Volume 19 Number 3 / June 2015
Hope et al
277
9. Hoogenraad TU, Tan KE. Inferior oblique muscle biopsy in Graves’ ophthalmopathy. J R Soc Med 1989;82:571. 10. Newman SA. Inferior oblique surgery for restrictive strabismus in patients with thyroid orbitopathy. Trans Am Ophthalmol Soc 2009;107: 72-90.
Peters anomaly in cri-du-chat syndrome William C. Hope, BS,a Jose A. Cordovez, MD,b Jenina E. Capasso, MS, LCGC,b Kristin M. Hammersmith, MD,a,c Ralph C. Eagle, MD,c,d Joel Lall-Trail, MD,e and Alex V. Levin, MD, MHSca,e The cri-du-chat syndrome is a rare genetic disorder caused by deletions in the short arm of chromosome 5. It presents with a distinctive catlike high-pitched cry, psychomotor delays, microcephaly, craniofacial abnormalities, and, in many cases, ocular findings. We report the first child with cri-du-chat and the findings of unilateral corneal staphyloma due to Peters anomaly and retinal dysplasia.
FIG 1. Photograph of the patient’s left eye showing paracentral keratinized corneal staphyloma. Note also the superior nasal sector (optical) iridectomy and iridocorneal adhesions.
Case Report
A
10-month-old girl with a previous diagnosis of cridu-chat was referred to Wills Eye Hospital for an enlarging left corneal mass. The patient had short neck, micrognathia, small ears, short palpebral fissures, flat nasal bridge, wide-spaced nipples, palmar crease, abnormal catlike cry, a functional anteriorly placed rectum and microcephaly (\3rd percentile). No internal defects had been detected except for a left normally functional ptotic kidney. Oligo-single nucleotide polymorphism (SNP) chromosomal microarray (Affymetrix 6.0; Santa Clara, CA), performed in a CLIA-approved laboratory, showed a deletion of approximately 26.7 Mb involving 5p15.33p14.1. A duplication of 210kb at band 10q21.1
Author affiliations: aSidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania; bPediatric Ophthalmology and Ocular Genetics, cCornea Service, and dPathology, Wills Eye Hospital, Philadelphia, Pennsylvania; eChildren’s Hospital of the King’s Daughters, Norfolk, Virginia Institution of Study: Wills Eye Hospital, 840 Walnut St., Philadelphia, PA 191075109. Study was funded in part by the Robison D. Harley, MD, Endowed Chair in Pediatric Ophthalmology and Ocular Genetics (AVL) and The Foerderer Fund (AVL). Submitted October 10, 2014. Revision accepted January 27, 2015. Correspondence: Alex V. Levin, MD, MHSc, Chief, Pediatric Ophthalmology and Ocular Genetics, Wills Eye Hospital, Suite 1210, 840 Walnut St., Philadelphia, PA 19107-5109 (email: [email protected]). J AAPOS 2015;19:277-279. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.01.018
Journal of AAPOS
FIG 2. Ultrasound biomicroscopy of the left eye prior to corneal transplantation showing iridolenticular (IL) adhesions to the ectactic cornea (arrows) displaying cysts (C). The small lens (L) is coming through the pupil.
was also present. Neither parent had these copy number variations or a balanced translocation. On ophthalmological examination at our center, the right eye had normal visual responses for age. The left eye was able to fix and follow but was not the preferred eye. There was no strabismus or nystagmus. On examination under general anesthesia, handheld slit-lamp revealed a left paracentral corneal keratinized mass (corneal staphyloma) extending through the left palpebral fissure with nasal iridocorneal adhesions (Figure 1). There was no corneal edema or Haab striae that could be visualized. A previous temporal sector iridectomy performed before the patient was transferred to our care was noted. Ultrasound biomicroscopy showed a thick stalk of iridolenticular adhesions to the back of the ectatic cornea (Figure 2). Corneal diameters by template were 12 mm in each eye. Corneal pachymetry was 501 mm in the right eye and, eccentrically temporal to the lesion, 616 in the left eye.
278
Hope et al
Volume 19 Number 3 / June 2015
FIG 3. Fundus photographs of the (A) right eye and (B) left eye demonstrating multiple white areas and anomalous retinal vascularity consistent with retinal dysplasia.
