Acquired Ophthalmoplegia in Older Children and Adults.

Ophthalmoplegia or ophthalmoparesis are the terms given to paralysis or paresis of one or more of the extraocular muscles in one or both eyes. It may be representative of serious neurological or systemic disease. Other mechanisms may cause limitation of eye movement, including restrictive or myasthenic conditions, or combinations of several etiologies. Evaluating and differentiating the mechanisms causing ophthalmoplegia is important in determining the correct diagnosis and selecting the appropriate management in these complex cases.

Results of combined resection-recession on a single rectus muscle for incomitant deviations-an alternative to the posterior fixation suture.

PURPOSE: To compare outcomes of combined resection and recession on previously unoperated single horizontal or vertical rectus muscles in consecutive adult patients with acquired incomitant deviations minimal in primary gaze and maximal in an eccentric gaze position. METHODS: The surgical goal was to decrease the incomitance by 50%, expand the field of binocular single vision, and relieve diplopia. Recessions for the maximal deviations were combined with smaller resections using hang-back, nonadjustable sutures. Pre- and postoperative incomitance was compared using the paired t test. RESULTS: A total of 16 adults were studied (mean age at surgery, 49.3 ± 17.5 years), 12 with incomitant vertical deviations and 4 with incomitant horizontal deviations. Eight patients had preoperative deviations of <2Δ in primary gaze (mean, 9.2Δ ± 10.5Δ; range, 1Δ-35Δ). The mean maximum eccentric gaze deviation was 21.4Δ ± 9.9Δ (range, 10Δ-48Δ). Postoperatively, 12 patients (75%) had a decrease in incomitance of >50%, and all had expansion of the field of binocular single vision. Improvement in postoperative incomitance was highly statistically significant in the vertical incomitant group (P < 0.0001) but not statistically significant in the horizontal group (P = 0.39). The technique corrected downgaze deviations in 4 patients with canine tooth syndrome (93% ± 3.3, P < 0.0001) without worsening the hypotropia in upgaze. CONCLUSIONS: Combined resection-recession single muscle surgery significantly reduces incomitance with minimal effect on primary gaze. It is most effective for treating vertical deviations worse on downgaze with primary gaze deviations of <2Δ and for canine tooth syndrome; the technique was less successful in reducing horizontal incomitance and in cases involving gaze palsies and nystagmus.

Effectiveness of Bilateral Lateral Rectus Resection for Residual Esotropia in Dysthyroid Ophthalmopathy.

PURPOSE: To report one surgeon's experiences with bilateral lateral rectus resections in dysthyroid ophthalmopathy patients with residual esotropia after initial bilateral medial rectus recession. DESIGN: Retrospective interventional case series evaluating outcomes. METHODS: Medical records for patients with dysthyroid ophthalmopathy who underwent bilateral lateral rectus resections for persistent esotropia by a single surgeon from June 2012 to June 2015 were retrospectively reviewed. All patients had residual esodeviations following initial bilateral medial rectus recession. The goal of surgery was to obtain fusion in primary gaze and the reading position without prism, with a postoperative deviation equal to or less than 8Δ at distance and a phoria at near without diplopia. RESULTS: Seven of the 9 patients were successful in achieving this goal. Preoperative esotropia before resection ranged from 12Δ to 30Δ (23.1Δ ± 10.3Δ) at distance and -2Δ to 40Δ (14.9Δ ± 12.3Δ) at near. No exodeviation was created at near by the resections, but 1 patient had an asymptomatic exophoria both pre- and postoperatively. CONCLUSION: Patients with large horizontal misalignment may have residual esodeviations that are too large for correction with recession alone. Given our findings, we believe resection may be an effective tool in resolving esotropia in certain patients with restrictive strabismus.

Acquired nonparalytic causes of superior oblique dysfunction.

