Selective portal embolization combined with chemotherapy treatment in non-resectable liver tumors reduces tumor volume and generates contralateral compensating hypertrophy allowing for a full resection while avoiding liver transplantation. / La embolización portal selectiva coadyuvante al tratamiento quimioterápico en tumores hepáticos no resecables produce una reducción tumoral e hipertrofia compensadora contralateral que permite una resección completa evitando el trasplante hepático.

INTRODUCTION: Non-resectable liver tumors are one of the most frequent indications of pediatric liver transplantation. However, the usefulness of interventional techniques is still to be exploited. This is the case of a patient with a non-resectable liver sarcoma proposed for liver transplantation, which could be avoided as the tumor was fully resected following selective portal embolization combined with chemotherapy treatment. CLINICAL CASE: The patient was a 10-year-old female with a large hepatic mass compatible with undifferentiated PRETEXT III sarcoma treated according to the EpSSG RMS2005 protocol for high-risk tumors, but without achieving any reduction in volume. Given the risk of developing a small-for-size syndrome following a potential resection, a right portal embolization was performed in order to induce left hemi-liver hypertrophy. No response was observed after two months, so the patient was included on the liver transplantation list. However, one month later, a significant reduction in mass volume was noted at a control CT-scan, which meant the tumor could be resected while avoiding transplantation. The one-year follow-up was uneventful. DISCUSSSION: Interventional techniques should be considered as the main therapeutic option in non-resectable liver tumors, since they could potentially avoid transplantation in selected patients.

INTRODUCCION: Los tumores hepáticos irresecables figuran entre las indicaciones más frecuentes del trasplante hepático pediátrico. Sin embargo, las técnicas intervencionistas suponen un campo terapéutico con utilidades aún por explotar. Presentamos el caso de una paciente con un sarcoma hepático irresecable propuesta para trasplante hepático, que pudo evitarlo al ser completamente resecado tras realizar una embolización portal selectiva coadyuvante al tratamiento quimioterápico. CASO CLINICO: Paciente mujer de 10 años con masa hepática de gran tamaño compatible con sarcoma indiferenciado PRETEXT III tratado según el protocolo EpSSG RMS2005 para tumores de alto riesgo sin conseguir reducción del mismo. Debido al riesgo de desarrollar un síndrome small-for-size tras una eventual resección, se llevó a cabo una embolización portal derecha para inducir la hipertrofia del hemi-hígado izquierdo. Tras dos meses no se evidenció respuesta alguna, por lo que la paciente fue incluida en lista de trasplante hepático. Sin embargo, un mes después se observó una clara disminución de la masa en un TC de control y la resección pudo realizarse con éxito evitando así el trasplante. Tras un año de seguimiento, la paciente no presentó ningún evento clínico de interés. DISCUSION: Las técnicas intervencionistas deben ser consideradas como una opción terapéutica en tumores hepáticos irresecables, ya que podrían evitar el trasplante en pacientes seleccionados.

La embolización portal selectiva coadyuvante al tratamiento quimioterápico en tumores hepáticos no resecables produce una reducción tumoral e hipertrofia compensadora contralateral que permite una resección completa evitando el trasplante hepático / Selective portal embolization combined with chemotherapy treatment in non-resectable liver tumors reduces tumor volume and generates contralateral compensating hypertrophy allowing for a full resection while avoiding liver transplantation

Introducción: Los tumores hepáticos irresecables figuran entrelas indicaciones más frecuentes del trasplante hepático pediátrico. Sinembargo, las técnicas intervencionistas suponen un campo terapéuticocon utilidades aún por explotar. Presentamos el caso de una pacientecon un sarcoma hepático irresecable propuesta para trasplante hepático,que pudo evitarlo al ser completamente resecado tras realizar una embolización portal selectiva coadyuvante al tratamiento quimioterápico. Caso clínico: Paciente mujer de 10 años con masa hepática de grantamaño compatible con sarcoma indiferenciado PRETEXT III tratadosegún el protocolo EpSSG RMS2005 para tumores de alto riesgo, sinconseguir reducción del mismo. Debido al riesgo de desarrollar un síndrome small-for-size tras una eventual resección, se llevó a cabo unaembolización portal derecha para inducir la hipertrofia del hemi-hígadoizquierdo. Tras dos meses no se evidenció respuesta alguna, por lo quela paciente fue incluida en lista de trasplante hepático. Sin embargo,un mes después se observó una clara disminución de la masa en unTC de control y la resección pudo realizarse con éxito evitando así eltrasplante. Tras un año de seguimiento, la paciente no presentó ningúnevento clínico de interés.Discusión: Las técnicas intervencionistas deben ser consideradascomo una opción terapéutica en tumores hepáticos irresecables, ya quepodrían evitar el trasplante en pacientes seleccionados.(AU)

