Evolución y tendencias de un programa de resonancia magnética cardiaca pediátrica en un hospital terciario durante 14 años / Evolution and trends in a pediatric cardiac magnetic resonance imaging program in a tertiary hospital over a 14-year period

OBJETIVOS: 1) Revisar la actividad del programa de resonancia magnética cardiaca (RMC) de nuestro hospital desde su inicio hasta la actualidad; 2) evaluar la evolución del número de pacientes, estudios bajo anestesia, estudios con contraste (angiografía y realce tardío) y patologías representativas, y 3) estimar la tendencia de los mismos parámetros evaluados. MATERIAL Y MÉTODOS: El programa de RMC pediátrica de nuestro hospital comenzó el 14 de febrero de 2005. Revisamos los estudios hasta el 31 de diciembre de 2018. Los casos son incluidos en una tabla de cálculo que incluye sexo, fecha de nacimiento, fecha de exploración, clínica, diagnóstico radiológico, secuencias realizadas y anestesia. Obtenemos datos por años de la edad de los pacientes, la realización de estudios bajo anestesia, realización de angiografía por resonancia magnética con contraste, estudios de realce tardío y estudios posquirúrgicos. También valoramos la evolución de un grupo de patologías representativas: coartación aórtica, tetralogía de Fallot, D-transposición de las grandes arterias, correcciones univentriculares, síndrome del corazón izquierdo hipoplásico, retornos venosos pulmonares anómalos y miocardiopatías. Realizamos gráficos de barras, evolución de las medias y curvas logarítmicas de tendencia. RESULTADOS: El número total de casos fue 2.606. Se registró un incremento gradual del número de casos. La media de edad de los pacientes fue de 12,5 años y también se incrementó a lo largo del periodo; el 42% de los casos se realizaron con anestesia. En el 57,6% de los casos se realizó angio-RM con contraste, y en el 42,13%, estudio de realce tardío. La coartación aórtica ha representado la patología más frecuente (16,39%), aunque su número ha descendido paulatinamente a lo largo del periodo, y también lo ha hecho el corazón izquierdo hipoplásico. La patología del miocardio (7,25% de casos) ha aumentado paulatinamente, hasta representar el 9,35% en 2018. CONCLUSIÓN: A lo largo de estos 14 años, la patología estudiada, el tipo de pacientes y la técnica empleada han ido variando, con un aumento del número de pacientes y de su edad, una disminución de los estudios de angio-RM, y cambios en la prevalencia de los distintos grupos de patologías

OBJECTIVES: 1. To review the activity in our hospital's pediatric cardiac magnetic resonance imaging (cMRI) program from its inception to the present.2. To evaluate changes in the number of patients, in the number of studies done under anesthesia, in the number of studies done with contrast material (magnetic resonance angiography (MRA) and delayed enhancement), and in representative diseases studied.3. To estimate trends in the parameters evaluated in objective 2. MATERIAL AND METHODS: The pediatric cMRI program at our hospital started on February 14, 2005. We assessed cMRI studies done between the inception of the program and December 31, 2018. The cases were entered in a calculation table that included sex, date of birth, date of examination, clinical presentation, radiologic diagnosis, sequences done, and anesthesia. For each year, we obtained data about patients' age, studies done under anesthesia, contrast-enhanced MRA, delayed enhancement studies, and postoperative studies. We also evaluated the evolution of the number of patients studied for a group of representative diseases (coarctation of the aorta; tetralogy of Fallot; dextro-transposition of the great arteries; corrections of univentricular heart; hypoplastic left heart syndrome; anomalous pulmonary venous return; and cardiomyopathy). We analyzed these data with bar graphs, evolutions of means, and logarithmic trend curves. RESULTS: A total of 2606 cases were included. The number of cases per year increased gradually. The mean age of all patients was 12.5 years, and the age of the patients studied also increased during the 14-year period. Anesthesia was used in 42%. Contrast-enhanced MRA was done in 57.6% and delayed enhancement in 42.13%. The most common condition was aortic coarctation (16.39%), although the frequency of aortic coarctation and hypoplastic left heart syndrome decreased slightly during the period. By contrast, the frequency of cardiomyopathy (7.25% of cases) increased slightly, to the point where it represented 9.35% in 2018. CONCLUSION: During the 14-year period in which pediatric cMRI has been done at our hospital, the conditions studied, the type of patients, and the techniques used has varied; the number of patients and patients' age has increased, where as the frequency of MRA studies has decreased. The prevalence of the different conditions studied has also changed

Evolution and trends in a pediatric cardiac magnetic resonance imaging program in a tertiary hospital over a 14-year period. / Evolución y tendencias de un programa de resonancia magnética cardiaca pediátrica en un hospital terciario durante 14 años.

