Successful Case of Teprotumumab Treatment in an Adolescent Patient With Thyroid Eye Disease.

A 16-year-old black female presented with a 4-month history of significant proptosis and diplopia in the setting of diagnosed Graves disease. The patient underwent 8 infusions of teprotumumab. She had migraines and diplopia that were resolved with treatment. There was also a dramatic improvement in her proptosis. The authors present the first reported case of successful teprotumumab treatment in an adolescent patient, describing outcomes and proposing a mechanism for her transient side effects.

A case of necrotizing fasciitis of the orbit secondary to Aspergillus fumigatus and mixed flora.

A 79-year-old female presented to the emergency department for sudden-onset ocular pain, edema, and erythema around her left eye. She also had a left-sided migraine and frontal fullness for 2 weeks. She had attentive care for the diagnosis of orbital cellulitis and prompt recognition of necrotizing fasciitis. Wound cultures were positive for over 5 strains of bacteria in addition to Aspergillus. In a combined effort by our institution's Ophthalmology and Otolaryngology departments, the patient was successfully treated with debridement, porcine bladder matrix, antibiotics, and antifungals. The authors describe the first reported case of eyelid and periorbital necrotizing fasciitis, caused by mixed flora and Aspergillus fumigatus, that showed promising wound healing with the outlined treatment paradigm.

Orbital subperiosteal abscess in adolescents and adults: predictors and outcomes of nonsurgical management and surgical approaches.

PURPOSE: The aim of this study was to assess predictors and outcomes of subperiosteal abscess (SPA) management in adolescents and adults at two tertiary care centers. METHODS: This retrospective cohort study included cases of SPA from January 1 2000 to October 9 2022 at two institutions. Patients 9 years or older were categorized into surgical and nonsurgical cohorts. Surgical subgroups included those who underwent functional endoscopic sinus surgery (FESS) alone, external (transcutaneous or transconjunctival) orbitotomy alone, or combined FESS and external surgery. The presented features were assessed as potential treatment predictors. Outcomes included length of stay (LOS), final best-corrected visual acuity (BCVA), readmission rate, and reoperation rate. RESULTS: Of the 159 SPA cases included, 127 (79.9%) underwent surgery and 32 (20.1%) were managed nonsurgically. The nonsurgical cohort was younger (p = .003) with smaller abscesses (p < .001) that were more likely to be medial (p < .001). The nonsurgical cohort had shorter LOS (p < .001); final BCVA and readmission rates were similarly favorable. Abscess location was correlated with surgical approach. Superior SPA that underwent FESS or external surgery alone had higher reoperation rates (57.1.0% and 58.3%, respectively) than combined (17.9%). External approach and FESS alone resulted in lower reoperation rates (15.4% and 15.0%, respectively) than combined (27.3%) for medial SPA. Subgroup analysis in the sinusitis cohort yielded similar results. CONCLUSIONS: A trial of nonsurgical management may be safe and effective for select patients aged 9 years and older with sinusitis-derived, medial, and small SPA. When surgery is indicated, approach should be guided by abscess location to minimize reoperation risk.

Threading the Needle: A Case of Periorbital Gold Threading.

While a rare periorbital finding, the aesthetic practice of gold threading is increasingly identified in Western care setting and may be misidentified as the practice of inserting charm needles (susuk). The authors present a unique case of gold threading discovered incidentally during workup of chronic sinusitis and report a rarely seen delayed local site reaction. The practice of gold threading and mimickers including the practice of inserting charm needles (susuk) are reviewed with emphasis on clinical and radiographic differentiation by oculoplastic surgeons.

Solitary sclerotic fibroma of the palpebral conjunctiva: a unique presentation of a rare entity.

Sclerotic fibroma (SF) is an uncommon yet benign tumor that may occasionally be associated with Cowden's disease that presents as an asymptomatic, well-circumscribed lesion. We present a rare case of a patient with a solitary SF of the palpebral conjunctiva. The patient was an 85-year-old male who presented with a right lower lid nodule that was initially treated as a chalazion. Excision yielded a dense mass that was sent to pathology for evaluation. Histologically, the lesion showed a bland storiform spindle cell proliferation embedded in a sclerotic stroma with prominent clefting.

