Neumonectomía en niños / Pneumonectomy in children

Objetivo. La neumonectomía es raramente necesaria en niños y sus efectos a largo plazo son mal conocidos. El objetivo de este estudio es evaluar a los pacientes neumonectomizados en nuestro centro. Material y métodos. Revisamos retrospectivamente los pacientes neumonectomizados entre 1969 y 2008, recogiendo datos sobre la indicación, edad, supervivencia, función respiratoria y escoliosis. Resultados. Encontramos 10 pacientes (varón: mujer 1,5:1) con edad media de 102 ± 66 meses. Las indicaciones más frecuentes fueron las bronquiectasias (4, 1 con fibrosis quística), los tumores (1 carcinoide bronquial, 1 pseudotumor inflamatorio, 1 metástasis de osteosarcoma, 1 PNET pulmonar) y las malformaciones (1 secuestro pulmonar, 1enfisema lobar congénito). La supervivencia global fue del 90% a los (..) (AU)

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-termsequelae 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 (..) (AU)

[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.

[Pediatric patient in adult age. Long-terms results of esophageal replacement]. / El paciente pediátrico en la edad adulta: revisión a largo plazo de los resultados de la sustitución esofágica.

BACKGROUND: Esophagocoloplasty is one of the most used procedures for esophageal replacement in children. Considering high life expectancy in these patients, long-term results must be considered when evaluating this technique. The aim of our study is to evaluate quality of life of adult patients who underwent surgery at pediatric age. PATIENTS AND METHODS: We report a retrospective study of 99 patients who underwent esophageal replacement in our institution between 1966 and 2006. Eight of them have died and 63 out of the remaining 91 are over 18 years now and represent our study serie. Long-term results and actual situation of those patients, considering psychological, physic and social aspects, were evaluated through clinical review and telephonic interview. Karnofsky index was applied to mesure functional ability from 0-100% (bad, medium, good-excellent) according to the answers the patients gave to our questions. We also recorded their health personal experience and subjective evaluation of their quality of life. RESULTS: Sixty-three patients were reviewed (43 males and 20 females) with a mean age of 4.3 +/- 3.4 D.S. Mean follow-up time was 29.6 +/-7.7 years. Indications for esophageal replacement were as follows: caustication (n = 32), type III esophageal atresia (n = 15), type I AE (n = 13) and others (n = 3). In 48 patients the graft was placed in retroestenal position and in 15 cases retromediastic location was used. Postoperative period was uneventful in 44% of the patients, being the most frequent early complications in the remaining, cervical leakage and stenosis. Long-term, 56,8% did not have any sequelae, 28.5% required further surgery and the remaining 43.13% presented the following complications: symptomatic graft reflux (22), scoliosis and thoracic asymmetry (12), colonic redundancy or cervical diverticulum (7), food impaction (6) and failure to thrive (5). Only one 38 year old patient does not have intestinal tract continuity nowadays. Thirty-one patients have a Karnofsky index > or = 80-100%, being considered healthy and able to have a normal activity. Eighteen patients are included between 40-80%, being the most frequent limitation the need of medication to avoid reflux, backache and occasional episodes of food impact. Only 2 patients have Karnofsky index inferior to 40%. None of them are under 20%. CONCLUSIONS: Esophagocoloplasty allows restoration of intestinal tract continuity in almost all cases and the mortality of this procedure has decreased over time. Even though some risks are still remarkable, it offers long-term good results with little repercussion on functional ability in adult age. Most of the patients consider themselves healthy and enjoy an acceptable quality of life.

[Congenital cystic adenomatoid malformation: prenatal diagnosis, surgical treatment results and long-term follow-up]. / Malformación adenomatoidea quística: diagnóstico prenatal, resultados de la cirugía y seguimiento a largo plazo.

PURPOSE: (PD), possibility of regresion and hidden mortality are open questions in congenital cystic adenomatoid malformation (CCAM) treatment. METHODS: Children with CCAM were reviewed focused on: PD, postnatal diagnosis, clinic, radiology, histology and evolution. RESULTS: Seventeen fetus had PD of CCAM. Five gestations were electively finished (41%) with PD of CCAM previous to 20th week, 3/5 (60%) were type III and 2/5 (40%) type I; 4/5 (80%) presented mediastinal shift and 1/5 (20%) hidrops. Two fetuses (11%) suffered fetal demise in 20th and 32th week; 1 type 1 and 1 type III; 1/2 (50%) presented hidrops and 2/2 (100%) mediastinal shift. Two (12%) died before 24 hours after birth without intervention possibility due to respiratory instability, 1 type II and 1 type III, both with mediastinal shift (100%). In one fetus with a type III malformation the image disappeared completely in 32th week and no intervention was done. Fourteen patients were operated (8 girls and 6 boys); 7/14 (50%) had PD, average diagnosis week was 21.9 (range 19.1-35.5), 5/7 (71%) was type I, 1/7 (14%) type II and 1/7 (14%) type III. None had mediastinal shift or hidrops. Average postnatal diagnosis week was 7 months (range 0.1-29). In 10/14 (71.4%) there were not respiratory difficulty during neonatal period and 3/10 (30%) suffered respiratory infections afterwards. Average week of operation was 8 months (range 0.1-30). PD was according with histology in 6/7 (86%) patients. After an average follow-up period of 4.3 years (range 1-9.5) the only complication is a pectus excavatum. CONCLUSIONS: More than half of patients with PD of CCAM died without intervention. Half of cases of CCAM are diagnosed prenatally. Type of CCAM in PD is according to histology in 86% of the cases. Fetuses with hidrops present a worse prognosis. Surgical timing do not seem to influence on outcome.

