Chemodenervation of extraocular muscles with botulinum toxin in thyroid eye disease.

BACKGROUND: Thyroid eye disease (TED) presents a management dilemma for strabismologists due to the variability of its clinical course. Prisms may be prescribed to relieve diplopia in small deviations. Surgical intervention, on the other hand, should not be done until the active phase of the disease has subsided. We report our experience with chemodenervation utilizing botulinum toxin (BT) injection in the management of TED-related strabismus. METHODS: A retrospective chart review was done on twenty-two (22) consecutive patients receiving BT injections at the University of California, San Diego (UCSD) Thyroid Eye Center. All BT injections were administered by a single physician under electromyographic guidance. RESULTS: The clinical records of 22 patients (18 females) were reviewed. Seven patients (32 %) had a reduction of their deviation to a point where surgery was not required. In six patients (27 %), surgery was required but an improvement in ocular deviation was found, altering the original surgical plan. In four patients (18 %), the deviation continued to progress after BT injection. Success rates were higher if pre-treatment deviation was less than 20 prism diopters ((∆)). CONCLUSION: One third of the chemodenervation-treated patients avoided surgical intervention, with an additional 27 % (total of 40 % of those who needed surgery) having a reduced deviation prior to surgery. Using BT injection to extraocular muscles to treat diplopia in TED patients is most effective in preventing surgery in those patients with 20(∆) or less of deviation.

A multicenter analysis of the ophthalmic knowledge assessment program and American Board of Ophthalmology written qualifying examination performance.

OBJECTIVE: To compare the performance on the American Board of Ophthalmology Written Qualifying Examination (WQE) with the performance on step 1 of the United States Medical Licensing Examination (USMLE) and the Ophthalmic Knowledge Assessment Program (OKAP) examination for residents in multiple residency programs. DESIGN: Comparative case series. PARTICIPANTS: Fifteen residency programs with 339 total residents participated in this study. The data were extracted from the 5-year American Board of Ophthalmology report to each participating program in 2009 and included residency graduating classes from 2003 through 2007. Residents were included if data were available for the USMLE, OKAP examination in ophthalmology years 1 through 3, and the WQE score. Residents were excluded if one or more of the test scores were not available. METHODS: Two-sample t tests, logistic regression analysis, and receiver operating characteristic (ROC) curves were used to examine the association of the various tests (USMLE, OKAP examination year 1, OKAP examination year 2, OKAP examination year 3, and maximum OKAP examination score) as a predictor for a passing or failing grade on the WQE. MAIN OUTCOME MEASURES: The primary outcome measure of this study was first time pass rate for the WQE. RESULTS: Using ROC analysis, the OKAP examination taken at the third year of ophthalmology residency best predicted performance on the WQE. For the OKAP examination taken during the third year of residency, the probability of passing the WQE was at least 80% for a score of 35 or higher and at least 95% for a score of 72 or higher. CONCLUSIONS: The OKAP examination, especially in the third year of residency, can be useful to residents to predict the likelihood of success on the high-stakes WQE examination.

Radiographic analysis of extraocular muscle volumetric changes in thyroid-related orbitopathy following orbital decompression.

PURPOSE: To evaluate radiographic volume changes in extraocular muscles (EOM) following orbital decompression for thyroid-related orbitopathy (TRO). METHODS: Medical records of 22 orbits in 12 patients undergoing postoperative orbital CT after orbital decompression for TRO were retrospectively reviewed. All orbits demonstrated no signs of clinical reactivation of TRO. EOM volumes were determined by the summation of each EOM's cross-sectional area in the coronal plane of the CT scans and multiplying the sum by the slice thickness. Main outcome measure was a comparison of EOM volumes preoperatively and postoperatively. RESULTS: All orbits demonstrated proptosis reduction postoperatively with a mean of 4.8 mm +/- 2.1 mm (p < 0.0001). The significant increase in the medial rectus muscle (p = 0.0010) postoperatively accounted for the primary change in the postoperative enlargement of total EOM volume (p = 0.028). The medial rectus muscle increased 27% from the preoperative volume. The lateral rectus, superior rectus/levator complex, inferior rectus, and superior oblique muscles did not demonstrate a significant volume increase postoperatively (p = 0.23, 0.21, 0.17, 0.40, respectively). Two patients who underwent unilateral orbital decompression demonstrated EOM enlargement postoperatively in the operative orbit only. CONCLUSIONS: There is a significant increase in medial rectus muscle volume postoperatively in orbits undergoing orbital decompression for TRO despite lacking clinical evidence of disease reactivation.

