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Aim: Sarcopenia is associated with poor prognosis in adult oncologic patients, with little evidence of this association in pediatric population, including hepatoblastoma. Methods: Retrospective study in patients with hepatoblastoma, divided into those with or without sarcopenia. Sarcopenia was assessed by measuring psoas muscle area (PMA) at L4-L5 level on the CT/MR and defined as z-score values ≤2. Relapse and mortality were analyzed. Results: Twenty-one patients (57.1% male) were included, with median age 35.7 months (IQR: 23.5-58.5). Seven (33.3%) had sarcopenia on initial studies compared to 14 (66.7%) who did not. No differences were found between groups in age, weight, PRETEX, surgical treatment or a-fetoprotein levels. Twelve (57.1%) underwent liver resection and 9 (42.9%) liver transplantation. Sarcopenia was associated with a higher rate of metastases at diagnosis (49.2% vs 0.0%; p=0.026) and surgical complications (57.1% vs 21.4%, p=0.047). After a median follow-up of 65.1 months (1.7-144.8), 2 patients (28.6%) had tumor relapse in sarcopenic group compared to 1 (7.1%) in non-sarcopenic group. Two patients died in sarcopenic group and 1 in non-sarcopenic group. Median event-free survival (EFS) was lower in sarcopenic group (100.38±25.63vs118.91±11.52 months) as well as overall survival (OS) (101.72±24.86 vs 121.78±8.75 months) with no statistical significance. Five-year EFS was also lower in sarcopenic group (71%vs93%) as well as 5-year OS (71%vs87%). Conclusions: Sarcopenia at diagnosis was associated with a higher rate of metastases and surgical complications in hepatoblastoma. Our data shows the first evidence of its role as a possible poor prognostic factor, influencing survival and risk of relapse.

CD19-directed immunotherapies have revolutionized the treatment of advanced B-cell acute lymphoblastic leukemia (B-ALL). Despite initial impressive rates of complete remission (CR) many patients ultimately relapse. Patients with B-ALL successfully treated with CD19-directed T cells eventually relapse, which, coupled with the early onset of CD22 expression during B-cell development, suggests that preexisting CD34+CD22+CD19− (pre)-leukemic cells represent an “early progenitor origin-related” mechanism underlying phenotypic escape to CD19-directed immunotherapies. We demonstrate that CD22 expression precedes CD19 expression during B-cell development. CD34+CD19−CD22+ cells are found in diagnostic and relapsed bone marrow samples of ∼70% of patients with B-ALL, and their frequency increases twofold in patients with B-ALL in CR after CD19 CAR T-cell therapy. The median of CD34+CD19−CD22+ cells before treatment was threefold higher in patients in whom B-ALL relapsed after CD19-directed immunotherapy (median follow-up, 24 months). Fluorescence in situ hybridization analysis in flow-sorted cell populations and xenograft modeling revealed that CD34+CD19−CD22+ cells harbor the genetic abnormalities present at diagnosis and initiate leukemogenesis in vivo. Our data suggest that preleukemic CD34+CD19−CD22+ progenitors underlie phenotypic escape after CD19-directed immunotherapies and reinforce ongoing clinical studies aimed at CD19/CD22 dual targeting as a strategy for reducing CD19− relapses. The implementation of CD34/CD19/CD22 immunophenotyping in clinical laboratories for initial diagnosis and subsequent monitoring of patients with B-ALL during CD19-targeted therapy is encouraged., The work in P.M. and C.B.’s Laboratory was supported by the European Research Council (CoG-2014-646903, PoC-2018-811220) and the Spanish Ministry of Economy and Competitiveness (SAF2016-80481R, PID2019-108160RB-I00) (P.M.); the ISCIII (ISCIII/FEDER, PI17/01028 and PI20/00822), the Spanish Association against Cancer (AECC), and the FERO Foundation (C.B.). P.M. and J.M.R. acknowledge the support of ISCIII-RICORS within the Next Generation EU program (Plan de Recuperación, Transformación y Resiliencia). N.E. is supported by Cancer Research UK and a Children and Young People’s Cancer Innovation Award (DRCPGM\100058). A.R. is supported by a Wellcome Trust Clinical Research Career Development Fellowship (216632/Z/19/Z). A.O. was supported by ISCIII (PI19/011183). F.S. was supported by AGAUR/Generalitat de Catalunya (SGR288). The single-cell transcriptomic analysis was supported by MRC Discovery award MRCDA 0816-11 and the MRC WIMM Single Cell Facility and MRC-funded Oxford Consortium for Single-Cell Biology (MR/M00919X/1) provided assistance.

