Beneficios de la estandarización en el manejo de la apendicitis aguda / Benefits of standardization in the management of acute appendicitis

bjetivos. La apendicitis aguda (AA) es la patología quirúrgica pediátrica urgente más frecuente, pero su manejo postoperatorio es variable. La aplicación de protocolos minimiza esta variabilidad. Presentamos los resultados tras la optimización del protocolo de manejo en nuestro centro con objetivo de determinar su efectividad en cuanto a número de complicaciones infecciosas y optimización de recursos en nuestro medio. Material y métodos. Se realiza un estudio observacional retrospectivo en pacientes intervenidos de AA entre enero 2018 y agosto 2022. Comparamos dos cohortes, antes (1) y después del protocolo (2), subdividiéndolas según gravedad para un análisis por subgrupos: flemonosas (F), gangrenosas (G) y perforadas (P). Resultados. Incluimos 771 pacientes (1: 390/ 2: 381), con distribución homogénea, con mediana de edad 9,3 ± 2,8 años. La solicitud de analíticas previo al alta disminuyó de forma significativa (F: 3,9% vs. 0,5%; p= 0,026; G: 97,6% vs. 13,4%, p< 0,001). Los días de estancia hospitalaria se redujeron en los subgrupos F (1,2 IQR 0,7 vs. 1 IQR 0,36; p< 0,001), y G (4 IQR 1 vs. 3 IQR 1 días; p< 0,001). No se observaron diferencias en el número de abscesos entre los grupos (41 vs. 43 p= 0,73); sí en el subgrupo G (9 vs. 2; p= 0,029). Observamos una reducción del gasto de recursos en F y G. Conclusiones. En nuestro estudio, el subgrupo más beneficiado tras la optimización del protocolo fue el de las apendicitis gangrenosas con una reducción significativa en el número de complicaciones y en la utilización de recursos hospitalarios.(AU)

Objective. Acute appendicitis (AA) is the most frequent urgent surgical pathology in the pediatric population, but postoperative management is variable, with protocols minimizing variability. We present our results following the optimization of the management protocol in ourinstitution in order to establish its efficacy in terms of number of infectious complications and optimization of resources in our environment. Materials and methods. An observational, retrospective study of patients undergoing AA surgery from January 2018 to August 2022 was carried out. Two cohorts were compared, both before (1) and after (2) the implementation of the new protocol. They were divided according to severity in order to conduct a subgroup-based analysis –phlegmonous (PH), gangrenous (G), and perforated (P) appendicitis. Results. 771 patients (1: 390; 2: 381) were included, with a homogeneous distribution and a median age of 9.3 ± 2.8 years. Blood tests requested prior to discharge experienced a significant reduction (PH: 3.9% vs. 0.5%; p= 0.026; G: 97.6% vs. 13.4%, p< 0.001). Days of hospital stay decreased in the PH (1.2 IQR: 0.7 vs. 1 IQR: 0.36; p< 0.001) and G (4 IQR: 1 vs. 3 IQR: 1 days; p< 0.001) subgroups. No differences in the number of abscesses were found between groups (41 vs. 43; p= 0.73), but they were noted within subgroup G (9 vs. 2; p= 0.029). A reduction in resource expenses was detected in PH and G appendicitis. Conclusions. In our study, the most widely benefited subgroup following protocol optimization was the gangrenous appendicitis subgroup, with a significant reduction in the number of complications and the use of hospital resources.(AU)

Benefits of standardization in the management of acute appendicitis. / Beneficios de la estandarización en el manejo de la apendicitis aguda.

