[Use of mitomycin C in esophageal stenosis]. / Empleo de la mitomicina C en las estenosis esofágicas.

INTRODUCTION: Esophageal stenosis, due to its important implications on the patients' quality of life, poses a serious problem, often difficult to resolve, that requires therapeutical solutions that could irreversibly affect vital conditions and quality of life of those affected. For this reason our group has tried over the last thirty years to employ conservative solutions in the damaged aesophagus, showing that a complete cure is possible in most cases although the way to achieve this can be dramatically long. MATERIAL AND METHODS: We present our experience employing topical Mitomicyn C over the last year and a half in eight patients, six of them affected with caustic stenosis and two with secuelae of esophageal atresia, highlighting the three cases that received at least five applications of the product. We describe the method employed that consists in the spraying of the correct dose using a flexible fibroscope on the dilated area, loading the dose in the container of the cleaning water of the fibroscope, employing a system that permits only to apply the exact quantity necessary of the product. The application time is five minutes and the dose is 0.4 mg/ml applying 5ml or 10 ml according to the patient's weight ( under 12 kg = 2.5 ml and over 25 kg=5 ml). Upon completion of the application, a washing of the product is performed using the same fibroscope. RESULTS: From the eight cases in which it was applied, we analysed the three that received at least five doses. These were two boys of seven and eight years and a 25 year-old woman. In the children the dilatations interval has passed from 4-5 weeks to 8-11 respectively. The woman has passed from 12 to15 weeks. The children have been in the dilatation program more than 3 years and the woman more than 18. CONCLUSIONS: Although the time period that we have been applying Mitomicyn C is still short, the symptom-free interval after dilatations is proving to be highly promising. This fact is highlighted in those patients included in the dilatation program recently. We have not seen any adverse side effect from the application of the product.

Empleo de la mitomicina C en las estenosis esofágicas / Use of mitomicyn C in esophageal stenosis

Introducción. Las estenosis esofágicas por las importantes implicaciones que tienen en la calidad de vida de los pacientes constituyen un grave problema, en general de difícil solución, que obliga a tomar decisiones terapéuticas que pueden condicionar de un modo irreversible las condiciones vitales y el confort de aquellos que las padecen. Por ese motivo, nuestro grupo optó desde hace más de 30 años por soluciones conservadoras sobre el esófago lesionado, demostrando que la curación completa es posible en la mayoría de los casos aunque el camino para conseguirla puede ser en ocasiones desesperantemente largo. Material y métodos. Presentamos el empleo tópico de mitomicina C en el ultimo año y medio en ocho pacientes afectos, seis de ellos de estenosis de tipo cáustico y dos casos de atresia de esófago, centrándonos fundamentalmente en los tres casos que han recibido al menos cinco aplicaciones del producto. Se describe el método empleado que consiste en el rociado de la dosis adecuada empleando un fibroscopio flexible sobre el área dilatada, cargando la dosis en el frasco de líquido de lavado del fibroscopio mediante un sistema que impulsa sólo la cantidad de producto deseado. El tiempo de aplicación es de cinco minutos y la dosis es de 0,4 mg/ml aplicando 2,5 o 5 ml según el peso del paciente (5 ml peso > 12 kg y 2,5 ml peso < 12 kg). Completada la aplicación se efectúa un lavado del producto con el mismo fibroscopio. Resultado. De los ocho casos en los que se ha aplicado, se analizan los tres que han recibido al menos cinco dosis. Se trata de dos niños de 7 y 8 años y una mujer de 25. En los niños el intervalo de dilataciones ha pasado de 4 y 5 semanas a 8 y 11 respectivamente. La mujer ha pasado de 12 a 15 semanas. Los niños llevaban más de tres años en programa de dilatación y la mujer más de 18. Conclusiones. Aunque el tiempo que llevamos aplicando la mitomicina C es todavía muy corto, resulta altamente prometedor el incremento del intervalo de tiempo libre de síntomas tras las dilataciones, hecho especialmente destacable en los pacientes con menos tiempo en programa de dilatación. No hemos evidenciado ningún efecto secundario tras la aplicación (AU)