Orbital Subperiosteal Abscess: The Role of Nonsurgical Management Among Adolescents and Adults.

PURPOSE: To investigate the role of nonsurgical management in the treatment of orbital cellulitis complicated by subperiosteal abscess (SPA) in adolescent and adult populations. METHODS: A retrospective cohort study to assess the demographic, clinical, and outcome profiles of adolescent and adult patients with orbital SPA treated nonsurgically versus those who received surgical intervention. Primary outcome measures included hospitalization length and best-corrected visual acuity (BCVA) in the final evaluation. RESULTS: The study comprised 76 patients diagnosed with SPA in the setting of orbital cellulitis. Twelve were stratified into the nonsurgical cohort, while 64 represented the surgical group. Sinusitis was the most prevalent risk factor among both populations, and the rate did not differ significantly. SPA was located medially at a significantly higher rate in the nonsurgical cohort compared with the surgical. No patients in the nonsurgical population had a relative afferent pupillary defect (RAPD) on presentation. The proportion of patients who received adjunctive systemic corticosteroid therapy was comparable in both groups. Hospitalization length was significantly higher in the surgery cohort. BCVA in the final evaluation was favourable in both groups. Sinusitis subgroup analysis including eight nonsurgical patients produced overall consistent results. CONCLUSIONS: Findings from the largest study of orbital cellulitis complicated by SPA to date demonstrate a role for nonsurgical management in adolescent and adult patients who present with a medial SPA and/or ophthalmic examination without an RAPD. Among these populations, visual prognosis is favourable whether management includes surgical debridement or not, and surgical intervention may not predict a shorter duration of hospitalization.

Electrical burn of the eye and orbit with severe delayed sequelae from an arc welding accident.

A 28-year-old male presented to the emergency room suffering an ocular burn injury from a welding rod. Given the mechanism of injury, severe delayed injury of the ocular adnexa occurred, requiring enucleation, partial exenteration of the superior orbit, and extensive reconstruction. Histopathology of the affected tissue was analyzed. This is the first report that details the clinical course of a patient with delayed high amperage and low voltage electrical burn injury.

Breaking the mold: a case of recalcitrant eyelid subconjunctival infection by Exophilia Phaeomuriformis.

While a rare ophthalmic pathogen, infections from Exophilia spp. are increasingly identified and have been associated with catastrophic vision loss. In this case report we present a previously undescribed manifestation of the melanin-producing fungus Exophilia Phaeomuriformis to the lower eyelid, establish an effective treatment, and review related cases. Previous cases of ophthalmic E. Phaeomuriformis were confined to the cornea and included iatrogenic tissue trauma. This case shares neither associations however includes a remote SJS history that likely led to changes in conjunctival tissue integrity. Previous cases of Exophilia spp. infecting the eyelid both included surgical source control and adjuvant antibiotic. In this case, topical therapy was deferred due to SJS-related ocular cicatricial disease. Fortunately, a full resolution was achieved with surgical resection and oral antifungal treatment.

Punctal Stenosis Associated with Topical Netarsudil Use.

PURPOSE: To report a series of patients who developed punctal stenosis secondary to the use of topical netarsudil 0.02% for treatment of glaucoma. DESIGN: Case series. PARTICIPANTS: Patients using topical netarsudil for management of glaucoma and noted to have punctal stenosis ipsilateral to the eye(s) being treated with netarsudil were included. METHODS: Each enrolled patient's chart was reviewed, and alternative causes of punctal stenosis were sought. Photographs were obtained to document punctal stenosis for some patients. MAIN OUTCOME MEASURES: Presence of punctal stenosis after topical netarsudil use and resolution of punctal stenosis after cessation of therapy. RESULTS: Sixteen patients had punctal stenosis; 13 developed unilateral punctal stenosis while using netarsudil unilaterally, and 3 patients developed bilateral punctal stenosis with bilateral use. Time from initiation of netarsudil to recognition of symptoms or documentation of punctal stenosis ranged from 2 to 35 months (median, 12; mean, 14.0 ± 8.7 months). Thirteen patients endorsed tearing, but 2 had no symptoms. Ectropion was seen in 1 eye. Corneal verticillata was noted in 14 patients (87.5%). In 8 cases, netarsudil was discontinued, and the punctal stenosis was reversed, with resolution of associated symptoms. CONCLUSIONS: Netarsudil use can lead to the development of reversible punctal stenosis. This inflammation-mediated stenosis may cause tearing and associated symptoms and may be of sufficient severity to necessitate discontinuation of treatment. In this case series, all patients who discontinued treatment had reversal of their punctal stenosis and associated symptoms.