OBJECTIVE: Acute appendicitis (AA) is the most frequent urgent surgical pathology in the pediatric population, but postoperative management is variable, with protocols minimizing variability. We present our results following the optimization of the management protocol in our institution in order to establish its efficacy in terms of number of infectious complications and optimization of resources in our environment. MATERIALS AND METHODS: An observational, retrospective study of patients undergoing AA surgery from January 2018 to August 2022 was carried out. Two cohorts were compared, both before (1) and after (2) the implementation of the new protocol. They were divided according to severity in order to conduct a subgroup-based analysis -phlegmonous (PH), gangrenous (G), and perforated (P) appendicitis. RESULTS: 771 patients (1: 390; 2: 381) were included, with a homogeneous distribution and a median age of 9.3 ± 2.8 years. Blood tests requested prior to discharge experienced a significant reduction (PH: 3.9% vs. 0.5%; p= 0.026; G: 97.6% vs. 13.4%, p< 0.001). Days of hospital stay decreased in the PH (1.2 IQR: 0.7 vs. 1 IQR: 0.36; p< 0.001) and G (4 IQR: 1 vs. 3 IQR: 1 days; p< 0.001) subgroups. No differences in the number of abscesses were found between groups (41 vs. 43; p= 0.73), but they were noted within subgroup G (9 vs. 2; p= 0.029). A reduction in resource expenses was detected in PH and G appendicitis. CONCLUSIONS: In our study, the most widely benefited subgroup following protocol optimization was the gangrenous appendicitis subgroup, with a significant reduction in the number of complications and the use of hospital resources.

OBJETIVOS: La apendicitis aguda (AA) es la patología quirúrgica pediátrica urgente más frecuente, pero su manejo postoperatorio es variable. La aplicación de protocolos minimiza esta variabilidad. Presentamos los resultados tras la optimización del protocolo de manejo en nuestro centro con objetivo de determinar su efectividad en cuanto a número de complicaciones infecciosas y optimización de recursos en nuestro medio. MATERIAL Y METODOS: Se realiza un estudio observacional retrospectivo en pacientes intervenidos de AA entre enero 2018 y agosto 2022. Comparamos dos cohortes, antes (1) y después del protocolo (2), subdividiéndolas según gravedad para un análisis por subgrupos: flemonosas (F), gangrenosas (G) y perforadas (P). RESULTADOS: Incluimos 771 pacientes (1: 390/ 2: 381), con distribución homogénea, con mediana de edad 9,3 ± 2,8 años. La solicitud de analíticas previo al alta disminuyó de forma significativa (F: 3,9% vs. 0,5%; p= 0,026; G: 97,6% vs. 13,4%, p< 0,001). Los días de estancia hospitalaria se redujeron en los subgrupos F (1,2 IQR 0,7 vs. 1 IQR 0,36; p< 0,001), y G (4 IQR 1 vs. 3 IQR 1 días; p< 0,001). No se observaron diferencias en el número de abscesos entre los grupos (41 vs. 43 p= 0,73); sí en el subgrupo G (9 vs. 2; p= 0,029). Observamos una reducción del gasto de recursos en F y G. CONCLUSIONES: En nuestro estudio, el subgrupo más beneficiado tras la optimización del protocolo fue el de las apendicitis gangrenosas con una reducción significativa en el número de complicaciones y en la utilización de recursos hospitalarios.

Thromboelastometry-guided surgery in neuroblastoma complicated with disseminated intravascular coagulation. / Cirugía guiada por tromboelastometría en neuroblastoma complicado con coagulación intravascular diseminada.

BACKGROUND: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery. OBSERVATION: A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle. She remained stable without clinical bleeding and emergent tumor resection guided by intraoperative-thromboelastometry was decided. DIC resolved early after surgery and complete remission was achieved. CONCLUSION: Treatment of the underlying condition is critical to manage DIC. Thromboelastometry can guide goal-directed therapy, including surgery in pediatric patients. However, larger studies are needed to examine its applicability in different clinical settings, such as cancer related DIC.

