Publications by authors named "Michael Kazim"

123 Publications

Teprotumumab and Hearing Loss: Case Series and Proposal for Audiologic Monitoring.

Ophthalmic Plast Reconstr Surg 2021 Jun 4. Epub 2021 Jun 4.

Department of Ophthalmology, New York University Langone Health, New York, New York Department of Otolaryngology, Johns Hopkins, Baltimore, Maryland Department of Ophthalmology, Wilmer Eye Institute, Johns Hopkins, Baltimore, Maryland Department of Ophthalmology, University of Missouri, Columbia, Missouri Department of Ophthalmology, Tufts University Medical Center, Boston, Massachusetts Ophthalmic Plastic and Reconstructive Surgery, Department of Ophthalmology, Columbia University Irving Medical Center, New York, New York Oculofacial Plastic and Orbital Surgery, Indianapolis, Indiana Ophthalmic Plastic Surgery Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A.

Purpose: To present a protocol for audiologic monitoring in the setting of teprotumumab treatment of thyroid eye disease, motivated by 4 cases of significant hearing loss, and review the relevant literature.

Methods: Cases of hearing loss in the setting of teprotumumab were retrospectively elicited as part of a multi-institutional focus group, including oculoplastic surgeons, a neurotologist and an endocrinologist. A literature review was performed.

Results: An aggregate of 4 cases of teprotumumab-associated hearing loss documented by formal audiologic testing were identified among 3 clinicians who had treated 28 patients.

Conclusions: Teprotumumab may cause a spectrum of potentially irreversible hearing loss ranging from mild to severe, likely resulting from the inhibition of the insulin-like growth factor-1 and the insulin-like growth factor-1 receptor pathway. Due to the novelty of teprotumumab and the lack of a comprehensive understanding of its effect on hearing, the authors endorse prospective investigations of hearing loss in the setting of teprotumumab treatment. Until the results of such studies are available, the authors think it prudent to adopt a surveillance protocol to include an audiogram and tympanometry before, during and after infusion, and when prompted by new symptoms of hearing dysfunction.
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http://dx.doi.org/10.1097/IOP.0000000000001995DOI Listing
June 2021

Globe Subluxation After Proning in a Coronavirus Disease 2019 Patient.

Ophthalmic Plast Reconstr Surg 2021 Jun 4. Epub 2021 Jun 4.

Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, New York, New York, U.S.A.

A 79-year-old male was hospitalized for respiratory failure secondary to coronavirus disease 2019 pneumonia. Approximately 6 hours after initiating proning, left globe subluxation with darkening of the superior sclera consistent with tache noir was noted. The left globe was thought to be hypotonus secondary to ciliary body shut down after a prolonged period of high intraocular pressure. The globe was reduced in the supine position with gentle traction of the eyelids. Over the next 24 hours, the left intraocular pressure improved to 10 mm Hg, and the tache-noir de la sclérotique resolved. The authors report the first known case of globe subluxation secondary to proning.
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http://dx.doi.org/10.1097/IOP.0000000000001998DOI Listing
June 2021

Atypical presentation of invasive myoepithelioma in a pediatric patient.

Orbit 2021 Apr 27:1-4. Epub 2021 Apr 27.

Columbia University Irving Medical Center - NewYork-Presbyterian Hospital, New York, New York, USA.

A 15-year-old girl presented with a mobile lesion with yellowish hue on the posterior lamella of the right lower eyelid adjacent to the punctum. Four years prior, a lesion thought to be a chalazion was excised from the same location. There was subsequent progressive painless enlargement. The patient ultimately underwent an internal excision of the mass. Histopathology demonstrated infiltrative nests and cords of epithelioid and plasmacytoid cells with abundant eosinophilic myxoid-collagenous stroma along with focal infiltration of skeletal muscle with immunohistochemical staining consistent with an invasive myoepithelioma of mixed-cell type. Given concern for invasive disease, the patient underwent subsequent Mohs resection resulting in a 25% full thickness eyelid defect, which was repaired with direct closure of the wedge defect. There has been no recurrence of the disease for 7 months since the Mohs resection. This case illustrates the atypical presentation of an invasive myoepithelioma of the eyelid in a pediatric patient.
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http://dx.doi.org/10.1080/01676830.2021.1918724DOI Listing
April 2021

Orbital Surgical Guidelines: Pediatric Considerations.

J Neurol Surg B Skull Base 2021 Feb 1;82(1):142-148. Epub 2021 Mar 1.

Oculoplastic and Orbital Surgery, Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, New York-Presbyterian Hospital, New York, New York, United States.

