Surgical Outcomes of Revision Orbital Reconstruction in Patients With Inadequate Primary Orbital Fracture Repair.

OBJECTIVE: This study aimed to investigate the clinical characteristics of patients with complications following inadequate primary orbital fracture repair and to evaluate surgical outcomes of secondary revision orbital reconstruction. METHODS: The authors retrospectively reviewed data from 41 patients who underwent revision orbital reconstruction by a single surgeon following complications from primary orbital fracture repair performed elsewhere. Clinical characteristics, including enophthalmos, exophthalmos, diplopia, ocular motility limitation, epiphora, infraorbital hypoesthesia, infection, eyelid malposition, lagophthalmos, hypoglobus, and compressive optic neuropathy, were assessed. Surgical outcomes of revision surgery were evaluated to determine improvements in clinical deficits and postoperative patient satisfaction. RESULTS: The most common postoperative complications of primary orbital fracture repair were enophthalmos (n=20/41) and diplopia (n=20/41). The mean time between primary and revision surgeries was 67.2 months (range: 1-276 mo). Revision surgery significantly improved enophthalmos, diplopia (Hess area ratio), epiphora (Munk score), periorbital pain, and exophthalmos (P=0.003, P=0.001, P<0.001, P<0.001, and P=0.007, respectively) compared to the pre-revision state. In addition, 6 patients experienced improved infraorbital hypoesthesia. Among the 41 patients, 23 were very satisfied, 17 were satisfied, and 1 was neutral after revision orbital reconstruction. CONCLUSIONS: Our study highlights the positive impact of revision orbital reconstruction in addressing complications from inadequate primary orbital fracture repair. Surgeons should consider revision surgery to address clinical deficits following prior surgery, especially when anatomic abnormalities are evident in imaging studies, regardless of the time lapse since the initial surgery or concerns about tissue fibrosis and fat atrophy.

Incidence and risk factors of age-related macular degeneration in patients with Parkinson's disease: a population-based study.

Background and objective: The association between age-related macular degeneration (AMD) and Parkinson' disease (PD) remains unclear. The aim of the present study was to assess the incidence of AMD in patients with PD, elucidate differences by age and sex, and investigate potential risk factors for AMD. Methods: Data were extracted from the Korean National Health Insurance System database, which covers 97% of the Korean population (2002 through 2019). We calculated the incidence of newly diagnosed AMD in patients with PD and used Cox proportional-hazards models to estimate risk factors for AMD, presenting adjusted hazard ratios (aHR) with 95% confidence intervals (CI). Results: Of 172,726 patients with PD, 15,800 were newly diagnosed with AMD during the follow-up, including 5,624 men and 10,176 women. The overall incidence of AMD in patients with PD was 13.59 per 1,000 person-years. Stratified by age group and sex, the incidence was higher in women aged 40-69, and conversely higher in men aged 70-89. Risk of AMD was high in older age groups (aHR = 4.36, 95% CI: 3.74-5.09 in the 70 s), female sex (aHR = 1.07, 95% CI: 1.04-1.11), patients with diabetes mellitus (DM) (aHR = 1.14, 95% CI: 1.10-1.18), and patients with hyperlipidemia (aHR = 1.17, 95% CI: 1.13-1.21). Conclusion: Our findings suggest that the AMD incidence is higher in patients with PD than in the general population, with varying patterns of sex differences across age groups. Particularly, old age, female sex, presence of DM, and hyperlipidemia are potential risk factors. Therefore, clinicians should pay greater attention to AMD in patients with PD.

Prognostic Significance of Tumor-Infiltrating Lymphocytes and High-Risk Human Papillomavirus in Ocular Sebaceous Carcinoma: A Comprehensive Analysis.

