Teprotumumab for thyroid eye disease in patients with hypothyroid/euthyroid state: a multicenter case series.

BACKGROUND: Teprotumumab, a novel IGF-1R antibody was recently shown to significantly reduce the signs of acute and chronic thyroid eye disease (TED) related to hyperthyroidism. Given the lower incidence of TED associated with hypothyroidism / euthyroidism, there is a paucity of data regarding the efficacy of teprotumumab in this group. METHODS: In this multicenter study, consecutive patients who had been diagnosed with TED, presenting with either hypothyroidism or euthyroidism as their baseline thyroid dysfunction and treated with teprotumumab were included. All patients had measurements of proptosis, clinical activity scores (CAS), diplopia scores and four-point strabismus scores before and after therapy. RESULTS: Twenty-six patients met the inclusion criteria. Mean age was 48 ± 14 years old and mean duration of TED prior to treatment was 31 ± 43 months. All patients received 8 infusions. Mean (SD) reduction in proptosis for study orbits was 2.7 mm (1.8) (p < 0.05) and 1.8 mm (2.0) for the fellow orbit (p < 0.05). In the study orbit, mean (SD) CAS was 2.3 (1.3) before therapy and 1.0 (1.0) following therapy (p < 0.05). At baseline, mean (SD) diplopia score was 1.2 (1.1) and 0.9 (1.1) following therapy (p < 0.05). CONCLUSION: Teprotumumab reduces proptosis and inflammation in patients presenting with TED associated with hypothyroidism and euthyroidism. The results of this study highlight the potential for teprotumumab therapy in this subgroup and also provide a unique insight into the potential role of the IGF-1R in these patients.

Thyroid eye disease with choroidal folds.

Purpose: To determine the clinical course of patients with chorioretinal folds (CRF) in thyroid eye disease (TED).Methods: A multi-center retrospective case series of patients with TED who developed CRF.Results: Ten patients (17 eyes) with CRF related to TED were identified. The mean age of presentation was 59.3 ± 8.3 years old. The majority of patients were male (70%), hyperthyroid (70%), hyperopic (53%), had a history of radioactive iodine (60%), and currently on methimazole treatment (30%). Three patients (3 eyes) had unilateral involvement of CRF with bilateral TED. The average clinical activity score was 3.6 ± 2.1 at the time of presentation. The most commonly enlarged extraocular muscles were medial (76%), inferior (64%), superior (64%) and lateral rectus (35%). Compressive optic neuropathy was seen in 47% of eyes. Treatment included oral prednisone (70%), orbital decompression (59%), thyroidectomy (20%) and tocilizumab (10%). The CRF did not resolve over a follow up period of 24.7 ± 23.7 months in 70% of eyes. There was no significant difference in average axial length (25.7 ± 4.9 mm) and optic nerve to optic strut distance (37.8 ± 3.9 mm) between patients with CRF and the eight age-and sex-matched TED control patients without CRF (p = 0.81 and 0.65 respectively). A univariable and multivariable analysis found an enlarged inferior rectus as a factor in TED patients with persistent CRF.Conclusions: CRF are often an indicator of visually threatening situations and often do not resolve despite treatment of TED.

Ophthalmic Plastic Surgery in Patients 100 Years and Older.

PURPOSE: The centenarian population is growing and ophthalmic plastic surgeons are providing care to an increasing number of elderly patients. Outcomes of centenarians have not been previously studied in the ophthalmic plastic surgery literature. The goal of the current review was to examine the baseline characteristics, surgical problems, and outcomes of this select group of patients. METHODS: A retrospective chart review was performed. Patients who underwent ophthalmic plastic surgery at age 100 or older between January 2000 and June 2016 by a member of the New England Oculoplastics Society were included in the study. RESULTS: Fifteen patients met inclusion criteria. The majority (66%) were female. More than half (60%) presented with a surgical problem of an urgent nature. Most disorders involved the lacrimal system or eyelids, and many were the result of trauma or infection. There were no cases of orbital tumor or thyroid eye disease. There were no surgical or anesthesia-related complications. Most patients (80%) had no documented history of dementia, and only 1 was diabetic. Notably, 33% of patients presented with no light perception vision in at least 1 eye. CONCLUSIONS: Ophthalmic plastic surgery can be performed safely in select patients 100 years of age and older. Formal prospective studies are needed to improve surgical care in this group.