OBJECTIVES: 1. To review the activity in our hospital's pediatric cardiac magnetic resonance imaging (cMRI) program from its inception to the present. 2. To evaluate changes in the number of patients, in the number of studies done under anesthesia, in the number of studies done with contrast material (magnetic resonance angiography (MRA) and delayed enhancement), and in representative diseases studied. 3. To estimate trends in the parameters evaluated in objective 2. MATERIAL AND METHODS: The pediatric cMRI program at our hospital started on February 14, 2005. We assessed cMRI studies done between the inception of the program and December 31, 2018. The cases were entered in a calculation table that included sex, date of birth, date of examination, clinical presentation, radiologic diagnosis, sequences done, and anesthesia. For each year, we obtained data about patients' age, studies done under anesthesia, contrast-enhanced MRA, delayed enhancement studies, and postoperative studies. We also evaluated the evolution of the number of patients studied for a group of representative diseases (coarctation of the aorta; tetralogy of Fallot; dextro-transposition of the great arteries; corrections of univentricular heart; hypoplastic left heart syndrome; anomalous pulmonary venous return; and cardiomyopathy). We analyzed these data with bar graphs, evolutions of means, and logarithmic trend curves. RESULTS: A total of 2606 cases were included. The number of cases per year increased gradually. The mean age of all patients was 12.5 years, and the age of the patients studied also increased during the 14-year period. Anesthesia was used in 42%. Contrast-enhanced MRA was done in 57.6% and delayed enhancement in 42.13%. The most common condition was aortic coarctation (16.39%), although the frequency of aortic coarctation and hypoplastic left heart syndrome decreased slightly during the period. By contrast, the frequency of cardiomyopathy (7.25% of cases) increased slightly, to the point where it represented 9.35% in 2018. CONCLUSION: During the 14-year period in which pediatric cMRI has been done at our hospital, the conditions studied, the type of patients, and the techniques used has varied; the number of patients and patients' age has increased, where as the frequency of MRA studies has decreased. The prevalence of the different conditions studied has also changed.

Radioprotección y uso de contrastes en pediatría: qué, cómo y cuándo / Radioprotection and contrast agent use in pediatrics: what, how, and when

La reducción de la radiación ionizante (RI) en el niño es fundamental por distintas causas. Ante una exposición a la misma dosis de radiación, el riesgo de desarrollo de un tumor es mayor en la infancia. Varias estrategias pueden emplearse para reducir la RI. La fundamental es evitar la realización de pruebas innecesarias o no indicadas, elegir una prueba alternativa que no utilice RI, y/o tomar una serie de medidas que reduzcan al máximo la dosis recibida por el paciente, como evitar repetir pruebas, emplear las proyecciones correctas, usar protector, ajustar el protocolo (mAs, Kv o pitch) al volumen del paciente, etc… En ecografía, se pueden utilizar contrastes sonográficos de uso endocavitario, aunque también se está extendiendo para uso endovascular. En fluoroscopia se deben utilizar de baja osmolaridad, como en TC donde ajustaremos la dosis y la velocidad de inyección al peso del paciente y calibre de la vía periférica respectivamente. En RM tan sólo están aprobados para utilización pediátrica tres tipos de contrastes. En ciertas situaciones clínicas es necesaria la utilización de dosis dobles o de contrastes organoespecíficos, indicaciones que aun no estando probadas en términos de seguridad farmacológica pueden emplearse como uso compasivo (AU)