Introduction. Esophageal stenosis, due to its important implications on the patients’ quality of life, poses a serious problem, often difficult to resolve, that requires therapeutical solutions that could irreversibly affect vital conditions and quality of life of those affected. For this reason our group has tried over the last thirty years to employ conservative solutions in the damaged aesophagus, showing that a complete cure is possible in most cases although the way to achieve this can be dramatically long. Material and Methods. We present our experience employing topical Mitomicyn C over the last year and a half in eight patients, six of them affected with caustic stenosis and two with secuelae of esophageal atresia, highlighting the three cases that received at least five applications of the product. We describe the method employed that consists in the spraying of the correct dose using a flexible fibroscope on the dilated area, loading the dose in the container of the cleaning water of the fibroscope, employing a system that permits only to apply the exact quantity necessary of the product. The application time is five minutes and the dose is 0.4 mg/ml applying 5ml or 10 ml according to the patient’s weight ( under 12 kg = 2.5 ml and over 25 kg=5 ml). Upon completion of the application, a washing of the product is performed using the same fibroscope. Results. From the eight cases in which it was applied, we analysed the three that received at least five doses. These were two boys of seven and eight years and a 25 year-old woman. In the children the dilatations interval has passed from 4-5 weeks to 8-11 respectively. The woman has passed from 12 to15 weeks. The children have been in the dilatation program more than 3 years and the woman more than 18. Conclusions. Although the time period that we have been applying Mitomicyn C is still short, the symptom-free interval after dilatations is proving to be highly promising. This fact is highlighted in those patients included in the dilatation program recently. We have not seen any adverse side effect from the application of the product (AU)

[Hirschsprung-neurointestinal dyspasia: diferential diagnosis and reliability of diagnostic procedures]. / Diagnóstico diferencial Hirschsprung-neurodisplasia intestinal. Fiabilidad de las pruebas diagnósticas.

The suspicion of Hirschsprung's disease (HD) or Neurointestinal Dysplasia (NID) arises with the appearance of a common symtomatology : delay in meconium evacuation, abdominal distention, vomiting, intestinal occlusion and ultimately, chronic constipation. The need to perform a correct differential diagnosis between both pathologies is essential, given that the treatment of HD is surgical while NID is expectant. The objective of this paper is to define the necessity of using or not all the diagnostic tests simultaneously to obtain a precise diagnosis. The authors performed a complete clinical review of a series of 42 HD and 18 NID analysing the results of diagnostic procedures (Radiology / transition zone, anorrectal manometry / absence of anal inhibitory reflex, and suction biopsy / AcHE study). The authors concluded that is necessary to perform all the three diagnostic procedures simultaneously in all patients with symtomatology given that not one test has the capacity to provide a diagnosis alone. Anorectal manometry has proven to be the most reliable test to diagnose HD. The authors agree with the guidelines published by Meier Ruge in 2004 to diagnose NID.

Diagnóstico diferencial Hirschsprung-neurodisplasia intestinal. Fiabilidad de las pruebas diagnósticas / Hirschsprung-neurointestinal dysplasia: differential diagnosis and reliability of diagnostic procedures

La sospecha de enfermedad de Hirschsprung (EH) o de neurodisplasia intestinal (NID) se produce ante la aparición de una misma sintomatología: retraso en la evacuación meconial, distensión abdominal, vómitos, oclusión-suboclusión, o más tardíamente estreñimiento crónico. La necesidad de realizar un diagnóstico diferencial correcto entre ambas patologías es imprescindible, dado que el tratamiento de la EH es quirúrgico y el de la NID, madurativo. El objetivo de este trabajo es conocer la necesidad de utilizar o no todas las pruebas diagnósticas simultáneamente para obtener un diagnóstico diferencial correcto. Se realiza una revisión de las historias clínicas en una serie de 42 EH y 18 NID, analizando los resultados de las pruebas diagnósticas (radiología- zona de transición, manometría anorrectal-ausencia reflejo inhibidor de ano, biopsia por succión-tinción de AChE). Se concluye la necesidad, hoy por hoy, de realizar las tres pruebas diagnósticas simultáneamente en todos los pacientes con sintomatología, ya que ninguna tiene la capacidad completa de orientar hacia un diagnóstico, aunque sí se ha mostrado en este trabajo como de mayor fiabilidad la manometría anorrectal en el diagnóstico de la enfermedad de Hirschsprung. Los autores se adhieren a los criterios publicados por Meier Ruge en 2004 para el diagnóstico de la NID (AU)