Introduction: Non-resectable liver tumors are one of the most frequent indications of pediatric liver transplantation. However, the usefulnessof interventional techniques is still to be exploited. This is the case of apatient with a non-resectable liver sarcoma proposed for liver transplantation, which could be avoided as the tumor was fully resected followingselective portal embolization combined with chemotherapy treatment. Clinical case: The patient was a 10-year-old female with a largehepatic mass compatible with undifferentiated PRETEXT III sarcomatreated according to the EpSSG RMS2005 protocol for high-risk tumors,but without achieving any reduction in volume. Given the risk of developing a small-for-size syndrome following a potential resection, a rightportal embolization was performed in order to induce left hemi-liverhypertrophy. No response was observed after two months, so the patientwas included on the liver transplantation list. However, one month later,a significant reduction in mass volume was noted at a control CT-scan,which meant the tumor could be resected while avoiding transplantation.The one-year follow-up was uneventful. Discussion: Interventional techniques should be considered as themain therapeutic option in non-resectable liver tumors, since they couldpotentially avoid transplantation in selected patients.(AU)

Health-related quality of life in pediatric intestinal transplantation.

To determine HRQOL after pediatric intestinal transplantation. Thirty-four IT survivors from 1999 to 2012 were asked to complete age-specific HRQOL non-disease-specific questionnaires: TAPQOL (0-4 yr), KINDL-R (5-7 yr; 8-12 yr; 13-17 yr), and SF-36v2 (>18 yr), all validated with Spanish population. Primary caregiver completed a SF-36 questionnaire and CBI. Thirty-one participants were included. Median age was 10.2 yr (1-29) and time after transplant 4.4 yr (0-13). Overall patient scores were 78.2 ± 10.6 (n = 8), 83.3 ± 9.7 (n = 6), 72.2 ± 9.21 (n = 6), 80.5 ± 12.4 (n = 7), and 82.2 ± 12.4 (n = 4) for each age group. Highest scores were obtained for vitality (group I), self-esteem (group IV), and physical and social functioning and emotions (group V). Lowest scores were obtained in appetite and behavior (I), family and school (III), and chronic disease perception (III, IV). No significant differences were found between caregivers and their children. CBI showed stress in 52%. SF-36 for caregivers was lower than general population. No significant differences were found depending on relevant clinical and sociodemographic data. HRQOL was acceptable and improved with age and time since transplantation. Parents had a slighter own QOL and worse perception of health than their children. When successful, intestinal transplantation allows a normal life in most patients and can be offered as an attractive option.

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.

[Technical aspects of experimental intestinal transplant]. / Aspectos técnicos del trasplante intestinal experimental.

PURPOSE: Our objective is to analyze the variables that influence the outcome of Small Bowel Transplantation (SBT) in rats in an experimental microsurgery program. The surgical technique and perioperative care are described in detail. METHODS: Retrospective study of the SBT in rats conducted in our experimental surgery laboratory from 2002 to 2010. The animals were divided into group A (those who survived more than 48 hours) and group B (those who died earlier without justificable cause). We compared in both groups: number of transplants performed by the surgeon, warm ischemia time, cold ischemia time and duration of the procedure. RESULTS: Five surgeons with different degrees of microsurgical training participated in the study. A total of 521 SBT were performed with an overall survival of 48%. The first successful transplant was performed after a median of 46 (25-68) transplants. Total procedure time (3.5 vs 2.9 hours) and warm ischemia time (51 vs 35 minutes) were higher in group B (p < 0.05). DISCUSSION: The number of transplants required for learning the technique is high. However, survival is acceptable when the time needed for vascular anastomosis is reduced. The SBT in rats is a valuable model for surgical training and research of the phenomena related to SBT.

