Contralateral orbicularis oculi muscle transposition in facial paralysis: functional, aesthetic and electromyographic outcomes. A case report and literature update.

PURPOSE: Many surgical approaches have been described for achieving satisfactory functionality in patients with facial paralysis, to ensure the protection of the cornea and the highest degree of physiological blinking. Out of all those available, dynamic techniques are indicated when motion recovery and synchrony are the goals pursued. Orbicularis oculi transposition (OOT) allows a genuine restoration of blinking by means of contralateral reinnervation, with minimal insult for the donor site. METHODS: We present the case of a 64-year-old man with unilateral facial paralysis. A physical examination revealed a lagophthalmos of more than 5 mm. A neurophysiological study showed a mild-to-moderate axonal injury of the left facial nerve. Contralateral OOT was indicated as the first therapy option since it can restore involuntary blinking. The orbicularis muscle flap was not divided into branches, as described previously by other authors; the whole flap was transposed to the paralysed upper eyelid to achieve the highest level of potential reinnervation. RESULTS: Lagophthalmos was fully corrected after the surgery. The patient reported subjective improvement of symptoms, less frequent instillation of artificial tear drops and, especially, synchronous blinking with significant improvement in involuntarity. These improvements were maintained after 1 year of follow-up. Postoperative electromyographic studies confirmed the improvement in neural function. CONCLUSIONS: OOT can be a safe and effective option for the treatment of patients with peripheral facial paralysis, as it achieves a good restoration of blinking function with minimal morbidity in the healthy donor eye.

Anti-IL-6 Receptor Tocilizumab in Refractory Graves' Orbitopathy: National Multicenter Observational Study of 48 Patients.

Graves' orbitopathy (GO) is the most common extrathyroidal manifestation of Graves' disease (GD). Our aim was to assess the efficacy and safety of Tocilizumab (TCZ) in GO refractory to conventional therapy. This was an open-label multicenter study of glucocorticoid-resistant GO treated with TCZ. The main outcomes were the best-corrected visual acuity (BVCA), Clinical Activity Score (CAS) and intraocular pressure (IOP). These outcome variables were assessed at baseline, 1st, 3rd, 6th and 12th month after TCZ therapy onset. The severity of GO was assessed according to the European Group on Graves' Orbitopathy (EUGOGO). We studied 48 (38 women and 10 men) patients (95 eyes); mean age ± standard deviation 51 ± 11.8 years. Before TCZ and besides oral glucocorticoids, they had received IV methylprednisolone (n = 43), or selenium (n = 11). GO disease was moderate (n =29) or severe (n = 19) and dysthyroid optic neuropathy (DON) (n = 7). TCZ was used in monotherapy (n = 45) or combined (n = 3) at a dose of 8 mg/kg IV every four weeks (n = 43) or 162 mg/s.c. every week (n = 5). TCZ yielded a significant improvement in all of the main outcomes at the 1st month that was maintained at one year. Comparing the baseline with data at 1 year all of the variables improved; BCVA (0.78 ± 0.25 vs. 0.9 ± 0.16; p = 0.0001), CAS (4.64 ± 1.5 vs. 1.05 ± 1.27; p = 0.0001) and intraocular pressure (IOP) (19.05 ± 4.1 vs. 16.73 ± 3.4 mmHg; p = 0.007). After a mean follow-up of 16.1 ± 2.1 months, low disease activity (CAS ≤ 3), was achieved in 88 eyes (92.6%) and TCZ was withdrawn in 29 cases due to low disease activity (n = 25) or inefficacy (n = 4). No serious adverse events were observed. In conclusion, TCZ is a useful and safe therapeutic option in refractory GO treatment.

Orbital and conjunctival metastasis from lobular breast carcinoma.

This article examines the clinical presentation of ocular metastasis from an infiltrating lobular breast carcinoma. We examined a conjunctival biopsy from a 69-year-old woman who developed unilateral conjunctival inflammation together with a neurotrophic corneal ulcer and proptosis. Infiltrating lobular breast carcinoma (ILBC) was diagnosed using routine histology and immunohistochemistry. She had a past history of a hormone receptor-positive infiltrating ILBC 11 years ago with cutaneous and diffuse osteoblastic metastases, and she was kept under treatment with lezotrol. Treatment was initiated with systemic corticosteroids but an annular conjunctival perilimbal infiltration was found to have spread, which did not respond either to local radiotherapy (total dose 60 Gy, 2 Gy per day). A new extensive corneal epithelial defect recurred, and because it had not responded to matrix therapy agent (RGTA, Cacicol®) eye drops, autologous serum eye drops and a therapeutic contact lens, a permanent total tarsorrhaphy was performed. Progression of the diffuse bone metastases was detected and the treatment with lezotrol was replaced by fulvestrant.Infiltrating lobular breast carcinoma is a rare cause of conjunctival metastasis. This aggressive malignancy did not respond to external beam radiotherapy.