Pediatric orbital and skull base surgery comprises a wide array of tumors. An understanding of the location of the lesion, nature of the disease, and surrounding anatomy is paramount to surgical planning in these small spaces. The goals of pediatric skull base surgery are to avoid injury to the surrounding structures, minimize cosmetic deformities, and remove some or all of the tumors based on anticipated pathology and biologic cost of removal. Safe surgery on many of these tumors requires an understanding of the location of the lesion relative to the optic nerve or orbit. This is particularly challenging because the dimensions of the orbital confines change continuously as one navigates from rostral to caudal. Management of these tumors may require a multidisciplinary approach including orbital surgery, neurosurgery, otolaryngology, oral maxillofacial surgery, plastic surgery, and interventional neuroradiology.
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http://dx.doi.org/10.1055/s-0040-1722637DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7987405PMC
February 2021

Orbital cavernous venous malformation with partial bone encasement.

Orbit 2021 Mar 16:1-2. Epub 2021 Mar 16.

Department of Ophthalmology, Weill Cornell Medicine and New York Presbyterian Hospital, New York, New York, USA.

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http://dx.doi.org/10.1080/01676830.2021.1893345DOI Listing
March 2021

Enrichment of IGF-1R and PPARγ signalling pathways in orbital inflammatory diseases: steps toward understanding pathogenesis.

Br J Ophthalmol 2021 Feb 26. Epub 2021 Feb 26.

Casey Eye Institute, Oregon Health & Science University Casey Eye Institute, Portland, Oregon, USA

Background: Orbital inflammatory disease (OID) encompasses a wide range of pathology including thyroid-associated orbitopathy (TAO), granulomatosis with polyangiitis (GPA), sarcoidosis and non-specific orbital inflammation (NSOI), accounting for up to 6% of orbital diseases. Understanding the underlying pathophysiology of OID can improve diagnosis and help target therapy.

Aims: To test the hypothesis that shared signalling pathways are activated in different forms of OID.

Methods: In this secondary analysis, pathway analysis was performed on the previously reported differentially expressed genes from orbital adipose tissue using patients with OID and healthy controls who were characterised by microarray. For the original publications, tissue specimens were collected from oculoplastic surgeons at 10 international centres representing four countries (USA, Canada, Australia and Saudi Arabia). Diagnoses were independently confirmed by two masked ocular pathologists (DJW, HEG). Gene expression profiling analysis was performed at the Oregon Health & Science University. Eighty-three participants were included: 25 with TAO, 6 with orbital GPA, 7 with orbital sarcoidosis, 25 with NSOI and 20 healthy controls.

Results: Among the 83 subjects (mean (SD) age, 52.8 (18.3) years; 70% (n=58) female), those with OID demonstrated perturbation of the downstream gene expressions of the IGF-1R (MAPK/RAS/RAF/MEK/ERK and PI3K/Akt/mTOR pathways), peroxisome proliferator-activated receptor-γ (PPARγ), adipocytokine and AMPK signalling pathways compared with healthy controls. Specifically, GPA samples differed from controls in gene expression within the insulin-like growth factor-1 receptor (IGF-1R, PI3K-Akt (p=0.001), RAS (p=0.005)), PPARγ (p=0.002), adipocytokine (p=0.004) or AMPK (p=<0.001) pathways. TAO, sarcoidosis and NSOI samples were also found to have statistically significant differential gene expression in these pathways.

Conclusions: Although OID includes a heterogenous group of pathologies, TAO, GPA, sarcoidosis and NSOI share enrichment of common gene signalling pathways, namely IGF-1R, PPARγ, adipocytokine and AMPK. Pathway analyses of gene expression suggest that other forms of orbital inflammation in addition to TAO may benefit from blockade of IGF-1R signalling pathways.
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http://dx.doi.org/10.1136/bjophthalmol-2020-318330DOI Listing
February 2021

Elephantiasis in a patient with thyroid eye disease.

Orbit 2020 Dec 22:1-2. Epub 2020 Dec 22.

Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, New York, NY, USA.

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http://dx.doi.org/10.1080/01676830.2020.1862249DOI Listing
December 2020

Thrombosed orbital arteriovenous malformation in a patient with lymphangioleiomyomatosis.

Orbit 2020 Nov 9:1-4. Epub 2020 Nov 9.

Manhattan Eye, Ear and Throat Hospital, Northwell Hospital, New York, NY, USA.

