIMC) developed consensus guidelines for the prevention and treatment of CMV infection in solid organ transplant recipients. Since then, new publications have clarified or questioned the aspects covered in the previous document. For that reason, a panel of experts revised the evidence on CMV management, including immunological monitoring, diagnostics, prevention, vaccines, indirect effects, treatment, drug resistance, immunotherapy, investigational drugs, and pediatric issues. This document summarizes the recommendations.

Comparative study between living and cadaveric donors in pediatric liver transplantation.

UNLABELLED: We examined whether the results in living-related hepatic transplantation (LRLT) are better than those from a cadaveric donor (CDLT). MATERIAL AND METHODS: The last 27 consecutive LRLT, performed from 1998 to 2005, were compared with 27 CDLT matched for age, weight, date, and diagnosis. Grafts in LRLT group were left lateral segment (n = 22), left lobe (n = 3), and right lobe (n = 2). In the CDLT group, the grafts were split in situ (n = 10), hepatic reduction (n = 9) and whole liver (n = 8). We analyzed the actuarial survivals (grafts and children), retransplantation, primary nonfunction, initial graft malfunction (liver enzymes >2000 U/L), surgical complications, rejection, and resource consumption. RESULTS: Patient survivals at 6 months, 1 year, and 5 years were 100%, 96%, and 96% in LRLT and 100%, 100%, and 100% in CDLT (P = NS). Graft survivals were 93%, 89%, and 89% versus 96%, 96%, and 96%, respectively (P = NS). Complications were biliary complications (LRLT, 25% vs CDLT, 3%; P = .021); portal vein thrombosis (LRLT, 7% vs CDLT, 3%; NS), and hepatic artery thrombosis (LRLT, 0% vs CDLT, 3%; NS). The overall incidence of acute rejection was slightly higher (NS) in LRLT (LRLT, 18% vs CDLT, 11%; NS). Liver enzyme levels were higher in the CDLT group, but initial malfunction rate was not statistically different. Regarding resource consumption: blood product needs were higher in LRLT (P < .05) and hospital stay and ICU stay were longer, although not significantly, among LRLT. CONCLUSIONS: The results in LRLT among children are similar to those obtained in CDLT. We found a trend towards less initial graft malfunction in LRLT. Blood product needs were higher in LRLT. Hospital and ICU stay were longer, but not significantly different in LRLT. The benefits of LRLT are saving a scarce resource: a cadaveric donor liver graft.

Intestinal transplantation in children: differences between isolated intestinal and composite grafts.

The results of the isolated intestinal grafts were compared with those of composite grafts (intestinal graft + liver) in a series of 18 transplantations performed in 17 children; 5 isolated intestinal grafts, 12 hepatointestinal grafts, and 1 multivisceral graft. Causes of intestinal failure were short bowel syndrome (n = 13), motility disorders (n = 2) and congenital epithelial disorders (n = 2). Transplantation was indicated due to end-stage liver disease (n = 14), loss of venous access (n = 2), untreatable diarrhea (n = 1) and high morbidity associated with a poor quality of life (n = 1). Six children, all with a composite graft, died after transplantation due to lymphoma (n = 2), sepsis (n = 1); intraabdominal bleeding (n = 1); pneumonia (n = 1); and overwhelming adenoviral infection (n = 1). Digestive autonomy was achieved in 16 of 18 grafts, the 11 surviving children are free of parenteral nutrition with a reasonably good quality of life. In conclusion, intestinal transplantation is a viable therapeutic alternative for children with permanent intestinal failure. The results of transplantation with an isolated intestine are clearly better that those with a composite graft.

Pediatric intestinal transplantation.

AIM: Analyze the results of a paediatric intestinal transplantation (IT) program in Spain. PATIENTS: During an 5-year period, 18 children were included as candidates for IT. The causes for intestinal failure (IF) were short bowel syndrome (n=13), motility disorders (n=3), and congenital epithelial disorders (n=2). Nine children were admitted for a combined liver-small bowel transplant (LSBT), seven for an isolated intestinal transplantation (IIT) and two for a multivisceral transplantation (MVT). In three of the candidates for IIT the indication had to be changed to LSBT because of progression of the liver damage. RESULTS: Eight candidates are on the waiting list: four for LSBT, two for IIT, and two for MVT. Four children died before transplantation. All were children under 1 year and candidates for LSBT. One child died during an attempted MVT. Five children underwent transplantation. Grafts were IIT in two and LSBT in three. Of these children, two are on a normal diet (respective follow-up times: 40 and 18 months), two died, both with functioning liver and intestinal grafts (hemorrage after liver biopsy and lymphoproliferative disease), and one developed an untreatable rejection that lead to loss of the intestinal graft; currently, she is on the waiting list for LSBT. CONCLUSIONS: The morbidity and mortality of IT are high, but it is the only possible treatment for children in IF who cannot be adequately managed with parenteral nutrition. A severe problem is the the scarcity of suitable donors for the very low weight children who are candidates for LSBT.

