Thoracic outlet syndrome in adolescents: a case series. / Síndrome del opérculo torácico en adolescentes: serie de casos.

OBJECTIVE: Thoracic Outlet Syndrome (TOS) is caused by a compression of the brachial plexus and the subclavian vessels in their passage to the upper limb. It mostly occurs in women aged 20-50, so it is infrequent in children. We present our results in the diagnosis and management of pediatric TOS. MATERIAL AND METHODS: Retrospective study of patients diagnosed with TOS between December 2017 and June 2018. Clinical, radiological, surgical, and evolution variables were assessed. RESULTS: Five TOS were diagnosed in 4 patients - one TOS was bilateral. Mean age at diagnosis was 12.5 years (7-15), and there was a delay in diagnosis of 153 days (10-36). TOS was either venous (3) or neurogenic (2). Patients presented with pain (5/5), edema (4/5), hypoesthesia (3/5), decreased strength (3/5), and cervical pain (2/5). One patient presented with sport-related pain. Neurophysiological study was normal in three cases. Two patients presented bone anomalies at CT-scan. Three surgeries were performed in two patients using the supraclavicular approach with resection of the anomalous first rib and scalenectomy. One patient refused surgery, and another patient remained expectant without reappearance of symptoms. Postoperative follow-up was 9 months (6-12), with progressive improvement of symptoms. CONCLUSIONS: TOS may occur in adolescents in the form of upper limb pain and edema. Imaging tests are recommended to detect abnormal anatomical structures. The supraclavicular approach represents a safe and effective technique in decompressing the thoracic outlet.

OBJETIVO: El síndrome del opérculo torácico (SOT) está causado por una compresión del plexo braquial y vasos subclavios en su paso hacia la extremidad superior. Patología típica de mujeres entre 20 y 50 años, que es infrecuente diagnosticar en niños. Presentamos nuestros resultados en el diagnóstico y tratamiento del SOT pediátrico. MATERIAL Y METODOS: Estudio retrospectivo de pacientes diagnosticados de SOT entre diciembre 2017 y junio 2018. Se analizaron variables clínicas, radiológicas, quirúrgicas y de evolución. RESULTADOS: Cinco SOT fueron diagnosticados en cuatro pacientes, uno de ellos bilateral. La edad media al diagnóstico fue de 12,5 años (7-15) y hubo una demora en el diagnóstico de 153 días (10-360). SOT venoso (3) y neurológico (2). Presentaron dolor (5/5), edema (4/5), hipoestesia (3/5), disminución de fuerza (3/5) y dolor cervical (2/5). Una paciente presentaba dolor asociado al deporte. El estudio neurofisiológico fue normal en tres casos. Dos pacientes presentaron anomalías óseas por TAC. Se realizaron tres intervenciones quirúrgicas en dos pacientes por abordaje supraclavicular realizando resección de la primera costilla anómala y escalenectomía. Una paciente rechazo la intervención y en otra se mantuvo en una actitud expectante sin reaparición de los síntomas. Seguimiento posoperatorio de 9 meses (6-12) con mejoría progresivas de los síntomas. CONCLUSION: El SOT puede darse en adolescentes siendo el dolor y edema de la extremidad superior lo más específico. Se recomienda la realización de pruebas de imagen para detectar estructuras anatómicas anómalas. El abordaje supraclavicular se presenta como una técnica segura y eficaz en la descompresión del desfiladero torácico.

A decision-tree approach for the differential diagnosis of chronic lymphoid leukemias and peripheral B-cell lymphomas.

BACKGROUND AND OBJECTIVE: Here we propose a decision-tree approach for the differential diagnosis of distinct WHO categories B-cell chronic lymphoproliferative disorders using flow cytometry data. Flow cytometry is the preferred method for the immunophenotypic characterization of leukemia and lymphoma, being able to process and register multiparametric data about tens of thousands of cells per second. METHODS: The proposed decision-tree is composed by logistic function nodes that branch throughout the tree into sets of (possible) distinct leukemia/lymphoma diagnoses. To avoid overfitting, regularization via the Lasso algorithm was used. The code can be run online at https://codeocean.com/2018/03/08/a-decision-tree-approach-for-the-differential-diagnosis-of-chronic-lymphoid-leukemias-and-peripheral-b-cell-lymphomas/ or downloaded from https://github.com/lauramoraes/bioinformatics-sourcecode to be executed in Matlab. RESULTS: The proposed approach was validated in diagnostic peripheral blood and bone marrow samples from 283 mature lymphoid leukemias/lymphomas patients. The proposed approach achieved 95% correctness in the cross-validation test phase (100% in-sample), 61% giving a single diagnosis and 34% (possible) multiple disease diagnoses. Similar results were obtained in an out-of-sample validation dataset. The generated tree reached the final diagnoses after up to seven decision nodes. CONCLUSIONS: Here we propose a decision-tree approach for the differential diagnosis of mature lymphoid leukemias/lymphomas which proved to be accurate during out-of-sample validation. The full process is accomplished through seven binary transparent decision nodes.

