[Urethroplasty with lateral pedunculated flap for complicated hypospadias]. / Uretroplastia con colgajo lateral pediculado para las hipospadias complicadas.

UNLABELLED: With the modern techniques, we can resolve almost the totallity of hypospadias. But there are patients with recurrent fistulas associated to uretral stenosis that can finish all the surgical possibilities. MATERIAL AND METHODS: Retrospective review. Lateral based flap uretroplasty consists in the exposition of the uretra, to reconstruct in one-stage the original neourethra with the lateral skin of the penis. RESULTS: From 2008, we have correct 5 patients with a mean age of 12,1 years (9-15) and a weight of 55,34 kg (22-98 kg). All of them were previously corrected 3 to 7 times, with recurrent fistulas. At the office, stenosis urethral was verified. Surgical correction was made in 90-110 minutes and results were positive in all patients, correcting the stenosis after a follow-up of more than a year. Only the oldest two patients had present minimally glans fistula. CONCLUSIONS: Lateral based flap urethroplasty is a useful technique for the correction of the complicated fistulas, allowing the correction of the fistulas and the stenosis of the urethra.

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)

Uretroplastia con colgajo lateral pediculado para las hipospadias complicadas / Urethroplasty with lateral pedunculated flap for complicated hypospadias

Las técnicas habituales de los hipospadias permiten resolver la práctica totalidad de esta patología con resultados muy positivos. Desgraciadamente, existen pacientes que se fistulizan repetidas veces, agotando las posibilidades quirúrgicas. Generalmente se asocian a estenosis uretral. Queremos presentar una técnica que nos ha facilitado la reparación de estos pacientes con resultados esperanzadores . Material y métodos. Revisión retrospectiva de una serie de casos. La técnica del colgajo lateral pediculado consiste en un desmontaje dela uretra y exposición de la misma, para proceder a su reconstrucción en un solo tiempo por medio de la piel lateral de la uretra que se tubulariza sobre la neouretra primitiva. Resultados. Desde 2008 hemos intervenido 5 pacientes con una edad media de 12,1 años (9-15) y un peso de 55,34 kg (22-98 kg).Previamente habían sufrido de 3 a 7 operaciones de corrección de hipospadias, siempre fistulizadas. En consulta se comprobó la estenosis uretral en todos los pacientes. La intervención se realizó en un tiempo de 90-110 minutos y los resultados fueron favorables en todos los pacientes, resolviéndose las estenosis, con un seguimiento medio superior al año. Tan solo los dos pacientes más mayores han presentado mínimas fístulas glanulares. Conclusiones. La uretroplastia con colgajo lateral pediculado es una técnica útil para el tratamiento de los hipospadias complicados permitiendo la resolución de las fístulas y, especialmente, de las estenosis de uretra (AU)

With the modern techniques, we can resolve almost the totallity of hypospadias. But there are patients with recurrent fi stulas associated touretral stenosis that can finish all the surgical possibilities. Material and methods. Retrospective review. Lateral based flapuretroplasty consists in the exposition of the uretra, to reconstruct in one-stage the original neourethra with the lateral skin of the penis. Results. From 2008, we have correct 5 patients with a mean age of 12,1 years (9-15) and a weight of 55,34 kg (22-98 kg). All of them were previously corrected 3 to 7 times, with recurrent fistulas. At the office, stenosis urethral was verified. Surgical correction was made in 90-110 minutes and results were positive in all patients, correcting the stenosis after a follow-up of more than a year. Only the oldest two patients had present minimally glansfistula. Conclusions. Lateral based flap urethroplasty is a useful technique for the correction of the complicated fistulas, allowing the correction of the fistulas and the stenosis of the urethra (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.

[PHACES syndrome]. / Síndrome de PHACES.

INTRODUCTION: PHACES syndrome associates a segmental facial hemangioma with cerebral malformations, aortic branches/cranial arteries anomalies, cardiac defects, eye anomalies or ventral wall defects. The aim of this study is to analyze our experience with this syndrome. MATERIAL AND METHODS: Retrospective study of the cases seen at our unit in the last year. RESULTS: We treat 4 cases; 3 girls and 1 child. Besides the segmental hemangioma they presented: 3 vascular cerebral malformations; 2 structural cardiopathies; 2 cerebral malformations, 1 microftalmia. We did not find ventral wall defects. A case received treatment with two cycles of metilprednisolone i.v. and oral prednisone, with favourable course; two cases received initial treatment with oral prednisone continued of oral propanolol in rising pattern up to 2 mg/kg/day, Obtaining both the detention of the tumour growth and regression of the lesion, with very good tolerance. A 7-year-old patient has been treated with colouring pulse laser for her residual lesions. CONCLUSIONS: When we see a segmental facial hemangioma we must perform a wide diagnostic study in order to discard a PHACES syndrome. Multidisciplinar approach to the patient by a wide expert's group gets an earlier diagnose and improves the outcome. Propranolol is a promising therapeutic alternative.

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.