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2739: severe anterior staphyloma

Surgical image of a severe anterior staphyloma in a patient with Axenfeld-Rieger syndrome and osteogenesis imperfecta, highlighting thinned sclera.

There are unusual cases which require incredible surgical skill such as this complex case of a young patient with Axenfeld-Rieger syndrome and osteogenesis imperfecta. Our guest surgeon is Professor Ramesh Ayyala (who is both a corneal specialist and a glaucoma specialist) doing an incredible surgical repair. If you are an ophthalmology resident who wants to have an incredible fellowship in complex anterior segment surgery, then look carefully into USF Tampa.

Surgical management of anterior staphyloma and scleral thinning in patients with osteogenesis imperfecta presents significant challenges due to the extreme fragility of ocular tissues. These patients often exhibit pronounced thinning of the sclera and cornea, increasing the risk of globe rupture and complicating surgical intervention. When staphyloma progresses and threatens vision or structural integrity, surgical reinforcement is considered. This typically involves tectonic grafting using preserved donor sclera, pericardium, or the patient’s own tissue to reinforce the weakened area. Careful dissection and minimal manipulation are essential to avoid further tissue damage. In this case, a full-thickness corneal transplant was also necessary. Postoperative healing may be compromised, and long-term follow-up is critical due to the potential for graft dehiscence or progressive thinning. Although outcomes vary, timely intervention can preserve globe integrity and restore sight. Multidisciplinary care is essential, given the systemic nature of osteogenesis imperfecta and its impact on healing.

video link here

https://youtu.be/NVZbLMgBfs0

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