Pediatric living donor liver transplantation.

AIM: The aim of this study was to analyze the results of living donor in a pediatric liver transplantation program. PATIENTS: Twenty-six living donor liver transplantations were performed in children from 0.5 to 14.8 years of age. The main indication was biliary atresia (72%) followed by tumors (2 hepatoblastomas and 1 hepatocarcinoma). Left lateral segments were used in 23 (1 transformed into a monosegment), 1 left lobe was used in 1, and right lobes were used in 2. Arterial reconstruction employed saphenous venous grafts in the first 3 cases and end-to-end anastomoses with a microsurgical technique in the following 22 cases. RESULTS: There has been no major morbidity in the donors, with a median hospitalization of 6 days. Four grafts have been lost; 2 in the first 3 cases. In only 1 case, the graft loss was related to the procedure saphenous venous graft thrombosis). Early biliary complications were frequent (23%). Six month, 1 year, and 5 year graft and patient survival rates were 91%, 85%, and 85% and 100%, 96%, and 96%, respectively. CONCLUSIONS: Living donor liver transplantation is an excellent option for transplantation in children.

Liver transplantation in patients with homozygotic familial hypercholesterolemia previously treated by end-to-side portocaval shunt and ileal bypass.

Familial hypercholesterolemia is the result of mutations in the gene that encodes the synthesis of the cellular receptor for low density lipoprotein (LDL). In the homozygous form of the disease (HFHC), cellular LDL receptors either do not form, or, when present, cannot bond LDL and mediate its cellular uptake LDL, and the cholesterol that it transports accumulate in plasma, producing severe premature atherosclerosis and death from coronary artery disease usually before the age of 20. Currently, the only effective treatment is liver transplantation, which, alone or in association with medications, normalizes plasma cholesterol levels. The authors report the cases of 2 siblings with HFHC who underwent portocaval shunt at the ages of 2.5 and 1.5 years, respectively. Portocaval shunt produced an immediate, but insufficient decrease in cholesterol (by 40% and 35%, respectively), leaving them with cholesterol concentrations of about 500 mg/dL. One year later they each underwent ileal bypass without obtaining any significant response. Liver transplantation at the ages of 18 and 16 years, respectively, reduced plasma cholesterol concentrations to 129 and 225 mg/dL, respectively. The earlier operations seriously increased the technical difficulty of liver transplantation and did not produce a favorable effect on the natural course of the disease, so portocaval shunt and ileal bypass are not indicated in HFHC, not even for the purpose of delaying liver transplantation.

Long-term survival expectancy after liver transplantation in children.

PURPOSE: The aim of this study was to assess the long-term survival rate in children who have undergone orthotopic liver transplantation (OLT) in the last 13 years. METHODS: The records of 198 consecutive patients under 18 years of age who underwent 249 OLTs between 1986 and 1998 were reviewed. Actuarial patient survival rates were assessed at 1, 3, 5, and 10 years in the whole series, in the last 5 years, and in patients surviving more than 1 year. Age, weight, and indications were analyzed, as well as type and incidence of posttransplant complications. The median follow-up period was 41 months (0 to 154 months). RESULTS: Biliary atresia was the most common indication (41.9%) followed by alpha-1 antitrypsin deficiency (8.1%), Alagille syndrome (7.6%), and fulminant hepatic failure (6.6%). One hundred forty-six patients (58.6%) were below 5 years, and 46 patients were (18.5%) younger than 1 year at operation. Sixty-eight patients (27.3%) weighed less than 10 kg. One hundred seventy whole organs and 70 reduced, 5 living-related donor, and 4 split-liver allografts were used. Hepatic artery thrombosis (n = 18), primary nonfunction (n = 15), and chronic rejection (n = 14) were the most common causes for allograft failure. Fourteen patients (7%) had posttransplant lymphoproliferative disorders (PTLD) at a median time of 28 months (4 to 124 months) postoperation (3 died). The 1-, 3-, 5-, and 10-year actuarial patient survival rates are 80%, 76%, 74%, and 74%, respectively; over the last 5 years it is 88% at 1 year and 82% at 3 and 5 years. For patients surviving more than 1 year, 3-, 5-, and 10-year actuarial survival rates are 95%, 93%, and 93%, respectively. CONCLUSIONS: (1) Overall results of OLT improve with increasing experience. (2) Children who survive more than 1 year after OLT have an excellent prognosis, although long-term complications of immunosuppression can be expected.

