Journal of Pediatric Neurology 2022; 20(02): 097-102
DOI: 10.1055/s-0041-1731027
Original Article

Clinical Spectrum of Ocular and Visual Dysfunction in Children with Periventricular Leukomalacia: A Need for an Interdisciplinary Approach

1   Department of Ophthalmology, Faculty of Medicine, Baskent University, Istanbul Hospital, Istanbul, Turkey
,
2   Department of Ophthalmology, Baskent University, Ankara Hospital, Ankara, Turkey
,
2   Department of Ophthalmology, Baskent University, Ankara Hospital, Ankara, Turkey
,
2   Department of Ophthalmology, Baskent University, Ankara Hospital, Ankara, Turkey
,
2   Department of Ophthalmology, Baskent University, Ankara Hospital, Ankara, Turkey
,
3   Department of Pediatric Neurology, Baskent University, Ankara Hospital, Ankara, Turkey
› Author Affiliations
Funding None.

Abstract

The study aimed to evaluate the ocular motility and visual and optic disc abnormalities in children diagnosed with periventricular leukomalacia (PVL). A retrospective analysis was performed on 51 consecutive children who had ophthalmic symptoms and were diagnosed with PVL by using magnetic resonance imaging. The patients were assessed for visual function, strabismus, cycloplegic refraction, fundus examination, and if appropriate, spectral-domain optical coherence tomography and visual field testing were applied. The primary outcome measures were the prevalence and visual and ocular motility dysfunctions. Mean age was 5.72 ± 2.6 years (range = 1–12), median birth weight was 2,740 g (range = 1,240–3,460), and median gestational age was 34 weeks (range = 28–38). In total, 21 patients (39.6%) had neurological deficit, 11 (21.5%) had intellectual disability, and 19 (37.2%) had no neurological symptom. In the spherical equivalent refractive error and cylinder power analysis, 10 patients had ≥3.0 D myopia, 15 had ≥3.0 D hyperopia, and eight had ≥2.50 D astigmatism. Thirteen (25.4%) children had a best-corrected visual acuity between 20/40 and 20/20 for Snellen card, while 9 (17.6%) had strabismic amblyopia and 6 (11.7%) had anisometropic amblyopia. Manifest strabismus was present in 35 patients (68.6%); of whom 12 had esotropia (23.5%), 16 had exotropia (31.3%) and 6 had vertical deviation (11.7%). Manifest or latent nystagmus was detected in 14 patients (27.4%). In 28 patients (54.9%), there was optic nerve abnormality. Two patients had hypoplastic disc, 14 had optic disc pallor, 7 had large cupping, and 5 had total optic atrophy. Six subjects underwent reliable visual field (VF) examinations, and all six had abnormal VFs, with inferior fields being most affected. Ocular motility disorders, optic nerve abnormalities, VF defects, and low visual acuity are common findings in this cohort of PVL patients and maybe the only presenting signs of the disease. The recognition of the visual disabilities and implementation of early rehabilitation may have a significant benefit in these children.



Publication History

Received: 12 January 2021

Accepted: 26 March 2021

Article published online:
22 June 2021

© 2021. Thieme. All rights reserved.

