[Slipping rib syndrome in paediatrics: report of 4 cases]. / Síndrome de la costilla deslizante. Serie de 4 casos clínicos.

AIM OF THE STUDY: The slipping rib syndrome (SRS) is an unknown pathology for the pediatric surgeon due to its low incidence in children. The weakness of the costal ligaments allowing an area of rib hypermobility has been postulated recently as the main etiology. It produces an intermittent pain in the lower thorax or upper abdomen that can affect to the daily activities and can be the origin of unspecific chronic pain. METHODS: A retrospective review of patients diagnosed with SRS between october 2012 and march 2017 was performed. Data of demographics, symptoms, imaging studies, surgical findings and long-term follow-up were collected. RESULTS: During this period, 4 patients were diagnosed with SRS. Median age at diagnosis was 13 years (12-15 years) with a mean duration of symptoms of 13 months (12-36 months). In 2 patients the SRS was associated with Costal Dysmorphia (CD). The initial diagnosis was clinical with posterior ultrasound confirmation. Resection of the affected cartilages was performed in 3 patients and after a follow-up of 6 months (3-30 months), they all are painless and refer a good cosmetic result. One patient refused the intervention. CONCLUSIONS: The SRS is an infrequent cause of thoracic pain with an etiology not well understood. The awareness of this disease and its typical presentation can avoid unnecessary studies. The resection of the affected cartilages is a safe and effective treatment.

INTRODUCCION: El síndrome de costilla deslizante (SCD) es una entidad poco frecuente en niños. Se cree que su causa es una debilidad en los ligamentos costales que permite una hipermovilidad de las costillas. Genera un dolor intermitente en la región baja del tórax o alta del abdomen que puede afectar a las actividades de la vida diaria o generar un dolor crónico. MATERIAL Y METODOS: Revisión retrospectiva de SCD entre octubre de 2012 y diciembre de 2017. Se recogió información acerca de los datos demográficos, síntomas, estudios de imagen, hallazgos intraoperatorios, material fotográfico y seguimiento a largo plazo. RESULTADOS: Durante este periodo, 4 pacientes fueron diagnosticados de SCD. La mediana de edad al diagnóstico fue de 13 años (12-15 años) con una duración previa de los síntomas de 13 meses (12-36 meses). En 2 pacientes se asoció una dismorfia costal (DC). El diagnóstico fue clínico con confirmación ecográfica. Se realizó resección de los cartílagos afectos en 3 pacientes con un seguimiento posterior de 6 meses (3-30 meses). Actualmente se encuentran sin dolor y con un resultado estético satisfactorio. Un paciente rechazó la intervención. CONCLUSIONES: El SCD aparece en pacientes preadolescentes que en algunos casos asocian DC. Una exploración física y ecografía enfocada son las claves para un diagnóstico certero. La resección de cartílagos es efectiva a largo plazo.

[Pulmonary lobectomy in children: the sooner the better?] / Lobectomía pulmonar en niños: ¿cuanto antes mejor?

AIM OF THE STUDY: Recommendation of early pulmonary resection in asymptomatic congenital pulmonary airway malformations (CPAMs) is based on the presumed compensatory lung growth during the first months of life. Our aim is to analyze the long-term pulmonary function after lobectomy before and after one year of age using spirometry. METHODS: We performed a retrospective review of children who underwent pulmonary lobectomy for CPAM between 2001 and 2016. Patients who were old enough (>5 years) to carry out a spirometry were included in the study and were divided into 2 groups (surgery before or after 12 months of age). Pulmonary function testing values were considered normal if they were >80% of predicted. MAIN RESULTS: Forty-seven patients underwent pulmonary lobectomy for CPAM, 23 of them met the inclusion criteria and prospectively performed a spirometry. Among them, 7 had surgery before and 16 after one year of age (0.1 vs. 2); being both groups comparable in terms of sex, type of CPAM and surgical approach. Time from surgery until pulmonary function testing was longer in patients who had surgery before one year of age (9.1 vs. 4.6 years, p = 0.003). After correcting results by time from surgery until spirometry, a better FEV1/FVC was found in patients who had surgery after one year of age (90% vs. 77%, p = 0.043). CONCLUSION: Although spirometry may be influenced by many other variables, these preliminary results do not support the current recommendation of performing early lobectomy in CPAMs. Further studies are required in order to resolve the best age to perform pulmonary lobectomy.

