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Ophthalmic Images
June 13, 2019

Lensectomy Vitrectomy for Ectopia Lentis and Rhegmatogenous Retinal Detachment in a Patient With Marfan Syndrome

Author Affiliations
  • 1Beijing Tongren Eye Center, Beijing Tongren Hospital, Beijing Ophthalmology & Visual Science Key Lab, Beijing Institute of Ophthalmology, Capital Medical University, Beijing, China
JAMA Ophthalmol. 2019;137(6):e185426. doi:10.1001/jamaophthalmol.2018.5426

A 33-year-old man with Marfan syndrome (the patient had a pathogenic variant c.649T>C/p. W217R in FBN1 gene) presented with declined vision after a fist injury to his left eye. Ocular color Doppler ultrasonography and fundus examination results revealed a vitreous opacity, dislocation of the lens, and retinal detachment. Pars plana lensectomy vitrectomy was performed and during the operation the dislocated lens was found on the optic nerve, first appearing as a pearl (Figure, A) and then as a “golden apple” (Figure, B) when impaled between the vitrector and light pipe. Retinal reattachment was achieved after deuteroxide injection, endophotocoagulation, air/fluid exchange, and silicone oil tamponade.

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