Megalocornea describes a condition in which the corneal diameter is larger than 13.0 mm and is associated with corresponding enlargement of the anterior segment of the eye.1 The anterior chamber is deep, the iris is atrophic (on transillumination) with pigment dispersion, and the lens might be dislocated or subluxated with early cataract.2 It is a bilateral, nonprogressive, and rare hereditary condition associated with an X-linked recessive trait; thus, 90% of cases affect men.3,4
Megalocornea can occur in isolation or associated with different ocular and systemic abnormalities, such as arcus lipoides, mosaic dystrophy, Marfan syndrome, albinism, and Neuhauser syndrome.5 The most common clinical findings involve the lens, the iris (iris atrophy), and the iridocorneal angle resulting in a condition that is also termed anterior megalophtalmos.6 The development of cataract in adulthood is frequent, and its management can be challenging.6,7 This is mainly because of the deep anterior chamber, enlarged ciliary ring, weakened zonular fibers, and large capsular bag.8 The capsular bag can be very elastic, as seen in children, making capsulorhexis difficult to perform.9 The crystalline lens can also be luxated or subluxated.7 All these elements make cataract surgery and intraocular lens (IOL) implantation in the bag difficult, requiring careful selection of the type and size of the IOL.
We report 6 eyes with megalocornea in 3 brothers in which cataract surgery was performed and 2 techniques of IOL implantation were used.
The operations were performed by the same surgeon (H.S.D.) using general anesthesia. In the eldest brother, a posterior chamber (PC) IOL (SK21RU in right eye and CZ70BD in left eye, both Alcon Laboratories, Inc.) was implanted in each eye. The IOLs were placed in the bag through a superior stepped corneal incision and sutured to the iris in planned extracapsular cataract extraction (ECCE). In the second and third brothers (4 eyes), after uneventful standard phacoemulsification, an aphakia anterior chamber iris-fixated IOL (Verisyse, Abbott Medical Optics, Inc. in Case 2 and Artisan, Ophtec BV in Case 3) was inserted through a superior 5.5 mm sclerocorneal tunnel and a peripheral iridectomy performed at the 12 o'clock position. Table 1 shows the biometry, IOL choices, and surgical techniques. In all cases, the incisions were closed with 10–0 nylon sutures. The extracapsular wound was closed with a running stitch and the phacoemulsification wounds with a single cross stich. The conjunctiva was advanced to cover the wound and sutured to episclera with 2 interrupted sutures and buried knots. Postoperatively, chloramphenicol 0.5% and dexamethasone 0.1% preservative-free drops, both 4 times a day for 4 weeks, were given.
A 38-year-old British man with anterior megalophthalmos was referred to our clinic reporting loss of vision in the left eye that had rapidly worsened over the past few weeks. The corrected distance visual acuity (CDVA) was 6/9 in the right eye and hand motion (HM) in the left eye. The refraction was −2.75 −2.50 × 20 in the right eye and −4.25 −2.50 × 145 in the left eye. A slitlamp examination showed an intumescent lens (Figure 1, A) and a crocodile shagreen (Figure 1, B) atrophy of the iris (Figure 1, C) in the left eye and Krukenberg spindle (Figure 1, D) in both eyes. Intraocular pressures (IOPs) were normal, and there was no iridodonesis or phakodonesis. Ultrasound biomicroscopy showed the choroid and retina to be flat; however, the lens diameter could not be accurately measured. The white-to-white diameter was 17.00 mm. Extracapsular cataract extraction was performed, and an iris-sutured PC IOL with a fixation hole in the haptics was implanted in the bag (Figure 2, A). The desired overall length of the IOL was 17.5 mm and such an IOL was not available in the market (maximum diameter available, 13.5 mm) for human use. Dog IOLs of 17.5 mm were available; however, the lowest power was 40.0 diopters (D) and thus they were not considered suitable. The iris-clip IOL was also not available at that time. Two months postoperatively, the CDVA was 6/6 with a refraction of +1.00 −4.00 × 170.
At this point, the vision in the right eye deteriorated rapidly to counting fingers. Extracapsular cataract extraction was performed, and an iris-sutured PC IOL with 4 eyelets in the optic was implanted in the bag (Figure 2, B and C) (Table 1). Two months postoperatively, the CDVA was 6/5 with a refractive error of +0.50 −2.00 × 35. Eleven years postoperatively, the patient returned with loss of vision in the right eye. There was no history of trauma. The IOL was subluxated, and the CDVA was 6/60. The suture had cut through the iris. The subluxated IOL was explanted, and a +9.5 D aphakia iris-clip IOL was implanted (Figure 2, D). The postoperative CDVA was restored to 6/6, and the final refraction in the right eye was +2.00 −1.75 × 28.