INTRODUCCION/OBJETIVO: La resección temprana de las malformaciones pulmonares asintomáticas ofrece el beneficio teórico de optimizar el crecimiento pulmonar compensatorio durante la infancia. El objetivo de este estudio es determinar si la lobectomía antes del año de vida se asocia con mejor función pulmonar a largo plazo. MATERIALES Y METODOS: Revisión de pacientes en los que se realizó lobectomía pulmonar desde 2001, incluyendo los que tenían edad suficiente para realizar una espirometría (>5 años). Fueron divididos en dos grupos: lobectomía antes o después de 12 meses de vida. Los parámetros espirométricos se consideraron normales cuando eran mayores del 80% esperado. RESULTADOS: Veintitrés de los 47 pacientes intervenidos cumplieron los criterios de inclusión. Siete fueron intervenidos antes (0,1 ± 0,4 años) y 16 después del año de vida (2 ± 3,6 años), siendo ambos grupos comparables en sexo, tipo de malformación y abordaje quirúrgico. El tiempo de seguimiento desde la cirugía hasta que se realizó la espirometría fue mayor en los pacientes intervenidos antes del año de vida (9,1 vs. 4,6 años, p = 0,003). Tras corregir los resultados por el tiempo de seguimiento, se objetivó un mejor cociente FEV1/FVC en los pacientes intervenidos después del año de vida (90% vs. 77%, p = 0,043). CONCLUSION: Aunque la espirometría puede estar influenciada por otras variables, los datos de nuestro estudio no apoyan la recomendación actual de realizar resección temprana en las malformaciones pulmonares congénitas asintomáticas. Se necesitan estudios prospectivos con mayor número de pacientes para determinar la mejor edad para realizar la lobectomía.

[Study of pulmonary hypertension and long-term respiratory clinic in children with congenital diaphragmatic hernia]. / Estudio de la hipertensión pulmonar y la clínica respiratoria a largo plazo en los niños con hernia diafragmática congénita.

INTRODUCTION AND OBJECTIVES: The aim of this study is to identify respiratory clinic and pulmonary arterial hypertension (PAH) in congenital diaphragmatic hernia (CDH) and whether these could be predicted by prenatal measures. MATERIAL AND METHODS: We studied fetal ultrasound: Observed/expected Lung to Head Ratio (O/E LHR) and classified patients according to their outcome (group 1: O/E LHR <25%, group 2: 26-35%, group 3: 36-45%, group 4: >55%) as well as the severity of PAH (group 0: non-PAH, group 1: mild, group 2: moderate, group 3: severe) in echocardiograms at birth, 1st, 6th, 12th and 24 months of life. We also evaluated gestational age, weight, bronchodilator treatment and number of hospital admissions. RESULTS: 58 patients with CDH, 13 without prenatal diagnosis. 36 patients out of 45 had O/E LHR calculated at 22.4 ± 5.8 weeks. O/E LHR had significant association with the severity of PAH at birth and in the 1st, 6th, 12th and 24th months (p <0.05). At 6 months, only 30.4% had PAH without any association with a higher risk of hospital admission [OR 1.07 (0.11-10.1)] and only three patients (5.1%) required bronchodilator treatment. CONCLUSION: In CDH, PAH and the respiratory clinic improve over time, being uncommon the need for treatment as of the 6th month. O/E LHR predicts the presence and severity of PAH in short and long term.

INTRODUCCION Y OBJETIVOS: Nuestro objetivo es estudiar la presencia en hernia diafragmática congénita (HDC) de clínica respiratoria e hipertensión pulmonar (HTP) a largo plazo y si estas pueden predecirse prenatalmente. MATERIAL Y METODOS: Estudiamos en ecografía fetal: Lung to Head Ratio observado/esperado (LHR O/E) y clasificamos a los pacientes según su resultado (grupo 1: LHR O/E <25%, grupo 2: 26-35%, grupo 3: 36-45%, grupo 4: >55%) así como la gravedad de HTP (grupo 0: no HTP, grupo 1: leve, grupo 2: moderada, grupo 3: grave) en los ecocardiogramas al nacimiento, 1º, 6º, 12º y 24º meses de vida. Estudiamos también edad gestacional, peso, tratamiento broncodilatador y número de ingresos hospitalarios. RESULTADOS: Se identificaron 58 pacientes con HDC, 13 de ellos sin diagnóstico prenatal. De los 45 restantes, 36 tenían calculado el LHR O/E registrado a las 22,4 ± 5,8 semanas. El LHR O/E se relacionó significativamente con la gravedad de la HTP al nacimiento y en los meses 1º, 6º, 12º y 24º (p <0,05). A los 6 meses únicamente el 30,4% presentaban HTP sin que ello asociara más riesgo de ingresos hospitalarios [OR 1,07 (0,11-10,1)] y siendo solo n = 3 (5,1%) los que precisaban algún tipo de tratamiento broncodilatador. CONCLUSION: En HDC, la HTP y la clínica respiratoria mejoran con el tiempo, siendo infrecuente la necesidad de tratamiento a partir del 6º mes. El LHR O/E predice la presencia y gravedad de HTP a corto y largo plazo.