Aspectos técnicos del trasplante intestinal experimental / Technical aspects of experimental intestinal transplant

Introducción. Nuestro objetivo es analizar las variables que in-fluyen en los resultados del trasplante de intestino delgado (TID) en ratas en un programa de microcirugía experimental. Se describe con detalle la técnica quirúrgica y los cuidados perioperatorios con objeto de favorecer el aprendizaje de la técnica. Material y métodos. Estudio retrospectivo de los TID en ratas realizados en nuestro laboratorio de cirugía experimental desde el año 2002 al 2010. Dividimos los animales en grupo A (los que sobrevivieron más de 48 horas) y grupo B (los que fallecieron precozmente sin causa justificable). Comparamos en ambos grupos: número de trasplantes realizados por el cirujano, tiempo de isquemia caliente, tiempo de isquemia fría y duración total del procedimiento. Resultados. Cinco cirujanos con distinto grado de formación microquirúrgica participaron en el estudio. Se realizaron en total 521 TID con una supervivencia global del 48%. El primer trasplante con éxito se realizó tras una mediana de 46 (25-68) trasplantes. El tiempo total del procedimiento (3,5 vs. 2,9 horas) y el tiempo de isquemia caliente (51 vs. 35 minutos) fueron superiores en el grupo B (p<0,05). Discusión. El número de trasplantes necesarios para el aprendizaje de la técnica es elevado. Sin embargo, la supervivencia es aceptable al reducir el tiempo empleado en las anastomosis vasculares. El TID en ratas constituye un modelo muy valioso para la formación del cirujano y para la investigación de los fenómenos relacionados con el TID (AU)

Purpose. Our objective is to analyze the variables that influence the outcome of Small Bowel Transplantation (SBT) in rats in an experimental microsurgery program. The surgical technique and perioperative care are described in detail. Methods. Retrospective study of the SBT in rats conducted in our experimental surgery laboratory from 2002 to 2010. The animals were divided into group A (those who survived more than 48 hours) and group B (those who died earlier without justificable cause). We compared in both groups: number of transplants performed by the surgeon, warm ischemia time, cold ischemia time and duration of the procedure. Results. Five surgeons with different degrees of microsurgical training participated in the study. A total of 521 SBT were performed with an overall survival of 48%. The first successful transplant was performed after a median of 46 (25-68) transplants. Total procedure time (3.5 vs 2.9 hours) and warm ischemia time (51 vs 35 minutes) were higher in group B (p<0.05).Discussion. The number of transplants required for learning the technique is high. However, survival is acceptable when the time needed for vascular anastomosis is reduced. The SBT in rats is a valuable model for surgical training and research of the phenomena related to SBT (AU)

Liver transplantation in children with cystic fibrosis: experience in our centre and preliminary results with a combined en bloc liver-pancreas graft.

AIM OF THE STUDY: Cystic fibrosis (CF) is a multisystemic disease, with some patients developing end-stage liver disease (ESLD), requiring liver transplantation (LT). These children usually present with severe mutations of the CFTR gene. Almost 100% of patients with severe mutations develop exocrine pancreatic insufficiency, leading later to endocrine insufficiency. Immunosuppression accelerates the development of insulin-dependent diabetes (IDD) in transplanted children with CF. Our aims were: (1) to analyze our experience with CF-related ESLD children who received LT, and the relationship to the development of IDD; (2) to report our preliminary results with en bloc liver-pancreas transplantation (CLPT). METHODS: 9 children (6M/3F) with CF and ESLD underwent LT between 1993 and 2010; median age and weight were 12.3 years (range: 5.4-17.0) and 36.7 kg (range: 14.2-58.5), respectively. 4 patients received a whole graft, 4 had reduced grafts (1 split) and 1 underwent CLPT. Immunosuppression followed the protocols at the time of transplantation. RESULTS: Liver function was restored in all patients and none of them needed re-transplantation. Median follow-up was 105 months (range: 4-206). 1 child died of respiratory failure at 23 months after transplantation while awaiting pulmonary transplantation. Survival (Kaplan-Meier) at 105 months was 87.5%. 4 children already had IDD before transplantation and 3 developed diabetes immediately after transplantation. 2 had not developed IDD at the end of the study: the youngest at the time of LT (5.4 years, follow-up 7.1 years) and the girl who had had CLPT and who recovered normal exocrine and endocrine pancreatic function after transplantation. CONCLUSIONS: LT is a realistic option to treat CF-related ESLD children. IDD is common in these patients. En bloc liver-pancreas transplantation is an appealing option, since it simultaneously restores exocrine function and prevents IDD. This procedure has clear technical advantages over simultaneous isolated liver and pancreas transplantation.

