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Case Report

Successful Visual Rehabilitation in a Case of Anterior Megalophthalmos

Vidya Hegde, Rashmi Jain, Anupama Bappal

ABSTRACT Access this article online Website: We report a case of 40-year-old female who presented with diminution of vision in both eyes. www.meajo.org Ocular evaluation showed presence of bilateral megalocornea with deep anterior chamber, DOI: iridodonesis, cataract, and anterior embryotoxon. She was diagnosed with bilateral anterior 10.4103/0974-9233.102763 megalophthalmos. She underwent an uneventful cataract extraction with standard posterior Quick Response Code: chamber intraocular implantation of overall large diameter in the left eye. Zonular dialysis was not evident intraoperatively despite the presence of iridodonesis. Postoperatively the intraocular lens was well centered throughout follow up. This case report reviews this rare disorder and highlights successful visual rehabilitation.

Key words: Anterior Megalophthalmos, Cataract, Posterior Chamber Intraocular Lens

INTRODUCTION challenges in the management of cataract is placing the correct size of intraocular lens (IOL) in the capsular bag to prevent egalocornea is a nonprogressive bilateral congenital IOL decentration. Possible options would include use of a large enlargement of the anterior segment in the absence of custom-made posterior chamber (PC) IOL or use of anterior M 3,4 raised intraocular pressure.1 It can occur in three forms—simple chamber sutured IOL. This case report describes the use megalocornea unassociated with other ocular abnormalities; of standard posterior chamber IOL of larger diameter in the anterior megalophthalmos with megalocornea; and iris and angle management of cataract in anterior megalophthalmos. abnormalities as well as in infantile glaucoma. CASE REPORT Anterior megalophthalmos is a rare hereditary disorder characterized by presence of megalocornea (horizontal corneal A 40-year-old healthy woman presented with gradual progressive diameter more than 13 mm) in association with enlarged lens painless diminution of vision in both eyes for 1 year duration. iris diaphragm and ciliary ring.2 Secondary complications There was no relevant family history. On examination, her best include presence of iridodonesis, miosis, atrophy of iris stroma, corrected visual acuity (BCVA) OD was 6/18 and light perception and cataractous lens. Marfan’s syndrome, Apert syndrome, with projection, OS. Anterior segment examination in both eyes , and mucolipidosis type 2 are some systemic revealed presence of megalocornea [Figure 1] and clear associations seen with this condition. with mild pigment on endothelium. Anterior chamber were deep bilaterally. Iridodonesis with uveae was also Premature development of cataract is the most common cause present. The pupil of either eye could not be fully dilated by for visual impairment in this condition. Cataract extraction in pharmacological mydriasis. There was grade I nuclear sclerosis these patients is prone to complications due to presence of (by Lens Opacity Classification System II) OD and total cortical weak zonules and ciliary ring enlargement. One of the major cataract with phacodonesis OS. Biometric measurements were

Department of Ophthalmology, Yenepoya Medical College, Deralakatte, Mangalore, India Corresponding Author: Dr. Vidya Hegde, Department of Ophthalmology, Yenepoya Medical College, Deralakatte, Mangalore, India. E-mail: [email protected]

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Hegde, et al.: Anterior Megalophthalmos and Cataract

as shown in Table 1. Gonioscopy showed open angles with broad pigmented trabecular meshwork and anterior embryotoxon [Figure 2]. Intraocular pressure by applanation was 16 mmHg OD and 14 mmHg OS. The central corneal thickness (CCT) using ultrasound pachymetry was 0.445 mm OD and 0.465 mm OS. Fundus examination of the right eye was normal. B scan of the left eye was normal. Vitreous index was 63% OD and 66% OS. Based on the above findings, the patient was diagnosed with anterior megalophthalmos. Systemic examination was normal.

Manual small incision cataract surgery with use of capsular tension ring (CTR) with peribulbar block was planned for the left eye. IOL power was calculated with the SRK II formula. Preoperatively we were not able to assess the capsular bag diameter. Cataract surgery was uneventful in the left eye. Figure 1: Horizontal corneal diameter measured by using a Castroviejo calipers The zonular dialysis was not evident intraoperatively despite iridodonesis precluding the use of CTR. A single piece rigid Poly Methyl MethaAcrylate posterior chamber IOL of +15.00 D power, with 6.5 mm diameter optic and an overall diameter of 13.5 mm (Aurolab, Tamil Nadu, India) was implanted [Figure 3]. Follow up was performed at 1, 3, 4, 6, 12 weeks postoperatively. At 3 months postoperatively, BCVA in the left eye was 6/9 with +2.00 D sphere and +1.0 D cylinder at 90° and the PCIOL was well centered.

DISCUSSION

Anterior megalophthalmos is a rare inherited condition that is characterized by the presence of megalocornea, ciliary ring

enlargement and secondary effects of iridodonesis, miosis, Figure 2: Gonioscopy photograph showing presence of broad pigmented portion 5 atrophy of iris stroma, and occurrence of cataract. X-linked of trabecular meshwork genetic transmission is found in 50% of cases of anterior megalophthalmos (possibly located on Xq21.3-q22), autosomal transmission in 40%, and sporadic transmission in the remaining 10%. It is commonly seen in males (90%) because X-linked inheritance is common. Our case being a female, with a negative family history probably suggests a sporadic occurrence. She had megalocornea, deep anterior chamber, hypoplasia of iris stroma as pupils failed to fully dilate pharmacologically as well as anterior embryotoxon with presenile cataract fitting into the diagnosis of anterior megalophthalmos.

