Short Subjects
Aplasia of the Optic Chiasm and Tracts With Unifocal Polymicrogyria in an Otherwise Healthy Infant

Khalid Waheed, FRCS; Wajanat Jan, FRCR and David M. Calver, FRCOphth

EXCERPT

Chiasmal aplasia or achiasmia, also referred to as "non-decussating retinal fiber syndrome," was first described by Williams¹ in a group of Belgian sheep dogs that manifested "see-saw" nystagmus. Chiasmal aplasia usually occurs in conjunction with other central midline developmental defects that can be serious and life threatening. There is only one reported case in the literature of achiasmia, aplasia of optic nerves and tracts, and associated asymmetrical microphthalmia in an otherwise healthy infant.² We report a case of achiasmia, hypoplasia of the optic nerves and tracts, associated with unifocal polymicrogyria.

AUTHORS

From the Department of Pediatric Ophthalmology (KW, DMC) and the Department of Radiological Sciences (WJ), Guy's and St Thomas' School of Medicine, London, England.

Originally submitted November 15, 2000.

Accepted for publication April 16, 2001.

Reprint requests: Khalid Waheed, FRCS, 61, Curie Court, Queens Medical Centre University Hospital, Derby Road, Nottingham NG7 2UH United Kingdom.