The middle brother, at the age of 44 years, was referred to our department with a family history of anterior megalophthalmos. Slitlamp examination showed an enlarged corneal diameter in both eyes, and Krukenberg spindle, posterior mosaic corneal dystrophy, iris transillumination defects, and mild cataract in the right eye. The CDVA was 6/9, and the patient was not symptomatic. However, by 3 months later, the CDVA in the right eye had rapidly dropped to HM. The refraction was +4.00 −0.75 × 15 in the right eye and +3.00 −0.50 × 160 in the left eye. Phacoemulsification with IOL implantation was planned for the right eye; however, at assessment (4 months later) the cataractous lens was dislocated into the vitreous chamber. A pars plana vitrectomy with lensectomy was performed by the vitreoretinal team and the eye was left aphakic. Ten months later, an aphakia iris-clip IOL was implanted through a 5.5 mm sclerocorneal incision (Figure 3, A). At the 1-month follow-up, the CDVA was 6/6 and the refraction was +2.75 −1.25 × 20.
One year later, nuclear cataract was detected in the left eye. The CDVA was 6/18, and the patient was symptomatic. Phacoemulsification with aphakia iris-clip IOL implantation was performed. At the 2-month follow-up, the CDVA was 6/6 with a refraction of +3.00 −0.75 × 160. Six years after the first IOL implantation, the patient returned reporting discomfort and blurred vision in the right eye. The examination showed mild sectorial (temporal) stromal corneal edema with some superficial punctate keratitis and loss of enclavation of the anterior chamber IOL (AC IOL) at the 9 o'clock position. The CDVA was 6/12. The iris-clip IOL was reenclavated (Figure 3, B). The corneal edema settled, and the CDVA was restored to 6/6.
At the age of 55 years, the youngest brother presented to our department with decreased vision, mainly in the right eye. As in his brothers, anterior segment examination showed an enlarged corneal diameter, atrophy of the stromal iris, and Krukenberg spindle with normal IOPs. Bilateral nuclear cataract was detected more in the right eye than in the left eye. The CDVA was 6/36 in the right eye and 6/9 in the left eye. Refraction was −2.50 −2.00 × 84 and −1.50 −2.00 × 93, respectively. Phacoemulsification with implantation of an aphakia iris-clip IOL was performed (Figure 3, C). After 1 week, the CDVA was 6/9 in the right eye. At the 1-month follow-up, the CDVA in the right eye was 6/5 with a refractive error of +1.25 D. A similar technique was used in the left eye (Figure 3, D), and the postoperative CDVA at the 1-month follow-up was 6/5 with a refractive error of +1.25 −1.25 × 180. This was maintained at the 6-month follow-up visit.
Patients affected by megalophthalmos are prone to develop early nuclear sclerotic cataract.7 Because of the challenges in performing the cataract surgery in these eyes, different surgical approaches have been tried.8 Of them, standard PC IOL implantation,10 a PC IOL using the anterior optic capture technique (optic within the capsulorhexis margin, haptics in the sulcus),11 implantation of a retropupillary6 or anterior chamber12 iris-clip aphakic IOL, and an iris-sutured acrylic PC IOL1,13 are the most described options.
The most important consideration is the size of the IOL. Standard PC IOLs and AC IOLs are most likely to decenter or luxate. As described in our previous study1 (Case 1), we implanted an iris-sutured PC IOL in both eyes after ECCE in 1 brother, with complete visual recovery. Complications such as IOL decentration, inflammation, or ocular hypertension were not detected in the left eye over a 19-year follow-up. However, 11 years after the surgery a subluxation occurred in the right eye and the PC IOL was replaced with an iris-clip AC IOL.
In the other 2 brothers, we opted for iris-clip AC IOLs (4 eyes), which had become readily available. The technique is well known to be effective and reasonably easy to perform but can be associated with raised IOP and endothelial cell loss.14,15 However, in megalocornea the anterior chamber depth is greater than normal; thus, the risk for endothelial cell loss by intraoperative manipulation or endothelial touch is low. Another important problem is the spontaneous disenclavation of 1 haptic16 or both haptics.17 Loss of enclavation of 1 haptic can occur spontaneously; however, disenclavation of both haptics is usually seen after postoperative trauma. In 1 eye of in Case 2, temporal disenclavation occurred 6 years after surgery with no apparent associated trauma. Reenclavation was successfully performed with no further complications over a 6-year follow-up. Enclavation of a generous fold of iris is recommended to avoid spontaneous disenclavation. However, over time iris atrophy, in particular at the site of enclavation, is common.16 In anterior megalophthalmos, this would be more likely because of the preexisting iris atrophy. Despite this complication, the iris-clip IOL appears to be the best choice for this condition. We used the iris-suture technique only because at that time, iris-clip IOLs were not a common choice.
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Disclosures:None of the authors has a financial or proprietary interest in any material or method mentioned.