Spanish primary care in pediatric trauma (AITP) consensus: An AITP checklist. / Consenso AITP español: Una lista de verificación AITP.

INTRODUCTION: The course in Primary Care in Pediatric Trauma (ATIP in Spanish) has been taught in Spain since 1997, and there are currently 9 accredited training centers. Care of polytraumatized pediatric patients often takes place in an environment conducive to errors resulting from forgetfulness, which is why checklists - mnemonic tools widely used in industry and medicine - are particularly useful to avoid such errors. Although several checklists exist for pediatric trauma care, none have been developed within the setting of our course. MATERIALS AND METHODS: The criteria for being selected as an expert in Primary Care in Pediatric Trauma were agreed upon with the scientific polytrauma committee of the Spanish Pediatric Surgery Society. The items that make up the checklist were obtained from a review of the literature and consultation with selected experts, using the Delphi Technique. RESULTS: 10 experts representing the 9 groups or training centers in Primary Care in Pediatric Trauma were selected, and a 28-item checklist was drawn up in accordance with their design recommendations. CONCLUSIONS: With the consensus of all the groups, a checklist for the treatment of polytraumatized pediatric patients was drawn up using the Delphi Technique, an essential requirement for the dissemination of this checklist, which should be adapted and validated for use in each healthcare center.

INTRODUCCION: El curso de Asistencia Inicial al Trauma Pediátrico se imparte en España desde 1997, existiendo en la actualidad 9 centros formadores acreditados. La asistencia al paciente pediátrico politraumatizado se produce muchas veces en un ambiente proclive al error por olvido, por lo que las listas de verificación, como herramientas mnemotécnicas de amplia difusión en la industria y en medicina, serían especialmente útiles para evitarlos. Aunque existen varias listas de verificación para la asistencia al traumatismo pediátrico, ninguna se ha desarrollado en el entorno de nuestro curso. MATERIAL Y METODOS: Se acordaron los criterios para ser seleccionado como experto en Asistencia Inicial al Trauma Pediátrico con la comisión científica de politrauma de la Sociedad Española de Cirugía Pediátrica. Los ítems para formar la lista de verificación se obtuvieron a partir de una revisión bibliográfica y de la consulta a los expertos seleccionados, empleando un método Delphi. RESULTADOS: Se seleccionaron 10 expertos que representan los 9 grupos o centros formadores en Asistencia Inicial al Trauma Pediátrico y se elaboró una lista de verificación con 28 ítems, siguiendo sus recomendaciones de diseño. CONCLUSIONES: Se diseñó una lista de verificación para el manejo del paciente pediátrico politraumatizado, con el consenso de todos los grupos empleando un método Delphi, requisito fundamental para facilitar la difusión de esta lista. Sería preciso adaptar y validar dicha lista para su uso en cada centro asistencial.

Thoracoscopic pneumonectomy in a 4 year-old-child with destroyed lung following viral pneumonia. / Tiroidectomía transoral endoscópica con abordaje vestibular (TOETVA): una nueva opción para el tratamiento de pacientes pediátricos.

Pediatric pneumonectomies are exceptional nowadays, being reserved for cases with destroyed lungs with frequent exacerbations and reinfections and only two cases of thoracoscopic pneumonectomy have been previously published. We present the case of a 4-year-old patient with no relevant history who developed complete atelectasis of the left lung (LL) after influenza A pneumonia, followed by secondary recurrent infections. A year later a diagnostic bronchoscopy without alterations was performed. A complete loss of volume and hypoperfusion of the LL (right lung perfusion 95%, LL perfusion: 5%) with bronchiectasis and hyperinsufflation and herniation of the right lung into the left hemithorax was observed in a pulmonary perfusion SPECT-CT. After unsuccessful conservative management and recurrent infections a pneumonectomy was indicated. The pneumonectomy was performed through a five-port thoracoscopy. The dissection of the hilum was made using hook electrocautery and sealing device. The left main bronchus was sectioned with an endostapler. There were no intraoperative complications. An endothoracic drain was removed the first postoperative day. The patient was discharged on the fourth postoperative day. The patient has not presented any complications 10 months after surgery. Although pneumonectomy is an exceptional surgery in children, it can be performed by minimally invasive surgery with success and safety in centers with extensive experience in pediatric thoracoscopic surgery.