Nasolacrimal Lymphangioma Presenting With Hemolacria.

A 22-year-old male presented with spontaneous unilateral hemolacria and was found to have complete opacification of the ipsilateral nasolacrimal sac and duct. Endoscopic biopsy with histopathologic analysis revealed the diagnosis of nasolacrimal lymphangioma. Additional management included nasolacrimal stenting, and there was no hemolacria recurrence or epiphora. This is the first report of a nasolacrimal lymphangioma and describes the vascular malformation as a potential cause of bloody tearing.

Metastatic Renal Cell Carcinoma Masquerading as a Sphenoid Wing Meningioma.

A 47-year-old man who presented with subacute binocular diplopia and a left head turn was found to have a right sixth nerve palsy and right optic disc edema. Radiologic imaging revealed a non-lytic right greater sphenoid wing mass with a dural tail, suggestive of a sphenoid wing meningioma. The patient underwent an orbitotomy with lesion biopsy; histopathologic analysis and subsequent imaging revealed the diagnosis of metastatic clear cell renal cell carcinoma. He developed new metastases despite systemic immunotherapy, and prognosis was guarded at last follow up 3 months after diagnosis. The authors present the first reported case of renal cell carcinoma metastatic to the sphenoid wing without sinus involvement, describing an atypical presentation of an aggressive malignancy that necessitates timely diagnosis for possible survival.

Dacryoadenitis due to gonococcal conjunctivitis complicated by corneal perforation.

Adnexal and periocular involvement in Neisseria gonorrhoeae (NG) infection is rare. This report describes the case of a patient with a delayed diagnosis of gonococcal dacryoadenitis with contiguous conjunctivitis and corneal involvement. She underwent extensive inpatient laboratory and infectious workup but rapidly progressed to corneal perforation requiring emergent penetrating keratoplasty prior to a positive culture confirming the diagnosis. To date, this is the first reported case of ophthalmologic NG infection with associated conjunctivitis, dacryoadenitis, and corneal perforation.

Periorbital Necrotizing Fasciitis Secondary to Candida parapsilosis and Streptococcus pyogenes.

Necrotizing fasciitis is most often caused by either polymicrobial bacterial infections or by Gram-positive organisms, such as Streptococcus or Staphylococcus; however, rare cases of fungal necrotizing fasciitis have been reported. Candida parapsilosis is an emerging fungal pathogen. This fungus grows in either a yeast or pseudohyphal form. C. parapsilosis has been reported to cause keratitis, intraocular infection, and seeding of frontalis slings. C. parapsilosis is a commensal of human skin and can be acquired by nosocomial spread. Necrotizing fasciitis due to Candida has rarely been reported, but to date C. parapsilosis has not been identified as the causative organism in necrotizing fasciitis. This is the first documented case of human periocular soft tissue infection by C. parapsilosis, and also the first report providing evidence of mycotic infection in a necrotizing fasciitis concurrently infected by Streptococcus pyogenes.

Contribution of alloantigens to hepatic ischemia/reperfusion injury: Roles of natural killer cells and innate immune recognition of nonself.

