Neonatal supraventricular extrasystole as early clinical debut of cardiac rhabdomyoma.

We are reporting the case of a newborn with a diagnosis of frequent supraventricular extrasystoles, up to 25% of beats at Holter monitoring, and partial response to beta-blockers. Initial echocardiographic studies were normal until the identification of a right atrial mass at 4 months of life. Given the progressive growth of the mass and the suspicion of myxoma or thrombus in the magnetic resonance study, surgical resection of the tumor was performed. The surgical specimen was histologically diagnostic of rhabdomyoma. Currently, the patient remains asymptomatic after a 6-year follow-up period. A single rhabdomyoma is described, located in an atypical situation, near the crista terminalis, and diagnosed from frequent extrasystoles which appeared before the echocardiographic resolution was able to identify it. Magnetic resonance showed nondiagnostic tissue enhancement characteristics.

Miocarditis aguda frente a miocardiopatía no compactada en el lactante: utilidad de la resonancia magnética / Acute myocarditis versus ventricular noncompaction cardiomyopathy in infancy: role of magnetic resonance

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Acute Myocarditis with Infarct-like Presentation in a Pediatric Population: Role of Cardiovascular Magnetic Resonance.

Chest pain is a typical symptom of acute myocarditis in adolescents. It may be indistinguishable from myocardial ischemia so it is called "infarct-like pattern." Cardiovascular magnetic resonance has an important role as a non-invasive diagnostic tool. The aim of our study is to provide a description of an acute myocarditis series with infarct-like pattern and to evaluate the cardiovascular magnetic resonance role in a pediatric population. We included all pediatric patients (0-16 years) admitted to our hospital (May 2007-May 2016) with clinical diagnosis of acute myocarditis and infarct-like presentation (chest pain, EKG alterations, and released cardiac biomarkers). Diagnosis was confirmed with cardiovascular magnetic resonance using Lake Louise criteria. Seven patients (five males, two females) with a median age of 14 years (12.5-15.2) were included. All patients showed ST-segment changes and released cardiac biomarkers. Three patients had left ventricular hypertrophy and two presented mild systolic left ventricular dysfunction. All patients had at least two positive Lake Louise criteria. Late gadolinium enhancement was positive in all of them. With a median follow-up of 23 months (8-47), all of them are alive, with no cardiac symptoms and normal ventricular function. Infarct-like pattern is a typical presentation of acute myocarditis in adolescents. CMR should be performed in this population and may be considered as a first-line diagnostic tool. Its high sensitivity in infarct-like acute myocarditis may allow us to avoid endomyocardial biopsy. Unlike what was described in adults, late gadolinium enhancement does not imply worse outcome in our series.

Utilidad de la resonancia magnética para el diagnóstico de miocarditis en niños / Role of magnetic resonance imaging in the diagnosis of myocarditis in children

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Tracheobronchomegaly following intrauterine tracheal occlusion for congenital diaphragmatic hernia.

BACKGROUND: Fetuses with severe congenital diaphragmatic hernia (CDH) and pulmonary hypoplasia may benefit from fetal endoluminal tracheal occlusion (FETO). Enlargement of the main bronchi and trachea appears to be a common complication of FETO. OBJECTIVE: To retrospectively evaluate the trachea and main bronchi of infants who underwent FETO for CDH and compare diameters with age-matched references. MATERIAL AND METHODS: Postnatal and follow-up chest radiographs were performed in seven children with unilateral CDH treated by FETO. Additional CT was performed in six of these (one neonate died before CT could be performed). Images were acquired from 3 days to 23 months of age. For each child, radiographs and CT images with optimal visualisation of the airways were selected for retrospective analysis. Tracheal and bronchial morphology was assessed by two experienced paediatric radiologists, and the diameters of these structures measured and compared with age-matched references. RESULTS: Mean diameters of the trachea and main bronchi were above the age-matched normal range in all patients, regardless of the side of the hernia or the degree of lung hypoplasia. CONCLUSION: Enlargement of the trachea and main bronchi appears following FETO and persists at least to the age of 5 years.

Correlation between US and MRI for prenatal lung volumetry in diaphragmatic hernia, and use of Doppler to identify the ipsilateral lung cap.