It is essential to minimize exposure to ionizing radiation in children for various reasons. The risk of developing a tumor from exposure to a given dose of radiation is greater in childhood. Various strategies can be used to reduce exposure to ionizing radiation. It is fundamental to avoid unnecessary tests and tests that are not indicated, to choose an alternative test that does not use ionizing radiation, and/or to take a series of measures that minimize the dose of radiation that the patient receives, such as avoiding having to repeat tests, using the appropriate projections, using shields, adjusting the protocol (mAs, Kv, or pitch) to the patient's body volume, etc… When contrast agents are necessary, intracavitary ultrasound agents can be used, although the use of ultrasound agents is also being extended to include intravenous administration. In fluoroscopy, contrast agents with low osmolarity must be used, as in CT where we must adjust the dose and speed of injection to the patient's weight and to the caliber of the peripheral line, respectively. In MRI, only three types of contrast agents have been approved for pediatric use. It is sometimes necessary to use double doses or organ-specific contrast agents in certain clinical situations; the safety of contrast agents for these indications has not been proven, so they must be used off label (AU)

Cuantificación de la circulación pulmonar y sistémica: Qp/Qs. Qué es, cómo se calcula y para qué sirve. Lo que el radiólogo debe saber / What radiologists need to know about the pulmonary-systemic flow ratio (Qp/Qs): what it is, how to calculate it, and what it is for

La resonancia magnética cardiaca aporta abundante información morfológica y funcional al estudio de las cardiopatías congénitas. Entre los datos funcionales se encuentran el gasto pulmonar y el gasto sistémico; el cociente entre ambos es el cociente Qp/Qs. Después del nacimiento, en condiciones normales el gasto pulmonar y el sistémico son prácticamente iguales, y el cociente Qp/Qs = 1. En los pacientes con cortocircuitos entre la circulación sistémica y la pulmonar este cociente se altera. Dependiendo de la localización del cortocircuito (intra o extracardiaco), y de las alteraciones estructurales o posquirúrgicas asociadas, la interpretación de los hallazgos es diferente. Revisamos el concepto de Qp/Qs, los métodos para calcularlo con especial énfasis en la RM, y el significado de los resultados obtenidos, haciendo hincapié en la relevancia de estos datos dependiendo de la patología de base y los procedimientos terapéuticos que se hayan realizado al paciente (AU)

Cardiac magnetic resonance imaging (cMRI) provides abundant morphological and functional information in the study of congenital heart disease. The functional information includes pulmonary output and systemic output; the ratio between these two (Qp/Qs) is the shunt fraction. After birth, in normal conditions the pulmonary output is practically identical to the systemic output, so Qp/Qs = 1. In patients with «shunts» between the systemic and pulmonary circulations, the ratio changes, and the interpretation of these findings varies in function of the location of the shunt (intracardiac or extracardiac) and of the associated structural or postsurgical changes. We review the concept of Qp/Qs; the methods to calculate it, with special emphasis on cMRI; and the meaning of the results obtained. We place special emphasis on the relevance of these findings depending on the underlying disease and the treatment the patient has undergone (AU)

Valoración radiológica de los tumores congénitos / Radiological evaluation of congenital tumors

Los tumores congénitos son aquellos tumores diagnosticados durante el embarazo y los tres primeros meses de vida. Constituyen un grupo heterogéneo de neoplasias con unas características biológicas y epidemiológicas especiales que los diferencian del resto de los tumores pediátricos y de la edad adulta. Su detección prenatal ha aumentado en las dos últimas décadas debido a la generalización del cribado ecográfico prenatal. El desarrollo de las técnicas de imagen, especialmente la resonancia magnética (RM) fetal, ha permitido mejorar el diagnóstico, el seguimiento y el manejo clínico y terapéutico perinatal de estos tumores. Presentamos una revisión basada en imágenes de los tumores congénitos más frecuentes, describiendo los tipos histológicos, localizaciones y sus características en las distintas técnicas de imagen empleadas (AU)

In this article, we consider tumors that are diagnosed during pregnancy or in the first three months of life. This is a heterogeneous group of neoplasms with special biological and epidemiological characteristics that differentiate them from tumors arising in children or adults. In the last two decades, the prenatal detection of congenital tumors has increased due to the generalized use of prenatal sonographic screening. Advances in imaging techniques, especially in fetal magnetic resonance imaging, have enabled improvements in the diagnosis, follow-up, clinical management, and perinatal treatment of these tumors. This image-based review of the most common congenital tumors describes their histologic types, locations, and characteristics on the different imaging techniques used (AU)

