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Year : 2020  |  Volume : 58  |  Issue : 3  |  Page : 203-205

A rare case of stickler marshall syndrome

Cataract, Cornea, External Disease and Refractive Services, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Puducherry, India

Correspondence Address:
Dr. Kirandeep Kaur
Fellow Pediatric Ophthalmology and Squint Services, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Puducherry - 605 007
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_29_20

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A 35 year old female presented to us with sudden painless diminution of vision in the right eye (RE) since 10 days. There was no history of trauma. She gave systemic history of hearing loss since 4 years of age and also complained of joint pains on and off for which she was taking medical treatment. Best corrected visual acuity in the RE was Hand movements (HM+) and in the left eye (LE) was 20/20 with -5D spherical correction. Anterior segment examination in the RE revealed horizontal jerky nystagmus, typical complete inferonasal iris coloboma with aphakia in primary gaze(Figure 1). On examining the eye in downgaze on slit lamp, heterogenous lenticular opacity could be seen in the anterior vitreous phase suggestive of spontaneously dislocated crystalline lens in the vitreous cavity(Figure 2). LE anterior segments was within normal limits. Fundoscopy of the RE revealed Type 4 Retinochoroidal(RC) coloboma (Ida Mann's classification) with posterior vitreous detachment (PVD) and LE fundus was normal. Ultrasound B scan of the RE revealed dislocated crystalline lens in the vitreous cavity with PVD and RC coloboma thus confirming the findings(Figure 3,4,5). The patient was planned for pars plana vitrectomy with lensectomy in the RE.

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