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Purpose: We report a patient with severe multi-organ dysfunction of unknown origin who presented with bilateral orbital and chorioretinal manifestations that led to the diagnosis of Erdheim-Chester Disease (ECD). Observations: ECD is a rare, histiocytic, proliferative disorder characterized by multi-systemic organ involvement that has historically lacked effective therapy. Our patient underwent genetic testing that was positive for the BRAF V600E mutation; therefore, the patient was treated with vemurafenib. Conclusions and importance: This case demonstrates the rare orbital and intraocular manifestations of ECD and the unfortunate impact of a delayed diagnosis, the importance of early gene therapy testing for management decisions, and the utilization of targeted directed therapy to improve visual outcomes and quality of life. Keywords: Histiocytosis, Vemurafenib, Orbital, Chorioretinal, Xanthomatous, Lipogranulomatous, BRAF

The purpose of this article is to report a case of upper eyelid mechanical ptosis caused by silicone oil migration in a Jordanian patient after pars plana vitrectomy and intraocular silicone oil injection. A 20-year-old male patient was referred to the oculoplastic clinic for the management of a left upper eyelid swelling and complete ptosis that developed 12 years after pars plana vitrectomy and silicone oil placement for retinal detachment secondary to perforating eye injury. Eye examination showed upper eyelid swelling and a complete ptosis induced by the weight of inflammatory tissue and silicone oil, as proved by histopathologic examination. We presumed that silicone oil had leaked through the pars plana vitrectomy ports or through a subtle traumatic perforation of the posterior segment, which might have been aggravated by increased intra-ocular pressure postoperatively. In conclusion, mechanical ptosis caused by silicone oil migration after retinal detachment surgery is rare and this is one of the few reports in the literature.