CASE REPORT
Bilateral Juvenile Onset Primary Open-Angle Glaucoma Among Keratoconus Patients Sahil Goel, MBBS, Anita Ganger, MD, and Viney Gupta, MD
Purpose: To report the occurrence of juvenile open-angle glaucoma (JOAG) in patients with keratoconus. Patients and Methods: In this observational case series we report 6 eyes of 3 patients with keratoconus who had concomitant JOAG. Corneal topography, intraocular pressure, gonioscopic, and fundus findings were recorded for all the eyes. Results: All 3 patients presented with corneal ectasia, high intraocular pressure, and advanced glaucomatous damage and had no family history of glaucoma or keratoconus. Two of the 3 patients needed collagen cross-linking with riboflavin for progression of keratoconus and trabeculectomy for control of intraocular pressure. One of the patients also underwent a lamellar keratoplasty for keratoconus. Conclusions: This is the first case series pointing toward a possible association of JOAG with keratoconus and highlights the importance of a thorough workup of glaucoma in patients with keratoconus. Key Words: juvenile glaucoma, keratoconus, glaucoma, juvenile open-angle glaucoma
(J Glaucoma 2015;24:e25–e27)
P
rimary juvenile open-angle glaucoma (JOAG) is an uncommon form of open-angle glaucoma, usually recognized during childhood or early adulthood, often showing a strong family history. Affected individuals and family members are known to be myopic. The association of JOAG with keratoconus is unknown. We present 3 unrelated patients who were diagnosed as keratoconus and later detected to have JOAG.
(Fig. 1B). His keratometry was RE: 50.6/47.7 at 38.4/141.6 degrees and LE 48.6/44.7 at 54.3/125.7 degrees (Fig. 1C). The central corneal thickness was 425 and 448 mm in RE and LE, respectively. The patient was started on brimonidine and travoprost. On serial follow ups the IOP was noted to be between 18 and 22 mm Hg. Four months later his corneal topography revealed progression of keratoconus for which collagen cross-linking with riboflavin was performed in both the eyes. His IOP at that time was RE: 22, LE: 20 mm Hg for which dorzolamide was added. One month later his IOP was 24 and 20 mm Hg in RE and LE, respectively (on travoprost, brimonidine, and dorzolamide). In view of IOP being much higher than the target IOP, both eyes trabeculectomy with mitomycin was performed (right followed by left). His postoperative course was uneventful.
CASE 2 A 21-year-old male presented with diminution of vision in BE. His best corrected visual acuity was 6/12 and 6/24 in RE and LE, respectively, and cup-disc ratio, as noted in records was 0.6:1 in both his eyes. He was diagnosed as a case of bilateral keratoconus. There was no family history of myopia or glaucoma. Right eye collagen cross-linking with riboflavin was performed in November 2008. Postoperatively his keratometry was noted as 56.87/56.50 at 180/90 degrees and 72.25/63.12 at 108/18 degrees and CCT was 424 and 385 mm, in RE and LE, respectively. His IOP recorded then was 26 and 28 mm Hg RE and LE, respectively. He was started on timolol twice a day in both eyes. A lamellar keratoplasty was done in August 2011 in left eye. The patient was subsequently using topical prednisolone eye drops off and on. Postoperatively his IOP was recorded as 16 and 38 mm Hg in RE and LE, respectively. Gonioscopy showed open angles with 2 + angle pigmentation (Fig. 2A). His cup-disc ratio in RE was 0.8:1 while in LE had progressed to 0.9:1 (Fig. 2B). A trabeculectomy with mitomycin was thus performed in his left eye. His postoperative course was uneventful.
