ABSTRACT
BACKGROUND: Heterotaxy refers to the abnormal arrangement of organs across the left-right axis and is typically associated with complex cardiovascular malformations. OBJECTIVE: To characterise the range of cardiac and extracardiac CT angiography findings in children with heterotaxy using the latest nomenclature consensus and to compare the different types of isomerism. MATERIALS AND METHODS: We retrospectively analysed the data of 64 consecutive paediatric patients referred to our tertiary paediatric cardiovascular centre who underwent CT angiography for the evaluation of known or suspected heterotaxy within a 52-month period. RESULTS: Right atrial isomerism was identified in 44 (69%) children, while left atrial isomerism was identified in 18 (28%) children. Atrial appendage anatomy and situs could not be determined in 2 children (3%). Associated heart defects included complete atrioventricular canal (CAVC) in 51 (80%) children, total anomalous pulmonary venous return in 43 (67%) and pulmonary atresia in 23 (36%). The bronchial branching pattern corresponded to atrial appendage morphology in all children except in the two in whom atrial appendage morphology could not be defined. In children with right atrial isomerism, the most common associated abnormalities were CAVC (n=41, 93%) and asplenia (n=34, 77%), while in those with left atrial isomerism, the most common associated abnormalities were polysplenia (n=17, 94%) and an interrupted inferior vena cava with azygos continuation (n=15, 83%). CONCLUSION: CT angiography provides useful cardiovascular and extracardiac data on heterotaxy, which frequently involves a pattern of side-related findings but has great anatomical variability.
Subject(s)
Computed Tomography Angiography/methods , Heterotaxy Syndrome/diagnostic imaging , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Retrospective StudiesABSTRACT
Segmental spinal dysgenesis (SSD) is a rare congenital spinal abnormality characterized by segmental dysgenesis or agenesis of the thoracolumbar or lumbar spine, congenital kyphosis, and abnormal configuration of the underlying spinal cord. A unique feature of SSD is that the vertebrae are present above and below the defect, and there is often a lower cord segment in the caudal spinal canal. We report a fetal MRI case of SSD with postmortem and neuropathological correlations. Our report confirms already published findings including the presence of a neurenteric cyst but is the first to document anterior spinal artery segmental agenesis in SSD.
Subject(s)
Kyphosis/complications , Spinal Diseases/complications , Spinal Diseases/pathology , Spine/abnormalities , Vertebral Artery/abnormalities , Adult , Diagnosis , Female , Humans , Magnetic Resonance Imaging , Pregnancy , Prenatal Diagnosis , Spinal Diseases/diagnostic imagingABSTRACT
Objetivo: Determinar la sensibilidad y especificidad de la Ultrasonografía en el diagnóstico de Apendicitis Aguda en niños en el hospital Daniel Alcides Carrión en el año 2012. Materiales y Métodos: Es un estudio para validación de prueba diagnóstica, transversal, retrospectiva. Se revisaron las historias clínicas, informes de ecografía abdominal e informe de anatomía patológica de todos los pacientes pediátricos, menores de 15 años, que fueron intervenidos quirúrgicamente con el diagnóstico presuntivo de Apendicitis aguda por el servicio de Cirugía Pediátrica del Hospital Nacional Daniel Alcides Carrión. Resultados: Se estudiaron 105 pacientes, de los cuales 70 fueron varones (67 por ciento) y 35 mujeres (33 por ciento). La edad promedio fue de 9.3 años y el tiempo promedio de enfermedad fue de 38 horas. Se encontraron 94 pacientes con diagnóstico anatomopatológico de Apendicitis aguda y 10 con apéndice sin alteraciones, representando un 10 por ciento de error diagnóstico. Se encontró una Sensibilidad de 82 por ciento, Especificidad de 82 por ciento, Valor predictivo positivo de 97 por ciento y Valor predictivo negativo de 35 por ciento. Se evaluó cada hallazgo ecográfico patológico encontrándose que la especificidad y valor predictivo positivo para la visualización de estructura tubular en FID y presencia de hiperecogenicidad de la grasa mesentérica en FID fue de 100 por ciento respectivamente, y valor predictivo negativo de 14 y 15 por ciento respectivamente. Conclusiones: La ultrasonografía mostró una Sensibilidad y Especificidad de 82 por ciento de acuerdo a lo reportado en la literatura. La ultrasonografía presentó un alto valor predictivo positivo mostrando gran utilidad frente a hallazgos eco gráficos patológicos. La ultrasonografía presentó un bajo valor predictivo negativo mostrando poca utilidad frente a hallazgos eco gráficos negativos, por lo que el paciente debe ser evaluado dentro de un contexto clínico y laboratorial. Los hallazgos ecográficos...
Objective: Determine the sensitivity and specificity of the ultrasound on the diagnosis of Acute Appendicitis in children at Hospital Daniel Alcides Carrion during 2012. Materials and Methods: This is a diagnostic test validation study, transversal, retrospective. We reviewed clinical histories, ultrasound reports, and pathological reports of all the pediatric patients, under 15 years, who underwent surgery with the diagnostic of Appendicitis on the Pediatric Surgery Service of the Hospital Daniel Alcides Carrion. Results: We study 105 patients, 70 were male (67 per cent) and 35 were female (33 per cent). The mean age was 9.3 years and the mean sick time was 38 hours. We found 94 patients with the pathological diagnosis of acute appendicitis and 10 with a normal appendix, which represents a 10 per cent of diagnostic error. We found a Sensibility of 82 per cent, Specificity of 82 per cent, Positive predictive value of 97 per cent and Negative predictive value of 35 per cent. We evaluated each ultrasound feature and found that the visualization of the inflamed appendix and the hyperecogenicity of the mesenteric fat have a Specificity and Positive predictive value of 100 per cent. Conclusions: The ultrasound shows a Sensibility and Specificity of 82 per cent, as has been reported. The ultrasound shows a high Positive predictive value, which is very helpful when we have abnormal ultrasound features. The ultrasound shows a low Negative predictive value, so we have to be very careful with a normal ultrasound features, and evaluate the patient with his clinical history and laboratory data. The visualization of the inflamed appendix and hyperecogenicity of the mesenteric fat have the highest Positive predictive values and Specificity, 100 per cent.