El paciente pediátrico en la edad adulta: revisión a largo plazo de los resultados de la sustitución esofágica / Pediatric patient in adult age. Long-terms results of esophageal replacement

Introducción. La esofagocoloplastia es uno de los procedimientos más empleados para la sustitución esofágica en niños. Dada la elevada esperanza de vida de estos pacientes deben evaluarse los resultados a largo plazo de dicha intervención. Nuestro objetivo es revisar nuestra serie para conocer la calidad de vida de los pacientes adultos operados en la edad pediátrica. Pacientes y métodos. Analizamos retrospectivamente los 99 pacientes sometidos a una sustitución esofágica en nuestro centro entre los años 1.966 y 2.006. De ellos, 8 han fallecido y de los 91 restantes, 63 son actualmente mayores de 18 años y constituyen la muestra de nuestro estudio. Mediante revisión clínica y/o encuesta telefónica evaluamos las complicaciones a largo plazo y la situación actual de estos pacientes desde el punto de vista psicológico, físico y social. Empleamos el índice de Karnofsky para medir la capacidad funcional (Quality of life, QOL) de 0-100% (baja-pésima, media, buena-excelente) en base a las respuestas de los pacientes al cuestionario, añadiendo a nuestros resultados la autopercepción del estado de salud de éstos y la valoración subjetiva de su calidad de vida. Resultados. De los 63 pacientes que constituyen nuestra serie, 43 eran hombres y 20 mujeres, con edades en el momento de la intervención, comprendidas entre los 9 meses y los 7 años (4,3±3,4 años, media ± desviación estándar). El seguimiento postoperatorio fue de 29,6±7,7 años. Las indicaciones de sustitución fueron las siguientes: causticación (n=32), atresia de esófago tipo III (n=15), atresia de esófago tipo I (n=13) y otras (n=3). En 48 casos la transposición fue retroesternal y en 15 mediastínica. El 44% de los casos presentó un postoperatorio sin incidencias, siendo las complicaciones precoces más frecuentes en los demás pacientes la fístula cervical y la estenosis. A largo plazo, el 56,8% no tenía secuelas, el 28,5% precisó alguna intervención posterior y el 43,13% presentaba las siguientes complicaciones: reflujo sintomático al injerto (22), escoliosis y asimetría torácica (12), redundancia cólica o divertículo cervical (7), impactación alimentaria (6) y escasa ganancia pondero-estatural (5). Tan sólo un paciente de 38 años no tiene restablecida la continuidad digestiva en la actualidad. Treinta y un pacientes presentan un índice de Karnofsky >= 80-100%, considerándose sanos y aptos para la actividad común. Dieciocho están comprendidos entre el 40-80%, siendo las limitaciones más frecuentes la necesidad de medicación y medidas dietéticas para evitar el reflujo, los dolores dorso-lumbares y los episodios ocasionales de impactación alimentaria. Sólo 2 pacientes actualmente presentan un Karnofsky inferior o igual al 40% y superior o igual al 20%. Ninguno de ellos tiene un índice inferior al 20%. Conclusiones. La esofagocoloplastia es una técnica no exenta de riesgos, de mortalidad decreciente y que permite, en casi todos los pacientes, restablecer la continuidad digestiva con buenos resultados a largo plazo y escasa repercusión en la capacidad funcional del adulto. La mayor parte de ellos se consideran sanos y gozan de una calidad de vida satisfactoria (AU)