CASE 3 CASE REPORTS CASE 1 A 21-year-old myopic male presented for refractive surgery. His best spectacle corrected visual acuity was RE: 6/12, LE: 6/9. Intraocular pressure (IOP; Goldman applanation tonometer) was recorded to be RE: 40 mm Hg, LE: 38 mm Hg. There was no history of trauma, steroid use, or any other cause for secondary glaucoma. There was no family history of glaucoma or myopia. Gonioscopy showed wide open angles with grade 2 angle pigmentation (Fig. 1A). The optic disc in both eyes was normal sized with a 0.9:1 cup-disc ratio in RE and 0.8:1 cup-disc ratio in LE Received for publication March 4, 2013; accepted September 11, 2013. From the Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India. Disclosure: The authors declare no conflict of interest. Reprints: Viney Gupta, MD, Department of Ophthalmology, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi 110029, India (e-mail: [email protected]). Copyright r 2013 Wolters Kluwer Health, Inc. All rights reserved. DOI: 10.1097/IJG.0000000000000024
J Glaucoma
Volume 24, Number 4, April/May 2015
A 23-year-old myopic male known to be suffering from schizophrenia was diagnosed as a case of bilateral keratoconus with advanced glaucomatous cupping. His best corrected visual acuity was 4/60 and 6/12 in RE and LE, respectively. We could not find records of his untreated IOP, however his IOP was noted to be 24 and 12 mm Hg on Travoprost and Levobunolol in RE and LE, respectively. Gonioscopy revealed wide open angles without significant pigmentation. Fundus evaluation showed large optic nerve heads with peripapillary atrophy and near total cupping in both eyes. His keratometry was noted as 50.6/41.9 at 146/56 degrees and 48.6/41.2 at 45/135 degrees RE and LE, respectively. His central corneal thickness was 443 mm in RE and 492 mm in LE. Dorzolamide 3 times a day was added in RE to control IOP.
DISCUSSION Three young males presented with bilateral keratoconus and bilateral high pressure JOAG. There was no family history of glaucoma or myopia in either of the 3. Although JOAG in the general population is rare, keratoconus on the contrary is not so uncommon. The prevalence of JOAG among glaucoma patients varies from www.glaucomajournal.com |
e25
Goel et al
J Glaucoma
Volume 24, Number 4, April/May 2015
FIGURE 1. A, Goniophotograph showing wide open angle with increased pigmentation. B, Optic disc morphology showing cup-disc ratio of 0.9:1 RE and 0.8:1 LE. C, Oculus pentacam images showing keratoconus grade II (Amsler-Krumeich grading system) in right eye of Case 1.
FIGURE 2. A, Goniophotograph showing wide open angle with pigmentation in Case 2. B, Optic disc morphology showing cup-disc ratio of 0.8:1 RE and 0.9:1 LE with inferior notching.
e26 | www.glaucomajournal.com
Copyright
r
2013 Wolters Kluwer Health, Inc. All rights reserved.
J Glaucoma
Volume 24, Number 4, April/May 2015
0.7% to 3.3%.1,2 The prevalence of keratoconus in the general population varies from 0.05% to 2.3% depending on the population studied.3,4 A 5-year record of our JOAG patients revealed these 3 patients with keratoconus among 200 unrelated JOAG patients examined. This highlights the association with keratoconus in at least 1.5% of JOAG patients. Prevalence of JOAG among keratoconus patients is likely to be lower, though the association of JOAG in patients with keratoconus has not been reported so far. Normal tension glaucoma, however, has been reported among adult patients with keratoconus.5 Glaucoma and keratoconus may be interlinked in many ways. Increased IOP may be associated with progression of keratoconus especially in young patients.6 A low corneal thickness may be an independent risk factor for increased susceptibility of optic nerve to glaucomatous damage.7 Corneal thickness may be related to biomechanical properties of lamina cribrosa, which affect optic nerve head.8 A genetic susceptibility of the association of thin CCT and keratoconus with glaucoma has been reported.9,10 Although the first case in this series presented with a baseline IOP (uncorrected for CCT) above 30 mm Hg in both eyes, the other 2 cases presented with an IOP
