[Debut and evolution MRI images of a child with a new genetic variant of vanishing white matter leukodystrophy]. / Imágenes de resonancia magnética de inicio y evolución de un niño con nueva variante genética de leucodistrofia evanescente.

TITLE: Imágenes de resonancia magnética de inicio y evolución de un niño con nueva variante genética de leucodistrofia evanescente.

La derivación lumbo-peritoneal en la edad pediátrica y el papel de la imagen en su valoración / Lumboperitoneal shunting in pediatric patients and the role of imaging in its evaluation

La derivación lumbo-peritoneal permite regular el flujo de líquido cefalorraquídeo estableciendo una conexión entre el saco tecal y la cavidad peritoneal. Entre las indicaciones en la población pediátrica se encuentra principalmente la hipertensión intracraneal idiopática, siendo también útil en el tratamiento de la hidrocefalia postinfecciosa, posthemorrágica y normotensiva, en el seudomeningocele posquirúrgico o ante una fuga de líquido cefalorraquídeo.En este artículo, mediante la revisión de 9 casos de nuestro centro, se pretende mostrar la normalidad del dispositivo en las pruebas de imagen y realizar una breve revisión de las posibles complicaciones asociadas, neurológicas y abdominales.(AU)

Lumboperitoneal shunting makes it possible to regulate the flow of cerebrospinal fluid by establishing a connection between the thecal sac and the peritoneal cavity. The main indication for lumboperitoneal shunting in children is idiopathic intracranial hypertension, but the technique is also useful in the treatment of postinfectious, posthemorrhagic, and normotensive hydrocephalus, as well as in the treatment of postsurgical pseudomeningocele or leakage of cerebrospinal fluid.This article reviews nine cases treated at our center to show the normal imaging findings for lumboperitoneal shunts in children and to provide a succinct review of the possible neurological and abdominal complications associated with this treatment.(AU)

Miofibromatosis infantil. Un caso estudiado con ecografía y resonancia magnética de cuerpo entero / Infantile myofibromatosis. Study of a case using whole body ultrasound and MRI

La miofibromatosis infantil, a pesar de ser considerada una entidad rara, representa el tumor fibroso más frecuente de la infancia. Se caracteriza por la presencia de lesiones fibroblásticas-miofibroblásticas benignas. Suele presentarse en niños menores de 2 años, aunque puede aparecer a cualquier edad. La forma solitaria (miofibromas) puede afectar a piel, tejido celular subcutáneo, músculo o hueso. En la forma multicéntrica (miofibromatosis), además, puede haber lesiones viscerales. Las lesiones suelen regresar espontáneamente en uno o dos años siendo el pronóstico excelente en estos casos. Sin embargo, cuando existe afectación visceral el pronóstico es malo y está indicado el tratamiento quimioterápico. La afectación pulmonar es la que se asocia a un peor pronóstico. Aunque el diagnóstico definitivo es anatomopatológico, las pruebas de imagen son fundamentales para caracterizar las lesiones, establecer la extensión de la enfermedad, valorar la afectación visceral y realizar un seguimiento evolutivo de las lesions (AU)

Infantile myofibromatosis, despite being considered a rare condition, is the most common fibrous tumour in infancy. It is characterised by the presence of benign fibroblasticmyofibroblastic lesions. It usually occurs in children under two years-old, but it can appear at any age. The solitary form (myofibromas) may affect the skin, subcutaneous cellular tissue, muscle or bone. In the multi-centred form (myofibromatosis), there may also be visceral lesions. The lesions usually regress spontaneously in one or two years, with the prognosis being excellent in these cases. However, when there is visceral involvement, the prognosis is poor and treatment with chemotherapy is indicated. Lung involvement is more associated with a poor prognosis. Although the definitive diagnosis is by histopathology, diagnostic imaging tests are essential for characterising the lesions, establishing the extent of the disease, assessing visceral involvement, and following up the progression of the lesions (AU)

[Infantile myofibromatosis. Study of a case using whole body ultrasound and MRI]. / Miofibromatosis infantil. Un caso estudiado con ecografía y resonancia magnética de cuerpo entero.