Combined eyelid and strabismus surgery: examining conventional surgical wisdom.

PURPOSE: To determine whether satisfactory results of both eyelid surgery and strabismus surgery can be obtained when these procedures are performed in the same setting in selected patients. METHODS: Nine patients (16 eyelids) who had undergone surgery in the same setting for eyelid malposition and strabismus of the horizontal rectus muscles, with or without concurrent vertical rectus muscle surgery, were retrospectively reviewed. Eyelid malposition surgery success was defined as a postoperative margin reflex distance (MRD1) within 1 mm of the target. Strabismus surgery success was defined as a postoperative deviation within 8 prism diopters of orthophoria or a decrease in binocular diplopia if the patient had a less than 8 prism diopters deviation in primary gaze preoperatively. RESULTS: Eyelid retraction repair was performed on 13 upper eyelids, levator advancement was performed on 2 eyelids, and frontalis suspension was performed on 1 eyelid. Four patients underwent bilateral medial rectus recession and the remaining 5 patients received varying strabismus surgery. Four patients received adjustable sutures. Mean follow-up was 11 months. Postoperatively, 13 of 16 eyelids (81%) achieved an MRD1 within 1 mm of the target. Eight of 9 patients (89%) demonstrated satisfactory strabismus correction. CONCLUSION: Combined eyelid malposition and strabismus surgery can be successfully performed in selected cases, particularly when the strabismus surgery involves the horizontal rectus muscles. Combined surgery is not recommended in cases that do not involve the horizontal rectus muscles.

Effect on intraocular pressure of extraocular muscle surgery for thyroid-associated ophthalmopathy.

PURPOSE: To study the effect of extraocular muscle surgery on intraocular pressure (IOP) in patients with thyroid-associated ophthalmopathy. DESIGN: Retrospective, observational case series. METHODS: The medical records of patients with restrictive myopathy secondary to thyroid-associated ophthalmopathy who underwent strabismus surgery from July 1, 1997 through July 31, 2003 were reviewed and analyzed retrospectively. Seventeen patients met the criteria and were included in this study. All patients were seen at the Thyroid Eye Center at the University of California, San Diego, a university-based tertiary referral center. The main outcome measure was IOP readings obtained before and after surgery in both primary gaze and upgaze. RESULTS: A statistically significant decrease in IOP in upgaze was noted after extraocular muscle recession. The mean IOP before surgery was 16.6 +/- 3.78 mm Hg in primary gaze and 23.2 +/- 7.27 mm Hg in upgaze. After strabismus surgery, the mean IOP after one month was 15.7 +/- 2.36 mm Hg (P = .215) in primary gaze and 18.9 +/- 2.96 mm Hg in upgaze (P = .001). CONCLUSIONS: Strabismus surgery resulted in a significant reduction in IOP in the early postoperative period in patients with restrictive myopathy secondary to thyroid-associated ophthalmopathy.

Optic disc melanocytoma and glaucoma.

An 82-year-old man presented with optic disc melanocytoma and glaucomatous optic neuropathy. The coexistence of these findings is particularly challenging, as there is no specific means for differentiating whether the optic nerve damage is glaucoma- or melanocytoma-related.

Psychological disturbance in graves ophthalmopathy.

OBJECTIVE: To study mood disturbance in Graves ophthalmopathy. METHODS: Forty-eight patients (mean age, 55 years; 40 women and 8 men) with Graves ophthalmopathy from a university-based referral center were classified into two groups, 24 with moderate/severe disease (study group) and 24 with negligible/mild disease (control group). The groups were matched with regard to demographic and medical characteristics. All participants completed a mood survey to assess differences in degree of emotional distress. MAIN OUTCOME MEASURE: The Profile of Mood States survey, a 65-item self-reported inventory designed to assess emotional distress, was the primary outcome measure. A total mood disturbance score was assigned by summing the scores derived on the 6 subscales of the survey--tension, depression, vigor, confusion, fatigue, and anger. RESULTS: Analysis of variance revealed that patients with moderate/severe Graves ophthalmopathy showed significantly greater emotional distress than patients with mild/negligible Graves ophthalmopathy on the Profile of Mood States mean total score (P<.001). Additionally, patients who had disfigurement (proptosis) as the predominant clinical feature had significantly elevated emotional distress compared with the control group (P = .01), whereas no significant difference was detected between the control group and patients with diplopia as the predominant clinical feature (P = .20). CONCLUSION: Patients with moderate to severe Graves ophthalmopathy have significant mood disturbance, especially when disfiguring signs are predominant. We propose that evaluation of the psychological burden of the disease should be considered in routine follow-up and in decisions regarding treatment.