INTRODUCCION: La coagulación intravascular diseminada (CID) es una urgencia oncológica poco común. Describimos el caso de un neuroblastoma pediátrico complicado con CID que precisó de cirugía guiada por tromboelastometría. CASO CLINICO: Paciente de seis años diagnosticada de neuroblastoma suprarrenal de riesgo intermedio que desarrolló CID asociada al tumor tras el primer ciclo de quimioterapia. Permaneció estable sin hemorragia clínica, decidiéndose una resección tumoral de urgencia guiada por tromboelastometría intraoperatoria. La CID se resolvió poco después de la cirugía, consiguiéndose una remisión total. CONCLUSION: El tratamiento de la patología subyacente es clave a la hora de manejar la CID. La tromboelastometría puede guiar la terapia orientada a objetivos, también en cirugías realizadas en pacientes pediátricos. No obstante, hacen falta mayores estudios que analicen su aplicabilidad en distintos contextos clínicos, como la CID relacionada con cáncer.

Cirugía guiada por tromboelastometría en neuroblastoma complicado con coagulación intravascular diseminada / Thromboelastometry-guided surgery in neuroblastoma complicated with disseminated intravascular coagulation

Introducción: La coagulación intravascular diseminada (CID) es una urgencia oncológica poco común. Describimos el caso de un neuroblastoma pediátrico complicado con CID que precisó de cirugía guiada por tromboelastometría. Caso clínico. Paciente de seis años diagnosticada de neuroblastoma suprarrenal de riesgo intermedio que desarrolló CID asociada al tumor tras el primer ciclo de quimioterapia. Permaneció estable sin hemorragia clínica, decidiéndose una resección tumoral de urgencia guiada por tromboelastometría intraoperatoria. La CID se resolvió poco después de la cirugía, consiguiéndose una remisión total. Conclusión. El tratamiento de la patología subyacente es clave a la hora de manejar la CID. La tromboelastometría puede guiar la terapia orientada a objetivos, también en cirugías realizadas en pacientes pediátricos. No obstante, hacen falta mayores estudios que analicen su aplicabilidad en distintos contextos clínicos, como la CID relacionada con cáncer.(AU)

Background: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery. Observation. A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle. She remained stable without clinical bleeding and emergent tumor resection guided by intraoperative-thromboelastometry was decided. DIC resolved early after surgery and complete remission was achieved. Conclusion. Treatment of the underlying condition is critical to manage DIC. Thromboelastometry can guide goal-directed therapy, including surgery in pediatric patients. However, larger studies are needed to examine its applicability in different clinical settings, such as cancer related DIC.(AU)

Hallazgos histológicos y seguimiento clínico de pacientes con mEn 2 tras tiroidectomía profiláctica / Clinical follow up and histological findings of patients With men2 treated With prophylactic thyroidectomy

Objetivo. Presentar nuestra experiencia en el manejo profiláctico mediante tiroidectomía del carcinoma medular tiroideo, revisando la correlación entre los hallazgos clínicos, analíticos, histopatológicos y genéticos en sujetos con síndrome de neoplasia endocrina múltiple tipo 2A (MEN 2A) y carcinoma medular familiar de tiroides (CMFT).materiales y métodos. Estudio retrospectivo mediante revisión de historias clínicas de pacientes con diagnóstico de MEN 2A o CMFT, derivados a nuestra consulta tras consejo genético desde 1997 hasta 2011. Se analizaron las variables sexo, edad al diagnóstico y a la cirugía, valores pre y postoperatorios de calcitonina (y metanefrinas en pacientes con MEN 2A), hallazgos histopatológicos, seguimiento y supervivencia. Resultados. Se identificaron 13 pacientes con antecedentes familiares de CMT, 9 mujeres y 4 varones, 11 portadores de mutación en protooncogén RET para MEN 2A y 2 no portadores (CMFT). La edad media al diagnóstico fue 4,2 años (rango: 1,8-8,2). Todos los pacientes fueron tratados mediante tiroidectomía total con una edad media de 6 años (rango: 4,1-8,5). Los hallazgos histopatológicos demostraron 7 casos de hiperplasia nodular de células C, 2 microcarcinomas, 1 carcinoma multicéntrico, 1 tiroiditis linfocitaria y 2 sin evidencia de enfermedad. La calcitonina preoperatoria estaba elevada en 3 casos, coincidiendo en uno con histopatología de microcarcinoma. Todos los pacientes se encuentran libres de enfermedad.Conclusiones. La tiroidectomía profiláctica precoz es la única herramienta preventiva y curativa para el CMT en pacientes susceptibles genéticamente de padecerlo. Es imprescindible la creación de equipos multidisciplinarios (Endocrinología, Genética Clínica y Cirugía Pediátrica) para el estudio, manejo y seguimiento de los pacientes con MEN 2A y sus familias (AU)

