Ectopia Lentis with Retinal Detachment in a Patient with Marfan Syndrome: A Case Report
Devwrath Suneet Upasani, Sachin Daigavane, Swapneel Mathurkar
1. Junior Resident, Department of Ophthalmology, Datta Meghe Institute of Higher Education and Research, Wardha, Maharashtra, India.
2. Professor and Head, Department of Ophthalmology, Datta Meghe Institute of Higher Education and Research, Wardha, Maharashtra, India.
3. Assistant Professor, Department of Ophthalmology, Datta Meghe Institute of Higher Education and Research, Wardha, Maharashtra, India.
Correspondence Address :
Devwrath Suneet Upasani,
Raghobaji PG Hostel, AVBRH, Sawangi (Meghe), Wardha-442001, Maharashtra, India.
E-mail: devd.upasani@gmail.com
Abstract
Marfan syndrome is an autosomal dominant hereditary connective tissue disease caused by mutations in the Fibrillin-1 gene located on chromosome 15q15-21. Fibrillin is a specific type of glycoprotein widely distributed throughout the body, contributing to the elasticity and load-bearing capacity of connective tissue. Marfan syndrome is a multisystem disorder that affects the cardiovascular system, musculoskeletal system, and the eyes. Patients with Marfan syndrome can experience life-threatening complications such as aortic aneurysms, aortic dissection, and mitral valve prolapse. However, most patients initially present to an ophthalmologist with ocular symptoms, necessitating the ophthalmologist to diagnose and counsel the patient about the disease and its complications. A 23-year-old female patient presented to the Ophthalmology Outpatient Department (OPD) with complaints of diminished visual acuity in both eyes since birth. The patient exhibited cataractous changes in the lens with dislocation in the superotemporal quadrant in both eyes. Furthermore, the patient experienced rhegmatogenous retinal detachment in her left eye. The patient underwent scleral belt buckling and pars plana vitrectomy with silicone oil insertion in the left eye, as well as cataract extraction with Posterior Chamber Intraocular Lens (PCIOL) implantation in the right eye. Marfan syndrome can impact visual function in various ways. The present case report underscores the ocular manifestations of Marfan syndrome and the management of patients with developmental cataracts, subluxation, and retinal detachment due to high myopia.
Keywords
Cataract surgery, Connective tissue disorder, Dislocated lens, Myopia, Vitrectomy
DOI and Others
DOI: 10.7860/JCDR/2024/66942.19220
Date of Submission: Aug 11, 2023
Date of Peer Review: Nov 09, 2023
Date of Acceptance: Feb 10, 2024
Date of Publishing: Apr 01, 2024
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Aug 11, 2023
• Manual Googling: Nov 14, 2023
• iThenticate Software: Feb 08, 2024 (14%)
ETYMOLOGY: Author Origin
EMENDATIONS: 6