Effects of extraocular muscle surgery on 15 patients with oculo-cutaneous albinism (OCA) and infantile nystagmus syndrome (INS).
OBJECTIVE
The purpose of this report is to characterize the clinical and electrophysiological effects of extraocular muscle surgery in 15 patients with oculo-cutaneous albinism (OCA) and infantile nystagmus syndrome (INS). Our hypothesis is that surgery on the extraocular muscles of patients with OCA and INS changes their nystagmus and their visual function.


METHODS
Interventional, prospective, cohort, noncomparative case series.


METHODS
All 15 patients had surgery on all four virgin horizontal recti; three for strabismus alone, three for nystagmus alone, five for an eccentric gaze null zone alone, and four for an eccentric gaze null zone plus strabismus. All patients have been followed for at least six months. All 15 patients had the subjective outcome measure of pre- and postoperative binocular best optically corrected acuity (BBOCA). Objective outcome measures included anomalous head posture (AHP) in nine patients, eye movement recording measures of expanded nystagmus acuity function (NAFX) in 10 patients, null zone position (NUZP) and null zone width (NUZW) in 10 patients, and foveation time (FOV) in nine patients.


RESULTS
The results are summarized as follows; BBOCA increased 0.1 LogMar or greater in 14 of 15 patients. In those operated on for an AHP with or without associated strabismus the AHP improved significantly (P < .01 for all). The NAFX, NUZP, NUZW, and FOV measured from eye movement recordings showed persistent, significant increases in all patients (P < .01 for all).


CONCLUSIONS
This report adds to the evidence that surgery on the extraocular muscles in patients with INS has independent neurologic and visual results.
Hertle
Richard W
RW
Department of Ophthalmology, Children's Hospital of Pittsburgh, the Pittsburgh Eye and Ear Institute, Pittsburgh, Pennsylvania 15213, USA. Richard.hertle@chp.edu
Anninger
William
W
Yang
Dongsheng
D
Shatnawi
Raed
R
Hill
Vanessa M
VM
D000293:Adolescent; D000328:Adult; D016115:Albinism, Oculocutaneous / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D004594:Electrophysiology; D005133:Eye Movements / Q000502:physiology; D005260:Female; D019416:Head Movements / Q000502:physiology; D006801:Humans; D008297:Male; D020417:Nystagmus, Congenital / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D009801:Oculomotor Muscles / Q000503:physiopathology / Q000601:surgery*; D013508:Ophthalmologic Surgical Procedures; D011187:Posture; D011446:Prospective Studies; D013577:Syndrome; D014792:Visual Acuity / Q000502:physiology