Takashi Haseoka1, Risako Inagaki1, Kentaro Kurata1, Shinji Arai1, Yuri Takagi1, Hiroko Suzuki1, Akiko Hikoya1, Kasumi Nishimura2,3, Yoshihiro Hotta1, Miho Sato4. 1. Department of Ophthalmology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Hamamatsu-shi, Higashi-ku, Shizuoka, 431-3192, Japan. 2. Shizuoka Children's Hospital, Shizuoka, Japan. 3. Ueno Ganka Clinic Ophthalmology, Shizouka, Japan. 4. Department of Ophthalmology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Hamamatsu-shi, Higashi-ku, Shizuoka, 431-3192, Japan. mihosato@hama-med.ac.jp.
Abstract
PURPOSE: To report the diagnosis of three childhood patients with blue-cone monochromatism (BCM) using S-cone electroretinograms (ERG) recorded with RETeval® Complete. STUDY DESIGN: Prospective clinical study. METHODS: We examined three boys initially suspected of having rod monochromatism. S-cone ERG was performed with red background and blue flashed light stimulation using two different intensities: 0.25 cd × s/m2 and 1 cd × s/m2. RESULTS: Case 1 was a 12-year-old boy with a visual acuity of 0.1 OU. Case 2 was an 8-year-old boy with a visual acuity of 0.3 OD and 0.2 OS. Both cases showed a myopic fundus and nystagmus without any other ocular abnormalities. Case 3 was a 6-year-old boy with a visual acuity of 0.3 OD and 0.4 OS. He also showed myopic fundus changes, but nystagmus was not observed. Rod and maximal responses recorded with RETeval® were likely to be within normal range; however, cone responses were absent in all cases. S-cone ERGs showed positive responses at 40 ms with 0.25 cd × s/m2 intensity in Case 2, and at approximately 30-40 ms with 1.0 cd × s/m2 intensity in all three cases. These ERG findings led to a diagnosis of BCM. CONCLUSIONS: S-cone ERG of RETeval® was helpful in diagnosing with minimal invasion BCM in childhood patients.
PURPOSE: To report the diagnosis of three childhood patients with blue-cone monochromatism (BCM) using S-cone electroretinograms (ERG) recorded with RETeval® Complete. STUDY DESIGN: Prospective clinical study. METHODS: We examined three boys initially suspected of having rod monochromatism. S-cone ERG was performed with red background and blue flashed light stimulation using two different intensities: 0.25 cd × s/m2 and 1 cd × s/m2. RESULTS: Case 1 was a 12-year-old boy with a visual acuity of 0.1 OU. Case 2 was an 8-year-old boy with a visual acuity of 0.3 OD and 0.2 OS. Both cases showed a myopic fundus and nystagmus without any other ocular abnormalities. Case 3 was a 6-year-old boy with a visual acuity of 0.3 OD and 0.4 OS. He also showed myopic fundus changes, but nystagmus was not observed. Rod and maximal responses recorded with RETeval® were likely to be within normal range; however, cone responses were absent in all cases. S-cone ERGs showed positive responses at 40 ms with 0.25 cd × s/m2 intensity in Case 2, and at approximately 30-40 ms with 1.0 cd × s/m2 intensity in all three cases. These ERG findings led to a diagnosis of BCM. CONCLUSIONS: S-cone ERG of RETeval® was helpful in diagnosing with minimal invasion BCM in childhood patients.
Authors: Xunda Luo; Artur V Cideciyan; Alessandro Iannaccone; Alejandro J Roman; Lauren C Ditta; Barbara J Jennings; Svetlana A Yatsenko; Rebecca Sheplock; Alexander Sumaroka; Malgorzata Swider; Sharon B Schwartz; Bernd Wissinger; Susanne Kohl; Samuel G Jacobson Journal: PLoS One Date: 2015-04-24 Impact factor: 3.240