A-scan ultrasound by immersion was 20.96 mm in the right eye and, by contact, 17.28 mm in the left eye. Dilated retinal examination of the left eye revealed large white areas of retina with aberrant pathways of the posterior pole vasculature, clinically suggesting retinal dysplasia. Other findings included hyperpigmentation of the macula, lack of foveal definition, and normal optic nerve (Figure 3). Dilated retinal examination of the right eye was normal. In consultation with the patient’s parents, the decision was made to proceed with removal of the staphyloma, in an attempt to restore a more normal anatomic picture and with the hope of some restoration of vision. A partial thickness trephination was initiated, but full-thickness pathology was realized and a penetrating keratoplasty was performed. Iridolenticular adhesions to the cornea were noted and lysed. A donor corneal graft was applied. Gross examination of the excised specimen revealed a white corneal button measuring 11 7.5 7 mm. Microscopic examination revealed an ectatic, scarred, and focally thickened cornea. Bowman’s layer was not present. Portions of the stroma were intensely cellular, featuring mostly spindle cells. Descemet’s membrane was only noted in the far periphery. Iridocorneal adhesions to most of the posterior cornea were present (Figure 4).
Discussion To our knowledge, this is the first reported child with both cri-du-chat and Peters anomaly. Cri-du-chat syndrome is characterized by terminal or interstitial deletion of 5p15 and, although rare, is thought to be one of the most frequent autosomal deletion syndromes.1,2 The incidence is estimated to be 1 in 50,000 newborn infants with de novo deletion in 85% and 10% to 15% resulting from a familial causative chromosomal aberration.2 Reported ophthalmic manifestations include: divergent strabismus, myopia, upward-slanting palpebral fissures, epicanthal folds, cataract, and hypertelorism. Less common manifestations include decreased tearing, optic atrophy, microspherophakia and tortuous retinal blood vessels.1-3 Cases of cri-du-chat and concurrent Goldenhar syndrome
FIG 4. Posterior surface of thickened, scarred, vascularized and chronically inflamed cornea is lined by thin layer of pigmented iris tissue; the corneal endothelium and Descemet membrane were absent (original magnification 25).
(oculo-auriculo-vertebral spectrum) with limbal dermoids have also been reported.1-3 Peters anomaly is an anterior segment dysgenesis characterized by incomplete separation of the nascent lens from surface ectoderm during embryogenesis. The estimated prevalence of congenital corneal opacity is 3-6 per 100,000, of which Peters anomaly is the most common cause.4 Thinning of the central posterior cornea with complete or partial absence of Descemet’s membrane is perhaps a constant finding.4 In a retrospective review of 37 patients with Peters anomaly, 9 displayed corneal staphyloma.5 Iridolenticulocorneal adhesions are common.5,6 We believe the partial absence of Descemet’s membrane, and the iridolenticulocorneal adhesions are consistent with a diagnosis of Peters anomaly in our patient. The scarred and ectatic cornea was lined posteriorly by uveal tissue, consistent with secondary staphyloma. The lesion did not resemble a solid epibulbar dermoid histopathologically. The surface epithelium had no epidermal appendages and the stroma, which was quite cellular, lacked coarse
Journal of AAPOS
Volume 19 Number 3 / June 2015 interweaving bundles of collagen. The patient had no clinical features of Goldenhar syndrome. Retinal dysplasia is an abnormal differentiation of the embryonic retina. It is often characterized histopathologically by rosettes, which are laminated tubules of retina that have failed to establish polarity with the retinal pigment epithelium.7 Although our patient did not have histologic analysis, the clinical appearance is consistent with this diagnosis. Retinal dysplasia can be caused by in utero exposures including viruses or teratogens, none of which occurred in our patient. It may also result from gene mutation.7 The incidence of retinal dysplasia has not previously been reported although it may be similar to that of retinoblastoma.7 Two authors cumulatively screened 66 patients with Peters anomaly and none were found to exhibit retinal dysplasia.5,6 Based on the incidence figures above, the average chance occurrence of both cri-du-chat and Peters anomaly would be approximately 1 per 1,000,000,000. Isolated Peters anomaly has been associated with a number of gene mutations including PAX6, CYP1B1, PITX2, FOXE3, and FOXC1.4 Unfortunately, we did not have the opportunity to sequence these genes in our patient. None of the genes known to be associated with Peters anomaly are located at 5p or 10q21. Ophthalmic anomalies are uncommon in 10q duplications. One patient with 10q21-q22 duplication is similar to our case, with microphthalmos, narrow palpebral fissures, opacities in the vitreous body, large coloboma and retinal dysplasia.8 The 10q21 region contains the gene cadherin 23 (CDH23), which plays a critical role in the function of photoreceptor cells and when mutated is associated with Usher syndrome.9 Perhaps duplication of this gene could be related to retinal dysplasia as seen in our patient. The d-catenin gene (CTNND2) is located at 5p15.2 and encodes an important downstream protein of PAX6. PAX6 and d-catenin have both been implicated in the development of brain and eye, and disruption of this pathway may be responsible for mental retardation and ocular findings in our patient.10 No other candidate genes for ocular malformations were found within our patient’s deleted locus.