Reliability of the Hirschsprung-Associated Enterocolitis Score in Clinical Practice.

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.

Is Intraoperative Fluoroscopy Necessary for Central Venous Port System Placement in Children?

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.

[Risk factors associated with the development of enterocolitis in Hirschsprung's disease]. / Factores de riesgo asociados al desarrollo de enterocolitis en la enfermedad de Hirschsprung.

OBJECTIVES: Hirschsprung's-associated enterocolitis (HAEC) is a live-threatening complication that remains badly understood. Our objective is to identify the risk factors related to the development of HAEC in the cohort of patients with Hirschsprung's disease (HD) treated in our center. METHODS: We reviewed the patients treated for HD between 2000 and 2016. Ninety four patients were included, and the clinical details related to the disease were evaluated. Our primary outcome measure was the development of HAEC. Relative risks are presented with 95% confidence intervals. RESULTS: Twenty seven patients out of the ninety four (28.7%) suffered HAEC. None of them died from this complication. The extended aganglionosis, the need of a preoperative stoma, a transabdominal surgery and the diagnosis before the age of 7.2 months were related to a higher risk of suffering HAEC. CONCLUSIONS: HAEC remains a common complication in patients suffering from HD, especially those with complex forms. The identification of the risk factors could result in a better control of the HAEC, which lead to a faster diagnosis and treatment, reducing the morbi-mortality related to HAEC.

OBJETIVO: La enterocolitis (EC) asociada a la enfermedad de Hirschsprung (EdH) es una entidad de etiopatogenia desconocida y potencialmente mortal. Nuestro objetivo es identificar los factores implicados en el desarrollo de EC en una cohorte de pacientes con EdH tratados en el nuestro centro. METODOS: Se revisaron los pacientes tratados por EdH en el periodo 2000-2016. Se incluyeron 94 pacientes, recogiendo de las historias clínicas variables demográficas y todas las relacionadas con la enfermedad. Se realizó un estudio de riesgo uni y multivariado usando como variable dependiente la existencia de un cuadro de enterocolitis. Los resultados se presentan con riesgo relativo (RR) con su intervalo de confianza al 95%. RESULTADOS: De los 94 pacientes estudiados, 27 (28,7%) sufrieron algún episodio de EC. Ninguno falleció por esta causa. La afectación intestinal extensa, la necesidad de una ostomía de descarga en el proceso diagnóstico, la cirugía abierta y el diagnóstico anterior a los 7,2 meses de vida se asociaron en el análisis univariante a un mayor riesgo de desarrollar EC. En el multivariante, tan solo el diagnostico precoz y la cirugía abierta quedaron como variables asociadas al riesgo de EC. CONCLUSIONES: La EC sigue siendo una complicación habitual en la EdH, especialmente en las formas más largas y complejas. La identificación de los factores que la favorecen permitiría un mayor control, una mejor prevención secundaria ante el comienzo de los síntomas y un tratamiento más precoz, lo que lograría disminuir su morbilidad.

[Nissen fundoplication in children under 1 year of age: is age important?] / Funduplicatura de Nissen en niños menores de 1 año: ¿la edad importa?

AIM: 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. MATERIALS AND METHODS: 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. RESULTS: 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, p <0.05) 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 (p <0.05). 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%). CONCLUSIONS: 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.

INTRODUCCION Y OBJETIVOS: 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. MATERIAL Y METODOS: 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. RESULTADOS: 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, p <0,05) 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 (p <0,05). 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%). CONCLUSIONES: 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.

Best oxygenation index on day 1: a reliable marker for outcome and survival in infants with congenital diaphragmatic hernia.

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<0.05 was considered significant. 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<0.0001), LHR >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.

Cephalic pancreaticoduodenectomy for bleeding duodenal arteriovenous malformation.

Introduction Treatment of recurrent severe gastrointestinal bleeding due to arteriovenous malformations may require complex resections. In some particular locations, extensive surgery is the only way out, as shown in this report. Case Report A 2.5-year-old child suffered repeated episodes of upper gastrointestinal bleeding since the first month of life. After an extensive diagnostic workout, the diagnosis of duodenal arteriovenous malformation was established. Cephalic pancreaticoduodenectomy with pyloric preservation was performed and no further episodes of bleeding occurred in the ensuing 2 years. Conclusion Bleeding malformations located in the pancreaticoduodenal area can be effectively treated in children by pylorus-preserving cephalic pancreaticoduodenectomy.

[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.

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.

[Noninvasive positive pressure ventilation in postoperative period of tracheal surgery]. / Ventilación con presión positiva no invasiva en el postoperatorio de cirugía traqueal.