Epithelioid hemangioma of the orbit or angiolymphoid hyperplasia with eosinophilia.

A patient was referred to the department for an incidentally discovered right orbital mass on MRI. After surgical excision, histopathologic examination led to the diagnosis of epithelioid hemangioma, a tumor rarely located in the orbit, whose main histopathologic feature is an exuberant proliferation of small vascular channels lined by enlarged endothelial cells. The clinical characteristics and histopathology of epithelioid hemangioma allow differentiation with Kimura disease, angiosarcoma, intravascular papillary endothelial hyperplasia, and other vascular tumors that share the common feature of an abnormal proliferation of endothelial cells. Although epithelioid hemangioma is extremely rare, it should be considered in the differential diagnosis of orbital tumors.

Use of hyaluronic acid gel in the management of cicatricial ectropion: results and complications.

AIMS: To report our experience using hyaluronic acid gel injection in the lower eyelid to treat cicatricial ectropion. METHODS: Clinical records and literature review. RESULTS: Between November 2009 and June 2011, 12 lower eyelids of 11 patients with cicatricial ectropion were treated with hyaluronic acid gel. All 11 patients demonstrated improvement in the eyelid position after treatment but only 3 patients (27.3%) experimented total correction after injection. Although the correction was partial in the majority of the patients, the signs and symptoms associated with conjunctival and corneal exposure improved in all patients. Seven patients (63.7%) developed irregular fullness which resolved over time, but in 4 patients (36.3%) fullness persisted for one year. CONCLUSIONS: In our experience, hyaluronic acid gel is a nonsurgical, minimally invasive and safe technique that improves cicatricial ectropion, but with a poor cosmetic result that limits its use. This treatment could be considered only in patients who decline surgery or are poor surgical candidates, as the aesthetic result would not be acceptable to many.

Use of carotid compression in a patient with arteriovenous malformation.

We report a case of a patient with a high flow arteriovenous malformation (AVM)i n the orbit, who developed a severe compartment syndrome, and was successfully treated with manual carotid compressions. The patient suffered a progressive proptosis, restriction of ocular motility and decreased of the visual acuity of the right eye. Embolization had a high risk of serious complications due to fistula location, so an alternative treatment consisting in carotid compressions was prescribed. Manual carotid compression is a non-invasive alternative technique to treat AVMs in patients with high risk of embolization episodes.

Optic neuropathy following orbital irradiation for Graves' ophthalmopathy: a case report and literature review.

Radiation optic neuropathy (RON) is a devastating complication of radiotherapy to the anterior visual pathway resulting in acute, profound and sometimes irreversible visual loss. Cumulative doses of radiation that exceed 50 Gy or radiation fractions of greater than 2 Gy are usually required for RON to develop. Several factors, such as diabetes and pre-existing compression of the optic nerve, are associated with a higher risk for developing RON at lower doses of radiation. We report a case of presumed subacute RON following orbital irradiation for Graves' ophthalmopathy, successfully treated with megadoses of intravenous corticosteroids.

Orbital nerve sheath myxoma: a case report.

A 72-year-old man was referred to the Service of Ophthalmology due to a 2-year history of ptosis and a painless mass in the lateral orbital margin of the right eye. Orbital MRI revealed a well-demarcated lesion in the superotemporal quadrant of the orbit. After surgical excision, histopathological examination led to the diagnosis of nerve sheath myxoma, a tumor composed of myxoid nodules separated by fibrous septa with spindle-shaped and stellate cells. Many of these cells were immunostained with antibodies to S-100 protein. This is the first case reported in the literature of such a tumor located in the orbit, and, though extremely rare, it should be considered in the differential diagnosis of orbital tumors.

Peripheral primitive neuroectodermal tumour of the orbit.

Peripheral primitive neuroectodermal tumours (pPNETs) are a group of soft-tissue tumours of neuroepithelial origin that arise outside the central and sympathetic nervous system. Orbital location is infrequent, and to the best of the authors' knowledge only 16 cases have been reported in the literature. With this article, the authors report the demographics and clinical characteristics, diagnostic features, differential diagnosis, prognosis and therapeutic options of primary orbital peripheral primitive neuroectodermal tumour, based on their patients and on the cases reported in the literature to date. A differential diagnosis should be made with other small round cell tumours; immunohistochemical and ultrastructural techniques are essential for this purpose. Although bone invasion and extraorbital extension are possible, systemic metastases are uncommon in the cases of orbital pPNETs. Surgery has been the initial treatment in most cases; chemotherapy with or without radiotherapy is considered the best additional treatment. The orbital pPNET could be less aggressive than other forms of pPNETs, since most of the patients reported were alive after the follow-up period (at least 6 months).