Transcutaneous retrobulbar amphotericin B for rhino-orbital-cerebral mucormycosis: a multi-center retrospective comparative study.

PURPOSE: To assess whether transcutaneous retrobulbar amphotericin B injections (TRAMB) reduce exenteration rate without increasing mortality in rhino-orbital-cerebral mucormycosis (ROCM). METHODS: In this retrospective case-control study, 46 patients (51 eyes) with biopsy-proven ROCM were evaluated at 9 tertiary care institutions from 1998 to 2021. Patients were stratified by radiographic evidence of local orbital versus extensive involvement at presentation. Extensive involvement was defined by MRI or CT evidence of abnormal or loss of contrast enhancement of the orbital apex with or without cavernous sinus, bilateral orbital, or intracranial extension. Cases (+TRAMB) received TRAMB as adjunctive therapy while controls (-TRAMB) did not. Patient survival, globe survival, and vision/motility loss were compared between +TRAMB and -TRAMB groups. A generalized linear mixed effects model including demographic and clinical covariates was used to evaluate the impact of TRAMB on orbital exenteration and disease-specific mortality. RESULTS: Among eyes with local orbital involvement, exenteration was significantly lower in the +TRAMB group (1/8) versus -TRAMB (8/14) (p = 0.04). No significant difference in mortality was observed between the ±TRAMB groups. Among eyes with extensive involvement, there was no significant difference in exenteration or mortality rates between the ±TRAMB groups. Across all eyes, the number of TRAMB injections correlated with a statistically significant decreased rate of exenteration (p = 0.048); there was no correlation with mortality. CONCLUSIONS: Patients with ROCM with local orbital involvement treated with adjunctive TRAMB demonstrated a lower exenteration rate and no increased risk of mortality. For extensive involvement, adjunctive TRAMB does not improve or worsen these outcomes.

Efficacy and Safety of Teprotumumab in Patients With Thyroid Eye Disease of Long Duration and Low Disease Activity.

CONTEXT: Early inflammatory thyroid eye disease (TED) can lead to symptomatic chronic disease, including disabling proptosis. Teprotumumab, an insulin-like growth factor-1 receptor (IGF-1R) inhibitor, previously demonstrated efficacy in acute, high-inflammation TED trials. OBJECTIVE: We present data from the first placebo-controlled trial with teprotumumab in chronic/low disease activity TED. METHODS: This randomized double-masked, placebo-controlled trial, conducted at 11 US centers, enrolled adult participants with TED duration of 2 to 10 years, Clinical Activity Score (CAS) ≤ 1 or no additional inflammation or progression in proptosis/diplopia for ≥1 year, proptosis ≥3 mm from before TED and/or from normal, euthyroid/mildly hypo/hyperthyroid, no prior teprotumumab, and no steroids within 3 weeks of baseline. Patients received (2:1) intravenous teprotumumab or placebo once every 3 weeks (total 8 infusions). The primary endpoint was proptosis (mm) improvement at Week 24. Adverse events (AEs) were assessed. RESULTS: A total of 62 (42 teprotumumab and 20 placebo) patients were randomized. At Week 24, least squares mean (SE) proptosis improvement was greater with teprotumumab (-2.41 [0.228]) than with placebo (-0.92 [0.323]), difference -1.48 (95% CI -2.28, -0.69; P = .0004). Proportions of patients with AEs were similar between groups. Hyperglycemia was reported in 6 (15%) vs 2 (10%) and hearing impairment in 9 (22%) vs 2 (10%) with teprotumumab and placebo, respectively. AEs led to discontinuation in 1 teprotumumab (left ear conductive hearing loss with congenital anomaly) and 1 placebo patient (infusion-related). There were no deaths. CONCLUSION: Teprotumumab significantly improved proptosis vs placebo in longstanding/low inflammation TED, demonstrating efficacy regardless of disease duration/activity. The safety profile was comparable to that previously reported.

Tattoo-associated lacrimal gland enlargement and sarcoidosis.