Objective. To present our experience in the prophylactic management of the medullary thyroid carcinoma (MTC), reviewing the correlation between clinical, analytical, histopathological, and genetic findings on subjects with type 2A multiple endocrine neoplasia (MEN 2A) and familial MTC.material and methods. A retrospective study was done by reviewing the medical records of patients diagnosed with MEN 2A or familial MTC, between 1997 and 2011. The variables studied were sex, age at the time of diagnosis, age at the time of surgery, pre and post operative Calcitonin levels, pre and post op Metanephrine levels in patients with MEN 2A, histopathological findings, follow up and overall survival.Results. Thirteen patients were identified with family history of MTC, 9 females and 4 males. Eleven carriers of mutation on RET proto-oncongene for MEN 2A and no carriers for Familial MTC. The median age at the time of diagnosis was 4.2 years (range: 1.8 to 8.2). All patients were treated with total thyroidectomy, with a median age of 6 years (range: 4.08 to 8.5). The histopathological findings demonstrated 7 cases of C-Cells nodular hyperplasia, 2 micro-carcinomas, 1 multicentric carcinoma, 1 lymphocytic thyroiditis and 2 without evidence of disease. Elevated pre operative Calcitonin levels were found in 3 cases, correlated with one histopathological finding of micro-carcinoma. All patients are disease free.Conclusion. In patients with genetic predisposition to suffer the disease, early prophylactic thyroidectomy is the only current available approach to prevent and cure MTC.The creation of a multidisciplinary team (Endocrinology, clinical genetics, and pediatric surgery), is necessary to study, manage and follow up patients with MEN 2A and their families (AU)

[Clinical follow up and histological findings of patients with MEN2 treated with prophylactic thyroidectomy]. / Hallazgos histológicos y seguimiento clínico de pacientes con MEN 2 tras tiroidectomía profiláctica.

OBJECTIVE: To present our experience in the prophylactic management of the medullary thyroid carcinoma (MTC), reviewing the correlation between clinical, analytical, histopathological, and genetic findings on subjects with type 2A multiple endocrine neoplasia (MEN 2A) and familial MTC. MATERIAL AND METHODS: A retrospective study was done by reviewing the medical records of patients diagnosed with MEN 2A or familial MTC, between 1997 and 2011. The variables studied were sex, age at the time of diagnosis, age at the time of surgery, pre and post operative Calcitonin levels, pre and post op Metanephrine levels in patients with MEN 2A, histopathological findings, follow up and overall survival. RESULTS: Thirteen patients were identified with family history of MTC, 9 females and 4 males. Eleven carriers of mutation on RET proto-oncongene for MEN 2A and no carriers for Familial MTC. The median age at the time of diagnosis was 4.2 years (range: 1.8 to 8.2). All patients were treated with total thyroidectomy, with a median age of 6 years (range: 4.08 to 8.5). The histopathological findings demonstrated 7 cases of C-Cells nodular hyperplasia, 2 micro-carcinomas, 1 multicentric carcinoma, 1 lymphocytic thyroiditis and 2 without evidence of disease. Elevated pre operative Calcitonin levels were found in 3 cases, correlated with one histopathological finding of micro-carcinoma. All patients are disease free. CONCLUSION: In patients with genetic predisposition to suffer the disease, early prophylactic thyroidectomy is the only current available approach to prevent and cure MTC. The creation of a multidisciplinary team (Endocrinology, clinical genetics, and pediatric surgery), is necessary to study, manage and follow up patients with MEN 2A and their families.