Introduction There is a lack of an agreed Hirschsprung-associated enterocolitis (HAEC) definition. In 2009, a HAEC score was proposed for the diagnosis of HAEC episodes. Our aim was to apply the HAEC score on HAEC episodes to determine its diagnostic efficiency and whether it correlated to its severity. Methods Retrospective study of patients with HAEC admitted between 2000 and 2016. Episodes of HAEC were identified and the HAEC score was calculated. A cut-off of ≥ 10 according to Pastor et al and ≥ 4 according to Frykman et al were used. A Pearson's correlation coefficient was performed for outcome variable: length-of-stay (LOS). Results Note that 21/93 (22.6%) patients with Hirschsprung's disease presented 50 HAEC episodes with a median of 2 (1–5) episodes during an 8.3-year (2–15.6) follow-up. The most common symptoms were foul-smelling (86% [43/50]) and explosive (60% [30/50]) diarrhea. Physical findings showed a distended abdomen (76% [38/50]) and fever (60% [30/50]) with dilated bowel (82% [41/50]) and rectosigmoid cut-off (80% [40/50]) identified on X-rays. Only 34% (17/50) showed abnormal laboratory findings. Patients were admitted with a median LOS of 7 days (1–28). A HAEC score of 9 (2–16) was found, and only 50% (25/50) of patients met the initial criteria (score of 10 points). However, the use of a 4-point cut-off would have allowed the diagnosis of 98% (49/50) of the patients. A positive linear correlation was found for LOS and HAEC score (r = 0.3, p = 0.014). Conclusion There is no standardized definition for HAEC. The initial HAEC score cut-off is restrictive and might fail to identify milder episodes. The positive correlation with LOS and thus HAEC severity might aid in patient information and anticipation of treatment.

Introduction Recurrence of ileocolic intussusception (ICI) has been related to residual bowel wall edema after enema reduction. Early oral tolerance has been associated with a higher risk of re-intussusception, so an imaging test (ultrasound) has traditionally been performed before restarting oral tolerance. Our aim is to analyze the cost-effectiveness of performing a routine ultrasound in patients who remain asymptomatic after successful enema reduction. Materials and Methods A retrospective observational study was performed in patients with ICI who underwent a successful enema reduction between 2005 and 2019 and distributed in two groups according to whether or not a routine ultrasound was performed before restarting oral tolerance: group A (ultrasound) or B (no ultrasound). We analyzed demographic, clinical and laboratory variables, length of hospital stay, and recurrence rate. Results We included 366 patients who presented 373 ICI episodes (165 in group A and 208 in group B), without significant differences in gender and age. Group A patients presented a higher percentage of vomiting and bloody stools than those in group B without differences in the other clinical features studied, time of evolution, or laboratory variables. Group A presented a higher length of hospital stay than group B (36 vs. 24 hours), although it was not statistically significant (p = 0.30). No statistically significant differences were observed in the recurrence rate between both groups (10.3% A vs. 10.8% B; p = 0.83). Conclusion Performing routine ultrasound before restarting oral tolerance in asymptomatic patients after successful ICI reduction does not decrease the risk of re-intussusception and should not be routinely encouraged.

Objective One of the principal objectives in treatment of facial nevus is to minimize psychological damage and encourage the child's schooling by the best possible cosmetic result. There are several therapeutic techniques: grafts, flaps, dermal regenerator, and tissue expanders. Materials and Methods We reviewed 10 patients with facial nevus higher than 10 cm treated in the past 8 years. Our treatment protocol includes serial expander implant to remove everything that does not involve the eyelid and nasal pyramid. Ten patients were included, between 8 and 36 months of age. A median of 4 (2–6) surgeries were conducted, and the number of implanted expanders was 1 to 3 in each session. There were only minor complications in two patients, infection that responded to antibiotic therapy and minimal dehiscence of incision forcing resuturing. The median follow-up was 1.6 (1.3–3.4) years. Conclusion Tissue expansion has become in recent years the treatment of choice for facial nevus in children, obtaining the best aesthetic result because the defect is covered with similar characteristics in color, texture, and relief skin. It is essential that the reconstruction is done by specialized units and we recommend complete surgical excision before starting school.