The suspicion of Hirschsprung’s disease (HD) or Neurointestinal Dysplasia (NID) arises with the appearance of a common symtomatology : delay in meconium evacuation, abdominal distention, vomiting, intestinal occlusion and ultimately, chronic constipation. The need to perform a correct differential diagnosis between both pathologies is essential, given that the treatment of HD is surgical while NID is expectant. The objective of this paper is to define the necessity of using or not all the diagnostic tests simultaneously to obtain a precise diagnosis. The authors performed a complete clinical review of a series of 42 HD and 18 NID analysing the results of diagnostic procedures ( Radiology / transition zone, anorrectal manometry / absence of anal inhibitory reflex, and suction biopsy / AcHE study ). The authors concluded that is necessary to perform all the three diagnostic procedures simultaneously in all patients with symtomatology given that not one test has the capacity to provide a diagnosis alone. Anorectal manometry has proven to be the most reliable test to diagnose HD. The authors agree with the guidelines published by Meier Ruge in 2004 to diagnose NID (AU)

[Biliary atresias operated with favourable results: predictable outcome]. / Atresias de vías biliares intervenidas con buen resultado. Evolución previsible.

Since 1975, our experience in the treatment of biliary atresia with Kasai's technique has improved little by little, achieving 65% favourable outcome in the last five years. We define "good results" as the complete restoration of biliary flow and normalization of bilirrubin levels. The long-term evolution of these good results can be diverse. The objective of the present work is to analyze the outcome of patients in our series in whom a favourable initial response was achieved, as well as evaluating their present situation and future perspectives. The authors present a total of 17 patients operated by Kasai's technique since 1985, that constitutes the group with good results in our series. The controls were based on general analysis, liver function and periodic ultrasound explorations. All received a standardized medical treatment consisting of vitamin supplements (A, D3, E, K) minerals (zinc, calcium, phosphate, iron) ursodexoxicolic acid, luminal,as well as close control of calorie intake. In two patients the levels of bilirrubine were progressively increased with time, stabilizing at between 5/6 mgs/100 ml, with progressive hepatic hardening, appearance of splenomegalia, indirect signs of portal hypertension and a slight deterioration of hepatic function. One received a transplant at age 12 with Quick levels below 50%. The other, aged 16, continues with an acceptable hepatic function and good quality of life under recommendation of transplant. Eleven patients with ages ranging from fourteen months to seventeen years presented slight and firm hepatomegalia, moderate portal hypertension, GOT 71 +/- 8 mg/100 ml, GPT 97 +/- 11 mg/100 ml and normal bilirrubine levels. From this group, 3 patients, all under five years of age, experienced bleeding from esophageal varices which were controlled by sclerosis and medical treatment (propanolol and isosorbide dinitrate). Recently, one three year-old patient developed a hepatocarcinoma of rapid, mortal evolution. Since then, the determination of alfa-feto protein in follow-up controls has been introduced. Four other patients of 5, 6, 14, 16, years of age are completely assymptomatic with an excellent clinical evolution. In our experience,the patients that overcome the third year after surgery without serious complications seem destined to reach puberty with a good quality of life. However, some cases show signs of hepatic fibrosis and portal hypertension, 77% in our series. Only 23% of patients with a favorable initial evolution appear to present a complete normalization of their hepatic lesion in the long term.

Atresias de vías biliares intervenidas con buen resultado. Evolución previsible / Biliary atresias operated with favourable results: predictable outcome

