Síndrome de Bonnet-Wybur-Mason: dos polos de un espectro / The Bonnet-Wyburn-Mason syndrome: two poles of one spectrum

Se comunican los casos de dos pacientes con síndrome de Bonnet, Wyburn-Mason en quienes existía un aneurisma cirsoide de la retina de diferente grado de desarrollo. En uno, el cuadro oftalmoscópico era obvio; en tanto que en el otro la manisfestación fundamental era una tortuosidad vascular acentuada y en quien solo la angiografía fluoresceínica del fondo ocular mostró sutiles cambios compatibles con una malformación arteriovenosa localizada. Otro elemento inusual en el comportamiento de este tipo de malformaciones fue la asociación a una coartación de la aorta torácica y multiples anomalías esqueléticas sencillas, así como la obstrucción de un segmento muy desarrollado de la malformación arteriovenosa con infarto hemorrágico retiniano e involución posterior de parte de ella

We communicate the cases of two patients with syndrome of Bonnet, Wyburn-Mason who had cirsoide aneurysms of the retina of differnt degrees of development. In one, the ophthalmoscopic picture was obvious, while on the other, The primary manifestation was a marked vascular tortuosity and in which only the ocular fundus fluorescein angiography showed subtle changes consistent with a located arteriovenous malformation. Another unusual element in the behavior of this type of malformations was a coarctation of the thoracic aorta and multiple skeletal anomalies simple associated, as well as the obstruction of a highly developed portion of the retinal arteriovenous malformation with hemorrhagic infarction and consecutive involution of part of it

Cirsoid aneurysm of the scalp: case report / Aneurisma cirsoide do couro cabeludo: relato de caso

We report a case of a 23-year-old man presented with a painful swelling over his right occipital region. Selective right carotid angiogram demonstrated a markedly tortuous and dilated right occipital artery feeding into a circular or cirsoid aneurysm in the right occipital scalp. Percutaneous occlusion of the cirsoid aneurysm was not attempted and the patient underwent a successful resection of the fistula with good outcome after three months followed up.

Relatamos um caso de paciente masculino de 23 anos com lesão pulsatile em região occipital direita. Angiografia digital seletiva da artéria occipital direita mostrou artéria occipital direita dilatada e tortuosa formando aneurisma cirsoide do couro cabeludo. Oclusão percutânea não era possível e o paciente foi submetido à ressecção cirúrgica da fístula com sucesso. O seguimento em três meses mostrou boa recuperação.