Purpose: To describe a case of tattoo-associated sarcoidosis presenting with cutaneous findings of tattoo granulomas and ophthalmic manifestation of isolated bilateral lacrimal gland enlargement. Observations: A 35-year-old female presented with bilateral upper eyelid swelling for over a year. She reported no associated episodes of ocular pain or visual decline since onset of eyelid edema. On examination, the lacrimal glands were firm and enlarged bilaterally. Slit-lamp examination demonstrated no evidence of active or prior ocular inflammation. Further systemic examination revealed multiple raised papules within a 4-year-old chest/shoulder tattoo. Histopathology from a lacrimal gland biopsy showed non-caseating granulomas consistent with sarcoidosis. Conclusions and importance: The authors report a rare case of a 35-year-old presenting with isolated dacryoadenitis and tattoo granulomas found to be a tattoo-associated sarcoidosis. Although uveitis is a commonly described ocular manifestation in tattoo-associated sarcoidosis, few reports have described lacrimal gland enlargement as a presenting ophthalmic feature in tattoo-associated sarcoidosis.

Alemtuzumab-Induced Thyroid Eye Disease: A Comprehensive Case Series and Review of the Literature.

PURPOSE: To present 5 cases of alemtuzumab-induced thyroid eye disease (AI-TED) and review the literature to highlight the natural history, severity, and outcomes as compared with conventional thyroid eye disease (TED). METHODS: A multi-institutional retrospective case series of patients with AI-TED was compiled. Chart review evaluated for clinical characteristics, imaging findings, and treatment for AI-TED. Additionally, a comprehensive review of the literature identified all previously published cases of AI-TED. RESULTS: Five new patients with AI-TED were included in this series. The average clinical activity score on presentation was 2.8 (range 1-4) and reached an average peak of 5.0 during the active phase of the disease (4-7). Patients were treated medically with selenium (40%) or monoclonal antibodies including teprotumumab or tocilizumab (40%). Surgical treatment with orbital decompression for compressive optic neuropathy was performed on 2 (40%) patients. Combined with 11 previously reported cases, these 16 patients with AI-TED had an average clinical activity score on presentation of 3.3. The average length of the AI-TED phase was 14.0 months, and all patients were treated with medical and/or surgical interventions for their disease. CONCLUSIONS: Clinical and imaging findings in AI-TED mirror that of conventional TED, however, AI-TED may present with greater severity. AI-TED may develop many months after Graves' disease; therefore, providers should be aware of this association and monitor patients for the development of severe TED.

A Contemporary Review of the Role of Facial Prostheses in Complex Facial Reconstruction.

BACKGROUND: Maxillofacial prostheses provide effective rehabilitation of complex facial defects as alternatives to surgical reconstruction. Although facial prostheses provide aesthetically pleasing reconstructions, multiple barriers exist that prevent their routine clinical use. The accessibility of facial prostheses is limited by the scarce supply of maxillofacial prosthodontists, significant time commitment and number of clinic appointments required of patients during prosthesis fabrication, short lifespan of prostheses, and limited outcomes data. METHODS: A literature review was completed using PubMed and Embase databases, with search phrases including face and maxillofacial prostheses. Patient cases are included to illustrate the use of facial prostheses to reconstruct complex facial defects. RESULTS: The clinical use of facial prostheses requires a multidisciplinary team including a reconstructive surgeon, a maxillofacial prosthodontist, and an anaplastologist, if available, to provide patients with aesthetically appropriate facial prostheses. Developing technology including computer-aided design and three-dimensional printing may improve the availability of facial prostheses by eliminating multiple steps during prosthesis fabrication, ultimately decreasing the time required to fabricate a prosthesis. In addition, enhanced materials may improve prosthesis durability. Long-term outcomes data using validated measures is needed to support the continued use of facial prostheses. CONCLUSIONS: Facial prostheses can be used to reconstruct complex facial defects, and bone-anchored prostheses are associated with high patient satisfaction. Multiple barriers prevent prostheses from being used for facial reconstruction. New technologies to assist the design and fabrication of prostheses, and cost reduction measures, may allow their use in the appropriately selected patient.