A 47 year-old female with lymphangioleiomyomatosis developed right periorbital pain worsened while chewing, six months prior. Neuroimaging demonstrated a heterogenous inferotemporal right orbital mass extending through the inferior orbital fissure into the temporalis fossa, with flow voids. Given the patient's past medical history, the lesion was presumed to be a perivascular epithelioid cell tumor, a manifestation of lymphangioleiomyomatosis. A lateral orbitotomy revealed a well-circumscribed bluish-red lesion with areas of hemorrhage that was resected in total. Histopathology, however, was consistent with a thrombosed orbital arteriovenous malformation likely arising from the zygomaticotemporal neurovascular bundle. Lymphangioleiomyomatosis is a rare progressive disease that causes cystic destruction of the lungs and is frequently associated with extrapulmonary tumor infiltration, typically of the kidney and liver. Lymphangiomyoleiomyomatosis cell pathogenesis includes a pro-angiogenic micro-environment, classically expressing vascular endothelial factor-C and -D, which we postulate may have contributed to the development of the orbital arteriovenous malformation.
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http://dx.doi.org/10.1080/01676830.2020.1842465DOI Listing
November 2020

Contour Variability in Thyroid Eye Disease with Compressive Optic Neuropathy Treated with Radiation Therapy.

Adv Radiat Oncol 2020 Sep-Oct;5(5):804-808. Epub 2020 Feb 29.

Department of Radiation Oncology, Columbia University Medical Center, New York, New York.

Purpose: Few studies have evaluated the methodology by which radiation therapy (RT) for thyroid eye disease and compressive optic neuropathy is performed. The objective of this study was to retrospectively review our experience from a radiation planning standpoint and to determine whether current treatment methods provide adequate dose to target and collateral structures.

Methods: A retrospective review of 52 patients (104 orbits) with bilateral thyroid eye disease and compressive optic neuropathy treated with RT (20 Gy in 10 fractions) at our institution. RT plans were analyzed for target volumes and doses. Visual fields, color plates, and visual acuity were assessed pretreatment and at last available follow-up post RT. A standardized, anatomic contour of the retro-orbital space was applied to these retrospective plans to determine dose to the entire space, rather than the self-selected target structure.

Results: Compared with the anatomic retro-orbital space, the original contour overlapped by only 68%. Maximum and mean dose was 2134 cGy and 1910 cGy to the anatomic retro-orbital space. Consequently, 39.8% of the orbits had a mean dose <19 Gy (<17 Gy 16.4%, <18 Gy 27.6% <19 Gy 37.8%, <20 Gy 59.2%, 20-21 Gy 35.8%, >21 Gy 5%). There was no significant association of improvement in color plates ( = .07), visual fields ( = .77), and visual acuity ( = .62), based on these dose differences. When beam placement was retrospectively adjusted to include a space of 0.5 cm between the lens and the anterior beam edge, there was a 39.4% and 20.3% decrease in max and mean dose to the lens.

Conclusions: Without a standardized protocol for contouring in thyroid eye disease, target delineation was found to be rather varied, even among the same practitioner. Differences in dose to the anatomic retro-orbital space did not affect outcomes in the follow-up period. Although precise contouring of the retro-orbital space may be of little clinical consequence overall, a >0.5 cm space from the lens may significantly reduce or delay cataractogenesis.
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http://dx.doi.org/10.1016/j.adro.2020.02.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7560569PMC
February 2020

Atypical Cogan Syndrome Featuring Orbital Myositis and Dacryoadenitis.

Ophthalmic Plast Reconstr Surg 2021 May-Jun 01;37(3S):S160-S162

Edward S. Harkness Eye Institute.

A 39-year-old male presented with bilateral hearing loss and progressive left eye vision loss over a 14-month period. The development of systemic symptoms including arthralgias, enlarged lymph nodes, and profound leg weakness, prompted a workup for lymphoproliferative disease, infection, and autoimmune inflammatory conditions which was unrevealing. Subsequently, the right visual acuity declined from 20/25 to 20/70 and the left to hand motions due to corneal interstitial keratitis. There was limitation of left infraduction. Neuroimaging revealed dural thickening of the internal auditory canals, cavernous sinuses, cerebellum, and along the optic nerves. There was fusiform enhancing enlargement of the left inferior and medial rectus muscles and pathologic enlargement of the left lacrimal gland. Biopsy of the left lacrimal gland and left inferior rectus revealed fibrosis and lymphocytic infiltration. The patient was diagnosed with atypical Cogan syndrome and treated with oral prednisone, with improvement in visual acuity of the right eye, motility of the left eye, and systemic weakness.
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http://dx.doi.org/10.1097/IOP.0000000000001835DOI Listing
May 2021

Orbital Osteomas: Report of 3 Rapidly Progressive Cases in Adolescents and Systematic Literature Review.

