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Crystalline coloboma
a r c h s o c e s p o f t a l m o l . 2 0 1 5;9 0(3):142–143
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Crystalline coloboma夽 R. Demetrio ∗ , D. Ruiz-Sancho, N. Rolón, A. del Campo, C. Blanco Servicio de Oftalmología, Hospital Universitario Marqués de Valdecilla, Santander, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Case report: We present a 73-year-old female diagnosed with lens coloboma when she was
Received 24 August 2012
going to be operated on for cataracts.
Accepted 9 February 2014
Discussion: The incidence of congenital coloboma of the eye is estimated to be 0.5 per
Available online 14 April 2015
10 000 newborns in Spain, 1.4 in France, 2.6 in United States, and 7.5 in China. The diagnosis is clinical by observing the defect. The visual prognosis depends on the extent and
Keywords:
severity of the coloboma. The treatment of the cataract was by phacoemulsification with an
Lens
intraocular lens using a capsular tension ring.
Crystalline
˜ © 2012 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
Coloboma Cataract Capsular tension ring
Coloboma de cristalino r e s u m e n Palabras clave:
˜ Caso clínico: Paciente mujer de 73 anos diagnosticada de coloboma de cristalino tras ser
Cristalino
derivada a consulta para cirugía de catarata.
Coloboma
˜ por cada 10.000 nacimientos es de 0,5, en Discusión: La incidencia de coloboma en Espana
Catarata
Francia de 1,4, en Estados Unidos de 2,6 y en China de 7,5. El diagnóstico es clínico mediante
Anillo de tensión capsular
visualización directa. El pronóstico visual depende de la extensión y gravedad del defecto. El tratamiento de la catarata se realizó mediante la técnica convencional de facoemulsificación más lente intraocular de cápsula posterior (FACO + LIO CP) con ayuda de anillo de tensión capsular. ˜ © 2012 Sociedad Espanola de Oftalmología. Publicado por Elsevier España, S.L.U. Todos los derechos reservados.
Introduction Lens coloboma is caused by the absence of tissue at the level of the Zinn zonule in the form of a hole, notch or fissure
located in the equatorial region of the lens. Toxic, inflammatory and genetic factors have been involved in the pathogenic mechanism of lens coloboma. These factors interfere with the tertiary vitreous differentiation and zonular development between the third and fourth month of gestation. The
夽
Please cite this article as: Demetrio R, Ruiz-Sancho D, Rolón N, del Campo A, Blanco C. Coloboma de cristalino. Arch Soc Esp Oftalmol. 2015;90:142–143. ∗ Corresponding author. E-mail address: [email protected] (R. Demetrio). ˜ 2173-5794/$ – see front matter © 2012 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
a r c h s o c e s p o f t a l m o l . 2 0 1 5;9 0(3):142–143
143
Discussion
Fig. 1 – Lens coloboma.
underlying etiology for each coloboma phenotype depends on the degree of lack of fusion of the choroidal fissure folds which may affect the cornea, the iris, the ciliary body, it lens, choroids and optic nerve.1 However, it is possible to find lens coloboma which does not affect other ocular structures as the crystalline vesicle is developed independently of the embryonary fissure.2,3
Clinic case A 73-year-old female who visited the practice for cataract surgery assessments with suspected associated dislocation in the left eye (LE) is presented. Said alteration was not observed in the right eye (RE). Examination revealed far visual acuity (FVA) of 0.2 in RE and 0.05 in LE. Refraction in RE was +1.75, −0.50, 140◦ and in LE −4.50, −2.50, 105◦ . Intraocular pressure (IOP) was 12 and 13 in RE and LE respectively. Slit lamp biomicroscopic examination revealed bilateral nuclear cataract. In addition, and the exhibited zonule absence at the inferior nasal level of the lens extending from 5 to 8 o’clock without associated dislocation (Fig. 1). Gonioscopy presented an open angle. Ocular fundus examination was carried out which discarded association of uveal or chorioretinal coloboma. The posterior segment exhibited posterior vitreous detachment. The surgical approach of the cataract was with the PHACO + IOL CP (23.5 D single block monofocal lens SN6CNS, Alcon, AJL OFTALMIC S.L. Álava, Spain) with the aid of a 12 mm capsular tension ring. The intervention was successful. At the one month post-surgery examination the patient exhibited refraction of +0.50, −0.75, 115◦ , as well as FVA in the LE of 0.5 which did not improve with stenopeic. After three consecutive monthly visits, the patient remains with the lens in place and requires +3D correction for near vision.