Infantile myofibromatosis, despite being considered a rare condition, is the most common fibrous tumour in infancy. It is characterised by the presence of benign fibroblastic-myofibroblastic lesions. It usually occurs in children under two years-old, but it can appear at any age. The solitary form (myofibromas) may affect the skin, subcutaneous cellular tissue, muscle or bone. In the multi-centred form (myofibromatosis), there may also be visceral lesions. The lesions usually regress spontaneously in one or two years, with the prognosis being excellent in these cases. However, when there is visceral involvement, the prognosis is poor and treatment with chemotherapy is indicated. Lung involvement is more associated with a poor prognosis. Although the definitive diagnosis is by histopathology, diagnostic imaging tests are essential for characterising the lesions, establishing the extent of the disease, assessing visceral involvement, and following up the progression of the lesions.

Detección precoz mediante resonancia magnética de un angioma cavernoso tras radioterapia craneal por ependimoma anaplásico en un niño / Early magnetic resonance imaging detection of a cavernous angioma after cranial radiotherapy for an anaplastic ependymoma in a boy

La radioterapia se incluye en gran parte de los regímenes terapéuticos, preventivos y de acondicionamiento, en oncología pediátrica. Numerosos efectos secundarios tardíos de la radiación craneal han sido descritos y son bien conocidos. Sin embargo, se han publicado casos esporádicos de angiomas cavernosos radio-inducidos (ACRI) y aún menos casos se han referido con presentación temprana de estas lesiones. En esta comunicación breve se muestra la aparición de un ACRI con un periodo de latencia corto entre la finalización de la radioterapia y el desarrollo del mismo, en el contexto de un paciente tratado por un tumor del SNC en edad infantil, ependimoma anaplásico infratentorial. Se comentan las diferentes variables propuestas para la formación de novo de estas lesiones y las características de imagen, el tratamiento, pronóstico y seguimiento de este tipo de cavernomas (AU)

Radiotherapy forms part of most therapeutic, preventive, and conditioning regimens in pediatric oncology. Numerous late secondary effects of cranial radiation are well known. However, radiation-induced cavernous angiomas (RICA) have been reported only sporadically and even fewer cases of earlier presentation of RICA have been reported. In this brief report, we describe a RICA that appeared in a boy treated for a CNS tumor (an infratentorial anaplastic ependymoma) after a short latency period between the end of radiotherapy and the development of the RICA (AU)

Síndrome de aorta media: presentación de tres casos pediátricos / Middle aortic syndrome: a report of three pediatric cases

El síndrome de aorta media (SAM) consiste en una estenosis de la aorta abdominal generalmente con inclusión de las arterias renales y viscerales. Es un síndrome muy infrecuente (0,5-2%), aunque una causa importante de hipertensión arterial (HTA) en niños y adolescentes. Se cree que su origen embriológico se debe a un fallo en la fusión de las dos aortas dorsales y la etiología es idiopática en un alto porcentaje. Su localización es interrenal en el 54% de los casos. Clínicamente cursa con HTA, sintomática o no. A la exploración puede apreciarse soplo abdominal, pulsos femorales disminuidos o ausentes y diferencia de TA entre miembros superiores e inferiores. La angiografía es considerada la técnica de elección aunque las técnicas angiográficas no invasivas mediante RM y TC se encuentran al mismo nivel diagnóstico. La ecografía es la primera técnica de despistaje. El tratamiento médico consiste en la combinación de diferentes antihipertensivos. El tratamiento quirúrgico puede ser curativo(AU)

The middle aortic syndrome (MAS) is caused by stenosis of the abdominal aorta, often accompanied by concomitant stenosis of the renal or visceral arteries. Although MAS is uncommon (0.5-2%), it is an important cause of hypertension in children and adolescents. It is thought to originate in a failure of the two dorsal aortas to fuse during embryological development, and a high percentage of cases are idiopathic. MAS affects the segment of the aorta between the renal arteries in 54% of cases. Clinically, it courses with symptomatic or asymptomatic arterial hypertension. On physical examination, findings include an abdominal bruit, diminished or absent femoral pulses, and a difference between the arterial pressure of the upper and lower limbs. Angiography is the technique of choice, although noninvasive MR angiography and CT angiography have similar diagnostic accuracy. Ultrasonography is the primary screening technique. Medical treatment consists of a combination of different antihypertensives. Surgical treatment can be curative(AU)