Botulinum A toxin injection for restrictive myopathy of thyroid-related orbitopathy: effects on intraocular pressure.

PURPOSE: To study the effect of extraocular muscle injections of botulinum A toxin on intraocular pressure in patients with thyroid-related orbitopathy. DESIGN: Retrospective observational case series. METHODS: The medical records of eight consecutive patients with restrictive myopathy secondary to thyroid related orbitopathy (TRO) who underwent botulinum A toxin injection from December 1997 to December 1998 were reviewed and analyzed retrospectively. All patients were seen at the University of California, San Diego (UCSD) Thyroid Eye Center, a university-based tertiary referral center. The main outcome measure was intraocular pressure (IOP) readings taken before and after injection in both primary gaze and upgaze (involving one eye in seven of the patients and both eyes in one patient). Intraocular pressure readings were measured by an unmasked physician using a Goldmann applanation tonometer. RESULTS: A statistically significant decrease in IOP in upgaze was noted 2 to 6 weeks following botulinum A toxin injection and in both fields of gaze (primary and upgaze) after 2 to 4 months. The mean IOP before injection was 21.4 +/- 3.0 mm Hg in primary gaze and 29.9 +/- 9.7 mm Hg in upgaze. The mean IOP, following injection at 2 to 6 weeks, was 19.2 +/- 4.2 mm Hg (P <.095) in primary gaze and 25.1 +/- 5.9 mm Hg (P <.023) in upgaze. At 2 to 4 months following injection, the mean IOP was 19.3 +/- 3.9 mm Hg (P <.044) in primary gaze and 27.7 +/- 8.5 mm Hg (P <.024) in upgaze. Six patients indicated improved ocular deviation, which was associated with a lowering of IOP. Two patients indicated no change in IOP or strabismic deviation following botulinum A toxin injection. CONCLUSIONS: Botulinum A toxin injections cause a secondary effect to lower IOP in patients with restrictive strabismus associated with thyroid-related orbitopathy.

When the darker eye has the smaller pupil.

Waardenburg and congenital Horner syndromes are both recognized causes of congenital hypochromic iris heterochromia. Each has been linked to disruptions in the pathway of tyrosinase induction, thus leading to a deficiency in melanin production of the iris. These syndromes must be considered in the differential diagnosis of a patient presenting with heterochromia iridis. We present the case of a 20-month old boy afflicted with both congenital Horner syndrome and Waardenburg syndrome, type II. In contrast to the more common presentation of congenital Horner syndrome, the affected iris in this case was the darker of the two because of the effects of the concomitant Waardenburg syndrome on the contralateral iris pigmentation. We are unaware of any other cases presenting with both Horner and Waardenburg syndromes and believe that this case serves as an excellent opportunity to briefly review the pathophysiology involved with these disorders.

The delayed cost of treatment.

A 78-year-old woman presented with acute decreased vision in both eyes. She had been treated for a pituitary mass with a total of 4,500 centigray of external beam radiation 8 months prior to presentation. She was diagnosed with radiation optic neuropathy. Treatment with hyperbaric oxygen and intravenous steroids were initiated but vision remained poor.

Reduction of orbital inflammation following decompression for thyroid-related orbitopathy.

PURPOSE: Thyroid-related orbitopathy (TRO) is associated with inflammation, expansion of orbital fat, enlargement of extraocular muscles, and optic neuropathy (ON). We examined the effects of orbital decompression on the inflammatory and congestive signs of TRO in patients who underwent emergent orbital decompression. METHODS: This retrospective, consecutive study included patients with ON from TRO who underwent orbital decompression. Pre- and postoperative orbital inflammatory signs in the operated and nonoperated, contralateral eyes were graded with the 10-item clinical activity score (CAS). RESULTS: Thirty-one orbits were included. Postoperatively, 22 patients and 29 orbits had resolution of ON while the remaining 2 patients had improvement in visual acuity. Mean preoperative CAS was 9.5 ± 0.4. At 12 months, postoperative CAS was 2.1 ± 0.6 (P < 0.01) in the operated eye and 3.2 ± 0.5 (P < 0.05) in the nonoperated, contralateral eye. CONCLUSION: In our series, 94% of orbits had resolution of ON. There was also a statistically significant postoperative reduction in the CAS in both the operated and nonoperated, contralateral eyes. This phenomenon may be due to lowered venous congestion, decreased intraorbital pressure, and diminution in inflammatory factors.