Bilateral Juvenile Onset Primary Open-Angle Glaucoma Among Keratoconus Patients Sahil Goel, MBBS, Anita Ganger, MD, and Viney Gupta, MD
Purpose: To report the occurrence of juvenile open-angle glaucoma (JOAG) in patients with keratoconus. Patients and Methods: In this observational case series we report 6 eyes of 3 patients with keratoconus who had concomitant JOAG. Corneal topography, intraocular pressure, gonioscopic, and fundus findings were recorded for all the eyes. Results: All 3 patients presented with corneal ectasia, high intraocular pressure, and advanced glaucomatous damage and had no family history of glaucoma or keratoconus. Two of the 3 patients needed collagen cross-linking with riboflavin for progression of keratoconus and trabeculectomy for control of intraocular pressure. One of the patients also underwent a lamellar keratoplasty for keratoconus. Conclusions: This is the first case series pointing toward a possible association of JOAG with keratoconus and highlights the importance of a thorough workup of glaucoma in patients with keratoconus. Key Words: juvenile glaucoma, keratoconus, glaucoma, juvenile open-angle glaucoma
(J Glaucoma 2015;24:e25–e27)
P
rimary juvenile open-angle glaucoma (JOAG) is an uncommon form of open-angle glaucoma, usually recognized during childhood or early adulthood, often showing a strong family history. Affected individuals and family members are known to be myopic. The association of JOAG with keratoconus is unknown. We present 3 unrelated patients who were diagnosed as keratoconus and later detected to have JOAG.
(Fig. 1B). His keratometry was RE: 50.6/47.7 at 38.4/141.6 degrees and LE 48.6/44.7 at 54.3/125.7 degrees (Fig. 1C). The central corneal thickness was 425 and 448 mm in RE and LE, respectively. The patient was started on brimonidine and travoprost. On serial follow ups the IOP was noted to be between 18 and 22 mm Hg. Four months later his corneal topography revealed progression of keratoconus for which collagen cross-linking with riboflavin was performed in both the eyes. His IOP at that time was RE: 22, LE: 20 mm Hg for which dorzolamide was added. One month later his IOP was 24 and 20 mm Hg in RE and LE, respectively (on travoprost, brimonidine, and dorzolamide). In view of IOP being much higher than the target IOP, both eyes trabeculectomy with mitomycin was performed (right followed by left). His postoperative course was uneventful.
CASE 2 A 21-year-old male presented with diminution of vision in BE. His best corrected visual acuity was 6/12 and 6/24 in RE and LE, respectively, and cup-disc ratio, as noted in records was 0.6:1 in both his eyes. He was diagnosed as a case of bilateral keratoconus. There was no family history of myopia or glaucoma. Right eye collagen cross-linking with riboflavin was performed in November 2008. Postoperatively his keratometry was noted as 56.87/56.50 at 180/90 degrees and 72.25/63.12 at 108/18 degrees and CCT was 424 and 385 mm, in RE and LE, respectively. His IOP recorded then was 26 and 28 mm Hg RE and LE, respectively. He was started on timolol twice a day in both eyes. A lamellar keratoplasty was done in August 2011 in left eye. The patient was subsequently using topical prednisolone eye drops off and on. Postoperatively his IOP was recorded as 16 and 38 mm Hg in RE and LE, respectively. Gonioscopy showed open angles with 2 + angle pigmentation (Fig. 2A). His cup-disc ratio in RE was 0.8:1 while in LE had progressed to 0.9:1 (Fig. 2B). A trabeculectomy with mitomycin was thus performed in his left eye. His postoperative course was uneventful.