High-risk human papillomavirus (hrHPV) and tumor-infiltrating lymphocytes (TILs) are known to have prognostic significance in oropharyngeal squamous cell carcinoma. However, their significance in ocular sebaceous carcinoma (OSC) remains unverified because of the rarity of the condition. This study aimed to investigate the association between clinicopathologic features, biomarkers, and hrHPV infection and their potential to predict prognosis in OSC patients. We analyzed the clinicopathologic features of 81 OSC patients from Asan Medical Center between 2000 and 2022. Seventeen biomarkers and hrHPV were examined using immunohistochemistry and DNA in situ hybridization on tissue microarray cores. hrHPV was identified in 31 cases (38.3%). Univariate analysis revealed that hrHPV infection was associated with comedonecrosis (P = .032), high Ki-67 labeling index (≥30%, P = .042), lower expression of E-cadherin (P = .033), and loss of expression of zinc finger protein 750 (P = .023). Multivariate analysis revealed that loss of expression of zinc finger protein 750 (P = .026) remained an independently associated factor for hrHPV. Progression-free survival analysis was performed on 28 patients who were continuously observed for more than 5 years. During a median follow-up duration of 86 months, recurrence or metastasis developed in 14 patients (50%) within the survival cohort, occurring at a median time of 48 months after excision. Univariate analysis indicated that recurrence or metastasis was associated with tumor size (P = .010), high TILs (≥10%; P = .025), lymphovascular invasion (P = 0.043), site of origin (P = .025), and high expression of bcl-2-associated athanogene 3 (P = .039). Multivariate analysis demonstrated that high TILs (P = .017) and site of origin (P = .025) were independent prognostic factors. The prognosis of OSC was hrHPV-independent, and a better prognosis was associated with the site of origin in the order of the gland of Zeis, meibomian gland, and multicentric site, as well as with high TILs.

Efficacy and safety of intravenous glucocorticoid therapy for IgG4-related ophthalmic disease.

PURPOSE: To evaluate and compare the efficacy and safety of intravenous (IV) glucocorticoid therapy with those of oral glucocorticoids as a first-line treatment for IgG4-related ophthalmic disease (IgG4-ROD). METHODS: We retrospectively reviewed the medical records of patients who underwent systemic glucocorticoid therapy for biopsy-proven IgG4-ROD from June 2012 to June 2022. Glucocorticoids were given either oral prednisolone at an initial dose of 0.6 mg/kg/day for four weeks with subsequent tapering or once weekly IV methylprednisolone (500 mg for six weeks, then 250 mg for six weeks), according to the date of treatment. Clinicoserological features, initial response, relapse during follow-ups, cumulative doses of glucocorticoids, and adverse effects of glucocorticoids were compared for the IV and oral steroid groups. RESULTS: Sixty one eyes of 35 patients were evaluated over a median follow-up period of 32.9 months. The complete response rate was significantly higher in the IV steroid group (n = 30 eyes) than in the oral steroid group (n = 31 eyes) (66.7% vs. 38.7%, p = 0.041). Kaplan-Meier analysis showed that the 2-year relapse-free survival was 71.5% (95% confidence interval: 51.6-91.4) and 21.5% (95% confidence interval: 4.5-38.5) in the IV steroid and oral steroid group, respectively (p < 0.001). Although the cumulative dose of glucocorticoids was significantly higher in the IV steroid group than in the oral steroid group (7.8 g vs. 4.9 g, p = 0.012), systemic and ophthalmic adverse effects were not significantly different between the two groups throughout follow-ups (all p > 0.05). CONCLUSIONS: As a first-line treatment for IgG4-ROD, IV glucocorticoid therapy was well-tolerated, led to better clinical remission and more effectively prevented inflammatory relapse than oral steroids. Further research is needed to establish guidelines on dosage regimens.

Effectiveness of An Intraoperative Lagophthalmos Formula for Levator Resection in Patients With Congenital Ptosis.

PURPOSE: To evaluate the effectiveness of an intraoperative lagophthalmos formula (IOLF) for levator resection in congenital ptosis and investigate the optimal preoperative conditions for IOLF application. METHODS: This retrospective interventional cohort study evaluated 30 eyelids of 22 patients with congenital ptosis who underwent levator resection using the IOLF to calculate the extent of surgical correction under general anesthesia. Surgical success was defined as margin reflex distance-1 (MRD1)≥3 mm in each eye and a difference of MRD1 1 mm between the eyes at 6 months postoperatively. Logistic regression was performed to investigate the preoperative conditions associated with surgical success. RESULTS: Among 30 eyelids, 19 had good-to-fair levator function (LF) (≥5 mm) and 11 had poor LF ( 4 mm). The overall success rate was 90.0% (n=27/30), whereas the under-correction rate was 10.0% (n=3/30). The surgical success rate was 100% (n=19/19) in eyelids with LF ≥5 mm and 72.7% (n=8/11) in eyelids with LF 4 mm. Patients with preoperative MRD1≥0 mm (versus MRD1<0 mm, odds ratio=34.5, P =0.0098) or a combination of preoperative MRD1≥0 mm and LF≥5 mm (versus MRD1<0 mm and LF 4 mm, odds ratio=48.0, P =0.0124) more likely had successful surgical outcomes. CONCLUSIONS: Levator resection using the IOLF can provide satisfactory results for congenital ptosis regardless of LF. Preoperative MRD1≥0 mm may be suitable for IOLF application, and the combination of preoperative MRD≥0 mm and LF≥5 mm may be the optimal preoperative condition for IOLF application.

