[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.

[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.

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.

[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.

[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.

[Pneumonectomy in children]. / Neumonectomía en niños.

BACKGROUND/AIM: Pneumonectomy is seldom indicated in children and its long-term effects are not well known. The aim of the present study was to examine retrospectively the indications and the long-term sequelae in a relatively large cohort of patients treated at our institution. MATERIAL AND METHODS: We reviewed the charts of children undergoing pneumonectomy between 1969 and 2009 with particular attention to indications, techniques, complications, survival, pulmonary function and spine deformities RESULTS: Ten patients (M:F=6/4) aged 102 +/- 66 months underwent pneumonectomy for bronchiectasis (4, one with cystic fibrosis), tumors (4, 1 myofibroblastic inflammatory pseudotumor, 1 bronchial carcinoid, 1 primary pulmonary PNET and 1 massive metastasis of osteosarcoma) and malformations (2, 1 pulmonary sequestration and 1 congenital bilobar emphysema). Overall survival was 90% at 5.6 (0.3-23) years. Patients with bronchiectasis improved markedly except the one with cystic fibrosis who died. All children with tumors cured. Four have some degree of scoliosis which is severe in one with malformative costal fusion. All survivors live unrestristricted normal lives. Postoperative respiratory function is well preserved with FVC of 58% (40-70%), FEV1 of 60% (47-84%) and FEV1/FVC of 92% (87-98%) of the theoretical value for their height and weight. CONCLUSIONS: Pneumonectomy does not affect by itself to the overall survival, and the respiratory function is good and there is no impairment of the quality of life.

[Laparoscopic living donor nephrectomy: functional repercussion in the pediatric recipient]. / Nefrectomía laparoscópica de donante vivo: repercusión funcional en el receptor pediátrico.

AIM: The impact of laparoscopic (LDN) vs. open nephrectomy (ODN) on early graft function and survival in pediatric kidney recipient remains unclear. MATERIAL AND METHOD: 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. RESULTS: 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. CONCLUSIONS: LDN delays the recovery of the graft function in pediatric recipient. Pediatric LDN recipient have graft outcomes comparable to those of ODN.

[The risk of necrotizing enterocolitis in newborns with congenital heart disease. a single institution-cohort study]. / El riesgo de enterocolitis necrosante en recién nacidos con cardiopatía congénita.

BACKGROUND/AIM: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

[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.

[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.

[Bean or blue rubber blue nevus syndrome. Presentation of 6 patients]. / Síndrome de bean o Blue rubber bleb nevus syndrome. presentación de 6 pacientes.

INTRODUCTION/AIMS: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

[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.

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.

[Atypical vascular tumors of the gastrointestinal tract: four uncommon cases]. / Tumores vasculares atípicos del tracto gastrointestinal: cuatro casos poco frecuentes.

AIM: A small but significant percentage of vascular tumors may develop at extracutaneous location. They are difficult to detect on the physical exam and usually they require immediate intervention. Pediatric surgeons must have acknowledge of its prognostic and therapeutic implications. PATIENTS AND RESULTS: We report 4 of these patients. Patient 1 was a healthy newborn who presented in the second week of life, recurrent severe gastrointestinal bleeding, thrombocytopenia and anemia. Diagnosis of multifocal linfangioendoteliomatosis with thrombocytopenia was established. Patient 2 had prenatal diagnosis of ascites and presented at birth sepsis, anemia, thrombocytopenia and hypoproteinemia. Upon laparotomy hemorrhagic ascites and thickening of rectum-sigmoid wall and mesentery were found. Pathologic diagnosis was Kaposiform hemangioendothelioma and the clinical course was consistent with Kassabach-Merrit phenomenon. Patient 3 had at birth, multifocal hepatic GLUT1- hemangiomatosis with severe cardiac insufficiency and coagulopathy. She died while waiting for a liver transplantation. Patient 4 is a girl who presented in the newborn period with vomiting and hematochezia. She required several transfusions and endoscopic biopsies showed a vascular tumor that infiltrated duodenum, jejunum and mesentery. Imaging studies and histologic findings on biopsy led to the diagnostic of juvenile hemangioma GLUT-1+. CONCLUSIONS: Vascular tumors of the digestive tract may be difficult to diagnosis and their classification is still incomplete. Pediatric surgeons must be acquainted with these varieties of tumors because they are always involved in diagnosis and therapeutic decision making.

[Liver transplant from living donor]. / Trasplante hepático de donante vivo.

AIM: Even though Spain has the highest donation rate in the world, our needs cannot be satisfied, specially in younger children. Living-related donor transplant is an alternative in those cases. PATIENTS AND METHOD: We performed a retrospective study of 57 living-related donor transplants performed in our hospital between June 1993 and December 2007. Median age and weight were 1.2 years old (0.5-14.8) and 8.5 kg (5-62). Indications for transplant were as follow: biliary atresia in 42 cases (73.7%), hepatic tumor in 8 (14%) and others in 7 patients. Type of graft was: monosegment (n=1), left lateral segment (n=45), extended left lateral segment (n=5), left liver (n=4), right liver (n=2). We studied the following factors: graft and patient survival (Kaplan Meier), perioperative conditions, complications, causes of graft lost, donor complications and technique difficulties. RESULTS: Patient survival at 3 months, 1 year, 5 years and 10 years was 98.2%, 98.2%, 95% and 95% respectively. Three grafts werelost due to arterial thrombosis, two due to rejection, one due to portal thrombosis and three due to other causes. Complications were as follow: biliary fistula in the cut surface (6), biliary anastomosis complications (6), cut surface abcess (1), portal stenosis (2), suprahepatic stenosis (1) and intestinal perforation (2). Most common complication in donors was biliary leak (4). Among the technique difficulties, 8 patients needed major reconstruction of suprahepatic vein; 4 needed complex portal reconstruction, 6 patients had double biliary tract and 4 patients needed multiple arterial anastomosis. Wall closure was delayed (Goretex) in 35% of cases (20). CONCLUSIONS: Despite technical complications, results after living-related donor transplantation are excellent. It is particularly favourable for children with low weight, since Spanish policy for organ allocation does not make easy to find an adecuate donor in short periods of time. Without living-related donor transplantations, mortality pretransplant would be much higher.

[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.

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.

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.