Hoy en día, las neumonectomías pediátricas son algo excepcional. El procedimiento se reserva para aquellos casos en los que los pulmones están destruidos y presentan exacerbaciones y reinfecciones frecuentes, con tan solo dos casos de neumonectomía toracoscópica publicados hasta la fecha. Presentamos el caso de un paciente de 4 años sin antecedentes de interés que desarrolló atelectasia completa del pulmón izquierdo (PI) tras neumonía por gripe A, seguido de infecciones secundarias recurrentes. Un año después, se le practicó broncoscopia diagnóstica, sin que esta mostrara alteraciones significativas. Tras realizársele un SPECT-CT de perfusión pulmonar, se evidenció pérdida completa de volumen e hipoperfusión del PI (perfusión del pulmón derecho: 95%; perfusión del pulmón izquierdo: 5%), con bronquiectasia e hiperinsuflación y herniación del pulmón derecho hacia el hemitórax izquierdo. Tras fracasar el manejo conservador y registrarse infecciones recurrentes, se estableció la indicación de neumonectomía. La neumonectomía se llevó a cabo mediante toracoscopia por cinco puertos. La disección del hilio se realizó mediante gancho de electrocoagulación y dispositivo de sellado. El bronquio principal izquierdo se seccionó con endograpadora. No se registraron complicaciones intraoperatorias. El drenaje endotorácico se retiró al día siguiente de la intervención, mientras que el paciente fue dado de alta a los cuatro días, sin que haya presentado complicaciones transcurridos 10 meses desde la cirugía. Aunque la neumonectomía es una intervención excepcional en niños, puede llevarse a cabo de manera exitosa y segura por cirugía mínimamente invasiva en centros con amplia experiencia en cirugía toracoscópica pediátrica.

Quality of life in adolescents who underwent congenital diaphragmatic hernia surgical repair. / Calidad de vida en adolescentes intervenidos de hernia diafragmática congénita.

OBJECTIVE: The advances made in the surgical and postnatal treatment of congenital diaphragmatic hernia (CDH) have considerably improved patient survival, but morbidity remains significant. The objective of this study was to analyze the effect these sequels have on the health-related quality of life (HRQL) of adolescents and young adults who have survived CDH, and to compare it with that of the general population. MATERIALS AND METHODS: A transversal descriptive study of patients diagnosed with CDH in our institution from 1997 to 2004 was carried out. Survival, location, hernia size, herniated organs, need for extracorporeal membrane oxygenation, and mechanical ventilation time were analyzed. In addition, a comparative study of the current HRQL of survivors was conducted using the SF-36 survey (36-Item Health Survey Short Form), which assessed physical function, physical role, body pain, general health, vitality, social function, emotional role, and mental health. Data of 24 healthy adolescents was used as a control group. RESULTS: Of the 29 survivors (70.7%), 21 were successfully contacted, and 16 responded to the survey. They all claimed their overall quality of life was good or very good. The group of adolescents who underwent CDH surgical repair had better results in the vitality (p= 0.001) and mental health (p<0.05) areas, but the overall HRQL score and the remaining health areas were similar. No significant differences were found regarding diaphragmatic size or need for ECMO. CONCLUSION: According to adolescent survivors who underwent CDH surgical repair, their quality of life is similar to that of other individuals of their age. Our results are encouraging and may prove useful for future parents of CDH patients.

OBJETIVO: Los avances en el tratamiento quirúrgico y posnatal han mejorado significativamente la supervivencia de pacientes con hernia diafragmática congénita (HDC). La morbilidad asociada sigue siendo significativa. El objetivo del estudio es evaluar efecto de estas secuelas sobre la calidad de vida relacionada con la salud (CVRS) de adolescentes y adultos jóvenes supervivientes de HDC y compararla con la población general. MATERIAL Y METODOS: Estudio descriptivo transversal de pacientes diagnosticados de HDC entre 1997 y 2004. Supervivencia, localización, tamaño de la hernia, órganos herniados, necesidad de oxigenación por membrana extracorpórea, tiempo de ventilación mecánica. Estudio comparativo de CVRS actual de pacientes supervivientes mediante la encuesta SF-36 (36-Item Health Survey Short Form): función física, rol físico, dolor corporal, salud general, vitalidad, función social, rol emocional, salud mental. Utilizamos datos de 24 adolescentes sanos como grupo control. RESULTADOS: De los 29 supervivientes (70,7%), fueron localizados 21 y contestaron la encuesta 16, que consideraron tener una calidad de vida global buena o muy buena. El grupo de adolescentes intervenidos tuvieron mejores resultados en las esferas de vitalidad (p = 0,001) y salud mental (p<0,05), pero la puntuación de la CVRS global y el resto de dimensiones de salud fueron similares. No se objetivaron diferencias significativas en relación con el tamaño diafragmático ni la necesidad de ECMO. CONCLUSIONES: Los adolescentes supervivientes intervenidos de HDC consideran tener una calidad de vida similar a jóvenes de su misma edad. Los resultados de nuestro estudio son alentadores y permiten un mejor asesoramiento para futuros pacientes con HDC.