Introduction Central venous port (CVP) placement is traditionally performed under fluoroscopy guidance. However, efforts for radiation dose reduction in children have allowed the introduction of ultrasound guidance (USG) and anatomic landmarks as an alternative technique for CVP placement. The aim of this study is to determine whether intraoperative fluoroscopy (IF) is required to confirm the correct position of the catheter tip in children. Patients and Methods A prospective, single-center study was performed between July and December 2017. Standard venous access site was the right internal jugular vein under USG. Estimated catheter length (ECL) was measured using anatomic landmarks. Ideal catheter length (ICL) was measured after placement under fluoroscopy guidance in the same patient. Age, sex, radiation dose, and complications were also analyzed. A t-test for paired samples and intraclass correlation coefficient were performed to analyze results. Results A total of 30 consecutive patients aged 7 ± 2 years underwent CVP placement. The mean ECL was 17.1 ± 1.8 cm, while the mean ICL was 17.7 ± 1.8 cm. The mean difference between measurements was 0.28 cm (95% confidence interval [CI], –0.29 to 0.86; p = 0.324). Intraclass correlation coefficient analysis showed an agreement of 0.95 (95% CI, 0.91–0.98) between measurements. Mean radiation exposure during the procedure was 1.060.78 mGym2 during 0.34 ± 0.6 minutes. There were no complications registered during CVP placement. Conclusion The correlation between IF and USG and anatomically guided catheter tip placement is optimal. These results suggest that fluoroscopy and the radiation exposure it entails can be safely avoided in selected children.

Aim of the Study Severe lung hypoplasia and persistent pulmonary hypertension are the main determining factors of survival in infants with congenital diaphragmatic hernia (CDH). The oxygenation index (ratio of delivered oxygen and its arterial level) closely reflects lung function. Single-institution studies have reported that best oxygenation index on day 1 of life (BOI-d1) is the most reliable postnatal predictor of survival in CDH. The aim of this study was to evaluate the predictive value of BOI-d1 in four disparate high volume centers in Europe. Methods A retrospective, multicenter study of infants with CDH born between 2000 and 2009 in four European tertiary institutions was conducted. Ethical approval was obtained from institutional review boards. Centers no. 1 and. 4 offered extracorporeal membrane oxygenation (ECMO), whereas center no. 3 offered fetal endoluminal tracheal occlusion (FETO) in fetuses defined as poor prognosis (lung-to-head ratio [LHR] ≤ 1.0 and “liver-up” position). Prenatal LHR and perinatal variables, including gestational age, birth weight, defect side, liver position, BOI-d1, and patch requirement, were analyzed. Receiver operating characteristic curves were used to determine cutoff values for continuous variables. Comparison was made between survivors and nonsurvivors using univariate analysis and logistic regression analysis, p Results A total of 235 infants (center no. 1, n = 29; no. 2, n = 64; no. 3, n = 113; and no. 4, n = 29) were included. One infant required (2%) ECMO and 66 (28%) had FETO. LHR was available in 83 patients (36%). Overall survival (discharge from hospital) and 28-day survival were 67.6% (n = 159) and 72.3% (n = 170), respectively. Univariate analysis showed that significant categorical predictors of 28-day survival were liver-down position (p 1 (p = 0.003), and primary repair (p = 0.02) but not defect side (p = 0.83). Area under the receiver operating characteristic (AUROC) curve for continuous variables; gestational age, birth weight, and BOI-d1 were 0.70, 0.68, and 0.88, respectively. AUROC for BOI-d1 (28-day survival) was 0.91 and had sensitivities (73 and 91%) and specificities (92 and 80%) for cutoffs of 40 and 82, respectively. Conclusion This multicenter study showed, that except from the defect side, all the prenatal variables studied have predictive value but the most useful is BOI-d1. This is simple to calculate and represents an excellent marker for lung function and a reliable early postnatal predictor of survival.

