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Dissociative phenomena in congenital monocular elevation deficiency
Insights
Dissociated vertical deviation can occur in children with congenital monocular elevation deficiency, particularly after strabismus surgery. This finding highlights a potential complication to monitor in pediatric ophthalmology patients.
Area of Science:
- Ophthalmology
- Pediatric Ophthalmology
- Strabismus
Background:
- Monocular elevation deficiency (MED) presents as a congenital, unilateral limitation of eye elevation.
- Dissociated vertical deviation (DVD) is less commonly associated with MED compared to other strabismus conditions.
Purpose of the Study:
- To investigate the occurrence of dissociated vertical deviation (DVD) in patients with congenital monocular elevation deficiency (MED).
Main Methods:
- Retrospective review of 31 patients diagnosed with congenital MED between 1971 and 1995.
- Exclusion of patients with acquired causes of MED or other specific conditions.
- Analysis of DVD development in relation to age, follow-up duration, and prior strabismus surgery.
Main Results:
- 29% (9 of 31) of patients with congenital MED developed DVD.
- DVD onset occurred after strabismus surgery in all affected patients (mean 3.5 years post-surgery).
- Patients who developed DVD were younger and had longer follow-up periods.
Conclusions:
- Congenital monocular elevation deficiency is associated with the development of dissociated vertical deviation.
- Strabismus surgery may be a factor in the development of DVD in these patients.
Introduction:
Monocular elevation deficiency is characterized by unilateral limitation of elevation in both adduction and abduction and is usually present at birth. Dissociative phenomena such as dissociated vertical deviation are well recognized in association with conditions such as congenital esotropia but much less so in association with conditions such as congenital monocular elevation deficiency.
Methods:
All 129 patients given the diagnosis of monocular elevation deficiency or double elevator palsy in the Pediatric Ophthalmology and Strabismus Clinic at the University of Iowa Hospitals and Clinics between 1971 and 1995 were reviewed. After those with history of trauma, myasthenia gravis, thyroid eye disease, orbital lesions, Brown syndrome, or monocular elevation deficiency with acquired onset were excluded, 31 patients with congenital monocular elevation deficiency remained for retrospective study.
Results:
First diagnosed at median age 2.6 years (although all were noted by parents at less than 6 months of age) with mean follow-up of 5.0 years (up to 15.5 years), 9 of 31 (29%) developed dissociated vertical deviation in the eye with monocular elevation deficiency, all of whom had undergone strabismus surgery 0 to 9.7 years previously (mean 3.5 years). Those who developed dissociated vertical deviation were generally younger, were followed up longer, and had more accompanying horizontal strabismus than did those who did not develop dissociated vertical deviation. The results did not reach significance.
Conclusion:
The current study demonstrates that dissociated vertical deviation occurs in association with monocular elevation deficiency.