[Primary cardiac tumours in infancy]. / Tumores cardíacos primarios en la infancia.

INTRODUCTION: Primary cardiac tumours are very rare in the paediatric age, their incidence varies from 0.0017 % to 0.28 %. More than 90 % are benign in nature. The most common variety is the rhabdomyoma, present in over 60 % of cases with tuberous sclerosis. MATERIAL AND METHODS: We performed a retrospective analysis of medical records with a diagnosis of primary cardiac tumor between March 1977 and March 2007, finding a total of 27 patients. RESULTS: The age of initial diagnosis is more prevalent in the neonatal period, beginning with the discovery of a heart murmur (11 cases). There was no difference in gender distribution. In 14 patients were found cardiomegaly on chest radiograph. According to the echocardiography characteristics there were diagnosed 20 rhabdomyomas, 2 fibromas, 2 pericardial teratomas and 3 non classifiable tumours. Most were located in the left ventricle. Echocardiography, cardiac catheterization was also performed in 3 cases and angioresonance in 5 cases. During their evolution, episodes of arrhythmias were observed in 11 patients, 5 patients required some sort of surgical procedure, which confirmed the histopathology diagnosis. In 3 patients the initial cause of death was cardiological. The 75 % of cases with rhabdomyomas presented or developed tuberous sclerosis. In most of the rhabdomyomas (13 cases), there was a spontaneous regression. CONCLUSIONS: Firstly, there is shown to be a low prevalence of this disorder in children. Rhabdomyoma is the most common primary cardiac tumour in our study and it was associated in 75 % of cases with tuberous sclerosis. The diagnosis is more common in the early neonatal period after auscultation of a cardiac murmur and echocardiography, the diagnostic technique of choice, other imaging techniques, such as angioMRI not being of much for diagnosis in children. The emergence of foetal echocardiography allows early detection. The course is benign in most tumours, rhabdomyomas tending to regress spontaneously. It must be monitored as the occurrence of arrhythmias during its evolution will require medical treatment. Surgery is needed in cases with severe symptoms, due to obstruction in the ventricular output tracts. One option is the surgical cardiac transplant in non-resectable life-threatening tumours.

Consulta de preanestesia no presencial en niños: experiencias y recomendaciones / Telemedicine pre-anesthesia evaluation in children: Experiences and recommendations

Desde hace años se realizan consultas y seguimiento de pacientes de forma no presencial. Durante la pandemia de COVID-19 diferentes sociedades han recomendado potenciar las consultas telemáticas. La consulta preanestésica no presencial es un acto médico que debe incluir los objetivos básicos de evaluación, preparación, información y obtención del consentimiento. Se debe disponer de medios y tiempo para realizarla. Al comienzo de la consulta debe identificarse el médico y el o los progenitores. La consulta preoperatoria no presencial es especialmente útil en niños ASA I y II que van a someterse a cirugías de bajo riesgo, a una reintervención o a procedimientos diagnósticos. Aquellos pacientes que requieran una exploración física, más allá de la posible de manera telemática, deberán citarse en la consulta presencial. El personal de enfermería puede participar de forma activa en este proceso siempre y cuando el anestesiólogo supervise todo el proceso, tome todas las decisiones sobre el procedimiento y sea el responsable de la información que se da a los padres y al niño, aclarando personalmente cualquier duda. El anestesiólogo debe informar del procedimiento, sus riesgos, incluidos los personalizados, y alternativas. Se registrará en la historia clínica que se ha informado, cuándo y a quién. EL anestesiólogo firmará el Consentimiento Informado haciendo figurar la fecha que da la información y los padres deberán firmarlo antes de la intervención.(AU)

Medical consult and follow-up of patients have been carried out remotely for years. During the COVID-19 pandemic, different societies have recommended promoting online consultations. The remote pre-anesthetic consultation is a medical act that must include the basic objectives of evaluation, preparation, information and obtaining consent. You must have the resources and time to do it. At the beginning of the consultation, the doctor and the parent(s) must be identified. Non-face-to-face preoperative consultation is especially useful in ASA I and II children evaluated for low-risk surgeries, reintervention, or diagnostic procedures. Those patients who require a physical examination, beyond that possible electronically, should make an appointment in the face-to-face consultation. The nursing staff can actively participate in this process as long as the anesthesiologist supervises the entire process, makes all decisions about the procedure and is responsible for the information given to the parents and the child, personally clarifying any doubts. The anesthesiologist must inform about the procedure, its risks, including personalized ones, and alternatives. It will be recorded in the medical history the information given, when and to whom. The anesthesiologist will sign the Informed Consent stating the date that the information is given, and the parents must sign it before the intervention.(AU)

Pseudoaneurisma ventricular izquierdo durante valvuloplastia aórtica neonatal / Left ventricular pseudoaneurysm during neonatal aortic valvuloplasty

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[Immature ovarian tumour and dilated myocardiopathy]. / Teratoma inmaduro de ovario y miocardiopatía dilatada.

