Neurosarcoidosis Presenting as Longitudinally Extensive Transverse Myelitis and Orbital Mass: A Case Report.

We describe a case of neurosarcoidosis in a 64-year-old female who presented with proptosis and orbital inflammation together with bilateral lower extremity neuropathy and longitudinally extensive transverse myelitis. These two entities are not commonly associated, and the etiology of the transverse myelitis was facilitated by an orbital biopsy. The transverse myelitis caused numbness in her lower extremities and tightness in her chest and abdomen, which progressed over weeks to difficulty walking and bilateral neuromuscular weakness. Magnetic resonance imaging (MRI) revealed longitudinally extensive transverse myelitis in the cervical and thoracic spine. Computed tomography (CT) imaging of the chest revealed right hilar and mediastinal lymphadenopathy and calcified subcarinal nodes. Positron emission tomography (PET) scan revealed hypermetabolism in the mediastinum and medial left orbit. Orbital biopsy revealed non-necrotizing granulomatous inflammation suggestive of sarcoidosis. The neurologic deficits and orbital inflammation responded well to intravenous corticosteroids. Neurosarcoidosis can present with unusual clinical manifestations, as evidenced by this patient.

Rituximab for thyroid eye disease.

PURPOSE: To assess the efficacy and safety of rituximab-mediated B-lymphocyte depletion as treatment for thyroid eye disease (TED). METHODS: Prospective, open-label, interventional clinical trial evaluating 12 patients with TED and Clinical Activity Scores (CAS) (VISA [vision, inflammation, strabismus and appearance/exposure] classification) of 4 or greater followed for 1 year after rituximab (1000 mg) treatment, administered intravenously on days 1 and 15. CAS, peripheral B-lymphocyte levels, thyroid autoantibody levels, and thyroid function tests were recorded at baseline, 4 weeks, 8 weeks, 12 weeks, 24 weeks, 36 weeks, and 52 weeks after the second infusion. The primary endpoint was a change from baseline in CAS. Thyroid-stimulating immunoglobulin and thyroid-stimulating hormone levels were also monitored over the 12-month postinfusion observation period. RESULTS: CAS scores demonstrated a statistically significant decrease from baseline at each of the follow-up visits. Thyroid-stimulating immunoglobulin and thyroid-stimulating hormone levels demonstrated no statistically significant change from baseline. B-cell depletion was observed within 1 month after rituximab treatment, and peripheral B-lymphocyte counts started to increase 36 weeks after the infusion. B-cell depletion was well tolerated, and there were no adverse effects of the rituximab infusions. CONCLUSIONS: CAS scores were significantly reduced over time in this group of 12 patients and appeared to be associated with rituximab infusion. The variable natural history of TED makes it difficult to definitively assign efficacy. The results support the continued investigation of rituximab for TED in a larger placebo-controlled trial.

Syringocystadenoma papilliferum of the eyelid.

PURPOSE: To describe 6 patients with syringocystadenoma papilliferum of the eyelid and to review the literature regarding this rare eyelid tumor. METHODS: Clinicopathologic series of 6 cases, diagnosed at the New York Eye and Ear Infirmary from 1990 to 2006. The PubMed database was searched for reported cases of syringocystadenoma papilliferum of the eyelid (keywords: syringocystadenoma, eyelid, apocrine, eccrine). RESULTS: Fourteen patients (6 from the current case series and 8 previously reported) with syringocystadenoma papilliferum of the eyelid were identified. Patient age ranged from 8 to 82 years (mean, 44.6 years). Most cases had a preoperative diagnosis of basal cell carcinoma or cyst. Histopathologic evaluation of excised lesions revealed proliferation of duct-like channels in the dermis. These channels were lined by nonkeratinizing epithelium and communicated with the epidermis. Papillary structures with plasma cell-rich stromal infiltrate protruded in the cystic invaginations. Nine lesions (64%) were associated with apocrine, eccrine, or sebaceous tumors or malformations. None of the lesions was associated with a malignant neoplasm. One lesion recurred after incomplete excision. None of the completely excised lesions recurred. CONCLUSIONS: Syringocystadenoma papilliferum can rarely affect eyelid skin. This lesion is frequently clinically misdiagnosed as basal cell carcinoma or cyst. Although syringocystadenoma papilliferum of the eyelid can be associated with other benign lesions, no malignant transformation or association with malignant neoplasms has been reported. The evidence suggests that this tumor should be managed with conservative complete excision.