Pseudoobstrucción intestinal idiopática crónica y síndrome de Berdon: todavía un desafío diagnóstico y terapéutico para el cirujano pediátrico / Chronic idiopathic intestinal pseudoobstruction and berdon syndrome: Still a diagnostic and therapeutic challenge for the pediatric surgeon

Introducción. La pseudoobstrucción intestinal idiopática crónica(POIIC) y el síndrome de Berdon (SB) son trastornos motores de etiopatogenia aún mal conocida, difícil diagnóstico y tratamiento. Pacientes/métodos. Se estudiaron retrospectivamente 26 pacientes (8H/18M) tratados en nuestro hospital por POIIC (21) o SB (5) desde 1982-2009 analizando aspectos clínicos, diagnósticos, terapéuticos y evolutivos. Resultados. El 77% debutaron en los primeros 3 meses de vida(5 tenían diagnóstico prenatal de megavejiga). Los principales signos/síntomas fueron la distensión abdominal (87%), episodios obstructivos recurrentes (70%) y malnutrición (60%), seguidos de vómitos, diarrea crónica, estreñimiento pertinaz y disfagia. Doce tenían afectación urológica (8 megavejiga, 8 RVU/ectasia); 2 asociaban arritmias, 1 sordera, 1 hidrocefalia y 5 mal rotación intestinal. El estudio radiológico fue compatible en todos (1 tenía afectación esofágica exclusiva, 6 presentaban micro colon, 3 dilataciones segmentarias y el resto dilatación generalizada). Se realizó manometría anorrectal en 12 (demostrando RIA+),esofágica en 9 (evidenciando aperistalsis en 4) y antroduodenal en 9(viendo patrón miopático en 4 y neuropático en 5). Las biopsias rectales por succión (16) y musculares (5) fueron normales en todos ellos;las de pared intestinal completa (en 18, tras cirugía) demostraron solo (..) (AU)

Aim/background. Chronic Intestinal Pseudo Obstruction (CIPO)and Berdon Syndrome (BS) are motility disorders with still unclear pathophysiology, and challenging diagnosis and management. Patient and methods. 26 patients (8M/18F) treated of CIPO (21)or BS (5) at our institution between 1982-2009 were retrospectivelyre viewed and clinical, diagnostic, therapeutic and follow-up data were analyzed. Results. 77% had a neonatal onset by the 3rd month of life (5had a prenatal diagnosis of megacystis). Abdominal distension (87%),recurrent suboclusive episodes (70%) and malnourishment (60%)were the main symptoms followed by vomits, chronic diarrhea, constipation and dysfagia. The urinary tract was involved in 12 patients(46%): 8 had megacystis, 8 had vesicoureteral reflux. Arrythmias were seen in 2, deafness in 1, hydrocephalia in 1 and mal rotation in5. Radiological studies were consistent for CIPO in all of them: the (..) (AU)

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

Objetivo. Analizar el beneficio del trasplante hepático (TH) con labipartición adulto-niño. Pacientes /métodos. 1) Análisis de la mortalidad pretrasplante calculadas sobre 228 inclusiones a TH (enero 2004-diciembre 2008).2) Impacto de las técnicas alternativas (donante vivo/bipartición) en la mortalidad pretrasplante de nuestros enfermos. 3) Análisis de los resultados de 33 biparticiones que dieron lugar a 66 trasplantes (1994-2008). Resultados. Referida por 1.000 enfermos y año de exposición, la mortalidad pretrasplante fue de 110 en niños mayores de 5 años, 180en niños de 2 a 5 años, 90 en niños entre 1 y 2 años, y 510 en menores de 1 año (p<0,05 respecto a restantes grupos). 36/66 injertos divididos fueron implantados por nuestro grupo. Cinco se perdieron, 3 por retrasplante, 2 por fallecimiento. La supervivencia actuarial a 10 años fue94,5% (enfermos) y 85,1% (injerto). Los 30 injertos restantes fueron trasplantados en otros hospitales, de los que 4 se perdieron precozmente. En el (..) (AU)

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 December2008. 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 than5 year old, 180 in children from 2 to 5 year-old, 90 in children between1 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 (..) (AU)

[Chronic idiopathic intestinal pseudoobstruction and Berdon syndrome: still a diagnostic and therapeutic challenge for the pediatric surgeon]. / Pseudoobstrucción intestinal idiopática crónica y síndrome de berdon: todavía un desafío diagnóstico y terapéutico para el cirujano pediátrico.