Calidad de la coronariografía 3D por resonancia magnética en los pacientes con D-transposición de las grandes arterias intervenidos con switch arterial de Jatene / Quality of 3D magnetic resonance imaging of coronary arteries in patients with D-transposition of the great arteries after the Jatene switch procedure

Objetivos. Valorar la calidad de imagen de la secuencia 3D de todo el corazón (3D balanced fast field echo, whole heart [WH3D]) para estudiar la anastomosis coronaria y las estenosis coronarias en los pacientes con D-transposición de las grandes arterias intervenidos con switch arterial de Jatene. Material y métodos. Rescatamos de la base de datos de RM cardiaca pediátrica 100 exploraciones en 83 pacientes intervenidos con la técnica de Jatene y secuencia WH3D. Cumplían criterios de inclusión en el estudio 84 exploraciones. Valoramos las estenosis coronarias mediante WH3D y su correlación con la TC coronaria o la angiografía por catéter. Estudiamos retrospectivamente la calidad de la imagen de las arterias coronarias proximales con una escala de 4 puntos y la correlación con la edad, frecuencia cardiaca y tamaño cardiaco. Resultados. Cuatro de los 84 estudios (4,8%) mostraron calidad «insuficiente» para el diagnóstico, 7 (8,3%) «discreta», 23 (27,4%) «buena» y 50 (59,5%) «excelente». La frecuencia cardiaca y la calidad de la imagen coronaria se relacionaron estadísticamente. Con la RM detectamos estenosis en el origen coronario en 9 exploraciones (10,7%). Conclusión. La calidad de imagen con la secuencia WH3D fue diagnóstica en la mayoría de los pacientes intervenidos con la técnica de Jatene, mejor en pacientes con menor frecuencia cardiaca. En el 10,7% se diagnosticó una estenosis del origen coronario que obligó a nuevas exploraciones (AU)

Objectives. To evaluate the quality of images obtained with 3D balanced fast-field echo whole heart (WH3D) MRI sequences for assessing the coronary anastomosis and coronary stenosis in patients with D-transposition of the great arteries who have undergone the Jatene switch procedure. Material and methods. We retrieved 100 WH3D studies done in 83 patients who had undergone the Jatene switch procedure from our pediatric cardiac MRI database; 84 of these studies fulfilled the criteria for inclusion in the study. We evaluated coronary stenoses on WH3D MR images and their correlation with coronary CT or angiography images. We retrospectively studied the quality of the images of the proximal coronary arteries using a four-point scale and correlating the findings with age, heart rate, and heart size. Results. Of the 84 studies, 4 (4.8%) were of a quality considered «insufficient for diagnosis», 7 (8.3%) were considered «fair», 23 (27.4%) «good», and 50 (59.5%) «excellent». The quality of the image of the coronary arteries was significantly correlated with heart rate. MRI detected stenosis in the origin of the coronary arteries in 9 (10.7%) studies. Conclusion. Images obtained with the WH3D MRI sequence in patients who had undergone the Jatene procedure were of diagnostic quality in most cases and were better in patients with lower heart rates. In 10.7%, stenosis in the origin of the coronary arteries that required new studies was detected (AU)

[Radiological evaluation of congenital tumors]. / Radiografía del abdomen en Urgencias. ¿Una exploración para el recuerdo?

In this article, we consider tumors that are diagnosed during pregnancy or in the first three months of life. This is a heterogeneous group of neoplasms with special biological and epidemiological characteristics that differentiate them from tumors arising in children or adults. In the last two decades, the prenatal detection of congenital tumors has increased due to the generalized use of prenatal sonographic screening. Advances in imaging techniques, especially in fetal magnetic resonance imaging, have enabled improvements in the diagnosis, follow-up, clinical management, and perinatal treatment of these tumors. This image-based review of the most common congenital tumors describes their histologic types, locations, and characteristics on the different imaging techniques used.

What radiologists need to know about the pulmonary-systemic flow ratio (Qp/Qs): what it is, how to calculate it, and what it is for.

