Primary exotropia: importance of cerebral MRI

Introduction: Primary exotropia is a divergent strabismus that appears from the first day of life to the second year. It can be isolated or associated with a pathological context requiring cerebral imaging to determine diagnosis and prognosis. The objective of this study was to report the sensorimotor state and the result of MRI in infantile exotropia. Patients and method: Forty-seven children with primary exotropia had a complete ophthalmologic assessment (visual acuity, binocular vision, refraction with cycloplegia, eye fundus) and cerebral MRI (average age, 16 months). Deviation was classified into four categories <4 degrees, >= 4<10 degrees, >= 10<20 degrees, >= 20 degrees. MRI results were classified into seven categories: white matter abnormalities (gliosis, delay of maturation, periventricular leukomalacia, aspecific hyperintense signal, and necroses); Virchow-Robin enlargement space and enlarged subarachnoid space, gray matter abnormalities (necroses, cerebral atrophy, occipital cortex, basal ganglia); ventriculomegaly; thin corpus callosum; cerebellar injury; and tumor. Results: There was a statistically significant increase in the rate of pathological MRI as the angle deviation increased: 76.6% of patients had a pathological ophthalmologic exam (amblyopia, ptosis, head posture, Duane's syndrome, cataract, albinism, or pigmentary retinopathy). We found 38% nystagmus and 38% optic nerve hypoplasia. There was a real pathological context in 61.7% of the exotropia cases: 27.7% prematurity, 31.9% fetal distress, 21.3% facial dysmorphy (plagiocephaly), 12.8% psychomotor delay, and 14.9% epilepsy. Only three children had isolated exotropia. Thirty-four cerebral MRI (72.3%) were not normal. In pathological MRI, there was 61.8% white matter injury, 41.2% gray matter injury, and 17.6% thin corpus callosum. Conclusion: This study demonstrates the fundamental contribution of cerebral MRI in infantile exotropia. The greater the deviation, the more abnormal the MRI results are. The pathological context and ophthalmological abnormalities are important in infantile exotropia.