Lens coloboma is the result of zonular involvement4 with localized absence in its developments. It frequently expresses unilaterally below the lens equator.2 It is differentiated from the lens dislocation or sub dislocation where there is no absence of zonular tissue but a rupture thereof or allegation of its fibers. In the Marfan syndrome associated to said disease, sub dislocation tends to be bilateral, symmetrical and generally superior and temporal, which is opposite to the case of coloboma. In lens coloboma, due to the absence of the force applied by the zonule, tension on the capsule is lost giving rise to alterations in its volume, which becomes thicker and spherical. This must be taken into account when planning cataract surgery to have at hand elements such as capsular tension rings which could be required for implantation.5,6 Due to the above characteristics, cataract intra-surgery complications may appear such as difficult capsulorhexis with inadequate size or problems for ocular lens centering and stabilization. This issue has been the subject of a recent publications comparing the phACO + IOL technique with manual small incision cataract surgery (MSICS) with the conclusion that a better surgical treatment is with phacoemulsification.7 In the case presented herein, the patient reached FVA of 0.5 despite having achieved adequate post surgery refraction. This could be related to possible previous amblyopia in relation to the astigmatism caused by the coloboma, which was confirmed in the presurgery refraction.
Conflict of interest No conflict of interest has been declared by the authors.
references
1. Pederna E, Méndez C. Embriología en la clínica. Casos médicos. 2006;21:125–7. 2. Aggarwal A, El-Bash AR, Inker S, Musarrella MA. Symmetrical bilateral lens coloboma in two brothers. J Pediatr Ophthalmol Strabismus. 2004;41:302–4. 3. Fuhrmann S. Eye morphogenesis and patterning of the optic vesicle. Curr Top Dev Biol. 2010;93:61–84. 4. Ilsen PF, Patel S. Coloboma of the crystalline lens. Optometry. 2003:74–80. 5. Gimbel HV, Sun R. Clinical applications of capsular tension rings in cataract surgery. Ophthalmic Surg Lasers. 2002;33:44–53. 6. Mizuno H, Yamada J, Nishiura M, Takahashi H, Hino Y, Miyatani H. Capsular tension ring use in a patient with congenital coloboma of the lens. J Cataract Refract Surg. 2004;30: 503–6. 7. Goel R, Kamal S, Khurana B, Kumar S, Malik KP, Bodh SA, et al. Minimal small incisión cataract surgery for subluxated cataract with lens coloboma. Cont Lens Anterior Eye. 2012;35: 89–91.
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Crystalline coloboma夽 R. Demetrio ∗ , D. Ruiz-Sancho, N. Rolón, A. del Campo, C. Blanco Servicio de Oftalmología, Hospital Universitario Marqués de Valdecilla, Santander, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Case report: We present a 73-year-old female diagnosed with lens coloboma when she was
Received 24 August 2012
going to be operated on for cataracts.
Accepted 9 February 2014
Discussion: The incidence of congenital coloboma of the eye is estimated to be 0.5 per
Available online 14 April 2015
10 000 newborns in Spain, 1.4 in France, 2.6 in United States, and 7.5 in China. The diagnosis is clinical by observing the defect. The visual prognosis depends on the extent and
Keywords:
severity of the coloboma. The treatment of the cataract was by phacoemulsification with an
Lens
intraocular lens using a capsular tension ring.
Crystalline
˜ © 2012 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
Coloboma Cataract Capsular tension ring
Coloboma de cristalino r e s u m e n Palabras clave:
˜ Caso clínico: Paciente mujer de 73 anos diagnosticada de coloboma de cristalino tras ser
Cristalino
derivada a consulta para cirugía de catarata.
Coloboma
˜ por cada 10.000 nacimientos es de 0,5, en Discusión: La incidencia de coloboma en Espana
Catarata
Francia de 1,4, en Estados Unidos de 2,6 y en China de 7,5. El diagnóstico es clínico mediante
Anillo de tensión capsular
visualización directa. El pronóstico visual depende de la extensión y gravedad del defecto. El tratamiento de la catarata se realizó mediante la técnica convencional de facoemulsificación más lente intraocular de cápsula posterior (FACO + LIO CP) con ayuda de anillo de tensión capsular. ˜ © 2012 Sociedad Espanola de Oftalmología. Publicado por Elsevier España, S.L.U. Todos los derechos reservados.
