An on-table diagnosis of brittle cornea syndrome

Session Details

Session Title: Presented Poster Session: Cornea: Surgical

Venue: Poster Village: Pod 3

First Author: : R.Solanki INDIA

Co Author(s): :                           

Abstract Details

Purpose:

To describe a rare case of Brittle Cornea Syndrome (BCS) which was diagnosed while operating on the patient in the operating theatre thereby confirming the spectrum of preoperative suspicious clinical findings revealed during examination in an outpatient department.

Setting:

Aravind Eye Hospital, Pondicherry India. Cornea and Refractive Surgery Services

Methods:

36 Year male Born to parents with non consanguineous marriage presented with painful sudden onset Loss of vision OD after an alleged minor finger nail trauma. Ocular Examination revealed OD visual acuity of Perception of Light(PL) positive, projection of rays(PR) accurate, full thickness linear corneal tear inferiorly and a flat chamber. Documented blue sclera OD on one of his previous visits. OS no light perception, previous childhood trauma and Pthysis Bulbi. General Examination revealed mental retardation, Short stubby nose, use of Hearing Aid, mild contractures of fingers (5th finger) clinodactyly, Small joint hypermobility and apparent Dentinogenesis Imperfecta like features.

Results:

A Corneal tear repair under local peribulbar anesthesia was undertaken. An additional side port entry was planned and made which spontaneously extended into a larger linear tear than the expected size. Subsequent cheese wiring of sutures and difficulty in sealing the wound under air and viscoelastic agents helped us make an on table diagnosis of Brittle Cornea Syndrome while operating on the patient. Eventually wound closure with air tamponade (c3f8 was not available as a first choice agent ) was done, anterior chamber was formed and patient was put on systemic carbonic anhydrase inhibitors.

Conclusions:

In BCS with corneal rupture, primary repair is required. Surgical repair of spontaneous corneal ruptures in brittle cornea syndrome is difficult. Corneal sealing can be achieved by a combination of cheese wiring sutures with bandage contact lens, pressure patches and systemic carbonic anhydrase inhibitors. Gas(C3F8)/air tamponade, tissue adhesives in dry operative field, viscoelastic agents and onlay epikeratoplasty can also be used. Prevention is always better than cure and thus BCS is a rare condition that is important to recognize, in order to permit appropriate management, including avoidance of misattribution of corneal damage to non-accidental injury, and to facilitate genetic counselling.

Financial Disclosure:

None