AIM/BACKGROUND: Chronic Intestinal Pseudo Obstruction (CIPO) and Berdon Syndrome (BS) are motility disorders with still unclear pathophysiology, and challenging diagnosis and management. Patient and methods. 26 patients (8M/18F) treated of CIPO (21) or BS (5) at our institution between 1982-2009 were retrospectively reviewed and clinical, diagnostic, therapeutic and follow-up data were analyzed. RESULTS: 77% had a neonatal onset by the 3rd month of life (5 had a prenatal diagnosis of megacystis). Abdominal distension (87%), recurrent suboclusive episodes (70%) and malnourishment (60%) were the main symptoms followed by vomits, chronic diarrhea, constipation and dysfagia. The urinary tract was involved in 12 patients (46%): 8 had megacystis, 8 had vesicoureteral reflux. Arrythmias were seen in 2, deafness in 1, hydrocephalia in 1 and malrotation in 5. Radiological studies were consistent for CIPO in all of them: the disease was limited to the esophagus in 1, 3 had segmentary involvement of the small bowel, 6 had microcolon, and the rest had all the digestive tract involved. Anorectal manometry ruled out aganglionosis in 12, esophageal manometry showed aperistalsis in 9 and antro-duodenal manometry confirmed the diagnosis in 9 (visceral myopathy in 4 y neuropathy in 5). Rectal biopsies (16) and muscular biopsies (5) were normal in all of them. Full thickness biopsies (in 18, after surgery) showed myopathy in 12 and neuropathy in 6. Prokinetics and antibiotics for bacterial overgrowth were employed in 100%, 17 required long-time parenteral nutrition (PN), 21 required surgery and 7 were transplanted (4 isolated small bowel, 3 multivisceral). Symptoms improved in 9/15 with an ileostomy. 19 weaned from PN. After a median follow-up of 7.9 years (range 5m-17a), 3 were lost, 17/23 patients are alive and only 2 on home PN. Six died, 3 after being transplanted. CONCLUSIONS: CIPO and/or BS have a wide clinical spectrum and a complex diagnosis; however, the knowledge of the disease and an appropriate treatment, generally aggressive, allows the patients with CIPO to enjoy a long-term acceptable quality of life.

[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.

[Liver bipartition as an alternative to the transplant]. / Bipartición hepática como alternativa en el trasplante.

AIM: Liver pediatric transplantation finds in the lack of donors its main limitation. An alternative in those cases is split liver grafts from bigger donors. PATIENTS AND METHOD: We performed a retrospective study of 56 hepatic split transplants performed between 1994 and 2007. Twenty-nine children were transplanted with a median age and weight of 1.8 years old (0.3-9) and 9.7 kg (6.2-23). In 16 cases (53.3%) liver transplant was performed in emergency situation. In one patient we performed a combined transplant (liver-kidney) and in another patient it was a second transplant due to primary graft failure after receiving an hepatointestinal allograft. Type of grafts used were: lateral left segment (n=26), extended lateral left segment (n=1) and extended right liver (n=3). Median donor age and weight were 20 years old (8-44) and 60 kg (24-80). We studied patient and graft survival (Kaplan Meier), perioperative factors, complications and net rate of early complications in adults recipients. RESULTS: Patient survival was 96.7% after 6 months, 1 year, 5 years and 10 years. Id for grafs 86.7%. Two grafts were lost due to arterial thrombosis, one due to primary non function and another due to recipient death secondary to a sepsis. Five children had major biliary complications and 2 of them developed multiple intrahepatic stenoses, one of them being on waiting list for retransplant. Early graft lost (retransplant or death before leaving the hospital) occurred in 4 out of the 25 grafts transplanted in other centers (25 adults, 1 kid); all of them occurred in the initial period (1994-2001). CONCLUSIONS: Even though it is clearly documented that benefit of transplant (measured in years of life won) is very good after split transplantation, nowadays criteria for organ allocation in Spain do not allow a more extensive diffusion of this technique and it is confined to urgent transplant. Even in those cases, results after split transplantation are excellent. Without this possibility our pretransplant mortality would be much higher.