[Perinatal factors for necrotizing enterocolitis (NEC). A case-control study]. / Factores perinatales en el desarrollo de enterocolitis necrotizante. Un estudio de casos y controles.

AIM OF THE STUDY: The aim of this study is to identify potential perinatal risk or protective factors associated with NEC. MATERIALS AND METHODS: Single-center, retrospective case-control study of newborns admitted to the neonatal intensive care unit with NEC from 2014 to 2015. Clinical charts were reviewed recording maternal factors (fever, positive recto-vaginal swab and signs of corioamnionitis or fetal distress), and neonatal factors analyzed were: birth-weight and weeks gestation, umbilical vessel catheterization, time of enteral feedings and the use of probiotics, antibiotics and antifungal agents. Cases and controls were matched for all of these factors. Parametric tests were used for statistical analysis and p < 0.05 deemed significant. RESULTS: We analyzed 500 newborns of which 44 developed NEC (cases) and 456 controls. Univariate analysis did not identify any maternal risk factors for NEC. We did not found statistical differences between patients either time of enteral feedings or probiotics. Nevertheless, patients with signs of fetal distress and early sepsis had a higher risk of NEC (p < 0.0001). CONCLUSIONS: Infants with history of fetal distress and signs of early sepsis are at a higher risk of NEC. The use of prophylactic catheter infection or orotracheal intubation with antifungal treatment seemed to elevate the incidence of NEC. However, antibiotic treatment couldn´t be demonstrated to increase the risk of NEC.

OBJETIVOS: Identificar factores perinatales que favorezcan el desarrollo de enterocolitis necrotizante (ECN) en las unidades de cuidados intensivos neonatales (UCIN). METODOS: Estudio de casos y controles de los recién nacidos (RN) tratados entre 2014-2015. Se evaluaron factores de riesgo materno-fetal (fiebre, corioamnionitis, cultivos rectovaginales y sufrimiento intrauterino) y neonatales (edad gestacional, canalización de vasos umbilicales, hemocultivos, sepsis, nutrición y probióticos) y su asociación a la ECN. Estudiamos también la existencia de tratamiento antibiótico y antifúngico intravenoso previo al cuadro de ECN. Se estimó la odds ratio con un nivel de significación p < 0,05. RESULTADOS: Se analizaron 500 neonatos: 44 ECN y 456 controles. En el análisis univariante ninguno de los factores de riesgo maternos se relacionó con el desarrollo de ECN. No se encontraron diferencias significativas en los RN que recibieron alimentación enteral o probióticos. Los RN con sufrimiento fetal y los diagnosticados de sepsis precoz presentaron mayor riesgo de desarrollo de ECN (p < 0,0001). CONCLUSION: La pérdida de bienestar fetal y la sepsis precoz favorecen el desarrollo de ECN, que también parece aumentar con el uso de antibioterapia sistémica así como el tratamiento antifúngico profiláctico para las infecciones de catéter o intubaciones orotraqueales prolongadas.

[Long-term results of the early endoscopic treatment of acquired tracheal-subglottic stenosis: 10 years of experience]. / Resultados a largo plazo del tratamiento endoscópico precoz de las estenosis subglóticas y traqueales adquiridas: 10 años de experiencia.

INTRODUCTION: Acquired stenosis of the airway is a common complication after endotracheal intubation. Endoscopic dilation has been accepted as the treatment of choice in cases detected precociously. Our goal is to know the current status of the patients treated in our hospital with endoscopic dilation in the last 10 years. MATERIAL AND METHODS: Retrospective cohort study of patients with subglottic and tracheal acquired stenosis (STAS) early treated endoscopically with balloon dilation at our center in the last 10 years. Bronchoscopy control at 2 weeks, a month, 3 and 6 months post-dilation were performed and later on depending on the symptoms. RESULTS: 32 patient were treated in the period considered. The median age was 4.5 (3-120) months. There were necessary 2.5 (1-5) dilations per patient. All cases were extubated in the operating room or in the following 24 hours. There were no complications during the procedure. Follow-up time was 6 (1-10) years. Only 1 of the 32 patients have had recurrence of stenosis 2 years after, it was secondary to reintubations due to new surgical interventions; which it was dilated successfully. CONCLUSIONS: Early endoscopic dilation in the acquired airway stenosis is a safe and effective long-term procedure. The results support the use of this technique as a treatment of choice in these patients.