Laparoscopic learning curves. / Curvas de aprendizaje en laparoscopia.

OBJECTIVES: Laparoscopic learning curves are slow, and there are no uniform surgical skill acquisition models. Therefore, our objective was to assess a laparoscopic skill learning program in individuals without any surgical experience, analyzing the learning curve by means of a certified custom-made simulator, and evaluating whether previous surgical experience had an impact on the learning curve. MATERIAL AND METHODS: A certified custom-made simulator and laparoscopic instruments were used to assess 20 university students who performed 10 repetitions of 3 exercises of growing difficulty (eye-hand coordination, hand-hand coordination, and cutting). Three parameters were analyzed: total time with each hand, total mistakes with each hand, and three items of the OSATS technical skill scale. The two first exercises were compared with a group of 14 experienced surgeons. Statistical analysis using repeated-measures Anova and Student's t-test was carried out (p < 0.05). RESULTS: Significant time improvement with each repetition was demonstrated in the three exercises. Curve stabilization was faster in surgeons (2-4 repetitions) than in students (8-9). Time reduction was noted in the first and second exercises in both groups, with 44.08% and 33.1% shorter times, respectively. CONCLUSIONS: Individuals without surgical experience acquired basic laparoscopic skills using a custom-made simulator, which allows simple surgical techniques to be carried out in an inexpensive, accessible fashion. Previous surgical experience was associated with a shorter learning curve. The custom-made simulator allowed individuals with and without surgical experience to be distinguished from each other.

OBJETIVOS: La curva de aprendizaje en cirugía laparoscópica es lenta y no existen modelos uniformes de adquisición de habilidades quirúrgicas. Tratamos de establecer la idoneidad de un programa de aprendizaje de habilidades laparoscópicas en sujetos sin experiencia quirúrgica, analizando la curva de aprendizaje utilizando un simulador artesanal homologado. Comprobar si la experiencia quirúrgica previa modifica la curva de aprendizaje. MATERIAL Y METODOS: Se empleó un simulador artesanal validado e instrumental laparoscópico para evaluar a 20 estudiantes universitarios que realizaron 10 repeticiones de tres ejercicios de dificultad creciente (coordinación ojo-mano, coordinación mano-mano y corte). Se evaluaron tres parámetros: tiempo total y con cada mano, errores totales y con cada mano y tres ítems de habilidad técnica OSATS. Comparación de los dos primeros ejercicios con un grupo de 14 cirujanos con experiencia. Análisis estadístico mediante Anova para medidas repetidas y t de Student (p < 0,05). RESULTADOS: Se demostró la mejoría significativa del tiempo con cada repetición en los tres ejercicios. La estabilización de la curva fue más precoz entre los cirujanos (2-4 repeticiones) que los estudiantes (8-9). Se comprobó la reducción del tiempo invertido para el primer y segundo ejercicio en ambos grupos, que en los estudiantes fue del 44,08% y 33,1% respectivamente. CONCLUSIONES: Individuos sin experiencia quirúrgica desarrollan habilidades laparoscópicas básicas utilizando un simulador artesanal, que permite practicar técnicas quirúrgicas sencillas de forma barata y accesible. La experiencia quirúrgica previa se asocia con el acortamiento de la curva de aprendizaje. El simulador artesanal permite discriminar entre sujetos con y sin experiencia quirúrgica.

[Esophageal impaction of button batteries in childhood. How to avoid tragedy!]. / Impactación esofágica de pilas de botón en la infancia. Cómo evitar la tragedia!

INTRODUCTION: Ingestion of button batteries (PB) represents less than 2% of all foreign bodies, but its incidence is increasing. Esophageal impaction produces severe damages, which can be devastating. Our aim is to present 3 new cases and the therapeutic protocol followed in our center. MATERIAL AND METHODS: We report 3 children who presented esophageal foreign body impaction, displaying a PB with the "double halo sign" on the radiograph, being situated in the upper, medium and low esophageal in each case. The protocol followed included: radiography of the neck, chest and abdomen; high doses of corticosteroids, inhibitors of proton pump, antibiotics and urgent endoscopy removal. A nasogastric tube was placed in the same act. RESULTS: The PB had been impacted for 10.3 hours (5-14) but had inflammation and necrosis of the esophageal mucosa. We applied the specified protocol. Oral feeding was initiated 8 +/- 2 days after with a normal esophagogastric study, which was repeated a month later to exclude stenosis. CONCLUSION: Adherence to a diagnostic and therapeutic protocol prevents the complications of esophageal impaction of button batteries. We consider the double halo image pathognomonic of the PB.