Bipartición hepática como alternativa en el trasplante / Liver bipartition as an alternative to the transplant

Objetivo. El trasplante hepático pediátrico, encuentra en la falta de donantes su principal limitación. Una alternativa en estos casos es recurrir a la bipartición (split) de hígados procedentes de donantes de mayor peso. Pacientes y método. Análisis retrospectivo de 56 trasplantes hepáticos con injerto procedente de bipartición entre 1994-2007. Fueron implantados a 29 niños con una mediana de edad y peso de 1,8 años (0,3-9,0) y 9,7 Kg (6,2-23). En 16 casos (53,3%) el TH fue realizado en situación de emergencia. En un caso el trasplante fue combinado (hígado riñón) y en otro se trataba de un retrasplante por fallo primario del injerto hepático tras haber recibido un aloinjerto hepatointestinal. El tipo de injerto fue: segmento lateral izquierdo (n = 26), segmento lateral izquierdo extendido (n = 1), hígado derecho extendido (n = 3). La mediana de (..) (AU)

Aim. Liver pediatric transplantation finds in the lack of donors its main limitation. An alternative in those cases is split liver grafts from bigger donors. Patients and method. We performed a retrospective study of 56hepatic split transplants performed between 1994 and 2007. Twenty-nine children were transplanted with a median age and weight of 1.8 years old (0.3-9) and 9.7 kg (6.2-23). In 16 cases (53.3%) liver transplant was performed in emergency situation. In one patient we performed a combined transplant (liver-kidney) and in another patient it was a second transplant due to primary graft failure after receiving an hepatointestinal allograft. Type of grafts used were: lateral left segment (n=26), extended lateral left segment (n=1) and extended right liver (n=3). Median donor (..) (AU)

Variant techniques for liver transplantation in pediatric programs.

INTRODUCTION: Several variant techniques have been developed as alternatives to whole liver transplantation to improve size matching, timing, or simply to increase the pool of donors. The aim of this study was to assess the requirements of these techniques and their outcomes in a pediatric transplant program. PATIENTS AND METHOD: A retrospective analysis of children on the waiting list in the last 4 years was carried out. Data of patients who died while on the waiting list (WL) were recorded. Transplanted patients were divided according to the type of graft: whole liver, split, living donor and reduced liver. The analyzed outcome variables were: age, weight, UNOS status, cause of liver failure, complications and graft and patient survival. Comparisons between types of graft were performed by using Kaplan-Meier, log-rank, chi (2) and Kruskal-Wallis tests. RESULTS: During the period studied, 116 children were listed for liver transplantation. Of these 116 children, nine (7.7 %) died after a mean period of 40.5 (5-175) days waiting for a suitable graft. Their median age at inclusion was 214 (35-1607) days, and median weight was 7.2 (12.3-3.6) kg. The cause of liver failure in this group was: 1 hemochromatosis, 1 hepatoblastoma, 2 biliary atresia, 2 acute liver failure, 2 primary non-function (PNF) and 1 chronic rejection. Liver transplantation was performed in 103 children: 25 (24 %) whole livers, 17 (16.5 %) split, 29 (28 %) living donor, 32 (31 %) reduced and 4 remain on the waiting list. Recipient age and weight were significantly lower in those receiving split and living donor than in those who given whole livers. Patient and graft survival were similar in all groups although there was a trend to lower graft survival in patients receiving whole livers. More than 50 % of patients with UNOS status I received a split graft and 5/6 children with hepatoblastoma underwent living donor transplantation. There were no differences in the rate of acute vascular complications, but long-term biliary complications were more frequent in split and living donor grafts. CONCLUSIONS: As long as the goal of zero mortality for children on the waiting list is not achieved, variant techniques will be necessary in pediatric liver transplantation programs. Split and living donor were employed mostly to treat younger children and particularly those with a higher UNOS status. Children with tumors were treated mainly with living donor grafts. Variant techniques, which are absolutely necessary in a pediatric program, need to be improved in order to avoid long-term biliary complications.

Long-term results of the treatment of total colonic aganglionosis with two different techniques.

AIM: Aim of this study was to assess the long-term results of the treatment of total colonic aganglionosis (TCA) with 2 different techniques in terms of growth, continence and quality of life (QOL). PATIENTS AND METHODS: Forty-one patients treated for TCA between 1972 and 2007 were reviewed retrospectively with special attention paid to the length of aganglionosis, complications, growth, continence and QOL. Until 1992, patients underwent subtotal colonic resection and side-to-side ileosigmoid anastomosis (modified Martin). Since 1992, straight ileo-anal pull-through was preferred. At the end of follow-up (median 18 years, range 1-35), the height and weight, continence and QOL (scoring feeding habits, school/work performance, family life and professional development) were assessed by clinical visit or phone interview. RESULTS: Twenty-eight patients were male and 13 female. Six had total intestinal aganglionosis and were excluded from this review together with the 2 who died before definitive treatment. The 33/41 persons in whom aganglionosis had involved less than 50 cm above the ileocecal valve and who had been considered suitable for the reestablishment of transanal fecal flow were included. Eighteen children underwent a modified Martin and 15 straight ileo-anal pull-through. Postoperative intestinal obstruction occurred in 4 cases, prolapse and prolonged TPN requirement in 2, and wound disruption and fistula in 1. Thirteen patients (39 %) had postoperative enterocolitis. Two children died after operation (1 wound disruption with sepsis and 1 pneumonia). Out of 31 survivors, 57 % and 53 % were > p50 with regard to height and weight whereas only 15 % and 19 % were