Literature Search The authors conducted a search of English-language articles in October 2014 in Scopus using combination of the following terms linked by Boolean operators AND/OR: cri-du-chat, retinal dysplasia, Peters anomaly, anterior segment dysgenesis, corneal staphyloma, anterior staphyloma, 10q21, delta catenin, cadherin 23, CDH23, ocular, eye, and ophthalmic. References 1. Cerruti Mainardi P. Cri du chat syndrome. Orphanet J Rare Dis 2006;1. 2. Niebuhr E. The cri du chat syndrome: epidemiology, cytogenetics, and clinical features. Hum Genet 1978;44:227-75.
Journal of AAPOS
Ngo, Araya, and Kraft
279
3. Howard RO. Ocular abnormalities in the cri du chat syndrome. Am J Ophthalmol 1972;73:949-54. 4. Ciralsky J, Colby K. Congenital corneal opacities: A review with a focus on genetics. Semin Ophthalmol 2007;22:241-6. 5. Ozeki H, Shirai S, Nozaki M, et al. Ocular and systemic features of Peters’ anomaly. Graefes Arch Clin Exp Ophthalmol 2000;238: 833-9. 6. Traboulsi EI, Maumenee IH. Peters’ anomaly and associated congenital malformations. Arch Ophthalmol 1992;110:1739-42. 7. Lahav M, Albert DM, Wyand S. Clinical and histopathologic classification of retinal dysplasia. Am J Ophthalmol 1973;75:648-67. 8. Koivisto M, Herva R, Linna SL. Serial duplication of 10 (q21/q22) in a mentally retarded boy with congenital malformations. Human Genetics 1981;57:224-5. 9. Ahmed ZM, Riazuddin S, Riazuddin S, Wilcox ER. The molecular genetics of Usher syndrome. Clin Genet 2003;63:431-44. 10. Duparc R-, Boutemmine D, Champagne M-, Tetreault N, Bernier G. Pax6 is required for delta-catenin/neurojugin expression during retinal, cerebellar and cortical development in mice. Dev Biol 2006; 300:647-55.
Cyclic strabismus in adults Cheryl S. Ngo, MBBS, FRCS(Ed), Marcela Perez Araya, MD, and Stephen P. Kraft, MD, FRCSC Cyclic strabismus is a rare condition that usually occurs in children and is characterized by alternating intervals of straight and strabismic eyes. In adults with the condition, strabismus surgery often eliminates the cycles. We report a case of adult-onset cyclic esotropia that was converted into a cyclic exotropia.
Case Report
A
44-year-old woman had a 3-year history of 24hour cycles of cyclic esodeviation of her left eye. Five years prior to the onset, she had suffered total loss of vision from a central retinal artery occlusion. She developed a cataract and neovascular glaucoma. When first examined at University Health Network, Toronto, Canada in July 2013, visual acuity was 6/6 in the right eye and no light perception in the left eye. Krimsky testing revealed a left comitant esotropia of 45D. She had 10% limited abduction in the left eye, but clinically
Author affiliations: Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada Submitted December 17, 2014. Revision accepted January 29, 2015. Correspondence: Cheryl S. Ngo, MBBS, FRCS(Ed), The Hospital for Sick Children, 555 University Avenue, Toronto, ON, Canada M5G 1X8 (email: [email protected]). J AAPOS 2015;19:279-281. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.01.019
Hope et al
277
9. Hoogenraad TU, Tan KE. Inferior oblique muscle biopsy in Graves’ ophthalmopathy. J R Soc Med 1989;82:571. 10. Newman SA. Inferior oblique surgery for restrictive strabismus in patients with thyroid orbitopathy. Trans Am Ophthalmol Soc 2009;107: 72-90.
