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Hashemi Eye Care - Neuroscience Institute and Research Center

Wallerian Degeneration of Optic Nerve due to Neonatal Intracranial Hemorrhage

Naser M, Hashemi N

Journal of Ophthalmology and Research, 2020 · DOI: 10.26502/fjor.2644-00240022

A case report of a 16-year-old former premature infant referred for glaucoma evaluation who was found to have bilateral optic atrophy and visual field defects attributable to Wallerian degeneration from neonatal intracranial hemorrhage.

6 min read

This case report describes a 16-year-old male born prematurely at 29 weeks gestation with a history of neonatal intracranial hemorrhage who was referred for glaucoma evaluation and ultimately diagnosed with bilateral optic atrophy secondary to Wallerian degeneration. Published in the Journal of Ophthalmology and Research in 2020, this case emphasizes that neonatal intracranial hemorrhage can lead to delayed optic nerve damage through Wallerian degeneration, a mechanism distinct from retinopathy of prematurity.

Key Findings

  • Glaucoma was excluded despite optic nerve cupping, as intraocular pressure was normal at 10 mmHg OU and gonioscopy showed open angles
  • Bilateral arcuate visual field defects were consistent with optic nerve pathology rather than glaucomatous damage
  • Diffuse RNFL thinning on OCT bilaterally confirmed significant optic nerve fiber loss
  • MRI confirmed bilateral optic atrophy with otherwise normal brain structures
  • Wallerian degeneration from neonatal intracranial hemorrhage was identified as the cause after thorough clinical evaluation and history review
  • Normal childhood vision screening did not detect the underlying optic nerve damage, which only became symptomatic when the patient noticed peripheral vision loss while learning to drive

Background

Wallerian degeneration is a process of anterograde (orthograde) axonal degeneration that occurs following nerve fiber damage, in which the portion of the axon distal to the injury site degenerates. In the central nervous system, the most common causes include hemorrhage, infarction, tumors, and head injury. In premature neonates, intracranial hemorrhage and hypoxic-ischemic injury are well-recognized complications that can lead to Wallerian degeneration along multiple neural pathways, including the visual system.

Children born prematurely are at risk for visual impairment through several mechanisms. While retinopathy of prematurity is the most widely recognized ocular complication of prematurity, neurological insults such as ischemic brain injury and intraventricular hemorrhage can also cause delayed visual pathway damage. Studies have shown that pre-Wallerian degeneration of the cerebral peduncles in neonates with hypoxia-ischemia is associated with poor motor development, and similar processes can affect the optic pathways.

The Patient

A 16-year-old Hispanic male was referred to the neuro-ophthalmology clinic with concern for glaucoma. Key history included:

  • Chief complaint of blurry peripheral vision, first noticed while learning to drive
  • Allergic conjunctivitis
  • No headaches, double vision, or history of head trauma
  • Born at 29 weeks gestation (premature)
  • Neonatal intracranial hemorrhage at birth
  • Required supplemental oxygen for 3 months in hospital and 5 months after discharge at home
  • No retinopathy of prematurity or other ophthalmic conditions documented as a newborn
  • Normal eye exams at hospital discharge; passed all childhood visual screening tests
  • No family history of glaucoma or hereditary ocular conditions

Diagnostic Workup

Ophthalmic Examination

Ancillary Testing

  • Visual field testing: bilateral arcuate defects consistent with optic nerve pathology
  • OCT of the optic nerve: diffuse retinal nerve fiber layer (RNFL) thinning bilaterally
  • MRI brain and orbits: normal brain structure with bilateral optic atrophy

Findings

The comprehensive evaluation revealed:

  • Bilateral optic nerve cupping with diffuse RNFL thinning on OCT, mimicking glaucomatous optic neuropathy
  • Bilateral arcuate visual field defects consistent with optic nerve fiber loss
  • Normal intraocular pressure (10 mmHg) and open angles on gonioscopy, effectively excluding glaucoma
  • MRI showing bilateral optic atrophy without other structural brain abnormalities
  • No evidence of glaucoma, retinopathy of prematurity, or other acquired pathology to account for the optic nerve findings

The authors concluded that the bilateral optic nerve atrophy was attributable to Wallerian degeneration secondary to the neonatal intracranial hemorrhage, a diagnosis supported by the patient's perinatal history and the exclusion of all other causes.

Clinical Significance

This case highlights several important clinical points:

  1. Optic nerve cupping is not always glaucoma. In young patients with cupped discs and visual field defects but normal intraocular pressure, a thorough workup for non-glaucomatous optic neuropathy is essential. A detailed birth and neonatal history can reveal the true etiology.

  2. Wallerian degeneration of the optic nerve can remain subclinical for years. This patient passed all routine childhood vision screening tests and only became aware of his peripheral vision loss at age 16 when learning to drive, despite having had optic nerve damage since infancy.

  3. Neonatal intracranial hemorrhage carries long-term visual risks beyond retinopathy of prematurity. Parents of neonates with intracranial hemorrhage should be counseled about the possibility of delayed optic nerve damage and peripheral vision limitation.

  4. Premature infants need long-term ophthalmic surveillance. Even when retinopathy of prematurity is absent, neurological complications of prematurity can manifest as optic nerve disease years later.

Clinical pearl: When evaluating a young patient referred for glaucoma suspect, always obtain a detailed birth and neonatal history. Optic nerve cupping with RNFL thinning and visual field defects in the setting of normal intraocular pressure should prompt consideration of non-glaucomatous causes, including Wallerian degeneration from remote neonatal intracranial hemorrhage. Normal visual acuity does not exclude significant optic nerve pathology, as peripheral visual field loss can be the predominant deficit.

Important for parents and providers: Retinopathy of prematurity is not the only visual outcome of concern in premature infants. Children with a history of neonatal intracranial hemorrhage may develop optic nerve damage through Wallerian degeneration, leading to peripheral vision loss that may not become apparent until adolescence or adulthood. Comprehensive ophthalmic evaluation including visual field testing and OCT should be considered in these patients, even if early childhood screening was normal.

Citation

Naser M, Hashemi N. Wallerian degeneration of optic nerve due to neonatal intracranial hemorrhage. J Ophthalmol Res. 2020;3(2):61-64. doi:10.26502/fjor.2644-00240022.

References

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