Background. Esophagocoloplasty is one of the most used procedures for esophageal replacement in children. Considering high life expectancy in these patients, long-term results must be considered when evaluating this technique. The aim of our study is to evaluate quality of life of adult patients who underwent surgery at pediatric age. Patients and methods. We report a retrospective study of 99 patients who underwent esophageal replacement in our institution between 1966 and 2006. Eight of them have died and 63 out of the remaining 91 are over 18 years now and represent our study serie. Long-term results and actual situation of those patients, considering psychological, physic and social aspects, were evaluated through clinical review and telephonic interview. Karnofsky index was applied to mesure functional ability from 0-100% (bad, medium, good-excellent) according to the answers the patients gave to our questions. We also recorded their health personal experience and subjective evaluation of their quality of life. Results. Sixty-three patients were reviewed (43 males and 20 females) with a mean age of 4,3±3,4 D.S. Mean follow-up time was 29.6±7,7years. Indications for esophageal replacement were as follows: caustication (n=32), type III esophageal atresia (n=15), type I AE (n=13) and others (n=3). In 48 patients the graft was placed in retroesternal position and in 15 cases retromediastic location was used. Postoperative period was uneventful in 44% of the patients, being the most frequent early complications in the remaining, cervical leakage and stenosis. Long-term, 56,8% did not have any sequelae, 28,5% required further surgery and the remaining 43,13% presented the following complications: symptomatic graft reflux (22), scoliosis and thoracic asymmetry (12), colonic redundancy or cervical diverticulum (7), food impaction (6) and failure to thrive (5). Only one 38 year old patient does not have intestinal tract continuity nowadays. Thirty-one patients have a Karnofsky index >=80-100%, being considered healthy and able to have a normal activity. Eighteen patients are included between 40-80%, being the most frequent limitation the need of medication to avoid reflux, backache and occasional episodes of food impact. Only 2 patients have Karnofsky index inferior to 40%. None of them are under 20%. Conclusions. Esophagocoloplasty allows restoration of intestinal tract continuity in almost all cases and the mortality of this procedure has decreased over time. Even though some risks are still remarkable, it offers longterm good results with little repercussion on functional ability in adult age. Most of the patients consider themselves healthy and enjoy an acceptable quality of life (AU)

Malformación adenomatoidea quística: diagnóstico prenatal, resultados de la cirugía y seguimiento a largo plazo / Congenital cystic adenomatoid malformation: prenatal diagnosis surgical treatment results and long-term follow-up

Introducción. El diagnóstico prenatal (DP) posibilidad de regresión, mortalidad oculta y momento de la cirugía son cuestiones abiertas en el seguimiento de niños con malformación adenomatoidea quística (MAQ). Pacientes y métodos. Revisamos en las historias de niños con MAQ entre los años 1995 y 2005: diagnóstico prenatal y posnatal, presentación clínica y radiológica, intervención quirúrgica, histología y evolución. Resultados. Diecisiete fetos tenían DP de MAQ. Cinco (41%) se abortaron de manera electiva con DP previo a la semana 20, 3/5 (60%) fueron tipo III y 2/5 (40%) tipo I; 4/5 (80%) presentaban desplazamiento cardíaco y 1/5 (20%) anasarca y ascitis. Dos (11%) fueron abortos espontáneos con DP en las semanas 20 y 32; 1 tipo I y 1 tipo III; 1/2 (50%) presentaba hidrops y 2/2 (100%) desplazamiento cardíaco. Dos (12%) murieron antes de las 24 horas de vida sin posibilidad de intervención por inestabilidad, 1 tipo II y 1 tipo III, ambos con desplazamiento cardíaco (100%). En 1 (6%) con DP de MAQ tipo III en la semana 20 desapareció la imagen en la semana 32; no fue intervenido y está asintomático. Catorce pacientes fueron intervenidos (8 niñas y 6 niños); 7/14 (50%) tenían DP, la semana media de diagnóstico fue 21,9 (rango 19,1- 35,5), 5/7 (71%) fueron tipo I, 1/7 (14%) tipo II y 1/7 (14%) tipo III. Ninguno tenía desplazamiento cardíaco o hidrops. La mediana de edad de diagnóstico posnatal fue 7 meses (rango 0,1-29). En 10/14 (71,4%) no existió dificultad respiratoria en el período neonatal desarrollando 3/10 (30%) infección posteriormente. La mediana de edad de la intervención quirúrgica fue 8 meses (rango 0,1-30). En 11/13 (84%) casos se hizo lobectomía y en 2 (16%) lobectomía acompañada de segmentectomía. En 1 caso sin lesiones radiológicas posnatales se hizo exploración quirúrgica sin resección. El resultado histológico fue MAQ tipo I 9/13 (69%), tipo II 1/13 (7,6%) y tipo III 3/13 (23%). El DP se correspondía con el histológico en 6/7 (86%) pacientes. Tras un seguimiento medio de 4.3 años (rango 1-9,5) la única complicación es un pectus leve. Conclusiones. Más de la mitad (52%) de los niños con DP de MAQ mueren sin ser intervenidos. El DP diagnostica MAQ en la mitad de los casos y el tipo de MAQ en el 86% de éstos. En el DP el desplazamiento cardíaco y el hidrops se asocian a una alta mortalidad por aborto espontáneo o en el período neonatal inmediato siendo estos pacientes susceptibles de cirugía fetal. El pronóstico de los niños intervenidos sin DP o con DP sin hidrops, anasarca o ascitis es excelente. No hemos encontrado cambios en la evolución relacionados con el tipo de resección o el momento de ésta (AU)

