Treatment of Thyroid Eye Disease-Associated Ophthalmopathy and Myopathy With Intraorbital Injection of 5-Fluorouracil and Triamcinolone Acetonide.

PURPOSE: The authors report a technique of local application of anti-metabolite and corticosteroid mixture in the orbit for treatment of thyroid orbitopathy with moderate-severe inflammation and muscle involvement. METHODS: Patients of one orbital surgeon seen between March 2019 and May 2020 with active thyroid eye disease and restrictive strabismus were considered for local treatment of the myopathic component of the disease. A mixture of 1 ml 5-FU 50 mg/ml, 0.25 ml triamcinolone 40 mg/ml, and 1 ml lidocaine 2% is injected through the skin using a 25-gauge, 1.5-inch needle into the orbit adjacent to the affected extraocular muscle. Six patients were treated in the outpatient setting and 3 patients have been treated with this intervention intraoperatively at the time of orbital decompression. One was treated with the mixture reconstituted with hyaluronic acid (Healon GV) to address postoperative medial rectus fibrosis to the medial wall, this mixture was applied topically in the operative field and not injected. RESULTS: All patients had subjective improvement in the eye movement limitation and 2 patients had a change in motility on exam that was temporally correlated to injections. One patient did not disclose high-dose aspirin intake before injection and experienced a retrobulbar hemorrhage immediately following injection which was successfully treated. No complications were noted as a result of the medication itself. DISCUSSION: The combination of 5-fluorouracil and triamcinolone acetonide for orbital treatment may be a useful adjunct in treating patients with ongoing inflammatory activity, both in the office and in the operating room. The novel combination may optimize ophthalmic outcomes, modifying disease course in some patients.

Neutrophil-to-Lymphocyte Ratios Distinguish Idiopathic Orbital Inflammation From Orbital Infectious Disease.

PURPOSE: The neutrophil-to-lymphocyte ratio (NLR) is a relatively novel biomarker to distinguish between acute stresses. This study was performed to determine whether the NLR may discern infectious orbital maladies from idiopathic orbital inflammation (IOI). METHODS: The NLR was calculated by a review of the initial blood draws of adult patients who presented to the emergency department at a single academic medical center. Statistical comparisons were performed to identify the significance of these results. RESULTS: Ten patients with IOI, 12 patients with necrotizing fasciitis (NF), and 12 patients with orbital cellulitis (OC) presented to the emergency department. The groups were not statistically significantly different in terms of age or gender. The mean NLRs were 3.48 (standard deviation = 1.80), 13.5 (standard deviation = 14.5), and 8.15 (standard deviation = 6.56) for IOI, NF, and OC, respectively. Patients with IOI had statistically significantly lower NLRs than patients with NF ( p = 0.037) and OC ( p = 0.034). However, the NLRs of patients with OC were not statistically significantly different from those of patients with NF ( p = 0.27). CONCLUSIONS: The NLR appears to distinguish IOI from infectious etiologies, but does not discern between variants of infection. These results should be juxtaposed against appropriate imaging and clinical evaluations, but elevated NLR values may heighten clinicians' concerns for an infectious process and encourage them to initiate appropriate management steps.

Misdiagnosis of fungal infections of the orbit.

OBJECTIVE: To evaluate the accuracy of the initial diagnosis in the case of fungal infections of the orbit and identify factors that may influence patient outcomes. METHODS: An institutional review board-approved retrospective chart review was conducted across 2 large academic centres to identify cases of fungal infections involving the orbit from January 1, 1998, to November 15, 2019. Data collected included patient demographics, past medical history, examination findings, diagnosis, treatment, imaging, and outcomes. RESULTS: Fifty cases of fungal infection involving the orbit were identified. Of these, 33 (66.0%) were initially misdiagnosed as nonfungal diagnoses. Sixteen patients (32.0%) received multiple initial diagnoses. The most common diagnoses on presentation were bacterial cellulitis (n = 12 of 50; 24.0%) and bacterial sinusitis (n = 12 of 50; 24.0%). These were followed by vascular and orbital inflammatory conditions (n = 9 of 50; 18.0%): 5 patients (10.0%) were clinically diagnosed with giant cell arteritis, 3 (6.0%) with nonspecific orbital inflammation, and 1 (2.0%) with optic neuritis. In this subset of patients, 77.8% (n = 7 of 9) were treated initially with systemic steroids. Additional initial diagnoses included neoplastic mass lesions, mucocele, dacryocystitis, cavernous sinus thrombosis, hemorrhage, tick-borne illness, allergic rhinitis, and allergic conjunctivitis. Misdiagnosis was significantly correlated with involvement of the masticator space on imaging (p = 0.04). CONCLUSION: Fungal infections of the orbit are misdiagnosed in 2 of 3 cases. Nearly 15% of patients who are later diagnosed with fungal disease of the orbit were initially treated with systemic steroids. Misdiagnosis is more frequent when the masticator space is involved.