Abscesos cerebelosos secundarios a infección de seno dérmico occipital / Cerebellar abscesses secondary to infection of an occipital dermal sinus

El seno dérmico es un defecto congénito del cierre del tubo neural que condiciona una comunicación en diferentes grados entre la piel y el sistema nervioso central. Se puede situar desde la raíz nasal hasta el cono medular, siendo la localización occipital la segunda en frecuencia. Se encuentra frecuentemente asociado con quiste dermoide o epidermoide, siendo más rara la asociación a teratomas. Los pacientes con quiste dermoide occipital asociado a seno dérmico pueden desarrollar meningitis y/o formación de abscesos como primera manifestación clínica, debido a la abscesificación del quiste dermoide en sí mismo o bien a la formación de abscesos secundarios, siendo esta última una entidad escasamente descrita en la literatura. Se presenta un caso de quiste dermoide asociado a seno dérmico infectado con desarrollo posterior de abscesos cerebelosos e hidrocefalia(AU)

A dermal sinus is a congenital defect arising from a closure failure of the neural tube that results in different degrees of communication between the skin and the central nervous system. A dermal sinus can occur anywhere from the root of the nose to the conus medullaris, and the occipital location is the second most common. Dermal sinuses are often found in association with dermoid or epidermoid cysts and less frequently with teratomas. Patients with an occipital dermoid cyst associated with a dermal sinus can develop meningitis and/or abscesses as the first clinical manifestation of the disease due to the dermoid cyst itself becoming abscessed or to the formation of secondary abscesses; few cases of the formation of secondary abscesses have been reported. We present a case of a dermoid cyst associated with an infected dermal sinus and posterior development of cerebellar abscesses and hydrocephalus(AU)

[Cerebellar abscesses secondary to infection of an occipital dermal sinus]. / Abscesos cerebelosos secundarios a infección de seno dérmico occipital.

A dermal sinus is a congenital defect arising from a closure failure of the neural tube that results in different degrees of communication between the skin and the central nervous system. A dermal sinus can occur anywhere from the root of the nose to the conus medullaris, and the occipital location is the second most common. Dermal sinuses are often found in association with dermoid or epidermoid cysts and less frequently with teratomas. Patients with an occipital dermoid cyst associated with a dermal sinus can develop meningitis and/or abscesses as the first clinical manifestation of the disease due to the dermoid cyst itself becoming abscessed or to the formation of secondary abscesses; few cases of the formation of secondary abscesses have been reported. We present a case of a dermoid cyst associated with an infected dermal sinus and posterior development of cerebellar abscesses and hydrocephalus.

[Middle aortic syndrome: a report of three pediatric cases]. / Síndrome de aorta media: presentación de tres casos pediátricos.

The middle aortic syndrome (MAS) is caused by stenosis of the abdominal aorta, often accompanied by concomitant stenosis of the renal or visceral arteries. Although MAS is uncommon (0.5-2%), it is an important cause of hypertension in children and adolescents. It is thought to originate in a failure of the two dorsal aortas to fuse during embryological development, and a high percentage of cases are idiopathic. MAS affects the segment of the aorta between the renal arteries in 54% of cases. Clinically, it courses with symptomatic or asymptomatic arterial hypertension. On physical examination, findings include an abdominal bruit, diminished or absent femoral pulses, and a difference between the arterial pressure of the upper and lower limbs. Angiography is the technique of choice, although noninvasive MR angiography and CT angiography have similar diagnostic accuracy. Ultrasonography is the primary screening technique. Medical treatment consists of a combination of different antihypertensives. Surgical treatment can be curative.

[Early magnetic resonance imaging detection of a cavernous angioma after cranial radiotherapy for an anaplastic ependymoma in a boy]. / Detección precoz mediante resonancia magnética de un angioma cavernoso tras radioterapia craneal por ependimoma anaplásico en un niño.

