Longitudinal neurological analysis of moderate and severe pediatric cerebral visual impairment
IntroductionCerebral visual impairment (CVI) results from damage to cerebral visual processing structures. It is the most common cause of pediatric visual impairment in developed countries and rising in prevalence in developing nations. There is currently limited understanding on how neurologic, dev...
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Frontiers Media S.A.
2022-08-01
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| Series: | Frontiers in Human Neuroscience |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fnhum.2022.772353/full |
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| author | Andres Jimenez-Gomez Kristen S. Fisher Kristen S. Fisher Kevin X. Zhang Kevin X. Zhang Chunyan Liu Qin Sun Veeral S. Shah Veeral S. Shah Veeral S. Shah Veeral S. Shah Veeral S. Shah |
| author_facet | Andres Jimenez-Gomez Kristen S. Fisher Kristen S. Fisher Kevin X. Zhang Kevin X. Zhang Chunyan Liu Qin Sun Veeral S. Shah Veeral S. Shah Veeral S. Shah Veeral S. Shah Veeral S. Shah |
| author_sort | Andres Jimenez-Gomez |
| collection | DOAJ |
| description | IntroductionCerebral visual impairment (CVI) results from damage to cerebral visual processing structures. It is the most common cause of pediatric visual impairment in developed countries and rising in prevalence in developing nations. There is currently limited understanding on how neurologic, developmental, and ophthalmic factors predict outcome for pediatric CVI.MethodA retrospective manual chart review of pediatric CVI patients seen at the tertiary pediatric hospital neurology and neuro-ophthalmology service between 2010 and 2019 was conducted. Patients were stratified into severity groups (based on a custom CVI grading score), and followed over time to identify outcome predictors. Collected baseline characteristics included perinatal, genetic, developmental, and neurologic history, along with neuroimaging and fundoscopic findings on examination. Longitudinal data collected included age, seizure control, and type of therapy received. Linear mixed-effect models were used for longitudinal CVI grade outcome analysis.ResultsA total of 249 individuals spanning 779 patient visits were identified. Mean age at diagnosis was 18.8 ± 16.8 months (2–108 months). About 64.3% were born at term age. Perinatal history revealed hypoxic ischemic encephalopathy (HIE) in 16.5%, intraventricular hemorrhage (IVH) in 11.6%, and seizures in 21.7%. At presentation, 60.3% had a diagnosis of cerebral palsy and 84.7% had developmental delay. Among all subjects, 78.6% had epilepsy; 33.8% had an epileptic encephalopathy, with spasms/hypsarrhythmia being most common. Abnormal neuroimaging was present in 93.8%. Genetic anomalies were present in 26.9%. Baseline visual examination revealed no blink-to-light (BTL) in 24.5%; only BTL in 34.5%, fixation/tracking in 26.5%, and optokinetic drum follow in 14.4%. Longitudinal data analysis showed that perinatal history of HIE, a positive epilepsy history, using multiple (≥3) epilepsy medications, cerebral palsy, and abnormal fundoscopic findings were all negatively associated with CVI grade change over time. After controlling for significant confounders, receiving any type of therapy [early childhood intervention (ECI), physical and occupational therapy (PT/OT), refractive error correction or glasses] was significantly associated with longitudinal improvement in CVI grade compared to patients who did not receive any therapy, with glasses yielding the largest benefit.ConclusionThis study offers extensive insights into neurologic, developmental and ophthalmologic features in patients with moderate to severe CVI. In concordance with previous findings, aspects of perinatal history and epilepsy/seizure control may help inform severity and prognosis in the general neurology or ophthalmology clinic. Conversely, these aspects, as well as genetic and specific epilepsy traits may alert vision health care providers in the clinic to pursue visual evaluation in at-risk individuals. Longitudinal follow-up of CVI patients showed that interventional therapies demonstrated vision function improvement greater than no therapy and maturational development. |
| first_indexed | 2024-04-11T22:02:14Z |
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| institution | Directory Open Access Journal |
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| language | English |
| last_indexed | 2024-04-11T22:02:14Z |
| publishDate | 2022-08-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Human Neuroscience |