Nuestra experiencia en el tratamiento de la atresia de vías biliares mediante la técnica de Kasai ha ido mejorando con el tiempo hasta conseguir en los últimos 5 años un 65% de «buenos resultados», incluyendo en ese término a pacientes con restauración del flujo biliar y normalización de las cifras de bilirrubina. El objetivo del presente trabajo es analizar el comportamiento de los pacientes de nuestra serie en los que se consiguió ese buen resultado inicial, valorar su situación en el momento actual y sus perspectivas de futuro. Presentamos un total de 17 pacientes intervenidos mediante técnica de Kasai desde 1985, con la característica común de pertenecer al grupo de buenos resultados de nuestra serie. Los controles se basaron en analítica general, función hepática y ecografías periódicas. A todos ellos desde el momento del diagnóstico se les aplicó un tratamiento médico estandarizado consistente en suplementos polivitamínicos (A, D3, E, K) minerales, zinc, calcio, fósforo, hierro, ácido ursodesoxicólico y luminal y una vigilancia estricta de los aportes calóricos. En dos pacientes se elevaron progresivamente las cifras de bilirrubina estabilizándose entre 5/6 mg/100 ml con progresivo endurecimiento hepático, aparición de esplenomegalia, signos indirectos de hipertensión portal y deterioro relativo de la función hepática. Uno de ellos con cifras de Quick inferiores al 50% se trasplantó a los 12 años de vida. El otro con 16 años continúa con una función hepática aceptable y buena calidad de vida en situación de trasplante aconsejable. Un grupo de 11 pacientes con edades comprendidas entre los 14 meses y los 17 años presentan discreta hepatomegalia firme, moderada hipertensión portal, moderada elevación de las transaminasas y bilirrubina normal. De este grupo 3 pacientes, siempre por debajo de los cinco años, han tenido sangrados por varices esofágicas controlados mediante esclerosis y tratamiento médico (propanolol y dinitrato de isosorbide). Un paciente desarrolló un hepatocarcinoma al tercer año de vida de evolución rápidamente fatal. Desde entonces se introdujo la determinación de alfa-fetoproteína en los protocolos de seguimiento. Cuatro pacientes de 5, 6, 14 y 16 años están clínica y exploratoriamente asintomáticos y previsiblemente continuarán así a largo plazo. En nuestra experiencia, los pacientes que superan sin incidencias graves el tercer año postoperatorio, parecen destinados a sobrepasar la pubertad con buena calidad de vida. Un 77% con signos de fibrosis hepática e hipertensión portal moderada y un 23% de ellos con la normalización completa de su lesión hepática a largo plazo (AU)

Since 1975, our experience in the treatment of biliary atresia with Kasai’s technique has improved little by little, achieving 65% favourable outcome in the last five years. We define «good results» as the complete restoration of biliary flow and normalization of bilirrubin levels. The long-term evolution of these good results can be diverse. The objective of the present work is to analyze the outcome of patients in our series in whom a favourable initial response was achieved, as well as evaluating their present situation and future perspectives. The authors present a total of 17 patients operated by Kasai’s technique since 1985, that constitutes the group with good results in our series. The controls were based on general analysis, liver function and periodic ultrasound explorations. All received a standardized medical treatment consisting of vitamin supplements (A, D3, E, K) minerals (zinc, calcium, phosphate, iron) ursodexoxicolic acid, luminal,as well as close control of calorie intake. In two patients the levels of bilirrubine were progressively increased with time, stabilizing at between 5/6 mgs /100 ml, with progressive hepatic hardening, appearance of splenomegalia, indirect signs of portal hypertension and a slight deterioration of hepatic function. One received a transplant at age 12 with Quick levels below 50%.The other, aged 16, continues with an acceptable hepatic function and good quality of life under recommendation of transplant. Eleven patients with ages ranging from fourteen months to seventeen years presented slight and firm hepatomegalia, moderate portal hypertension, GOT 71 +/- 8 mg/100 ml, GPT 97 +/-11 mg/100 ml and normal bilirrubine levels. From this group, 3 patients, all under five years of age, experienced bleeding from esophageal varices which were controlled by sclerosis and medical treatment (propanolol and isosorbide dinitrate). Recently, one three year-old patient developed a hepatocarcinoma of rapid, mortal evolution. Since then, the determination of alfa- feto protein in follow-up controls has been introduced. Four other patients of 5, 6, 14, 16, years of age are completely assymptomatic with an excellent clinical evolution. In our experience,the patients that overcome the third year after surgery without serious complications seem destined to reach puberty with a good quality of life. However, some cases show signs of hepatic fibrosis and portal hypertension, 77% in our series. Only 23% of patients with a favorable initial evolution appear to present a complete normalization of their hepatic lesion in the long term (AU)

Tratamiento endoscópico de las fístulas traqueoesofágicas. ¿Realidad o ficción? / Endoscopic treatment oftracheo-esophageal fistulas. Fact or fiction?