Lower eyelid margin reconstruction: results of five different techniques.

PURPOSE: This retrospective study was performed to examine surgical results of five different techniques for lower eyelid margin reconstruction after Mohs surgery: primary closure, semicircular flap, dermal matrix graft, sliding tarsal flap, and tarsoconjunctival flap. METHODS: Medical records were reviewed in 178 patients undergoing surgery between 2005 and 2020. Outcomes were evaluated (photographic review) by three oculoplastic observers masked to procedure type, both with and without knowledge of the eyelid defect. RESULTS: All patients achieved a good-excellent functional result and 90.4% were asymptomatic after surgery. Tarsoconjunctival flaps were associated with greater need for subsequent interventions (p < .001) and anterior lamellar deformities (p < .001). Semicircular flaps had a higher incidence of lateral canthal deformity (p < .001), but less eyelash disruption than other flap/graft techniques (p < .001). Mean cosmetic ratings (defect masked) were similar for dermal matrix grafts, semicircular, and sliding tarsal flaps; with each grading higher than tarsoconjunctival flaps (p ≤ .05). Among patients with 9-15 mm wide defects, results were better for semicircular and sliding tarsalflaps, than dermal matrix grafts (p ≤ .005) and tarsoconjunctival flaps (p ≤ .02). CONCLUSIONS: All patients achieved a good-excellent functional result and 87.1% a good-excellent cosmetic result. The semicircular flap was effective for repairing medium sized wounds that could not be closed primarily, creating a continuous lash line, although with a higher incidence of lateral canthal deformities. The sliding tarsal flap was effective for shallow wounds of varying widths. The single-staged dermal matrix graft provided similar results as the tarsoconjunctival flap. Subsequent interventions were more frequent after the tarsoconjunctival flap than other methods.

Outcomes of Patients With Thyroid Eye Disease Partially Treated With Teprotumumab.

PURPOSE: In response to the coronavirus (COVID-19) pandemic, teprotumumab production was temporarily halted with resources diverted toward vaccine production. Many patients who initiated treatment with teprotumumab for thyroid eye disease were forced to deviate from the standard protocol. This study investigates the response of teprotumumab when patients receive fewer than the standard 8-dose regimen. METHODS: This observational cross-sectional cohort study included patients from 15 institutions with active or minimal to no clinical activity thyroid eye disease treated with the standard teprotumumab infusion protocol. Patients were included if they had completed at least 1 teprotumumab infusion and had not yet completed all 8 planned infusions. Data were collected before teprotumumab initiation, within 3 weeks of last dose before interruption, and at the visit before teprotumumab reinitiation. The primary outcome measure was reduction in proptosis more than 2 mm. Secondary outcome measures included change in clinical activity score (CAS), extraocular motility restriction, margin reflex distance-1 (MRD1), and reported adverse events. RESULTS: The study included 74 patients. Mean age was 57.8 years, and 77% were female. There were 62 active and 12 minimal to no clinical activity patients. Patients completed an average of 4.2 teprotumumab infusions before interruption. A significant mean reduction in proptosis (-2.9 mm in active and -2.8 mm in minimal to no clinical activity patients, P < 0.01) was noted and maintained during interruption. For active patients, a 3.4-point reduction in CAS ( P < 0.01) and reduction in ocular motility restriction ( P < 0.01) were maintained during interruption. CONCLUSIONS: Patients partially treated with teprotumumab achieve significant reduction in proptosis, CAS, and extraocular muscle restriction and maintain these improvements through the period of interruption.

Teprotumumab (Tepezza) for Thyroid Eye Disease.

Thyroid eye disease (TED) is an immune mediated infiltration and inflammation of the orbital and periorbital soft tissues leading to facial disfigurement. Classically broken into two "phases," active inflammatory and quiescent, disease modifying therapy and surgical intervention are used to improve a number of clinical aspects of TED. Many medical modalities have been utilized to halt the inflammatory phase of the disease including steroids, orbital radiation, and targeted steroid-sparing chemotherapy. Teprotumumab is currently the only Federal Drug Administration approved therapy for the treatment of TED. Significant improvements in proptosis, diplopia and quality of life are noted following its 24-week course of therapy.

