Marfan syndrome is an autosomal dominant multisystem disorder caused by a mutation in the fibrillin-1 gene (FBN1).1 It is mainly characterized by ocular, cardiovascular, and skeletal abnormalities.2 In these patients, vision is compromised from subluxation of the lens that may render the visual axis aphakic, irregular astigmatism from the lens periphery, and lens opacity. Lens removal with capsule fixation and intraocular lens (IOL) implantation is a valuable strategy to address these problems.3 However, in the pediatric age group, challenges with manual capsulorhexis result from the high elasticity of the capsule, with a tendency toward posterior tears, damage to the weak zonules from stress during manipulation, and the fact that the desired position for capsulorhexis is off center, all often resulting in increased intraoperative complications.4
A new generation of femtosecond lasers for cataract surgery with an integrated imaging system was introduced into ophthalmology in 2009.5 These devices can cut a free-positioned and precisely sized capsulotomy after docking of the eye without zonular disturbance.6,7 We describe the first femtosecond laser capsulotomy as a novel technique in a case of Marfan syndrome.
A 10-year-old boy with Marfan syndrome and associated ectopia lentis in both eyes had femtosecond laser-assisted cataract surgery (Catalys Precision Laser System; OptiMedica, Sunnyvale, CA) in the right eye under general anesthesia and sterile conditions on the Catalys bed. Due to the young age of the patient, it was performed off label under an ethics committee-approved clinical protocol for cataract surgery. The preoperative corrected distance visual acuity in the right eye was 20/100. Other ocular parameters were essentially normal. IOLMaster (Carl Zeiss, Oberkochen, Germany) and A-scan were used for IOL calculation. He had a positive family history of Marfan syndrome and systemic evaluation by a pediatric cardiologist revealed an aortic arch dilatation.
After sterile draping of the eye, the non-applanating fluid-filled interface (Liquid Optics Interface; Opti-Medica, inner clear aperture 13.5 mm, outer diameter 21.5 mm) was placed on the sclera and the vacuum was activated. The suction ring was then filled with balanced salt solution and docked to the laser system. Imaging of the cornea and the lens was immediately performed by integrated three-dimensional spectral-domain optical coherence tomography (Figure 1A). After the ocular surfaces were identified with the integrated algorithms, the capsulotomy was automatically placed by the software at the center of the scanned capsule, which was at the superior, temporal periphery of the pupil (Figure 1B). The system allows a free positioning of the capsulotomy wherever intended within the 13.5-mm inner clear aperture of the optical system and within the iris safety zones. The selected capsulotomy diameter was 4.1 mm (incision depth: 1,000 μm, pulse energy: 10 μJ, laser time: 12.9 sec). No lens fragmentation was performed. The total suction time was 2 minutes and 45 seconds.
Figure 1. (A) High-resolution optical coherence tomography screenshot showing the automatically detected scanned capsule center (sagittal view). (B) Screenshot of the infrared camera during capsulotomy creation, centered on the scanned capsule.
Surgery was completed on the same bed, which is permanently mounted to the laser. Two 1.2-mm clear corneal side-port incisions were made manually at the 3- and 9-o’clock positions with a paracentesis knife. A 2.75-mm blade main cataract incision was created at the 12-o’clock position. A microforceps was used to remove the capsular disc and no radial tears were noted. The soft lens was aspirated with standard bimanual irrigation/aspiration devices, using the Stellaris phaco-emulsification device (Bausch & Lomb, Aliso Viejo, CA). The anterior chamber and capsular bag were filled with viscosurgical device (Healon 1.0%; Abbott Medical Optics, Santa Ana, CA). A 10-0 polypropylene suture was knotted to the single eyelet of the Cionni ring type 1L (Morcher, Stuttgart, Germany) and the ring was introduced into the capsular bag. A partial-thickness scleral incision was made at the 5-o’clock position. A 25-gauge needle was threaded with the suture in the Cionni ring and was passed via the superior incision into the anterior chamber and under the iris to exit the eye at the 5-o’clock position at the prepared scleral incision. Tension on the suture was increased and knots secured when centration of the capsular bag was judged adequate. A foldable plate-haptic IOL (AT Lisa 409 MP, 20 diopter; Carl Zeiss) was injected into the bag and bimanual irrigation/aspiration was used to remove all viscosurgical device from the capsular bag and anterior chamber. Finally, the corneal incisions were hydrated and the conjunctiva over the scleral incision was closed using 8-0 polyglactin. The total operation time was 48 minutes.
No complications were observed within 10 weeks of follow-up. The corrected distance visual acuity increased to 20/25. The postoperative refraction was sphere +0.25, cylinder −1.0 A 3°. Figure 2 demonstrates the well-centered capsulotomy and IOL.
Figure 2. Slit-lamp photograph after femtosecond laser-assisted capsulotomy and Cionni ring and intraocular lens implantation.
Cataract surgery in eyes with pediatric Marfan syndrome with ectopia lentis is often discouraged because of the high rate of intraoperative and postoperative complications. The risk of zonular damage and the high elasticity of the capsule make it difficult to perform a well-centered manual capsulorhexis. This may lead to a decentered IOL implantation or a loss of the entire capsular bag.4 Primary posterior capsulotomy was not performed in this patient because he was 10 years old and Nd:YAG laser capsulotomy is recommended in this age group.8 The current suction ring version of the patient interface is not intended for use in the pediatric age group and a lateral canthotomy might be necessary in younger children,7 but it was not performed in this case. The integrated laser software offers the possibility to position the capsulotomy on the center of the capsular bag, based on the available intraoperative optical coherence tomography. With this tool at hand, the capsulotomy can be placed at the correct position easily, even with a subluxated decentered capsular bag. A capsulotomy centered with respect to the capsular bag will help during Cionni ring implantation and simplifies the IOL positioning at the end of surgery. No radial tear was noted during the Cionni ring implantation, suggesting tensile strength of the capsulotomy. However, radio-frequency diathermy, plasma blade, and vitrectorrhexis are alternative techniques for a well-sized and reproducible capsulotomy of sufficient strength in children.9 The amount of capsular bag shrinkage in children with resultant lens decentration with the femtosecond laser is currently unknown. Single-piece IOLs may have a smaller amount of decentration than three-piece IOLs,10 but in the event of suture absorption, repositioning using the Cionni ring eyelet might be challenging with a plate-haptic IOL.
The use of a femtosecond laser with an integrated imaging system has potential to perform a circular, easy, and gentle to remove, complete and well-centered capsulotomy for subsequent capsular tension ring and IOL implantation without decentration in patients with Marfan syndrome.
- Dietz HC, Cutting GR, Pyeritz RE, et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature. 1991;352:337–339. doi:10.1038/352337a0 [CrossRef]
- Cañadas V, Vilacosta I, Bruna I, Fuster V. Marfan syndrome: Part 1. Pathophysiology and diagnosis. Nature Reviews Cardiology. 2010;7:256–265.
- Cionni RJ, Osher RH. Management of profound zonular dialysis or weakness with a new endocapsular ring designed for scleral fixation. J Cataract Refract Surg. 1998;24:1299–1306. doi:10.1016/S0886-3350(98)80218-6 [CrossRef]
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- Vasavada AR, Praveen MR, Tassignon MJ, et al. Posterior capsule management in congenital cataract surgery. J Cataract Refract Surg. 2011;37:173–193. doi:10.1016/j.jcrs.2010.10.036 [CrossRef]
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