BACKGROUND AND PURPOSE: To conduct a retrospective study to investigate the causes of acquired superior oblique dysfunction, excluding paralysis, in a consecutive series of adult patients and to compare presenting symptoms and clinical findings. METHODS: A retrospective review of all adult patients with superior oblique dysfunction between the ages of 18 and 80 who met the study profile was conducted at Saint Louis University Medical Center between January 2000 and April 2012. The presenting symptoms, clinical findings, and treatment course for each patient was recorded. The study was approved by the Institutional Review Board of our institution. RESULTS: Acquired forms of nonparalytic superior oblique dysfunction were identified in forty-eight patients. These included superior oblique myokymia (twenty-three patients), superior oblique click syndrome or variable Brown syndrome (nine), canine tooth syndrome (five), spontaneous acquired Brown syndrome (four), iatrogenic or traumatic Brown syndrome (four), and ocular neuromyotonia affecting the superior oblique (three). CONCLUSIONS: Several nonparalytic entities were identified that caused superior oblique dysfunction. Clinical findings may be similar despite entirely different mechanisms. Subjective symptoms may be difficult for the patient to describe or for the examiner to elicit on the day of the examination. Specific techniques can be used in eliciting, differentiating, and documenting the conditions. These included trochlear palpation, modified head tilt technique, interpretation of torsion, and Hess charts.

Cyclic (alternate day) vertical deviation--possible forme fruste of ocular neuromyotonia.

PURPOSE: Cyclic ocular deviations are relatively uncommon and are seldom seen in adults. We report 3 adult patients with cyclic hypotropia that has clinical characteristics similar to neuromyotonia, suggesting a possible common etiology. METHODS: Three consecutive patients with 48-hour cyclic hypotropia underwent full neuro-ophthalmologic, oculoplastics, and orthoptic evaluations as well as appropriate medical and neurologic studies. Examinations were arranged on consecutive days on multiple visits to document the cyclic pattern. RESULTS: All 3 patients had sustained contraction of a vertically acting extraocular muscle lasting 24 hours and demonstrated characteristics of ocular neuromyotonia. The contraction was absent for the next 24 hours. Two of the patients had thyroid eye disease; the third patient had unilateral ophthalmoparesis and had subsequent frameless robotic radiosurgery for a cavernous sinus schwannoma. Regular cycles lasting 6, 9, or 14 months were documented by all 3 patients. In 2 patients, treatment with carbamazepine and gabapentin effectively reduced or eliminated the cycle. The cyclic deviation in the third patient resolved spontaneously. CONCLUSIONS: On the basis of the response of these patients to membrane-stabilizing medications and the behavior noted as the cycle broke each day, we propose that cyclic vertical strabismus and ocular neuromyotonia may be related conditions with similar underlying physiology.

Ocular neuromyotonia: differential diagnosis and treatment.

Ocular neuromyotonia (ONM) is a rare but distinctive clinical entity characterized by involuntary episodic contraction of one or more muscles supplied by the ocular motor nerves. A retrospective review was conducted on all patients with ONM seen by the neuroophthalmology service in the past 20 years. Ten patients were identified with ONM; six affecting vertical muscles (superior oblique; inferior rectus; superior rectus) and four affecting lateral rectus muscles. Case 1 has been reported previously. Most episodes occurred every 10-40 min, lasted a few seconds to several minutes, and were repeated throughout the day. Only two patients had previously undergone cranial radiation. Two had thyroid eye disease. One patient presented with superior oblique myokymia and subsequently developed ONM. Membrane stabilizing medications were prescribed in 7 of the 10 patients with varied success. ONM episodes ceased after extraocular muscle surgery in one patient with thyroid eye disease.

Management of vertical ocular deviations secondary to restrictive conditions.

BACKGROUND AND PURPOSE: Management of vertical strabismus secondary to restrictive conditions can be challenging for both the patient and practitioner. The purpose of this paper is to identify both common and uncommon causes of vertical restrictive strabismus, to discuss the evaluation of these conditions, and to briefly review the etiology and recommendations for surgical management. METHOD: Current literature was reviewed as well as the surgical techniques that have been found to be most useful from experience over the past twenty years of practice. CONCLUSION: Surgical realignment and elimination of diplopia is both challenging and problematic. Surgery should be designed to relieve restriction, with attention to identifying and managing incomitance. Furthermore, it is important to establish realistic goals with patients.

