The authors describe two brothers with Marfan syndrome who developed severe complications after iris-suture fixation of an intraocular lens (IOL). A secondary iris cyst developed in one and the IOL dislocated into the vitreous in the other, requiring pars plana vitrectomy and IOL removal. Although the visual outcome was good in both cases, severe complications of iris-suture fixated IOLs must be considered when surgical correction of aphakia is attempted in children with Marfan syndrome.
From the Department of Ophthalmology, Charité-Universitätsmedizin Berlin, Berlin, Germany (DJS); and the Department of Pathology, Yale University School of Medicine, New Haven, Connecticut (JS).
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Daniel J. Salchow, MD, Department of Ophthalmology, Charité-Universitätsmedizin Berlin, Campus Virchow Klinikum, Augustenburger Platz 1, 13353 Berlin, Germany. E-mail: firstname.lastname@example.org
Received: November 08, 2012
Accepted: January 22, 2013
Posted Online: February 26, 2013
Correction of aphakia in patients with Marfan syndrome may be challenging. Although contact lenses or spectacles can be used to correct aphakia in most cases, suture fixation of an intraocular lens (IOL) to ocular structures such as the sclera or the iris may need to be considered in select cases.
The older of two brothers presented at age 5 years with bilateral lens subluxation in the superonasal direction, requiring lensectomy. Aphakia was recommended along with optical correction. The father had Marfan syndrome and had undergone lensectomy in childhood. Remembering contact lens manipulations as traumatic, he insisted that an intraocular lens (IOL) be implanted in his son and could not be convinced otherwise.
Uncomplicated lensectomy with iris-suture (10-0 polypropylene; Ethicon, Somerville, NJ) fixated posterior chamber IOL (three-piece hydrophobic acrylic, MA60AC; Alcon Laboratories, Inc., Fort Worth, TX) was performed in the left eye and 1 year later in the right eye, as described previously.1 Nine months later, the patient presented for follow-up care. He did not have any complaints; best-corrected visual acuity (BCVA) was 20/30 in both eyes. Intraocular pressure (IOP) was normal by palpation bilaterally. Slit-lamp examination showed a well-positioned iris-sutured IOL in each eye. In the superior anterior chamber of the right eye a translucent, non-mobile cyst (3 mm in diameter) was noted over the suture site. IOP was normal, the overlying cornea was clear, and dilated fundus examination was unremarkable.
Eight months later, visual acuity was stable but the cyst had enlarged to approximately 4.5 mm and a small concretion was seen inside the cyst (Figure 1). Because of the growth, surgical treatment was performed. Using a 27-gauge needle with a tuberculin syringe attached, the cyst was entered at its base from superiorly 1 mm behind the limbus. The fluid was aspirated to collapse the cyst. A small amount (less than 0.1 cc) of ethanol 96% was injected into the cyst and irrigated out after 30 seconds using balanced salt solution. The cyst was everted through a corneal limbal incision at its base; it was excised and examined histologically. The vitrectomy hand-piece was used to shave the cyst from the iris. Postoperative recovery was uncomplicated and BCVA remained stable. Three months later, the cyst had recurred, although it was smaller in size (1.5 mm in diameter). No secondary complications were noted.
Figure 1. Color photograph showing an iris cyst in the right eye of case 1, 17 months after lensectomy and iris-suture fixated posterior chamber intraocular lens implantation.
The cyst was received in the collapsed state. It was lined by non-keratinizing stratified squamous epithelium without pigmentation (Figure 2). No keratin or goblet cells were seen, and signs of inflammation were absent.
Figure 2. Light micrograph of the cyst wall, excised from the right eye of case 1. There is epithelial lining of the wall with a continuous layer of non-keratinizing stratified squamous epithelium, signs of inflammation are absent (hematoxylin–eosin; original magnification ×400).
The younger brother presented at the age of 5 years with nasally subluxated lenses in both eyes. BCVA was 20/100 bilaterally. Lensectomy and iris-suture (10-0 polypropylene; Ethicon) fixated posterior chamber IOL (MA60AC; Alcon Laboratories, Inc.) implantation was performed in both eyes, several weeks apart. The postoperative course was uneventful and BCVA improved to 20/40 bilaterally.
Fourteen months later, the patient presented with decreased vision in the left eye. Visual acuity with habitual correction was 20/600, and the IOL had dislocated into the vitreous. The patient and his family denied any history of trauma. Using 23-gauge instruments, a pars plana vitrectomy was performed. The IOL was brought forward into the anterior chamber and removed from the eye, which was left aphakic. Postoperatively, the patient recovered well and was fitted with a contact lens and glasses. BCVA improved to 20/50 (refraction: +14.25 −0.75 × 180) four months after IOL explantation. No secondary complications such as increased IOP, retinal detachment, or corneal de-compensation developed.
