“Shark Jaws” Sign in Macular Hole

A 67-year-old female presented with blurred vision in left eye for 1 month. Best corrected visual acuity was 20/200. Slit lamp evaluation showed pseudophakia, and the rest was unremarkable. Fundus evaluation revealed tiny retinal defect at 6 o’clock at midpoint between foveola and inferior margin of yellow ring at macula along with drusen (Fig. 1). Swept source optical coherence tomography through defect showed full-thickness macular hole with vitreo-macular traction and cone-shaped structure (CSS) adherent to opened roof (Fig. 2A). Adjacent to “defect”, multiple CSSs corresponding to adjacent intact ellipsoid zone were seen adherent to intact external limiting membrane (ELM) (Fig. 2B). There was also the presence of hyper-reflective deposits on retinal pigment epithelium (RPE) (Fig. 2B) along with patchy disorganization of outer retinal layers (Fig. 2C). Pattern of multiple CSSs adherent to ELM with hyper-reflective deposits on RPE resembled a “shark jaws” appearance.FIGURE 1: Color fundus image of the left eye showing tiny eccentric retinal defect (black arrow) within yellow ring (white arrow) at macula.FIGURE 2: Swept Source Optical Coherence Tomography scans of the left eye showing (A) Full thickness macular hole with vitreo-macular traction (yellow arrow), a cone-shaped structure (CSS) (white arrow) under open roof (white arrowhead). (B) Multiple CSSs (white arrow) adherent to intact external limiting membrane (yellow arrow) and hyper-reflective deposits on retinal pigment epithelium (white arrowhead) in a scan adjacent to retinal defect, giving an appearance of “shark jaws” (inset). (C) Patchy disorganization of outer retinal layers (white arrows).In macular hole, CSS comprising cone photoreceptors and hyper reflective deposits on RPE (remnants of photoreceptors outer segments) have been described independently.1,2 Multiple CSSs adherent to ELM with hyper-reflective deposits on RPE have not been described in same eye. Above findings along with patchy disorganization of outer retinal layers may be related to prefoveolar cortical vitreous traction with degenerative changes.1