Asymptomatic 2 month-old infant referred for evaluation of a hard abdominal mass on the left side. The ultrasound examination showed a solid-cystic tumour above the left kidney. The alpha-fetoprotein level was 2000ng/ml. The meta-iodobenzylguanidine (123-I-MIBG) showed no tumour uptake. At 48h, she showed signs of hypovolemic shock. The chest X-ray showed cardiomegaly with a cardiothoracic ratio of 0.7 and pulmonary congestion. The echocardiogram showed a dilated left ventricle with ejection fraction 35-40%. Anaemia, hypertension, hypervolemia and hyper-secretion of catecholamines were ruled out. The virology and metabolic screens were normal. The highly vascularised retroperitoneal tumour was resected without incident and confirmed the diagnosis of an immature Norris grade 2 teratoma grade. At 3 months the outcome was satisfactory. Teratomas are rare tumours in childhood that generally have a benign course. Dilated cardiomyopathy (DCM) secondary to, chromaffin cell tumours (phaeochromocytoma, neuroblastoma, ganglioneuroblastoma), leukaemia infiltrates, and treatment with anthracyclines have been described, but there is no case reported in the literature regarding a teratoma with dilated cardiomyopathy. Various cytokines, such as INF-α, IL-1, IL-6 may be secreted by tumour, promoting fibroblast activity in the heart and inducing apoptosis and myocardial fibrosis. Thus, in the case presented resection of the tumour mass responsible for this production, enables the heart to return to normal.

Afectación renal en las cardiopatías congénitas / Renal involvement in congenital heart disease

Las cardiopatías congénitas (CC) son las anomalías congénitas estructurales más frecuentes, y pueden asociar problemas renales congénitos de forma no desdeñable. Aunque la afectación cardiaca secundaria a problemas renales está bien determinada, los efectos que producen las CC en sus distintas variantes sintomáticas sobre los riñones son bastante desconocidos. La mejora de los medios técnicos y humanos desde los comienzos en esta área hasta nuestros días ha permitido el aumento de la supervivencia y la calidad de vida a medio-largo plazo de estos pacientes. Esto ha diversificado la morbilidad asociada a su evolución y tratamiento, y es menor el daño derivado de la evolución natural de las CC. Existe una mayor concienciación sobre la profilaxis y el tratamiento precoz de los problemas derivados, fundamentalmente, de la cirugía con circulación extracorpórea, las cirugías paliativas y el trasplante cardiaco. Asimismo, se tiene un mayor conocimiento y se toman más precauciones en la administración de los múltiples fármacos con efectos adversos renales que pueden usarse en los pacientes con CC

Congenital heart disease (CHD) are the most common structural birth defects, it could be often associated with congenital kidney problems. While it is well known cardiac involvement secondary to kidney problems, are largely unknown effects produced by the CHD in its different symptomatic variants on the kidneys. The technical and human improvement from the beginning in this area to nowadays, have allowed increased survival and quality of life in the medium-long term follow-up of these patients. It has diversified the morbidity associated with the development and treatment of itself, with much less damage resulting from the natural evolution of the disease. There is a growing awareness in the prophylaxis and early treatment of problems arising mainly from the surgery under cardiopulmonary bypass, the palliative surgeries and heart transplant. Likewise, there is also greater awareness and caution in the use of multiple drugs with adverse renal effects that can be used in patients with CHD

Anillo vascular completo: diagnóstico prenatal de doble arco aórtico / Complete vascular ring: Prenatal diagnosis of double aortic arch

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Miocarditis y síndrome coronario agudo en la infancia / Myocarditis and acute coronary syndrome in childhood

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Edema escrotal idiopático / Idiopathic scrotal edema

El edema escrotal agudo idiopático es una patología autolimitada que afecta fundamentalmente a niños prepúberes, caracterizado por la presencia de edema y eritema, de aparición súbita en uno o ambos hemiescrotos, con aspecto normal de ambos testes y epidídimos. Su etiología es desconocida y, sin antecedentes traumáticos, el diagnóstico es esencialmente clínico, en la mayoría de las veces por exclusión, aunque la realización de una eco-Doppler escrotal es esencial para el diagnóstico, ya que permite determinar el engrosamiento de la piel y las cubiertas subcutáneas escrotales y la ausencia de alteraciones testiculares. Es muy importante la identificación correcta de esta entidad dentro del diagnóstico diferencial del escroto agudo para evitar intervenciones quirúrgicas innecesarias. Presentamos 3 casos y 1 imagen para ayudar a reconocer esta patología, así como una revisión de la escasa literatura publicada hasta la fecha sobre este tema (AU)

Acute idiopathic scrotal edema is a self-limited disease that primarily affects prepubertal children, characterized by erythema and edema, sudden onset of one or both scrotal bags with both normal epididymis and testis. Of unknown etiology and with no related previous trauma, the diagnosis is clinical, in most cases by exclusion, although the realization of scrotal Doppler ultrasound should be essential to diagnose, in order to prove the thickening of the skin and subcutaneous tissues and normal testis. It is very important to correctly identify this entity in the differential diagnosis of acute scrotum, to avoid unnecessary surgery. We present three cases and a picture to help recognize this disease, and a review of the limited literature published to date (AU)

Genetic and environmental factors affecting menarcheal age in Spanish women.

Genetic and environmental contributions to menarcheal age were studied in 267 Spanish girls and women born between 1948 and 1981, and their mothers born between 1922 and 1959 (n = 200). Recalled age at menarche, as well as family environment characteristics (family size, birth order, father's occupation and father's educational level) were obtained from each woman. Mean age at menarche in mothers (13.45 years; SD = 1.51) was significantly (p < 0.01) greater than in daughters (13.03 +/- 1.28 years). Year of birth and family size accounted for a significant amount of the variation in age at menarche in both mothers and daughters, whereas occupation of the father only appeared related to menarcheal age in the mothers subsample. Birth order and father's educational level did not contribute significantly to the age at menarche. The mother-daughter and sister-sister correlations (maximum likelihood estimates) for age at menarche were 0.30 and 0.35, respectively (both significant at p < 0.001). This supports the genetic and environmental contribution to the age at menarche, even though the influence of environmental variables may change over time.