Ophthalmic Plast Reconstr Surg 2021 Mar-Apr 01;37(2):118-124

Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, New York.

Purpose: The purpose of this study was to describe the clinical characteristics, management, and outcomes of orbital osteomas in the pediatric age group, and compare these to osteomas managed in the adult population.

Methods: A retrospective review of 3 cases of rapidly progressive orbital osteomas in young patients was performed. In addition, a systematic review of the literature following Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines was conducted to describe the clinical characteristics, management, and outcomes of orbital osteomas in the pediatric age group. The PubMed/MEDLINE database was searched in December 2019 for studies reporting data on patients with orbital osteomas.

Results: Fifty-nine patients were included in this pediatric review, 39 male patients (68%), 18 female patients (32%), and 2 cases with unreported genders. Average age was 15.9 years with a range of 4-21 years. Average osteoma size measured 30.7 mm with a range of 9-60 mm. Adult osteoma size for comparison was smaller than 10 mm in majority of cases. The most common location of pediatric osteomas was the ethmoid sinus. The frontal sinus was the most common location in adult patients. Ninety-seven percent of reported pediatric cases required surgical intervention, compared with only 6.5% in adults.

Conclusions: Orbital osteomas in younger patients are larger when identified, grow faster, are more likely to be symptomatic, and more likely to require surgical intervention compared with those identified in older patients. We recommend close monitoring of osteomas identified in young patients as well as counseling regarding the potential need for future resection.
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http://dx.doi.org/10.1097/IOP.0000000000001755DOI Listing
April 2021

Prospective Correlation of Risk of Obstructive Sleep Apnea With Severe Clinical Features of Thyroid Eye Disease.

Ophthalmic Plast Reconstr Surg 2021 May-Jun 01;37(3S):S58-S61

Columbia University Edward S. Harkness Eye Institute.

Purpose: To evaluate the association between risk of obstructive sleep apnea (OSA) and severity of thyroid eye disease (TED) using a validated OSA screening tool.

Methods: A prospective, observational cohort study was performed. New adult TED patients were offered OSA screening with the Snoring Tired Observed Pressure (STOP)-Bang survey during their initial evaluation. Clinical examination and treatment for TED were standard of care and utilized the International Thyroid Eye Disease Society Vision Inflammation Strabismus Appearance system. At the conclusion of the study period, analysis was performed correlating maximum severity of TED signs and symptoms between high- and low-risk OSA groups. Multivariate logistic and linear regression analyses were also performed to analyze the association between the numerical STOP-Bang score and maximum severity of the potentially actionable clinical features of TED (compressive optic neuropathy, vertical prism deviation, horizontal prism deviation, exophthalmos, vertical fissure height).

Results: Eighty-five patients met inclusion criteria. Twenty-eight percent were at high risk of OSA (STOP-Bang score of 3 or higher). When comparing the low- and high-risk cohorts, increased risk of OSA was significantly associated with the development of TED compressive optic neuropathy (p = 0.014), conjunctival injection (p = 0.027), chemosis (p = 0.013), upper eyelid edema (p = 0.024), lower eyelid edema (p = 0.003), eyelid erythema (p = 0.037), and vertical strabismus (p = 0.047). In the multivariate regression analyses, higher STOP-Bang scores correlated with increased risk of TED compressive optic neuropathy (p = 0.006), vertical strabismus (p = 0.019), and higher subjective diplopia scores (p = 0.045).

Conclusions: Increased risk of OSA, as determined by the STOP-Bang survey, is associated with increased severity of multiple clinical features of TED, including TED compressive optic neuropathy and strabismus.
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http://dx.doi.org/10.1097/IOP.0000000000001809DOI Listing
May 2021

Basaloid follicular hamartoma of the eyelid in a pediatric patient.

Am J Ophthalmol Case Rep 2020 Sep 2;19:100855. Epub 2020 Aug 2.

Edward S. Harkness Eye Institute Columbia University College of Physicians and Surgeons, New York, NY, USA.

Purpose: We report a case a pediatric patient with an eyelid lesion found to be a basaloid follicular hamartoma.

Observations: A six-year-old female with juvenile diabetes who presented with a benign eyelid lesion harboring an aberrant eyelash.

