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JAMA Ophthalmology Clinical Challenge
April 2018

Congenital Restrictive Strabismus

Author Affiliations
  • 1Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota
  • 2Department of Neurology, Mayo Clinic, Rochester, Minnesota
JAMA Ophthalmol. 2018;136(4):442-443. doi:10.1001/jamaophthalmol.2017.4345

A healthy 1-year-old boy had a history of restricted movements of the left eye since birth. He had been diagnosed as having an orbital capillary hemangioma at 3 months of age after orbital magnetic resonance imaging (MRI), which had reportedly revealed an orbital mass with enlarged extraocular muscles. He was treated with oral propranolol with no improvement.

On examination, the patient had inferior displacement of the left globe with a severe limitation of supraduction and a mild limitation of horizontal movement in the left eye. He was unable to maintain fixation with the left eye. Results of modified Krimsky testing showed 6 prism diopters (PD) of exotropia and 16 PD of left hypotropia. Retinoscopy disclosed a cycloplegic refraction of +2.00 diopters (D) in the right eye and +6.00 D in the left eye. Thyrotropin, thyroxine, and thyroid-stimulating immunoglobulin levels were all within reference ranges. Repeated MRI with coronal images showed massive enlargement of the superior, inferior, and medial rectus muscles (Figure 1). The medial rectus mass extended nasally to produce bowing of the lamina papyrecea, with no extension into adjacent structures. The enlarged extraocular muscles were hypointense on T1- and T2-weighted sequences. An abnormal curvilinear signal in the intraconal fat was isointense with muscle (Figure 1).