J Korean Ophthalmol Soc.  2013 Jun;54(6):987-993. 10.3341/jkos.2013.54.6.987.

A Case of Optic Atrophy and Visual Field Defect in Periventricular Leukomalacia

• Kim HY
• Choi HY
• Lee JW

• KMID: 2217280
• DOI: http://doi.org/10.3341/jkos.2013.54.6.987

Abstract

PURPOSE
To report a case of visual field defect and optic atrophy in a patient with periventricular leukomalacia.
CASE SUMMARY
A 22-year-old woman was referred to our hospital for further glaucoma evaluation. She was diagnosed with normal tension glaucoma by her local ophthalmologist. Best corrected visual acuity and intraocular pressure were 1.0 and 16 mm Hg in both eyes, respectively. Fundus examination showed a cup disc ratio of 0.36 in the right eye and 0.34 in the left eye. However, the optic disc had temporal pallor. Red-free photograph and optical coherence tomography showed supratemporal retinal nerve fiber layer atrophy. Visual field examinations revealed inferior visual field defect in both eyes. The patient was born at 34 weeks of gestation with a birth weight of 1600 g. Brain magnetic resonance imaging (MRI) showed periventricular leukomalacia.
CONCLUSIONS
The author presents a case of periventricular leukomalacia with inferior visual field defect and optic atrophy in a patient who was previously diagnosed with glaucoma. If non-specific clinical features that differ from typical glaucomatous clinical features are observed in a patient with a history of prematurity, a brain MRI can aid the diagnosis.

Keyword

Optic atrophy; Periventricular leukomalasia; Visual field defect

MeSH Terms

Atrophy
Birth Weight
Brain
Eye
Female
Glaucoma
Humans
Infant, Newborn
Intraocular Pressure
Leukomalacia, Periventricular
Low Tension Glaucoma
Magnetic Resonance Imaging
Nerve Fibers
Optic Atrophy
Pallor
Pregnancy
Retinaldehyde
Tomography, Optical Coherence
Visual Acuity
Visual Fields
Retinaldehyde

Figure

• 

  Figure 1. Retinal nerve fiber layer (RNFL) and color fundus photographs of the patient at initial visit. Cup-disc ratio is 0.36 in the right eye (A) and 0.34 in the left eye (B). Optic discs have temporal pallor in both eyes. Superotemporal RNFL defects with intact neuroretinal rim in the both eyes (C, D).

• 

  Figure 2. Optical coherence tomography (OCT) (A) and Heidelberg retinal tomography (HRT) (B). Superotemporal retinal nerve fi-ber layer (RNFL) defects were detected in the RNFL thickness deviation map on OCT with normal Moorfield Regression Analysis (MRA) on HRT in both eyes.

• 

  Figure 3. Automated visual field examinations showed bilateral inferior visual field defect in the left eye (A) and right eye (B).

• 

  Figure 4. The patient’s brain MRI. Magnetic resonance imaging (fluid-attenuated inversion recovery sequence) shows high signal intensity in the optic radiations (black arrows) and the corticospinal pathways (white arrow). Posterior ventricles were enlarged.

Reference

References

1. Miller SP, Ferriero DM, Leonard C. . Early brain injury in pre-mature newborns detected with magnetic resonance imaging is as-sociated with adverse early neurodevelopmental outcome. J Pediatr. 2005; 147:609–16.

2. Litt J, Taylor HG, Klein N, Hack M. Learning disabilities in chil-dren with very low birthweight: prevalence, neuropsychological correlates, and educational interventions. J Learn Disabil. 2005; 38:130–41.

3. Hernández-Cabrera MA, Flores-Santos R, García-Quintanilla JF. . Periventricular leukomalacia prevalence in premature newborn. Rev Med Inst Mex Seguro Soc. 2009; 47:147–50.

4. Liu J, Li J, Qin GL. . Periventricular leukomalacia in pre-mature infants in mainland China. Am J Perinatol. 2008; 25:535–40.

5. Olsén P, Pääkkö E, Vainionpää L. . Magnetic resonance imaging of periventricular leukomalacia and its clinical correlation in children. Ann Neurol. 1997; 41:754–61.

