Subject(s)
Eye/pathology , High-Throughput Nucleotide Sequencing , Membrane Glycoproteins/genetics , Molecular Chaperones/genetics , Neuronal Ceroid-Lipofuscinoses/diagnosis , Atrophy/etiology , Atrophy/pathology , Child , Diagnostic Techniques, Ophthalmological , Female , Humans , Neuronal Ceroid-Lipofuscinoses/genetics , Optic Nerve/pathology , Visual Acuity/physiologyABSTRACT
PURPOSE: To determine whether unilateral strabismus surgery creates lateral incomitance in patients with exotropia. METHODS: Patients >7 years of age with intermittent or constant exotropia who underwent unilateral horizontal rectus muscle surgery between December 2009 and January 2012 were prospectively evaluated. Prism and alternate cover testing was performed with distance fixation in primary position, right gaze, and left gaze after 1 hour of monocular occlusion. Measurements were obtained within 1 month prior to surgery, within 1 week after surgery, and >3 months after surgery. The surgical procedure varied according to the surgeon's discretion. The change in deviation induced by strabismus surgery in lateral gaze was expressed as a percentage of the change in deviation induced in primary position. RESULTS: A total of 12 patients met inclusion criteria. Of the 11 patients with postoperative examinations within 1 week after surgery, 10 (91%) had greater surgical effect with gaze toward the operated eye (P = 0.007). All 9 patients with >3 months' follow-up had greater surgical effect with gaze toward the operated eye (P = 0.003). On average, the surgical effect in gaze toward the operated eye was 120% of that achieved in primary position; in gaze away from the operated eye, 75% (P < 0.001). Three patients had diplopia in lateral gaze toward the operated eye that remained >6 months after surgery. CONCLUSIONS: Unilateral strabismus surgery induces lateral incomitance that may cause diplopia >6 months after surgery in patients with exotropia. This should be considered when planning strabismus surgery and counseling patients.
Subject(s)
Diplopia/etiology , Exotropia/surgery , Oculomotor Muscles/surgery , Ophthalmologic Surgical Procedures , Postoperative Complications , Adult , Aged , Female , Follow-Up Studies , Functional Laterality , Humans , Male , Middle Aged , Vision, BinocularABSTRACT
We present an 11-month-old girl with congenital ocular motor apraxia (COMA) and Joubert syndrome found to have a compound heterozygous mutation in the NPHP1 gene that is responsible for juvenile nephronophthisis type 1. The association of congenital ocular motor apraxia and juvenile nephronophthisis is reviewed. The patient does not currently manifest signs of renal failure, although her mutation indicates that she is at risk for the development of juvenile nephronophthisis type 1.