Eyelash resection procedure for severe, recurrent, or segmental cicatricial entropion.

PURPOSE: To study the safety, efficacy, and cosmetic outcome of the eyelash resection procedure for treatment of severe, recurrent, or segmental cicatricial entropion. METHODS: Retrospective consecutive case series of patients with severe, recurrent, or segmental cicatricial entropion treated with eyelash resection at the Moran Eye Center and the University of Vermont. Investigators performed chart reviews of these patients and evaluated effectiveness of the treatment and outcome data, including age, gender, diagnoses, method of repair, recurrence of trichiasis, and cosmetic satisfaction. There were no exclusionary characteristics specified in the study. RESULTS: A total of 26 eyelids were operated on in 5 male and 11 female patients. The mean age was 74 years, with the following diagnoses: idiopathic (6), ocular cicatricial pemphigoid (2), postoperative (2), ocular pseudopemphigoid (drug related) (1), graft-versus-host disease (1), Stevens-Johnson syndrome (1), trachoma (1), linear IgA bullous dermatosis (1), and trauma (1). Mean postoperative follow-up was 13 months. The functional success rate was 90.5%, and the cosmetic success rate was 100%. CONCLUSION: The eyelash resection procedure is a safe, effective, and cosmetically acceptable procedure for treatment of severe, recurrent, or segmental cicatricial entropion.

Blood-tinged stools and blood-shot eyes.

A 37-year-old woman developed red painful eyes, diarrhea, and painful oral and genital ulcerations. Optic disk swelling was noted bilaterally. Colonoscopy was significant for granulomatous lesions consistent with Crohn disease. Epidemiologic factors, diagnostic criteria, and differentiating characteristics for Behcet disease and Crohn disease are discussed.

Ocular myasthenia gravis.

Myasthenia gravis may be limited solely to the eyes (ocular myasthenia) or may have systemic manifestations (generalized myasthenia). Most patients display blepharoptosis or ophthalmoparesis, resulting in diplopia, but the ocular motility disorder may take many forms. The diagnosis is often elucidated by variable muscle weakness with fatigability noted in the history and on examination. The ice test has supplanted the need for the edrophonium (Tensilon) test in many cases of ocular myasthenia. Therapy may be challenging, because many ocular myasthenics do not achieve significant clinical improvement with acetylcholinesterase inhibitors alone. Immunomodulatory treatment,such as systemic corticosteroids, is frequently effective, and emerging data suggest that this type of therapy may reduce the likelihood of ocular myasthenia becoming generalized.

Müller's muscle-conjunctival resection for blepharoptosis with poor levator function.

We describe a patient with blepharoptosis, poor levator function, and a positive phenylephrine test who responded favorably to Müller's muscle-conjunctival resection, alleviating the need for a frontalis suspension ptosis repair in the presence of very deep superior sulci.

Snip punctoplasty offers little additive benefit to lower eyelid tightening in the treatment of pure lacrimal pump failure.

Lacrimal pump failure refers to epiphora due to reduced tear outflow in the presence of a patent lacrimal drainage system and well-positioned puncta. This condition has been managed with dacryocystorhinostomy and lower eyelid tightening, which carry success rates of 50-94% and 84-91%, respectively. In this retrospective study, 23 eyes with symptomatic epiphora attributed to lacrimal pump failure underwent the lateral tarsal strip procedure and lower eyelid snip punctoplasty, and 20 eyes (87%) experienced complete or nearly complete resolution of tearing. This success rate is quite similar to that of prior studies of eyelid tightening alone for lacrimal pump failure, suggesting that the addition of the snip punctoplasty may not offer any additional benefit in patients with little or no punctal stenosis. All patients displayed some degree of lower eyelid laxity on lid distraction testing preoperatively, and those with only mild lid laxity responded at least as well as those with more severe laxity.

Contralateral orbital melanoma 28 years following enucleation for choroidal melanoma.

A 79-year-old man underwent right enucleation for a choroidal melanoma. Twenty eight years later he presented with a nine month history of progressive left proptosis. Orbital imaging revealed a large left lateral orbital mass, extending back to the orbital apex, which was found on subtotal resection to represent an orbital melanoma. Skin survey was negative, and the prior right choroidal melanoma was the most likely metastatic source. He underwent radiotherapy of the residual tumor at the left orbital apex, as well as radiotherapy of small liver and lung nodules felt to likely represent metastatic melanoma. Five years later, he was still alive and well, with no further tumor demonstrable in the orbit, lung or liver. This case demonstrates the indolence and favorable prognosis of some orbital melanomas. We discuss some hypotheses that may explain such tumor behavior. While melanoma is often considered a highly malignant and lethal tumor, some melanomas are characterized by a more benign course. In addition, certain melanomas may respond to radiotherapy.