Manejo quirúrgico del tumor de Wilms bilateral: Nuestra experiencia con 18 casos / Surgical management of the bilateral wilm's tumor: Our experience with 18 cases

Objetivo. Mostrar nuestra experiencia en el manejo quirúrgico del tumor de Wilms bilateral. Material y métodos. Se analizaron los datos de 18 niños con diagnóstico de TW bilateral entre 1971 y 2007, evaluando la edad al momento del diagnóstico, sexo, presentación clínica, métodos diagnósticos, histología, tratamiento quimioterápico, radioterápico y quirúrgico, complicaciones y el estado clínico actual de los pacientes. Resultados. El 65% de los TW sincrónicos se encontraban en estadios I / II; el 30% mostraban al menos un tumor en estadio III. Un caso en estadio IV (5%). Los pacientes con TW metacrónicos se encontraban en el 100% de los casos en estadios I /II. Todos los tumores fueron de bajo o intermedio grado de malignidad, con predominio del tipo mixto. Las complicaciones quirúrgicas fueron 4 suboclusiones intestinales,2 fístulas ureteropiélicas y 1 quiste urinario.15 niños permanecen convida (83%) con un período libre de enfermedad entre 1 y 24 años, delos cuales 3 se han trasplantado con buena evolución. Un paciente falleció por insuficiencia renal progresiva y otros dos pacientes por evolución de la enfermedad. Conclusiones. La quimioterapia preoperatoria citorreductora permite una cirugía renal más conservadora con una alta tasa de supervivencia (80-90%). El tratamiento quirúrgico individualizado conlleva resecciones más conservadoras y una menor incidencia de insuficiencia renal a largo plazo (AU)

Aim. To show our experience in the surgical management of bilateral Wilms’ tumor. Methods. We have reviewed the medical records of 18 patients diagnosed of bilateral Wilms’ tumor between 1971 and 2007, evaluating age, sex, clinical situation, imaging studies, histology, treatment, complications and follow-up. Results. 65% of patients with synchronous Wilms’ tumor was stageI-II, 30% stage III and 5% stage IV. 100% of patients with metachronous Wilms’ tumor was stage I-II. All the tumors had favourable histology. Surgical complications were: 4 bowel pseudobstructions, 2ureteropielic fistulae and 1 urinary cyst. 15 patients are alive (83%) with a mean follow-up of 12 years.3 of these patients had a renal transplant with a good evolution. One patient died of a progressive renal failure and two patients died of the evolution of the oncological disease. Conclusions. Preoperative chemotherapy allows a conservative surgical resection with a high overall survival (80-90%). Individualized surgical treatment offers a conservative surgical resection with a lower incidence of long-term renal failure (AU)

Protocolo antibiótico “fast-track” en la apendicitis aguda / Fast track antibiotic protocol in acute appendicitis

Introducción. El auge de las resistencias antimicrobianas, ha producido un descenso en la efectividad de la tradicional triple terapia antibiótica, con el consiguiente aumento de las complicaciones. En este contexto, decidimos cambiar a Cefuroxima-Metronidazol o Ertapenem en función del riesgo de presentar resistencias. Este nuevo protocolo se ajusta a la filosofía Fast-Track, siendo factible el alta tras 72 horas de antibioterapia. Nuestro objetivo es conocer si ha mejorado nuestra tasa de complicaciones infecciosas y si es factible el alta precoz. Material y métodos. Realizamos un estudio de cohortes históricas:– A la cohorte histórica (CH) pertenecen pacientes apendicectomizados entre octubre 05 a octubre 06.– La cohorte actual (CA) comienza a recogerse en junio 2007.En ambos grupos se procede con idéntico protocolo de recogida de datos (un solo observador). Se procede a comparar la homogeneidad entre las cohortes y posteriormente al análisis de los resultados ( a<0,05).El análisis estadístico se realizó con el SPSS 15.0.Resultados. Se revisan 226 pacientes cursando 110 como apendicitis complicadas (CH: 61/135; CA: 49/91). No existen diferencias (..) (AU)