Attitudes and Awareness of Cornea Specialists and Oculoplastic Surgeons toward Methicillin-Resistant Staphylococcus aureus Decolonization.

Background: Methicillin-resistant Staphylococcus aureus (MRSA) decolonization is widely utilized in many medical subspecialities to reduce surgical site infections, but routine ophthalmic implementation has been limited. The aim of this study was to investigate the attitudes and actual practice of corneal specialists and oculoplastic surgeons toward MRSA decolonization as a preventive measure in ophthalmic surgery. Materials and Methods: A web-based survey was sent to cornea specialists and oculoplastic surgeons to assess their knowledge, beliefs, and practices regarding MRSA prophylaxis and the use of MRSA decolonization to prevent post-operative infections. Results: A total of 180 surgeons participated in this study: 71% of respondents agreed that MRSA colonization plays a role in post-operative infection of the eye and adnexal structures; 65% stated that MRSA decolonization could help prevent MRSA infection. Although 41% of respondents would change their management in response to a positive pre-operative MRSA screening result, only 18% performed pre-operative screening. Seventeen percent of respondents indicated that they offer pre-operative decolonization for MRSA-positive patients; the most frequently applied technique was the use of nasal antibiotic agents such as mupirocin, followed by antiseptic baths. Peri-operative MRSA prophylaxis was used by 18% of respondents; pre-operative MRSA decolonization was used in conjunction by 8.5 % of respondents. Conclusions: Although MRSA decolonization has been validated in fields outside of ophthalmology, there has not been widespread adoption of this practice among oculoplastic surgeons and cornea specialists. Prospective MRSA decolonization ophthalmic studies are necessary if evidence-based management guidelines are to be developed.

Recession-Resection of the Vertical Rectus Muscles for Chin-up Vertical Abnormal Head Position Associated With Infantile Nystagmus Syndrome.

PURPOSE: To review the correction of chin-up abnormal head position (AHP) due to infantile nystagmus syndrome via surgery on the vertical rectus muscles, typically a combined recession-resection of these muscles. METHODS: This was a review of 6 patients who underwent surgical correction of chin-up vertical AHP in the context of infantile nystagmus syndrome at an academic institution. The correction of AHP, visual acuity, ductions, and ocular alignment were noted both preoperatively and postoperatively. The need for repeat surgery, induced strabismus, or correction of AHP were also noted. RESULTS: Six patients underwent surgery for chin-up AHP. A combined recession-resection of the vertical rectus muscles (bilateral inferior rectus muscle recession of 5 to 8 mm; bilateral superior rectus muscle resection of 7 to 8 mm) was performed in 4 of 6 patients, and isolated bilateral recession of the inferior rectus muscles was performed in the remaining 2 patients. Four of 6 patients (67%) achieved complete correction of their AHP at the last follow-up visit, with a mild residual chin-up AHP persisting in the other 2 patients. One patient developed large angle exotropia, one had restrictive hypertropia and horizontal plane null position, and another developed an incomitant horizontal strabismus with exotropia in right gaze. Reoperation was performed in the former 2 patients, with successful correction of the strabismus in each. CONCLUSIONS: Surgery on the vertical rectus muscles can reduce or eliminate a chin-up head position in patients with infantile nystagmus syndrome. Care should be taken to avoid producing a restriction of depression in abduction if the amount of resection is too large. [J Pediatr Ophthalmol Strabismus. 2022;59(6):410-415.].

