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Pan Afr Med J ; 33: 312, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31692927


Congenital malformations of the aortic arches are a heterogeneous group of diseases associated with developmental disorders of the primitive branchial arches during fetal life. The coarctation of the aorta is a common congenital vascular malformation which is a congenital narrowing of the aortic isthmus, a segment of the aorta between the left subclavian artery and the ductus arteriosus. The interruption of the aortic arch is considered by some authors as an extreme coarctation of the aorta, characterized by discontinuity between ascending and descending aorta. These abnormalities are integrated, in most cases, in the context of cardiac malformations from which they are indivisible. CT angiogram plays an essential role in the examination of these abnormalities, their preoperative assessment and their follow-up in the long term. We conducted a retrospective study of 42 patients undergoing CT angiogram following the detection of heart disease on echocardiography. CT angiogram was performed in 6 cases. The average age of patients was 2 years, ranging from 6 days to 14 years; a male predominance was reported with a sex ratio of 1,6. The main diseases were: coarctation of the aorta: 18 cases; hypoplasies of the aortic arch: 8 cases; interruptions of the aortic arch: 7 cases; abnormalities of the aortic arches: 9 cases. Some of these abnormalities were associated. Extracardiac abnormalities associated with congenital heart diseases are relatively frequent; multislice scanner allows for good analysis of the cardiac afferent and efferent pathways. CT complements echocardiogram for pre-treatment assessment of the main malformative diseases, especially for the detection of the associated extra-cardiac vascular abnormalities, thanks to its satisfactory tridimensional multiplanar exploration. It tends to supplant angiography in many pathological malformations for several reasons: it is less invasive; it provides high-resolution 3D images useful to surgeons; it established the anatomical diagnosis, assesses tracheal compression and any associated malformation; it guides surgical treatment.

Aorta Torácica/diagnóstico por imagem , Coartação Aórtica/diagnóstico por imagem , Angiografia por Tomografia Computadorizada/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Adolescente , Aorta Torácica/anormalidades , Criança , Pré-Escolar , Ecocardiografia/métodos , Feminino , Humanos , Imagem Tridimensional/métodos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos
Pan Afr Med J ; 23: 38, 2016.
Artigo em Francês | MEDLINE | ID: mdl-27200143


The Williams-Beuren syndrome is a rare genetic disease. It combines classically specific facial dysmorphism, cardiovascular malformations and specific neuropsychological profile. We report three cases of Williams-Beuren syndrome in children with particular emphasis on vascular abnormalities observed on CT angiography and MR angiography.

Angiografia por Tomografia Computadorizada/métodos , Angiografia por Ressonância Magnética/métodos , Malformações Vasculares/etiologia , Síndrome de Williams/fisiopatologia , Adolescente , Pré-Escolar , Humanos , Masculino , Malformações Vasculares/diagnóstico por imagem , Síndrome de Williams/diagnóstico por imagem
Childs Nerv Syst ; 19(7-8): 555-60, 2003 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-12845458


INTRODUCTION: Diastematomyelia, also termed split cord malformation, is a form of occult spinal dysraphism characterized by a cleft in the spinal cord. Prenatal diagnosis of this anomaly is possible by ultrasonography (US), and fetal MRI can be used to diagnose the type of diastematomyelia precisely. Diastematomyelia can be isolated or associated with other dysraphisms, segmental anomalies of the vertebral bodies, or visceral malformations (horseshoe or ectopic kidney, utero-ovarian malformation, and anorectal malformation). We present three cases of fetal diastematomyelia investigated using a multimodal prenatal work-up (US, MRI, 3D-CT). CASES: The first case, detected at 20 weeks' gestation, had a lumbar meningocele. At 30 weeks' gestation, direct US visualization revealed the division of the spinal cord into two hemicords. This patient illustrates an isolated type II diastematomyelia with a favorable prognosis. The second case, detected at 22 weeks' gestation, presented with disorganization of bony process of the vertebral column with a midline echogenic bony spur, asymmetrical hemicords, and a foot malposition. Fetal MRI at 26 weeks' gestation and CT/3D reconstructed at 32 weeks' gestation confirmed a type I diastematomyelia with orthopedic malposition. The third case, detected at 22 weeks' gestation, presented with widening of the lumbar canal and scoliosis. Prenatal work-up (US, MRI) disclosed other visceral malformations (pelvic kidney), which led to the assumption of a complex polymalformative syndrome. The pregnancy was terminated. Fetopathologic examination disclosed even more visceral malformations (anal atresia and unicorn uterus).

Doenças Fetais/diagnóstico por imagem , Defeitos do Tubo Neural/diagnóstico por imagem , Diagnóstico Pré-Natal/métodos , Adulto , Feminino , Doenças Fetais/embriologia , Doenças Fetais/cirurgia , Feto , Idade Gestacional , Humanos , Imagem Tridimensional/instrumentação , Imagem Tridimensional/mortalidade , Recém-Nascido , Região Lombossacral/patologia , Imagem por Ressonância Magnética/instrumentação , Imagem por Ressonância Magnética/métodos , Meningocele/patologia , Defeitos do Tubo Neural/embriologia , Defeitos do Tubo Neural/cirurgia , Gravidez , Espinha Bífida Oculta/patologia , Medula Espinal/patologia , Ultrassonografia Pré-Natal/instrumentação , Ultrassonografia Pré-Natal/métodos