Introduction. The increase of antimicrobial resistances, has affected the efficacy of antimicrobial triple therapy, increasing appendicitis morbidity. We decided to change to a fast-track protocol of 72 hours ofCefuroxime-Metronidazol or Ertapenem. Aim: to know if our infectious morbidity rate has improved and if early dischargement is possible. Material and methods. Analytic historic cohort study:– Historical cohort (HC): patients intervened of appendicitis between October 2005and October 2006.– Current cohort (CC): started in June 2007.A data collection protocol is designed for both groups. Homogeneity among cohorts is proved and data are analysed (a=0,05). Statistics are analyzed by SPSS 15.0.Results. 226 patients were controlled, being 110 complicated appendicitis (HC: 61/135; AC: 49/91). There are no differences among cohorts about evolution time, temperature and leukocytes rate at admission, interval to intervention, use of laparoscopy, drainage or antimicrobial (..) (AU)

[Surgical management of bilateral Wilms' tumor: our experience with 18 cases]. / Manejo quirúrgico del tumor de Wilms bilateral: nuestra experiencia con 18 casos.

AIM: To show our experience in the surgical management of bilateral Wilms' tumor. METHODS: We have reviewed the medical records of 18 patients diagnosed of bilateral Wilms' tumor between 1971 and 2007, evaluating age, sex, clinical situation, imaging studies, histology, treatment, complications and follow-up. RESULTS: 65% of patients with synchronous Wilms' tumor was stage I-II, 30% stage III and 5% stage IV. 100% of patients with metachronous Wilms' tumor was stage I-II. All the tumors had favourable histology. Surgical complications were: 4 bowel pseudobstructions, 2 ureteropielic fistulae and 1 urinary cyst. 15 patients are alive (83%) with a mean follow-up of 12 years. 3 of these patients had a renal trasplant with a good evolution. One patient died of a progressive renal failure and two patients died of the evolution of the oncological disease. CONCLUSIONS: Preoperative chemotherapy allows a conservative surgical resection with a high overall survival (80-90%). Individualized surgical treatment offers a conservative surgical resection with a lower incidence of long-term renal failure.

[Chromophobe renal cell carcinoma, an exceptional entity in childhood: a case report]. / Carcinoma renal de células cromófobas, una entidad excepcional en la infancia: caso clínico.

Chromophobe renal cell carcinoma (CRCC) is a rare variant of renal carcinomas arising from the intercalated cells of the distal renal tubule and representing 5% among all renal tumors. Its biological behaviour is variable, less aggresive than clear cell renal carcinoma. Histochemical, ultrastructural and molecular genetic characteristics are different from other renal carcinomas. Age at presentation is about the 6th decade of life. We report an exceptional 10 year-old boy case with a CRCC. Diagnostic and therapeutic aspects for the management of this tumor are reviewed.

Carcinoma renal de células cromófobas, una entidad excepcional en la infancia: caso clínico / Chromophobe renal cell carcinoma, an exceptional entiy in childhood: a case report

El carcinoma renal de células cromófobas (CRCC) es una variante rara de carcinoma renal que se origina de las células intercaladas del epitelio tubular distal y constituye un 5% de los tumores renales. Es un tumor de comportamiento biológico variable, menos agresivo que el carcinoma convencional de células claras. Presenta características histoquímicas, ultra estructurales y genéticas diferentes al resto de carcinomas renales. La edad de presentación suele ser hacia la 6ª década de vida. Presentamos un caso excepcional de CRCC en un niño de 10 años de edad. Se revisan los aspectos diagnósticos y terapéuticos del manejo de este tumor (AU)

Chromophobe renal cell carcinoma (CRCC) is a rare variant of renal carcinomas arising from the intercalated cells of the distal renal tubule and representing 5% among all renal tumors. Its biological behaviouris variable, less aggresive than clear cell renal carcinoma. Histochemical, ultrastructural and molecular genetic characteristics are different from other renal carcinomas. Age at presentation is about the 6th decade of life. We report an exceptional 10 year-old boy case with a CRCC. Diagnostic and therapeutic aspects for the management of this tumor are reviewed (AU)