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  • References

  • 1 Marret S, Zupan V, Gressens P, Lagercrantz H, Evrard P. [Periventricular leukomalacia. I. Histological and pathophysiological aspects]. Arch Pediatr 1998; 5 (05) 525-537
  • 2 Spinillo A, Capuzzo E, Stronati M, Ometto A, De Santolo A, Acciano S. Obstetric risk factors for periventricular leukomalacia among preterm infants. Br J Obstet Gynaecol 1998; 105 (08) 865-871
  • 3 Jacobson LK, Dutton GN. Periventricular leukomalacia: an important cause of visual and ocular motility dysfunction in children. Surv Ophthalmol 2000; 45 (01) 1-13
  • 4 Jacobson L, Flodmark O, Martin L. Visual field defects in prematurely born patients with white matter damage of immaturity: a multiple-case study. Acta Ophthalmol Scand 2006; 84 (03) 357-362
  • 5 Olsén P, Pääkkö E, Vainionpää L, Pyhtinen J, Järvelin MR. Magnetic resonance imaging of periventricular leukomalacia and its clinical correlation in children. Ann Neurol 1997; 41 (06) 754-761
  • 6 Hoyt CS. Visual function in the brain-damaged child. Eye (Lond) 2003; 17 (03) 369-384
  • 7 Fazzi E, Bova SM, Uggetti C. et al. Visual-perceptual impairment in children with periventricular leukomalacia. Brain Dev 2004; 26 (08) 506-512
  • 8 Jacobson L, Ek U, Fernell E, Flodmark O, Broberger U. Visual impairment in preterm children with periventricular leukomalacia--visual, cognitive and neuropaediatric characteristics related to cerebral imaging. Dev Med Child Neurol 1996; 38 (08) 724-735
  • 9 Kumar P, Suresh G. Complications after preterm birth: an overview for emergency physicians. Clin Pediatr Emerg Med 2008; 9: 191-199
  • 10 Cioni G, Fazzi B, Coluccini M, Bartalena L, Boldrini A, van Hof-van Duin J. Cerebral visual impairment in preterm infants with periventricular leukomalacia. Pediatr Neurol 1997; 17 (04) 331-338
  • 11 Lanzi G, Fazzi E, Uggetti C. et al. Cerebral visual impairment in periventricular leukomalacia. Neuropediatrics 1998; 29 (03) 145-150
  • 12 Jacobson L, Ygge J, Flodmark O, Ek U. Visual and perceptual characteristics, ocular motility and strabismus in children with periventricular leukomalacia. Strabismus 2002; 10 (02) 179-183
  • 13 Brodsky MC, Fray KJ, Glasier CM. Perinatal cortical and subcortical visual loss: mechanisms of injury and associated ophthalmologic signs. Ophthalmology 2002; 109 (01) 85-94
  • 14 Brodsky MC. Semiology of periventricular leucomalacia and its optic disc morphology. Br J Ophthalmol 2003; 87 (11) 1309-1310
  • 15 Jacobson L, Ygge J, Flodmark O. Nystagmus in periventricular leucomalacia. Br J Ophthalmol 1998; 82 (09) 1026-1032
  • 16 Cioni G, Bertuccelli B, Boldrini A. et al. Correlation between visual function, neurodevelopmental outcome, and magnetic resonance imaging findings in infants with periventricular leucomalacia. Arch Dis Child Fetal Neonatal Ed 2000; 82 (02) F134-F140
  • 17 Volpe JJ. Brain injury in premature infants: a complex amalgam of destructive and developmental disturbances. Lancet Neurol 2009; 8 (01) 110-124
  • 18 Resch B, Vollaard E, Maurer U, Haas J, Rosegger H, Müller W. Risk factors and determinants of neurodevelopmental outcome in cystic periventricular leucomalacia. Eur J Pediatr 2000; 159 (09) 663-670
  • 19 Muen WJ, Saeed MU, Kaleem M, Abernethy L, Chandna A. Unsuspected periventricular leukomalacia in children with strabismus: a case series. Acta Ophthalmol Scand 2007; 85 (06) 677-680
  • 20 Ganesh S, Khurana R, Wallang B, Sharma S. Ophthalmic Manifestations in Children with Periventricular Leukomalacia. Indian J Pediatr 2018; 85 (07) 572
  • 21 Groth SL, Donahue SP, Reddy A, Sarma A, Wushensky C. Periventricular leukomalacia in patients with pseudo-glaucomatous cupping. Am J Ophthalmol 2020; 211: 31-41
  • 22 Jacobson L, Hellström A, Flodmark O. Large cups in normal-sized optic discs: a variant of optic nerve hypoplasia in children with periventricular leukomalacia. Arch Ophthalmol 1997; 115 (10) 1263-1269
  • 23 Wechsler D. Wechsler Intelligence Scales for Children. 5th ed.. Bloomington, MN: Pearson; 2014
  • 24 Tychsen L, Lisberger SG. Maldevelopment of visual motion processing in humans who had strabismus with onset in infancy. J Neurosci 1986; 6 (09) 2495-2508
  • 25 Tychsen L, Burkhalter A, Boothe RG. [Functional and structural abnormalities in the visual cortex in early childhood strabismus]. Klin Monatsbl Augenheilkd 1996; 208 (01) 18-22
  • 26 Jeon H, Jung J, Kim H, Yeom JA, Choi H. Strabismus in children with white matter damage of immaturity: MRI correlation. Br J Ophthalmol 2017; 101 (04) 467-471
  • 27 Pennefather PM, Clarke MP, Strong NP, Cottrell DG, Dutton J, Tin W. Risk factors for strabismus in children born before 32 weeks' gestation. Br J Ophthalmol 1999; 83 (05) 514-518
  • 28 Fieß A, Kölb-Keerl R, Schuster AK. et al. Prevalence and associated factors of strabismus in former preterm and full-term infants between 4 and 10 Years of age. BMC Ophthalmol 2017; 17 (01) 228
  • 29 Gursoy H, Basmak H, Bilgin B, Erol N, Colak E. The effects of mild-to-severe retinopathy of prematurity on the development of refractive errors and strabismus. Strabismus 2014; 22 (02) 68-73
  • 30 Yang CS, Wang AG, Sung CS, Hsu WM, Lee FL, Lee SM. Long-term visual outcomes of laser-treated threshold retinopathy of prematurity: a study of refractive status at 7 years. Eye (Lond) 2010; 24 (01) 14-20
  • 31 Sahni J, Subhedar NV, Clark D. Treated threshold stage 3 versus spontaneously regressed subthreshold stage 3 retinopathy of prematurity: a study of motility, refractive, and anatomical outcomes at 6 months and 36 months. Br J Ophthalmol 2005; 89 (02) 154-159
  • 32 Brodsky MC. Periventricular leukomalacia: an intracranial cause of pseudoglaucomatous cupping. Arch Ophthalmol 2001; 119 (04) 626-627
  • 33 Brodsky MC, Glasier CM. Optic nerve hypoplasia. Clinical significance of associated central nervous system abnormalities on magnetic resonance imaging. Arch Ophthalmol 1993; 111 (01) 66-74
  • 34 Jacobson L, Hård A-L, Svensson E, Flodmark O, Hellström A. Optic disc morphology may reveal timing of insult in children with periventricular leucomalacia and/or periventricular haemorrhage. Br J Ophthalmol 2003; 87 (11) 1345-1349
  • 35 Luna B, Dobson V, Scher MS, Guthrie RD. Grating acuity and visual field development in infants following perinatal asphyxia. Dev Med Child Neurol 1995; 37 (04) 330-344
  • 36 Sobrado P, Suárez J, García-Sánchez FA, Usón E. Refractive errors in children with cerebral palsy, psychomotor retardation, and other non-cerebral palsy neuromotor disabilities. Dev Med Child Neurol 1999; 41 (06) 396-403
  • 37 Schenk-Rootlieb AJ, van Nieuwenhuizen O, van Waes PF, van der Graaf Y. Cerebral visual impairment in cerebral palsy: relation to structural abnormalities of the cerebrum. Neuropediatrics 1994; 25 (02) 68-72
  • 38 Pediatric Eye Disease Investigator Group. A randomized trial of atropine vs. patching for treatment of moderate amblyopia in children. Arch Ophthalmol 2002; 120 (03) 268-278