Incidence of Strabismus Post-Plaque Brachytherapy in Patients With Uveal Melanoma.

PURPOSE: To determine the incidence and type of strabismus in patients with uveal melanoma treated with plaque brachytherapy. DESIGN: Multicenter, retrospective incidence estimation study. METHODS: A total of 438 eyes of 438 patients with uveal melanoma treated with plaque brachytherapy between October 2011 and May 2021. Intervention was Iodine 125, and Palladium 103 plaque brachytherapy. The variables reviewed included incidence of nonresolving strabismus post-plaque brachytherapy, type of strabismus developed, extraocular muscles operated, and modality of treatment received. RESULTS: A total of 438 patients underwent plaque brachytherapy treatment for uveal melanoma. Eleven patients developed strabismus post-plaque brachytherapy (2.5%, n = 11/438). Of these patients, 5 (1.1%, n = 5/438) developed strabismus immediately postoperation. Specifically, 2 patients (0.5%, n = 2/438) developed strabismus immediately postoperation due to slipped muscles, 2 patients (0.5%, n = 2/438) due to decompensated phorias, and 1 patient (0.5%, n = 1/438) due to a fibrotic muscle. Six patients (1.4%, n = 6/438) developed late-onset sensory strabismus. A total of 355 patients (81.1%, n = 355/438) had their extraocular muscles disinserted during surgery, with the lateral rectus being the most common, accounting for 45.4% (n = 161/355), followed by the superior rectus at 26.8% (n = 95/355). Strabismus surgery was the most common treatment modality, comprising 72.7% (n = 8/11) of patients. CONCLUSIONS: The incidence of strabismus after plaque brachytherapy treatment for uveal melanoma was low and primarily classified as late-onset sensory strabismus. Previous studies may underestimate the long-term incidence of strabismus after plaque brachytherapy by focusing primarily on strabismus present immediately postoperatively.

Clinical study of the radiotherapy with EDGE accelerator in the treatment of the moderate and severe thyroid associated ophthalmopathy.

OBJECTIVE: To explore the short-term efficacy, acute complications and response factors after the radiotherapy with EDGE accelerator in patients with moderate and severe thyroid-associated ophthalmopathy (TAO). PATIENTS AND METHODS: A total of 68 patients with moderate and severe TAO who received the radiotherapy with EDGE accelerator between August 1st, 2017 and May 1st, 2011 were enrolled in the present study. The clinical data were collected, and the efficacy and acute complications were followed up, and the response factors were analyzed. RESULTS: Sixty-eight patients (136 eyes) were followed up for 6 months after radiotherapy. The total score after radiotherapy was significantly lower compared to that before the therapy (p<0.05), and the effective rate was 75.74%. After the radiotherapy, the patient's exophthalmia, soft tissue involvement, eye external muscle involvement, corneal involvement, decreased vision and diplopia, tearing and eyelid pain have improved. Acute complications included increased local inflammation, hair loss, pigmentation and xerophthalmias. In addition, multivariate logistic regression analysis demonstrated that thyroid hormone level was the independent factor for the response to the radiotherapy. CONCLUSIONS: For patients with moderate and severe TAO, radiotherapy with EDGE accelerator is a safe and effective treatment option. Maintaining normal thyroid hormone level can improve the effective rate of radiotherapy.

Vulvar Melanoma with Isolated Metastasis to the Extraocular Muscles: Case Report and Brief Literature Review.

BACKGROUND/AIM: Orbital metastasis of systemic cancer is exceedingly rare. This is a case report of a patient treated for locally recurrent vulvar melanoma who later presented with unilateral proptosis and was found to have an isolated biopsy-proven extraocular muscle metastasis. PATIENTS AND METHODS: A 94-year-old female with locally recurrent vulvar melanoma presented with eye discomfort and blurry vision. Patient underwent histopathological, genetic, and imaging studies. RESULTS: All prior work-up, including brain MRI and PET/CT, was negative for disease elsewhere from local recurrence. Orbital MRI demonstrated a mass involving the extraocular muscle, and immunohistochemistry staining of biopsy was consistent with metastasis. The patient underwent radiation therapy and tolerated treatment well. CONCLUSION: This is the first reported case of vulvar melanoma with extraocular muscle metastasis. The absence of findings on imaging as part of the staging work-up underscores the importance of considering extraocular muscle (EOM) metastasis as a differential for patients with vulvar melanoma who present with proptosis.