Peters anomaly in cri-du-chat syndrome William C. Hope, BS,a Jose A. Cordovez, MD,b Jenina E. Capasso, MS, LCGC,b Kristin M. Hammersmith, MD,a,c Ralph C. Eagle, MD,c,d Joel Lall-Trail, MD,e and Alex V. Levin, MD, MHSca,e The cri-du-chat syndrome is a rare genetic disorder caused by deletions in the short arm of chromosome 5. It presents with a distinctive catlike high-pitched cry, psychomotor delays, microcephaly, craniofacial abnormalities, and, in many cases, ocular findings. We report the first child with cri-du-chat and the findings of unilateral corneal staphyloma due to Peters anomaly and retinal dysplasia.
FIG 1. Photograph of the patient’s left eye showing paracentral keratinized corneal staphyloma. Note also the superior nasal sector (optical) iridectomy and iridocorneal adhesions.
Case Report
A
10-month-old girl with a previous diagnosis of cridu-chat was referred to Wills Eye Hospital for an enlarging left corneal mass. The patient had short neck, micrognathia, small ears, short palpebral fissures, flat nasal bridge, wide-spaced nipples, palmar crease, abnormal catlike cry, a functional anteriorly placed rectum and microcephaly (\3rd percentile). No internal defects had been detected except for a left normally functional ptotic kidney. Oligo-single nucleotide polymorphism (SNP) chromosomal microarray (Affymetrix 6.0; Santa Clara, CA), performed in a CLIA-approved laboratory, showed a deletion of approximately 26.7 Mb involving 5p15.33p14.1. A duplication of 210kb at band 10q21.1
Author affiliations: aSidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania; bPediatric Ophthalmology and Ocular Genetics, cCornea Service, and dPathology, Wills Eye Hospital, Philadelphia, Pennsylvania; eChildren’s Hospital of the King’s Daughters, Norfolk, Virginia Institution of Study: Wills Eye Hospital, 840 Walnut St., Philadelphia, PA 191075109. Study was funded in part by the Robison D. Harley, MD, Endowed Chair in Pediatric Ophthalmology and Ocular Genetics (AVL) and The Foerderer Fund (AVL). Submitted October 10, 2014. Revision accepted January 27, 2015. Correspondence: Alex V. Levin, MD, MHSc, Chief, Pediatric Ophthalmology and Ocular Genetics, Wills Eye Hospital, Suite 1210, 840 Walnut St., Philadelphia, PA 19107-5109 (email: [email protected]). J AAPOS 2015;19:277-279. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.01.018
Journal of AAPOS
FIG 2. Ultrasound biomicroscopy of the left eye prior to corneal transplantation showing iridolenticular (IL) adhesions to the ectactic cornea (arrows) displaying cysts (C). The small lens (L) is coming through the pupil.
was also present. Neither parent had these copy number variations or a balanced translocation. On ophthalmological examination at our center, the right eye had normal visual responses for age. The left eye was able to fix and follow but was not the preferred eye. There was no strabismus or nystagmus. On examination under general anesthesia, handheld slit-lamp revealed a left paracentral corneal keratinized mass (corneal staphyloma) extending through the left palpebral fissure with nasal iridocorneal adhesions (Figure 1). There was no corneal edema or Haab striae that could be visualized. A previous temporal sector iridectomy performed before the patient was transferred to our care was noted. Ultrasound biomicroscopy showed a thick stalk of iridolenticular adhesions to the back of the ectatic cornea (Figure 2). Corneal diameters by template were 12 mm in each eye. Corneal pachymetry was 501 mm in the right eye and, eccentrically temporal to the lesion, 616 in the left eye.
278
Hope et al
Volume 19 Number 3 / June 2015
FIG 3. Fundus photographs of the (A) right eye and (B) left eye demonstrating multiple white areas and anomalous retinal vascularity consistent with retinal dysplasia.
