The opportunistic masquerader microspherophakia

An 8-year-old girl presented with a history of loss of vision and pain in the left eye (LE) associated with watering and redness, allegedly after sustaining some sort of trauma while playing few months back. The parents also reported that the child had decreased vision. The visual acuity in the right eye (RE) was finger counting at 5 m and perception of light (PL) with accurate projection of rays (PR) in the LE. The examination of LE revealed congested conjunctiva and diffuse corneal edema [Figure 1]. The anterior chamber was faintly visible showing the presence of a dislocated lens. The pupil was faintly visible, dilated, and nonreacting. The intraocular pressure was 20 mmHg in the RE and 35 mmHg in the LE. B-scan in the LE revealed no posterior segment abnormality. The autorefractometer reading of the RE was − 22.75 D sphere /−8.00 D Cylinder at 28°. The RE on dilated examination showed the presence of small, globular lens with equator visible within the pupillary border suggestive of microspherophakia [Figure 2]. Detailed systemic examination did not reveal any abnormality linking to any syndrome. Hence, the final diagnosis of isolated microspherophakia in both eyes with secondary glaucoma, and corneal decompensation due to anteriorly dislocated lens in the LE was made and managed accordingly.Figure 1: External appearance of the left eye showing corneal edemaFigure 2: Slit lamp image of the right eye showing small, globular lens with equator visible within the pupillary borderDISCUSSION Microspherophakia is a rare congenital anomaly characterized by the abnormal spherical shape of the crystalline lens. There is an increased anteroposterior thickness of the lens associated with reduced equatorial diameter.[1] It can be found in systemic or ocular conditions, such as Marfan’s syndrome, Weill–Marchesani syndrome, Iridocorneal endothelial syndrome, and Axenfeld–Rieger syndrome.[2] It can be associated with complications secondary to the abnormal shape of the lens resulting in high myopia, subluxation, or even complete anterior or posterior dislocation. The anterior subluxation can lead to secondary glaucoma, corneo-lenticular touch causing endothelial cell loss or endothelial decompensation[3] and can masquerade these conditions. It is thus imperative to do a detailed examination of the other eye in cases of unilateral atypical presentations because it may lead you to open up a Pandora box! 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 Nil. Conflicts of interest There are no conflicts of interest.