Late biliary complications in pediatric liver transplantation.

PURPOSE: The aim of this study was to review the biliary complications occurring in late follow-up after liver transplantation in children. METHODS: The medical records of 135 children who received orthotopic liver transplantations (OLT) and had graft survival of more than 1 year were reviewed. Technical variants using a reduced-size graft were applied in 32 (23.7%). For biliary reconstruction, 15 patients had choledochocholedochostomy and 120 a Roux-en-Y loop. Biliary reoperation in the early post-OLT period was needed in 24 patients (17.7%). Routine checking of liver function and duplex Doppler ultrasonography (DDS) were performed during the follow-up period, which averaged 58 months. Late biliary complication was defined as that occurring after the first hospital discharge. RESULTS: Late biliary complications occurred in 18 children (13.3%); 16 showed symptoms or analytical disturbances in liver function tests. The Diagnoses included uncomplicated cholangitis (n = 6), anastomotic biliary stricture (n = 7), ischaemic damage of the biliary tree (n = 3) including one late (28 months) hepatic artery thrombosis leading to an intrahepatic biloma. and bile leak after T-tube removal (n = 2). The six children with uncomplicated cholangitis had no repeat episodes in follow-up despite persistent aerobilia. Six patients affected by anastomotic strictures were treated successfully with percutaneous dilatation and, if present, stone removal. Persisting dysfunction and cholangitis occurred in one case affected by ischaemic biliary disease. Biliary leaks after T tube removal settled spontaneously. Risk factors for late biliary complications were determined. There was no relation to the cold ischaemia time, type of graft or biliary reconstruction, or previous early post-OLT biliary reoperation. Aerobilia (affecting 21.5% of OLT patients) was related to cholangitis (P = .001). CONCLUSIONS: Anastomotic strictures, reflux of intestinal contents via the Roux-en-Y loop, and residual ischaemic damage led to late biliary complications in 12% of paediatric OLT patients. Evidence of biliary dilatation on DDS may be delayed in anastomotic strictures; in these cases the results of percutaneous treatment were excellent. Children with aerobilia have and increased risk of cholangitis.

Donor vascular grafts for arterial reconstruction in pediatric liver transplantation.

The authors compared the results of 48 orthotopic liver transplantations (OLT) in which revascularization was achieved with a conduit interposed between the receptor aorta and the graft (vascular graft [VG] group) with those obtained for 56 OLT performed during the same period (1991 to 1994) in which end-to-end anastomosis (EEA) of the hepatic arteries or celiac trunk was used (EEA group). In the VG group, the interposed conduits were the cadaveric iliac artery (37) the living-donor saphenous vein (3), or nonthrombosed conduits from previous transplants (8) (7 iliac arteries, 1 saphenous vein). There were significant differences between the two groups with respect to recipient age, recipient weight, the retransplant:first transplant ratio, the number of emergency transplantations, the use of reduced-size grafts, and intraoperative transfusion requirements. Twenty-nine grafts in the VG group (60.4%) and 43 in the EEA group (76.7%) currently are functioning. The actuarial 3-year graft survival rates are 60% and 71.5% for the VG and EEA groups (P < .05), respectively. The rate of arterial thrombosis did not differ between the two groups. The authors conclude that, although EEA of the hepatic artery is still the preferred revascularization technique for OLT, revascularization of the liver graft by conduit interposition is safe when EEA is not possible. Reutilization of the interposed conduit during retransplantation proved to be safe in the absence of hepatic artery thrombosis.

Our first 100 consecutive pediatric liver transplants.