A-scan ultrasound by immersion was 20.96 mm in the right eye and, by contact, 17.28 mm in the left eye. Dilated retinal examination of the left eye revealed large white areas of retina with aberrant pathways of the posterior pole vasculature, clinically suggesting retinal dysplasia. Other findings included hyperpigmentation of the macula, lack of foveal definition, and normal optic nerve (Figure 3). Dilated retinal examination of the right eye was normal. In consultation with the patient’s parents, the decision was made to proceed with removal of the staphyloma, in an attempt to restore a more normal anatomic picture and with the hope of some restoration of vision. A partial thickness trephination was initiated, but full-thickness pathology was realized and a penetrating keratoplasty was performed. Iridolenticular adhesions to the cornea were noted and lysed. A donor corneal graft was applied. Gross examination of the excised specimen revealed a white corneal button measuring 11 7.5 7 mm. Microscopic examination revealed an ectatic, scarred, and focally thickened cornea. Bowman’s layer was not present. Portions of the stroma were intensely cellular, featuring mostly spindle cells. Descemet’s membrane was only noted in the far periphery. Iridocorneal adhesions to most of the posterior cornea were present (Figure 4).
Discussion To our knowledge, this is the first reported child with both cri-du-chat and Peters anomaly. Cri-du-chat syndrome is characterized by terminal or interstitial deletion of 5p15 and, although rare, is thought to be one of the most frequent autosomal deletion syndromes.1,2 The incidence is estimated to be 1 in 50,000 newborn infants with de novo deletion in 85% and 10% to 15% resulting from a familial causative chromosomal aberration.2 Reported ophthalmic manifestations include: divergent strabismus, myopia, upward-slanting palpebral fissures, epicanthal folds, cataract, and hypertelorism. Less common manifestations include decreased tearing, optic atrophy, microspherophakia and tortuous retinal blood vessels.1-3 Cases of cri-du-chat and concurrent Goldenhar syndrome
FIG 4. Posterior surface of thickened, scarred, vascularized and chronically inflamed cornea is lined by thin layer of pigmented iris tissue; the corneal endothelium and Descemet membrane were absent (original magnification 25).
(oculo-auriculo-vertebral spectrum) with limbal dermoids have also been reported.1-3 Peters anomaly is an anterior segment dysgenesis characterized by incomplete separation of the nascent lens from surface ectoderm during embryogenesis. The estimated prevalence of congenital corneal opacity is 3-6 per 100,000, of which Peters anomaly is the most common cause.4 Thinning of the central posterior cornea with complete or partial absence of Descemet’s membrane is perhaps a constant finding.4 In a retrospective review of 37 patients with Peters anomaly, 9 displayed corneal staphyloma.5 Iridolenticulocorneal adhesions are common.5,6 We believe the partial absence of Descemet’s membrane, and the iridolenticulocorneal adhesions are consistent with a diagnosis of Peters anomaly in our patient. The scarred and ectatic cornea was lined posteriorly by uveal tissue, consistent with secondary staphyloma. The lesion did not resemble a solid epibulbar dermoid histopathologically. The surface epithelium had no epidermal appendages and the stroma, which was quite cellular, lacked coarse
Journal of AAPOS
Volume 19 Number 3 / June 2015 interweaving bundles of collagen. The patient had no clinical features of Goldenhar syndrome. Retinal dysplasia is an abnormal differentiation of the embryonic retina. It is often characterized histopathologically by rosettes, which are laminated tubules of retina that have failed to establish polarity with the retinal pigment epithelium.7 Although our patient did not have histologic analysis, the clinical appearance is consistent with this diagnosis. Retinal dysplasia can be caused by in utero exposures including viruses or teratogens, none of which occurred in our patient. It may also result from gene mutation.7 The incidence of retinal dysplasia has not previously been reported although it may be similar to that of retinoblastoma.7 Two authors cumulatively screened 66 patients with Peters anomaly and none were found to exhibit retinal dysplasia.5,6 Based on the incidence figures above, the average chance occurrence of both cri-du-chat and Peters anomaly would be approximately 1 per 1,000,000,000. Isolated Peters anomaly has been associated with a number of gene mutations including PAX6, CYP1B1, PITX2, FOXE3, and FOXC1.4 Unfortunately, we did not have the opportunity to sequence these genes in our patient. None of the genes known to be associated with Peters anomaly are located at 5p or 10q21. Ophthalmic anomalies are uncommon in 10q duplications. One patient with 10q21-q22 duplication is similar to our case, with microphthalmos, narrow palpebral fissures, opacities in the vitreous body, large coloboma and retinal dysplasia.8 The 10q21 region contains the gene cadherin 23 (CDH23), which plays a critical role in the function of photoreceptor cells and when mutated is associated with Usher syndrome.9 Perhaps duplication of this gene could be related to retinal dysplasia as seen in our patient. The d-catenin gene (CTNND2) is located at 5p15.2 and encodes an important downstream protein of PAX6. PAX6 and d-catenin have both been implicated in the development of brain and eye, and disruption of this pathway may be responsible for mental retardation and ocular findings in our patient.10 No other candidate genes for ocular malformations were found within our patient’s deleted locus.