INTRODUCCION: La estenosis adquirida de la vía aérea es una complicación frecuente tras la intubación endotraqueal. La dilatación endoscópica ha sido aceptada como tratamiento de elección en los casos detectados de forma precoz. Nuestro objetivo es conocer el estado actual de los pacientes tratados en nuestro centro mediante dilatación endoscópica en los últimos 10 años. MATERIAL Y METODOS: Estudio de cohorte retrospectivo de pacientes con estenosis subglóticas y traqueales adquiridas (ESTA) tratadas endoscópicamente mediante dilatación con balón en nuestro centro en los últimos 10 años. Se realizaron broncoscopias de control a las 2 semanas, al mes, a los 3 y 6 meses postdilatación y posteriormente en función de la clínica. RESULTADOS: Se trataron 32 pacientes de ESTA de reciente aparición en dicho periodo. La mediana de edad fue de 4,5 (3-120) meses. Fueron necesarias 2,5 (1-5) dilataciones por paciente. Todos los pacientes fueron extubados en quirófano o en las 24 horas siguientes al procedimiento. No hubo complicaciones durante los procedimientos ni durante el postoperatorio. El tiempo de seguimiento fue de 6 (1-10) años. Solo 1 de los 32 pacientes presentó recidiva de la estenosis 2 años después que fue secundaria a reintubaciones por nuevas intervenciones quirúrgicas; la cual se dilató nuevamente. CONCLUSIONES: La dilatación endoscópica precoz en las estenosis adquiridas de la vía aérea es un procedimiento seguro y eficaz a largo plazo. Los resultados avalan el uso de esta técnica como tratamiento de elección en estos pacientes.

[The role of fetal magnetic resonance imaging in the study of congenital diaphragmatic hernia]. / El papel de la resonancia magnética fetal en el estudio de la hernia diafragmática congénita.

INTRODUCTION AND OBJECTIVES: Different echographic and fetal magnetic resonance (MRI) measurements have been described in the diagnosis of associated malformations and the prognosis of congenital diaphragmatic hernia (CDH). We have reviewed our experience searching for useful isolated or combined parameters and how MRI can complement ultrasound. MATERIAL AND METHODS: We evaluated 29 fetuses with CDH. We examined ultrasonography: Lung to Head (LHR o/e) and in MRI: ipsilateral lung volume (IPV) and total expressed as percentage of observed / expected lung volume (VPT o/e) and percentage of herniated liver (PHH). We studied: survival, ECMO and associated malformations. RESULTS: LHR o/e was the measure that best predicted survival (p< 0.05). VPT o/e did not predict survival or the need of ECMO (p> 0.05). PHH ≥19% was related to the need of ECMO. IPV < 2 cc required ECMO more frequently (p< 0.018) and when it was 0 cc in all cases. No combination of MR measurements was superior to LHR o/e in prediction of survival. MRI complemented the ultrasound in 4 cases: diaphragmatic eventration diagnosed with HDC, right HDC with fluid in the sac that suggested thoracic cyst, differentiation between spleen and lung that measured together overestimated the LHR and/or suspicion of Cornelia de Lange due to facial malformations. CONCLUSIONS: Not a single or combined MRI measurement exceeds LHR o/e in survival prediction. MRI is related to prognosis and can be used to support ultrasound in making decisions. MRI occasionally provides complementary morphological information.