Association of Age-adjusted Charlson Comorbidity Index With Orbital Fungal Disease Outcomes.

PURPOSE: To determine whether the age-adjusted Charlson comorbidity index (age-CCI) in sino-orbital fungal disease patients correlates with disease-specific mortality. METHODS: Hospital billing systems at 2 academic institutions were queried for patients with ICD-9, ICD-10, and CPT codes used in fungal disease who also had orbital disease and significant visual loss. Thirty-two patients at Institution A and 18 patients at Institution B met the inclusion criteria of microbiologic or pathologic confirmation of fungal infection and completion of inpatient ophthalmology evaluation. Patients without radiographic abnormality in the sinus or orbit were excluded. Demographic, diagnostic, treatment, and outcome variables were recorded. Our primary outcome was death due to fungal disease. RESULTS: Of the 50 medical records examined, 44 patients met the criteria for fungal-related death outcome on multivariate analysis. The regression coefficient for age-CCI and fungal-related mortality was 0.242 (95% CI, 0.012-0.779) with a p value of 0.038. CONCLUSIONS: Age-CCI is significantly associated with fungal-related mortality. This relationship remains significant when controlling for 5 covariates of fungal organism phylum, presence or absence of CNS disease, exenteration, local treatment use, and presence or absence of an immunosuppressive diagnosis. Age-CCI shows promise as a clinical and research tool in the evaluation of invasive fungal disease involving the orbit.

Ocular adnexal phenotype and management of a patient with mosaic expression of a mutation in TWIST2.

Ablepharon-macrostomia syndrome (AMS) and Barber-Say syndrome (BSS) are congenital ectodermal dysplasias associated with mutations in the TWIST2 gene. Among the ophthalmic anomalies that occur in these syndromes, underdevelopment of the anterior lamella of the eyelid is a defining feature. Reports of mosaic expression of TWIST2 mutations are extremely rare, with only five confirmed or suspected cases described to date. Mosaic expression of TWIST2 variants is correlated with a less severe phenotype than that reported for the typical expression of TWIST2 variants associated with BSS or AMS. Abnormal development of the anterior lamella appears to be a common feature in all cases of AMS with mosaic expression. Here, we describe the phenotype of a patient with mosaic expression of a TWIST2 mutation that is typically associated with AMS. We additionally describe the surgical approach employed in the treatment of this patient.

Blunderbuss: Ocular and Orbital Injuries Due to Muzzle-Loading Firearms.

PURPOSE: Injury to the eye and/or orbital and adnexal structures associated with antiquated muzzle-loading firearms has not, to our knowledge, been characterized with the exception of a single case report. METHODS: A retrospective chart review of 7 patients treated at Vanderbilt University Medical Center from 2003 to 2017 who sustained traumatic injuries to the ocular and/or orbital structures secondary to the discharge of muzzle-loading firearms. The study was approved by the Vanderbilt Institutional Review Board. RESULTS: In 6/7 cases, injuries occurred secondary to the muzzle-loading firearm exploding due to dysfunction or misuse. Foreign material deriving from the firearm was retained in 3/7 patients. Initial examination of the orbit and adnexa revealed 5/7 individuals sustaining orbital fractures and 6/7 with facial lacerations (including 2 with eyelid lacerations); none had evidence of a lacrimal duct injury. Three patients suffered globe injuries (1 closed-globe and 2 open-globe). Visual acuity at last follow-up was ≥20/20 in 12/14 eyes examined. Surgical intervention was required in the treatment of 4/7 individuals (including 3/7 requiring intervention for sustained orbital fractures). No individuals were wearing eye protection at the time of injury. All individuals survived their injuries. CONCLUSIONS: The operation of muzzle-loading firearms poses a unique risk of injury to the operator. The resultant injuries in this case series were primarily due to the explosion of the firearm, which subsequently appear similar to orbital and ocular blast injuries caused by explosive weapons. Orbital injuries and more potentially more devastating ocular injuries may have been prevented by protective eyewear.

Correlation of Automated Computed Tomography Volumetric Analysis Metrics With Motility Disturbances in Thyroid Eye Disease.