CASE 3 CASE REPORTS CASE 1 A 21-year-old myopic male presented for refractive surgery. His best spectacle corrected visual acuity was RE: 6/12, LE: 6/9. Intraocular pressure (IOP; Goldman applanation tonometer) was recorded to be RE: 40 mm Hg, LE: 38 mm Hg. There was no history of trauma, steroid use, or any other cause for secondary glaucoma. There was no family history of glaucoma or myopia. Gonioscopy showed wide open angles with grade 2 angle pigmentation (Fig. 1A). The optic disc in both eyes was normal sized with a 0.9:1 cup-disc ratio in RE and 0.8:1 cup-disc ratio in LE Received for publication March 4, 2013; accepted September 11, 2013. From the Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India. Disclosure: The authors declare no conflict of interest. Reprints: Viney Gupta, MD, Department of Ophthalmology, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi 110029, India (e-mail: [email protected]). Copyright r 2013 Wolters Kluwer Health, Inc. All rights reserved. DOI: 10.1097/IJG.0000000000000024
J Glaucoma
Volume 24, Number 4, April/May 2015
A 23-year-old myopic male known to be suffering from schizophrenia was diagnosed as a case of bilateral keratoconus with advanced glaucomatous cupping. His best corrected visual acuity was 4/60 and 6/12 in RE and LE, respectively. We could not find records of his untreated IOP, however his IOP was noted to be 24 and 12 mm Hg on Travoprost and Levobunolol in RE and LE, respectively. Gonioscopy revealed wide open angles without significant pigmentation. Fundus evaluation showed large optic nerve heads with peripapillary atrophy and near total cupping in both eyes. His keratometry was noted as 50.6/41.9 at 146/56 degrees and 48.6/41.2 at 45/135 degrees RE and LE, respectively. His central corneal thickness was 443 mm in RE and 492 mm in LE. Dorzolamide 3 times a day was added in RE to control IOP.
DISCUSSION Three young males presented with bilateral keratoconus and bilateral high pressure JOAG. There was no family history of glaucoma or myopia in either of the 3. Although JOAG in the general population is rare, keratoconus on the contrary is not so uncommon. The prevalence of JOAG among glaucoma patients varies from www.glaucomajournal.com |
e25
Goel et al
J Glaucoma
Volume 24, Number 4, April/May 2015
FIGURE 1. A, Goniophotograph showing wide open angle with increased pigmentation. B, Optic disc morphology showing cup-disc ratio of 0.9:1 RE and 0.8:1 LE. C, Oculus pentacam images showing keratoconus grade II (Amsler-Krumeich grading system) in right eye of Case 1.
FIGURE 2. A, Goniophotograph showing wide open angle with pigmentation in Case 2. B, Optic disc morphology showing cup-disc ratio of 0.8:1 RE and 0.9:1 LE with inferior notching.
e26 | www.glaucomajournal.com
Copyright
r
2013 Wolters Kluwer Health, Inc. All rights reserved.
J Glaucoma
Volume 24, Number 4, April/May 2015
0.7% to 3.3%.1,2 The prevalence of keratoconus in the general population varies from 0.05% to 2.3% depending on the population studied.3,4 A 5-year record of our JOAG patients revealed these 3 patients with keratoconus among 200 unrelated JOAG patients examined. This highlights the association with keratoconus in at least 1.5% of JOAG patients. Prevalence of JOAG among keratoconus patients is likely to be lower, though the association of JOAG in patients with keratoconus has not been reported so far. Normal tension glaucoma, however, has been reported among adult patients with keratoconus.5 Glaucoma and keratoconus may be interlinked in many ways. Increased IOP may be associated with progression of keratoconus especially in young patients.6 A low corneal thickness may be an independent risk factor for increased susceptibility of optic nerve to glaucomatous damage.7 Corneal thickness may be related to biomechanical properties of lamina cribrosa, which affect optic nerve head.8 A genetic susceptibility of the association of thin CCT and keratoconus with glaucoma has been reported.9,10 Although the first case in this series presented with a baseline IOP (uncorrected for CCT) above 30 mm Hg in both eyes, the other 2 cases presented with an IOP
