PURPOSE: To describe a case of spontaneous retrobulbar hemorrhage in type IV Ehlers-Danlos syndrome, a disorder characterized by vascular fragility and associated severe bleeding problems. METHODS: Observational case report. Spontaneous unilateral retrobulbar hemorrhage in a 22-year-old man with type IV Ehlers-Danlos syndrome was documented by clinical and radiographic examination. RESULTS: The retrobulbar hemorrhage was self-limited and resolved without visual sequelae. CONCLUSION: Spontaneous retrobulbar hemorrhage should be recognized as part of the spectrum of type IV Ehlers-Danlos syndrome, and clinicians must be alert to diagnosis and treatment of this vision-threatening problem. (Am J Ophthalmol 2002;133:422-424. (C) 2002 by Elsevier Science Inc. All rights reserved).
机构:Assistance Publique – Hôpitaux de Paris,Centre de référence pour la maladie de Fabry et les maladies héréditaires du tissu conjonctif (syndromes d'Ehlers