Not just exotropia: a case of persistent visual complaints in a 10-year-old boy

Intermittent exotropia is a common childhood condition often considered benign. However, atypical features such as reduced distance visual acuity due to accommodative spasm, rapid progression without clear cause, or unusually late onset may point to an underlying neurological cause.

We report the case of a 10-year-old boy referred for a second opinion due to progressive intermittent exotropia and reduced distance visual acuity.

At the time of referral, clinical examination revealed an accommodative spasm, which explained the reduced acuity. This spasm was initially interpreted as a compensatory mechanism used by the patient to maintain control of the exotropic deviation. After successful spectacle correction and the establishment of a stable deviation, strabismus surgery was performed. Postoperatively, visual complaints persisted. Atropine improved his distance vision, but symptoms recurred after discontinuation. MRI then was initiated for the recurrent accommodation spasm without a clear cause and revealed a lesion in the right thalamus and cerebral peduncle, initially raising suspicion for glioma. Follow-up imaging, however, confirmed regression and suggested a hemorrhagic infarct without evidence of tumor or vascular malformation.

This case underscores the importance of recognizing red flags in seemingly straightforward strabismus. Persistent accommodative spasm with reduced distance visual acuity, particularly when unexplained, and a rapidly decompensating intermittent exotropia should prompt consideration of neuroimaging. Early recognition of these atypical features can significantly alter both the diagnostic process and management in pediatric strabismus.