Cardiac magnetic resonance imaging (cMRI) provides abundant morphological and functional information in the study of congenital heart disease. The functional information includes pulmonary output and systemic output; the ratio between these two (Qp/Qs) is the shunt fraction. After birth, in normal conditions the pulmonary output is practically identical to the systemic output, so Qp/Qs = 1. In patients with « shunts ¼ between the systemic and pulmonary circulations, the ratio changes, and the interpretation of these findings varies in function of the location of the shunt (intracardiac or extracardiac) and of the associated structural or postsurgical changes. We review the concept of Qp/Qs; the methods to calculate it, with special emphasis on cMRI; and the meaning of the results obtained. We place special emphasis on the relevance of these findings depending on the underlying disease and the treatment the patient has undergone.

Vena innominada subaórtica en resonancia magnética / Subaortic innominate vein on magnetic resonance imaging

No disponible

[Quality of 3D magnetic resonance imaging of coronary arteries in patients with D-transposition of the great arteries after the Jatene switch procedure]. / Calidad de la coronariografía 3D por resonancia magnética en los pacientes con D-transposición de las grandes arterias intervenidos con switch arterial de Jatene.

OBJECTIVES: To evaluate the quality of images obtained with 3D balanced fast-field echo whole heart (WH3D) MRI sequences for assessing the coronary anastomosis and coronary stenosis in patients with D-transposition of the great arteries who have undergone the Jatene switch procedure. MATERIAL AND METHODS: We retrieved 100 WH3D studies done in 83 patients who had undergone the Jatene switch procedure from our pediatric cardiac MRI database; 84 of these studies fulfilled the criteria for inclusion in the study. We evaluated coronary stenoses on WH3D MR images and their correlation with coronary CT or angiography images. We retrospectively studied the quality of the images of the proximal coronary arteries using a four-point scale and correlating the findings with age, heart rate, and heart size. RESULTS: Of the 84 studies, 4 (4.8%) were of a quality considered «insufficient for diagnosis¼, 7 (8.3%) were considered «fair¼, 23 (27.4%) «good¼, and 50 (59.5%) «excellent¼. The quality of the image of the coronary arteries was significantly correlated with heart rate. MRI detected stenosis in the origin of the coronary arteries in 9 (10.7%) studies. CONCLUSION: Images obtained with the WH3D MRI sequence in patients who had undergone the Jatene procedure were of diagnostic quality in most cases and were better in patients with lower heart rates. In 10.7%, stenosis in the origin of the coronary arteries that required new studies was detected.

Solución del caso 17. Tumor neuroectodérmico primitivo de la pared torácica / No disponible

No disponible

Ausencia de realce tardío por resonancia magnética en la no compactación del ventrículo izquierdo en lactantes y niños pequeños / Lack of MR late-enhancement in left ventricular non-compaction in infants and young children

Objetivo: La miocardiopatía no compactada o la no compactación del ventrículo izquierdo (NCVI) es una enfermedad rara y probablemente infradiagnosticada. El diagnóstico es ecográfico, si bien la resonancia magnética (RM) aporta importante información morfológica y funcional. El realce tardío es un hallazgo importante que refleja fibrosis o infarto en las zonas afectadas, y se refiere en adultos y niños mayores de forma generalizada. El objetivo de este trabajo es revisar el realce tardío en esta enfermedad en lactantes y niños pequeños. Material y métodos: En nuestra base de datos de RM cardíaca encontramos 5 pacientes (rango de edad: un mes a 5 años; media de 29,4 meses). Se revisaron los hallazgos morfológicos y funcionales, incluyendo el realce tardío de contraste. Resultados: Todos los pacientes se diagnosticaron previamente por ecocardiografía. Los hallazgos morfológicos y el cociente miocardio no compactado/miocardio compactado fueron compatibles con NCVI. Ninguno de los casos mostró realce tardío poscontraste. Conclusiones: Al contrario que en adultos y niños mayores, ninguno de nuestros pacientes mostró realce tardío. Este hallazgo podría reflejar la naturaleza evolutiva de la fibrosis subendocárdica en estos pacientes (AU)