The lens in anterior megalophthalmos is of normal size,6 but the ciliary ring is enlarged, which is responsible for weakened zonules and subluxation of lens. One of the major challenges in the management of cataract in patients with anterior megalophthalmos is prevention of IOL decentration. Various techniques have been Figure 3: Per operative photograph after the intraocular lens implantation recommended including placement of iris clip IOL in the posterior chamber7 and custom IOL.8 Javadi et al.9 have reported safe In our case we were able to implant a standard IOL (13.5 mm implantation of standard IOL (13.4 mm in length with 7 mm optic) overall length with 6.5 mm optic) successfully. in six eyes without complications. Tsai et al.10 have also reported the use of standard IOLs (13 mm in length and 6 mm optic) in a case Introduced in 1991, CTRs have become indispensable in of anterior megalophthalmos who underwent phacoemulsification. stabilizing the capsular bag in the presence of generalized

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Hegde, et al.: Anterior Megalophthalmos and Cataract

Table 1: Biometric data of a patient with anterior REFERENCES megalophthalmos OD OS 1. Kraft SP, Judisch GF, Grayson DM. Megalocornea: A clinical Horizontal corneal 14 14 and echographic study of an autosomal dominant pedigree. diameter (Using Castroviejo J Pediatr Ophthalmol Strabismus 1984;21:190-3. calipers) (mm) 2. Duke-Elder S. Anomalies of the size of the cornea: Anterior Anterior Chamber depth by 4.84 4.92 megalophthalmos. In: Duke-Elder S, editor. Congenital A scan (mm) Keratometry (D) 45/47 x 90 44.25/46.75 x 90 deformities. Part 2. Normal and abnormal development. Axial Length by A scan (mm) 23.37 24.62 System of ophthalmology. vol 3. St Louis, MO: CV Mosby; Lens thickness by A scan (mm) 3.92 3.21 1964. p. 498-505. 3. Kwitko S, Belfort R Jr, Omi CA. Intraocular lens implantation in anterior megalophthalmus; Case report. Cornea 1991;10:539-41. 11 weakness or dehiscence of less than 150°. Hence, placement 4. Neumann AC. Anterior megalophthalmos and intraocular lens of conventional CTRs are not limited to only zonular dialysis implantation. Am Intra-Ocular Implant Soc J 1984;10:220-2. and can be used in generalized weakness of zonules. Even 5. Vail DT. Adult hereditary anterior megalophthalmos sine though there was phacodonesis, zonular dialysis was not glaucoma. A definite disease entity, with special reference evident and IOL implantation was possible in our case without to extraction of the cataract. Arch Ophthalmol 1931;6:39-62. the use of CTR. 6. Sharan S, Billson FA. Anterior megalophthalmos in a family with 3 female siblings. J Cataract Refract Surg 2005;31:1433-36. 7. Lee GA, Hann JV, Braga-Mele R. Phacoemulsification in anterior 12 Zare et al. have suggested preoperative use of ultrasound megalophthalmos. J Cataract Refract Surg 2006;32:1081-4. biomicroscopy (UBM) in anterior megalophthalmos to measure 8. Vaz FM, Osher RH. Cataract surgery and anterior 12 the actual size of the capsular bag. Zare et al. were able to megalophthalmos: Custom intraocular lens and special implant a standard foldable IOL as the capsular bag diameter in considerations. J Cataract Refract Surg 2007;33:2147-50. their case was found to be normal by ultrasound biomicroscopy. 9. Javadi MA, Jafarinasab MR, Mirdehghan SA. Cataract surgery The hyperopic shift could be explained with greater posterior and intraocular lens implantation in anterior megalophthalmos. shift of the IOL due to long zonules. In our case it was not J Cataract Refract Surg 2000;26:1687-90. possible to assess the capsular bag diameter, we assume that it 10. Tsai CK, Lai IC, Kuo HK, Teng MC, Fang PC. Anterior megalophthalmos. Chang Gung Med J 2005;28:191-5. would have been of normal size as there was no IOL decentration 11. Jacob S, Agarwal A, Agarwal A, Agarwal S, Patel N, Lal V. for the entire follow-up period. Efficacy of a capsular tension ring for phacoemulsification in eyes with zonular dialysis. Cataract Refract Surg 2003;29:315-21. Anterior megalophthalmos is a rare condition with a male 12. Zare MA, Eshraghi B, Kiarudi MY, Masoule EA. Application of preponderance. However, there is relative paucity of female ultrasound biomicro-scopy in the planning of cataract surgery in cases (such as the current case) in the literature. Presence of anterior megalophthalmos. Indian J Ophthalmol 2011;59:400-2. iris stromal atrophy and zonular dialysis can cause difficulties during cataract surgery. However, good visual rehabilitation can Cite this article as: Hegde V, Jain R, Bappal A. Successful visual rehabilitation be achieved with standard IOL in these anatomically challenged in a case of anterior megalophthalmos. Middle East Afr J Ophthalmol eyes, even in centers with limited or no access to investigative 2012;19:413-5. techniques such as UBM. Source of Support: Nil, Conflict of Interest: No.

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