Short Reports Lensectomy, vitrectomy, and transvitreal ciliary body photocoagulation as primary treatment for glaucoma in microspherophakia Neha Goel, MS, FRCS (Glasg), Ravi Sharma, MBBS, Amrita Sawhney, MBBS, DNB, Madhullika Mandal, MS, and Reena M. Choudhry, MD, FRCS (Glasg) Microspherophakia is a rare, bilateral developmental anomaly of the crystalline lens. It can occur in isolation or as a component of a familial disorder. It has been associated with the Weill- Marchesani syndrome and Marfan syndrome. Angle clousure glaucoma can occur in microspherophakia and is the primary cause of visual loss. We describe the management of 2 sisters with bilateral microspherophakia and advanced angle closure glaucoma. Case Report T wo sisters, aged 7 (patient 1) and 10 years (patient 2), presented to the outpatient department of ICARE eye hospital and postgraduate institute with complaints of gradual progressive diminution of vision in both eyes since early childhood. They were the product of a nonconsanguineous marriage. Both girls were of normal height, normal skeletal proportions, normal intel- lect, with no abnormalities of the skeletal or cardiovascular system. Complete examination of the parents was normal. Patient 1 had alternating exotropia of about 60 D . Best- corrected visual acuity was counting fingers close to the face, with accurate projection of rays in both eyes. Slit- lamp examination revealed bilateral clear corneas, shallow anterior chambers, marked iridodonesis and phacodonesis. After pupillary dilatation, the entire lens equator was visible within the pupil; there was an increased curvature of the anterior aspect of the lenses, suggestive of micro- spherophakia (Figure 1). The zonules were stretched but unbroken. Fundus evaluation revealed a pale optic disk, with a cup:disk ratio of 0.9:1 and a thinned neuroretinal rim bilaterally. The horizontal and vertical dimensions of both corneas were 11.50 mm and 11 mm respectively. Intraocular pressure (IOP) measured by Goldmann appla- nation tonometry was 36 mm Hg in the right eye and 38 mm Hg in the left eye. Indentation gonioscopy showed bilateral closed angles, with peripheral anterior synechiae (PAS). Ultrasonic A-scan biometry revealed an axial length of 26.11 in the right eye and 25.26 in the left eye. Patient 2 was orthophoric, with best-corrected visual acuity of counting fingers at .5 meters, with accurate projec- tion of rays in both eyes. Bilateral microspherophakia with pale optic disks and near total cupping was noted. The hor- izontal and vertical dimensions of both corneas were 11.75 mm and 11.5 mm, respectively. IOP by Goldmann applana- tion tonometry was 32 mm Hg in the right eye and 30 mm Hg in the left eye. Indentation gonioscopy showed bilateral closed angles with PAS. Axial length calculated by A-scan biometry was 28.64 in the right eye and 29.22 in the left eye. With written informed consent of the parents, both eyes of both patients underwent 23-gauge pars plana lensec- tomy and vitrectomy with transvitreal endocyclophotocoa- gulation (TVCP) under general aaesthesia, at 2 week intervals. After lensectomy and vitrectomy, a 23-gauge diode endolaser (DC-3300, Nidek, Japan) was used along- with scleral indentation by a cotton-tipped applicator and transpupillary visualization to apply laser directly to 270  of pars plicata until the entire length of the ciliary tips turned white and shrinkage of the processes was achieved (Figure 2). Typical laser parameters used were 200 mW and continuous duration. Postoperative treatment included topical antibiotic ste- roid with cycloplegic eyedrops. The IOP in both eyes ranged between 12–16 mm Hg in patient 1 and 14–16 mm Hg in patient 2. No complications occurred and no ex- tremes of IOP ( \8 mm Hg or .22 mm Hg) were noted at any time. At 1 month’s follow-up, best-corrected visual acu- ity improved to counting fingers at 3 meters in both eyes in patient 1 and 20/200 in both eyes in patient 2. IOP was be- tween 12–14 mm Hg without any antiglaucoma medica- tions. This was maintained through 1 year of follow-up. Discussion The term microspherophakia describes a lens with a decreased equatorial diameter and an increased anteropos- terior diameter, making it relatively spherical. Clinically, it is recognized when the equator of the whole lens is visible after full mydriasis. 1 Ocular involvement in microsphero- phakia includes subluxation or dislocation of the lens, progressive myopia, defective accommodation, glaucoma, and retinal detachment. 2 Glaucoma is the predominant sight-threatening compli- cation of microspherophakia and may be present in up to 51% of eyes. 3 Although acute rise in IOP can occur due to pupillary block by the spherical lens or by subluxation or dislocation of the lens in anterior chamber, chronic IOP elevation has been attributed to PAS formation and irreversible trabecular damage due to recurrent, unrelieved pupillary block, or a developmental anomaly of the angle. 3,4 Author affiliations: ICARE Eye Hospital and Postgraduate Institute, NOIDA, UP, India. Submitted October 1, 2014. Revision accepted February 14, 2015. Correspondence: Neha Goel, MS, FRCS (Glasg), 57, Sadar apartments, Mayur Vihar Phase 1 Extension, New Delhi – 110091, India (email: nehadoc@hotmail.com). J AAPOS 2015;19:366-368. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.02.008 366 Journal of AAPOS