Rituximab immunotherapy for ocular adnexal lymphoma: clinicopathologic correlation with 5-year follow-up.

Two patients with primary ocular adnexal mucosa-associated lymphoid tumor lymphoma were treated with rituximab immunotherapy. One patient had refused radiotherapy. The other had bilateral ischemic retinopathy, a relative contraindication to radiotherapy. Biopsies were performed 6 months after treatment. One patient had complete resolution of local disease, and the other had a partial response, remaining stable without signs of progression. During the 5 years of clinical follow-up, there was no evidence of systemic disease in either patient. Rituximab immunotherapy is an alternative to regional radiotherapy for ocular adnexal mucosa-associated lymphoid tumor lymphoma.

Endoscopic endonasal surgery simulator as a training tool for ophthalmology residents.

PURPOSE: Surgical training on the endoscopic endonasal surgery simulator had proven efficacy for otorhinolaryngology residents in preparation for endoscopic endonasal and sinus surgery. Its use for ophthalmology residents in preparation for endoscopic endonasal dacryocystorhinostomy has not been previously studied. METHODS: Eight of 15 ophthalmology residents recruited for this experimental study underwent training on the endoscopic endonasal surgery simulator, completing the novice and intermediate modules. All 15 residents then participated in cadaver surgical training, performing defined surgical tasks including endoscopic navigation, identification of nasal anatomy, endonasal injection, and middle turbinate medialization. Performance on these tasks was videotaped and graded by 2 masked observers. Total mean scores and variance by task category were compared between subjects and controls and interobserver variance was compared between observers. RESULTS: Correlation between the 2 masked observers' scores was strong (R = 0.677), with total mean scores of 2.34 and 2.38, respectively. Total mean scores were 2.79 for subjects, and 1.86 for controls (F value 0.735, p = 0.01). Residents who trained on the simulator performed significantly better during endonasal navigation (mean scores 2.58 for subjects versus 1.74 for controls, p = 0.04) and endonasal injection (mean scores 2.73 for subjects versus 1.72 for controls, p = 0.03) and minimally better at identification of nasal anatomy (mean scores 2.93 for subjects versus 1.88 for controls, p = 0.18) and middle turbinate medialization (mean scores 3.13 for subjects versus 2.78 for controls, p = 0.36). CONCLUSIONS: Ophthalmology residents who trained on the surgical simulator had significantly enhanced endoscopic endonasal surgical skills for endonasal navigation and injection.

Oral montelukast and cetirizine for thyroid eye disease.

PURPOSE: To study the efficacy of oral montelukast, a cysteinyl leukotriene receptor antagonist, in combination with cetirizine, a histamine-1 receptor antagonist, in the treatment of thyroid eye disease. METHODS: Patients considering surgical correction of eyelid retraction for inflammatory symptoms of thyroid eye disease were offered a preoperative medical regimen of oral montelukast/cetirizine. Exclusion criteria included prior use of oral montelukast (i.e., for seasonal allergy or asthma), compressive optic neuropathy, severe ophthalmopathy requiring systemic corticosteroids, and orbital and/or muscle surgery. A 6-week course of oral cetirizine (10 mg every morning) and oral montelukast (10 mg every evening) was administered and patients subjectively rated their ocular surface dryness, tearing, itching, injection, eyelid swelling, eyelid retraction, double vision, proptosis, and visual clarity, at baseline, after 3 weeks and 6 weeks of medical therapy, and after 3 weeks off of the medications. RESULTS: Six of the 12 patients recruited for the study reported a subjective improvement in tearing, dryness, and itching. Less effect on diplopia and proptosis was noted after 6 weeks of medical therapy. Two of the patients who did not report response chose to proceed with eyelid retraction surgery and both had evidence of mast cell infiltration in their Müller muscle specimens. CONCLUSION: The response observed in this open-label trial suggests that oral montelukast and cetirizine may be an effective medical regimen for patients with thyroid eye disease who experience mild to moderate orbital congestion and inflammation.