Surgical management of strabismus following choroidal melanoma plaque brachytherapy.

PURPOSE: To characterize intraoperative findings, surgical approach, and postoperative outcomes in patients undergoing strabismus surgery following plaque brachytherapy for ocular melanoma. METHODS: The records of all patients who underwent plaque brachytherapy for choroidal melanoma between May 2007 and June 2016 were reviewed retrospectively to identify those who subsequently required strabismus surgery. RESULTS: Of the 461 patients who underwent plaque brachytherapy during the study period, 13 (2.8%) met inclusion criteria. Visual acuity of the affected eye was 20/40 or better in 9 patients (69%). Preoperative horizontal deviation ranged from 0Δ to 52Δ; vertical deviation, from 2Δ to 25Δ. At final follow-up mean horizontal deviation ranged from 0 to 4Δ; vertical deviation, from 0Δ to 12Δ. Intraoperatively, all muscles directly adjacent to the treated area appeared macroscopically thicker than normal despite being functionally underacting. Magnetic resonance imaging showed enlarged muscles adjacent to the plaque radiotherapy. Microscopic examination of muscles in 2 patients showed reactive enlargement of the muscle fibers, granulation tissue, and inflammation. CONCLUSIONS: Persistent strabismus after plaque brachytherapy is rare. Typical findings include enlarged, underacting rectus muscles adjacent to the area of the plaque, restrictive connective tissue, and incomitant strabismus. Previously disinserted muscles may be found in abnormal locations. In this patient cohort scar tissue removal in conjunction with tightening procedures on the muscle adjacent to the plaque combined with recession of the antagonist muscle frequently resulted in good anatomical outcome.

Portable infrared pupillometry: a review.

Portable infrared pupillometers provide an objective measure of pupil size and pupillary reflexes, which for most clinicians was previously only a visual impression. But despite the fact that pupillometry can uncover aspects of how the human pupil reacts to drugs and noxious stimulation, the use of pupillometry has not gained widespread use among anesthesiologists and critical care physicians. The present review is an introduction to the physiology of pupillary reflexes and the currently established clinical applications of infrared pupillometry, which will hopefully encourage physicians to use this diagnostic tool in their clinical practice. Portable infrared pupillometry was introduced in 1989. The technology involves flooding the eye with infrared light and then measuring the reflected image on an infrared sensor. Pupil size, along with variables of the pupillary light reflex and pupillary reflex dilation, is calculated by the instrument and displayed on a screen immediately after each time-stamped measurement. Use of these instruments has uncovered aspects of how the human pupil reacts to drugs and noxious stimulation. The primary clinical applications for portable pupillometry have been in the assessment of brainstem function. Portable pupillometry is useful in the management of pain because it allows for assessments of the effect of opioids and in the titration of combined regional-general anesthetics.

Dystrophic changes in extraocular muscles after gamma irradiation in mdx:utrophin(+/-) mice.

