Validation of the Script Concordance Test as an instrument to assess clinical reasoning of residents in pediatric emergency medicine in Mexico

Background. Clinical reasoning is a crucial skill to be acquired during a residency training program. In pediatric emergency medicine, physicians are challenged by diagnostic, investigative, and treatment uncertainties. The Script Concordance Test (SCT) uses authentic clinical scenarios to compare trainee’s judgement skills with those of experts. The purpose of this study was to evaluate the clinical reasoning using a pediatric emergency medicine SCT and to determine if it would be able to differentiate between different levels of residency training, validating it in Mexico. Methods. A SCT containing 58 questions nested in 14 cases was administered to pediatric and senior residents at one academic institution. Fourteen experienced emergency medicine pediatricians were part of a reference panel to establish the basis for the scoring process. Results. Ninety six residents consented to participate. Thirteen residents were fellows of pediatric intensive care medicine or pediatric emergency medicine, and there were 83 pediatric residents. Overall, there was a significant difference in performance across levels of training. The difference between all levels was significant. Conclusions. This pediatric emergency medicine Script Concordance Test was useful to assess the progression of clinical reasoning during residency training in Mexico.

Factores etiológicos relacionados al pronóstico de la epilepsia en niños con síndrome de Möebius / Etiological factors related to prognosis of epilepsy in children with möebius syndrome

Introducción. El síndrome de Möebius (SM) es una enfermedad congénita, sin etiología determinada, multifactorial, se considera rara y se caracteriza por la afección del VI y VII pares craneales o sus núcleos, es unilateral o bilateral, teniendo como manifestación clínica diplejía facial con limitación de la mirada lateral. Se describe en literatura más reciente la afección de otros pares craneales. Hasta 90% de los casos tienen inteligencia normal. Objetivo: determinar las características de las crisis convulsivas de los pacientes con SM y epilepsia. Material y métodos. Estudio descriptivo, analítico y retrolectivo, en el cual se revisaron los casos registrados con diagnóstico de SM en el Hospital Infantil de México Federico Gómez, en el período de 1994-2004, analizando las diferentes variables y revisando las causas de la epilepsia en estos pacientes. Resultados. De 31 pacientes con SM, se encontraron 8 con epilepsia (25.8%): 2 casos (6.4%) con epilepsia parcial, 4 (12.9%) con epilepsia generalizada, 1 caso (3.22%) con crisis febriles plus y 1 caso (3.22%) con espasmos infantil. En 7 de los 8 casos de epilepsia, el EEG fue anormal, así como la neuro-imagen donde se observaron múltiples anormalidades. Conclusiones. La epilepsia en pacientes con SM depende más de la asociación de éste con otras enfermedades que del síndrome en sí, por lo cual el riesgo de epilepsia en estos pacientes está asociado a la etiología acompañante del SM.

Introduction. Möebius syndrome (MS) is a rare congenital disease of multifactorial etiology; VI and VII cranial nerves or their nucleus are involved in its clinical presentation, with facial diplegia and inability to abduct the eyes beyond the midline; other cranial nerves are also involved. As far as 90% of patients have normal intelligence. Objective: to describe the characteristics of seizure in children with MS and epilepsy. Material and methods. A descriptive retrolective study of children with MS attended in the Hospital Infantil de Mexico Federico Gomez from 1994 to 2004. Clinical characteristics of seizures were determined. Results. Thirty one patients with MS were analyzed; of these, 8 had epilepsy (25.8%), 2 cases (6.4%) partial epilepsy, 4 cases (12.9%) generalized epilepsy; one case (3.22%) with epilepsy plus and 1 case (3.22%) with infantile spasms. In 7 of 8 cases of epilepsy, the EEG was abnormal and there were multiple abnormalities in neuroimaging. Conclusion. Epilepsy in children with MS is probably determined by other associated diseases, it is not the result of MS itself.

Neurocutaneous melanosis in association with the Dandy-Walker complex, complicated by melanoma: report of a case and literature review.

Neurocutaneous melanosis is a rare congenital neurocutaneous syndrome in which benign and malignant melanocytic tumors of the leptomeninges with large or numerous congenital melanocytic nevi develop. The Dandy-Walker malformation occurs as a broad posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilation of the fourth ventricle communicating with the posterior fossa. Association of these entities is very unusual and only 10 previous reports were found in the literature. Our patient had multiple, medium-size to small melanocytic nevi present since birth. At 5 years of age the patient has intracranial pressure secondary to hydrocephalus. A diagnosis of Dandy-Walker malformation and suspected neurocutaneous melanosis was established after a skull computed tomography (CT) scan. Three months later the patient developed a right frontal tumor shown on the CT scan. The histologic finding was nevomelanocytic infiltration with strong pleomorphism. The tumor grew rapidly, producing neurogenic shock and death. The postmortem report indicated malignant melanoma.