PURPOSE: (PD), possibility of regresion and hidden mortality are open questions in congenital cystic adenomatoid malformation (CCAM) treatment. METHODS: Children with CCAM were reviewed focused on: PD, postnatal diagnosis, clinic, radiology, histology and evolution. RESULTS: Seventeen fetus had PD of CCAM. Five gestations were electively finished (41%) with PD of CCAM previous to 20th week, 3/5 (60%) were type III and 2/5 (40%) type I; 4/5 (80%) presented mediastinal shift and 1/5 (20%) hidrops. Two fetuses (11%) suffered fetal demise in 20th and 32th week; 1 type 1 and 1 type III; 1/2 (50%) presented hidrops and 2/2 (100%) mediastinal shift. Two (12%) died before 24 hours after birth without intervention possibility due to respiratory instability, 1 type II and 1 type III, both with mediastinal shift (100%). In one fetus with a type III malformation the image disappeared completely in 32th week and no intervention was done. Fourteen patients were operated (8 girls and 6 boys); 7/14 (50%) had PD, average diagnosis week was 21.9 (range 19.1-35.5), 5/7 (71%) was type I, 1/7 (14%) type II and 1/7 (14%) type III. None had mediastinal shift or hidrops. Average postnatal diagnosis week was 7 months (range 0.1-29). In 10/14 (71.4%) there were not respiratory difficulty during neonatal period and 3/10 (30%) suffered respiratory infections afterwards. Average week of operation was 8 months (range 0.1-30). PD was according with histology in 6/7 (86%) patients. After an average follow-up period of 4.3 years (range 1-9.5) the only complication is a pectus excavatum. CONCLUSIONS: More than half of patients with PD of CCAM died without intervention. Half of cases of CCAM are diagnosed prenatally. Type of CCAM in PD is according to histology in 86% of the cases. Fetuses with hidrops present a worse prognosis. Surgical timing do not seem to influence on outcome (AU)

Sarcomas de partes blandas 1991-2004 / Soft tissue sarcomas (1991-2004)

: Introducción. El objetivo de este estudio es revisar los resultados del tratamiento de los sarcomas de partes blandas (SPB) en nuestro centro en los últimos trece años. Material y métodos. Tratamos 57 niños, 39 con rabdomiosarcomas (RMS) (67%) y 18 con otros tipos de sarcomas (33%). Dentro del primer grupo, excluimos 13 tumores orbitarios, 4 parameníngeos y 2 faciales tratados por otros servicios. Analizamos la edad, sexo, localización, histología, estadio inicial, tratamiento médico-quirúrgico y resultados. Evaluamos cada uno de estos datos como factor pronóstico mediante curvas de sobrevivencia actuarial y pruebas de log-rank. Resultados. La mediana de edad al diagnóstico fue de 2,3 años en los RMS (rango 6 m-16 a), y 10,9 años (0-16 a) en los otros SPB. La relación varón/mujer fue de 2,8:1 y 2:1 respectivamente. Doce RMS fueron urogenitales, 3 torácicos, 3 abdominales, 1 de extremidades y 1 cervical. Trece tumores (65%) eran embrionarios, 5 (25%) botrioides, 1 alveolar y otro fusiforme. Al diagnóstico, 74% se hallaban en estadio 1 o 2, y 26% en 3 o 4. En 5 niños (25 %) se realizó punción por aspiración de aguja fina (PAAF), que dio un diagnóstico impreciso o erróneo. En 12 (60%) se realizó biopsia antes de la cirugía definitiva y en 8 (40%) el diagnóstico se obtuvo tras analizar la pieza quirúrgica, aunque en 3 casos este diagnóstico inicial fue erróneo (neuroblastoma, tumor neuroectodérmico primitivo y leiomiosarcoma) y no se obtuvo el definitivo hasta la recidiva. Todos recibieron quimioterapia, el 60% antes de la extirpación quirúrgica, los restantes después. Sólo 2 pacientes (..) (AU)

Background.The aim of this study is to review the results of the treatment of soft tissue sarcomas (STS) in our Department during the last 13 years. Material and methods. Fifty-seven children (39 rhabdomyosarcomas (RMS) and 18 other types of sarcomas) have been treated. Nineteen RMS were excluded because they were treated by oher departments. The charts of 39 chidren were analysed evaluating several parameters (age, sex, location, histology, initial stage, clinical and surgical treatment and results) as prognostic factors using actuarial survival analyses and log-rank tests. Results. 1. RMS: Median age at diagnosis was 2.3 years (range 6 m- 16y).Twelve were genitourinary, 3 thoracic, 3 abdominal, 1 was located in limb and 1 in the neck. Histologically, 13 were embryonal, 5 botryoid, 1 alveolar and 1 fusiform. At diagnosis, 74% were in stages I or II. Fine needle aspiration biopsy (FNAB) was made in 5 children and the result was always imprecise or mistaken. Surgical biopsy was made before the definitive surgery in 12 cases. In the remaining 8 children the diagnosis was made only after surgical resection. With an mean follow-up of 70 ± 43 moths, 6 children died. The prognostic factors associated with poor outcomes were genitourinary (..) (AU)