Extraocular muscles (EOM) have a strikingly different disease profile than limb skeletal muscles. It has long been known that they are spared in Duchenne (DMD) and other forms of muscular dystrophy. Despite many studies, the cause for this sparing is not understood. We have proposed that differences in myogenic precursor cell properties in EOM maintain normal morphology over the lifetime of individuals with DMD due to either greater proliferative potential or greater resistance to injury. This hypothesis was tested by exposing wild type and mdx:utrophin(+/-) (het) mouse EOM and limb skeletal muscles to 18 Gy gamma irradiation, a dose known to inhibit satellite cell proliferation in limb muscles. As expected, over time het limb skeletal muscles displayed reduced central nucleation mirrored by a reduction in Pax7-positive cells, demonstrating a significant loss in regenerative potential. In contrast, in the first month post-irradiation in the het EOM, myofiber cross-sectional areas first decreased, then increased, but ultimately returned to normal compared to non-irradiated het EOM. Central nucleation significantly increased in the first post-irradiation month, resembling the dystrophic limb phenotype. This correlated with decreased EECD34 stem cells and a concomitant increase and subsequent return to normalcy of both Pax7 and Pitx2-positive cell density. By two months, normal het EOM morphology returned. It appears that irradiation disrupts the normal method of EOM remodeling, which react paradoxically to produce increased numbers of myogenic precursor cells. This suggests that the EOM contain myogenic precursor cell types resistant to 18 Gy gamma irradiation, allowing return to normal morphology 2 months post-irradiation. This supports our hypothesis that ongoing proliferation of specialized regenerative populations in the het EOM actively maintains normal EOM morphology in DMD. Ongoing studies are working to define the differences in the myogenic precursor cells in EOM as well as the cellular milieu in which they reside.

Orbital paraganglioma presenting as lateral rectus enlargement and its novel management: a case report and review of literature.

Paragangliomas of the orbit are extremely rare. We report on a case of paraganglioma manifesting as enlargement of the lateral rectus muscle. Magnetic resonance imaging (MRI) of the orbit showed typical salt and pepper appearance of the mass and pathologic examination was consistent with paraganglioma. The patient underwent surgery with total removal of lateral rectus muscle. Alignment was preserved by a half tendon transposition of the vertical rectus muscles to the insertion of the resected lateral rectus muscle. Isolated lateral rectus enlargement has not been previously reported as a manifestation of paraganglioma.

Protracted hypofractionated radiotherapy for Graves' ophthalmopathy: a pilot study of clinical and radiologic response.

PURPOSE: To evaluate the clinical and radiologic response of patients with Graves' ophthalmopathy given low-dose orbital radiotherapy (RT) with a protracted fractionation. METHODS AND MATERIALS: Eighteen patients (36 orbits) received orbital RT with a total dose of 10 Gy, fractionated in 1 Gy once a week over 10 weeks. Of these, 9 patients received steroid therapy as well. Patients were evaluated clinically and radiologically at 6 months after treatment. Clinical response assessment was carried out using three criteria: by physical examination, by a modified clinical activity score, and by a verbal questionnaire considering the 10 most common signs and symptoms of the disease. Radiologic response was assessed by magnetic resonance imaging. RESULTS: Improvement in ocular pain, palpebral edema, visual acuity, and ocular motility was observed in all patients. Significant decrease in symptoms such as tearing (p < 0.001) diplopia (p = 0.008), conjunctival hyperemia (p = 0.002), and ocular grittiness (p = 0.031) also occurred. Magnetic resonance imaging showed decrease in ocular muscle thickness and in the intensity of the T2 sequence signal in the majority of patients. Treatments were well tolerated, and to date no complications from treatment have been observed. There was no statistical difference in clinical and radiologic response between patients receiving RT alone and those receiving RT plus steroid therapy. CONCLUSION: RT delivered in at a low dose and in a protracted scheme should be considered as a useful therapeutic option for patients with Graves' ophthalmopathy.

Extraocular muscle involvement in marginal zone B-cell lymphomas of the orbit.