Nissen fundoplication (NF) is a procedure with technical difficulties and variable functional prognosis the lower the patient's age is. Our objective is to analyze the peculiarities of this procedure when performed in children under 1 year.Retrospective study of the NF in our center from 1999 to 2014. We review the differences between children under 1 year of age and the leftover of the series: history, indications, surgical approach and postoperative outcomes.A total of 233 patients (57.1% male) were operated at a median age of 2.3years (1 month-17.31years), of which 82 (35.2%) were younger than 1 year. It Open surgery was performed in 118 patients (86.6% of children under 1 year and 31.1% over 1 year, p0.05) and laparoscopic in 115. The median follow-up was 3.92 ± 3.24 years. Patients under 1 year had a higher number of comorbidities (91.5% vs 81.5%), respiratory symptoms (76.8% vs 49.7%) and postoperative complications (20.7% vs 9.9% OR = 2.4), with statistically significant differences (p0.05). There were not differences in the Nissen's failure rate (15.9% vs 8.6%) or the need of reoperation (15.9% vs 7.9%).Patients under 1 year operated by NF form a group with particular indications and comorbidities. Although the outcomes among these patients are favourable, surgical complications are more frequent than in older children.La funduplicatura de Nissen (FN) es un procedimiento que plantea dificultades técnicas y un pronóstico funcional variable cuanto menor es la edad del paciente. Nuestro objetivo es analizar las peculiaridades de esta intervención cuando se realiza a niños menores de 1 año.Estudio retrospectivo de las FN en nuestro centro de 1999 a 2014. Se evaluaron las diferencias entre menores de 1 año de vida y el resto de la serie en cuanto a antecedentes, indicaciones, abordaje quirúrgico y evolución postoperatoria.Un total de 233 pacientes (57,1% varones) fueron intervenidos a una mediana de edad de 2,3 a (1 mes-17,31 a), de los que 82 (35,2%) eran menores de 1 año. La cirugía fue abierta en 118 pacientes (86,6% de los menores de 1 año y 31,1% de los mayores de 1 año, p0,05) y laparoscópica en 115. La mediana de seguimiento fue de 3,92 años. Los pacientes menores de 1 año presentaron mayor número de comorbilidades (91,5% vs 81,5%), sintomatología respiratoria (76,8% vs 49,7%) y complicaciones postoperatorias (20,7% vs 9,9%, OR=2,4), siendo estas diferencias estadísticamente significativas (p0,05). No hubo diferencias en cuanto al fallo del Nissen (15,9% vs 8,6%) ni a la necesidad de reintervención (15,9% vs 7,9%).Los pacientes menores de 1 año operados mediante FN constituyen un grupo con indicaciones y comorbilidades particulares. Aunque los resultados son favorables, las complicaciones quirúrgicas son más frecuentes.

Introduction Ganglioneuroma (GN) is a benign, differentiated variety of neurogenic tumor. It is often asymptomatic and may be diagnosed by serendipity. Surgical removal is the treatment of choice. However, it has been suggested that postoperative complications and sequelae might outweigh the benefits of this approach. The purpose of the present study was to examine these issues in a large experience of neural tumors. Methods Patients treated between 1992 and 2012 were retrospectively reviewed. Modern imaging, measurement of catecholamine metabolite excretion and metaiodobenzylguanidine were used for workup. Surgical treatment aimed at complete resection. Complications and sequelae were recorded. Literature was searched for regrowth or malignant transformation of GN. Results Of 227 patients with neural tumors, 24 were GN patients (12 abdominal, 11 thoracic and 1 cervical with 8 dumbbell extensions). Six children were symptomatic (three with abdominal pain and mass, one with stridor or dysphonia, and one each with anisocoria and opsomyoclonus). However, 18 (75%) were asymptomatic and the diagnosis was incidental. Several tumors were large and involved more than one body space. There were no neurologic symptoms in eight cases with dumbbell extension. Complete resection was achieved in 20 children (83%) whereas gross residual was left in four. Postoperative complications were: Horner syndrome (3 patients), mild scoliosis (1 patient), adhesive bowel obstruction (1 patient) and acute urinary retention (1 patient). There was no evidence of either regrowth or malignant behavior in residual masses left in place after follow-up of 84 (1–194) months. Conclusions There were a limited number of general minor complications in this series that did not include cases of regrowth or malignant transformation. However, these unfavorable events were occasionally reported in the literature. Since diagnosis of GN cannot be ascertained before removal of the mass, this should remain the aim of the treatment, although limiting the chances of complications to a minimum even if incomplete resection is the price to pay. Nonoperative attitudes should not be recommended in all cases, but they are certainly justified in some.