Sustitución esofágica. Experiencia de 12 años / Esophageal replacement. 12 years experience

Objetivo. La sustitución esofágica está indicada en unos pocos niños con atresia esofágica tipos I y III no anastomosable, con esofagitis cáustica u otras enfermedades. Analizamos los resultados de una serie amplia de sustituciones esofágicas en nuestro Servicio con el fin de destacar sus modalidades, ventajas y riesgos. Material y métodos. Revisamos retrospectivamente las sustituciones esofágicas llevadas a cabo entre 1992 y 2004 en 29 pacientes (15 niñas y 14 niños) de entre 2 meses y 14 años (mediana 24 meses). Once (37,9%) sufrían atresia de esófago tipo I, 7 (24,1%) atresia de esófago tipo III no anastomosable, 8 (27,5%) esofagitis cáusticas, 1 esofagitis herpética, 1 esofagitis candidiásica y 1 necrosis esofágica por endoesclerosis. En 25 casos (86,2%) se usó el colon, y en 4 (13,8%) el estómago. La colocación del injerto fue retromediastínica en 25 niños (86,2%), retroesternal en 3 y subcutánea en 1. El esófago nativo se extirpó en todos excepto en 2 de los 3 casos de localización retroesternal. Resultados. El injerto es funcional en todos los niños y en el momento actual todos toleran satisfactoriamente la alimentación oral, con un tiempo de seguimiento entre 7 y 145 meses (mediana 76 meses), siendo el desarrollo estaturoponderal en la mayoría de los niños normal. Hubo estenosis de la anastomosis cervical en 3 (10,3%), obstrucción pilórica postoperatoria en otros 3 (10,3%), evisceración de herida (..) (AU)

Esophageal replacement is a surgical procedure rarely indicated in children. It is used in esophageal atresia type I and long-gap atresia when anastomosis is not possible, corrosive strictures and other unusual causes. Type and location of the graft depend on etiology and surgeon preferences. We analyse our results of a large series of esophageal replacement. Methods. We reviewed esophageal replacements carried out in our department between january-1992 and december-2004. We report 29 patients (15 girls and 14 boys) with ages ranging from 2 months until 14 years old (median 24 months). 11 (37.9%) had esophageal atresia type I, 7 (24.1%) long-gap esophageal atresia, 8 (27.5%) caustic esophagitis, 1 herpetic esophagitis, 1 candida esophagitis and 1 esophageal necrosis due to sclerotherapy. Colon was used for substitution in 25 cases (86.2%) and stomach in 4 (13.8%). Graft location was retromediastinal in 25 children (86.2%), retrosternal in 3 and subcutaneous in 1. Native esophagus was removed in all but 2 out of 3 retrosternal cases. Results. After a follow up between 7 and 145 months (median 76 months) all children have a functional graft. Actually all patients tolerate oral feeding in a satisfactory way, and have had a normal pondostatural growth. Post-operative complications were pyloric obstruction in 3 patients (10.3%), upper anastomosis stricture in 3 (10.3%), 2 (6.9%) surgical wound evisceration, 2 (6.9%) diaphragmatic hernia, 1 (3.4%) retromediastinal abscess and 1 (3.4%) colo-gastric emptying difficulties. Also 2 pleural effusions, 1 cervical wound abscess and 1 abdominal (..) (AU)

Congenital segmental fibromuscular hypertrophy of the esophagus and esophageal atresia: an uncommon case.

Although a rare entity, congenital esophageal stenosis due to segmental hypertrophy of the muscularis and submucosal layers with diffuse fibrosis should be considered a possibility in patients with esophageal stricture associated to congenital esophageal atresia. The efficacy of dilatation seems to be limited, and may even result in severe complications such as an esophageal rupture. Surgical repair for congenital esophageal stenosis is the authors' preferred treatment, although initial dilatation may be effective for some patients.