Introduction Lens coloboma is caused by the absence of tissue at the level of the Zinn zonule in the form of a hole, notch or fissure
located in the equatorial region of the lens. Toxic, inflammatory and genetic factors have been involved in the pathogenic mechanism of lens coloboma. These factors interfere with the tertiary vitreous differentiation and zonular development between the third and fourth month of gestation. The
夽
Please cite this article as: Demetrio R, Ruiz-Sancho D, Rolón N, del Campo A, Blanco C. Coloboma de cristalino. Arch Soc Esp Oftalmol. 2015;90:142–143. ∗ Corresponding author. E-mail address: [email protected] (R. Demetrio). ˜ 2173-5794/$ – see front matter © 2012 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
a r c h s o c e s p o f t a l m o l . 2 0 1 5;9 0(3):142–143
143
Discussion
Fig. 1 – Lens coloboma.
underlying etiology for each coloboma phenotype depends on the degree of lack of fusion of the choroidal fissure folds which may affect the cornea, the iris, the ciliary body, it lens, choroids and optic nerve.1 However, it is possible to find lens coloboma which does not affect other ocular structures as the crystalline vesicle is developed independently of the embryonary fissure.2,3
Clinic case A 73-year-old female who visited the practice for cataract surgery assessments with suspected associated dislocation in the left eye (LE) is presented. Said alteration was not observed in the right eye (RE). Examination revealed far visual acuity (FVA) of 0.2 in RE and 0.05 in LE. Refraction in RE was +1.75, −0.50, 140◦ and in LE −4.50, −2.50, 105◦ . Intraocular pressure (IOP) was 12 and 13 in RE and LE respectively. Slit lamp biomicroscopic examination revealed bilateral nuclear cataract. In addition, and the exhibited zonule absence at the inferior nasal level of the lens extending from 5 to 8 o’clock without associated dislocation (Fig. 1). Gonioscopy presented an open angle. Ocular fundus examination was carried out which discarded association of uveal or chorioretinal coloboma. The posterior segment exhibited posterior vitreous detachment. The surgical approach of the cataract was with the PHACO + IOL CP (23.5 D single block monofocal lens SN6CNS, Alcon, AJL OFTALMIC S.L. Álava, Spain) with the aid of a 12 mm capsular tension ring. The intervention was successful. At the one month post-surgery examination the patient exhibited refraction of +0.50, −0.75, 115◦ , as well as FVA in the LE of 0.5 which did not improve with stenopeic. After three consecutive monthly visits, the patient remains with the lens in place and requires +3D correction for near vision.
Lens coloboma is the result of zonular involvement4 with localized absence in its developments. It frequently expresses unilaterally below the lens equator.2 It is differentiated from the lens dislocation or sub dislocation where there is no absence of zonular tissue but a rupture thereof or allegation of its fibers. In the Marfan syndrome associated to said disease, sub dislocation tends to be bilateral, symmetrical and generally superior and temporal, which is opposite to the case of coloboma. In lens coloboma, due to the absence of the force applied by the zonule, tension on the capsule is lost giving rise to alterations in its volume, which becomes thicker and spherical. This must be taken into account when planning cataract surgery to have at hand elements such as capsular tension rings which could be required for implantation.5,6 Due to the above characteristics, cataract intra-surgery complications may appear such as difficult capsulorhexis with inadequate size or problems for ocular lens centering and stabilization. This issue has been the subject of a recent publications comparing the phACO + IOL technique with manual small incision cataract surgery (MSICS) with the conclusion that a better surgical treatment is with phacoemulsification.7 In the case presented herein, the patient reached FVA of 0.5 despite having achieved adequate post surgery refraction. This could be related to possible previous amblyopia in relation to the astigmatism caused by the coloboma, which was confirmed in the presurgery refraction.
Conflict of interest No conflict of interest has been declared by the authors.
references
1. Pederna E, Méndez C. Embriología en la clínica. Casos médicos. 2006;21:125–7. 2. Aggarwal A, El-Bash AR, Inker S, Musarrella MA. Symmetrical bilateral lens coloboma in two brothers. J Pediatr Ophthalmol Strabismus. 2004;41:302–4. 3. Fuhrmann S. Eye morphogenesis and patterning of the optic vesicle. Curr Top Dev Biol. 2010;93:61–84. 4. Ilsen PF, Patel S. Coloboma of the crystalline lens. Optometry. 2003:74–80. 5. Gimbel HV, Sun R. Clinical applications of capsular tension rings in cataract surgery. Ophthalmic Surg Lasers. 2002;33:44–53. 6. Mizuno H, Yamada J, Nishiura M, Takahashi H, Hino Y, Miyatani H. Capsular tension ring use in a patient with congenital coloboma of the lens. J Cataract Refract Surg. 2004;30: 503–6. 7. Goel R, Kamal S, Khurana B, Kumar S, Malik KP, Bodh SA, et al. Minimal small incisión cataract surgery for subluxated cataract with lens coloboma. Cont Lens Anterior Eye. 2012;35: 89–91.