INTRODUCTION: Marginal zone B-cell lymphoma is the most frequent ocular adnexal lymphoma. It may involve the conjunctiva, lacrimal gland, eyelid, extraocular muscle, and orbital connective tissue. Extraocular muscle infiltration is rare. METHODS: We report here a series of 5 patients presenting with extraocular muscle infiltration due to marginal zone B-cell lymphoma, extracted from a retrospective study of 39 patients with primitive ocular adnexal marginal zone B-cell lymphoma presenting within a 15-year period, from 1993 to 2007, at two university hospitals. RESULTS: Out of 39 patients, two females and three males presented with extraocular muscle involvement (one levator muscle, one medial rectus muscle, one lateral rectus muscle, one inferior rectus muscle, and one inferior oblique muscle). In 4 cases, the right eye was involved. The median age of presentation was 60 years. Proptosis and diplopia were the main clinical signs. A mean duration of symptoms was 12 months before diagnosis was given. Three patients were stage IE at the diagnosis, according to Ann Arbor classification, and one was at stage IV. Three patients received radiotherapy and two received chemotherapy. Three patients underwent local relapses at a median time of 40 months. The patients with relapses were treated with chemotherapy alone in one case, radiotherapy alone in one case, and immunotherapy and chemotherapy in one case. The mean follow-up period was 54 months. DISCUSSION: When compared to other locations of marginal zone B-cell lymphomas of the orbit, extraocular involvement occurred in younger patients and had similar prognosis. CONCLUSION: Extraocular muscle involvement is a rare location of marginal zone B-cell lymphoma that had to be known and can simulate thyroid orbitopathy.

Rhabdomyosarcoma metastases to all extraocular muscles.

A 24-year-old woman developed acute bilateral proptosis. She had a history of rhabdomyosarcoma of the left orbit treated 2 years previously with chemotherapy and radiation. Computed tomography demonstrated enlargement of each of the extraocular muscles in both orbits. Extraocular muscle biopsy confirmed rhabdomyosarcoma. She was treated with radiation but died 2 months after presentation.

Prostate carcinoma metastasis to extraocular muscles.

Prostate carcinoma, when metastatic, typically involves bone and produces both osteoblastic and osteolytic changes. Orbital involvement is uncommon and extraocular muscle enlargement is a rare presentation of metastatic prostate adenocarcinoma. The authors present 2 patients with prostatic tumor metastasis to extraocular muscles. One patient had single-muscle involvement; the other presented with bilateral progressive proptosis, upper eyelid retraction, and bilateral multiple extraocular muscle enlargement mimicking thyroid-associated orbitopathy. Clinicians should be aware that, although rare, prostate cancer can involve the extraocular muscles.

Anterior segment ischemia after two-muscle surgery in a patient with radiation-treated nasopharyngeal carcinoma.

Anterior segment ischemia after strabismus surgery is a rare occurrence. Risk factors include single-stage surgery of three or more muscles in one eye, dysthyroid eye disease, and advanced age. The authors report a case of anterior segment ischemia after single-stage surgery of two horizontal muscles without previous reported risk factors. However, the patient had received radiotherapy for nasopharyngeal carcinoma more than 20 years ago. The case was successfully treated with systemic steroids but with reduced best-corrected visual acuity. Previous radiotherapy in the related orbital field may be a new risk factor for this condition.

Ultrastructural alterations in extraocular muscles following iodine-125 brachytherapy for uveal melanoma.

PURPOSE: This study investigated the ultrastructural changes in extraocular muscles under which radioactive plaques had been placed for the treatment of uveal melanoma. METHODS: At the time of plaque removal, biopsies were taken from four horizontal recti that had been left in situ over plaques and from one lateral rectus muscle that had been disinserted before brachytherapy. Normal lateral recti from enucleated eyes were used as controls. Iodine-125 seeds were used with a mean total activity of 54.04 mCi, remaining for an average of 149.62 hours over the sclera. RESULTS: Muscles that had been left in situ over the radioactive plaques demonstrated a focal decrease in muscular tissue and increased fibroblasts and collagen. Electron microscopy showed increased collagen, loss of sarcoplasmic reticulum and swollen mitochondria. The disinserted muscle in the plaque group appeared unaffected. CONCLUSIONS: Despite the theoretical shielding properties of plaques, leaving an extraocular muscle over the plaque may lead to several non-specific ultrastructural changes.

Strabismus in adults with uveal melanoma following episcleral plaque brachytherapy.