Correction of aphakia in patients with Marfan syndrome can be challenging because of the lack of capsular support. Contact lenses and glasses remain the standard approach, but may not be optimal for all patients. Although different techniques for IOL implantation in the absence of capsular support have been described, no method has been shown to be superior to the others.2 In a cohort of patients with Marfan syndrome, Yeung and Weissman found that 70% of eyes developed some form of complication of contact lens wear.3 The physician may therefore consider implanting an IOL in select patients. Implantation of a posterior chamber IOL into the ciliary sulcus with transscleral suture fixation is a widely used technique. However, IOL dislocation secondary to suture breakage is a recognized late complication. Buckley reported that 3 of 26 children developed spontaneous dislocation of transs-clerally sutured IOLs 3.5 to 9 years after surgery.4 Asadi and Kheirkhah noted suture breakage in 6 of 25 eyes of children 7 to 10 years after implantation.5 It has been suggested that 9-0 polypropylene should be used instead of 10-0 polypropylene to lower the risk for suture breakage, but we are not aware of clinical studies on this topic.
Iris-claw IOLs are another way to correct aphakia in the absence of capsular support. Aspiotis et al. reported good visual results in adults and children with Marfan syndrome6; all 7 eyes of 5 patients had improved BCVA. Corneal decompensation, raised IOP, and irregular pupils were not observed, but one eye developed a retinal detachment 3 months after surgery. In the United States, iris-claw IOLs are currently not approved to treat aphakia in children. Although it has been used more widely in Europe, large series with long-term follow-up are lacking. Sminia et al. reported their results in two children with Marfan syndrome who had an iris-claw IOL implanted in both eyes.7 Comparing endothelial cell counts from their patients with published normative data and their own control database, the authors found that after more than 12 years the endothelial cell density was within the normal range. These encouraging findings warrant confirmation in larger long-term studies.
Suturing a posterior chamber IOL to the back surface of the iris has also been reported in children.1,8,9 Kopel et al. found good visual outcomes in a cohort children with ectopia lentis, but reported IOL dislocation in 4 of 12 eyes.1 Yen et al. found dislocation of iris-suture fixated IOLs in 5 of 17 eyes; dislocation was more common in patients with Marfan syndrome (45%) than in patients with traumatic or surgical aphakia (0%).9 Because some patients with Marfan syndrome have an increased risk for retinal detachment, surgical manipulation of the vitreous should be minimized.
Wong and Salchow reported an iris cyst after iris-suture fixated IOL in a boy with Marfan syndrome,10 and we provide follow-up and histology on this case. Primary iris cysts develop spontaneously and usually involve the pigmented neuroepithelium. Histologic evaluation showed that in our patient the cyst wall consisted of non-keratinizing squamous epithelium, it is therefore a secondary iris cyst. The presumptive origin of secondary iris cysts is corneal or conjunctival surface epithelium introduced by surgical or non-surgical trauma. The treatment of secondary iris cysts may be difficult, recurrences are common, and corneal decompensation, raised IOP, and inflammation can complicate the course.11 Repeated cyst aspiration has been recommended as a less invasive treatment for secondary iris cysts.
Iris-suture fixation of IOLs in children is an effective procedure to correct aphakia, but postoperative complications are relatively common and may be severe.
- Kopel AC, Carvounis PE, Hamill MB, Weikert MP, Holz ER. Iris-sutured intraocular lenses for ectopia lentis in children. J Cataract Refract Surg. 2008;34:596–600 doi:10.1016/j.jcrs.2007.11.044 [CrossRef] .
- Wagoner MD, Cox TA, Ariyasu RG, Jacobs DS, Karp CL. Intraocular lens implantation in the absence of capsular support: a report by the American Academy of Ophthalmology. Ophthalmology. 2003;110:840–859 doi:10.1016/S0161-6420(02)02000-6 [CrossRef] .
- Yeung KK, Weissman BA. Contact lens correction of patients with Marfan syndrome. J Am Optom Assoc. 1997;68:367–372.
- Buckley EG. Hanging by a thread: the long-term efficacy and safety of transscleral sutured intraocular lenses in children (an American Ophthalmological Society thesis). Trans Am Ophthalmol Soc. 2007;105:294–311.
- Asadi R, Kheirkhah A. Long-term results of scleral fixation of posterior chamber intraocular lenses in children. Ophthalmology. 2008;115:67–72 doi:10.1016/j.ophtha.2007.02.018 [CrossRef] .
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- Shields JA, Shields CL, Lois N, Mercado G. Iris cysts in children: classification, incidence, and management. The 1998 Torrence A Makley Jr Lecture. Br J Ophthalmol. 1999;83:334–338 doi:10.1136/bjo.83.3.334 [CrossRef] .