[Modifiable prognostic factors in the morbidity-mortality of gastroschisis]. / Factores pronósticos modificables en la morbi-mortalidad de la gastrosquisis.

OBJECTIVE: The low incidence of gastroschisis makes impossible a consistently study of the factors that determine its evolution. The presence of other alterations associated is an important determinant of prognosis known. We analyze the factors implicated in morbidity and mortality in our center that can be modified. MATERIAL AND METHODS: We performed a retrospective study from hospital records. We analyzed the morbidity and mortality versus gestational age, mode of delivery, surgery performed, presence of prenatal diagnosis, herniated viscera and associated anomalies. We studied the postoperative differences occurred as a result of implantation of fetal surgery group. The variables were analyzed with SPSS 15.0 using non-parametric test. RESULTS: Since 1987 25 patients have been operated (12 men) with a mean birth weight of 2,328 g +/- 364. The 44% of them had prenatal diagnosis and 72% were born by cesarean. Only 4 had intestinal atresia. Preterm birth (< 36 weeks) did not improve the complications, but did reduce hospital stay in 10.68 days and the time of parenteral nutrition in 6 days. Cesarean delivery and prenatal diagnosis was improved all the previous factors. Primary closure however was associated with higher rates of postsurgical complications (46.2% vs. 18.2%). The 5 patients who died was during the immediate postoperative period, all before developing the fetal diagnostic program. CONCLUSIONS: Prenatal diagnosis and preterm delivery by elective cesarean reduces the complications of gastroschisis. Interdisciplinary coordination is essential to improve the prognosis of these patients.

[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.

[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.

[Relationship between the radiological length of the opaque enema and the aganglionic length of the segment in Hirschsprung's disease]. / Relación entre la longitud radiológica del enema opaco y la longitud agangliónica de la pieza en la enfermedad de Hirschsprung.

INTRODUCTION: Barium enema was the first method used for the diagnosis of Hirschsprung's disease, with the appearance of anorectal manometry and its combination with rectal suction biopsy, barium enema has lost value as a diagnosis method but it has also gain importance to decide the surgical technique that will be used for the correction of the disease. AIM: To determine the correlation between the length of the affected segment showed by barium enema valued and the length of the removed piece. MATERIALS AND METHODS: We have studied all Hirschsprung disease's cases diagnosed and treated in our center since 1998, 127 patients underwent Soave-Boley's technique and 51 De La Torre's descent technique. Routinely preoperative barium enema was performed in all cases and determine the location of the transition zone and compared with the aganglionic segment's length specified by pathologist. RESULTS: At 90% of cases the transition zone could be seen at barium enema, it's most common location was rectosigmoid. After statistically analysis the length measured at radiology tests and the length of the anatomic piece showed a low correlation (kappa index 0.0159), being highest values at rectosigmoid transition and very low values at long affected segments. CONCLUSIONS: Barium enema in Hirschsprung's disease is valuable to decide the best surgical technique in each singular case but not to determine the exact length of affected segments. In case of a aganglionic long-segment suspicion, biopsies may be necessary to determinate preoperative length of affected segments.

[Results of living donor kidney transplant in pediatric recipients, with graft obtained by laparoscopy]. / Resultados del trasplante renal de donante vivo en receptores pediátricos, con injertos obtenidos por laparoscopia.

OBJECTIVE: Analyze the results of the living related donor kidney transplant (LRDKT) in our center. PATIENTS AND METHODS: Between December 2005 and 2008, 34 kidney transplants in pediatric recipients were performed in our center, 7 (20.58%) of which were LRDKT with grafts obtained via laparoscopy. The donor was the mother in 4 cases and the father in 3. Mean age of the donors was 43 years (38-48) and of the recipients 12.5 years (9-17). Four were a first transplant (one of them planned) and three retransplantations. RESULTS: Cold ischemia time was < 2 hours in every case while warm ischemia did not show significant differences with the cadaveric donor transplant. None had initial graft dysfunction. No vascular complications occurred, but there was a urinary fistula secondary to ureteral necrosis, resolved with a new reimplantation. Survival of the patient and graft is 100%, superior to that of the cadaveric grafts within the same period. Current mean plasma creatinine is 0.8 mg/dl (0.7-1.39) and mean creatinine clearance is 80 cc/min/1.73 m2 (75-90). No donor had surgical complication and all maintain good kidney function. CONCLUSIONS: The short term results of the LRDKT with grafts obtained by laparoscopy in our center are similar to those described by groups with large experience, which makes it possible to continue offering it with guarantee.