Las fístulas traqueo esofágicas como secuela de la cirugía de la atresia de esófago suelen ser una severa complicación para cuya corrección se requiere una importante agresión quirúrgica, en ocasiones con resultados inciertos. Tras la aparición en el mercado de diversos productos con capacidad de oclusión la posibilidad de tratar esta problemática mediante el empleo de métodos endoscópicos de menor agresividad ha estado presente en la literatura médica en los últimos años, con resultados discordantes en las diferentes publicaciones. Presentamos nuestra experiencia en la oclusión endoscópica sobre tres casos de fístulas traqueo esofágicas. Describimos la modalidad técnica de abordaje endoscópico transtraqueal que se ha basado como novedad en el empleo de la mascarilla laríngea lo cual facilita la ventilación durante la realización del procedimiento y la posibilidad de utilizar material endoscópico de mayor diámetro. Se comentan los diferentes materiales de oclusión empleados (Tissucol e Histoacryl) y las dificultades de su manejo. Se han efectuado en un caso dos intentos de oclusión y tres en los otros doscasos variando el modo de aplicación y el producto. En dos casos las fístulas se han repermeabilizado inicialmente y en otro se consiguió una oclusión demostrable mediante esofagograma y con remisión de los síntomas respiratorios durante ocho meses. En un control posterior reaparece una fístula filiforme manteniendo la mejoría clínica. La posibilidad de empleo de técnicas endoscópicas inicialmente nos pareció muy prometedora para este tipo de patología. La aparición en el mercado de diversas sustancias con capacidad de oclusión nos permitió su empleo comprobando las evidentes ventajas de la mascarilla laríngea en este tipo de actuaciones. Los resultados muestran la completa inefectividad de unas y las posibilidades relativas de otras aunque en nuestra experiencia no se ha conseguido ninguna oclusión definitiva aunque si mejorías transitorias (AU)

[Nineteen years experience with posterior sagittal anorectoplasty as a treatment of anorectal malformation]. / Diecinueve años de anorrectoplastia sagital posterior como tratamiento de la malformación anorrectal.

UNLABELLED: Posterior sagittal anorectoplasty (PSARP) is considered today the best surgical technique for the treatment of the anorectal malformations. With the aim of evaluating the results of the PSARP in our patients, the charts of 39 children (27 male, 12 female) were reviewed. Mean age was 11 years (3 to 20 years) and the mean follow-up period from the closure of the colostomy was 9.4 years (1 to 18 years). Patients with any alteration of the extrinsic innervation or those with rare malformations were excluded of the study. According to the Wingspread classification, 14 patients had a high defect, 13 had an intermediate type and 12 had a low one. One patient had a PSARP in the newborn period whereas the rest of them had a descending loop colostomy. Definitive repair consisted in PSARP in low and intermediate forms and in six of the high type patients. The rest (8) of the high type patients had a Rehbein abdominoperineal pull-through (DA) plus a PSARP. Evaluation for the surgical results was made by means of an anorectal manometry (existence or not of anal inhibitory reflex [RIA], the symmetry and pressure of the canal anal, and the existence of extrinsic innervation) and a clinical questionnaire. Patients or parents were asked for fecal continence, age at which the continence was achieved and the existence of severe constipation. RESULTS: Low defects: Eleven patients were continent between 3 and 5 years old. Seven patients had RIA. Intermediate defects: Seven patients were continent. Five (all with RIA) achieved continence between 2 and 8 years old; the other 2 were continent at the age of 10. The six incontinent patients had absence of RIA and/or low-pressure anal canal. High defects: Five patients were continent, between 11 and 16 years old. All had an DA plus PSARP. None of them had RIA. All the incontinent patients had an anomalous anal canal. CONCLUSIONS: The lowest the type of an anorectal malformation, the better the prognoses. In these patients, there is a relationship between the achievement of continence, the presence of RIA and the symmetry and high pressures at the anal canal. Among the patients with high defects, the results are better when they had an abdominoperineal pull-through plus PSARP.

Total anterior urethral reconstruction with the 'BAES flap' in a spinal cord-injured patient.

STUDY DESIGN: A case report of urethral destruction in a spinal cord injured (SCI) patient. OBJECTIVES: To report the reconstruction of the whole anterior urethra in one-stage using an epilated scrotal flap. SETTING: Institut Guttmann, Hospital de Neurorehabilitació, Barcelona, Spain. METHODS: A one-stage tubular substitution urethroplasty based on a bi-axial epilated scrotal flap design ('BAES flap') was performed successfully. RESULTS: Long-term follow-up of 6 years has confirmed the excellent adaptation of the flap to its urethral function. CONCLUSION: The bipedicle epilated scrotal flap can effectively resolve this challenging urethral pathology.

