Brittle cornea syndrome: current perspectives [Letter]

Dear editor

I have read with great interest the review article by Walkden et al on “Brittle Cornea Syndrome: Current Perspectives”.¹ I would like to express my appreciation to the authors for an elaborate and comprehensive review of this complex disease which has devastating complications. I agree with the authors on various surgical techniques to manage the eyes with brittle cornea syndrome (BCS).

I have managed a few children with BCS in our hospital with spontaneous corneal ruptures after a trivial trauma. Based on our experience, I would like to add a few surgical tips in managing a corneal tear in patients with BCS.

The configuration of the corneal tear can be irregular usually resembling a globe rupture. It is preferable to perform the surgery under general anesthesia rather than local anesthesia. It is difficult to take bites into the corneal tissue using a suture needle as it can result in cheese wiring of the cornea and this can lead to extension of the tear. Hence, utmost care must be taken while titrating the strength of the knot. It is better to take full thickness bites on to the cornea than partial thickness as it can lead to further tearing of the cornea. Always keep a cyanoacrylate tissue adhesive as a standby as it can be used to close the minor leaks. It is advisable to keep the knot of the suture unburied, as the very act of burying can lead to further extension of the tear. Also, the knots which are exposed externally will induce more inflammation and lead to faster healing of the wound. Postoperative topical steroid drops must be tapered off very quickly to induce more inflammation and cause faster healing. Sutures must be kept for reasonably longer time than routine cases.

While removing the sutures after adequate healing, it is safe to remove them in the operating room. This gives the surgeon ample scope of handling any complications like wound rupture and if required resuturing during suture removal. The child is advised not to rub the eyes and to wear protective goggles all the time. Contact lens should not be prescribed in the postoperative period as a simple fingernail touch can lead to globe rupture.