OBJECTIVES: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Late surgery for spinal deformities in children previously treated for neural tumors.

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.

Surgical treatment of chronic inflammatory bowel disease in children.

Surgery for chronic inflammatory bowel disease (IBD) is increasingly often necessary in children. This study aimed at assessing the results of these operations in order to facilitate adequate preoperative counseling. We reviewed patients treated from 1992 to 2009. The operations, complications and functional outcome were recorded. For those with preserved rectal defecation, continence (Koivusalo score) and quality of life (standardized questionnaire) were assessed in the long term. Eighty five of 192 patients had Crohn disease (CD), 107 of 192 had ulcerative colitis (UC), and 3 of 192 had indeterminate colitis (IC). 12 of 85 CD patients (15%) aged 14 (12-19) years required 13 resections, 1 stricturoplasty, 1 transplantation and 6 other operations including 3 permanent enterostomies for anorectal involvement. Removal of the involved bowel led to significant improvement of nutritional status, growth and quality of life. The transplanted patient had a striking recovery but eventually died 1 year later of unrelated complications. 29 of 107 UC patients (26%) aged 11 (2-15) years required 87 operations. Nine had emergency colectomy for toxic megacolon (3, one death) or severe hemorrhage (6). 28 had restorative proctocolectomy and ileoanostomy (RPCIA) without (16) or with (12) J-pouch under protective ileostomy. Complications were frequent (40%). Permanent ileostomy was required in five children (17%). Twelve months postoperatively, RPCIA patients had 6.5 (2-13) stools/day; all were continent during daytime, and 25% have nocturnal leaks. Mean Koivusalo score (5-12) was 8.8 ± 2. Quality of life was good in all. All attended normal school and 7 the university, 4 work and 60% of those older than 18 years have sexual partners. Three of 107 children treated as UC with RPCIA had ultimately IC (3%) and were permanently diverted. The nature of IBD involves frustrating surgery. However, it may change life for CD patients and provide a reasonably good quality of life for UC after the first year. Pediatric surgeons should be able to provide adequate preoperative counseling to patients and families.

High-risk hepatoblastoma: results in a pediatric liver transplantation center.

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 <100 ng/ml serum AFP) were identified and imaging and biopsies were reviewed. The treatment consisted of total removal of the tumor, involving extended hepatectomies and LT if necessary, together with SIOPEL-guided chemotherapy. RESULTS: 23/57 hepatoblastomas were HRH (11F/12M). 17 were considered unresectable by standard techniques, 3 had extrahepatic disease, and 3 fulfilled both criteria. Mean age at diagnosis was 2.3 ± 2.4 years. 3 children (referred after chemotherapy) died without surgery. 4 had resections (2 left and 2 right trisegmentectomies). Primary LT was required in 15 children (7 cadaveric donors and 8 living related donor transplantations (LRDT), 2 of them with retrohepatic vena cava replacement), and 1 patient had rescue LT after recurrence. Mean follow-up was 4.8 ± 2.9 years. 2 children who had undergone liver resection developed pulmonary metastases at 1.7 and 1.6 years postoperatively and survived after surgical treatment. 2 children with LT developed EBV-related lymphoma and leukemia respectively but survived. Event-free survival (EFS) at 1, 5, and 10 years was 78.3 ± 8.6%, 63.1 ± 10.5%, and 63.1 ± 10.5%, respectively. 6 children died (3 without surgery, 1 after liver resection, 1 after primary LT and 1 after rescue LT). Overall survival at 1, 5 and 10 years was 78.3 ± 21.7%, 73.2 ± 26.8% and 73.2 ± 26.8%. Of those with primary LT, survival at 1, 5 and 10 years was 93.3 ± 6.4%, 93.3 ± 6.4% and 93.3 ± 6.4%. CONCLUSIONS: Outstanding results in the treatment of HRH are possible in tertiary centers when referral is early (preferably at diagnosis) and specialized liver surgery and transplantation facilities are available.

[Traumatic diaphragmatic hernia: a vital need for an early diagnosis]. / Hernia diafragmática traumática: necesidad vital de un diagnóstico precoz.

INTRODUCTION: 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. PATIENTS AND METHODS: 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). RESULTS: 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. DISCUSSION: 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.

Monoclonal free light chains can be found in heavy chain diseases.