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.

Background/aim Surgery for neural tumors occasionally involves opening of the neural canal and/or costovertebral resection that may be followed by radiotherapy. Spinal deformities (SD) may occur in these cases. This study investigated the incidence, surgical indications and results of repair of late SD in a large series of patients with neural tumors. Methods A retrospective review of neural tumors treated between 1991 and 2009 at our institution identified children with SD who subsequently required spinal surgery. SD was meaÂ-sured using Cobb's angle. Instrumentation of the spine was indicated in patients with a Cobb's angle of more than 60° or rapidly progressing SD. The operation was adapted to the location, type and degree of SD. Patients were followed up and the remaining deviations were measured annually. Results Out of a series of 188 patients with neural tumors, 5 of 173 patients with neuroblastomas/ganglioneuromas (NB/GN) and 3 of 15 patients with PNET/Askin tumors developed SD over time. NB/GN children had intraspinal tumors requiring laminotomy (n=3) or laminectomy (n=2). PNET/Askin tumor patients had undergone largescale rib and partial vertebral body resection and radiotherapy. Scoliosis or kyphosis were located in the thoracic spine in 4, in the thoracolumbar spine in 3 and in the cervicothoracic spine in 1. The median preoperative Cobb's angle was 65° (50°-90°) prior to spinal surgery performed 2.9 (0.4-11) years after tumor removal. 3 growth bars, 2 vertical expandable prosthetic titanium ribs, 1 pediatric ISOLA device and anterior instrumentation were used. Arthrodesis was necessary in 3 patients. Median post-instrumentation deformity after 3.1 (0.2-12.2) years was 40° (20°-50°), giving an improvement of 38.4%. Conclusions With increasing numbers of children surviving cancer, attention must focus on sequelae. A sizeable number of survivors of NB/GN and PNET/Askin tumors develop serious SD requiring surgical correction. Pediatric surgeons, patients and relatives must be aware of these possible sequelae.

AIM Aim of the study was assess the results of the treatment of High-Risk Hepatoblastoma (HRH) in a tertiary center where all liver surgery facilities, including pediatric transplantation (LT), are available. METHODS 91 primary liver tumors treated between 1991 and 2009 were retrospectively reviewed. HRHs as defined by the SIOP criteria (PRETEXT IV or any stage with venous involvement, extrahepatic disease, tumor rupture and

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.

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.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.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 (p0.05).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.

Reconstructive surgery of the airway often means prolonged periods of intubation during the post-operatory period, increasing the needs for drugs and favoring the appearance of infectious complications. We present an original system of ventilatory support with non-invasive positive pressure ventilation (NIPPV) using in patients subjected to reconstructive surgery of the airway.A retrospective study in patients undergoing reconstructive procedures of the airway in the year 2009 was carried out. We exclude those treated endoscopically and those who had vascular rings. The positive pressure mechanism used in the Surgery Critical Care Unit was a design made by the unit based on the circuit devised by Mapleson that provides optimum levels of ventilation without need for connection to a respiratory. We analyze the results, postoperatory intubation time, time dependent on NIPPV and medical treatment received.A total of 7 patients (1 Female and 6 Males) with median age of 1.6 (0.1-7.5) years were included. The diagnoses were: 4 subglottic stenosis, 2 had tracheal stenosis and 1 subcarinal stenosis with involvement of both principal bronchioles. The techniques used were: laryngotracheoplasty with costal cartilage graft (4), tracheoplasty with costal cartilage (1) and sliding tracheoplasty (2) with bilateral bronchoplasty in one of them. The mean time of nasotracheal intubation was 3 days, and mean time of NIPPV was 2.3. No patient required reintubation and none had infectious complications.Ventilatory support by VPPNI allows effective extubation in these patients, it being possible to maintain a safe airway. Infectious complications, frequent in prolonged intubations, were not observed in any of the cases.