Radiotherapy forms part of most therapeutic, preventive, and conditioning regimens in pediatric oncology. Numerous late secondary effects of cranial radiation are well known. However, radiation-induced cavernous angiomas (RICA) have been reported only sporadically and even fewer cases of earlier presentation of RICA have been reported. In this brief report, we describe a RICA that appeared in a boy treated for a CNS tumor (an infratentorial anaplastic ependymoma) after a short latency period between the end of radiotherapy and the development of the RICA. We comment on the different variables proposed to explain the formation of these lesions, as well as on their imaging features, treatment, prognosis, and follow-up.

Tumores corticosuprarrenales pediátricos: imagen de adenomas y carcinomas / Adrenocortical tumors in children: imaging adenomas and carcinomas

Objetivo. El propósito de la revisión es mostrar las características de imagen que presentan los tumores corticosuprarrenales pediátricos (TCSP). Material y métodos. Se realiza una revisión retrospectiva de los pacientes diagnosticados de TCSP en nuestro hospital terciario en el periodo comprendido entre los años 2000 y 2010, desde el punto de vista radiológico y anatomopatológico. Se estudian las características radiológicas mediante ecografía, tomografía computarizada (TC) y resonancia magnética (RM), que ayudarán a orientar la lesión hacia benignidad o malignidad, y el seguimiento de imagen. Resultados Se presentan 8 TCSP: 5 carcinomas, 2 adenomas y un tumor borderline; se clasifican 2 en estadio I, uno en estadio II, 3 en estadio III y 2 en estadio IV. La radiología permitió el diagnóstico de carcinoma en estadio IV en 2 casos, dada la presencia de metástasis iniciales en un paciente y el gran tamaño y desestructuración tumoral en otro, desarrollando posteriormente metástasis. En los otros 6 casos el diagnóstico radiológico fue de aproximación respecto a su naturaleza de carcinoma o adenoma. Conclusiones. Los TCSP son raros en la infancia. Engloban las entidades de adenoma y carcinoma, siendo difíciles de diferenciar histológica y radiológicamente en ausencia de infiltración vascular y/o metástasis. En un paciente en edad pediátrica la combinación de una masa suprarrenal y signos clínicos de hiperfunción corticosuprarrenal es virtualmente diagnóstica de TCSP (AU)

Objective. This article aims to show the imaging characteristics of pediatric adrenocortical tumors. Material and methods. We review the imaging and histological findings in patients diagnosed with pediatric adrenocortical tumors at our tertiary hospital between 2000 and 2010. We analyze the findings at ultrasonography, computed tomography, and magnetic resonance imaging that can help orient the diagnosis toward benign or malignant lesions and guide imaging follow-up. Outcome. We found 8 adrenocortical tumors in children: 5 carcinomas, 2 adenomas, and 1 borderline tumor. Two tumors were classified as stage I, 1 as stage II, 3 as stage III, and 2 as stage IV. Imaging enabled the diagnosis of stage IV carcinoma in 2 cases, due to the presence of initial metastases in one patient and to size of the tumor and structural changes in the other, who later developed metastases. In the other 6 cases, the imaging studies oriented the diagnosis toward carcinoma or adenoma. Conclusions. Adrenocortical tumors are rare in children. Adrenocortical tumors include adenomas and carcinomas, and in the absence of vascular infiltration and/or metastases it is difficult to differentiate between the two types by imaging and histology. The combination of an adrenal mass and clinical signs of adrenocortical hyperfunction in a child is virtually diagnostic of an adrenocortical tumor (AU)

Trombosis venosa como complicación en paciente pediátrico tratado con L-asparraginasa / Venous thrombosis as a complication of L-asparaginase treatment in a child

No disponible

Etiología y diagnóstico de la neumonía adquirida en la comunidad y sus formas complicadas / Aetiology and diagnosis of community acquired pneumonia and its complicated forms