BACKGROUND: Pulmonary hypoplasia is a common cause of neonatal death. OBJECTIVE: To describe the correlation between relative fetal lung volume (RFLV) and lung-to-head ratio (LHR) in fetuses with unilateral diaphragmatic hernia. Additionally, to describe identification of the ipsilateral lung cap by power Doppler. MATERIALS AND METHODS: Single-institution study of consecutive fetuses with diaphragmatic hernia. LHR (by US) and RFLV (by MRI) were correlated in fetuses with and without an ipsilateral lung cap seen at MRI. In four, color/power Doppler was used to follow the pulmonary artery of the ipsilateral lung to identify the compressed cap. RESULTS: The study included 48 fetuses of 20-38 weeks' gestational age (mean, 26 weeks). Mean LHR was 1.52 (range, 0.6-3) in fetuses with a lung cap and 1.15 (range, 0.6-2.58) in fetuses without (P = 0.043). Mean RFLV was 47.4% (range, 18-80%) in fetuses with and 32.9% (range, 14-57%) in fetuses without a lung cap (P = 0.005). RFLV and LHR correlated (r = 0.41, P = 0.01 in those with a cap; r = 0.50, P = 0.05 in those without). Power Doppler identified the ipsilateral lung cap and pulsed Doppler confirmed pulmonary vascularization in four of four fetuses. CONCLUSION: LHR underestimates lung volume in fetuses with an ipsilateral lung cap. Power Doppler may be useful for identifying the cap.

Congenital tumours involving the head, neck and central nervous system.

Congenital intracranial tumours are uncommon and differ from those occurring in older children in clinical presentation, imaging characteristics and prognosis. These tumours are often detected incidentally on routine prenatal US and/or fetal MRI. Hence, the paediatric radiologist should be familiar with the features of those lesions that should be included in the differential diagnosis. In general, the prognosis of these conditions is poor owing to large tumour size and the limitations of adjuvant therapy at such a young age. Congenital lesions involving the head and neck region require a meticulous imaging approach using both US and MRI techniques to better guide prenatal planning and fetal or neonatal surgical procedures.

Selective muscle fiber loss and molecular compensation in mitochondrial myopathy due to TK2 deficiency.

A 12-year-old patient with mitochondrial DNA (mtDNA) depletion syndrome due to TK2 gene mutations has been evaluated serially over the last 10 years. We observed progressive muscle atrophy with selective loss of type 2 muscle fibers and, despite severe depletion of mtDNA, normal activities of respiratory chain (RC) complexes and levels of COX II mitochondrial protein in the remaining muscle fibers. These results indicate that compensatory mechanisms account for the slow progression of the disease. Identification of factors that ameliorate mtDNA depletion may reveal new therapeutic targets for these devastating disorders.

Conservative versus surgical treatment for complex neonatal ovarian cysts: outcomes study.

OBJECTIVE: Prenatally diagnosed complex ovarian cysts are most often managed surgically in an attempt to save the ovary. Nevertheless, published surgical results disclose that most patients undergo oophorectomy or salpingo-oophorectomy. We assessed whether a surgical or conservative approach was more appropriate by comparing the long-term outcome of infants treated by both methods. A hypothesis for the cause of complex cysts is presented. CONCLUSION: Clinical evidence questions the use of surgery for asymptomatic complex ovarian cysts. Histologic analysis suggests gonad maldevelopment as the origin of complex neonatal ovarian cysts.

Nena de 2 anys amb tumoració en extremitat superior detra i febre / Two year-old girl with a right upper extremity mass and febrile illness

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Complicated acute pediatric bacterial sinusitis: Imaging updated approach.

Acute bacterial sinusitis is usually a clinical diagnosis. Orbital complications require emergent evaluation with computed tomography. Using the orbital septum as an anatomic landmark, such infections can be classified as pre- or postseptal and treated with the most adequate therapy, ie, oral or intravenous antibiotics or surgical endonasal drainage. Intracranial complications can be seen in 3.7% to 11% of these patients, often with subtle clinical symptoms and signs. Radiologists play a decisive role in the final management of these patients and should be familiar with the most relevant complications. In this article, we present a retrospective review of all pediatric patients referred to our department for paranasal sinuses and orbital computed tomography because of acute complicated bacterial sinusitis. They were studied with an emergent enhanced facial and cranial computed tomography within 24 hours of admission, followed by magnetic resonance imaging when intracranial complications were suspected. Particular emphasis is placed on the imaging algorithm and the most relevant complications; we correlate imaging findings with clinical and bacteriological data.