| spelling | doaj.art-7b9171a7b07d41888b5e0efa4824d7fd2022-12-22T04:00:53ZengFrontiers Media S.A.Frontiers in Human Neuroscience1662-51612022-08-011610.3389/fnhum.2022.772353772353Longitudinal neurological analysis of moderate and severe pediatric cerebral visual impairmentAndres Jimenez-Gomez0Kristen S. Fisher1Kristen S. Fisher2Kevin X. Zhang3Kevin X. Zhang4Chunyan Liu5Qin Sun6Veeral S. Shah7Veeral S. Shah8Veeral S. Shah9Veeral S. Shah10Veeral S. Shah11Neuroscience Center, Joe DiMaggio Children’s Hospital, Hollywood, FL, United StatesDivision of Pediatric Neurology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United StatesDepartment of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United StatesDivision of Pediatric Ophthalmology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United StatesMedical Scientist Training Program, University of Cincinnati College of Medicine, Cincinnati, OH, United StatesDivision of Biostatistics and Epidemiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United StatesDivision of Biostatistics and Epidemiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United StatesDivision of Pediatric Neurology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United StatesDivision of Pediatric Ophthalmology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United StatesDepartment of Ophthalmology, University of Cincinnati, Cincinnati, OH, United StatesBaylor College of Medicine, Cullen Eye Institute, Houston, TX, United StatesDepartment of Ophthalmology, Texas Children’s Hospital, Houston, TX, United StatesIntroductionCerebral visual impairment (CVI) results from damage to cerebral visual processing structures. It is the most common cause of pediatric visual impairment in developed countries and rising in prevalence in developing nations. There is currently limited understanding on how neurologic, developmental, and ophthalmic factors predict outcome for pediatric CVI.MethodA retrospective manual chart review of pediatric CVI patients seen at the tertiary pediatric hospital neurology and neuro-ophthalmology service between 2010 and 2019 was conducted. Patients were stratified into severity groups (based on a custom CVI grading score), and followed over time to identify outcome predictors. Collected baseline characteristics included perinatal, genetic, developmental, and neurologic history, along with neuroimaging and fundoscopic findings on examination. Longitudinal data collected included age, seizure control, and type of therapy received. Linear mixed-effect models were used for longitudinal CVI grade outcome analysis.ResultsA total of 249 individuals spanning 779 patient visits were identified. Mean age at diagnosis was 18.8 ± 16.8 months (2–108 months). About 64.3% were born at term age. Perinatal history revealed hypoxic ischemic encephalopathy (HIE) in 16.5%, intraventricular hemorrhage (IVH) in 11.6%, and seizures in 21.7%. At presentation, 60.3% had a diagnosis of cerebral palsy and 84.7% had developmental delay. Among all subjects, 78.6% had epilepsy; 33.8% had an epileptic encephalopathy, with spasms/hypsarrhythmia being most common. Abnormal neuroimaging was present in 93.8%. Genetic anomalies were present in 26.9%. Baseline visual examination revealed no blink-to-light (BTL) in 24.5%; only BTL in 34.5%, fixation/tracking in 26.5%, and optokinetic drum follow in 14.4%. Longitudinal data analysis showed that perinatal history of HIE, a positive epilepsy history, using multiple (≥3) epilepsy medications, cerebral palsy, and abnormal fundoscopic findings were all negatively associated with CVI grade change over time. After controlling for significant confounders, receiving any type of therapy [early childhood intervention (ECI), physical and occupational therapy (PT/OT), refractive error correction or glasses] was significantly associated with longitudinal improvement in CVI grade compared to patients who did not receive any therapy, with glasses yielding the largest benefit.ConclusionThis study offers extensive insights into neurologic, developmental and ophthalmologic features in patients with moderate to severe CVI. In concordance with previous findings, aspects of perinatal history and epilepsy/seizure control may help inform severity and prognosis in the general neurology or ophthalmology clinic. Conversely, these aspects, as well as genetic and specific epilepsy traits may alert vision health care providers in the clinic to pursue visual evaluation in at-risk individuals. Longitudinal follow-up of CVI patients showed that interventional therapies demonstrated vision function improvement greater than no therapy and maturational development.https://www.frontiersin.org/articles/10.3389/fnhum.2022.772353/fullcerebral vision impairmentcortical visual impairmentbrain based visual impairmentepilepsycerebral palsyprematurity |
| spellingShingle | Andres Jimenez-Gomez Kristen S. Fisher Kristen S. Fisher Kevin X. Zhang Kevin X. Zhang Chunyan Liu Qin Sun Veeral S. Shah Veeral S. Shah Veeral S. Shah Veeral S. Shah Veeral S. Shah Longitudinal neurological analysis of moderate and severe pediatric cerebral visual impairment Frontiers in Human Neuroscience cerebral vision impairment cortical visual impairment brain based visual impairment epilepsy cerebral palsy prematurity |
| title | Longitudinal neurological analysis of moderate and severe pediatric cerebral visual impairment |
| title_full | Longitudinal neurological analysis of moderate and severe pediatric cerebral visual impairment |
| title_fullStr | Longitudinal neurological analysis of moderate and severe pediatric cerebral visual impairment |
| title_full_unstemmed | Longitudinal neurological analysis of moderate and severe pediatric cerebral visual impairment |
| title_short | Longitudinal neurological analysis of moderate and severe pediatric cerebral visual impairment |
| title_sort | longitudinal neurological analysis of moderate and severe pediatric cerebral visual impairment |
| topic | cerebral vision impairment cortical visual impairment brain based visual impairment epilepsy cerebral palsy prematurity |
| url | https://www.frontiersin.org/articles/10.3389/fnhum.2022.772353/full |
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