Orthotopic liver transplantation (OLT) is nowadays accepted as the best therapy for end-stage liver disease. The difficulties involved are even greater in children than in adults, and it is debatable whether exclusively pediatric programs are warranted. The aim of this paper is to analyze our experience at the Children's Hospital "La Paz", with the first consecutive 100 OLT in children, 61% of whom weighed less than 20 kg. Since 1986, 220 pediatric patients were evaluated as candidates, 100 OLT were performed in 78 patients and 13 died on the waiting list, currently maintained below 15 cases. Indications were: cholestasis (45), metabolic disease (18), fulminant hepatic failure (3), primary liver tumors (2) and cirrhosis (10). Mean age was 66 months (range = 7 to 216) with a mean weight of 21 kg (range = 6 to 60), twenty patients weighed less than 13 kilograms. OLT was performed by standard technique. Reduced or segmental grafts were necessary in 8 instances. Twenty-two patients were retransplanted and 2 received three grafts. Indications for retransplantation were: hepatic artery thrombosis (8), primary nonfunction (4), chronic rejection (7), portal thrombosis (2) and Budd-Chiari recurrence (1). Acute rejection was observed in 52 patients, and eight cases developed a chronic rejection. These episodes were treated with "bolus" of steroids, monoclonal antibodies (OKT-3) and FK-506. Surgical complications included: hepatic artery thrombosis 12%, portal vein thrombosis 3% and biliary fistula or stenosis 13%. The incidence of primary non-function was 7%. Actuarial survival rate at 5 years was 75%.(ABSTRACT TRUNCATED AT 250 WORDS)

Innovative techniques in pediatric liver transplantation: reduced-, split- and living-donor related liver transplantation.

In spite of the increased number of organ donations, the amount of livers available for pediatric patients does not meet requirements. In an attempt to expand the pool of grafts, several techniques of size reduction (RST) have been developed: reduced-liver transplantation (RLT), liver segment transplantation (LST), "split" liver transplantation (SLT) and living-donor liver transplantation (LDLT). The aim of this work is to study the contribution of these techniques to reduction of mortality in the waiting list and the increased risks that we inflict to our patients by using these procedures. We report our experience on RST in the last nine years (23 RLT, 5 LST, 2 SLT and 3 LDLT). More than a half of them were performed in an emergency. Overall survival in children with RST (67%) is similar to that of patients receiving a "whole graft" (74%). We did not find significant differences in the incidence of graft-related complications, only reintervention for abdominal bleeding was needed more often in the RST group (30%) than in whole graft group (24%). Our data confirm the safety of the RST procedures in the pediatric liver transplantation programs and they decrease the waiting-list mortality (2.5%) among the pediatric patients.

[Children with intestinal failure as candidates for intestinal transplantation]. / Niños con fallo intestinal candidatos a trasplante intestinal.

Small bowel is not anymore considered a forbidden organ for transplantation, and intestinal transplantation (IT) is currently used as a therapeutic option in selected cases of permanent intestinal failure (PIF). Nevertheless, the experience is still scarce, and despite recent improvements, IT is nowadays only accepted as a life-saving option. However, small children are prone to suffer complications related to TPN, particularly end stage liver disease; moreover, suitable donor for the small baby who needs an IT is seldom available. Subsequently, a high pretransplantation mortality has been reported in the pediatric series. In those cases, the indication of IT shouldn't be delayed, and these children must be referred early for IT. Since we started our IT program, 17 children have been assessed, and 3 are currently on the waiting list; two for combined liver-small bowel transplantation (LSB), both with short bowel syndrome and end stage liver disease, and one patient with microuvillous dysplasia for isolated IT (indication loss of venous access). 3 children were referred too late and died, so did a fourth patient, candidate for LSB, before he could be transplanted.

[Reduced-size liver transplants from cadaver and living donors: an alternative to waiting lists]. / Trasplante hepático reducido de cadáver y donantevivo: una alternativa a las listas de espera.

The ever expanding of indications of liver transplantation in children make pediatric donor pool unable to fulfill the needs. The reduced and partial transplants from cadaveric and living related donors reveal an effort to increase the number of pediatric grafts aid to reduce the mortality in the waiting list. Among 126 pediatric transplants performed in a 8-year period, 18 (15%) were reduced (n = 3) and partial livers (with preservation of the recipient vena cava) (n = 15). In 1993, 41% (n = 12) of the 29 liver transplantations performed were partial segments. In two of them the graft was harvested from a living-related donor. Eight transplants were made on an emergency basis and ten were elective. Eight patients were retransplanted. Considering the transplants performed in the Wisconsin era, after completing the "learning curve" the actuarial survival at five years reaches 65% approaching 70% for patients younger than 1 year and/or weighing less than 13 kg. The arterial complications in this group are limited to a single case of thrombosis. Despite our limited experience we conclude that reduced and partial transplants are useful to reduce the mortality in the waiting list with a survival similar to that of the whole grafts and with less vascular complications. The living-related transplant represents another step ahead that allows a further reduction of the shortage of organ available for the small children.