Quantitative Analysis of Preoperative and Postoperative Photographs Posted on Social Media by Oculoplastic Surgeons.

PURPOSE: To investigate and quantitatively analyze preoperative and postoperative photographs posted on social media by oculoplastic surgeons. METHODS: Pre- and postoperative blepharoplasty photographs posted by oculoplastic surgeons on the social media platform Instagram were identified over a 4-month period. Photographs demonstrating a frontal pose of the periocular region were selected, and image analysis was performed to compare the preoperative and postoperative photographs. Quantitative image analysis was performed with a semiautomated script using ImageJ (v1.52k) software. Image analysis compared magnification, patient position, luminosity, relative color profiles, and edge detection/sharpness. Three oculoplastic surgeons graded these imaging metrics for the same photoset and the consensus grade was assigned to each category. Finally, the quantitative calculations were then compared with the consensus grades to develop receiver operating characteristic curves for further analysis. RESULTS: The study included 104 posts from 35 members of ASOPRS. Regarding patient positioning, 9 postings were 5%-10% offset from the patients' center, and 6 postings were more than 10% from the patients' center. The majority of photosets demonstrated less than or equal to 2.5% difference in magnification (69/104), with 7 postings demonstrating a greater than 5% difference, and 3 postings exhibiting greater than 10% difference in magnification. In the periocular region, 17.3% (18/104) of postings were found to have a greater than 10% difference in luminosity (>25.5 pixel-value). A more than 5% difference (>12.75 pixel-value) in pre- and postoperative edge-detection measurements was identified in 12 of 104 postings. In 22 of 104 photosets, the mean absolute deviation in color channel ratios was more than 0.01 and 15 of 104 photosets were found to have a mean absolute deviation more than 0.05. CONCLUSIONS: Numerous quantifiable photographic inconsistencies were identified in surgical photographs posted on social media by members of ASOPRS. Adoption of an automated image analysis tool that compares before-and-after surgical photographs could promote standardized oculoplastic surgery photography on social media.

Psychogenic Ptosis.

PURPOSE: Psychogenic ptosis is a rare ophthalmic manifestation of conversion disorder. The aim of this study was to describe the clinical parameters, etiology, psychological, and clinical aspects of psychogenic ptosis. METHODS: A retrospective case series was conducted of patients with psychogenic ptosis seen between 1990 to 2020. Medical records were reviewed for patient demographics, including psychiatric history, clinical findings, diagnostic studies, treatment, and resolution rates. A literature review was performed to identify cases of psychogenic ptosis previously published between 1990 and 2020. RESULTS: Six female patients (aged 14-60 years) were diagnosed with unilateral psychogenic ptosis. Physical trauma preceded the onset of ptosis in all cases. Imaging studies had been previously obtained in all patients, none of who were correctly diagnosed at time of referral. Associated signs included concurrent brow ptosis, orbicularis oculi spasm, squint on upgaze, and variable levator function and eyelid margin measurements. Four patients had preexisting psychological conditions. Patients were primarily managed with reassurance. CONCLUSIONS: Psychogenic ptosis is an often delayed or misdiagnosed condition, resulting in unnecessary referrals and imaging. Psychogenic ptosis should be considered in patients with atypical findings of ptosis including ipsilateral brow depression, orbicularis oculi spasm, squint on upgaze, and variable eyelid measurements. A prior history of minor trauma and female sex were common in this series. Our experience suggests that psychogenic ptosis can often be treated with reassurance, leading to partial or complete resolution. Given the number of patients with comorbid psychiatric conditions, the authors recommend a low threshold for psychiatric or psychological evaluation.

Surgical management of eyelid lesions in adult-onset asthma and periocular xanthogranuloma.

