Prominent protuberances: A peep over corneal striae.

A 4-year-old male child presented with a recent history of diminution of vision, pain, and redness in both eyes (BE). He was treated for allergic conjunctivitis with topical steroids since past 2 years.

On examination, uncorrected visual acuity was 20/120 and 20/200 in the right (RE) and left eye (LE), respectively. SLE of BE revealed conjunctival congestion and upper tarsal papillae. Corneal findings of microcystic epithelial and stromal edema (LE > RE) were documented along with horizontal tracks and retro-corneal pigmented bands. Fundus showed near-total cupping in BE. IOP in BE was 32 mm Hg.

What is your next step?

Instillation of topical steroids

Control IOP with anti-glaucoma medications

Start topical hypertonic saline

Perform Pentacam in both eyes.

Correct Answer: B.

Diagnosis: Congenital Glaucoma.

Findings

The child was born full-term Cesarian section, with uneventful natal and developmental history. His presentation was bilaterally asymmetric [Fig. 1a and c]. The keratometry values did not show any corneal steepening. Anterior segment optical coherence tomography (ASOCT) of RE showed hyperreflective Descemet’s membrane (DM) with prominent endothelial protuberances [Fig. 1b and d] and left eye revealed localized stromal thickening with endothelial protuberances. Patient vision and IOP improved on starting anti-glaucoma medications.

Figure 1

(a) Anterior segment slit-lamp photograph of the right eye showing corneal edema under diffuse illumination. (b) High-resolution cross-sectional image (AS-OCT) of the right eye showing prominent protuberances (red arrow). (c) Anterior segment slit-lamp photograph of the left eye showing nasal corneal edema with horizontal tracks under diffuse illumination. (d) High-resolution cross-sectional image (AS-OCT) of the left eye showing localized stromal thickening with protuberance (red arrow)

Discussion

We enumerate here various clinically resembling differentials of corneal band-like structures. Haab’s striae seen in congenital glaucoma are torn DM’s edges that are thickened and curled with their intervening area being thin and smooth. Posterior polymorphous corneal dystrophy (PPCD) presents with thickened DM having scalloped and irregular edges. Due to dystrophic endothelial cell changes, PPCD can be associated with characteristic vesicles. Traumatic DM rupture, seen commonly with forceps birth delivery, can be secondarily associated with corneal edema. Interestingly such striaes are oriented vertically or obliquely vis-a-vis horizontal striaes of congenital glaucoma. In severe keratectic eyes, aqueous seepage through torn DM into stroma leads to rolled DM edges and marked corneal edema.[1] In this case, ancillary diagnostic modality like ASOCT added to the dilemma and challenge as it revealed hyperreflective DM along with protuberances in both congenital glaucoma and PPCD.[23]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

The authors have not declared a specific grant for this research from any funding agency in the public, commercial, or not-for-profit sectors.

Conflicts of interest

There are no conflicts of interest.