Objective: Non compaction cardiomyopathy or left ventricular non compaction is a rare disease that is probably underdiagnosed. The diagnosis is reached by echocardiography, although MRI provides additional morphological and functional information. Late MRI enhancement is a hallmark of the disease that reflects fibrosis or infarction of affected areas in adults and older children. We aimed to review the presence of late enhancement in left ventricular non compaction in infants and young children. Material and methods: We found five very young patients (mean age, 29.4 months; range 1 month to 5 years) with left ventricular non compaction in our cardiac MRI database. We reviewed the morphological and functional findings, including late enhancement after the administration of contrast material. Results: All patients had been previously diagnosed by echocardiography. At MRI, the morphological findings and the ratio of non compacted myocardium to compacted myocardium were compatible with left ventricular non compaction. None of the cases showed late enhancement after the administration of contrast material. Conclusions: Unlike in adults and older children, none of the infants and young children we studied had late enhancement. This finding might reflect the natural history of the disease, with subendocardial fibrosis developing over time (AU)

[Lack of MR late-enhancement in left ventricular non-compaction in infants and young children]. / Ausencia de realce tardío por resonancia magnética en la no compactación del ventrículo izquierdo en lactantes y niños pequeños.

OBJECTIVE: Noncompaction cardiomyopathy or left ventricular noncompaction is a rare disease that is probably underdiagnosed. The diagnosis is reached by echocardiography, although MRI provides additional morphological and functional information. Late MRI enhancement is a hallmark of the disease that reflects fibrosis or infarction of affected areas in adults and older children. We aimed to review the presence of late enhancement in left ventricular noncompaction in infants and young children. MATERIAL AND METHODS: We found five very young patients (mean age, 29.4 months; range 1 month to 5 years) with left ventricular noncompaction in our cardiac MRI database. We reviewed the morphological and functional findings, including late enhancement after the administration of contrast material. RESULTS: All patients had been previously diagnosed by echocardiography. At MRI, the morphological findings and the ratio of noncompacted myocardium to compacted myocardium were compatible with left ventricular noncompaction. None of the cases showed late enhancement after the administration of contrast material. CONCLUSIONS: Unlike in adults and older children, none of the infants and young children we studied had late enhancement. This finding might reflect the natural history of the disease, with subendocardial fibrosis developing over time.

[Contrast-enhanced magnetic resonance angiography in congenital heart disease]. / Angiografía con contraste por resonancia magnética en las cardiopatías congénitas.

Contrast-enhanced MR angiography is one of the greatest achievements brought about by advances in body MRI. The noninvasive evaluation of arteries and veins can obviate heart catheterization, the administration of iodinated contrast, and exposure to ionizing radiation in many patients and spare them the risks associated with these factors. These gains are even more important in children with congenital heart disease, who will have to undergo numerous vascular studies in their lifetimes and are more susceptible to the effects of ionizing radiation. Contrast-enhanced MR angiography provides abundant information for diagnosis and postoperative follow-up in these patients, who reach advanced age thanks to advances in medical and surgical treatment and thus receive more and more imaging studies during their lifetimes. In this review, we analyze the contrast-enhanced MR angiography technique in these patients, the problems and precautions related to the use of gadolinium, the indications for the test, and the relevant imaging findings in patients with congenital heart disease.

Angiografía con contraste por resonancia magnética en las cardiopatías congénitas / Contrast-enhanced magnetic resonance angiography in congenital heart disease

El avance de la resonancia magnética (RM) de cuerpo ha visto en la angiografía con contraste por RM (ACRM) uno de sus mayores logros. La evaluación no invasiva de arterias y venas evita la realización de cateterismo, la administración de contraste yodado y la exposición a radiaciones ionizantes a un gran número de pacientes. Este hecho es, si cabe, de mayor relevancia en los niños con cardiopatías congénitas, al tratarse de pacientes a los que habrá que realizar numerosos estudios vasculares a lo largo de su vida, con los riesgos inherentes del cateterismo, los debidos al contraste yodado, y la mayor susceptibilidad de estos pacientes a los efectos de las radiaciones ionizantes. La ACRM aporta abundante información para el diagnóstico y seguimiento posquirúrgico en este grupo de pacientes que, con los avances terapéuticos médicos y quirúrgicos, hoy en día alcanzan edades avanzadas y reciben, cada vez más, numerosos estudios de imagen a lo largo de su vida. En esta revisión se analiza la técnica de la ACRM en estos pacientes, los problemas y precauciones relativas al uso del gadolinio, las indicaciones y los hallazgos radiológicos a los que hay que prestar especial atención en esta patología (AU)