La neumonía adquirida en la comunidad (NAC) es una enfermedad frecuente en la infancia, en cuyo diagnóstico y tratamiento participan diversas especialidades pediátricas. Esto ha motivado que la Sociedad Española de Neumología Pediátrica (SENP) y la Sociedad Española de Infectología Pediátrica (SEIP) elaboren un documento de consenso sobre el diagnóstico de la NAC, revisando mediante la medicina basada en la evidencia aquellos aspectos prácticos sobre el mismo. Se analizan la etiología y la epidemiología, con los cambios actuales, así como la validez de ciertas pruebas complementarias, como los reactantes de fase aguda, los métodos microbiológicos y los métodos de imagen, orientando al pediatra en la utilidad real de los mismos(AU)

Community Acquired Pneumonia (CAP) is a common childhood disease, involving several paediatric subspecialties in its diagnosis and treatment. This has prompted the Spanish Society of Paediatric Pulmonology (SENP) and the Spanish Society of Paediatric Infectious Diseases (SEIP) to prepare a consensus document on the diagnosis of CAP, assessing the practical aspects by means of evidence-based medicine. It discusses the aetiology and epidemiology, with the current changes and the validity of certain laboratory tests, such as acute phase reactants, microbiological and imaging techniques, guiding the paediatricians in the real value of these tests(AU)

Resonancia magnética en el ependimoma anaplásico infratentorial pediátrico / Magnetic resonance imaging of infratentorial anaplastic ependymoma in children

Objetivo. El objetivo es mostrar las principales características radiológicas que el ependimoma anaplásico puede presentar en las imágenes de resonancia magnética (RM). Material y métodos. Se recogen los pacientes diagnosticados de ependimoma infratentorial de tipo anaplásico en los últimos 6 años en nuestro hospital terciario. Se estudian las características de imagen mediante RM (secuencias convencionales protocolizadas para estudio tumoral del SNC, difusión, estudio con contraste, espectroscopia) de este tipo tumoral. Resultados. El estudio de nuestra serie de 7 pacientes pediátricos con ependimoma anaplásico infratentorial no mostró características definitivas que ayudaran en la distinción entre grado II y grado III previamente al diagnóstico anatomopatológico, al no haber presentado ninguno de ellos diseminación al líquido cefalorraquídeo (LCR) en el momento del diagnóstico ni aumento de la restricción tumoral en la secuencia de difusión. Conclusiones. Las características radiológicas no son definitivas para distinguir entre los ependimomas grado II y los ependimomas anaplásicos grado III. Únicamente algunos detalles sobre la difusión y la mayor propensión a la diseminación al LCR, si se presentan, pueden diferenciarlos desde el punto de vista de la imagen (AU)

Objective. To show the main findings for anaplastic ependymoma on MRI. Material and methods. We reviewed all patients diagnosed with anaplastic ependymoma at our tertiary hospital during a six-year period. We recorded the MRI findings for this type of tumor (on conventional sequences following the protocol for the study of CNS tumors, diffusion-weighted imaging, contrast-enhanced sequences, and MR spectroscopy). Results. Our series comprises seven children with infratentorial anaplastic ependymoma. We found no definitive characteristics to distinguish between grade II and grade III tumors before histology, as none of the lesions had spread to the cerebrospinal fluid at diagnosis or showed increased restriction in the diffusion-weighted sequence. Conclusions. The MRI characteristics cannot definitively distinguish between grade II ependymomas and anaplastic grade III ependymomas. Only a few details about diffusion and dissemination to the cerebrospinal fluid, if present, can distinguish between these types at imaging (AU)

[Magnetic resonance imaging of infratentorial anaplastic ependymoma in children]. / Resonancia magnética en el ependimoma anaplásico infratentorial pediátrico.

OBJECTIVE: To show the main findings for anaplastic ependymoma on MRI. MATERIAL AND METHODS: We reviewed all patients diagnosed with anaplastic ependymoma at our tertiary hospital during a six-year period. We recorded the MRI findings for this type of tumor (on conventional sequences following the protocol for the study of CNS tumors, diffusion-weighted imaging, contrast-enhanced sequences, and MR spectroscopy). RESULTS: Our series comprises seven children with infratentorial anaplastic ependymoma. We found no definitive characteristics to distinguish between grade II and grade III tumors before histology, as none of the lesions had spread to the cerebrospinal fluid at diagnosis or showed increased restriction in the diffusion-weighted sequence. CONCLUSIONS: The MRI characteristics cannot definitively distinguish between grade II ependymomas and anaplastic grade III ependymomas. Only a few details about diffusion and dissemination to the cerebrospinal fluid, if present, can distinguish between these types at imaging.