Bean or Blue Rubber Bleb Nevus Syndrome (BRBNS) is an uncommon disease characterized by multifocal venous malformations that predominantly affect the skin, soft tissues and gastrointestinal tract. Our objective is to familiarize the pediatric surgeon with this condition, promoting early diagnosis and seriated surgical treatment as therapeutic options. We have reviewed the diagnosis and treatment of 6 patients treated from 1993 to 2009.All the patients had a venous malformation (10 cm) that acted as a marker. After, characteristic skin lesions appeared as well as chronic anemia due to digestive bleeding associated to hypofibrinogenemia. Age of appearance of the anemic picture varied from 6 months to 7 years, it being more aggressive the earlier its appearance. The diagnosis of the disease was made by endoscopically. All the patients required transfusion with blood derivatives 5 to 25 times a year. Treatment was multidisciplinary: periodic transfusions in every case, endoscopic sclerosis in lesions that affect the colon or stomach and surgical treatment for lesions of the jejunum and ileum. In two of these patients, 23 and 46 lesions were excised, respectively. In the last year, the tendency has been to use enterotomy and fulguration with Argon.The patients who have undergone excision of the digestive malformations have experienced a significant decrease in their transfusion needs. One female patient with miliary dissemination died.BRBNS is a difficult to diagnose due to its low frequency. Treatment of gastrointestinal tract lesions is multidisciplinary, combining endoscopic and surgical techniques that decrease transfusions and improve the quality of life of the patients.

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.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.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.

Introduction: Gastric pull-up has become the predominant technique for esophageal replacement because of allegedly deficient results of colon grafts. This retrospective study examines the long-term results in a large series of colonic interpositions. Patients and Method: One hundred six children (median, 2.9 years; range, 0.32-15 years) had their esophagus replaced between 1965 and 2008, of which 96 had colon grafts. Those survivors who were 18 years and older were contacted and, if willing, interviewed, examined, and had their functional ability rated using the Karnofsky index. Results: Ninety-six children had undergone a colon graft, and of these, 9 (9.3%) died. There were 65 long-term survivors whose indications for surgery included caustic injury (n = 32), failed tracheoesophageal fistula repair (n = 16), pure esophageal atresia (n = 14), and others (n = 3). The graft was either retrosternal (n = 49) or mediastinal (n = 16 patients). Twelve patients were unavailable for long-term assessment. After a median follow-up of 33.3 (11-41) years, 23 (43%) of 53 individuals experienced mild symptoms of reflux; scoliosis, 12 (22%) of 53, and/or other complications, 15 (27%) of 53 required further surgery. Thirty-two patients (60%) with Karnofsky indexes of 80% or higher felt healthy, 19 (36%) had mild life-style limitations (index, 40%-80%), and 2 had indexes less than 40%. Most patients live normal lives. Conclusions: Colon conduits restored gastrointestinal continuity with limited mortality and considerable morbidity but good functional outcome and health perception in the long-term. Our study suggests that colon grafts are no worse than gastric pull-ups in the long-term.

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

In this report the history and main activities of the Grupo de Estudios Peirceanos at the University of Navarra is presented. It was created in 1994 to promote the study of the work of Charles S. Peirce (1839-1914), especially in Spain and in the Spanish-speaking countries. An outstanding result of the work of this group is the massive installment in the web of Peirce’s texts translated into Spanish.