Diecinuieve años de anorrectoplastia sagital posterior como tratamiento de la malformación anorrectal / Nineteen years experience with posterior sagittal anorectoplasty as a treatment for anorectal malformation

La anorrectoplasia sagital posterior (PSARP) es actualmente la técnica quirúrgica de elección para el tratamiento de las malformaciones anorrectales. Se han valorado 39 pacientes; según la clasificación de Wingspread, 14 fueron formas altas, 13 intermedias y 12 bajas. Con el objetivo de evaluar sólo el resultado de la intervención, fueron excluidos del estudio los casos con alteraciones de la inervación extrínseca y las malformaciones raras. En un caso se practicó una sigmoidostomía. Para la corrección definitiva se practicó una PSARP en las formas bajas e intermedias, y en 6 de las altas; en las 8 altas restantes se reaizó un descenso abdominal tipo Rehbein (DA) asociado a una PSARP. Media de edad, 11 años (rango de 20 a 3), con 9,4 años de media transcurridos desde el cierre de la derivación hasta la actualidad (rango de 18 a 1 año). Se realizó manometría anorrectal valorando la existencia de reflejo inhibidor de ano (RIA), inervación extrínseca, y perfil del canal anal (PCA). Clínicamente se valoró la continencia y la edad de su adquisión, así como la existencia de estreñimiento grave. Resultados. Formas bajas (12 casos): En 11 se obtuvo continencia entre los 3 y 5 años. en 7 se identificó RIA. Formas intermedias (13 casos): En 7 se obtuvo continencia; de ellos, 5 entre los 2 y 8 años; todos con RIA; los otros dos la adquirieron a los 10 años. Los seis incontinentes están relacionados con ausencia de RIA o PCA con baja presión, situándose su edad actual entre los 9 y los 12 años. Formas altas (14 casos): En 8 se realizó DA + PSARP, y en 6 PSARP. Adquirieron continencia 5 de ellos entre los 11 y 16 años de vida; todos del primer grupo. En ninguno se desmostró RIA. Todos los incontinentes presentan alteraciones del PCA, hallándose entre los 8 y 20 años de edad. Conclusiones. El pronóstico de las MAR, en cuanto a la adquisición de continencia, es mejor cuanto más baja es la malformación. La continencia está relacionada con la presencia de RIA y con el valor de presión y la simetría del canal anal. Se han obtenido mejores resultados en cuanto a continencia en las formas altas que fueron intervenidas añadiendo a la PSARP un descenso abdómino-perineal (AU)

[Treatment of caustic stenosis of the esophagus with self-expanding devices]. / Tratamiento de las estenosis cáusticas del esófago mediante dispositivos de autoexpansión.

Caustic stenosis is a serious problem in children due to its complicated resolution and implications in important areas like nutrition, as well as the child's tolerance to the measures taken to correct them. After dealing extensively with this problem over the last twenty five years, always from a conservative approach using traditional methods like dilatations, we believe we have found a technique that brings together all the necessary conditions to achieve a favorable and definitive solution. Our ideal goal to achieve a device that provides a well-tolerated permanent esophageal expansion during the scarring process, also permitting normal swallowing, seems to have been reached through the use of the new generation of silicone stents. The authors present their experience in the first seven cases of caustic stenosis treatment through the placement of silicone stents, describing a precise placement technique while establishing a standard protocol for the use of these devices.

Tratamiento de las estenosis cáusticas del esófago mediante dispositivos de autoexpansión / Stenting procedure in the correction of caustic esophageal stenosis in children

Los autores presentan su experiencia en un nuevo método de tratamiento de las estenosis cáusticas de esófago en los niños.Se baca en la colocación de un dispositivo autoexpandible y biocompalible que se coloca mediante un aplicados especial sobre la zona causticada tras realizar una dilatación y se mantiene colocado durante el periodo de cicatrización. El dispositivo permite la alimentación del paciente por vía oral.Se describe el procedimiento técnico de colocación, los controles de seguimiento y mantenimiento y se comentan los resultados preliminares de la serie inicial de 7 pacientes.Todos ellos presentaban severas causticaciones esofágicas, con un tiempo mínimo en programa de dilataciones convencionales que oscilaba entre seis meses y 14 años.La tolerancia a largo plazo al dispositivo fue perfecta en todos los casos. Todos ellos presentaron inicialmente nauseas y vómitos durante periodos de tiempo que oscilaron entre las 12 y 48 horas, siempre en relación con la altura y longitud de la lesión. El tratamiento con sedación y Ondansetrón mejoró los síntomas.A dos pacientes se les retiró el dispositivo a los tres meses y estan libres de síntomas, A otro niño, el mas pequeño de la serie, se le desplazó el dispositivo hasta el estómago a los siete dial, y tuvo que serle retirado por endoscopia. Otra paciente, la mas mayor, sufrió estenosis por encima y debajo del dispositivo en zonas no cubiertas y continua en programa de dilataciones. Tres pacientes siguen en la actualidad el tratamiento con el dispositivo con buena tolerancia (AU)