[Adrenocortical tumors in children: imaging adenomas and carcinomas]. / Tumores corticosuprarrenales pediátricos: imagen de adenomas y carcinomas.

OBJECTIVE: This article aims to show the imaging characteristics of pediatric adrenocortical tumors. MATERIAL AND METHODS: We review the imaging and histological findings in patients diagnosed with pediatric adrenocortical tumors at our tertiary hospital between 2000 and 2010. We analyze the findings at ultrasonography, computed tomography, and magnetic resonance imaging that can help orient the diagnosis toward benign or malignant lesions and guide imaging follow-up. OUTCOME: We found 8 adrenocortical tumors in children: 5 carcinomas, 2 adenomas, and 1 borderline tumor. Two tumors were classified as stage I, 1 as stage II, 3 as stage III, and 2 as stage IV. Imaging enabled the diagnosis of stage IV carcinoma in 2 cases, due to the presence of initial metastases in one patient and to size of the tumor and structural changes in the other, who later developed metastases. In the other 6 cases, the imaging studies oriented the diagnosis toward carcinoma or adenoma. CONCLUSIONS: Adrenocortical tumors are rare in children. Adrenocortical tumors include adenomas and carcinomas, and in the absence of vascular infiltration and/or metastases it is difficult to differentiate between the two types by imaging and histology. The combination of an adrenal mass and clinical signs of adrenocortical hyperfunction in a child is virtually diagnostic of an adrenocortical tumor.

[Review and update about medulloblastoma in children]. / Meduloblastoma pediátrico, revisión y puesta al día.

Medulloblastoma is the most common malignant CNS tumor in children. Although all medulloblastomas are classified as grade IV lesions, the wide histological and molecular variation among these tumors means that the risk and prognosis involved also vary widely. Imaging studies are important not only because the initial diagnostic evaluation indicates what type of surgery will be performed and has prognostic value, but also because it influences the postoperative treatment approach, providing, among other details, information about the dissemination of disease and remnants of the tumor after surgery, which are both risk factors in medulloblastomas. Improvements in our understanding of the biological and molecular characteristics of medulloblastoma promise a dramatic change in the accuracy of staging and treatment of this tumor in the near future that are sure to bring about further improvements in survival.

Meduloblastoma pediátrico, revisión y puesta al día / Review and update about medulloblastoma in children

El meduloblastoma es el tumor maligno más frecuente del SNC en pediatría. Presenta una variabilidad histológica y molecular tan importante que hace que, aunque todos los tipos de meduloblastoma se clasifiquen como grado IV, el riesgo y el pronóstico de los mismos sean muy amplios. Las pruebas de imagen son importantes no sólo porque la valoración diagnóstica inicial indica el tipo de cirugía a realizar, que presenta un valor pronóstico, sino para el planteamiento terapéutico posterior, teniendo en cuenta que determinan, entre otras, la diseminación y el resto tumoral posquirúrgico, factores de riesgo en este tumor. Una mejor comprensión de las características biológicas y moleculares del meduloblastoma promete un cambio dramático hacia la precisión en la estadificación y el tratamiento tumoral en un futuro próximo, favoreciendo aún más la actual mejora de la supervivencia (AU)

Medulloblastoma is the most common malignant CNS tumor in children. Although all medulloblastomas are classified as grade IV lesions, the wide histological and molecular variation among these tumors means that the risk and prognosis involved also vary widely. Imaging studies are important not only because the initial diagnostic evaluation indicates what type of surgery will be performed and has prognostic value, but also because it influences the postoperative treatment approach, providing, among other details, information about the dissemination of disease and remnants of the tumor after surgery, which are both risk factors in medulloblastomas. Improvements in our understanding of the biological and molecular characteristics of medulloblastoma promise a dramatic change in the accuracy of staging and treatment of this tumor in the near future that are sure to bring about further improvements in survival (AU)