PURPOSE: To ascertain the incidence of persistent strabismus in patients treated with plaque brachytherapy and its subsequent treatment. METHODS: A single center retrospective case note review of adult patients with persistent diplopia or strabismus following plaque brachytherapy for all types of intraocular tumors between 1996 and 2004. RESULTS: A total of 929 consecutive adults underwent plaque brachytherapy during the study period at a single center. Sixteen patients (1.7%) with treated uveal melanoma developed persistent diplopia or strabismus. In 11 patients (69%) the timing of onset was in the first year, in 2 (13%) in the second year, and one each (6% each) in years 5, 7, and 8. Two patients (13%) did not require any intervention. Fourteen patients (88%) required treatment: 7 (50%) were treated with prisms only, 3 (21%) underwent botulinum toxin (BTXA) injections, and 4 (29%) were treated with extraocular muscle surgery (3 required one operation and one required 2 procedures). CONCLUSIONS: The incidence of ocular motility disorders following plaque brachytherapy in our cohort was 1.7% over 8 years and we include this in the consent process for conservative treatment of intraocular tumors. Options for treatment for persistent diplopia or strabismus include prisms, botulinum toxin injection, or surgery.

Orbital lymphoid tumour located within an extraocular muscle.

A rare case of an orbital lymphoid tumour within an extraocular muscle is presented. The tumour displayed features of a progressive indolent systemic lymphoma. There was no response to a trial of chemotherapy and so local radiotherapy was instituted. At follow up 2 months later there was no evidence of disease.

Ocular motility disturbances after episcleral plaque brachytherapy for uveal melanoma.

OBJECTIVE: To evaluate contributions of various factors in the development of strabismus after iodine-125 brachytherapy for uveal melanoma. METHODS: Twenty consecutive patients who underwent episcleral plaque brachytherapy for uveal melanoma underwent a full orthoptic examination before and after the surgery. Iodine-125 seeds at 5-mCi strengths were used with a mean total dose of 10.400 cGy toward the tumor apex. RESULTS: Average follow-up time was 25.4 months (range, 14 to 40). At 12 months all tumors showed regression, 9 patients had visual acuity of 20/200 or better, and 4 patients had ambulatory vision. After tumor treatment, 8 patients were orthophoric, 9 patients developed exotropia, 1 patient became hypertropic, 2 patients developed exotropia and hypertropia. Of 2 patients who developed significant diplopia, 1 received 10 U botulinum-toxin A injection (Botox, Allergan, Irvine, CA) for exotropia, and the other was managed with superior rectus advancement and superior oblique tenectomy for surgery-induced iatrogenic Brown's syndrome and slipped superior rectus muscle. CONCLUSION: Extensive mechanical injury, possible ionizing radiation of the plaques, and low visual acuity may cause strabismus after episcleral plaque brachytherapy. Diplopia and cosmesis could be improved with Botox injection or strabismus surgery.

Metastatic gastroesophageal junction adenocarcinoma to the extraocular muscles.

OBJECTIVE: To present one definitive and one probable case of gastroesophageal adenocarcinoma metastases to the extraocular muscles. DESIGN: Two observational case reports. METHODS: Two interventional case reports with clinical pathologic correlation and review of the literature. Vanderbilt University Institutional Review Board approval was obtained for this report. MAIN OUTCOME MEASURES: We analyzed the presenting symptoms, visual acuity, motility, and external examination. RESULTS: Computed tomography showed nodular enlargement of an isolated rectus muscle. Histopathologic examination confirmed the diagnosis of metastatic gastrointestinal carcinoma in one case. The orbital neoplasm contained signet ring cells, classically seen in gastrointestinal malignancy. CONCLUSIONS: Metastasis from gastrointestinal carcinoma should be considered in the differential diagnosis of a patient presenting with diplopia or visual loss, particularly with a recent history of gastrointestinal malignancy.

Ocular neuromyotonia: a case report.

Ocular neuromyotonia is a rare motility disorder occurring after tumor irradiation near the skull base or as a consequence of vascular abnormalities. Ocular myasthenia, convergence spasm and a cyclic third nerve palsy must be considered as differential diagnoses. The case of a 32-year-old woman suffering from intermittent diplopia six months after radiation therapy of a recurrent pituary gland adenoma is presented.