PURPOSE: The authors sought to examine relationships between CT metrics derived via an automated method and clinical parameters of extraocular muscle changes in thyroid eye disease (TED). METHODS: CT images of 204 orbits in the setting of TED were analyzed with an automated segmentation tool developed at the institution. Labels were applied to orbital structures of interest on the study images, which were then registered against a previously established atlas of manually indexed orbits derived from 35 healthy individuals. Point-wise correspondences between study and atlas images were then compared via a fusion algorithm to highlight metrics of interest where TED orbits differed from healthy orbits. RESULTS: Univariate analysis demonstrated several correlations between CT metrics and clinical data. Metrics pertaining to the extraocular muscles-including average diameter, maximum diameter, and muscle volume-were strongly correlated (p < 0.05) with the presence of ocular motility deficits with regards to the superior, inferior, and lateral recti (with exception of superior rectus motility deficits being mildly correlated with muscle volume [p = 0.09]). Motility defects of the medial rectus were strongly correlated with muscle volume, and only weakly correlated with average and maximum muscle diameter. CONCLUSIONS: The novel method of automated imaging metrics may provide objective, rapid clinical information which may have utility in prevention and recognition of visual impairments in TED before they reach an advanced or irreversible stage and while they are able to be improved with immunomodulatory treatments.

Porcine Urinary Bladder Extracellular Matrix for Treatment of Periocular Skin Defects.

PURPOSE: To describe the experience of 4 oculoplastic surgeons with porcine bladder matrix for periocular anterior lamella and donor site skin defects either as stand-alone treatment or in conjunction with other reconstructive procedures. The authors hypothesized that defect size and location influence the requirement for additional matrix treatments or ancillary procedures. METHODS: Following the Institutional Review Board approval, the authors conducted a retrospective review of 17 patients treated with porcine bladder matrix at 2 oculoplastic practices between 2016 and 2018. Powdered matrix was applied to the skin defect and overlaid with a matrix sheet. Subsequent rounds of matrix treatment or other reconstructive procedures were performed as necessary. Defect size and location were correlated to the number of ancillary matrix treatments or surgical procedures via univariate analysis. RESULTS: Twenty-five sites (21 primary and 4 donor) in 17 individuals (8-95 years, M = 58.8 years, 10 males) were treated with porcine bladder matrix. All wounds healed successfully. Additional matrix treatments were administered at 5 sites. Ancillary procedures were performed for 7 sites. Upper lid involvement and larger defect size tended to require additional ancillary procedures (p = 0.006), while lower eyelid and other periocular defects required fewer procedures (p < 0.001). CONCLUSION: Porcine bladder matrices are useful adjuncts to healing periocular anterior lamella defects in various settings. Such repairs are useful in nonsurgical candidates, but must take into account varying levels of complexity based on lesion location. Smaller defects are more conducive to application of matrices as stand-alone treatment, while larger or upper eyelid defects often require additional procedures.

Comparison of Techniques for Correction of Chin-down Vertical Abnormal Head Position Associated with Infantile Nystagmus Syndrome.

PURPOSE: We evaluated the relative effectiveness of combined recession-resection of vertical rectus muscles versus superior rectus recession with inferior oblique weakening for patients who underwent surgical correction of chin-down abnormal head position (AHP) associated with infantile nystagmus syndrome (INS). DESIGN: Retrospective interventional case series. METHODS: This is a review of 22 patients who underwent surgical correction of chin-down vertical AHP associated with INS at an academic institution. The primary outcome was collapse of AHP. Unfavorable outcomes included repeat surgery and induced strabismus, in addition to failure of collapse of AHP. RESULTS: Twenty-two patients had chin-down AHP. Recession-resection (bilateral superior rectus recession 6-9 mm; bilateral inferior rectus resection 5-9 mm) was performed in 11 cases; weakening of both elevators (bilateral superior rectus recession 5-8 mm, bilateral inferior oblique recession or myectomy) occurred in 11 cases. Unfavorable outcome rates were 64% (7/11) compared with 18% (2/11), respectively (P = .03). Reoperation was performed for 6 of 22 patients. Five patients were from the recession-resection group, namely 3 for induced V-pattern esotropia, 1 for alternating esotropia, and 1 to correct recurrent AHP. The last of the 6 who required reoperation was in the elevator weakening group, and required correction of a recurrent AHP (P = .06). CONCLUSIONS: While recession-resection of the vertical recti and weakening of both elevators each produce acceptable collapse of chin-down AHP, the former frequently induces a V-pattern esotropia requiring reoperation.

