Accepted indications for surgical treatment include luxation of the lens into the anterior chamber or into the vitreous, progressive subluxation accompanied by worsening visual acuity or uncorrectable refractive changes, and prevention of amblyopia.

In the past, according to the patient’s age and the surgeon’s experience, lensectomy was performed using a limbal or pars plana access or by vitrectomy—leaving the patient aphakic [37, 38]. In 1979, it was Peyman et al. who reported on 32 cases of lens subluxation and dislocation due to several causes, two of which were secondary to Marfan syndrome [39]. The lens was removed by a vitrectomy using a pars plana access, achieving significant visual improvement.

In 1996, Halpert et al. presented a group of 59 eyes that underwent pars plana lensectomy combined with anterior vitrectomy [40]. The longterm follow-up results were encouraging: 84% reached a postoperative visual acuity better than 20/40. Retinal detachment occurred in 1 out of 18 eyes, which completed more than 11 years of follow-up. More recently, the same group published 38 eyes with hereditary lens subluxation (22 of them due to Marfan syndrome) that underwent within-the-bag lensectomy with similar results [41].

Shortt et al. showed pars plana lensectomy to be safe in the management of ectopia lentis in children [42]. Seventy-seven percent reached a visual acuity of 6/9 and better. Thirteen out of 24 cases were secondary to Marfan syndrome.

The issue of refractive correction of aphakia is another challenging problem in Marfan patients—especially in children for preventing amblyopia. Glasses or contact lenses can present a nonsurgical solution, but aniseikonia, intolerance, and noncompliance frequently limit their use. In those cases, secondary intraocular lens (IOL) placement can be chosen. Today, secondary IOL implantation is not the method of choice, although it still presents an option in prepuberty and high myopia. Hence, primary implantation of an IOL is desirable and has become the accepted treatment.

In cases of secondary IOL placement or in eyes without adequate capsular support, an anterior

chamber IOL, scleral-fixated IOL, or iris-fixated posterior chamber IOL can be inserted.

Aspiotis described a series of seven eyes with subluxated lens due to Marfan syndrome that underwent lensectomy, anterior vitrectomy, and implantation of Artisan intraocular lens to the anterior chamber [43]. He achieved good refractive correction; all patients reached a visual acuity better than 20/40. Minimal complications, nonsignificant endothelial cell loss, and easy implantation were reported.

A review of the literature could not state the superiority of one lens type or fixation site in the capsular bag or ciliary sulcus [44].

Retinal Detachment

Retinal detachment is the most serious ocular complication in Marfan syndrome. Marfan patients are predisposed to develop retinal detachment; the incidence lies between 8% and 25.6% [23, 45]. It is more common in the younger age, with an average age of 22 years.

Clinical Findings

As discussed previously, the changes in eyes of patients with Marfan syndrome resemble myopic changes. Retinal holes and tears are frequent findings. The prevalence of giant retinal tears is significantly increased compared to the general population [46]. Retinal detachment is estimated to occur in about 10% of Marfan patients (Fig. 20.3). In the presence of a dislocated lens, the prevalence is significantly higher [23]. The rate of bilateral retinal detachment varies between different reports from 17.7% to 69% [47, 48]. Sharma et al. described anatomic findings of 53 eyes with retinal detachment in Marfan patients and their surgical results: 71% had associated ectopia lentis; in three-thirds, retinal detachment was complete; and in half of the cases, proliferative vitreoretinopathy was present. The vast majority of retinal breaks were found in the temporal half.

Poor mydriasis, lens opacities, and lens dislocation can impair visualization and make retinal examination difficult [49].

Fig. 20.3 A 15-year-old boy with high myopia, Marfan syndrome, and macula-off retinal detachment. Visual acuity was 20/200 (Courtesy of Manuel Diaz-LLopis, M.D. and Roberto Gallego-Pinazo, M.D.)

Pathogenesis

Myopia and increased axial length are evident risk factors for developing retinal tears. Furthermore, vitreous liquefaction is a frequent finding in Marfan patients and involves the central and posterior vitreous body. Posterior vitreous detachment in absence of dehiscence at the vitreoretinal interface and abnormal vitreoretinal adhesions at the equator forward the development of large retinal tears by causing traction.

Ectopia lentis and zonular deficiency cause additional traction on the ora serrata, leading to small tears or holes in the retinal periphery. The high prevalence of retinal breaks in the temporal half could indeed be connected to the frequent direction of lens subluxation in the superior temporal quadrant. Zonules deficiency in the lower nasal quadrant may cause free movement of vitreous, resulting in traction on the opposite site, hereby producing more breaks.

In fact, most of the patients with retinal detachment have a subluxated lens. Retinal detachment in these cases has features resembling those of retinal detachment in aphakic and pseudophakic patients.

Cataract extraction and removal of subluxated lens were shown to raise the risk of retinal detachment [23, 35]. As previously mentioned,

vitreous loss and incarceration were frequent complications of earlier lens surgery and presented a major risk factor for developing retinal detachment. Modern operation techniques and instruments significantly reduced this complication.

Therapy

Management of retinal detachment in Marfan patients is difficult for several reasons: Multiple breaks in different meridians can be present. Surgery is complicated in these patients by young age, lattice degeneration, thin sclera, poorly dilating pupils, and possible ectopia lentis demanding high surgical skills.

Visual outcome was poor in the first reports of surgical repair. Almost half of the cases shown by Jarrett in 1967 ended with a visual acuity of no light perception; in the report of Maumenee, it was a third [23, 35]. Advancement of operative instruments and methods improved the visual and anatomic outcome during the last decades.

In 1993, Greco and Ambrosino showed successful pars plana lensectomy and vitrectomy in five out of six eyes [50]. Dotrelova et al. reported 18 eyes; retinal reattachment was achieved in 89% of uncomplicated and in 56% of complicated retinal detachments [51]. These results were comparable with the outcome in patients without Marfan syndrome. A part of those cases was managed without removal of the dislocated lens.

Abboud showed good long-term results with a 75% reattachment rate after combined scleral buckling and vitrectomy [47]. Proliferative vitreoretinopathy was the main cause for failure of attachment.

In the previously cited study of Sharma et al., 45% of the cases were treated by scleral buckle operation. The reattachment rate following the primary surgical intervention was 62%; eight eyes needed additional operative correction. The overall anatomical success in this group, including reoperation, was 87.5%. The other group underwent pars plana vitrectomy with scleral buckle. The overall anatomical success in those cases was 86.2%. In half of the cases, the eye was filled with air or gas, and in the other half, with silicone oil. In all phakic eyes that had vitrectomy,