Enfermedad de Hirschsprung: enseñanzas de los últimos 100 casos / Hirschsprung disease: lessons learned from the last 100 cases

Introducción. Analizamos nuestra experiencia en el diagnóstico y tratamiento de esta enfermedad comparando los casos más antiguos con los más recientes, investigando la existencia de diferencias en morbimortalidad y evolución a largo plazo entre ambos grupos. Material y métodos. Revisamos 100 casos consecutivos de EH (72 varones y 28 mujeres) tratados en nuestro hospital entre 1992 y 2004. Doce tenían antecedentes familiares. Cinco niños sufrían Síndrome de Down. Tabulamos datos sobre el modo de presentación, necesidad de derivación intestinal, técnicas utilizadas en el tratamiento definitivo y morbimortalidad. Evaluamos la continencia a largo plazo utilizando la escala clínica de Holschneider modificada(1-3) que evalúa frecuencia y consistencia de las deposiciones, manchado, sensación de contenido fecal en ampolla rectal, necesidad de enemas o fármacos frente al estreñimiento y existencia de dolor con la defecación. Dividimos la serie en dos grupos de 50 según los pacientes hubieran sido tratados entre 1992-1997 y 1998-2004 y comparamos los resultados mediante métodos estadísticos comunes. Resultados. El 50% manifestaron la enfermedad en el período neonatal y el 25% se operaron en dicho momento. En 74 casos se trataba (..) (AU)

We analize our experience in the management of the last consecutive 100 Hirschsprung´s disease (HD) patients divided into two periods: 1992-1997 and 1998-2004, in order to find out differences in morbidity, mortality and outcome between them. Material and methods. During this period, 72 males and 28 females were treated. Twelve had family history and five suffered from Down´s syndrome. Information about clinical onset, need of stomas, surgical procedures, continence, outcome and mortality was recorded. We compared the results between the two groups with non-parametrics stadistics test. Results. 50% of patients were symptomatic in the newborn period and 25% of them needed some surgical procedures. Seventy four patients suffered from rectosigmoid forms, fourteen colic forms and twelve were total colonic HD (7 with small bowel extension). Hystochemistry was diagnostic in 98%. Nursing was effective in 47 cases. Differences (..) (AU)

Prenatal diagnosis of gastric triplication.

Duplications of the intestinal tract are rare malformations, and triplications are even less common; only two cases are found in the literature. The authors describe a case of prenatal diagnosis of a gastric triplication and the surgical treatment.

[Soft tissue sarcomas (1991-2004)]. / Sarcomas de partes blandas 1991-2004.

BACKGROUND: The aim of this study is to review the results of the treatment of soft tissue sarcomas (STS) in our Department during the last 13 years. MATERIAL AND METHODS: Fifty-seven children (39 rhabdomyosarcomas (RMS) and 18 other types of sarcomas) have been treated. Nineteen RMS were excluded because they were treated by oher departments. The charts of 39 chidren were analysed evaluating several parameters (age, sex, location, histology, initial stage, clinical and surgical treatment and results) as prognostic factors using actuarial survival analyses and log-rank tests. RESULTS: 1. RMS: Median age at diagnosis was 2.3 years (range 6 m-16y). Twelve were genitourinary, 3 thoracic, 3 abdominal, 1 was located in limb and 1 in the neck. Histologically, 13 were embryonal, 5 botryoid, 1 alveolar and 1 fusiform. At diagnosis, 74% were in stages I or II. Fine needle aspiration biopsy (FNAB) was made in 5 children and the result was always imprecise or mistaken. Surgical biopsy was made before the definitive surgery in 12 cases. In the remaining 8 children the diagnosis was made only after surgical resection. With an mean follow-up of 70 +/- 43 moths, 6 children died. The prognostic factors associated with poor outcomes were genitourinary location, non radical excission, the presence of distant metastases at onset and alveolar histology. 2. Other sarcomas: Median age at diagnosis was 10.9 years (range 4 days-15 years). Among this group, there were 6 fibrosarcomas, 4 indifferentiated sarcomas, 3 synovial sarcomas, 2 abdominal desmoplastic small round cell tumours, 2 neurofibrosarcomas and 1 leiomyosarcoma. Only 9 received chemotherapy and one radiotherapy. All but one were operated. Five out ot the 19 died. CONCLUSIONS: Although the role of surgery is crucial, it is necessary to refine the initial histological diagnosis, because neither the PAAF or the biopsy have always been correct. The negative prognostic factors in our series were metastases present at diagnosis, genitourinary location and alveolar (RMS), desmoplastic or indifferenciated histology.

[Esophageal replacement. 12 years experience]. / Sustitución esofágica. Experiencia de 12 años.

