Descolamento regmatogênico de retina na síndrome de Wyburn-Mason: relato de caso / Rhegmatogenous retinal detachment in Wyburn-Mason syndrome: case report

Wyburn-Mason is a rare vascular disorder, comprised of arteriovenous malformations (AVMs) of the midbrain and retina. It can cause visual symptoms depending on its localization and extension. Vitreous and intraretinal hemorrhage and neovascular glaucoma have been previously described. A case of rhegmatogenous retinal detachment in a patient with Wyburn-Mason syndrome is described. A 27 year-old woman previously diagnosed with Wyburn-Mason syndrome, sought attendance with sudden low vision in right eye 3 months before. She presented moderate vitreous hemorrhage and retinal detachment with a superior tear. She underwent a successful posterior vitrectomy with implantation of silicone oil, with reattachment of the retina. Rhegmatogenous retinal detachment in a patient with Wyburn-Mason syndrome has been not previously described in the literature. Vitrectomy in this case present challenges related to intraoperatory bleeding risk, to a posterior pole tear among AVMs and the difficulty of obtaining free retina for photocoagulation.

A síndrome de Wyburn-Mason é uma desordem vascular rara, caracterizada por malformações arteriovenosas (MAV) do mesencéfalo e retina. Pode causar sintomas visuais por sua localização e extensão. Hemorragia vítrea, intrarretiniana e glaucoma neovascular já foram descritos. Descreve-se um caso de descolamento regmatogênico de retina em uma paciente com síndrome de Wyburn-Mason. Uma paciente feminina de 27 anos com diagnóstico prévio de síndrome de Wyburn-Mason procurou atendimento com queixa de baixa acuidade visual súbita no olho direito há três meses. Apresentava hemorragia vítrea moderada e descolamento de retina com rotura superior. Foi submetida a vitrectomia posterior com implante intravítreo de óleo de silicone, com reaplicação da retina. Descolamento regmatogênico de retina em paciente com síndrome de Wyburn-Mason não havia sido previamente descrito na literatura. A vitrectomia nesse caso apresentou dificuldades relacionadas ao risco de sangramento intraoperatório, à presença de rotura no polo posterior entre as MAVs e à dificuldade de obtenção de retina livre para fotocoagulação.

Premacular haemorrhage associated with arteriovenous communications of the retina induced by a valsalva-like mechanism: an observational case report.

A 26-year-old woman presented with sudden defective vision in the right eye following lifting a heavy bucket of water. Examination showed a dense premacular subhyaloid haemorrhage associated with arteriovenous communications of the retina (AVCRs). Spontaneous absorption of the premacular haemorrhage with consequent improvement in the visual acuity was seen after two months. The possible aetiopathogenesis of the case is also discussed.

Unusual retinal manifestation in a combination of Sturge-Weber and Klipplel-Trenaunay syndrome--a case report.

An unusual retinal manifestation of arteriovenous communications is reported in a case showing a combination of Sturge-Weber and Kippel-Trenaunay syndromes. The arteriovenous communications are thought to be similar to those seen in the limbs in Parkes-Weber syndrome. This retinal finding indicates that the three syndromes are intricately related to each other and may be an incomplete manifestation of a single pathophysiologic entity which may be named neurocutaneous angiomatosis.

Congenital papillary venous ring

A 31-year-old woman had an extremely rare congenital anomaly of a venous circle on her right optic disc. All of the nasal and some of the temporal retinal veins drained into this ring, which itself drained into the central retinal vein. Fluorescein angiography revealed no disturbance of vascular structure. The eye was otherwise normal with 6/6 [20/20] visual acuity and full field of vision