Conclusions And Importance: Basaloid follicular hamartoma is a rare benign neoplasm arising from hair follicles. These lesions can resemble basal cell carcinomas and require complete excision.
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http://dx.doi.org/10.1016/j.ajoc.2020.100855DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7417745PMC
September 2020

Reliability of 3 Strategies of Orbital Tumor Volume Measurement Using Phantom Modeling.

Ophthalmic Plast Reconstr Surg 2021 May-Jun 01;37(3S):S33-S38

Department of Ophthalmology, Columbia University Medical Center.

Purpose: The reliability of 3 volume measurement strategies was investigated using MRI and a simple method for creating phantom orbit tumors.

Methods: Water-based starch was molded into orbital "tumors" of 3 shapes (sphere, ovoid, diffuse); water displacement was used to calculate volume. "Tumors" were placed into 3D-printed orbit phantoms, MRIs were obtained and volume analysis was performed. Observers measured tumor volume using ellipsoid volume (EV), manual segmentation, and semi-automated segmentation strategies. Intraclass correlation coefficients were calculated comparing observer measurements to true volumes. The coefficient of repeatability determined the percentage of tumor volume change required for each method to detect tumor growth.

Results: Intraclass correlation coefficients comparing measured volumes to true volumes using EV, manual segmentation, and semi-automated segmentation were 0.61, 0.98, and 0.99 for spherical, 0.64, 0.97, and 0.98 for ovoid, and 0.18, 0.82, and 0.87 for diffuse tumors. Semi-automated segmentation followed by manual segmentation had the highest correlation between measured and true tumor volume for all 3 tumor geometries. EV had low correlation with true volume for all tumor geometries. Diffuse tumors had high variability and low correlation for all 3 measurement techniques.

Conclusions: This study shows the reliability of 3 strategies to measure orbital tumor volume with MRI based on tumor geometry, using a simple phantom model. EV, the most commonly employed strategy in clinical practice, had low correlation and high variability across tumor shapes. Using manual segmentation and semi-automated segmentation, a measured change in volume greater than 25% may be considered true growth, while the EV strategy required a 40%-400% change in volume to reliably measure tumor growth.
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http://dx.doi.org/10.1097/IOP.0000000000001785DOI Listing
May 2021

Compound Coherent Plane-Wave Ultrasound Imaging of Vascular Malformations of the Orbit.

Ophthalmic Plast Reconstr Surg 2021 Mar-Apr 01;37(2):138-140

Ophthalmic Plastic and Reconstructive, and Orbital Surgery Division, Department of Ophthalmology, New York Presbyterian Hospital, Columbia University Irving Medical Center, New York, New York, U.S.A.

Purpose: Prior color-flow Doppler ultrasound studies of the eye have been performed with systems that exceed US Food and Drug Administration permissible ophthalmic ultrasonic energy limits. The authors report a study of orbital vascular malformations using a novel, Food and Drug Administration compliant, ultrafast compound coherent plane-wave ultrasound device to produce power Doppler images.

Methods: Using a Verasonics Vantage 128 ultrasound engine and a user-developed MATLAB program with a 5-MHz linear-array probe, compound coherent plane-wave ultrasound data were collected on patients with orbital vascular malformations. Real-time color-flow Doppler visualized orbital blood flow. Power Doppler images were produced by post-processing compound coherent plane-wave ultrasound data acquired continuously for 2 seconds.

Results: Compound coherent plane-wave ultrasound was performed on 3 orbital vascular malformations (1 venolymphatic malformation, 1 infantile hemangioma, and 1 arteriovenous malformation). Compound coherent plane-wave ultrasound produced a high-resolution depiction of orbital blood flow for orbital vascular malformations with high sensitivity to slow flow.

Conclusions: Analysis of blood flow within orbital lesions informs treatment planning. Compound coherent plane-wave ultrasound is an emerging ultrasound modality that falls within the Food and Drug Administration guidelines for use in the orbit and provides information to characterize orbital vascular malformations.
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http://dx.doi.org/10.1097/IOP.0000000000001732DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7714699PMC
April 2021

Carcinoid Tumors of the Orbit and Ocular Adnexa.

Ophthalmic Plast Reconstr Surg 2021 May-Jun 01;37(3):217-225

Division of Orbital and Ophthalmic Plastic Surgery.

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.
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http://dx.doi.org/10.1097/IOP.0000000000001715DOI Listing
May 2021

Comparing Image Segmentation Techniques for Determining 3D Orbital Cavernous Hemangioma Size on MRI.

Ophthalmic Plast Reconstr Surg 2020 Nov/Dec;36(6):569-574

Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, New York.