Clinical and Radiological Features of Diffuse Lacrimal Gland Enlargement: Comparisons among Various Etiologies in 91 Biopsy-Confirmed Patients.

OBJECTIVE: To compare the clinical and radiological features of various etiologies of chronic diffuse lacrimal gland enlargement. MATERIALS AND METHODS: We retrospectively reviewed 91 consecutive patients who underwent surgical biopsy for chronic diffuse lacrimal gland enlargement and were diagnosed with non-specific dacryoadenitis (DA) (n = 42), immunoglobulin G4-related dacryoadenitis (IgG4-RD) (n = 33), and lymphoma (n = 16). Data on patient demographics, clinical presentation, and CT imaging findings (n = 73) and MRI (n = 43) were collected. The following radiologic features of lacrimal gland enlargement were evaluated: size, unilaterality, wedge sign, angle with the orbital wall, heterogeneity, signal intensity, degree of enhancement, patterns of dynamic contrast-enhanced, and apparent diffusion coefficient value. Radiological features outside the lacrimal glands, such as extra-lacrimal orbital involvement and extra-orbital head and neck involvement, were also evaluated. The clinical and radiological findings were compared among the three diseases. RESULTS: Compared to the DA and IgG4-RD groups, the lymphoma group was significantly older (mean 59.9 vs. 46.0 and 49.4 years, respectively; p = 0.001) and had a higher frequency of unilateral involvement (62.5% vs. 31.0% and 15.2%, respectively; p = 0.004). Compared to the IgG4-RD and lymphoma groups, the DA group had significantly smaller lacrimal glands (2.3 vs. 2.8 and 3.3 cm, respectively; p < 0.001) and a lower proportion of cases with a wedge sign (54.8% vs. 84.8% and 87.5%, respectively; p = 0.005). The IgG4-RD group showed more frequent involvement of the extra-orbital head and neck structures, including the infraorbital nerve (36.4%), paranasal sinus (72.7%), and salivary gland (58.6%) compared to the DA and lymphoma groups (4.8%-28.6%) (all p < 0.005). CONCLUSION: Patient age, unilaterality, lacrimal gland size, wedge sign, and extra-orbital head and neck involvement differed significantly different between lymphoma, DA, and IgG4-RD. Our results will be useful for the differential diagnosis and proper management of chronic lacrimal gland enlargement.

Update on Immune Checkpoint Inhibitors for Conjunctival Melanoma.

The management of conjunctival melanoma is challenging due to the more frequent local recurrence and metastasis compared to other conjunctival neoplasms. Locally advanced conjunctival melanoma may require an orbital exenteration, and treatment options for metastatic conjunctival melanoma have been limited until recently. This review aims to provide comprehensive updates on immunotherapy for conjunctival melanoma, focusing on immune checkpoint inhibitors. We reviewed the available literature on the use of immunotherapy for the treatment of conjunctival melanoma. Systemic immunotherapy, particularly with checkpoint inhibitors, has recently been reported to have improved outcomes for patients with conjunctival melanoma. Immune checkpoint inhibitors that are currently approved by the US Food and Drug Administration for melanoma include anti-PD-1 (nivolumab and pembrolizumab), anti-PDL-1 (avelumab and atezolizumab), and anti-CTLA-4 inhibitors (ipilimumab). Most recent reports described using immune checkpoint inhibitors in patients with locally advanced conjunctival melanoma in an attempt to avoid orbital exenteration or in patients with metastatic conjunctival melanoma.Although the current data are limited to case reports and small case series, eye care providers should be aware of the potential role of immunotherapy for patients with locally advanced, recurrent, or metastatic conjunctival melanoma.

Quantitative analysis of morphological and functional alterations of the meibomian glands in eyes with marginal entropion.

