Bilateral Crowning of Lens Equator with Secondary Angle Closure Glaucoma in a Middle-Aged Female with Microspherophakia

Abstract

Microspherophakia is a rare congenital anomaly characterised by an increased anteroposterior thickness of the lens associated with a reduced equatorial diameter. The primary pathology lies in the development of zonules. Glaucoma is a serious complication associated with this disorder. We report a rare instance of bilateral crowning of the lens equator with secondary angle closure glaucoma in a middle-aged female with microspherophakia.

CASE REPORT

A 42 years old young female, a known case of glaucoma on dorzolamide eye drops two times a day in both eyes (BE) since 1 year, presented to us with the best corrected visual acuity (BCVA) of 6/6P with -8 Diopter spherical in the right eye (RE) and 6/9 with -9.5 Diopter spherical in the left eye (LE). The intra-ocular pressure was 20 mmHg in BE. Slit lamp examination BE revealed a variable depth of the anterior chamber much shallower inferiorly with patent peripheral iridotomy and 360⁰ occludable angles [Figure 1a-b]. The patient was accidentally dilated following which crowning of the lens in the pupillary margin was noted in BE [Figure 1c, d and 2a, b]. Ultrasound biomicroscopy revealed a decreased anterior chamber depth of 1.43 mm and 1.59 mm and an increased lens thickness of 4.69 mm and 4.80 mm in RE and LE, respectively, with stretched zonules in all the quadrants [Figure 3a, 3b]. Fundus examination showed 0.65 cup disc ratio and 0.85 in RE and LE, respectively. Systemic examination was unremarkable. Owing to the advanced optic nerve damage in LE, antiglaucoma medication was stepped up to Misopt eyedrops (dorzolamide hydrochloride 2% + timolol maleate 0.5% BD) in BE. In view of very shallow anterior chambers, the limbal approach was avoided and was planned for sequential pars plana vitrectomy (PPV) + pars plana lensectomy (PPL) + scleral fixation of intra-ocular lens (SFIOL). Unfortunately, the patient lost to follow-up.

Figure 1: (a-d): Slit lamp examination of BE showing a variable depth of the anterior chamber much shallower inferiorly and crowning of the lens equator in the pupillary margin (a-b). Retro-illumination images of BE showing lens equator in front of the pupillary margin inferiorly with pigment dispersion behind the posterior capsule (c-d)

Figure 2: (a-b): Gonioscopic photo of the inferior angles in BE showing completely occludable angles with an anteriorly subluxated lens

Figure 3: (a-b): Ultrasound biomicroscopy of BE showing crowded angle structures (white arrow), a decreased anterior chamber depth (green line) of 1.43 mm and 1.59 mm, and an increased lens thickness (yellow line) of 4.69 mm and 4.80 mm in RE and LE, respectively, with stretched zonules (arrow heads)

DISCUSSION

Microspherophakia is a rare but potentially blinding disease with a variety of associated systemic syndromes and varied clinical presentations.[¹] The appropriate lens surgery and intra-ocular lens (IOL) fixation technique have to be selected, keeping in mind the progressive nature of the zonulopathy.[²] In a prospective, non-randomised, interventional case series by Yang et al.,[³] patients with spherophakia and secondary glaucoma were split into two groups and they underwent either phacoemulsification + CTR + IOL or PPL + SFIOL. The authors found that both techniques were effective in lowering the intra-ocular pressure (IOP) and improving visual acuity over a follow-up period of 3 years. The authors also reported the need for medical/surgical control of IOP in the long term after successful lensectomy and IOL implantation.[³] Lensectomy alone cannot be effective if there is significant PAS or angle anomaly. Early identification of the disease, timely visual rehabilitation, and appropriate management of the lens and glaucoma can help us prevent blindness from this condition.[²]

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.