[Morbility and mortality associated with prosthetic patch in congenital diaphragmatic hernia]. / Morbimortalidad en pacientes con parche protésico para el cierre de la hernia diafragmática congénita.

AIM: To analyze morbility, mortality and neonatal intensive care management in CDH patients who required a prosthetic patch to close the diaphragmatic defects, and to compare these results with those who were made a primary closure. MATERIAL AND METHODS: We reviewed the clinical charts of CDH patients managed at our institution between January 1994 and December 2006, including demographic data, clinical management, treatment options, complications and mortality. Appropriate statistical tests were used to evaluate the data: mortality, need of high frequency oscillatory ventilation (HFOV), days of intubation, days of total parenteral nutrition (TPN), days of admission, reherniation, need of Nissen funduplication and intestinal obstruction; a p value less than 0.05 was considered statistically significant. RESULTS: Eighty-seven CDH patients were reviewed, 68 right, 17 left and 2 bilateral defects. Seventeen died before surgery (19.5%). Among the 70 operated patients, 21 (24.1%) required a prosthetic patch, 18 in the left and 3 in the right side. Overall mortality, need of HFOV, intubation days, need of TPN, days of admission, reherniation rate and need of Nissen funduplication were all significantly worse in the group who required a patch. Rate of intestinal obstruction was similar in both groups. CONCLUSIONS: Patients who required a prosthetic patch to close the diaphragmatic defect suffered from higher morbidity and presented a higher mortality than those who did not required the patch.

Morbimortalidad en pacientes con parche protésico para el cierre de la hernia diafragmática congénita / Morbility and mortality associated with prosthetic patch in congenital diaphragmatic hernia

Objetivo. El objetivo de nuestro estudio es analizar la asistencia intensiva neonatal y la morbimortalidad en pacientes con hernia diafragmática congénita (HDC) en quienes se requirió parche protésico y compararlas con la de aquellos en quienes se practicó cierre diafragmático primario. Material y métodos. Estudio retrospectivo mediante revisión de historias clínicas de los pacientes con HDC intervenidos en nuestro hospital entre enero de 1994 y diciembre de 2006 incluyendo demografía, clínica, intervenciones, tratamiento, complicaciones y mortalidad. Se utilizaron test estadísticos para evaluar las diferentes variables: mortalidad, uso de ventilación con alta frecuencia oscilatoria (VAFO), días de intubación, días de nutrición parenteral, días de estancia hospitalaria, reherniación, funduplicatura y obstrucción intestinal, considerando en todas ellas una diferencia estadísticamente significativa cuando p<0,05.Resultados. Revisamos 87 pacientes con HDC, de los que 17 fallecieron antes de la cirugía (19,5%), 68 eran izquierdas, 17 derechas y 2bilaterales. De los 70 intervenidos, se usó un parche protésico durante el cierre del defecto en 21 pacientes (24,1%), 18 en el lado izquierdo y 3 en el derecho. Se analizaron parámetros como la mortalidad global y por grupos de los pacientes con parche protésico y sin él, siendo peor en los del primer grupo, parámetros ventilatorios como la utilización de ventilación con alta frecuencia y los días de intubación, que fueron mayores en los pacientes con defectos grandes. El uso de nutrición parenteral prolongada y la estancia media también fue más prolongado en los pacientes con parche diafragmático. Las complicaciones como la reherniación también fue más frecuente en los pacientes con parche protésico, sin embargo no se encontraron diferencias significativas con respecto a la obstrucción intestinal en ambos grupos. El reflujo gastroesofágico severo y la necesidad de funduplicatura para su corrección estuvo presente con más frecuencia en los pacientes con grandes defectos. En todos los resultados, excepto en la obstrucción intestinal, se encontraron diferencias estadísticamente significativas. Conclusiones. Los pacientes que precisan la colocación de una parche protésico para el cierre diafragmático presentaron una mayor mortalidad, requirieron más asistencia ventilatoria y nutritiva y sufrieron mayor morbilidad respiratoria y digestiva. La frecuencia de reintervenciones quirúrgicas (reherniación, y funduplicatura) fue más alta en los niños que precisaron prótesis (AU)