UNLABELLED: Esophageal replacement is a surgical procedure rarely indicated in children. It is used in esophageal atresia type I and long-gap atresia when anastomosis is not possible, corrosive strictures and other unusual causes. Type and location of the graft depend on etiology and surgeon preferences. We analyse our results of a large series of esophageal replacement. METHODS: . We reviewed esophageal replacements carried out in our department between January-1992 and December-2004. We report 29 patients (15 girls and 14 boys) with ages ranging from 2 months until 14 years old (median 24 months). 11 (37.9%) had esophageal atresia type I, 7 (24.1%) long-gap esophageal atresia, 8 (27.5%) caustic esophagitis, 1 herpetic esophagitis, 1 candida esophagitis and 1 esophageal necrosis due to sclerotherapy. Colon was used for substitution in 25 cases (86.2%) and stomach in 4 (13.8%). Graft location was retromediastinal in 25 children (86.2%), retrosternal in 3 and subcutaneous in 1. Native esophagus was removed in all but 2 out of 3 retrosternal cases. RESULTS: After a follow up between 7 and 145 months (median 76 months) all children have a functional graft. Actually all patients tolerate oral feeding in a satisfactory way, and have had a normal pondostatural growth. Post-operative complications were pyloric obstruction in 3 patients (10.3%), upper anastomosis stricture in 3 (10.3%), 2 (6.9%) surgical wound evisceration, 2 (6.9%) diaphragmatic hernia, 1 (3.4%) retro-mediastinal abscess and 1 (3.4%) colo-gastric emptying difficulties. Also 2 pleural effusions, 1 cervical wound abscess and 1 abdominal wound one. Re-operation was needed in 11 patients (38%) due to these adverse events. Other complications were conservatively solved: 6 (20.7%) salivary fistula, 1 intestinal suboclusion and a dumping syndrome. One girl died due to a mycotic mediastinal abscess with perforation of the aorta 11 days after surgery. Overall survival was 96.5%. CONCLUSIONS: Esophageal replacement has limited indications. It allow a good functional result, with adequate oral feeding and normal growth. We believe that both colon and stomach have similar outcomes, but gastric pull-up is easier to perform. It is a major surgery whose risk of complications is higher in early post-operative time.

[Hirschsprung disease: lessons learned from the last 100 cases]. / Enfermedad de Hirschsprung: enseñanzas de los últimos 100 casos.

UNLABELLED: We analyize our experience in the management of the last consecutive 100 Hirschsprung's disease (HD) patients divided into two periods: 1992-1997 and 1998-2004, in order to find out differences in morbidity, mortality and outcome between them. MATERIAL AND METHODS: During this period, 72 males and 28 females were treated. Twelve had family history and five suffered from Down's syndrome. Information about clinical onset, need of stomas, surgical procedures, continence, outcome and mortality was recorded. We compared the results between the two groups with non-parametrics stadistics test. RESULTS: 50% of patients were symptomatic in the newborn period and 25% of them needed some surgical procedures. Seventy four patients suffered from rectosigmoid forms, fourteen colic forms and twelve were total colonic HD (7 with small bowel extension). Hystochemistry was diagnostic in 98%. Nursing was effective in 47 cases. Differences in the need of stomas were found between the two periods: 30% during the first period and 6% during the second one (p<0,05). Twenty percent (20) of the patients suffered from enterocolitis (with no differences between both groups), and 13 of them still had enterocolitis episodes in spite of stomas or pull-through procedures. We performed 49 Swenson, 29 Soave, 14 transanal and 2 Lester-Martin procedures. The median age at definitive operation was smaller in the last period when compared to the first (p< 0.05). We found good results on continence in 86%, with no relation with definitive surgical procedure nor with the period of time studied. CONCLUSIONS: The younger age at definitive treatment, the performance of stomas and the increase of transanal procedures were the principal differences between the two groups.

Body wall dysplasia: a rare case of abdominal hernia.

Malformations of the fetal abdominal wall include a broad spectrum of anomalies, and prenatal sonography provides the possibility of detecting most of them. Omphalocele and gastroschisis are the most common conditions, but there are other rare forms. We describe here a rare case of body wall dysplasia that we called abdominal hernia that appeared upon prenatal sonography as an omphalocele-like defect.

[Congenital neuroblastomas]. / Neuroblastomas congénitos.

BACKGROUND: We consider congenital neuroblastomas (CN) those detected in pregnancy or at the very first hours of life. Due to perinatal sonography, its incidence has increased in the last years. We present herein our experience in the treatment of this condition and we try to find out any different clinical pattern from those neuroblastomas diagnosed later in life. METHODS: We review the CN treated in our hospital from 1990 to 2003, analyzing diagnosis, localization, tumor staging, N-myc amplification, treatment and evolution. RESULTS: Among the 107 neural tumors managed during this period (89 neuroblastomas, 18 ganglioneuromas), 8 were congenital neuroblastomas (7 girls, 1 boy). Two patients had prenatal diagnosis and 6 tumours were detected in routine exploration or casual findings upon neonatal examination. Six were abdominal, 1 thoracoabdominal and 1 abdominopelvic with dumbbell invasion. Three tumours were classified like stage 1, 1 stage 2, 1 stage 3, 1 stage 4 and 2 stage 4S. Although most of them had unfavorable histology, we didn't find N-myc amplification in any tumor. All patients were operated upon, with preoperative chemotherapy in 2 of them. Resection was complete in 7 out of the 8 tumors. The patient who presented neurological symptoms at birth recovered neither motility nor bladder function after resection. All of them survive after 60+/-53 months of follow-up. CONCLUSIONS: The outcome in this group of neuroblastomas is better that expected, probably because of its abdominal location. On the contrary in dumbbell neuroblastomas, neurological damage at birth seems to be irreversible.

