GLOBE RUPTURE REVEALING OSTEOGENISIS IMPERFECTA

Published 2022 - 26th ESCRS Winter Meeting

Reference: P177 | Type: Abstracts | DOI: 10.82333/x2zk-p503

Authors: Imad Messafi* ¹ , Siham CHARIBA ¹ , Asmae MAADANE ¹ , Rachid SEKHSOUKH ¹

¹Ophthalmology,Mohammed VI University Hospital,Oujda,Morocco

Purpose

Osteogenesis imperfecta (OI) is a connective tissue disorder resulting from abnormal collagen type1 . It is characterized by different clinical manifestations including skeleton, ears, eyes, teeth, skin and joints. Ocular features of OI are various , from which we site megalocornea, keratoconus , keratoglobus, blue sclera, spontaneous globe rupture, primary optic atrophy, papilledema, congenital glaucoma, cataracts, dislocated lens and choroidal sclerosis .Treatment is essentially based on prevention of traumas, physical aids and surgery

Setting

ophtalmology departement, Mohammed VI university hospital, Oujda, morocco

Methods

we report a case of spontaneous globe rupture revealing OI in young woman

Results

A 31-old women was admitted for painful left eye occurring spontaneously. Her medical history showed congenital deafness. Examination : light perception, circular corneo-limbal wound from 5h to 12h with choroid protrusion, a keratoglobus in the right eye with central opacity, which made corneal topography difficult .We noticed that patient had joints laxity, scoliosis and bone deformities related to multiple traumas, confirmed by radiographies. The mother confirmed that “blue sclera “presence at birth. We concluded to a brittle cornea syndrome overlapping with OI. Visual acuity was still limited to light perception and she was addressed to ENT and orthopaedics specialists.

Conclusion

We report a unique case of osteogenisis imperfecta overlapping with brittle cornea syndrome, causing a spontaneous globe rupture occurring in a fragility zone of a thinned cornea. The diagnosis was essentially made by discovering keratoglobus, then linking it to the other systemic manifestations. Our case enlightens the importance of the early recognition of OI, in order to prevent ocular and extra-ocular complications and provide a better quality of life to these patients.