You would think this would be an easy diagnosis, but anyone with experience examining babies knows a high refractive error and a small angle tropia can be a challenge.  Once the refractive error hits this level the retinoscopic reflex often looks like it is the same as neutral.  With a squirmy baby and questionable fixation, sometimes these cases can be written off as "normal." 

Although the photos in this study show an older child, we first saw her as an infant assessment.

We did have a history of mom and dad both with high myopia, but we did not know at this initial exam that mom was anisometropic with a subtle amblyopia. 

This diagnosis was initially missed, but with persistence - and the help of this photorefractive image we noticed there was extreme myopia in the right eye and significant astigmatism in the left.

"The Princess" (she loves wearing her tiara) did give us some clues in the visual acuity portion of the exam.  There was resistance to occlusion of the left eye (indicating a possible problem with the right eye) and poor response to preferential looking paddles in both eyes.  The fix and follow was acceptable in each eye.

The cover test was sketchy with poor cooperation.  Ocular motilities were normal in each eye.

Retinoscopy was difficult with poor patient cooperation, but once we studied the photo we had our starting point making study of refractive error much easier.

Princess was OD -10.50 - 2.50 X 180 and OS +0.50 - 2.50 X 180 with amblyopia of the right eye and a small angle esotopia of the right eye. 

Dilated fundus exam was normal eye health with clear mediae, healthy optic nerve heads OU, healthy maculae OU, and no peripheral retinal disease OU.

Treatment of this case was difficult even with an early diagnosis.  The goal was to achieve the best acuity possible in the amblyopic eye.  Binocularity had a very guarded prognosis due to the small angle esotropia.  We also had issues with motor skills development, form and shape recognition, as well as other vision perception issues.

We began with full vision correction and aggressive patching of the left eye.  At age three we did a summer of in-office vision therapy for acuity and vision skills.  After 4 years of treatment we have achieved OD 20/40- and OS 20/25 vision with eyeglass correction.  More notably we have worked with the preschool program to help prepare Princess for school.

High myopia OD and much less myopia OS axis 180.
High myopia OD and no indication of refractive error OS.
Proper refractive correction with eyeglasses, photo indicating axis 180.  Note Hirschberg reflex and positive Bruckner's reflex indicating a small angle esotropia OD.
Proper refractive correction with eyeglasses, photo indicating axis 090.  Hirschberg and Bruckner's reflexes again indicating the small angel esotropia OD.