Necrotizing enterocolitis (NEC) is the most common gastrointestinal surgical disease among neonates. Congenital heart disease (CHD) is also a significant cause of infant morbidity and mortality and it is usually associated with prematurity, low birth weight, pulmonary and gastrointestinal disease, conditions that also account for the development of NEC. Our aim is to analyze the incidence and the risk factors for NEC in neonates with CDH admitted to the Neonatal Intensive Care Unit (NICU) in a 6 years period.All patients admitted in the NICU for any type of CDH from January 2003 to December 2008 comprised the cohort of this study. The primary outcome measure was the development of NEC in any stage. Sixteen independent variables were selected to include those factors previously correlated with NEC. Uni and multivariate statistical analysis were performed with SPSS 15.0 for Windows. Relative Risks (RR) are presented with 95% confidence intervals.935 out of the 7803 neonates admitted during this period, 935 (11.9%) presented a CHD. Among them, 70 also had NEC (RR 8.4-17.8), 31 requiring surgical treatment (Bell stages III and IV). The risk for NEC was specially increased if CHD needed surgical correction (2.1-5.1) and among babies with very low birth weight (VLBW,1,500 g) (2-4.8). After multivariate analysis the only independent variables associated with an increased risk of NEC were birth weight less than 1,500 g (2.2-7.4), the need of surgical correction of CHD (2.2-7.1) and a clinical picture of septicaemia (1.1-3.9).The incidence of NEC is greater in neonates with CDH than in the normal newborn population. Early recognition of NEC should be warranted among CHD patients, specially in those with VLBW, surgical treatment of CHD and sepsis. In this sub-cohort of patients an increased risk of NEC should be suspected.

Traumatic diaphragmatic hernia (HDT) is exceptional in children. Its diagnosis is usually delayed for appearing in the context of severe polytrauma. The aim is to analyze our series in relation to the severity scores.Five patients with HDT are presented. Age, biomechanics, clinical, ISS (injury severity score), ITP (pediatric trauma index), GCS (Glasgow coma scale), RTS (Revised Trauma Score) and treatment were studied. The probability of survival (Ps) was calculated with the TRISS method, Ps = 1/(1 + eb), b = b0 + bl (RTS) + b2 (ISS) + B3 (age).There were two males and three females, mean age was six years old (range = 3-10). Traffic accident was the mechanism of the thoracoabdominal trauma in 80% and one was crushed by garage door. The affected side was right in two cases and left in three. Mean ISS was 41 (range = 32-57), ITP 6 (range = 2-9) and RTS 6.1 (range = 5.1 to 6.9). The main symptom was respiratory. Plain chest radiography was diagnostic in three patients, one by CT scann, and another was a finding at laparotomy. Two had liver herniation, one had tension gastrothorax and two had gastric perforations. All patients underwent surgery through the abdomen. Estimated survival by the TRISS method was respectively 86.6%, 78.2%, 57.2%, 84.7% and 57.1%, while the actual was 100%. One has a severe disability.To study the biomechanics and semiology is essential in the early diagnosis of TDH in pediatric polytrauma and suspect it is mandatory prior to realize percutaneous chest procedures. The TRISS method has a great importance to assess the adult polytrauma, but specific scores are needed for children.

To analyze the benefits of Split (for adult and for child) in liver transplantation.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).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 (p0.05 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.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.

The impact of laparoscopic (LDN) vs. open nephrectomy (ODN) on early graft function and survival in pediatric kidney recipient remains unclear.We retrospectively review the records of 63 pediatric recipient of living donor renal trasplant from 1994 to 2007. We compared those who recieved allograft recovered by LDN (n: 16) with those by ODN (n: 47). The mean recipient age was 9.3 +/- 5 years and the mean donor age was 40.8 +/- 7 years. Outcomes of interest included the incidence of delayer graft function, acute rejection and long-term graft function.Donor, recipient, demographic data and the total time of cold ischemia (1.9 +/- 0.3 hours) were similar in both groups. The mean of kidney warm ischemia was (195 LDN vs. 20 ODN seconds). There were not any significant differences between the two groups, not even in the patient survival after 36 months (100% LDN vs. 98% ODN), neither in the graft survival with proportional hazards Cox analysis. The LDN group needed more days (9.56 +/- 2.3 vs. 4.72 +/- 0.57 ODN) to reach the minimum serum creatinine, but the GF (ml/min/1.73 m2) was similar at 6 months (122 +/- 12 LDN vs. 87 +/- 17 ODN), one year (139 +/- 45 LDN vs. 88 +/- 27 ODN), and two years (110 +/- 64 LDN vs. 82 +/- 30 ODN) after transplant.LDN delays the recovery of the graft function in pediatric recipient. Pediatric LDN recipient have graft outcomes comparable to those of ODN.