Medical therapy is the mainstay of initial treatment for periocular xanthogranulomas. Here we depict surgical management of biopsy-proven adult-onset asthma and periocular xanthogranuloma (AAPOX). This 27-year-old female presented with seven years of progressive bilateral periorbital swelling, weight gain, and severe asthma refractory to immunosuppressive therapy including methotrexate, rituximab, and cyclophosphamide. Pre-operative and post-operative photos show excellent resolution of lesions. Orbitotomy with excision involved an upper eyelid crease incision marked at 9 mm centrally. A flap was dissected between the skin and orbicularis-involving xanthogranuloma, extended superiorly to orbital rim and laterally and medially until normal orbicularis was encountered. The xanthogranuloma was excised en-bloc. Infiltrated preaponeurotic fat and portions of the orbital lobe of the lacrimal gland were resected. After achieving hemostasis, the skin was closed with 6-0 polypropylene suture. Surgical pathology was consistent with the diagnosis of AAPOX.

Contralateral eyelid elevation following unilateral upper eyelid retraction repair.

PURPOSE: To report the influence of unilateral upper eyelid retraction repair on the upper eyelid position of the contralateral, non-operative side. METHODS: An IRB-approved retrospective chart review was performed to identify patients who underwent unilateral upper eyelid retraction repair. Patient demographics, etiology of upper eyelid retraction, previous surgeries, and periocular measurements from pre-operative and follow-up visits were reviewed. RESULTS: Thirteen patients who underwent unilateral upper eyelid retraction repair due to thyroid eye disease or iatrogenic retraction secondary to ptosis repair demonstrated an elevation in contralateral eyelid height post-operatively. The mean pre-operative MRD-1 was 6.6 mm in the operative eye and 2.6 mm in the contralateral eye. All patients demonstrated a decreased MRD-1 in the operative eye (mean change: -2.6 mm [-38.4%]; standard deviation [SD]: 1.2 mm) and an increased MRD-1 in the contralateral eye (mean change: +1.3 mm [+62.4%]; SD: 0.6 mm) following unilateral upper eyelid retraction repair. The mean post-operative MRD-1 measurements were 4.0 mm ± 0.5 mm and 3.9 mm ± 0.6 mm in the operative and contralateral eyes, respectively. CONCLUSIONS: Unilateral upper eyelid retraction repair may induce an elevation of the contralateral upper eyelid position in some patients. The findings in this report may support unilateral intervention in patients with upper lid retraction, especially in the setting of contralateral blepharoptosis.

Carcinoid Tumors of the Orbit and Ocular Adnexa.

PURPOSE: Well-differentiated neuroendocrine or carcinoid tumors are found most commonly in the gastrointestinal tract. When metastatic to the orbit, they tend to have a propensity for the extraocular muscles. The purpose of this study was to better understand the diversity in presentation of orbital carcinoid disease and to determine predictors for survival. METHODS: In this observational cross-sectional cohort study, data from 8 tertiary orbital practices were compiled. Demographic, clinical, pathologic, American Joint Committee on Cancer stage and grade, imaging, and management data were extracted for all the patients. Descriptive statistics were calculated. Subgroups were compared utilizing analysis of variance analyses and Kaplan-Meier curves. Time to progression and disease-specific and overall mortality were calculated. Comparisons were performed for the following a priori pairs: unknown versus known primary tumor, single versus multiple extraocular muscle involvement, unilateral versus bilateral orbital disease, extraocular muscle versus other orbital involvement, and excisional versus incisional surgery. RESULTS: A total of 28 patients with carcinoid tumors of the orbit were identified. Of these, 57.1% of patients were female, the mean age at diagnosis of the primary tumor was 58.8 years and the mean age at diagnosis of orbital disease was 62.6 years. At primary presentation, all patients were American Joint Committee on Cancer stage III or IV and 21.4% demonstrated carcinoid syndrome. Muscle involvement was noted in 78.6% of patients, and of these, 72% were noted to have single muscle disease. Eight patients had no primary tumor identified; 3 of these 8 demonstrated disseminated disease at the time of diagnosis. The overall 5-year survival rate was 81.8% from diagnosis of primary tumor and 50% from diagnosis of orbital disease. Subgroup analysis revealed that patients with unilateral orbital disease when compared with bilateral orbital disease had a longer progression-free survival and time to death from all causes (p = 0.025). Patients with disease localized to the orbit at presentation had longer time to death than those with disseminated disease. Treatment with surgery, radiation, or octreotide did not appear to affect survival. Patients managed with systemic chemotherapy had a shorter time of survival than the rest of the group. All other subgroup comparisons were not found to be statistically significant. CONCLUSIONS: Neuroendocrine tumors of the orbit represent a wide spectrum of disease, with some cases being part of disseminated disease, while others being localized presentations. This heterogeneity may be responsible for the slightly higher overall survival in these patients than others with metastatic carcinoid tumors in other locations.