Second malignancies in pediatric patients: imaging findings and differential diagnosis.

Therapeutic advances in the treatment of pediatric neoplasms have improved the prognosis but have also increased the risk of developing rare second malignant neoplasms (SMNs). Primary neoplasms that are often associated with SMNs include lymphoma, retinoblastoma, medulloblastoma, neuroblastoma, and leukemia. The most common SMNs are central nervous system (CNS) tumors, sarcomas, thyroid and parotid gland carcinomas, and leukemia, particularly acute myeloblastic leukemia. Genetic predisposition, chemotherapy, and especially radiation therapy are implicated as pathogenic factors in SMN. All survivors of childhood cancer should have lifelong follow-up, preferably with magnetic resonance imaging, which does not require ionizing radiation and provides greater anatomic detail and resolution in the head and neck region and the CNS. A new or progressive lesion may represent recurrence of the primitive neoplastic process, late radiation injury, or, more infrequently, an SMN. Differential diagnosis can be very difficult, and outcome is often fatal. Treatment protocols should be modified to reduce the risk for SMN without compromising the effectiveness of initial therapy. Clinicians should individualize treatment for patients who are genetically predisposed to SMN. In addition, radiologists should be familiar with the long-term consequences of antineoplastic therapy to facilitate diagnosis and anticipate adverse outcomes.

Imaging of complications of acute mastoiditis in children.

Acute mastoiditis is a serious complication of acute otitis media in children. Suppurative disease in the mastoid region occasionally spreads to the adjacent dura mater of the posterior and middle cranial fossae and the sigmoid sinus by means of thrombophlebitis, osseous erosion, or anatomic pathways, producing intracranial complications. Computed tomography (CT) should be performed early in the course of the disease to classify the mastoiditis as incipient or coalescent and to detect intracranial complications. On the basis of the clinical features and imaging findings, the disease is managed conservatively with intravenously administered antibiotics or treated with mastoidectomy and drainage plus antibiotic therapy. CT is therefore a decisive diagnostic tool in determining the type of therapy. In addition, magnetic resonance imaging is performed in patients with clinical symptoms or CT findings suggestive of intracranial complications because of its higher sensitivity for detection of extraaxial fluid collections and associated vascular problems.

Neuroimaging in pediatric leukemia and lymphoma: differential diagnosis.

Recent advances in therapy for pediatric hematologic neoplasms have greatly improved the prognosis but have resulted in an increased incidence of associated complications and toxic effects. The main neuroimaging features in pediatric patients with leukemia or lymphoma treated with chemotherapy or radiation therapy were retrospectively reviewed. To simplify the approach and facilitate differential diagnosis, the neuroimaging features have been classified into three main categories: central nervous system manifestations of primary disease, side effects of therapeutic procedures (radiation therapy, chemotherapy, bone marrow transplantation), and complications due to immunosuppression, particularly infections. Manifestations of primary disease include cerebrovascular complications (hemorrhage, cerebral infarction) and central nervous system involvement (infiltration of the meninges, parenchyma, bone marrow, orbit, and spine). Effects of radiation therapy include white matter disease, mineralizing microangiopathy, parenchymal brain volume loss, radiation-induced cryptic vascular malformations, and second neoplasms. Effects of chemotherapy and bone marrow transplantation include hemorrhage, dural venous thrombosis, white matter disease, reversible posterior leukoencephalopathy syndrome, and anterior lumbosacral radiculopathy. Both the underlying malignancy and antineoplastic therapy can cause immunosuppression. Fungi are the most frequent causal microorganisms in immunosuppressed patients with infection. Familiarity with the imaging findings is essential for proper diagnosis of neurologic symptoms in pediatric patients with oncohematologic disease.