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Timely intervention of spontaneous scleral melt in Marfan syndrome
Nibha Mishra1, Priyanshi Priya2, Niraj Kumar Yadav2
1Department of Ophthalmology, King George's Medical University, Lucknow, Uttar Pradesh, India drnibhamishra@gmail.com.
Abstract:
A young man in his late adolescence presented with a gradually progressive diminution of vision in both eyes for 3 years. A general physical examination revealed features suggestive of Marfan syndrome, with a positive family history. Ocular examination revealed a superonasal lens subluxation with a myopic fundus in both eyes. The patient underwent pars plana lensectomy with anterior vitrectomy in the right eye followed by the left eye. The right eye surgery was uneventful, and the patient was left aphakic. On the first postoperative day, the left eye developed hypotony. By the second day, spontaneous scleral melt was noted posterior to the surgical wound with severe hypotony and loss of globe architecture. The patient was taken up for emergency surgery with an amniotic membrane graft over the area of scleral melt and was started on oral ciclosporin (100 mg). Postoperatively, the patient's globe stabilised. After 1 week, the patient's vision increased to 6/24 with normal intraocular pressure.
Insights
Marfan syndrome patients undergoing lens surgery may face rare complications like scleral melt. Prompt intervention with amniotic membrane grafts and immunosuppressants can stabilize the globe and improve vision.
Area of Science:
- Ophthalmology
- Genetics
- Surgical Complications
Background:
- Marfan syndrome is a genetic connective tissue disorder often associated with ocular abnormalities, including lens subluxation.
- Progressive vision loss is a common concern in patients with Marfan syndrome due to lens and refractive errors.
Purpose of the Study:
- To report a rare case of spontaneous scleral melt following pars plana lensectomy in a patient with Marfan syndrome.
- To describe the management and outcome of this severe ocular complication.
Main Methods:
- Pars plana lensectomy with anterior vitrectomy was performed on both eyes.
- Emergency surgery involving an amniotic membrane graft and oral ciclosporin was administered for scleral melt and hypotony in the left eye.
Main Results:
- The right eye surgery was uneventful, resulting in aphakia.
- The left eye developed hypotony and spontaneous scleral melt postoperatively, leading to loss of globe architecture.
- The emergency surgical intervention stabilized the left eye, with vision improving to 6/24 and normal intraocular pressure within one week.
Conclusions:
- Scleral melt is a potentially devastating but treatable complication after ocular surgery in Marfan syndrome patients.
- A multidisciplinary approach combining surgical repair and immunosuppressive therapy can salvage the globe and restore visual function.
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