CONSULTATION SECTION
341
lens implantation in eyes with deficient posterior capsules. J Cataract Refract Surg 2008; 34:1433–1438 4. Singh D, Singh K, Verma A, Singh RSJ. The iris-claw (Artisan) lens. In: Wilson ME Jr, Trivedi RH, Pandey SK, eds, Pediatric Cataract Surgery; Techniques, Complications, and Management. Philadelphia, PA, Lippincott Williams & Wilkins, 2005; 150–153
OTHER CITED MATERIAL A. Rajan R, Mahadevan R. Customized lens fitting in microcornea. Contact Lens Spectrum August 2013. Available at: http://www. clspectrum.com/printarticle.aspx?articleIDZ108722. Accessed November 9, 2013
- This patient's vision is reduced because of numerous ocular pathologies; however, the only correctable one is the cataract and despite the surgical difficulties and the expected limited improvement, cataract removal is undoubtedly indicated. Coloboma of the iris and ciliary body is usually segmental, and in most cases only the zonular fibers at the affected area are weakened or missing. Therefore, lens subluxation and phacodonesis are not common and an IOL can usually be implanted in the capsular bag. Careful slitlamp examination should distinguish between inferior subluxation of the entire lens–capsule complex secondary to weakening of the entire zonular apparatus and intracapsular inferior dislocation of a dense Morgagnian cataract in a stable capsular bag. In the latter, a PC IOL can be safely secured in the bag, whereas weakened zonular fibers all around would not be able to maintain a stable and central position of a capsule-fixated IOL. The inferior location of the anterior capsule scar and the phacodonesis support inferior displacement of the entire lens; therefore, a capsule-fixated IOL is not the best option unless the capsule is secured to the scleral wall using a capsule-stabilizing device such as a CTR, a modified CTR, or a capsule anchor. The large fibrotic scar may not allow creation of a continuous capsulotomy; therefore, an intact anterior CCC may not be achieved and the ability to use any of the above devices may not be an option. Lens removal can be performed by phacoemulsification or manual extracapsular cataract extraction. The nucleus is dense and hard, and a significant amount of ultrasound energy might be required, jeopardizing the small number (not density) of endothelial cells in an eye with a microcornea. Manual extraction through a scleral tunnel might be a good, or sometimes even preferred, alternative. The capsule–zonule complex may not provide sufficient support for an IOL. A PC IOL can be sutured to the sclera or the iris or glued using a variety of techniques. Another option is an iris-supported AC IOL. Enclavation may not be easy through the microcornea;
Figure 3. An iris-supported AC IOL and inferior pupilloplasty in a patient with congenital coloboma.
however, the globe is large (AL 25.12 mm; mean K 41.0 D). Therefore, there should be enough space for the AC IOL. The displaced pupil can be corrected by enlarging the superior opening with scissors or a vitrectome and suturing the inferior coloboma to achieve a central pupil (Figure 3). The surgeon should be prepared for all options and discuss them with the patient. The final decision is often made during surgery according to intraoperative circumstances. Ehud I. Assia, MD Kfar-Saba, Israel
Figure 4. Postoperative slitlamp photograph shows a well-centered and stable posterior chamber IOL. Corneoscleral sutures are noted temporally.
J CATARACT REFRACT SURG - VOL 40, FEBRUARY 2014
342
CONSULTATION SECTION
EDITOR’S COMMENT Microcornea–coloboma–cataract syndrome is a rare combination of ocular anomalies that may be associated with craniofacial and auricular developmental abnormalities; there is no specific inheritance pattern. Involved eyes present marked visual and surgical challenges, and a case similar to the present appeared in the Consultation Section of the April 2003 issue of the journal. In that patient, a steep cornea and axial elongation allowed aphakia to offer an acceptable outcome. The patient had ICCE by cryoextraction through an inferior 180-degree corneal incision and, fortunately, did remarkably well; the postoperative optical error was +1.75 diopter spherical equivalent. However, the present case presents a greater challenge in that high-powered aphakic spectacles would be required unless an IOL were to be implanted. As the respondents all suggest, traditional means of implanting an IOL in the capsular bag, ciliary sulcus, or anterior chamber are not options because of the nature of the cataract and the inherent anatomy of the eye. In his reply, Beiko mentions a specially designed iris-claw IOL for
microcornea; however, a device of that nature is not available in the United States. Scleral fixation of an IOL is the only likely solution as an alternative to aphakia. One could consider a variety of IOL suture techniques; however, the small anterior segment precludes use of the only available IOL (in the US) with suture fixation holes on the loops (CZ70BD, Alcon Laboratories, Inc.). Given the features of this case, I elected to perform intracapsular cryoextraction and implant a 3-piece silicone IOL (6.0 mm optic with 13.0 mm PMMA loops) by sutureless intrascleral fixation of the loops as described by Scharioth and modified as the glued IOL method by Agarwal (Video, available at http://jcrsjournal.org). However, in view of the large incision needed for the cataract removal, suturing of the scleral flaps was necessary. The patient had a marked improvement in vision along with a reduction in nystagmus. Figure 4 shows the postoperative appearance of the right eye.