Hepatic ischemia/reperfusion injury (IRI) remains a major clinical problem and involves the innate immune system's recognition of "nonself." Considering the efficient nonself recognition by natural killer (NK) cells, we hypothesize in this study that hepatic IRI associated with liver transplantation (LT) could be augmented in allogeneic rather than in syngeneic (Syn) grafts due to alloantigen recognition by innate immune cells, especially by NK cells. Using green fluorescent protein (GFP)/Sprague-Dawley rats, we tested our hypothesis in a rat LT model with 18 hours of cold storage in University of Wisconsin solution. Hepatic IRI was significantly augmented in allografts with higher alanine transaminase levels, increased necrosis, and vigorous proinflammatory mediator up-regulation compared to Syn grafts. Injury increased in allografts associated with augmented GFP+ host leukocyte infiltration due to significantly increased host CD11b/c+ and RP-1(+) neutrophil recruitment. A large number of liver-resident (donor) mature CD11b/c+ NK cells quickly diminished from allografts, but not from Syn grafts. Depletion of mature NK cells from liver grafts with anti-asialo monosialotetrahexosylganglioside significantly improved hepatic IRI and reduced neutrophil infiltration and proinflammatory mediators. In conclusion, early innate immune responses were more significantly enhanced in allografts than in Syn grafts during hepatic IRI, in part through NK cell recognition of "missing self."

Roles of dendritic cells in murine hepatic warm and liver transplantation-induced cold ischemia/reperfusion injury.

UNLABELLED: Dendritic cells (DCs) induce and regulate both innate and adaptive immune responses; however, their in vivo functional importance in hepatic ischemia/reperfusion (IR) injury is perplexing. We hypothesized that liver-resident DC and locally recruited blood-borne DC might have distinctive roles in hepatic IR injury. We tested this hypothesis by using DC-deficient, fms-like tyrosine kinase 3 ligand (Flt3L) knockout (KO) mice in hepatic warm (70% partial clamping for 60 minutes) and cold IR injury (liver transplant [LTx] with 24-hour cold storage). Flt3L KO liver and lymphoid organs contained virtually no CD11c+ F4/80- DC. Hepatic warm IR injury was significantly lower in Flt3L KO than in wildtype (WT) mice with lower alanine aminotransferase (ALT) levels, reduced hepatic necrosis, and lower neutrophil infiltration. Hepatic messenger RNA (mRNA) and protein levels for inflammatory cytokines (tumor necrosis factor alpha [TNFα], interleukin [IL]-6) and chemokines (CCL2, CXCL2) were also significantly lower in Flt3L KO than in WT mice, indicating that lack of both liver-resident and blood-borne DC ameliorated hepatic warm IR injury. Adoptive transfer of splenic or hepatic WT DC into Flt3L KO or WT mice increased hepatic warm IR injury, suggesting injurious roles of DC infusion. When Flt3L KO liver was transplanted into WT mice, ALT levels were significantly higher than in WT to WT LTx, with enhanced hepatic necrosis and neutrophil infiltration, indicating a protective role of liver-resident DC. CONCLUSION: Using both warm and cold hepatic IR models, this study suggests differential roles of liver-resident versus blood-borne DC, and points to the importance of the local microenvironment in determining DC function during hepatic IR injury.

Utility of diffusion-weighted imaging to differentiate benign and malignant solid orbital tumours.

OBJECTIVE: We assessed the utility of apparent diffusion coefficients (ADCs) derived from diffusion-weighted imaging to differentiate benign and malignant orbital tumours by oculoplastic surgeons in the clinical setting and sought to validate observed ADC cut-off values. DESIGN AND PARTICIPANTS: Retrospective review of patients with benign or malignant biopsy-confirmed orbital tumours. METHODS: Blinded graders including 2 oculoplastic surgeons, 1 neuroradiologist, and 1 medical student located and measured orbital tumour ADCs (10-6 mm2/s) using the Region of Interest tool. OUTCOME MEASURES: Nonradiologist measurements were compared with each other to assess reliability and with an expert neuroradiologist measurement and final pathology to assess accuracy. RESULTS: Twenty-nine orbital tumours met inclusion criteria, consisting of 6 benign tumours and 23 malignant tumours. Mean ADC values for benign orbital tumours were 1430.59 ± 254.81 and 798.68 ± 309.12 mm2/s for malignant tumours. Our calculated optimized ADC cut-off to differentiate benign from malignant orbital tumours was 1120.84 × 10-6 mm2/s (sensitivity 1, specificity 0.9). Inter-rater reliability was excellent (intraclass correlation coefficient = 0.92; 95% CI, 0.86-0.96). Our 3 graders had a combined accuracy of 84.5% (92.3%, 92.3%, and 65.4%). CONCLUSIONS: Our ADC cut-off of 1120.84 × 10-6 mm2/s for benign and malignant orbital tumours agrees with previously established values in literature. Without priming with instructions, training, or access to patient characteristics, most tumours were correctly classified using rapid ADC measurements. Surgeons without radiologic expertise can use the ADC tool to quickly risk stratify orbital tumours during clinic visits to guide patient expectations and further work-up.

