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Progressive cerebellar tonsillar herniation with recurrent divergence insufficiency esotropia
Dipesh Pokharel1, R Michael Siatkowski
1College of Medicine, University of Oklahoma, Oklahoma City, OK 73104, USA.
Summary
Chiari I malformation, a hindbrain herniation, can present with rare symptoms like acquired esotropia. Progressive herniation was observed in a case initially below diagnostic thresholds.
Area of Science:
- Neurology
- Ophthalmology
- Neurosurgery
Background:
- Chiari malformations involve posterior fossa content herniation through the foramen magnum.
- Chiari I malformation is defined by cerebellar tonsil ectopia >5 mm below the foramen magnum.
- Symptoms typically manifest in adulthood, including headache, ataxia, and cranial nerve palsies.
Observation:
- A rare case of acquired esotropia with divergence insufficiency was observed.
- Initial neuroimaging showed tonsillar herniation below the diagnostic threshold for Chiari I.
- Recurrence of strabismus prompted follow-up imaging.
Findings:
- Follow-up MRI revealed progressive cerebellar tonsil herniation.
- The case highlights a subtle presentation of Chiari I malformation.
- Magnetic resonance imaging (MRI) is crucial for diagnosis.
Implications:
- This case expands the spectrum of Chiari I malformation presentations.
- Early recognition of subtle signs may prevent delayed diagnosis.
- Understanding progressive herniation is vital for patient management.