Atresia de vías biliares: Boceto para una solución / Biliary atresia: Outline for a solution

La atresia de vías biliares continúa siendo una relativamente rara enfermedad (1/50.000 nacidos), cuyo pronóstico a largo plazo ha cambiado drásticamente desde la aparición del trasplante hepático (TH) como arma terapéutica. La combinación de dos factores, diagnóstico precoz y técnica correcta, son imprescindibles para obtener suficiente flujo biliar y resultados aceptables, permitiendo al niño superar la barrera crítica de los 7 kg y situarlo en un rango de peso de menor morbi-mortalidad en relación a un posible TH. A pesar de sus detractores, la técnica de Kasai, tanto en nuestra experiencia, como en largas series revisadas, puede proporcionar supervivencias actuariales a los 10 años que rondan el 50 por ciento con función hepática correcta, normalidad clínica y calidad de vida claramente superior a la del primer año postrasplante. Se presenta la evolución de un grupo de 20 pacientes afectos de atresia de vías biliares diagnosticados y tratados en nuestro centro desde 1985, año en el que el TH pediátrico comenzó a usarse como procedimiento terapéutico en nuestro país. Todos los pacientes, actualmente con un rango de edades entre 2 y 14 años, fueron intervenidos mediante técnica de Kasai. Se valoran la edad de intervención, técnica empleada, resultados inmediatos y tardíos, así como la evolución y la necesidad de TH. Se clasifican los resultados en buenos, regulares o malos en relación al flujo biliar obtenido, normalización de las cifras de bilirrubina y evolución clínica. De los 16 pacientes que presentaron flujo biliar, a 14 los consideramos buenos resultados, pues normalizaron completamente sus cifras de bilirrubina. Otros dos, actualmente de 14 y 8 años, presentan niveles de bilirrubina de 2,5 y 5,5 mg/100 ml, respectivamente, y función hepática aceptable. Los consideramos resultados regulares y están en situación de TH aconsejable. Cuatro pacientes los consideramos malos resultados. Sólo el primero de ellos no presentó ningún flujo biliar y falleció a los 6 meses en espera de TH. Otros tres casos del mismo grupo presentaron insuficiente eliminación biliar y fueron trasplantados con 7, 11 y 12,5 kg de peso, respectivamente. El segundo de ellos falleció en el primer año postrasplante. Conclusiones. En nuestra opinión la actuación con las atresias de vías biliares debe ser lo más precoz posible basada en la correcta aplicación de la técnica de Kasai, intentando conseguir un flujo biliar que elimine o aleje lo más posible en el tiempo la necesidad de un posible TH. Tres líneas convergen para obtener este diseño: un diagnóstico precoz, una correcta ejecución técnica de la portoenterostomía, y una implicación en el tratamiento y seguimiento de estos niños de los grupos de trasplante hepático. Todo esto orienta, como ya se hace en otros países, a la creación de centros de referencia para el estudio de la colestasis neonatal, donde pueda sacarse provecho de la experiencia acumulada en el tratamiento de esta relativamente rara patología (AU)

[Bile duct atresia: outline for a solution]. / Atresia de vías biliares: boceto para una solución.