6. Jacobson L, Hård A-L, Svensson E. . Optic disc morphology may reveal timing of insult in children with periventricular leuco-malacia and/or periventricular haemorrhage. Br J Ophthalmol. 2003; 87:1345–9.

7. Fazzi E, Bova SM, Uggetti C. . Visual-perceptual impairment in children with periventricular leukomalacia. Brain Dev. 2004; 26:506–12.

8. Brodsky MC. Periventricular leukomalacia: an intracranial cause of pseudoglaucomatous cupping. Arch Ophthalmol. 2001; 119:626–7.

9. Allingham RR, Damji KF, Freedman S. . The clinical form of glaucoma. Shields’ textbook of glaucoma. 6th ed. Philadelphia: Lippincott Williams & Wilkins;2011; chap. 11.

10. Cioni G, Fazzi B, Coluccini M. . Cerebral visual impairment in preterm infants with periventricular leukomalacia. Pediatr Neurol. 1997; 17:331–8.

11. Brodsky MC. Congenital optic disc anomalies. In: Pediatric Neuro- Ophthalmology. 18th ed.Springer;2010; v. 18:chap. 2.

12. Fazzi E, Bova S, Giovenzana A. . Cognitive visual dysfunctions in preterm children with periventricular leukomalacia. Dev Med Child Neurol. 2009; 51:974–81.

13. Park SJ, Chang BL. Strabismus, amblyopia and refractory errors in patients with cerebral palsy. J Korean Ophthalmol Soc. 1999; 40:2898–903.

14. Keith CG, Kitchen WH. The significance of ocular morbidity in very-low-birthweight infants to the Australian health service. Aust J Ophthalmol. 1983; 11:29–31.

15. Khetpal V, Donahue SP. Cortical visual impairment: etiology, asso-ciated findings, and prognosis in a tertiary care setting. J AAPOS. 2007; 11:235–9.

16. Jacobson L, Ygge J, Flodmark O, Ek U. Visual and perceptual characteristics, ocular motility and strabismus in children with per-iventricular leukomalacia. Strabismus. 2002; 10:179–83.

17. Jacobson L, Ygge J, Flodmark O. Nystagmus in periventricular leucomalacia. Br J Ophthalmol. 1998; 82:1026–32.

18. Jacobson LK, Dutton GN. Periventricular leukomalacia: an im-portant cause of visual and ocular motility dysfunction in children. Surv Ophthalmol. 2000; 45:1–13.

19. Brodsky MC. Semiology of periventricular leucomalacia and its optic disc morphology. Br J Ophthalmol. 2003; 87:1309–10.

20. Jacobson L, Hellström A, Flodmark O. Large cups in normal-sized optic discs: a variant of optic nerve hypoplasia in children with per-iventricular leukomalacia. Arch Ophthalmol. 1997; 115:1263–9.

21. Brodsky MC, Glasier CM. Optic nerve hypoplasia. Clinical sig-nificance of associated central nervous system abnormalities on magnetic resonance imaging. Arch Ophthalmol. 1993; 111:66–74.

22. Lee DW, Ahn HB, Roh SH. Topographic measurement of the optic nerve head with confocal scanning laser tomography in normal third decade of Korean. J Korean Ophthalmol Soc. 1999; 40:489–95.

23. Minagawa K, Tsuji Y, Ueda H. . Possible correlation between high levels of IL-18 in the cord blood of pre-term infants and neo-natal development of periventricular leukomalacia and cerebral palsy. Cytokine. 2002; 17:164–70.

24. Mewasingh LD, Demil A, Christiaens FJ. . Motor strategies in standing up in leukomalacic spastic diplegia. Brain Dev. 2002; 24:291–5.

25. Flodmark O, Roland EH, Hill A, Whitfield MF. Periventricular leukomalacia : radiologic diagnosis. Radiology. 1987; 162:(1 Pt 1). 119–24.

26. Lee YS, Yoo DS. Cystic periventricular leukomalacia in the neo-nate: analysis of sequential sonographic findings and neurologic outcomes. J Korean Radiol Soc. 2003; 49:57–62.

27. Park HK. Hypoxic-ischemic encephalopathy in premature infants: update on periventricular leukomalacia. Korean J Perinatol. 2009; 20:106–13.