Management of acute orbital hemorrhage with obstruction of the ophthalmic artery during attempted coil embolization of a dural arteriovenous fistula of the cavernous sinus.

Because of inability to access a cavernous-carotid fistula through conventional means, a superior ophthalmic vein approach was used to allow access for embolization. Although there was initially robust flow through both the fistula and the ophthalmic artery on angiography, the flow stopped during the procedure, and the patient was found to have an orbital hemorrhage. Immediately on emergent canthotomy and evacuation of the hemorrhage, angiographic evidence of restoration of flow was noted. This finding suggests that vision loss in orbital compartment syndrome may be due to arterial occlusion in some cases, and may be reversible if prompt action is taken.

Preoperative assessment of the eye and periocular region.

Proper preoperative assessment of the eye and periocular region is essential to verify ocular health and vision and to obtain an optimal surgical result. A systematic approach to reviewing the ocular history, review of systems, and pertinent physical exam will be discussed. This is important to identify not only underlying ocular conditions but also potentially serious systemic conditions with ocular manifestations that can impact the patient's overall health. A thorough exam by a qualified provider is needed if there are any management issues regarding the presence of a condition(s) that can impact the surgical outcome.

Eyelid mucous membrane grafts: a histologic study of hard palate, nasal turbinate, and buccal mucosal grafts.

PURPOSE: To histologically evaluate the outcome of mucous membrane grafts to the eyelid. METHODS: Case series of 31 eyes from 24 patients who underwent transplantation of hard palate (25 eyes), buccal (1 eye), or nasal turbinate (5 eyes) mucosa to the posterior eyelid surface. These grafts were biopsied at 0.5 months to 84 months (mean, 20 months) postoperatively. They were examined with light microscopy and compared with either the donor mucosa from the same patient (2 patients) or the typical donor site histology (22 patients). RESULTS: Graft biopsies revealed general epithelial morphology that was quite similar to the respective donor sites in virtually all cases. Six (25%) of 24 hard palate graft biopsies, which were obtained at 8 months to 49 months (mean, 22 months) postoperatively, displayed orthokeratosis alternating with parakeratosis, while 12 (50%) demonstrated parakeratosis alone, and another 6 (25%) showed adjacent regions of parakeratotic and nonkeratinized epithelium. No hard palate grafts showed complete absence of keratinization after transplantation. Other significant findings included loss of goblet cells in nasal turbinate grafts and few submucosal glands remaining in any specimen. CONCLUSIONS: Full-thickness mucosal grafts typically maintain their native epithelial morphology following transplantation to the ocular surface. Submucosal glands usually do not survive transplantation, which could be the result of intentional thinning of the graft at the time of transplantation. Contrary to the opinion that hard palate graft epithelium usually undergoes metaplasia from keratinized to nonkeratinized within 6 months following transplantation to the eye, all hard palate grafts in this study remained orthokeratotic and/or parakeratotic.

Spontaneous orbital and periocular hemorrhage in a patient with epithelioid hemangioma.

Epithelioid hemangioma, or angiolymphoid hyperplasia with eosinophilia, is a benign vascular lesion that infrequently involves the orbit. We report a case of spontaneous orbital and periocular hemorrhage in a patient with epithelioid hemangioma of the orbit. Ophthalmic examination was accompanied by CT and MRI, followed by histopathologic diagnosis. The patient presented with headache, eyelid bruising, and double vision. Ophthalmic examination showed ecchymosis, proptosis, and limited extraocular movements of the affected eye. Imaging studies showed a cystic right orbital mass. Histologic examination showed plump endothelial cells accompanied by lymphocytes, plasma cells, and eosinophils. A diagnosis of epithelioid hemangioma was rendered. To our knowledge, this is the first reported case of spontaneous orbital and periocular hemorrhage associated with epithelioid hemangioma. Epithelioid hemangioma should be considered in the differential diagnosis for patients presenting with spontaneous orbital or periocular hemorrhage.