INTRODUCCION Y OBJETIVOS: Distintas medidas ecográficas y de resonancia magnética fetal (RM) han sido descritas en el diagnóstico de malformaciones asociadas y el pronóstico de la hernia diafragmática congénita (HDC). Hemos revisado nuestra experiencia buscando parámetros aislados o combinados útiles y cómo la RM puede complementar a la ecografía. MATERIAL Y METODOS: Evaluamos 29 fetos con HDC. Revisamos en ecografía: Lung to Head Ratio observado/esperado (LHR o/e) y en RM: volumen pulmonar ipsilateral (VPI) y total expresado como porcentaje del volumen pulmonar observado/esperado (VPT o/e) y porcentaje de hígado herniado (PHH). Estudiamos: supervivencia, oxigenación con membrana extracorpórea (ECMO) y malformaciones asociadas. RESULTADOS: El LHR o/e fue la medida que mejor predijo supervivencia (p< 0,05). El VPT o/e no predijo supervivencia ni la necesidad de ECMO (p> 0,05). El PHH ≥19% se relacionó con necesidad de ECMO. El VPI menor de 2 cc requirió ECMO más frecuentemente (p< 0,018) y cuando fue de 0 cc en todos los casos. Ninguna combinación de medidas de RM fue superior al LHR o/e en predicción de supervivencia. La RM complementó a la ecografía en 4 casos: eventración diafragmática diagnosticada como HDC, HDC derecha con líquido en el saco que sugería quiste torácico, diferenciación entre bazo y pulmón que medidos juntos sobreestimaba el LHR o/e y sospecha de síndrome de Cornelia de Lange por malformaciones faciales. CONCLUSIONES: Ninguna medida aislada o combinada de RM supera al LHR o/e en la predicción de supervivencia. La RM se relaciona con el pronóstico y puede usarse como apoyo de la ecografía en la toma de decisiones. La RM aporta ocasionalmente información morfológica complementaria.

[Intestinal complications in twin-to-twin transfusion syndrome (TTTS) treated by laser coagulation (LC)]. / Complicaciones intestinales en el síndrome de trasfusión feto fetal (STFF).

AIM OF THE STUDY: The hemodynamic imbalance due to placental vascular anastomoses in TTTS but also vascular changes generated after intrauterine treatment may lead to hypoxic-ischemic complications. Different intestinal complications in TTTS are reviewed in this paper. METHODS: Retrospective review of TTTS cases treated by laser coagulation (LC) from 2012-2015. Demographic data, fetal therapy, prenatal diagnosis (US, MRI) and perinatal outcome were recorded. We describe cases with intestinal complications and their postnatal management. Results are expressed by median and range. RESULTS: 29 monochorionic pregnancies with TTTS were treated (23 LC, 4 cord occlusions and 2 cord occlusions after LC). The diagnosis was made at 19 (16-26) weeks and 86% presented stage of Quintero ≥ II. In 70% of mothers survived at least one fetus with a median of 31 (24-37) weeks at birth. Four patients had intestinal complications (1 jejunal atresia, 2 ileal atresia, 1 perforated necrotizing enterocolitis), half of them had prenatal diagnosis. Postnatal resections of the affected segments and ostomies were performed. Intestinal transit was restored and there were no severe digestive sequelae after 21 (8-38) months of follow up. CONCLUSIONS: Different types of intestinal complications were associated with TTTS and LC. US and MRI enable prenatal diagnosis of these complications and this allows prompt decisions after birth.

INTRODUCCION: El desequilibrio hemodinámico secundario a la presencia de anastomosis vasculares placentarias en el STFF así como los cambios hemodinámicos generados durante y tras su tratamiento mediante fotocoagulación con láser (FC) puede dar lugar a complicaciones hipóxico-isquémicas en distintos sistemas. Revisamos nuestra experiencia en el tratamiento del STFF con FC y presentamos las complicaciones intestinales encontradas. MATERIAL Y METODOS: Estudio retrospectivo de casos tratados intraútero entre 2012 y 2015. Recogimos datos sociodemográficos, terapia fetal, pruebas diagnósticas prenatales (ecografía, RM) y resultados perinatales. Expresamos las medidas en medianas y rangos. RESULTADOS: Se procedió al tratamiento intraútero de 29 gestaciones monocoriales complicadas con STFF (23 FC, 4 oclusiones de cordón y 2 FC seguidas de oclusión). La edad gestacional en el procedimiento fue 19 (16-26) semanas y en el 86% de los casos se trataba de un estadio de Quintero ≥ II. El 70% de las madres tuvieron al menos 1 recién nacido vivo, con mediana de edad gestacional al parto de 31 (24-37) semanas. Presentaron problemas intestinales 4 pacientes (1 atresia yeyunal, 2 atresias ileales, 1 enterocolitis necrotizante con perforación), con sospecha prenatal diagnóstica en 2 de ellos. Postnatalmente se realizó resección del segmento afecto y ostomía. Actualmente se ha restablecido el tránsito intestinal en todos sin secuelas digestivas graves tras 21(8-38) meses de seguimiento. CONCLUSIONES: Hemos descrito distintos tipos de complicaciones intestinales asociadas al STFF y/o su tratamiento con FC. Es posible hacer el diagnóstico prenatal de dichas complicaciones mediante ecografía y RM. Su conocimiento pone al cirujano en alerta y es importante en la toma de decisiones postnatales.

