Sjögren–Larsson syndrome

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A girl was referred to our clinic for alternating esotropia. The angle of deviation was measured at +45 prism diopters (Δ). The esotropia gradually turned to exotropia over the last 9 years (about −15Δ). Fundoscopy revealed crystalline retinopathy and in combination with the additional clinical findings of ichthyosis, spastic diplegia, and genetic testing, the diagnosis of Sjögren–Larsson syndrome (SLS) was made. The esotropia in this case, which gradually turned to exotropia without any intervention, may reflect the change in the muscle tone and the neurological damage in SLS. Thus, early strabismus surgery should be avoided to prevent its unpredictable outcomes.