Inverted Papilloma of the Orbit and Nasolacrimal System.

PURPOSE: Periocular inverted papilloma (IP) is a rare, locally aggressive tumor with a propensity for recurrence and malignant transformation. Historically treated via wide excision, this study examines the characteristics and management of periocular IP, comparing those confined to the nasolacrimal system with those invading the orbit. METHODS: An Institutional Review Board-approved, Health Insurance Portability and Accountability Act-compliant retrospective, comparative case series was conducted in patients with IP of the orbit or nasolacrimal system across 15 clinical sites. RESULTS: Of 25 patients, 22 met inclusion criteria with 9 limited to the nasolacrimal system and 13 invading the orbit. Mean age was 60.4 years, 55% were women, all were unilateral. Mean follow-up was 48 months. Rates of smoking, dust and/or aerosol exposure, human papillomavirus (HPV) status, and inflammatory polyps were elevated compared to rates in the general population (45%, 18%, 18%, and 14%, respectively). Bony erosion on computed tomography scans was statistically significantly associated with orbit-invading IP (p = 0.002). Treatment involved all confined IP undergoing surgery alone while 39% of orbit-invading IP also received radiation therapy and/or chemotherapy (p = 0.054). Orbit-invading IP was more likely to be excised with wide margins than IP confined to the nasolacrimal system (85% vs. 22%, p = 0.007). Overall rates of malignancy, recurrence, and patient mortality from IP were found to be 27%, 23%, and 9%, respectively. CONCLUSIONS: IP invading the orbit typically requires aggressive therapy, while IP confined to the nasolacrimal system may be treated more conservatively. Using risk factors, characteristics, and outcomes, a treatment algorithm was created to guide management.

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.

Atypical presentation of Cat Eye Syndrome in an infant with Peters anomaly and microphthalmia with cyst.

PURPOSE: To describe a unique ocular presentation of Cat Eye Syndrome and review the ocular and systemic findings associated with the syndrome. METHODS: Case report with multimodal imaging. RESULTS: A newborn female presented with a unilateral Peters anomaly with contralateral microphthalmia with cyst. The patient's other systemic findings included a hypoplastic right heart, persistent ductus arteriosus, intrauterine growth retardation, bilateral anotia, preauricular ear pits and skin tags, micrognathia, hypoplastic female genitalia, and unilateral cranial nerve VII palsy. Chromosomal microarray testing showed tetrasomy of chromosome 22 in the q11.1-q11.21 region consistent with Cat Eye Syndrome. The patient ultimately underwent a successful optical iridectomy on one side and orbitotomy with excision of the cystic mass on the other. CONCLUSIONS: The co-occurrence of unilateral Peters anomaly with contralateral microphthalmia with cyst in Cat Eye Syndrome is rare and demonstrative of the syndrome's phenotypic variability. The medical and surgical management of these patients may require a multidisciplinary approach and must be tailored to the individual findings and overall systemic health of the patient.

Efficient Coding by Midget and Parasol Ganglion Cells in the Human Retina.