[Primary versus secondary closure: ventilatory and nutritional differences in patients with gastrosquisis]. / Cierre primario frente a cierre diferido: diferencias ventilatorias y nutricionales en los pacientes con gastrosquisis.

AIM OF THE STUDY: Optimal surgical treatment of patients with gastroschisis remains controversial. Recent studies suggest better outcomes with secondary closure techniques (surgical or preformed silo). The purpose of the study is to identify differences in outcome of infants treated with traditional primary closure (PC) versus surgical silo (SS). PATIENTS AND METHODS: Retrospective study of patients primarily treated of gastroschisis between 2004 and 2014. Patients were divided in PC and SS according to abdominal wall closure. Non-parametric statistical analysis was used with p< 0.05 regarded as significant. RESULTS: Twenty-seven patients were included (14M/13F). Primary closure was performed on 17 and 10 underwent surgical silo placement with a median of 6 (5-26) days till secondary closure. Prenatal diagnosis was available in most patients (74%) by the 20th week of gestation. There were no significant differences regarding sex, gestational age or birthweight between groups. Fewer ventilation days were required in PC group compared to SS (4 vs 13, p< 0,05), however, there was no difference in type of ventilation or oxygen needs. Sedation and parenteral nutrition requirements were also lower in PC patients 4 vs 10 and 12 vs 20 days respectively (p< 0,05). Post-operative complications (5vs6) and median length of stay (36vs43 days) were also similar in PC and SS patients. One patient ultimately died due to catheter-related sepsis. Mean length of stay in hospital was 42 days (20-195). CONCLUSION: Patients with gastroschisis who underwent primary closure showed shorter ventilator support and PN dependency than those treated with surgical silo. However, SS is as safe and effective technique as PC and led to similar outcome regarding digestive autonomy and hospital length of stay.

INTRODUCCION/OBJETTIVO: El tratamiento óptimo de la gastrosquisis es controvertido. Algunos autores sugieren mejores resultados del cierre diferido (CD) frente al cierre primario (CP). El propósito del estudio es comparar las necesidades de ventilación mecánica y sedación, así como los aspectos nutricionales y resultados a medio plazo entre el CP y CD. PACIENTES Y METODOS: Estudio retrospectivo de pacientes con gastrosquisis entre 2004 y 2014. Se dividieron en CP y CD según el cierre de pared abdominal. RESULTADOS: Se incluyeron 27 pacientes (14V,13M). En 74% se realizó el diagnóstico prenatal antes de las 20 semanas de gestación. La edad gestacional y peso al nacimiento fueron 36 (31-39) semanas y 2.200 (1.680-3.150) gramos, respectivamente. Se realizó CP en 17 (63%) y a los 6 (5-26) días, un CD en 10 (37%). El grupo con CP precisó menos días de ventilación mecánica que el CD (4 vs 13, p< 0,05), sin diferencias en el tipo de soporte ni requerimiento de oxígeno. La necesidad de sedación y de nutrición parenteral (NP) fueron también menores en el grupo de CP 4 vs 10 y 12 vs 20 días, respectivamente, p< 0,05, pero sin diferencias para alcanzar la autonomía digestiva. Once (41%) presentaron complicaciones postoperatorias, distribuidas por igual en ambos grupos. Un paciente del grupo CD falleció por sepsis asociada a catéter central. La estancia mediana hospitalaria fue de 42 días (20-195). CONCLUSION: Los pacientes con CP de gastrosquisis requirieron menos soporte ventilatorio, sedación y NP exclusiva. Sin embargo, nuestra experiencia demuestra que el CD es una técnica segura que presenta resultados similares al CP en cuanto a estancia hospitalaria y autonomía digestiva.

Bladder changes after several coverage modalities in the surgically induced model of myelomeningocele in lambs.

OBJECTIVE: To assess the presence of early bladder abnormalities in a prenatally corrected and uncorrected animal model of Myelomeningocele (MMC). METHOD: A MMC-like lesion was surgically created in 18 fetal lambs between the 60th and the 80th day of gestation. Eight of them did not undergo fetal repair (group A), three were repaired with an open two-layer closure (group B), three using BioGlue® (groupC) and four fetoscopically (group D). At term, bladders were examined macroscopically and histopathological changes were assessed using H-E and Masson Trichrome. RESULTS: Five animals in group A (5/8, 62%), two in group B (2/3, 66%), one in group C (1/3, 33%) and one in group D (1/4, 25%) survived. Macroscopically bladders in group A were severely dilated and showed thinner walls. Microscopically they showed a thin layer of colagenous tissue (Blue layer. BL) lying immediately subjacent to the urothelium. The muscular layers were thinner. Non compliant pattern with thick wall and low capacity was also found in the non corrected model. Group B and the control showed preservation of muscular layers and absence of BL. Groups C and D presented BL but also preservation of muscular layers. CONCLUSION: Bladder changes in a surgically-induced model of MMC can be described using histopathological data. Both extremes of bladder changes can be observed in the model. These changes were completely prevented with open fetal surgery and partially with other coverage modalities.