J CATARACT REFRACT SURG - VOL 40, FEBRUARY 2014
Samuel Masket, MD Los Angeles, California, USA
341
lens implantation in eyes with deficient posterior capsules. J Cataract Refract Surg 2008; 34:1433–1438 4. Singh D, Singh K, Verma A, Singh RSJ. The iris-claw (Artisan) lens. In: Wilson ME Jr, Trivedi RH, Pandey SK, eds, Pediatric Cataract Surgery; Techniques, Complications, and Management. Philadelphia, PA, Lippincott Williams & Wilkins, 2005; 150–153
OTHER CITED MATERIAL A. Rajan R, Mahadevan R. Customized lens fitting in microcornea. Contact Lens Spectrum August 2013. Available at: http://www. clspectrum.com/printarticle.aspx?articleIDZ108722. Accessed November 9, 2013
- This patient's vision is reduced because of numerous ocular pathologies; however, the only correctable one is the cataract and despite the surgical difficulties and the expected limited improvement, cataract removal is undoubtedly indicated. Coloboma of the iris and ciliary body is usually segmental, and in most cases only the zonular fibers at the affected area are weakened or missing. Therefore, lens subluxation and phacodonesis are not common and an IOL can usually be implanted in the capsular bag. Careful slitlamp examination should distinguish between inferior subluxation of the entire lens–capsule complex secondary to weakening of the entire zonular apparatus and intracapsular inferior dislocation of a dense Morgagnian cataract in a stable capsular bag. In the latter, a PC IOL can be safely secured in the bag, whereas weakened zonular fibers all around would not be able to maintain a stable and central position of a capsule-fixated IOL. The inferior location of the anterior capsule scar and the phacodonesis support inferior displacement of the entire lens; therefore, a capsule-fixated IOL is not the best option unless the capsule is secured to the scleral wall using a capsule-stabilizing device such as a CTR, a modified CTR, or a capsule anchor. The large fibrotic scar may not allow creation of a continuous capsulotomy; therefore, an intact anterior CCC may not be achieved and the ability to use any of the above devices may not be an option. Lens removal can be performed by phacoemulsification or manual extracapsular cataract extraction. The nucleus is dense and hard, and a significant amount of ultrasound energy might be required, jeopardizing the small number (not density) of endothelial cells in an eye with a microcornea. Manual extraction through a scleral tunnel might be a good, or sometimes even preferred, alternative. The capsule–zonule complex may not provide sufficient support for an IOL. A PC IOL can be sutured to the sclera or the iris or glued using a variety of techniques. Another option is an iris-supported AC IOL. Enclavation may not be easy through the microcornea;
Figure 3. An iris-supported AC IOL and inferior pupilloplasty in a patient with congenital coloboma.
however, the globe is large (AL 25.12 mm; mean K 41.0 D). Therefore, there should be enough space for the AC IOL. The displaced pupil can be corrected by enlarging the superior opening with scissors or a vitrectome and suturing the inferior coloboma to achieve a central pupil (Figure 3). The surgeon should be prepared for all options and discuss them with the patient. The final decision is often made during surgery according to intraoperative circumstances. Ehud I. Assia, MD Kfar-Saba, Israel
Figure 4. Postoperative slitlamp photograph shows a well-centered and stable posterior chamber IOL. Corneoscleral sutures are noted temporally.
J CATARACT REFRACT SURG - VOL 40, FEBRUARY 2014
342
CONSULTATION SECTION
EDITOR’S COMMENT Microcornea–coloboma–cataract syndrome is a rare combination of ocular anomalies that may be associated with craniofacial and auricular developmental abnormalities; there is no specific inheritance pattern. Involved eyes present marked visual and surgical challenges, and a case similar to the present appeared in the Consultation Section of the April 2003 issue of the journal. In that patient, a steep cornea and axial elongation allowed aphakia to offer an acceptable outcome. The patient had ICCE by cryoextraction through an inferior 180-degree corneal incision and, fortunately, did remarkably well; the postoperative optical error was +1.75 diopter spherical equivalent. However, the present case presents a greater challenge in that high-powered aphakic spectacles would be required unless an IOL were to be implanted. As the respondents all suggest, traditional means of implanting an IOL in the capsular bag, ciliary sulcus, or anterior chamber are not options because of the nature of the cataract and the inherent anatomy of the eye. In his reply, Beiko mentions a specially designed iris-claw IOL for
microcornea; however, a device of that nature is not available in the United States. Scleral fixation of an IOL is the only likely solution as an alternative to aphakia. One could consider a variety of IOL suture techniques; however, the small anterior segment precludes use of the only available IOL (in the US) with suture fixation holes on the loops (CZ70BD, Alcon Laboratories, Inc.). Given the features of this case, I elected to perform intracapsular cryoextraction and implant a 3-piece silicone IOL (6.0 mm optic with 13.0 mm PMMA loops) by sutureless intrascleral fixation of the loops as described by Scharioth and modified as the glued IOL method by Agarwal (Video, available at http://jcrsjournal.org). However, in view of the large incision needed for the cataract removal, suturing of the scleral flaps was necessary. The patient had a marked improvement in vision along with a reduction in nystagmus. Figure 4 shows the postoperative appearance of the right eye.
J CATARACT REFRACT SURG - VOL 40, FEBRUARY 2014
Samuel Masket, MD Los Angeles, California, USA
