- Email: [email protected]
065: Irreversible visual loss in children with acquired optic neuropathy
Volume 13 Number 1 / February 2009
e17
the reliability of measurements of retinal vessel tortuosity, width, and variation of width using 2 semiautomated digital image vessel analysis systems. Methods: Digital images of 4 eyes with none to severe ROP were obtained with a NM200D camera. Two graders measured retinal vessel tortuosity and diameter with 2 programs, Computer-Aided Image Analysis of the Retina (CAIAR) and ROPnet. Intra- and inter-grader reliability for each program was calculated. Results: Intra- and inter-grader correlations for CAIAR ranged from 0.992 to 0.999 for width, tortuosity, and variation of width. Intra- and inter-grader correlations for ROPnet were also significant for width and tortuosity from 0.988 to 0.998, but not for variation of width. Intra- and inter-grader correlations for width and tortuosity when analyzed by vessel type and quadrant were significant for CAIAR from 0.958 to 0.999 and for ROPnet from 0.920 to 0.999. Discussion: Both programs showed very high intra- and intergrader reliability for vessel width and tortuosity. Reliability for each was also excellent when analyzed by vessel type and quadrant. Variation of vessel width was not significant for ROPnet. Conclusions: Semiautomated digital image analysis can reliably determine width and tortuosity of posterior pole vessels in eyes at risk for ROP. This may permit the development of a quantitative, rather than qualitative, scoring system for determining the presence of severe ROP with the goal of developing a screening tool that is both practical and reliable.
066 Nystagmus and reduced visual acuity secondary to drug exposure in utero: Long-term follow-up. Manish Gupta, Alan O. Mulvihill, Brian W. Fleck, Nick L. George Introduction: We previously reported nystagmus secondary to opiate and benzodiazepine exposure in utero among a cohort of 12 children. To date, no other reports of this have been published. Here, we present an enlarged cohort of 24 children and long-term follow-up. Methods: Clinical examination and case note review of 24 children with nystagmus who were exposed to controlled drugs. Results: Twenty-two children were exposed to opiates during pregnancy, and 12 were also exposed to benzodiazepines. Two children were exposed to benzodiazepines alone. Eight children were examined once whereas 16 were followed for an average of 26 months. The nystagmus was a fine horizontal pendular type in 41.7%; fine horizontal jerk nystagmus in 45.5% and, latent in 12.5%. Where the time of onset of nystagmus was known, it was always before 6 months of age. At least 9 children (37.5%) had delayed visual maturation. Visual acuity could not always be formerly measured until several years of age. The mean earliest measurable binocular best-corrected visual acuity was 0.6 logMAR at an average of 21 months of age, improving to 0.39 by an average of 42 months of age. Electroretinogram testing was normal in the 4 children tested. Discussion: Nystagmus secondary to exposure to opiates and benzodiazepines in utero is associated with persistently reduced vision and is most likely due to insult(s) in the central nervous system rather than the eyes.
065 Irreversible visual loss in children with acquired optic neuropathy. Nitza Goldenberg-Cohen, Helen Toledano, Ian J. Cohen, Shalom Michowiz, Moshe Snir Purpose: To characterize the visual loss induced by optic neuropathy in children. Methods: The files of 14 children evaluated at a pediatric tertiary center for visual loss due to optic neuropathy were reviewed. Results: The patients included 5 boys and 9 girls of mean age 6.5 3.5 years at diagnosis; mean follow-up was 4.5 4.8 years. Causes of the optic neuropathy included intracranial tumor (n 5 9), Burkitt's lymphoma (n 5 1), intracranial hypertension (n 5 1), thromboembolic event (n 5 1), and trauma (n 5 1). Ten asymptomatic children were diagnosed incidentally on routine examination or during evaluation for neurofibromatosis-1 or new-onset strabismus. Symptoms in the remainder included headaches (n 5 3), blurred vision (n 5 1), and/ or diplopia (n 5 2). Vision findings on initial examination were available for 7 children, as follows: bilateral severe visual loss with counting fingers in the better eye (n 5 1); no light perception (n 5 2), counting fingers (n 5 1), or 6/60 in the worse eye (n 5 2), and good vision (n 5 1). Further evaluation revealed proptosis in 2 children, post-traumatic periorbital hematoma with normal disc appearance in 1, chronic papilledema in 4, and optic atrophy in 4. No data were available for the remainder. Four children had rapid severe bilateral visual loss in spite of urgent shunting (n 5 4), optic nerve sheath fenestration (n 5 1), and tumor resection (n 5 3). Treatment failed to inhibit severe bilateral (n 5 1) or unilateral (n 5 9) visual loss in the remainder. Conclusions: The most common etiologies of optic neuropathyinduced visual loss are compressive neuropathy and long-standing papilledema. Although most patients are asymptomatic, eye examination may reveal reduced vision, papilledema, optic atrophy or strabismus. Clinicians should be alerted to this risk because early diagnosis and treatment might prevent irreversible loss.