Artificial Intelligence Automation of Proptosis Measurement: An Indicator for Pediatric Orbital Abscess Surgery.

INTRODUCTION: To evaluate the ability of artificial intelligence (AI) software to quantify proptosis for identifying patients who need surgical drainage. METHODS: We pursued a retrospective study including 56 subjects with a clinical diagnosis of subperiosteal orbital abscess (SPOA) secondary to sinusitis at a tertiary pediatric hospital from 2002 to 2016. AI computer software was developed to perform 3D visualization and quantitative assessment of proptosis from computed tomography (CT) images acquired at the time of hospital admission. The AI software automatically computed linear and volume metrics of proptosis to provide more practice-consistent and informative measures. Two experienced physicians independently measured proptosis using the interzygomatic line method on axial CT images. The AI software and physician proptosis assessments were evaluated for association with eventual treatment procedures as standalone markers and in combination with the standard predictors. RESULTS: To treat the SPOA, 31 of 56 (55%) children underwent surgical intervention, including 18 early surgeries (performed within 24 h of admission), and 25 (45%) were managed medically. The physician measurements of proptosis were strongly correlated (Spearman r = 0.89, 95% CI 0.82-0.93) with 95% limits of agreement of ± 1.8 mm. The AI linear measurement was on average 1.2 mm larger (p = 0.007) and only moderately correlated with the average physicians' measurements (r = 0.53, 95% CI 0.31-0.69). Increased proptosis of both AI volumetric and linear measurements were moderately predictive of surgery (AUCs of 0.79, 95% CI 0.68-0.91, and 0.78, 95% CI 0.65-0.90, respectively) with the average physician measurement being poorly to fairly predictive (AUC of 0.70, 95% CI 0.56-0.84). The AI proptosis measures were also significantly greater in the early as compared to the late surgery groups (p = 0.02, and p = 0.04, respectively). The surgical and medical groups showed a substantial difference in the abscess volume (p < 0.001). CONCLUSION: AI proptosis measures significantly differed from physician assessments and showed a good overall ability to predict the eventual treatment. The volumetric AI proptosis measurement significantly improved the ability to predict the likelihood of surgery compared to abscess volume alone. Further studies are needed to better characterize and incorporate the AI proptosis measurements for assisting in clinical decision-making.

Orbital resection by intranasal technique (ORBIT): A new classification system for reporting endoscopically resectable primary benign orbital tumors.

BACKGROUND: The Cavernous Hemangioma Exclusively Endonasal Resection (CHEER) staging system has become the gold standard for outcomes reporting in endoscopic orbital surgery for orbital cavernous hemangiomas (OCHs). A recent systematic review demonstrated similar outcomes between OCHs and other primary benign orbital tumors (PBOTs). Therefore, we hypothesized that a simplified and more comprehensive classification system could be developed to predict surgical outcomes of other PBOTs. METHODS: Patient and tumor characteristics as well as surgical outcomes from 11 international centers were recorded. All tumors were retrospectively assigned an Orbital Resection by Intranasal Technique (ORBIT) class and stratified based on surgical approach as either exclusively endoscopic or combined (endoscopic and open). Outcomes based on approach were compared using chi-squared or Fisher's exact tests. The Cochrane-Armitage test for trend was used to analyze outcomes by class. RESULTS: Findings from 110 PBOTs from 110 patients (age 49.0 ± 15.0 years, 51.9% female) were included in the analysis. Higher ORBIT class was associated with a lower likelihood of gross total resection (GTR). GTR was more likely to be achieved when an exclusively endoscopic approach was utilized (p < 0.05). Tumors resected using a combined approach tended to be larger, to present with diplopia, and to have an immediate postoperative cranial nerve palsy (p < 0.05). CONCLUSION: Endoscopic treatment of PBOTs is an effective approach, with favorable short-term and long-term postoperative outcomes as well as low rate of adverse events. The ORBIT classification system is an anatomic-based framework that effectively facilitates high-quality outcomes reporting for all PBOTs.