UNLABELLED: Biliary atresia continues to be a serious and relatively rare disease (1/50,000 newborns) and whose long-term prognosis has changed drastically since the appearance of liver transplant (LT) as a therapeutic weapon. The combination of two factors, early diagnosis and correct application of Kasai's surgical technique, is essential to obtain acceptable results and sufficient biliary drainage allowing the children to overcome the critical 7 kg barrier and place them in the lesser morbi-mortality range in relation to a possible LT. But we must keep in mind that despite its critics, Kasai's technique can guarantee, both in our own experience and in the literature, ten years survival percentages over 50% with correct hepatic function, as well as clinical normality and a quality of life clearly superior to first years post-LT. We present the evolution of a group of 20 patients affected with biliary atresia, diagnosed in our center since 1985, the year when pediatric LT began to be used as a therapeutic procedure in this country. We valued the age of intervention, technique, immediate and long-term results and the evolution and necessity of LT. All 20 patients were analyzed individually, and they currently have an age range from 2-14 years and were all operated by Kasai's technique. We classified the patients as having good, regular or poor results with regards to biliary flow, normalization of billirubin levels and clinical evolution. Sixteen patients presented biliary flow of such an extent that 14 of them, classified as good, completely normalized the billirubin levels. Two others, presently aged 14 and 8 years respectively, present average levels of 2.5-5.5 mg/100 ml and are classified as regular in a situation of advisable transplant, although with an acceptable hepatic function. Only one case, the first in the poor group, did not initially present biliary elimination and died at age six months while on the waiting list. Three other cases in the same group presented insufficient biliary elimination and were transplanted with 7, 11 and 12.5 kg, respectively. The second died in the first year post-transplant. CONCLUSIONS: In our opinion, action in biliary atresia must be early and based on the correct application of Kasai's technique, seeking to achieve a biliar flow that eliminates or distances the patient as far as possible from the necessity of a future LT. Three lines come together to obtain this target: an early diagnosis, a correct application of Kasai's technique, and an implication in the follow-up and treatment of these children by the hepatic transplant groups. All this advises us, as is done in other countries, to create reference centers for the study of neonatal cholestasis where an accumulated experience of a relatively rare pathology can be taken advantage of.

[Hydraulic prosthesis for regulating the sling tension in the treatment of urinary incontinence in women]. / Prótesis hidráulica para regular la tensión del "sling" en el tratamiento de la incontinencia urinaria en la mujer.

OBJECTIVE: To improve the results of corrective surgery for severe urinary incontinence in the female (sphincteric lesion) utilizing a new prosthesis that allows adjusting the degree of tension of the band or urethrocervical sling. METHODS: We have added the use of a subcutaneous hydraulic device to the classical pubovaginal sling procedure. The device can be gradually filled by percutaneous punction to adjust the urethral closure pressure. RESULTS: All patients had previously undergone different procedures and had grade III stress urinary incontinence. With the hydraulic device, the success rate (complete continence) was 95.5% (21/22 patients) and the complication rate was 9% (2 cases; one developed infection and the other chronic urinary retention). CONCLUSIONS: The hydraulic device described herein is easy to place, low-cost and carries a minimum morbidity. Its use is indicated in patients who have previously undergone surgery, with a sphincteric lesion and who require precise adjustment of the urethrocervical sling. The degree of tautness can be adjusted repeatedly according to the clinical course of a patient without requiring reoperation.

[Intraesophageal stent in the prevention of stenosis caused by caustic ingestion]. / Tutor intraesofágico para la prevención de estenosis en las ingestas de cáusticos.

Esophageal stenosis is a frequent consequence of lye ingestion, and their treatment is of longstanding and complex matter. The search of new solutions for this problem is challenging and always attractive if its associated with less aggressiveness to the patient. The use of intraesophageal stents is not new in the treatment of stenosis, but it was anecdotal, until histocompatible material came out in the market. The stent is designed in the OR, having the same length as the stenosis, previously observed by endoscopy, its made out of a silicone tube 30 or 36 French, mounted over an ng tube 16, all this is fixed in the ends of the silicone tube, that way won't slip over the ng tube. It is placed with a laryngoscope assistant, the proximal end of the ng tube comes out the nasal nares. Once the location of the tube is checked, the stent is left in place for six weeks. Our experience, with seven patients, has shown excellent tolerance to the stent; at the third post procedure day the patients were eating soft diet by mouth, we believe that the esophageal spasm had resolved by this day. Once the stent was withdraw, five cases were free of esophageal lesions, in the other two small areas of bleeding were visualized. In the follow-up the five cases that were free of lesions, had a normal esophageal diameter, in the other two, one had a moderate restenosis and the other case was severe.

Achalasia and Down's syndrome: coincidental association or something else?

Achalasia is an uncommon esophageal motor disorder. It has been associated with other diseases such as Parkinson's disease and depressive disorders, but coincidence of achalasia and Down's syndrome is rare. We report five cases of achalasia in Down's syndrome patients seen in our institution. Two of the five cases were diagnosed at pediatric age. Respiratory symptoms and growth retardation were the main clinical manifestations in pediatric patients, whereas adult patients mainly complained of dysphagia. Taking into account the prevalence rate of both disorders, the association seems higher than that expected by chance. The possible etiopathogenic implications of this association, as well as its clinical relevance, are discussed.