Modelo experimental de brida amniótica en ratas: descripción del modelo y estudio morfológico inicial / Experimental model of amniotic band in rats: model description and initial morphological study

Objetivo. Describir un nuevo modelo experimental de brida amnió tica en ratas y mostrar los resultados morfológicos iniciales. Material y métodos. El modelo se creó mediante ligadura con seda de una pata trasera de los fetos el día 17 de gestación; en determinados fetos la ligadura fue retirada el día 19 de gestación. Los fetos se extrajeron mediante cesárea el día 21 de gestación, analizando sus pesos y las características morfológicas de sus patas traseras. Utilizamos 10 ratas SD gestantes, con un peso de 263 g (231-279). Se analizaron 113 fetos repartidos en 6 grupos: fetos de ratas control (n=28), apertura y cierre uterino (n=7), creación de brida (n=28), creación y retirada de la brida (n=14) y fetos a los que no se intervino pero formaban parte de las camadas de los grupos en que se creó la brida (n=19) y de los que se creó y se retiró (n=17). Las comparaciones entre grupos se realizaron mediante test paramétricos considerando significativas diferencias con p<0,05.Resultados. Los fetos intervenidos (ligadura y ligadura con retirada) mostraron diferencias estadísticamente significativas en el peso (medias=4,56 g y 4,4 g) y longitud de patas traseras (medias=3,97 mm y 5,34 mm) respecto al resto de grupos, no así el grupo de apertura y cierre uterino sin actuación sobre el feto. La tasa de abortos en fetos intervenidos fue del 16,6% y la de amputaciones del 8,3%.Discusión. Se describe un modelo experimental viable y reproducible de bridas amnióticas, con posibles aplicaciones, tanto en el estudio de la enfermedad como en el papel de la cirugía fetal sobre la misma (AU)

Purpose. Our objective is to describe a new experimental model of amniotic band in rats and to show the initial morphological results. Methods. The model was created by ligation of a hind limb with silk on day 17 of gestation; in selected fetuses ligation was withdrawn on day 19 of gestation. Fetuses were removed by cesarean section on day 21 of gestation, analyzing their weights and morphological charcteristics of their hind legs. We used 10 pregnant SD rats, weighing 263 g (231-279). One hundred and thirteen fetuses were analyzed divided into 6 groups: fetuses from control rats (n=28), uterine opening and closing (n=7), band creation (n=28), band creation and subsequent removal (n=14) and not intervened fetuses of the litters in which the band was created (n=19) and that the band was created and removed (n=17). Comparisons between groups were made using parametric tests considering p<0.05 as a threshold for significance. Results. Operated fetuses (band creation and band creation and removal) showed statistically significant differences in weight (mean=4.56 g and 4.4 g) and length of their hind limbs (mean=3.97 mm and 5.34 mm) compared to the rest of the groups. The rate of abortions in operated fetuses was 16.6% and the rate of amputations was 8.3%.Discussion. A viable and reproducible experimental model of amniotic band is described, with potential applications in both the study of the disease and the role of fetal surgery thereon (AU)

Preservation of the native spleen in multivisceral transplantation.

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

[Experimental model of amniotic band in rats: model description and initial morphological study]. / Modelo experimental de brida amniótica en ratas: descripción del modelo y estudio morfológico inicial.