067 Botulinum toxin in infantile esotropia: Long-term outcome. Veronica C. Hauviller, Marcela Arrufat, Maria V. Sors, Maria S. Gamio Purpose: To review the long-term outcome in pediatric patients undergoing botulinum toxin treatment for infantile esotropia. Methods: Clinical records of 168 congenital esotropic patients treated with Botulinum Toxin from November 1996 to March 2006 were reviewed. Mean age was 12 months (range, 3-192). Average pretreatment deviation was 40D (range, 14D-90D). One dose of 2.53.75 U of botulinum toxin was injected in both medial rectus under general anesthesia in uncooperative children. Mean number of treatments/patient was 2. Results: A total of 45 of 168 remained orthotropic (26.78%); 77 palpebral ptosis were registered (50.33%). When the Kaplan-Meier analysis was used, cumulative treatment success in the 45 patients with ortho(10D) postinjection was 79.69% at 6 months, 77.28% at 18 months, and 66.41% at 120 months. Mean follow-up was of 31 months (range, 1 month postorthotropization to 121 months). 62.22% (28/45) received only 1 treatment. Mean time between first and second injection was 4 months. Conclusions: After 10 years of follow-up only 17.86% of the patients remained orthotropic. Regardless of the age and angle, botulinum toxin does not appear to be the best option for treatment in infantile esotropia. In our series of patients the best response to Botulinum toxin appears in patients younger than 1 year of age and amount of deviation less than 40D.
Journal of AAPOS
068 Evidence for a new recessive congenital fibrosis of the extraocular muscles (CFEOM) locus. Gena Heidary, Noriko Miyake, Koki Yamada, Caroline Andrews, Elias I. Traboulsi, Elizabeth Engle Introduction: CFEOM refers to several rare strabismus disorders characterized by nonprogressive ophthalmoplegia primarily affecting extraocular muscles innervated by the oculomotor nerve. One recessive (FEOM2) and 3 dominant (FEOM1, 3, 4) CFEOM genetic loci are mapped, and PHOX2A and KIF21A identified as the FEOM2 and
e17
the reliability of measurements of retinal vessel tortuosity, width, and variation of width using 2 semiautomated digital image vessel analysis systems. Methods: Digital images of 4 eyes with none to severe ROP were obtained with a NM200D camera. Two graders measured retinal vessel tortuosity and diameter with 2 programs, Computer-Aided Image Analysis of the Retina (CAIAR) and ROPnet. Intra- and inter-grader reliability for each program was calculated. Results: Intra- and inter-grader correlations for CAIAR ranged from 0.992 to 0.999 for width, tortuosity, and variation of width. Intra- and inter-grader correlations for ROPnet were also significant for width and tortuosity from 0.988 to 0.998, but not for variation of width. Intra- and inter-grader correlations for width and tortuosity when analyzed by vessel type and quadrant were significant for CAIAR from 0.958 to 0.999 and for ROPnet from 0.920 to 0.999. Discussion: Both programs showed very high intra- and intergrader reliability for vessel width and tortuosity. Reliability for each was also excellent when analyzed by vessel type and quadrant. Variation of vessel width was not significant for ROPnet. Conclusions: Semiautomated digital image analysis can reliably determine width and tortuosity of posterior pole vessels in eyes at risk for ROP. This may permit the development of a quantitative, rather than qualitative, scoring system for determining the presence of severe ROP with the goal of developing a screening tool that is both practical and reliable.
066 Nystagmus and reduced visual acuity secondary to drug exposure in utero: Long-term follow-up. Manish Gupta, Alan O. Mulvihill, Brian W. Fleck, Nick L. George Introduction: We previously reported nystagmus secondary to opiate and benzodiazepine exposure in utero among a cohort of 12 children. To date, no other reports of this have been published. Here, we present an enlarged cohort of 24 children and long-term follow-up. Methods: Clinical examination and case note review of 24 children with nystagmus who were exposed to controlled drugs. Results: Twenty-two children were exposed to opiates during pregnancy, and 12 were also exposed to benzodiazepines. Two children were exposed to benzodiazepines alone. Eight children were examined once whereas 16 were followed for an average of 26 months. The nystagmus was a fine horizontal pendular type in 41.7%; fine horizontal jerk nystagmus in 45.5% and, latent in 12.5%. Where the time of onset of nystagmus was known, it was always before 6 months of age. At least 9 children (37.5%) had delayed visual maturation. Visual acuity could not always be formerly measured until several years of age. The mean earliest measurable binocular best-corrected visual acuity was 0.6 logMAR at an average of 21 months of age, improving to 0.39 by an average of 42 months of age. Electroretinogram testing was normal in the 4 children tested. Discussion: Nystagmus secondary to exposure to opiates and benzodiazepines in utero is associated with persistently reduced vision and is most likely due to insult(s) in the central nervous system rather than the eyes.