Contrast-enhanced MR angiography is one of the greatest achievements brought about byadvances in body MRI. The non invasive evaluation of arteries and veins can obviate heartcatheterization, the administration of iodinated contrast, and exposure to ionizingradiation in many patients and spare them the risks associated with these factors. These gains are even more important in children with congenital heart disease, who will have toundergo numerous vascular studies in their life times and are more susceptible to theeffects of ionizing radiation. Contrast-enhanced MR angiography provides abundantinformation for diagnosis and postoperative follow-up in these patients, who reachadvanced age thanks to advances in medical and surgical treatment and thus receive moreand more imaging studies during their life times. In this review, we analyze the contrast-enhanced MR angiography technique in these patients, the problems and precautionsrelated to the use of gadolinium, the indications for the test, and the relevant imagingfindings in patients with congenital heart disease (AU)

[Möbius syndrome and an apparently life-threatening event]. / Síndrome de Möbius y episodio aparentemente letal.

Möbius syndrome is characterized by congenital facial weakness with impairment of ocular abduction. A subgroup of these patients have associated apneas because of involvement of brainstem respiratory centers located slightly lateral to the abducens nuclei. We report a 7-month old infant admitted to the pediatric intensive care unit because of an episode of cyanosis, hypotonia and unresponsiveness. The patient then became respirator dependent afterwards. On examination, facial diplegia, impairment of ocular abduction and hypotonia were evident. Magnetic resonance imaging (MRI) revealed abnormal signal intensity in brainstem tegmentum. At the age of 11 months he was discharged but required a home ventilator. He died 5 months later due to an infection. Möbius syndrome is associated with central respiratory dysfunction. The finding of abnormal signal intensity in brainstem tegmentum on MRI is a possible predictor of apnea in these patients.

Síndrome de Möbius y episodio aparentemente letal / Möbius syndrome and an apparently life-threatening event

El síndrome de Möbius se caracteriza por debilidad facial congénita junto con déficit de la abducción ocular. Un subgrupo de pacientes asocia apneas por afectación de los centros generadores del impulso respiratorio que se localizan junto al núcleo del VI par craneal. Se presenta un lactante que ingresó a los 7meses de vida en la unidad de cuidados intensivos pediátricos tras un episodio de cianosis, hipotonía y ausencia de respuesta a estímulos, y precisó ventilación mecánica continuada a partir de ese momento. La exploración física reveló debilidad de la musculatura facial bilateral, incapacidad para la abducción de ambos ojos e hipotonía. Una resonancia magnética (RM) craneal mostró alteración de la señal de la sustancia gris de mesencéfalo, protuberancia y bulbo. A los 11 meses de vida fue dado de alta con ventilación mecánica domiciliaria, y falleció 5 meses más tarde en el curso de un proceso séptico. El síndrome de Möbius se asocia con disfunción respiratoria central. La presencia en la RM craneal de alteración de la intensidad de la señal en el tegmento del tronco del encéfalo puede ayudar a identificar pacientes con síndrome de Möbius que tienen riesgo de apnea (AU)

Möbius syndrome is characterized by congenital facial weakness with impairment of ocular abduction. A subgroup of these patients have associated apneas because of involvement of brainstem respiratory centers located slightly lateral to the abducens nuclei. We report a 7-month old infant admitted to the pediatric intensive care unit because of an episode of cyanosis, hypotonia and unresponsiveness. The patient then became respirator dependent afterwards. On examination, facial diplegia, impairment of ocular abduction and hypotonia were evident. Magnetic resonance imaging (MRI) revealed abnormal signal intensity in brainstem tegmentum. At the age of 11 months he was discharged but required a home ventilator. He died 5 months later due to an infection (AU)

Torsion of a Meckel's diverticulum: sonographic findings.

There are few reports of the sonographic appearance of Meckel's diverticulum. We present a case of torsion of a Meckel's diverticulum that was suggested by sonography and confirmed pathologically. We discuss the sonographic differential diagnosis, which includes acute appendicitis, enteric duplication cyst and intestinal volvulus.