[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.

[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.

[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.

[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.

[Spondylodiscitis: Diagnosis and medium-long term follow up of 18 cases]. / Espondilodiscitis: diagnóstico y seguimiento a medio-largo plazo de 18 casos.

INTRODUCTION: Spondylodiscitis is an uncommon disease in children. It is often misdiagnosed or the diagnosis is made late in the course of illness. OBJECTIVES: To review the clinical, analytical and radiological characteristics of children with spondylodiscitis in the Materno-Infantil Hospital of Malaga. PATIENTS AND METHODS: Retrospective cohort study on children diagnosed with spondylodiscitis, during a period of 11 years. RESULTS: Eighteen patients were included in the study. Spondylodiscitis was more frequent in patients younger than 3 years old and older than 12 years old. The average time of delay in diagnosis was 26.9 days. In 8 cases the diagnosis was missed initially. Three clinical patterns appeared: in children under 3 years of age, refusing to walk and sit (100%) and irritability (42%); between 3 and 12 years of age, limb (100%) and abdominal pain (100%); in adolescents, back pain (75%). Fever was present in 38% of the cases, and low-grade fever in 8 (44%). A total of 50% of the patients had a moderate leucocytosis, and a slight increase in ESR; the most frequent location was L3-L4. X-ray diagnosis was pathological in 88% of the cases. The initial MRI diagnosed 100% of the cases. In addition, nerve roots damage (5 cases), inflammatory masses/paravertebral abscesses (5), epidural abscess (1) and psoas abscesses (2) were detected. A total of 94% of the children received antibiotics and 100% of the children underwent immobilization. All patients recovered early after the beginning of treatment, with the exception of those affected by psoas abscesses. The radiological follow-up was done in 17 patients (12 by MRI). After a mean of 14 months (rank 1-48), persistent diminution of disc space was seen in 100% of the children, and improvement of soft-tissue inflammation. Clinically (follow-up only in 11 patients) all patients regained normal mobility and only 27% had moderate pain. CONCLUSION: Spondylodiscitis, whose delay in the diagnosis is frequent, is a serious illness. Complications include abscesses and nerve root damage. MRI is the study of choice to determine the extension to neighbouring tissues.

[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.

[Papilloma and carcinoma of the choroid plexus in pediatric patients]. / Papiloma y carcinoma de plexos coroideos en la edad pediátrica.

Papillomas of the choroid plexus are rare tumors of neuroectodermal origin; they represent less than 5% of all central nervous system (CNS) tumors in pediatric patients. Choroid plexus carcinomas are even rarer. We reviewed the incidence of these neoplasms at our reference hospital and found six tumors of the choroid plexus (five papillomas and one carcinoma) in five patients. Patient age ranged from prenatal to 25 months. All five patients underwent computed tomography (CT) examination. Four perinatal patients underwent ultrasound examination, four magnetic resonance imaging (MRI), and one (years ago) angiography. All patients had tumors located in the lateral ventricles, and one patient had a second tumor located in the third ventricle. These tumors are predominantly solid, intraventricular, with well-defined polylobulated margins. They show intense vascularization on Doppler studies and marked contrast enhancement on CT and MRI studies. Hydrocephalus was present in three cases. All patients underwent surgery; total resection was achieved in the five papillomas, whereas the carcinoma was partially resected and the patient is currently undergoing chemotherapy. The three patients with a single papilloma are disease free at follow-up (range 7 months to 11 years). The patient with two papillomas shows good recovery at follow-up, whereas the patient with carcinoma of the choroid plexus has a poor prognosis.

[Intrathoracic lipoblastoma with costal involvement]. / Lipoblastoma intratorácico con afectación costal.

We report the case of a two-year-old girl with a lipoblastoma arising from the chest wall with intrathoracic extension and costal involvement. The diagnosis was confirmed histologically after surgery. Plain-film chest x-rays showed an extrapleural mass; computed tomography (CT) and magnetic resonance imaging (MRI) suggested fatty contents. The differential diagnosis is mainly versus liposarcoma, which is extremely rare in children.

Nephrogenic rests, nephroblastomatosis, and associated lesions of the kidney.

The fetal kidney is formed by the development of nephrons from fetal metanephric blastema surrounding the ureteric bud. The fetal renal tissue matures into normal renal parenchyma during gestation, but, occasionally, fetal tissue persists into infancy as microscopic foci called nephrogenic rests. Nephrogenic rests are found in approximately 1% of infant kidneys at autopsy. Nephrogenic rests are associated with an increased risk of Wilms tumor, and it is theorized that nephrogenic rests undergo neoplastic change into Wilms tumor. Fortunately, this transformation occurs in less than 1% of young children with nephrogenic rests. Nephrogenic rests are associated with many syndromes, including Beckwith-Wiedemann syndrome, hemihypertrophy, and sporadic aniridia. Children with identifiable syndromes, once diagnosed, should be screened for the development of Wilms tumor. Nephrogenic rests are associated with other lesions such as multilocular cystic nephroma and multicystic dysplasia, usually without malignant complications.

[Kearns-Sayre syndrome: pediatric neuroradiologic findings in computed tomography and magnetic resonance imaging]. / Síndrome de Kearns-Sayre: hallazgos en tomografía computarizada y resonancia magnética del sistema nervioso central.

Clinical presentation of mitochondrial disorders is heterogeneous because the affected organs are those depending on a high rate of aerobic metabolism. They can appear at any age and evolution is progressive. Signs that guide diagnostic suspicion, especially in the pediatric age group, are heterogeneous clinical presentation and multisystem involvement. Within the spectrum of diseases caused by mitochondrial myopathy, there are clearly defined syndromes such as Kearns-Sayre syndrome. Muscle biopsy shows ragged red fibers and approximately 80 % of patients present sporadic deletions in mitochondrial DNA. Imaging studies reveal areas of hypointensity in basal ganglia and midbrain that are not visible after administration of contrast enhancement in computed tomography, and symmetric T2 hyperintensity lesions in these areas in magnetic resonance imaging. We present a patient with Kearns-Sayre syndrome, in whom radiological alterations were helpful in reaching the diagnosis.