In humans, midget and parasol ganglion cells account for most of the input from the eyes to the brain. Yet, how they encode visual information is unknown. Here, we perform large-scale multi-electrode array recordings from retinas of treatment-naive patients who underwent enucleation surgery for choroidal malignant melanomas. We identify robust differences in the function of midget and parasol ganglion cells, consistent asymmetries between their ON and OFF types (that signal light increments and decrements, respectively) and divergence in the function of human versus non-human primate retinas. Our computational analyses reveal that the receptive fields of human midget and parasol ganglion cells divide naturalistic movies into adjacent spatiotemporal frequency domains with equal stimulus power, while the asymmetric response functions of their ON and OFF types simultaneously maximize stimulus coverage and information transmission and minimize metabolic cost. Thus, midget and parasol ganglion cells in the human retina efficiently encode our visual environment.

An Update on Endocrine Mucin-producing Sweat Gland Carcinoma: Clinicopathologic Study of 63 Cases and Comparative Analysis.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade adnexal neoplasm with predilection for the periorbital skin of older women. Histologically and immunophenotypically, EMPSGC is analogous to another neoplasm with neuroendocrine differentiation, solid papillary carcinoma of the breast. Both lesions are spatially associated with neuroendocrine mucinous adenocarcinomas of the skin and breast, respectively. EMPSGC is ostensibly a precursor of neuroendocrine-type mucinous sweat gland adenocarcinoma (MSC), a lesion of uncertain prognosis. Non-neuroendocrine MSC has been deemed locally aggressive with metastatic potential, and previous works speculated that EMPSGC-associated (neuroendocrine-type) MSC had similar recurrence and metastatic potential with implications for patient follow-up. Only 96 cases of EMPSGC have been reported (12 cases in the largest case series). Herein, we present 63 cases diagnosed as "EMPSGC" in comparison with aggregated results from known published EMPSGC cases. We aim to clarify the clinicopathologic features and prognostic significance of the neuroendocrine differentiation of EMPSGC and its associated adenocarcinoma and to determine the nosological relevance of EMPSGC association in the spectrum of MSC histopathogenesis. Results established an overall female predominance (66.7%) and average presenting age of 64 years. EMPSGC lesions were associated with adjacent MSC in 33.3% of cases. The recurrence rate for neuroendocrine-type MSC was ~21%, less than the reported 30% for non-neuroendocrine MSC. There were no cases of metastasis. EMPSGC and neuroendocrine-type MSC are distinct entities with more indolent behavior than previously reported, supporting a favorable prognosis for patients.

Thyroid eye disease presenting with superior rectus/levator complex enlargement.

Purpose: To describe the demographic and clinical characteristics of patients with thyroid eye disease (TED) who present with predominate superior rectus/levator complex involvement.Methods: A multi-institutional retrospective review was performed to identify patients with TED who presented with superior isolated or predominate rectus/levator involvement. Baseline and subsequent visits were reviewed to characterize the clinical course.Results: Nineteen patients were identified. All patients had imaging demonstrating an enlarged levator/superior rectus complex. At presentation, the mean clinical activity score (CAS) was 2.1 (range: 0-5). Nineteen (100%) patients had proptosis on the affected side. Lid abnormalities, including upper/lower eyelid retraction and ptosis were higher on affected side compared to the unaffected side. Eleven (58%) patients had vertical misalignment. Mean thyroid stimulating immunoglobulin (TSI) was 3.7 (range: 1-7.1). Mean follow-up time was 18 months (range: 0-60 months). At last follow-up, the mean CAS was 1.3 (range 0-5). Ten (53%) patients had proptosis. Eleven (58%) patients had vertical misalignment. Repeat imaging in eight patients showed interval enlargement of other extraocular muscles.Conclusions: The presentation of TED with superior rectus/levator complex enlargement may be under-appreciated. Orbital imaging, as well as laboratory evaluation, may help support a diagnosis of TED. In the setting of abnormal TSI and/or thyrotropin receptor antibody, presence of upper eyelid retraction, and an otherwise unremarkable laboratory and systemic evaluation, a presumptive diagnosis of TED may be made, and the patient can be followed closely, as he/she is likely to develop involvement of other extraocular muscles, consistent with a more typical presentation of TED.