Calendario quirúrgico infantil / Pediatric surgical timetable

El calendario quirúrgico es una guía que intenta dar a conocer el momento más oportuno para la realización de la mayoría de las intervenciones quirúrgicas en el niño. Este calendario se modifica por el desarrollo de nuevas técnicas quirúrgicas. La elaboración de un calendario quirúrgico es beneficioso para obtener mejores resultados y ayuda al pediatra en la toma de decisiones

The surgical timetable is a practical guide that attempts to indicate the best moment for carrying out most of the surgical procedures to be performed in children. This timetable is modified depending on the introduction of new surgical techniques. The development of a surgical timetable improves results and helps the pediatrician in the decisionmaking process

[Prophylactic thyroidectomy in children at risk for medullary thyroid carcinoma]. / Tiroidectomía profiláctica en pacientes pediátricos con riesgo de carcinoma medular tiroideo.

The medullary thyroid carcinoma (MTC) is a rare neoplasia occurring during childhood. At present time the molecular examination of the proto-oncogen RET, related to syndromes of multiple endocrine neoplasia (MEN II) and familial medullary thyroid carcinoma (FMTC) to allows identify patients with risk of suffering of medullary thyroid carcinoma in early ages, before the disease becomes clinically pronunced. Children with familial antecedents of MEN II or FMTC were biochemically (pentagastrin-stimulated) and genetically studied with the purpose of determining the risk of developing a MTC and in order to assess the possibilities of making a prophylactic thyroidectomy.

[Spigelian hernia: two cases associated to cryptorchidism]. / Hernia de Spiegel: a propósito de 2 casos asociados a criptorquidia.

Spigelian hernia (SH) is rare in children, it occurs primarily en adults the mean age of presentacion is 50 years. We present two patients with hernia associated to cryptorchidism one of them bilateral.

Tiroidectomía profiláctica en pacientes pediátricos con riesgo de carcinoma medular tiroideo / Prophylactic thyroidectomy in children at risk for medullary thyroid carcinoma

El carcinoma medular de tiroides (CMT) es una rara neoplasiaen la infancia. En la actualidad el estudio molecular del proto-oncogén RET, relacionado con los síndromes de neoplasia endocrina múltiple (MEN II) y carcinoma medular familiar de tiroides (CMFT)permiten identificar pacientes con riesgo de padecer carcinoma medularde tiroides en edades precoces, antes de que se manifieste la enfermedad clínicamente. Niños con antecedentes familiares de MEN II o CMFT fueron estudiados bioquímica (test de pentagastrina) y genéticamente con el fin dedeterminar el riesgo de padecer CMT y evaluar la posibilidad de realizar tiroidectomía profiláctica (AU)

The medullary thyroid carcinoma (MTC) is a rare neoplasiaoccurring during childhood. At present time the molecular examinationof the proto-oncogen RET, related to syndromes of multipleendocrine neoplasia (MEN II) and familial medullary thyroid carcinoma(FMTC) to allows identify patients with risk of suffering of medullarythyroid carcinoma in early ages, before the disease becomes clinicallypronunced. Children with familial antecedents of MEN II orFMTC were biochemically (pentagastrin-stimulated) and geneticallystudied with the purpose of determining the risk of developing a MTCand in order to assess the possibilities of making a prophylactic thyroidectomyThe medullary thyroid carcinoma (MTC) is a rare neoplasia occurring during childhood. At present time the molecular examinationof the proto-oncogen RET, related to syndromes of multiple endocrine neoplasia (MEN II) and familial medullary thyroid carcinoma(FMTC) to allows identify patients with risk of suffering of medullary thyroid carcinoma in early ages, before the disease becomes clinically pronunced. Children with familial antecedents of MEN II orFMTC were biochemically (pentagastrin-stimulated) and genetically studied with the purpose of determining the risk of developing a MTCand in order to assess the possibilities of making a prophylactic thyroidectomy (AU)

Hernia de Spiegel: A propósito de 2 casos asociados a criptorquidia / Spigelian: two cases associated to cryptorchidism

La hernia de Spiegel es una hernia rara en la población infantil, más común en la población adulta con predominio en la 5ª década de la vida. Presentamos dos pacientes con hernia asociada a criptorquidia, una de ellas bilateral (AU)

Spigelian hernia (SH) is rare in children, it occurs primarily en adults the mean age of presentacion is 50 years. We present two patients with hernia associated to cryptorchidism one of them bilateral (AU)

[Doppler echocardiographic evaluation of pulmonary hypertension in children]. / Valoración con ecocardiografía Doppler de la hipertensión pulmonar del niño.