Purpose: To measure orbital cavernous hemangioma size using 3 segmentation methods requiring different degrees of subjective judgment, and to evaluate interobserver agreement using these methods.

Methods: Fourteen patients with orbital cavernous hemangiomas were included in the study. Pretreatment T2-weighted MRIs were analyzed by 2 observers using 3 methods, including 1 user-dependent image segmentation method that required high degrees of subjective judgment (ellipsoid) and 2 parameter-dependent methods that required low degree of subjective judgment (GrowCut and k-means clustering segmentation). Interobserver agreement was assessed using Lin's concordance correlation coefficients.

Results: Using the ellipsoid method, the average tumor sizes calculated by the 2 observers were 1.68 ml (standard deviation [SD] 1.45 ml) and 1.48 ml (SD 1.19 ml). Using the GrowCut method, the average tumor sizes calculated by the 2 observers were 3.00 ml (SD 2.46 ml) and 6.34 ml (SD 3.78 ml). Using k-means clustering segmentation, the average tumor sizes calculated by the 2 observers were 2.31 ml (SD 1.83 ml) and 2.12 ml (SD 1.87 ml). The concordance correlation coefficient for the ellipsoid, GrowCut, and k-means clustering methods were 0.92 (95% CI, 0.83-0.99), 0.12 (95% CI, -0.21 to 0.44), and 0.95 (95% CI, 0.90-0.99), respectively.

Conclusions: k-means clustering, a parameter-dependent method with low degree of subjective judgment, showed better interobserver agreement compared with the ellipsoid and GrowCut methods. k-means clustering clearly delineated tumor boundaries and outlined components of the tumor with different signal intensities.
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http://dx.doi.org/10.1097/IOP.0000000000001651DOI Listing
March 2021

Orbital marginal cell lymphoma and mantle cell lymphoma subclone in patient with monoclonal gammopathy of unknown significance.

Orbit 2021 Jun 3;40(3):247-251. Epub 2020 May 3.

Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, NY, USA.

Indolent lymphomas have the potential to transform into more aggressive phenotypes. This phenomenon is best exemplified by the transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma into diffuse large B-cell lymphoma. However, it is less common to find multiple small cell lymphomas in the orbit, particularly mantle cell lymphoma. We present a case of an extranodal marginal zone lymphoma arising in the orbit that acquired a subclonal t(11;14) (q13;32) translocation, giving rise to a subclone of mantle cell lymphoma. The management of this tumor was targeted towards the more aggressive mantle cell subclone. The tumor exhibited an incomplete response to rituximab alone. To the best of the authors' knowledge, this represents the first such case to be described irrespective of site of origin.
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http://dx.doi.org/10.1080/01676830.2020.1760312DOI Listing
June 2021

Frontal osteoplastic flap without frontal sinus obliteration for orbital roof decompression.

Orbit 2021 Apr 27;40(2):145-149. Epub 2020 Apr 27.

Division of Rhinology and Anterior Skull Base Surgery, Department of Otolaryngology - Head and Neck Surgery, Columbia University Irving Medical Center , New York, New York, USA.

: We describe a novel surgical approach for bilateral orbital roof decompression using a frontal osteoplastic flap without frontal sinus obliteration. This technique utilizes a combined external and endonasal endoscopic approach for wide exposure to the orbital roof bilaterally. We demonstrate this technique for the resection of a massive frontal fibrous dysplasia lesion in a healthy male with bilateral orbital roof involvement. The endonasal endoscopic portion of the technique includes a Draf III frontal sinusotomy (endoscopic modified Lothrop procedure) which precludes the need for frontal sinus obliteration, restores normal frontal sinus function, and allows for postoperative endoscopic surveillance. : Report of novel surgical technique with video demonstration. : This technique for orbital roof decompression allows for removal of a frontal lesion, wide decompression of the bilateral orbital roof, and post-operative endonasal endoscopic surveillance of the cavity. The patient in whom we demonstrate this technique had complete resolution of his orbital symptoms and minimal residual fibrous dysplasia postoperatively. : Bilateral orbital roof decompression for frontal lesions can be performed safely and effectively with a frontal osteoplastic flap without frontal sinus obliteration, restoring normal orbital and sinus function.
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http://dx.doi.org/10.1080/01676830.2020.1757126DOI Listing
April 2021

Acute angle plication of optic nerve glioma as a mechanism of rapidly progressive visual loss.

Orbit 2021 Feb 24;40(1):30-33. Epub 2020 Feb 24.