Thyroid eye disease: honing your skills to improve outcomes.

Thyroid eye disease affects the eyelids, orbital compartment, and extraocular muscles, resulting in a highly variable degree of chemosis and enlargement of the preorbital fat pads, eyelid retraction, proptosis, restrictive strabismus, torticollis, and, rarely, compressive or congestive optic neuropathy. Although most patients with thyroid eye disease are best treated conservatively, those more severely affected may benefit from orbital decompression, strabismus surgery, or eyelid retraction repair after stabilization has occurred. Botulinum A toxin, high-dose intravenous corticosteroids, and radiation treatment are therapeutic options in select cases. Compressive or congestive optic neuropathy and severe corneal exposure warrant consideration of surgical intervention on an urgent basis without waiting for stabilization. Epidemiology and risks and benefits of high-dose steroids and radiation therapy are reviewed along with recommendations to improve conservative as well as surgical management of this disease. Strategies to manage strabismus and optimize outcomes are provided.

The influence of the vergence system on strabismus diagnosis and management.

The vergence system is an important element in human eye movement control. It comprises horizontal, vertical, and torsional components, the largest of which is convergence. Vergence performance is largely involuntary, although voluntary effort can influence convergence. Vergence function can be damaged by trauma or organic disease and, because convergence can be exerted voluntarily, it is susceptible to purposeful subjective disturbances. Vergence function is important in the maintenance of binocular control, being synonymous with motor fusion. The most common vergence dysfunction affects convergence and may be exhibited as an excess or weakness such as convergence spasm or paralysis. Divergence dysfunction generally presents with diminished function such as divergence weakness; vertical and cyclovergence disturbances are rare. An update is given on the neuroanatomical areas that subserve the vergence system. Clinical examples of the most common vergence disorders are presented. The influence of the vergence system on the diagnosis and management in common and less well-known clinical entities is described and discussed.

Results of extraocular muscle surgery in WEBINO bilateral internuclear ophthalmoplegia patients.

PURPOSE: To evaluate the results of extraocular surgery in patients with large exotropias from "wall-eyed" bilateral internuclear ophthalmoplegia (WEBINO). METHODS: Eight patients with WEBINO evaluated between November 1994 and October 2006 underwent extraocular muscle surgery. Three patients required a second procedure. RESULTS: An 85% reduction in exodeviation occurred postoperatively. Fusion responses were demonstrable in 87.5% of patients. Other ocular signs of internuclear ophthalmoplegia (INO) such as adduction limitation, abducting nystagmus, and ocular dysmetria also improved. These results were obtained following two-staged unilateral recession-resection procedures in seven patients; the remaining patient underwent bilateral lateral rectus recessions. CONCLUSIONS: Binocular outcomes are achievable in patients with WEBINO following extraocular muscle surgery, and associated ocular signs may be reduced or eliminated.

Neurological significance of small esodeviations.

A small esodeviation detected in an adult may represent an acquired event caused by a number of different mechanisms. These deviations may be comitant or incomitant, occur with or without diplopia and appear in association with other ocular or neurological findings or in isolation. The mechanisms include lateral rectus dysfunction, supranuclear gaze disturbances, anomalies in the vergence system, the influence of the near synkinesis and certain restrictive components. The presence of a small, acquired esodeviation may have neurological significance that may influence diagnosis and management decisions.1,2 Conditions such as divergence paralysis and convergence spasm are described in the classic literature with large esodeviations and obvious clinical features.3-7 They are not a common occurrence in most strabismus practices but are recognized readily when they occur. More subtle presentations of these entities are seen far more frequently and may be so small, or co-exist with other ocular findings, as to be overlooked. It is likely that with many clinical disorders like divergence paresis and convergence spasm that they present as a spectrum of dysfunction; it is the small end of that spectrum that will be discussed.