Long-term Outcomes Following Surgery for Infantile Nystagmus Syndrome With Abnormal Head Positioning.

PURPOSE: To evaluate long-term outcomes of surgical treatment for abnormal head positioning (AHP) associated with infantile nystagmus syndrome (INS). DESIGN: Retrospective observational case series. METHODS: Review of 150 patients who underwent surgery for AHP associated with nystagmus. Outcomes included head positioning, duction limitations, and strabismus, and were evaluated several times postoperatively. Successful collapse of AHP was defined as being ≤10°. RESULTS: Thirty-one patients had surgery for AHP in the pitch (chin up/down) position, whereas 119 had surgery for a horizontal AHP. In addition, 54 underwent 50%-60% augmentation, 19 underwent 40% augmentation, 5 underwent less than 40% augmentation. Thirty-eight had surgical dose modified to correct strabismus, and 3 underwent surgery different from standard Kestenbaum procedures. Collapse of AHP: At the 1-3-week follow-up (n = 131), 125 patients (95%) had collapse of AHP. The percentage trended down at the 2-5-month (91%, n = 106) and 2-year follow-ups (83%, n = 57). However, at 5 and 10 years, it was 93% (n = 42) and 93% (n = 14), respectively, due to reoperation in a small minority. Over- and undercorrection: At 1-3 weeks, 5% of patients were overcorrected whereas 0% were undercorrected. Over- and undercorrection rates peaked at 2 years postoperatively. Ten years out, there were no overcorrections and 7% undercorrections. Four percent of patients required reoperation for overcorrection (mean 2.7 years) and 5% did for undercorrection (mean 3.9 years). CONCLUSION: Surgery for the head positioning associated with INS produces excellent outcomes throughout 10 years postoperatively. Overcorrection presents early and resolves either over time or with additional surgery. Undercorrection develops later and can persist despite reoperation.

Management of Strabismus Associated With Infantile Nystagmus Syndrome: A Novel Classification to Assist in Surgical Planning.

PURPOSE: There is no consensus on the surgical management of head position associated with infantile nystagmus syndrome (INS) when strabismus coexists, and few outcome data have been published. We propose classifying strabismus into concordant or discordant based on the relationship between head positioning and strabismus and then modifying surgery accordingly. Our objective is to describe this system and to review surgical outcomes. DESIGN: Retrospective observational case series. METHODS: Twenty-eight patients with INS and coexisting horizontal strabismus underwent surgery for horizontal head positioning and had ≥2 months of follow-up from 1995-2018 at the Vanderbilt Eye Institute. Outcome variables included head positioning (minimal, ≤10°; mild, 11-30°; moderate 31-44°; and severe ≥45°), strabismus (range 0-70 prism diopters [PDs]; minimal ≤10 PD), and reoperation rates. Nonparametric Wilcoxon signed rank, Fisher exact, and Mann-Whitney U tests were used for statistical analysis. RESULTS: Twenty-one cases were concordant and 7 were discordant; the mean follow-up was 4.1 years. Ninety-six percent of patients had moderate to severe head positioning at baseline. Correction rates (to minimal) were 100% at 2-5 months postoperatively and 86% at last follow-up (P < .0001 at both time points compared with preoperatively). The magnitude of strabismus decreased compared with preoperative strabismus (30.8 ± 10.8 PDs; n = 28), strabismus at 2-5 months (9.1 ± 11.9 PDs; P = .0001; n = 26), and last follow-up (12.0 ± 14.1 PDs; P = .0003; n = 28). The overall reoperation rate was 32%. CONCLUSIONS: Our classification system in patients with INS allows a systematic way to surgically improve head positioning and strabismus in cases of moderate to severe baseline head positioning.