Department of Ophthalmology, Harkness Eye Institute, Columbia University , New York, New York, USA.

: The majority of pediatric patients with optic nerve gliomas (ONG) remain asymptomatic; however, a subset of patients suffer rapid, irreversible visual loss. The purpose of this study was to determine anatomic differences of ONG noted either by imaging or by intra-operative observation between patients with gradual visual dysfunction and those with rapid visual loss. : A retrospective review was performed in patients with visual loss secondary to ONG. The clinical records, pathology, and radiographic images were reviewed for all patients. The degree of folding or plication of the optic nerve (ON) was determined by analyzing the most acute angle present in the course of the ON. : The primary outcome measure was the angle of plication or folding of the ON and the relationship of this to the course of visual dysfunction. : Six patients with ONG were included in the study. A structural difference in the ON was identified in four patients with rapid vision loss compared with two patients with more gradual visual dysfunction. In patients with rapid progressive visual loss, the ONG had a 90-degree or more acute plication of the ON. Those with more gradual visual loss had more obtuse bends in the ONG. : We have identified that the intrinsic structure of the ONG may contribute to the rare but devastatingly rapid progression of visual dysfunction in some patients. Recognizing these changes may guide clinicians to intervene prior to the development of irreversible visual loss.
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http://dx.doi.org/10.1080/01676830.2020.1733027DOI Listing
February 2021

Trans-septal suture retraction for endoscopic orbital surgery.

Orbit 2020 Oct 18;39(5):336-341. Epub 2019 Nov 18.

Oculoplastic and Orbital Surgery, Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, NewYork-Presbyterian Hospital , New York, NY, USA.

Purpose: We describe a novel technique for endonasal endoscopic orbital surgery. Previously described strategies for minimally invasive orbital surgery include mobilization of the medial rectus muscle and cryosurgical retraction of the pathologic lesion. Herein we describe endoscopic trans-septal suture retraction of an orbital cavernous hemangioma to facilitate intra-orbital dissection from an external approach.

Methods: A 36-year-old male presented with blurred vision in his left eye for several years, along with several months of progressive left proptosis and decreasing visual acuity. Imaging revealed a 2.5 cm orbital mass consistent with a cavernous hemangioma. The patient underwent a combined transconjunctival and endoscopic approach for resection.

Results: Following left medial orbital wall and floor decompression, endoscopic trans-septal suture retraction facilitated dissection of the lesion from intraorbital structures. A complete extracapsular resection of the lesion was achieved. The inferomedial orbit was reconstructed with a synthetic implant.

Conclusion: Trans-septal suture retraction is an effective adjunctive technique for endonasal endoscopic orbital tumor surgery. This technique facilitates safe retraction and dissection of soft tissue lesions from critical intraorbital structures.
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http://dx.doi.org/10.1080/01676830.2019.1692040DOI Listing
October 2020

SETD2 Mutation in an Aggressive Optic Nerve Glioma.

JAMA Ophthalmol 2020 01;138(1):102-104

Ophthalmic Plastic and Reconstructive Surgery, Orbital Surgery Division, Department of Ophthalmology, New York Presbyterian Hospital, Columbia University Irving Medical Center, New York.

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http://dx.doi.org/10.1001/jamaophthalmol.2019.4511DOI Listing
January 2020

Extraocular muscle enlargement in retinoencephalofacial angiomatosis.

Orbit 2020 Jun 28;39(3):221-223. Epub 2019 Oct 28.

Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, NY, USA.

A 22-year-old female presented for evaluation of five years of progressive left exophthalmos and intermittent blurred vision. She had previously received laser treatment for peripheral retinal neovascularization and had undergone lip reconstruction for a left-sided congenital vascular facial malformation. Magnetic resonance imaging demonstrated diffuse enlargement of the left extraocular and temporalis muscles, with prominent vessels in the temporalis muscle and intraconal fat. Left fundoscopic examination revealed grossly enlarged, tortuous retinal vessels extending from the optic disc to the peripheral retina and an abnormal network of capillaries. On the basis of these findings, a diagnosis of retinoencephalofacial angiomatosis was established. Retinoencephalofacial angiomatosis is a rare, non-hereditary disorder associated with ipsilateral retinal, brain, and facial arteriovenous malformations. This is the first report, to the authors' knowledge, of retinoencephalofacial angiomatosis presenting with exophthalmos secondary to extraocular muscle enlargement.
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http://dx.doi.org/10.1080/01676830.2019.1677727DOI Listing
June 2020

Renal Medullary Carcinoma With Metastasis to the Temporal Fossa and Orbit.

