Butter like brittle cornea.

An 11-year-old girl presented with a corneal tear in her left eye (LE) of one day's duration, following a slap on her cheek by her brother while playing. There was no direct trauma to the eye. On examination, visual acuity in the right eye (RE) was 6/6 and that of LE was hand movements. Slit lamp examination of both eyes revealed a blue sclera and extreme corneal thinning from limbus to limbus without any obvious ectasia [Figs. 1a and b, 2b]. The LE showed full thickness corneal tear [Fig. 2a]. Topography of the RE showed a pachymetry of 350 μm without any ectasia [Fig. 2c]. The child's parents gave a history of consanguineous marriage. The child suffered from hearing disability as well as small joint laxity. The clinical diagnosis was brittle cornea syndrome (BCS). The child underwent corneal tear repair under general anaesthesia. During suturing there was 'cheese wiring' of the cornea due to the needle track. Full thickness bites of the cornea were taken to prevent further tearing of the cornea. Cyanoacrylate tissue adhesive was used at the end to close the minor leaks. Suture knots were left unburied, as the very act of burying can lead to further extension of the tear and externally exposed knots induce more inflammation, leading to faster healing of the wound along with a quick taper of postoperative topical steroid drops. After 8 weeks the visual acuity improved to 20/80 with an well apposed wound [Fig. 1b].

Figure 1

(a) Diffuse slitlamp examination of left eye showing a large 10 mm irregular ‘J’ shaped corneal tear from limbus to limbus with iris prolapse and flat anterior chamber. (b) Postoperative diffuse slitlamp image showing sutured corneal tear with tissue adhesive

Figure 2

(a) Right eye showing bluish discoloration of the sclera. (b) Slit section of the right eye showing a thin cornea from limbus to limbus. (c) Topography of the right eye showing a thin pachymetry and normal keratometric values

BCS is a rare genetic, autosomal recessive, connective tissue disease with typical ocular, auditory, musculoskeletal, and cutaneous disorders.[1] The most common ophthalmic findings include keratoconus, progressive central corneal thinning, high myopia, irregular astigmatism, retinal detachment, and high risk for spontaneous corneal or scleral rupture.[2] Cornea in BCS is unable to withstand normal biomechanical stresses and hence cannot maintain their structural integrity and is susceptible to spontaneous corneal tear.[3]

In conclusion spontaneous corneal ruptures can happen in Brittle Cornea Syndrome. These eyes are at a life time risk of corneal tears even due to trivial injuries. Also, suturing the cornea is very challenging and can lead to further extension of the tear.

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