Superior segmental optic nerve hypoplasia in youth.
OBJECTIVE
The clinical characteristics of superior segmental optic nerve hypoplasia (SSOH) in youth were investigated to help establish diagnostic criteria.


METHODS
Eleven eyes of seven young patients (male/female ratio, 3/4; age, 15.1 +/- 3.4 years) who had good visual acuity and inferior visual field defects (VFDs) were evaluated. Goldmann and Humphrey perimetries and optic disc morphology were analyzed, and the patients were prospectively followed for a long period.


RESULTS
Visual field defects were wedge shaped and oriented to the blind spot, but discontinuous in mild cases. Nerve fiber layer defects (NFLDs) were consistent with the VFDs. The optic disc appearance was variable, with double ring signs in seven eyes, small discs in three eyes, and an incomplete topless disc in one eye. The mothers of none of the patients had gestational diabetes. Visual field defects did not progress during the prospective 8.3 +/- 1.3 years follow-up.


CONCLUSIONS
Characteristic VFD patterns on Goldmann perimetry and corresponding NFLDs are important in the diagnosis of SSOH, but not optic disc morphology.
Takagi
Mineo
M
Division of Ophthalmology and Visual Science, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan. mtakagi@med.niigata-u.ac.jp.
Abe
Haruki
H
Division of Ophthalmology and Visual Science, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan.
Hatase
Tetsuhisa
T
Division of Ophthalmology and Visual Science, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan.
Yaoeda
Kiyoshi
K
Division of Ophthalmology and Visual Science, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan.
Miki
Atsushi
A
Division of Ophthalmology and Visual Science, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan.
Shirakashi
Motohiro
M
Division of Ophthalmology and Visual Science, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan.
D000293:Adolescent; D002648:Child; D018450:Disease Progression; D005124:Eye Abnormalities / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D005654:Fundus Oculi; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009412:Nerve Fibers / Q000473:pathology; D009898:Optic Disk / Q000473:pathology; D009900:Optic Nerve / Q000002:abnormalities* / Q000473:pathology; D011446:Prospective Studies; D014786:Vision Disorders / Q000175:diagnosis / Q000209:etiology; D058609:Visual Field Tests; D014794:Visual Fields; D055815:Young Adult