Ophthalmic Plast Reconstr Surg 2019 Nov/Dec;35(6):e149-e151

Department of Ophthalmology, Columbia University Medical Center.

A 22-year-old Hispanic man with sickle cell trait presented with blurred vision, double vision, and pain with OD movement. MRI demonstrated an extra-axial mass centered around the temporal bone with extension into the middle cranial fossa and lateral aspect of the extra-conal right orbit, and mass effect on the lateral rectus muscle. Biopsy of the lesion was consistent with renal medullary carcinoma. CT chest/abdomen/pelvis confirmed a primary tumor in the right kidney. No additional metastases were found. Renal medullary carcinoma is a rare, highly aggressive malignancy, which almost exclusively affects young men of African descent with sickle cell trait or sickle cell disease. The authors present the second confirmed case of renal medullary carcinoma metastatic to the orbit, with ocular symptoms prior the typical presenting symptoms of flank pain and hematuria.Renal medullary carcinoma is a highly aggressive malignancy, most commonly seen in African American patients with sickle cell disease. Involvement of the orbit is rare and visual symptoms may precede systemic diagnosis.
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http://dx.doi.org/10.1097/IOP.0000000000001478DOI Listing
January 2020

Adult xanthogranulomatous disease of the orbit: case report of spontaneous regression and review of treatment modalities.

Orbit 2020 Feb 25;39(1):31-37. Epub 2019 Apr 25.

Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, NY, USA.

Adult xanthogranulomatous disease of the orbit is a rare and incompletely understood entity. It can be limited to the orbit or be associated with systemic disease. While routine systemic surveillance is the standard of care for patients with orbital xanthogranulomatous disease, there is no universally accepted protocol for managing periorbital and orbital involvement. The authors report a case of adult orbital xanthogranuloma, without systemic disease, who, after 10 years of observation, demonstrated sustained radiographic regression of the lesions. To the authors' knowledge, this represents one of the first reports of spontaneous regression of untreated orbital xanthogranuloma, and supports observation of lesions that do not compromise ocular function. We present our case in the context of a major review of various treatment strategies described in the literature, including surgical resection, radiotherapy, plasmapheresis, corticosteroids, multiple immunomodulatory medications.
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http://dx.doi.org/10.1080/01676830.2019.1590421DOI Listing
February 2020

Bipedicle Dermis Fat Graft for Orbital Volume Augmentation and Repair of Lower Eyelid Retraction in an Anophthalmic Socket With Prior Orbital Implant Placement.

Ophthalmic Plast Reconstr Surg 2019 Mar/Apr;35(2):e39-e41

Division of Oculoplastic and Orbital Surgery, Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, New York, U.S.A.

Buphthalmos can create treatment challenges for socket rehabilitation following enucleation due to compression atrophy and mechanical stretching of adnexal tissues. The authors report a case demonstrating a surgical technique for simultaneous volume augmentation of the anophthalmic socket, expansion of the conjunctival fornix, and repair of lower eyelid retraction in a patient with history of buphthalmos and a previously placed 22 mm orbital implant at the time of enucleation. The authors utilized a single-stage, bipedicle dermis fat graft. Postoperatively, the patient demonstrated improvement in lower eyelid position, lower eyelid volume, improved prosthesis fit, improved three-dimensional projection of the ocular prosthesis, and an improvement in the superior sulcus deformity. Bipedicle dermis fat grafts are an option for surgical rehabilitation of the anophthalmic socket when orbital volume deficiency, conjunctival fornix contracture, and eyelid retraction are present and an adequately sized orbital implant has previously been placed.
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http://dx.doi.org/10.1097/IOP.0000000000001309DOI Listing
December 2019

Preseptal Upper Eyelid Lipoma.

Ophthalmic Plast Reconstr Surg 2019 Jan/Feb;35(1):e2-e3

Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, New York, U.S.A.

Superficial subcutaneous lipomas are benign, slow-growing neoplasms comprised mature adipose cells that may be surrounded by a fibrous tissue capsule. While found commonly on the body, simple lipomas are rarely described in the eyelids. Reports of two eyelid lipomas in the literature describe the anatomy to be posterior to the orbital septum. The presenting authors report the case of a simple lipoma of the eyelid found fully in the preseptal plane. Lipomas can occur anterior to the orbital septum and should be considered in the differential when presented with a painless, slow-growing eyelid mass in adults.
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http://dx.doi.org/10.1097/IOP.0000000000001268DOI Listing
December 2019