[Aggressive conservative treatment remains the best option for oesophageal perforation in children]. / Perforaciones esofágicas: el tratamiento conservador agresivo como la mejor opción en la edad pediátrica.

UNLABELLED: Oesophageal perforation (OP) requires prompt and vigorous treatment. In contrast with adult patients in whom surgical closure of perforation is preferred, non-operative treatment has been the usual approach in children. The present report aims at assessing whether this strategy stands the passage of time. We studied retrospectively the charts of patients treated at our institution for OP between 1991 and 2001. Between these years, we treated 19 episodes of OP in 17 patients aged 5.3 +/- 0,94 years. In 9 cases (4 lye burns, 3 oesophageal atresias, 1 bullous epidermolysis and 1 mucocutaneous candidiasis) OP occurred during dilatation of strictures. Foreign body extraction was the cause in 3 cases, and blunt trauma and sclerosis of varices were the causes in 2 cases each. The last child had multiple gastrointestinal perforation during treatment for leukaemia. Subcutaneous emphysema was seen in 7 instances, pneumomediastinum/pneumothorax in 14, pleural effusion in 9, dyspnoea in 9, severe thoracic pain in 1 and pericardial effusion in 1. The diagnosis was intraoperative in only 2 children but the symptoms and imaging signs prompted vigorous treatment within the first 24 hours in 15 instances. One or more pleural tubes were inserted in 11 cases and pericardial drainage was required once. Perforations closed without direct surgery in 18/19 episodes (16/17 children). Five gastrotomies and 2 jejunostomies were performed and several major abdominal operations were necessary to repair concurrent lesions in a child who sustained severe blunt abdominal trauma and in the one with leukaemic perforations. All these patients survive and all recovered oesophageal function although 2 with intractable lye structures ultimately required oesophageal replacement 6 and 10 months after OP. The only patient in whom direct approach for esophageal necrosis after variceal endosclerosis was unavoidable lost her organ and had a replacement after a successful porto-systemic shunt. CONCLUSIONS: Prompt and aggressive non-operative approach of oesophageal perforations in children allows survival and conservation of the organ and its function in most cases and should remain the first therapeutic choice at this age.

Aggressive conservative treatment of esophageal perforations in children.

BACKGROUND/PURPOSE: In contrast with adult patients in whom surgical closure of the defect is preferred, nonoperative treatment has been the usual approach for esophageal perforation (EP) in children. This report aims to assess whether this strategy stands the passage of time. METHODS: We reviewed retrospectively the charts of 17 patients aged 5.3 +/- 0.9 years (mean +/- SD) treated at our institution for EP between 1991 and 2001. RESULTS: Nineteen episodes of EP were caused by stricture dilation in 9 cases, foreign body extraction in 3, and blunt trauma and sclerosis of varices in 2 cases each. The remaining child had multiple gastrointestinal perforations in the course of chemotherapy for leukemia. Vigorous treatment, consisting of nasopharyngeal aspiration, wide spectrum antibiotics, prompt drainage of effusions and either parenteral or infraesophageal nutritition, was implemented immediately after diagnosis. Perforations were closed without direct surgery in 18 of 19 episodes (16 of 17 children). One or more pleural drains were inserted in 12 cases, and pericardial drainage was required once. Seven gastrostomies, 2 jejunostomies, and one esophagostomy were performed. Several major abdominal operations were necessary to repair concomitant lesions in a child who sustained severe blunt abdominal trauma and in the patient with leukemic perforations. All patients survived, and all recovered esophageal function. However, 2 with intractable lye strictures ultimately required esophageal replacement. The only patient in whom a direct approach for esophageal necrosis due to variceal endosclerosis was unavoidable, lost her organ and had a retrosternal colonic interposition after a successful portosystemic shunt. Excluding patients with other concomitant lesions and the patient who underwent surgery, median length of stay was 11 days (range, 6 to 47). CONCLUSIONS: Prompt and aggressive nonoperative treatment of esophageal perforations in children allows survival with conservation of the organ in most cases and remains, in the authors' hands, the first therapeutic choice at this age.

Pancreatic pseudocyst caused by valproic acid: case report and review of the literature.

The author reports one case of pseudocyst of the pancreas, an uncommon disorder in children, secondary to pancreatitis in a 9-year-old girl treated with valproic acid for epilepsy.