INTRODUCTION: We analysed the usefulness of Doppler echocardiography to determine the presence and severity of pulmonary hypertension (PH) in children. METHOD: The whole group consisted of 63 patients, 42 with congenital heart disease that underwent cardiac catheterization (32 of whom had PH = study group) and 21 healthy children. These 21 patients and the remaining 10 without PH at cardiac catheterization made up the control group. All children were studied with Doppler Echocardiography to evaluate the pulmonary flow pattern with the sample volume placed in the pulmonary artery trunk, 1 cm distal to the pulmonic valve. The preejection period (PEP), ejection period (EP), acceleration time (AcT), the indexes PEP/EP, PEP/AcT, AcT/EP and the morphologic pattern of the pulmonary flow (type I: with peak flow velocity at midsystole; type II: with peak flow velocity in early systole; type III: with midsystolic notching) were analysed and quantitative parameters corrected according to hear rate by dividing theirs value by the square root of R-R interval. In the hemodynamic study we analysed the systolic (SPAP), diastolic and mean pulmonary artery pressure, and the mean pulmonary pressure/mean systemic pressure ratio (Pp/Sp). We compared the echocardiographic variables in both, study and control groups, and analysed the hemodynamic and echocardiographic correlation between the variables in question. RESULTS: Pattern I of pulmonary flow was associated with absence of PH and pattern II and III with PH (p < 0.001). The best results of quantitative variables were either corrected AcT (AccT) rather less in the study group than in control group (2.89 +/- 0.56 vs 4.05 +/- 0.56 ms, p < 0.001) and PPE/AcT index, 1.28 +/- 0.3 in the hypertensive group and 0.78 +/- 0.16 in the control group (p < 0.001). The best correlation were AcT with SPAP (r = -0.82) and Act with Pp/Sp ratio (r = -0.84). CONCLUSIONS: We consider that pulmonary flow analysed with Doppler echocardiography is a reliable, suitable and non-invasive method to evaluate PH in children.

[Evaluation of the surgical correction in complete atrioventricular channels]. / Evaluación de la corrección quirúrgica en los canales auriculoventriculares completos.

We review our experience of surgical correction in atrioventricular canals between 1979 and 1991. 81 patients, ranging in ages from 5 to 109 months (with an average of 33 months) and weight from 4 to 25 kg (with an average of 9 kg), underwent primary repair. Another cardiac anomalies associated were ruled out, except patency of the arterial ductus. 33 patients (46%) presented the complete form of atrioventricular canals, 27 (33%) the partial form and 17 (21%) the transitional form. 55 patients (68%) of the sample had Down's syndrome. Regarding the cases with the complete form they were frequently associated to Down's syndrome. Thus, 89% of the cases of complete form had Down's syndrome. All operations for complete form cases used a two-patch technique and physiological reconstruction of the left atrioventricular valve. No patient underwent pulmonary artery banding or was discharged from surgery correction due to pulmonary pressure or resistance. The average age of the children with complete form was 19.5 months (11 months from 1986). The main hemodynamic parameters were mean pulmonary arterial pressure of 57 +/- 12 mmHg, pulmonary-systemic pressure relation of 0.87 +/- 0.12 and total pulmonary resistance of 6.3 +/- 4.0 U/m2. The hospital mortality was 32% in the complete form, comparable to samples of similar characteristics. We related this mortality with the hemodynamic profile at the time of surgical correction, compatible with pulmonary vascular obstructive disease, with the elevated percentage of Down's syndrome and with the delay in the surgical operation age.