Bilateral transient pupil closure after iris supported intraocular lens implantation in a case with Marfan syndrome.

A 16-year-old woman presented with Marfan syndrome and bilateral ectopia lentis. The surgical treatment including removal of the crystalline lens and implantation of an iris-supported intraocular lens in both eyes at a week interval. Postoperatively, the biomicroscopic examination showed total pupil closure bilaterally. After the topical tropicamide treatment, the pupil returned to normal shape.

INTRODUCTION

Ectopia lentis can be congenital, acquired, isolated, or associated with systemic abnormalities such as Marfan syndrome, homocystinuria, Weill-Marchesani syndrome, hyperlysinemia, sulfite oxidase deficiency, and Ehlers Danlos syndrome.1–3

Several surgical techniques have been described for ectopia lentis in children. Intracapsular and extracapsular extraction of the lens with anterior or posterior IOL implantation and pars plana lensectomy with primary and secondary IOL implantation are the current treatment options.14

However, difficulties in these cases may result in perioperative and postoperative complications such as crystalline lens dislocation, intraocular lens (IOL) decentration, cystoid macular edema, uveitis, and retinal detachment.4

We present a case report of transient bilateral pupil closure following crystalline lens extraction and iris-fixated intraocular lens implantation in a patient with Marfan syndrome.

CASE REPORT

A 16-year-old female presented with Marfan syndrome. The best-corrected visual acuity (BCVA) was 0.3 (decimal notation) in the right eye and 0.3 in the left eye. Slit-lamp examination revealed an bilateral inferior dislocation of the crystalline lens [Figure 1]. Intraocular pressure was 15 mmHg in the right eye and 13 mmHg in the left eye. Dilated fundus examination was normal bilaterally. Surgery was planned with a week interval between eyes. The patient was fully informed about the nature of the procedure, its complications, and a written consent was obtained.

Figure 1

Preoperative photograph of the left eye showing the subluxated crystalline lens

Preoperatively, mydriasis was achieved with topical tropicamide (Tropamid®, Bilim, Istanbul, Turkey) and phenylephrine 2.5% (Mydfrine®, Alcon, USA). The patient underwent surgery under general anesthesia. Two paracenteses were placed at 10 o’clock and 2 o’clock. The anterior chamber was filled with sodium chondroitin sulfate 4%, sodium hyaluronate 3%, and sodium hyaluronate 1% (DuoVisc®, Alcon Inc., Fort Worth, TX, USA). A small capsulotomy was created with a 27-gauge needle. The lens material and the complete capsular bag were removed by anterior vitrectomy. A 6.0 mm corneal incision was placed at the 12 o’clock position. Carbachol intraocular solution (Miostat®, Alcon Inc., Fort Worth, TX, USA) was injected into the anterior chamber to constrict the pupil. An iris-supported aphakic IOL (Verisyse®, AMO Inc., Abbott Park, IL, USA) was implanted in the anterior chamber with forceps and fixated to the iris with enclavation needles. A peripheral iridotomy was performed at the 12 o’clock position. The corneal wound was closed with 10-0 nylon sutures, and the viscoelastic material was aspirated. At the end of the surgery the pupils were round and 2-3 mm in diameter [Figure 2]. Subconjunctival injection of gentamycin (1%) and dexamethasone (0.4%) were delivered at the end of surgery.

Figure 2

Photograph of the left eye at the end of the surgery

At 1 day postoperatively the BCVA was light perception in both eyes. Slit-lamp examination showed total pupil closure in both eyes [Figure 3a]. The intraocular pressure (IOP) was 14 mmHg in the right eye and 16 mmHg in the left eye. B-scan ultrasonography was normal in both eyes. At this visit, topical tropicamid was instilled at 5-minute intervals three times. Six hours later, the BCVA improved to 0.5 in the right eye and 0.4 in the left eye and the pupil shape was normal bilaterally [Figure 3b]. At 1 week postoperatively, the pupil shape was normal bilaterally.

Figure 3a

The appearance of the left eye 1 day postoperatively

Figure 3b

Photograph of the left eye after tropicamide therapy showing the normal pupil shape

DISCUSSION

Marfan syndrome is a connective tissue disorder that is caused by mutations in the fibrillin gene. Fibrillin is widely distributed in ocular connective tissues such as iris, ciliary body, and ciliary processes.5 Ocular abnormalities of Marfan syndrome include subluxation of the crystalline lens, extreme myopia, and astigmatism.13

Subluxated clear crystalline lenses are one of the most surgically challenging cases. In recent years, crystalline lens extraction with an aphakic iris-supported IOL implantation in cases with subluxated lenses has become the favoured procedure.36 In our case, the lens and capsular bag was removed with a vitrector and an iris enclavated aphakic IOL was implanted. However, the most frequent complications following iris-fixated IOL implantation are an irregular pupil, damage to the endothelial cells, transient corneal edema, transient IOP elevation, cataract formation, and IOL decentration.78 Lifshitz et al.3 reported four eyes with subluxated crystalline lenses and successfully implanted Artisan (Ophtec BV, the Netherlands) aphakic IOL. Three of the eyes required intraoperative anterior vitrectomy and no complications such as irregular pupil were observed postoperatively.3

Irregular pupil occurs in 0.4% to 1.2% of similar case postoperatively. This complication also can occur secondarily to chronic inflammation, atrophic iris changes, asymmetric enclavation, or dislocation of iris-fixated IOLs.3910 In the current case, we did not observe inflammation, asymmetric enclavation or IOL dislocation. We believe that this pupil closure was caused by excessive mechanical effects of the iris-supported IOL in the more flaccid iris tissue, related to Marfan syndrome. Although there is no support in literature, patients with Marfan syndrome may have floppy iris especially after anterior vitrectomy. Presumably, after implantation of an iris enclavated aphakic IOL, an excessive and chronic traction of the haptic at the midperipheral iris might induce iris retraction and pupil closure. Alternately, the effect of the carbachol on the iris could have caused pupil closure. Further investigation on iris elasticity in similar cases is required.

In conclusion, pupil changes can occur following iris-supported intraocular lens implantation. These changes can be excessive in special conditions such as Marfan syndrome due to characteristics of the iris tissue. Therefore cases with Marfan syndrome should be carefully monitored in terms of pupil changes after crystalline lens extraction and iris enclavated aphakic IOL implantation.