Heavy chain diseases (HCDs) are rare B-cell lymphoproliferative neoplasias characterized by the production of a monoclonal component consisting of a truncated monoclonal Ig heavy chain without the associated light chain. Among them, patients with gamma-HCD are so rare that no more than 150 cases can be found in the literature. In this paper, we report one additional case: an 83-year-old man with a gamma-HCD, in whom a kappa light chain component was detected in the serum by using the serum free light-chain assessment and in addition monoclonal kappa cytoplasmic expression was detected in bone marrow plasma cells by flow cytometric analysis. In the work-up of the patient, the underlying anatomopathological lymphoproliferative disease corresponded to a lymphoplasmacytic lymphoma, as it is stated in the current World Health Organization classification (2008), with both lymphadenopathic and bone marrow infiltration. As in other cases, several autoimmune manifestations (antiphospholipidic syndrome and immune thrombocytopenia) were present during the course of the disease in this patient. This case report illustrates a new case of gamma-HCD, in which serum free light-chain analysis and flow cytometry represented a valuable tool for diagnosis, a finding that could be very important for the future management of these patients.

Automated pattern-guided principal component analysis vs expert-based immunophenotypic classification of B-cell chronic lymphoproliferative disorders: a step forward in the standardization of clinical immunophenotyping.

Immunophenotypic characterization of B-cell chronic lymphoproliferative disorders (B-CLPD) is becoming increasingly complex due to usage of progressively larger panels of reagents and a high number of World Health Organization (WHO) entities. Typically, data analysis is performed separately for each stained aliquot of a sample; subsequently, an expert interprets the overall immunophenotypic profile (IP) of neoplastic B-cells and assigns it to specific diagnostic categories. We constructed a principal component analysis (PCA)-based tool to guide immunophenotypic classification of B-CLPD. Three reference groups of immunophenotypic data files-B-cell chronic lymphocytic leukemias (B-CLL; n = 10), mantle cell (MCL; n = 10) and follicular lymphomas (FL; n = 10)--were built. Subsequently, each of the 175 cases studied was evaluated and assigned to either one of the three reference groups or to none of them (other B-CLPD). Most cases (89%) were correctly assigned to their corresponding WHO diagnostic group with overall positive and negative predictive values of 89 and 96%, respectively. The efficiency of the PCA-based approach was particularly high among typical B-CLL, MCL and FL vs other B-CLPD cases. In summary, PCA-guided immunophenotypic classification of B-CLPD is a promising tool for standardized interpretation of tumor IP, their classification into well-defined entities and comprehensive evaluation of antibody panels.

[Long-term results in ulcerative colitis treated with proctocolectomy and ileoanostomy in children]. / Resultados a largo plazo en la colitis ulcerosa tratada con proctocolectomía e ileoanostomía en niños.

AIM: The treatment of Ulcerative Colitis (UC) unresponsive to current medical treatment involves total proctocolectomy and ileanostomy. The aim of this study was to assess the long term results in such patients. MATERIAL AND METHODS: We reviewed children treated from 1992 to 2008. The operations, complications and functional outcome were recorded. For those with preserved rectal defecation, continence (Holschneider score) and quality of life (standardized phone questionnaire) were assessed in the long term. RESULTS: 104 patients had Ulcerative Colitis (UC) and 3 undetermined colitis (excluded from the study). 26/104 UC patients (26%) aged 10.7 +/- 4.1 years required 82 operations. Eight had emergency colectomy for toxic megacolon (3, one death) or severe hemorrhage (5). 25 had restorative proctocolectomy and ileoanostomy (RPCIA) without (16) or with (9) J-pouch under protective ileostomy. Complications were frequent (40%). Permanent ileostomy was required in 5 children (20%). Twelve months postoperatively, RPCIA patients had 6.3 +/- 3 stools/day, all were continent during daytime and 25% have nocturnal leaks. Mean Holschneider score (0-12) was 8.8 +/- 2. Quality of life was good in all with normal school (all) or university attendance (7), 4 work and 60% of those older than 18 have sexual partners. CONCLUSIONS: The nature of UC involves risky and frustrating surgery. However, it may provide a reasonably good quality of life for UC patients after the first year. Pediatric surgeons should be able to provide adequate preoperative counseling to patients and families.

[Laparoscopic resection of the prostatic utricle in children]. / Resección laparoscópica del utrículo prostático en niños.

INTRODUCTION: The prostatic utricle is an embryological remnant from Müllerian duct tissue. Most prostatic utricles are asympomatic, but they may manifest as a urinary tract infection or an incontinence. MATERIAL AND METHOD: Surgery consists in a cysto-urethroscopy and cannulation of the prostatic utricle with a ureteral catheter (Fogarty ner 4) and the cystoscopy left in situ to facilitate identification and mobilization. A 10 mm port through a umbilical incision and two more 5 mm working ports were inserted. After mobilization, the ureteral defect was closed by an absorbable suture and ultrasonic coagulation. RESULTS: Five boys with symptomatic prostatic utricles underwent surgery at a mean (range) age of 7 (2-11) years. The laparoscopic excision was successful in all but one due to a bleeding. The mean (range) operative duration was 165 (120-240) min. CONCLUSION: Laparoscopic excision under cystoscopic guidance offers a good surgical view and allowing easy dissection.