Visual field defects in optic nerve hypoplasia.

Case 1—A nine-year-old white girl was seen at the Bascom Palmer Eye Institute in December, 1969, because of poor vision. The parents first sus­ pected that she had decreased vision at age two and one-half years after a bout of measles. At the age of six years, two examiners had recorded her visual acuity at 20/200 in each eye. At the age of seven years, because of the poor vision and pale disks, skull x-ray examination and a pneumoencephalogram had been performed and were negative. Past history and family history were otherwise normal. Examination revealed a corrected visual acuity of 14/200 in each eye. External eye examination was normal. Quantitative perimetry revealed dense bilateral cecocentral scotomas with relatively intact peripheral fields (Fig. 1). Roentgenograms dis­ closed the presence of small optic canals bilaterally. Ophthalmoscopy revealed bilateral hypoplasia of the optic nerves. Both parents and the one sibling were also examined and had normal eye findings. Serum VDRL and FT A-AB S tests on the patient's mother were nonreactive. At a recent follow-up examination, the patient's vision, fields, and fundi were unchanged, and she had merited the honor roll in school during the past two years. Case 2—This 16-year-old white boy was first seen at the Bascom Palmer Eye Institute in Febru­ ary, 1970, because of poor vision. Although he was a premature baby, he did reasonably well until about the age of two years. He then developed whooping cough and pneumonia, followed by a no­ table hearing loss. His hearing was said to have been previously normal. Between the ages of two and nine years, he did rather well although he was a little slow because of the hearing loss. At the age of nine years, at a routine school vision test, the patient was first noted to have a visual deficit. His vision was said to be 20/50 at that time, and the optic disks were considered abnormal. The past his­ tory was otherwise unremarkable. Family history