PURPOSE: To quantitatively analyze morphological and functional alterations of the meibomian glands in eyes with marginal entropion and their changes after surgery. METHODS: Sixty eyes of 52 patients with marginal entropion and underwent meibography and interferometer were included. One-hundred and seventeen age- and sex-matched eyes with minimal to mild meibomian gland dysfunction (MGD) were recruited as control eyes. Meibomian gland loss (MGL) and lipid layer thickness (LLT) were compared between eyes with marginal entropion and control eyes. Subgroup analysis was performed according to the extent of entropion. MGL and average LLT at 1 and 5 months after surgery were compared with those of 20 eyes with marginal entropion followed without surgery. RESULTS: In eyes with marginal entropion, MGL was higher (27.7% vs. 12.8%, P = 0.014), and average LLT was thinner (64 nm vs. 86 nm, P = 0.005) than those in control eyes. MGL was higher in eyes with more extensive entropion (> 2/3 eyelid width) than in eyes with less extensive entropion (≤ 1/3 eyelid width) (40.5% vs. 13.2%, P = 0.001). Average LLT increased after surgery (97 nm at 1 month, P = 0.003; 75 nm at 5 months, P = 0.319), and thicker than that of eyes followed without surgery (97 nm vs. 66 nm, P = 0.046). MGLs after surgery remained unchanged from the preoperative MGL (all P > 0.7). CONCLUSION: Marginal entropion is associated with morphological and functional alterations of the meibomian glands. Functional improvement after entropion repair suggests that marginal entropion could cause or exacerbate MGD. Further studies are required to establish the role of entropion repair in managing MGD.

Bony nasolacrimal duct size and outcomes of nasolacrimal silicone intubation for incomplete primary acquired nasolacrimal duct obstruction.

PURPOSE: To investigate the association between the bony nasolacrimal duct (NLD) size and outcomes of nasolacrimal silicone intubation for incomplete primary acquired nasolacrimal duct obstruction (PANDO). METHODS: Patients who underwent silicone intubation for incomplete PANDO and had undergone facial computed tomography (CT) were included. Surgical success was judged by both epiphora improvement and normalized tear meniscus height (TMH; < 300 µm) on anterior segment optical coherence tomography at 3 months after tube removal. The area, major axis diameter, and minor axis diameter of the elliptic bony NLD sections were measured in 1.0 mm-thick axial CT images. These bony NLD sizes were analyzed for associations with surgical success and TMH normalization. RESULTS: Eighty-one eyes of 48 patients were investigated. The smallest area and the smallest minor axis diameter were significantly larger in the success group (49 eyes), compared with those in the failure group (median smallest minor axis diameter: 4.7 mm vs. 3.8 mm, P = 0.008, Mann-Whitney U test). There was also a tendency for the TMH normalization rate to significantly increase as the smallest area and the smallest minor axis diameter increased (P = 0.028 and 0.037, respectively, Fisher's 2 × 4 tests). Under multivariable logistic regression analysis using generalized estimating equation, a larger smallest minor axis diameter was associated with success of the nasolacrimal silicone intubation (odds ratio: 2.481, 95% confidence interval: 1.143-5.384). CONCLUSION: Surgical success of the nasolacrimal silicone intubation in incomplete PANDO is associated with a larger smallest minor axis diameter of the bony NLD. This finding will help understand the pathophysiology of surgical failure after nasolacrimal silicone intubation.

Clinical analysis of ocular adnexal mucosa-associated lymphoid tissue lymphoma with IgG4-related ophthalmic disease.

