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Periodic alternating esotropia associated with disorders of neural tube closure

James R Lewis1, Andrew H Malem2, J Raymond Buncic3

  • 1Department of Ophthalmology, Royal Alexandra Hospital Regional, Edmonton, Alberta, Canada.

Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
|May 23, 2025
PubMed

Insights

This study identifies periodic alternating esotropia (PAE) in children with neural tube defects and hydrocephalus. PAE, often with gaze paresis and head turn, suggests a distinct clinical entity in this population.

Area of Science:

  • Ophthalmology
  • Pediatric Neurology
  • Neuroscience

Background:

  • Periodic alternating esotropia (PAE) is a rare condition affecting eye movement.
  • Neural tube defects (NTDs) and hydrocephalus are serious congenital conditions.
  • The association between PAE and these neurological conditions requires further investigation.

Purpose of the Study:

  • To describe a series of pediatric patients presenting with periodic alternating esotropia (PAE).
  • To investigate the co-occurrence of PAE with neural tube defects and hydrocephalus.
  • To characterize the ocular motility and neurological features in affected children.

Main Methods:

  • Retrospective review of medical records for patients with PAE over a 20-year period.
  • Detailed analysis of ocular motility characteristics and associated neurological pathologies.
  • Inclusion of previously published cases for a comprehensive review.

Main Results:

  • Five pediatric patients with PAE were identified.
  • All patients exhibited PAE in conjunction with periodic alternating gaze paresis (PAG) and contraversive head turn.
  • Patients had congenital neural tube defects and hydrocephalus, requiring early ventriculoperitoneal shunt insertion.

Conclusions:

  • The findings suggest a consistent pattern of ocular motility abnormalities in children with NTDs and hydrocephalus.
  • Periodic alternating esotropia (PAE) combined with head turn may represent a specific clinical entity in this patient group.
  • Consideration of PAE is recommended for patients with neural tube defects and associated head turns.
Abstract

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