Biomedical subjects
Publications and source records attributed to E L Raab.
We describe a mother and daughter with a distinct phenotype that is different from previous reports. This is likely to constitute a new syndrome for which we propose the mnemonic GMS for G goniodysgenesis, M mental deficiency, and S short stature. The pattern of occurrence is compatible with either autosomal dominant or X-linked inheritance.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
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Eight patients with large-angle exotropia and unimprovable poor vision in one eye were treated with conventional maximum horizontal rectus recession/resection plus weakening of both obliques of the poorly seeing eye. This appears to be a useful alternative to "supermaximal" recession/resection procedures, which are more likely to lead to deficient or restricted rotations. The effect is probably due to the release of additional sites of contracture that occur in the oblique muscles. The principal disadvantage is a small postsurgical hypertropia of the operated eye, for which routine infraplacement of both horizontal recti can compensate adequately.
It is now recognized that the clinical findings of Brown's superior oblique tendon sheath syndrome can be caused by a variety of abnormalities. This has led to classification into "true" and "simulated" sheath syndromes. This report describes a case of simulated sheath syndrome caused by an anomalous fibrous band between the superior oblique tendon sheath and the upper nasal intermuscular quadrant. This unusual anomaly results in functional shortening of the sheath. Division of the band eliminated the restrictions on traction test and improved elevation in all fields postoperatively.
The preoperative evaluation of the strabismus patient may require one or several examination sessions. Since general anesthesia is most often employed, the patient's general health, hematological and neurological status, and concurrent medications should be evaluated. The age of onset and whether the deviation was intermittent in character for some part of its course are important clues to fusion potential. The examination should emphasize close observation of the eyes when they are stationary and during rotations; it should be conducted so as to encourage, rather than frustrate, binocular cooperation. This requires test selection that takes into account both variations in alignment (at different test distances) and amblyopia. Sensory evaluation can involve many tests, but specifically for surgical planning it can be brief and apply principal to justification of surgery for small-angle, nonsymptomatic deviations. The phenomenon of intractable diplopia is unusual and probably should not be considered a strong deterrent to surgical correction of a long-standing and cosmetically unpleasant misalignment.
The importance of both preoperative fusion and the distance-near alignment relationship to the results of recession of the lateral recti for correction of exodeviation was studied. Satisfactory alignment was achieved after this procedure in 78% of patients with constant exodeviation and 80% of patients with intermittent exodeviation. In each fusion status, initial overcorrection of up to 20 prism diopters gave the highest percentage of good results (constant exodeviation patients, 88%; intermittent, 89%). Fifty-six percent of the satisfactorily realigned constant exodeviation patients and 69% of intermittent exodeviation patients remained satisfactorily realigned over an average follow-up interval of five years. If convergence insufficiency was present, satisfactory realignments noted at five to eight weeks could be maintained thereafter in 40% of patients. Where there was no preoperative convergence insufficiency, satisfactory results were maintained in 64% of patients with "high" and 68% of patients with "normal" distance-near ratios.
This study reports observations on the subsequent course of the accommodative component in 139 patients with mixed esotropia after surgery for the nonaccommodative portion of the deviation. An unexpected finding was the presence of a normal distance/near relationship at the time of operation in 69% of these patients. In 40 cases (29%), the accommodative component subsided during the 1st postoperative year. In the remaining patients, the occurrence rate of improvement at longer intervals was similar to that for patients with pure accommodative esotropia observed in prior studies. Persistence was associated with preoperative spherical equivalent hypermetropia of more than 3.00 diopters, but not with a history of strabismus in close relatives. Accommodative esotropia appears to behave the same in pure and in mixed forms.
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