065 Irreversible visual loss in children with acquired optic neuropathy. Nitza Goldenberg-Cohen, Helen Toledano, Ian J. Cohen, Shalom Michowiz, Moshe Snir Purpose: To characterize the visual loss induced by optic neuropathy in children. Methods: The files of 14 children evaluated at a pediatric tertiary center for visual loss due to optic neuropathy were reviewed. Results: The patients included 5 boys and 9 girls of mean age 6.5 3.5 years at diagnosis; mean follow-up was 4.5 4.8 years. Causes of the optic neuropathy included intracranial tumor (n 5 9), Burkitt's lymphoma (n 5 1), intracranial hypertension (n 5 1), thromboembolic event (n 5 1), and trauma (n 5 1). Ten asymptomatic children were diagnosed incidentally on routine examination or during evaluation for neurofibromatosis-1 or new-onset strabismus. Symptoms in the remainder included headaches (n 5 3), blurred vision (n 5 1), and/ or diplopia (n 5 2). Vision findings on initial examination were available for 7 children, as follows: bilateral severe visual loss with counting fingers in the better eye (n 5 1); no light perception (n 5 2), counting fingers (n 5 1), or 6/60 in the worse eye (n 5 2), and good vision (n 5 1). Further evaluation revealed proptosis in 2 children, post-traumatic periorbital hematoma with normal disc appearance in 1, chronic papilledema in 4, and optic atrophy in 4. No data were available for the remainder. Four children had rapid severe bilateral visual loss in spite of urgent shunting (n 5 4), optic nerve sheath fenestration (n 5 1), and tumor resection (n 5 3). Treatment failed to inhibit severe bilateral (n 5 1) or unilateral (n 5 9) visual loss in the remainder. Conclusions: The most common etiologies of optic neuropathyinduced visual loss are compressive neuropathy and long-standing papilledema. Although most patients are asymptomatic, eye examination may reveal reduced vision, papilledema, optic atrophy or strabismus. Clinicians should be alerted to this risk because early diagnosis and treatment might prevent irreversible loss.
067 Botulinum toxin in infantile esotropia: Long-term outcome. Veronica C. Hauviller, Marcela Arrufat, Maria V. Sors, Maria S. Gamio Purpose: To review the long-term outcome in pediatric patients undergoing botulinum toxin treatment for infantile esotropia. Methods: Clinical records of 168 congenital esotropic patients treated with Botulinum Toxin from November 1996 to March 2006 were reviewed. Mean age was 12 months (range, 3-192). Average pretreatment deviation was 40D (range, 14D-90D). One dose of 2.53.75 U of botulinum toxin was injected in both medial rectus under general anesthesia in uncooperative children. Mean number of treatments/patient was 2. Results: A total of 45 of 168 remained orthotropic (26.78%); 77 palpebral ptosis were registered (50.33%). When the Kaplan-Meier analysis was used, cumulative treatment success in the 45 patients with ortho(10D) postinjection was 79.69% at 6 months, 77.28% at 18 months, and 66.41% at 120 months. Mean follow-up was of 31 months (range, 1 month postorthotropization to 121 months). 62.22% (28/45) received only 1 treatment. Mean time between first and second injection was 4 months. Conclusions: After 10 years of follow-up only 17.86% of the patients remained orthotropic. Regardless of the age and angle, botulinum toxin does not appear to be the best option for treatment in infantile esotropia. In our series of patients the best response to Botulinum toxin appears in patients younger than 1 year of age and amount of deviation less than 40D.
Journal of AAPOS
068 Evidence for a new recessive congenital fibrosis of the extraocular muscles (CFEOM) locus. Gena Heidary, Noriko Miyake, Koki Yamada, Caroline Andrews, Elias I. Traboulsi, Elizabeth Engle Introduction: CFEOM refers to several rare strabismus disorders characterized by nonprogressive ophthalmoplegia primarily affecting extraocular muscles innervated by the oculomotor nerve. One recessive (FEOM2) and 3 dominant (FEOM1, 3, 4) CFEOM genetic loci are mapped, and PHOX2A and KIF21A identified as the FEOM2 and