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Objective To evaluate the clinical features of Rathke cleft cysts(RCCs) in children diagnosed by pituitary magnetic resonance(MR) and their features on MR.Methods Twenty-two children with RCCs aged 2-18 years old who visited the Affiliated Hospital of Qingdao University Medical College between Jan.2002 and Feb.2012 were enrolled.RCCs was conformed by pituitary MR.The clinical symptoms and imaging features were reviewed retrospectively.Results The clinical presentation of symptomatic children were as follows:endocrinopathy in 13 cases (59.1％),headache in 5 cases(22.7％) and visual disturbance in 1 case(4.5％) and variety of symptoms in 3 cases (13.6％),which including 1 case of short stature and dysgenitalism,1 case of type 1 diabetes with electrolyte disorder and the other of headache associated with visual impairment.Endocrinopathy included short stature 5 cases(22.7％),precocious puberty 4 cases(18.2％)and diabetes insipidus 4 cases(18.2％).Generally,RCCs appeared various on Tl-weighted MR,whereas on T2-weighted sequences the signal intensity was mostly high.High signals in the T1-weighted image on brain MR were related to pituitary hormone deficiency.Hypointensity of the cysts in T1-weighted was appeared when enhanced images.Conclusions The most common clinical manifestation of children with RCCs is endocrinopathy.Pituitary MR shows a certain characteristics and it is favorable in agreement with pathological diagnosis.MR may be of predictive value for the preoperative diagnosis.
RESUMO
Las malformaciones vasculares son enfermedades raras ocasionadas por errores en el desarrollo embriológico de los vasos sanguíneos. Estas lesiones, junto con los tumores vasculares, constituyen las anomalías vasculares. En el caso de las malformaciones vasculares, éstas se denominan de acuerdo al vaso que conforma la lesión y se clasifican según las características del flujo en malformaciones de bajo o alto flujo. Si bien la mayor parte son diagnosticadas según criterios clínicos, algunas lesiones requieren de estudios complementarios tanto para el establecimiento de un correcto diagnóstico como para la evaluación de su extensión. La ecografía Doppler suele ser el estudio inicial para conocer las características hemodinámicas y contribuir al diagnóstico diferencial entre un tumor y una malformación vascular. La resonancia magnética (RM) es el mejor método para evaluar la extensión de las lesiones y su relación con estructuras adyacentes. El diagnóstico preciso del tipo de malformación vascular es importante por su pronóstico y tratamiento diferentes. La Radiología Intervencionista constituye uno de los pilares fundamentales en el manejo terapéutico. Es altamente recomendable un abordaje multidisciplinario dada la complejidad de estas lesiones, particularmente en la elección de las opciones terapéuticas y en el seguimiento clínico de los pacientes.
Vascular malformations: diagnostic clues for the radiologist. Vascular malformations are rare conditions resulting from the abnormal development of blood vessels. These lesions, together with vascular tumors, represent the vascular anomalies. Vascular malformations are named according to which type of vessel is affected and are classified into high- or low-flow malformations on the basis of flow characteristics. Most vascular malformations are diagnosed according to clinical criteria. However, some require imaging studies to confirm the diagnosis or assess its extension. Doppler ultrasound is the usual initial study for hemodynamic assessment and forits contribution to differential diagnosis between vascular tumors and malformations. Magnetic resonance imaging(MRI) is the best technique for evaluating the extent of the lesions and their relationship with adjacent structures. An accurate diagnosis of the type of vascular malformation is of importance because of the different prognosis and treatment. Interventional radiology is one of the cornerstones in the therapeutic management. A multidisciplinary approach is highly recommended given the complexity of these lesions, particularly for selecting therapeutic options and for the patient clinical follow-up.