Aim. To analyze morbility, mortality and neonatal intensive care management in CDH patients who required a prosthetic patch to close the diaphragmatic defects, and to compare these results with those who were made a primary closure. Material and methods. We reviewed the clinical charts of CDH patients managed at our institution between January 1994 and December2006, including demographic data, clinical management, treatment options, complications and mortality. Appropriate statistical tests were used to evaluate the data: mortality, need of high frequency oscillatory ventilation (HFOV), days of intubation, days of total parenteral nutrition(TPN), days of admission, reherniation, need of Nissen fundoplication and intestinal obstruction; a p value less than 0.05 was considered statistically significant. Results. Eighty-seven CDH patients were reviewed, 68 right, 17left and 2 bilateral defects. Seventeen died before surgery (19.5%).Among the 70 operated patients, 21 (24.1%) required a prosthetic patch,18 in the left and 3 in the right side. Overall mortality, need of HFOV, intubation days, need of TPN, days of admission, reherniation rate and need of Nissen fundoplication were all significantly worse in the group who required a patch. Rate of intestinal obstruction was similar in both groups. Conclusions: Patients who required a prosthetic patch to close the diaphragmatic defect suffered from higher morbidity and presented a higher mortality than those who did not required the patch (AU)

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.

[Pediatric patient in adult age. Long-terms results of esophageal replacement]. / El paciente pediátrico en la edad adulta: revisión a largo plazo de los resultados de la sustitución esofágica.

BACKGROUND: Esophagocoloplasty is one of the most used procedures for esophageal replacement in children. Considering high life expectancy in these patients, long-term results must be considered when evaluating this technique. The aim of our study is to evaluate quality of life of adult patients who underwent surgery at pediatric age. PATIENTS AND METHODS: We report a retrospective study of 99 patients who underwent esophageal replacement in our institution between 1966 and 2006. Eight of them have died and 63 out of the remaining 91 are over 18 years now and represent our study serie. Long-term results and actual situation of those patients, considering psychological, physic and social aspects, were evaluated through clinical review and telephonic interview. Karnofsky index was applied to mesure functional ability from 0-100% (bad, medium, good-excellent) according to the answers the patients gave to our questions. We also recorded their health personal experience and subjective evaluation of their quality of life. RESULTS: Sixty-three patients were reviewed (43 males and 20 females) with a mean age of 4.3 +/- 3.4 D.S. Mean follow-up time was 29.6 +/-7.7 years. Indications for esophageal replacement were as follows: caustication (n = 32), type III esophageal atresia (n = 15), type I AE (n = 13) and others (n = 3). In 48 patients the graft was placed in retroestenal position and in 15 cases retromediastic location was used. Postoperative period was uneventful in 44% of the patients, being the most frequent early complications in the remaining, cervical leakage and stenosis. Long-term, 56,8% did not have any sequelae, 28.5% required further surgery and the remaining 43.13% presented the following complications: symptomatic graft reflux (22), scoliosis and thoracic asymmetry (12), colonic redundancy or cervical diverticulum (7), food impaction (6) and failure to thrive (5). Only one 38 year old patient does not have intestinal tract continuity nowadays. Thirty-one patients have a Karnofsky index > or = 80-100%, being considered healthy and able to have a normal activity. Eighteen patients are included between 40-80%, being the most frequent limitation the need of medication to avoid reflux, backache and occasional episodes of food impact. Only 2 patients have Karnofsky index inferior to 40%. None of them are under 20%. CONCLUSIONS: Esophagocoloplasty allows restoration of intestinal tract continuity in almost all cases and the mortality of this procedure has decreased over time. Even though some risks are still remarkable, it offers long-term good results with little repercussion on functional ability in adult age. Most of the patients consider themselves healthy and enjoy an acceptable quality of life.

Sternal cleft associated with enterogenous cyst treated during the newborn period.

We present the case of a newborn with sternal cleft (SC) and presternal enterogenous cyst operated on during the neonatal period. SC is an uncommon congenital malformation of the thoracic wall which can occur as an isolated form or in association with other malformations. To our knowledge, the presence of SC and enterogenous cyst has not been described to date. Early surgical repair of SC gives good aesthetic and functional results and is usually the preferred approach.