[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.

[Incidence of metachronic contralateral inguinal hernia]. / Incidencia de la hernia inguinal contralateral metácrona.

INTRODUCTION: There are many observational studies about the incidence of metachronous contralateral inguinal hernia (MIH). Metaanalysis allows to resume the results of individual studies in one with a higher level of evidence. AIM: to know the incidence of MIH in order to decide the contralateral exploration. MATERIAL AND METHODS: We do an extensive bibliographic review in Medline, Embase and Cochrane Central. Data analysis is done with RevMan 4.2. RESULTS: 632 abstracts were screened by only one revisor who selected 27 studies: 17802 inguinal hernias and 1209 MIH. Follow up is between 6 months and 10 years. Due to high heterogeneity, a randomized effect analysis (Der Simonian and Lard) is done. The incidence of MIH is 6,96% (6,07-7,85); 14 hernia must be operated to avoid one MIH (NNT). If the original side of the hernia is left, the probability of MIH is 1.81 times higher (NNT =10). The secondary analysis indicates that there is no important publication bias. CONCLUSIONS: Rutinary bilateral inguinal exploration is not justified. Only in high anaesthetic risk patients, bilateral exploration should be suggested.

[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.

[Intestinal neuronal dysplasia: association with digestive malformations]. / Displasia neuronal intestinal asociada a otras malfomaciones congénitas.

Isolated Intestinal neuronal dysplasia is a disease characterized by symptoms of diarrhea or intestinal obstruction along with histopathologic diagnosis based on hyperplasia of submucous plexus with giant ganglia containing more than seven nerve cells, increased acetylcholinesterase activity in the lamina propria and around the submucosal blood vessels and heterotopic ganglia in the lamina propria. The aims of this study have been to determine the incidence of the congenital malformations associated with the isolated intestinal dysplasia type B (not associated to another anomaly of the enteric nervous system) and to correlate them with the severity of the symptoms and their possible familial involvement. We have retrospectively reviewed all the patients diagnosed with IND type B nonassociated to Hirschsprung's disease in our hospital from 1981 to 2002. Our serie consists of 44 cases, 1 for every 7500 newborn. 35% of the patients presented associated congenital anomalies, with digestive malformations being the most commonly found (20% of the total). 40% of the patients studied had previous history of constipation in the family. Onset of symptoms was detected in 75% of children during newborn period.

[Meckel's diverticulum as a cause of massive rectal bleeding]. / Divertículo de Meckel como causa de rectorragia masiva.

A fit three months old male baby was admitted in our hospital with a history of massive painless fresh rectal bleeding. Blood transfusion, hemodynamic stabilization and emergency laparotomy were necessary because of the high suspect of Meckel's diverticulum as the cause of bleeding. It is an uncommon case due to the low age of the patient and the severity of clinical presentation.

[Congenital sternal cleft]. / Hendidura esternal congenita.

INTRODUCTION: Congenital sternal cleft is a rare malformation. Little more than one hundred cases have been published, and rarely more than two cases are published in a single publication. We present five new cases that show the entire spectrum of defects. METHODS: They were 3 males and 2 females. Age range between 1 day and 5 years of age. There were 2 supraxiphoid clefts (SC), 2 gladiolar defects and 1 xiphoid cleft. We evaluate the demographics, associated malformations, mode of treatments and results. RESULTS: The SC were not associated to congenital herat malformations, but with a many other malformative spectrum (anterior cervical web, hemangiomatosis, CNS malformations, coloboma and pectus excavatum). The two females had SC and were operated on the 24th and 30th days of age. The first one developed a superior vena cava síndrome for a few days after surgery and a moderate pectus excavatum that did not required surgery. A midline cervical web was also surgically repaired at 10 years of age. The second patient with SC was treated with interferon alfa-2a and local infiltration of corticosteroid for treatment of hemangiomatosis. Both patients had excellent final results (follow up 11 and 5 years respectively). Gladiolar clefts were observed durign surgical repair of congenital Herat malformations (ventricular septal defect and partial anomalous of pulmonary venous draninage). They were repaired by direct closure of the defect. The patient with the xiphoid cleft had associaed a Cantrell pentalogy. He died 4 days after operation because of extreme prematurity and pulmonary vascular hypertenion. CONCLUSIONS: 1) We have observed five new cases of sternal cleft. 2) Each anatomical variety had their own mode of presentation, a different malformative spectrum and clinical evolution that are based on a distinct embryonic origin. 3) Excluding the xiphoid clefts, the long term surviving is 100%, but they are associated with a great variety of other congenital defects.