Literature Search The authors conducted a search of English-language articles in October 2014 in Scopus using combination of the following terms linked by Boolean operators AND/OR: cri-du-chat, retinal dysplasia, Peters anomaly, anterior segment dysgenesis, corneal staphyloma, anterior staphyloma, 10q21, delta catenin, cadherin 23, CDH23, ocular, eye, and ophthalmic. References 1. Cerruti Mainardi P. Cri du chat syndrome. Orphanet J Rare Dis 2006;1. 2. Niebuhr E. The cri du chat syndrome: epidemiology, cytogenetics, and clinical features. Hum Genet 1978;44:227-75.
Journal of AAPOS
Ngo, Araya, and Kraft
279
3. Howard RO. Ocular abnormalities in the cri du chat syndrome. Am J Ophthalmol 1972;73:949-54. 4. Ciralsky J, Colby K. Congenital corneal opacities: A review with a focus on genetics. Semin Ophthalmol 2007;22:241-6. 5. Ozeki H, Shirai S, Nozaki M, et al. Ocular and systemic features of Peters’ anomaly. Graefes Arch Clin Exp Ophthalmol 2000;238: 833-9. 6. Traboulsi EI, Maumenee IH. Peters’ anomaly and associated congenital malformations. Arch Ophthalmol 1992;110:1739-42. 7. Lahav M, Albert DM, Wyand S. Clinical and histopathologic classification of retinal dysplasia. Am J Ophthalmol 1973;75:648-67. 8. Koivisto M, Herva R, Linna SL. Serial duplication of 10 (q21/q22) in a mentally retarded boy with congenital malformations. Human Genetics 1981;57:224-5. 9. Ahmed ZM, Riazuddin S, Riazuddin S, Wilcox ER. The molecular genetics of Usher syndrome. Clin Genet 2003;63:431-44. 10. Duparc R-, Boutemmine D, Champagne M-, Tetreault N, Bernier G. Pax6 is required for delta-catenin/neurojugin expression during retinal, cerebellar and cortical development in mice. Dev Biol 2006; 300:647-55.
Cyclic strabismus in adults Cheryl S. Ngo, MBBS, FRCS(Ed), Marcela Perez Araya, MD, and Stephen P. Kraft, MD, FRCSC Cyclic strabismus is a rare condition that usually occurs in children and is characterized by alternating intervals of straight and strabismic eyes. In adults with the condition, strabismus surgery often eliminates the cycles. We report a case of adult-onset cyclic esotropia that was converted into a cyclic exotropia.
Case Report
A
44-year-old woman had a 3-year history of 24hour cycles of cyclic esodeviation of her left eye. Five years prior to the onset, she had suffered total loss of vision from a central retinal artery occlusion. She developed a cataract and neovascular glaucoma. When first examined at University Health Network, Toronto, Canada in July 2013, visual acuity was 6/6 in the right eye and no light perception in the left eye. Krimsky testing revealed a left comitant esotropia of 45D. She had 10% limited abduction in the left eye, but clinically
Author affiliations: Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada Submitted December 17, 2014. Revision accepted January 29, 2015. Correspondence: Cheryl S. Ngo, MBBS, FRCS(Ed), The Hospital for Sick Children, 555 University Avenue, Toronto, ON, Canada M5G 1X8 (email: [email protected]). J AAPOS 2015;19:279-281. Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.01.019
