Ciliary processes and ciliary sulcus hypoplasia in Marfan syndrome

Introduction: Lens dislocation is a major ophthalmological complication of Marfan syndrome. However, to our knowledge, no previous study has reported direct observation of the ciliary sulcus in living eyes of patients with Marfan syndrome. Therefore, in this study, we report hypoplasia of the ciliary process and sulcus observed using intraocular endoscopy in patients with Marfan syndrome and lens dislocation. Patient and Clinical Findings: We performed the ciliary sulcus suture fixation of intraocular lens in both eyes of 3 cases with Marfan syndrome and lens dislocation. The ciliary sulcus observed using an intraocular endoscope revealed hypoplasia of the ciliary process in all 3 cases. Therefore, no ciliary sulcus was formed. Diagnosis, Intervention, and Outcomes: Lens luxation was caused by rupture of the zonules of Zinn, most likely caused by hypoplasia of the ciliary process. Hypoplasia of the ciliary processes has been suggested to cause hypoplasia or excessive elongation of the zonules of Zinn, leading to their rupture. Conclusions: Anatomical hypoplasia of the ciliary process was recognized in Marfan syndrome, possibly leading to zonal rupture and lens dislocation. Ciliary process hypoplasia may be associated with Marfan syndrome.