The loss of renal dendritic cells and activation of host adaptive immunity are long-term effects of ischemia/reperfusion injury following syngeneic kidney transplantation.

Ischemia/reperfusion injury associated with kidney transplantation induces profound acute injury, influences early graft function, and affects long-term graft outcomes. To determine whether renal dendritic cells play any role during initial innate ischemia/reperfusion injury and the subsequent development of adaptive immune responses, we studied the behavior and function of renal graft and host infiltrating dendritic cells during early and late phases of renal ischemia/reperfusion injury. Wild type to green fluorescent protein (GFP) transgenic rat kidney transplantation was performed with and without 24-h cold storage. Ischemia/reperfusion injury in cold-stored grafts resulted in histopathological changes of interstitial fibrosis and tubular atrophy by 10 weeks, accompanied by upregulation of mRNAs of mediators of interstitial fibrosis and inflammation. In normal rat kidneys, we identified two populations of renal dendritic cells, predominant CD103(-)CD11b/c(+) and minor CD103(+)CD11b/c(+) cells. After transplantation without cold storage, grafts maintained CD103(-) but not CD103(+) GFP-negative renal dendritic cells for 10 weeks. In contrast, both cell subsets disappeared from cold-stored grafts, which associated with a significant GFP-expressing host CD11b/c(+) cell infiltration that included CD103(+) dendritic cells with a TNF-α-producing phenotype. These changes in graft/host dendritic cell populations were associated with progressive infiltration of host CD4(+) T cells with effector/effector-memory phenotypes and IFN-γ secretion. Thus, renal graft ischemia/reperfusion injury caused graft dendritic cell loss and was associated with progressive host dendritic cell and T-cell recruitment. Renal-resident dendritic cells might function as a protective regulatory network.

Hepatic B7 homolog 1 expression is essential for controlling cold ischemia/reperfusion injury after mouse liver transplantation.

UNLABELLED: Ischemia/reperfusion (I/R) injury remains a key risk factor significantly affecting morbidity and mortality after liver transplantation (LT). B7 homolog 1 (B7-H1), a recently identified member of the B7 family, is known to play important roles in regulating local immune responses. We hypothesized that B7-H1 plays crucial roles during innate immune responses induced by hepatic I/R injury, and using B7-H1 knockout (KO) liver grafts, we tested this hypothesis in the mouse LT model with 24 hours of cold storage. Cold I/R injury in wild type (WT)-to-WT LT enhanced constitutive B7-H1 expression on dendritic cells and sinusoidal endothelial cells and promptly induced B7-H1 on hepatocytes. When B7-H1 KO liver grafts were transplanted into WT recipients, serum alanine aminotransferase (ALT) and graft necrosis levels were significantly higher than those after WT-to-WT LT. Augmented tissue injury in B7-H1 KO grafts was associated with increased frequencies and absolute numbers of graft CD3(+) T cells (particularly CD8(+) T cells). B7-H1 KO grafts had significantly fewer annexin V(+) CD8(+) T cells, and this indicated a failure to delete infiltrating CD8(+) T cells. To evaluate the relative contributions of parenchymal cell and bone marrow-derived cell (BMDC) B7-H1 expression, we generated and transplanted into WT recipients chimeric liver grafts lacking B7-H1 on parenchymal cells or BMDCs. A selective B7-H1 deficiency on parenchymal cells or BMDCs resulted in similar levels of ALT and liver injury, and this suggested that parenchymal cell and BMDC B7-H1 expression was involved in liver damage control. Human livers up-regulated B7-H1 expression after LT. CONCLUSION: The study demonstrates that graft tissue expression of B7-H1 plays a critical role in regulating inflammatory responses during LT-induced hepatic I/R injury, and negative coregulatory signals may have an important function in hepatic innate immune responses.