Progressive cerebellar tonsillar herniation with recurrent divergence insufficiency esotropia

Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
Dipesh Pokharel, R Michael Siatkowski

Abstract

The Chiari malformations are characterized by herniation of posterior fossa contents through the foramen magnum. Chiari I malformation is currently defined as ectopia of the cerebellar tonsils more than 5 mm below the foramen magnum. Extension of the cerebellar tonsils up to 3 mm may be found in the normal population. Although Chiari malformations are congenital, symptoms often do not manifest until the third and fourth decades of life, or even later. Patients usually present with headache, lower cranial nerve palsies, downbeat nystagmus, ataxia, or dissociated anesthesia of the trunk and extremities. Definitive diagnosis is made by magnetic resonance imaging (MRI), which shows the compressed tonsils extending through the foramen magnum into the cervical subarachnoid space. One of the rare presenting signs of Chiari I malformations is acquired esotropia with a divergence insufficiency pattern. We report such a case in which the initial neuroimaging showed tonsillar herniation, but of insufficient magnitude to meet diagnostic criteria for Chiari I malformation. When the strabismus recurred after initially successful eye muscle surgery, follow-up scan showed progressive tonsillar herniation.

References

Dec 1, 1996·Brain : a Journal of Neurology·M VersinoD S Zee
Dec 22, 1999·Ophthalmology·C L Weeks, L M Hamed
Sep 27, 2000·American Journal of Ophthalmology·V BiousseS R Lambert
Apr 26, 2001·Ophthalmology·R K Imes, T A Quinn
May 7, 2002·American Journal of Ophthalmology·Sabine Defoort-DhellemmesPatrick Dhellemmes

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Citations

Oct 13, 2009·International Journal of Epidemiology·Knut LönnrothChristopher Dye
Jul 21, 2020·Critical Reviews in Food Science and Nutrition·Maria Αntoniadou, Theodoros Varzakas
Mar 12, 2013·Pulmonary Medicine·Padmanesan NarasimhanDilip Mathai
Oct 12, 2016·Seminars in Ophthalmology·Aubrey L GilbertGena Heidary
Jan 1, 2005·The American Orthoptic Journal·Christine Berscheid
Jul 1, 2015·Journal of Neurology·Daniel R Gold, David S Zee
Jun 1, 2014·International Ophthalmology Clinics·Laura Kirkeby
Feb 5, 2016·Expert Review of Ophthalmology·Aasef G Shaikh, Fatema F Ghasia

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