PURPOSE: Our objective is to describe a new experimental model of amniotic band in rats and to show the initial morphological results. METHODS: The model was created by ligation of a hind limb with silk on day 17 of gestation; in selected fetuses ligation was withdrawn on day 19 of gestation. Fetuses were removed by cesarean section on day 21 of gestation, analyzing their weights and morphological characteristics of their hind legs. We used 10 pregnant SD rats, weighing 263 g (231-279). One hundred and thirteen fetuses were analyzed divided into 6 groups: fetuses from control rats (n=28), uterine opening and closing (n=7), band creation (n=28), band creation and subsequent removal (n=14) and not intervened fetuses of the litters in which the band was created (n=19) and that the band was created and removed (n=17). Comparisons between groups were made using parametric tests considering p<0.05 as a threshold for significance. RESULTS: Operated fetuses (band creation and band creation and removal) showed statistically significant differences in weight (mean=4.56 g and 4.4 g) and length of their hind limbs (mean=3.97 mm and 5.34 mm) compared to the rest of the groups. The rate of abortions in operated fetuses was 16.6% and the rate of amputations was 8.3%. DISCUSSION: A viable and reproducible experimental model of amniotic band is described, with potential applications in both the study of the disease and the role of fetal surgery thereon.

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

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

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

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

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

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

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

Parafollicular C-cells of the thyroid are decreased in patients with congenital diaphragmatic hernia.

BACKGROUND: Parathyroid and thymic anomalies related to embryonic neural crest dysfunction have been demonstrated in rats with congenital diaphragmatic hernia (CDH). These rats, like infants with CDH, have conotruncal, facial, and other neurocristal defects. The present study examines whether parafollicular C-cells (CC) of the thyroid, whose embryogenesis is related to that of the parathyroids and thymus, might also be abnormal in babies with CDH. MATERIAL AND METHODS: Autopsy sections of the thyroids of 12 babies dead from CDH and of 11 controls were stained with anti-calcitonin antibodies. Calcitonin-stained areas and the histological distribution of CC within the thyroid gland were assessed. Mann-Whitney tests were used for comparison, with p<0.05 considered significant. RESULTS: The proportion of stained surface to total thyroid surface was significantly smaller in CDH babies than in controls (0.035±0.030% vs. 0.072±0.052%, p<0.05). A normal central CC location was demonstrated in both groups. CONCLUSIONS: Parafollicular thyroid C-cells are deficient in patients with CDH. These findings further support the involvement of neural crest dysregulation in the pathogenesis of CDH and the pertinence of using this experimental model to investigate the human condition. The clinical effects of this anomaly are unknown and probably irrelevant, but they are currently under scrutiny.

Microscopic magnetic resonance imaging of the thoracic venous system in rats with congenital diaphragmatic hernia.

BACKGROUND/AIM: Infants and rats with congenital diaphragmatic hernia (CDH) have malformations of the heart and the great arteries caused by neural crest (NC) dysregulation during embryogenesis. Abnormally narrow jugular veins have been found in babies during cannulation for ECMO. However, the venous system has not been examined in depth so far. We hypothesized that abnormal patterning and/or size of the thoracic veins could occur in rats with CDH. This hypothesis was tested by microscopic magnetic resonance imaging (MMRI), a high-resolution tool able to detect subtle changes of vessels in small animals. MATERIAL/METHODS: Fetuses from pregnant rats fed either 100 mg i.g. nitrofen or vehicle on E9.5 were recovered near term. A 7 T MMRI system with a coronal multislice fast spin echo sequence allowed diagnosis of CDH (n = 19), and T2 SE high-resolution sequences made assessment of the pattern and width of cervico-thoracic veins possible. Values were corrected for body size by dividing them by the length of thoracic vertebrae T3-T5. The results in nitrofen and control (n = 11) groups were compared by non-parametric tests (*p < 0.05). RESULTS: Congenital diaphragmatic hernia fetuses were smaller than controls (4.5 ± 0.26 vs. 5.3 ± 0.2 g*). The widths (corrected for body size) of left external, both innominate, right superior vena cava and azygos veins were significantly smaller in CDH rats than in controls. CONCLUSIONS: The cervico-thoracic veins are normally patterned but abnormally narrow (except the internal jugulars) in rats with CDH. The same embryonic NC dysregulation that accounts for cardiovascular malformations could also explain these venous anomalies in CDH.

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.

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

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

Aim. To analyze the benefits of Split (for adult and for child) in liver transplantation. Patient/methods. 1) Analysis of the waiting list mortality estimated on 228 inclusions for transplant since January 2004 to December2008. 2) Impact of the variant techniques (living-related donor and split)on the waiting list mortality in our patients. 3) Analysis of the outcome of 33 split livers which allowed to perform 66 transplants (1994-2008). Results. Estimated as number of patients by 1,000 candidates by year of exposure, the waiting list mortality was 110 in children older than5 year old, 180 in children from 2 to 5 year-old, 90 in children between1 and 2 year-old and 510 in younger than 1 year (p<0.05 for the last group). 36/66 split grafts were implanted by our group. Five grafts were (..) (AU)