Optic neuropathy associated with botulinum A toxin in thyroid-related orbitopathy.

PURPOSE: To report the development of optic neuropathy after botulinum A toxin injection for restrictive myopathy from thyroid-related orbitopathy (TRO). METHODS: We retrospectively reviewed the records of three patients with TRO who underwent botulinum A toxin injection for restrictive myopathy and subsequently developed optic neuropathy. Development of optic neuropathy was measured by visual acuity, color vision testing, visual field testing, and relative afferent pupillary testing. RESULTS: At 3 week follow-up after botulinum A toxin injection, three patients were noted to have clinical signs and symptoms of optic neuropathy in the ipsilateral eye following injection of botulinum A toxin for restrictive myopathy. Treatment with oral steroids followed by orbital wall decompression reversed the optic neuropathy. CONCLUSIONS: To our knowledge, this is the first report of optic neuropathy associated with botulinum A toxin injection in TRO. Clinicians should be aware of this potential vision threatening complication.

Change in proptosis following extraocular muscle surgery: effects of muscle recession in thyroid-associated orbitopathy.

PURPOSE: To evaluate the effect of strabismus surgery on proptosis in patients with thyroid-associated orbitopathy. METHODS: The medical records of 22 consecutive patients with thyroid-associated orbitopathy undergoing strabismus surgery were reviewed. Data pertaining to the number of muscles operated on, amount of muscle recession, prior orbital decompression, and exophthalmometry were evaluated. RESULTS: Thirty-eight eyes in 22 patients with thyroid-associated orbitopathy were studied before and after strabismus surgery. The mean change in exophthalmometry following strabismus surgery in all eyes was +0.6 mm (p < 0.01). Eyes with prior decompression averaged a 0.9 mm increase following strabismus surgery (p < 0.01); those without decompression averaged a 0.2 mm decrease (p = 0.658). In eyes that underwent two rectus muscle recessions the increase in Hertel measurements averaged 1.2 mm; when only one muscle was recessed, the average increase was 0.2 mm. In the eyes with muscle recession < or =5 mm, the mean exophthalmometric increase was 0.7 mm. When a muscle recession of more than 5 mm was performed, the exophthalmometry showed a mean increase of 0.5 mm. CONCLUSIONS: Strabismus surgery on patients with thyroid-associated orbitopathy can worsen proptosis, especially in those with prior decompression. When planning for orbital decompression, the surgeon should consider this effect. Moreover, patients should be made aware of the possible changes to their appearance.

Graded orbital decompression based on severity of proptosis.

OBJECTIVE: To study the results of orbital decompression based on the severity of preoperative proptosis. DESIGN: A retrospective noncomparative interventional case series. PARTICIPANTS: Thirty-nine orbits in 23 patients with thyroid-related orbitopathy at a university-based referral center. INTERVENTION: Graded orbital decompression was performed in all patients based on the severity of preoperative exophthalmometry. MAIN OUTCOME MEASURES: Exophthalmometry, visual acuity, margin-to-reflex distance, prism cover testing, and intraocular pressure. RESULTS: Mean proptosis reduction in all orbits was 6.4 +/- 2.7 mm (P < 0.01). In group 1 (preoperative exophthalmometry <22 mm), proptosis decreased with a mean of 4.8 +/- 1.3 mm (P < 0.01); mean proptosis reduction was 6.0 +/- 2.3 mm (P < 0.01) and 8.9 +/- 3.4 mm (P < 0.01) in group 2 (exophthalmometry between 22-25 mm) and group 3 (exophthalmometry >25 mm), respectively. In four of five eyes with compressive optic neuropathy there was an improvement of best-corrected visual acuity of 2 lines or more. Margin-to-reflex distance of the upper and lower lids and intraocular pressure were reduced in all groups. New-onset diplopia developed in two patients (8.7%); 13 of 15 patients (86.7%) who had diplopia preoperatively had persistent diplopia postoperatively. Two patients (13.3%) had relief of diplopia postoperatively. CONCLUSIONS: Graded orbital decompression based on the severity of preoperative exophthalmometry is useful to determine the type and amount of orbital surgery to be performed.