PURPOSE: To analyse the incidence and characteristics of ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) with immunoglobulin G4 (IgG4)-related ophthalmic disease (IgG4-ROD), and to compare with IgG4-negative OAML. METHODS: We enrolled 130 patients with pathology-confirmed OAML at a single tertiary medical centre. Patients were divided into IgG4-positive and IgG4-negative groups based on the pathologic criteria for IgG4-ROD. The data were reviewed and compared between the groups. RESULTS: IgG4-positive OAML was identified in 5.4% (7/130) of OAML and 13.5% (7/52) of non-conjunctival OAML. IgG4-positive group had a higher incidence of involvement of the lacrimal gland (7/7 vs. 18/123, p = .001), extraocular muscles (3/7 vs. 10/123, p = .022) and infraorbital nerve (2/7 vs. 0/123, p < .001), and had a lower incidence of involvement of the conjunctiva (0/7 vs. 84/123; p < .001) than IgG4-negative group. IgG4-positive group had higher T and N categories of the American Joint Committee on Cancer classification (T1:T2:T3:T4 ratio of 0:6:0:1 vs. 78:32:8:5, p < .001; and ≥N1 ratio of 2/7 vs. 7/123, p = .021). There were no differences in the response rate to initial treatment (5/6 vs. 39/41, p = .343) or the relapse rate (1/6 vs. 5/41, p = 1.000) between the groups. CONCLUSION: IgG4-positive OAML showed clinical features similar to IgG4-ROD, such as predominant disease involvement of the lacrimal gland, extraocular muscles, infraorbital nerve and lymph nodes, but not the conjunctiva. However, treatment outcomes were favourable with or without background IgG4-ROD. Physicians should be aware that IgG4-ROD might undergo a malignant transformation and that thorough treatment and surveillance of IgG4-ROD are important.

Efficacy and Safety of Inhalation Sedation during Office Probing for Congenital Nasolacrimal Duct Obstruction.

We compared the effectiveness of inhaled sevoflurane versus physical restraint during probing in children with congenital nasolacrimal duct obstruction (CNLDO). We performed a retrospective review of children with CNLDO who underwent office probing procedures by a single surgeon under sedation or restraint. Patients' characteristics at the time of probing, including age, sex, laterality, previous non-surgical treatment, presence of dacryocystitis, outcomes of probing, and complications were compared between the sedation and restraint groups. A multivariable logistic regression analysis was performed to investigate the prognostic factors associated with the success of probing. A subgroup analysis by 12 months of age was also conducted. The overall success rate was 88.6% in 202 eyes of 180 consecutive children (mean age, 15.1 ± 7.7 months). The sedation group had a marginally higher success rates than the restraint group (93.8% vs. 85.1%, p = 0.056). The success rate was not significantly different between the two groups in children aged <12 months (90.9% vs. 93.1%, p = 0.739), but it was significantly higher in the sedation group (94.7% vs. 77.8%. p = 0.006) in children aged ≥12 months. Inhalation sedation was the most potent factor associated with success (adjusted odds ratio = 5.56, 95% confidence interval = 1.33-23.13, p = 0.018) in children aged ≥12 months. There were no surgical or sedation-related complications intra- and postoperatively. Inhaled sevoflurane sedation resulted in more successful, controlled, painless probing, particularly in children aged ≥12 months. It represents a safe, efficient alternative to general anesthesia.

The clinical spectrum of periorbital vascular complications after facial injection.

BACKGROUND: Serious complications due to periorbital vascular occlusion can occur after facial injections, including skin necrosis, ophthalmoplegia, blepharoptosis, and visual loss. Visual loss after facial filler injection is particularly rare, but it is known to have a poor prognosis despite treatment. AIMS: This study aimed to describe the prognosis and various clinical features of periorbital vascular complications after facial injection of cosmetic filler or local anesthetic. PATIENTS/METHODS: This single-center retrospective study included 10 consecutive patients who presented with occluded periorbital vessels after facial injection. RESULTS: Nine patients were injected with cosmetic facial fillers: seven with hyaluronic acid, one with collagen, and one with poly-Llactic acid. The other patient was injected with lidocaine mixed with epinephrine. Injection sites included the glabella (n = 5), nasal dorsum (n = 4), and temporal fossa (n = 1). Presumed arteries affected included the central (n = 2) or branch (n = 3) retinal artery, ophthalmic artery (n = 4), and angular artery (n = 1). Nine patients (90%) had purpura and blisters, and eight patients (80%) had ophthalmoplegia at presentation, but all of them recovered within 3 months. Six patients (60%) were blind at the last follow-up, and five of them had occlusion of the central retinal artery or ophthalmic artery. There was a patient with sequelae of phthisis bulbi, which was cosmetically managed with retrobulbar filler injections. CONCLUSION: Facial injections can cause periorbital arterial occlusion, and the clinical features are diverse according to the site and extent of vascular occlusion and injection materials. Visual prognosis was associated with the site of vascular occlusion and initial visual acuity. Other common complications, such as skin lesions, blepharoptosis, and limited extraocular movement, can fully resolve only with supportive treatments in most cases.

Correlation between body composition and blepharoptosis in adults.

BACKGROUND: The present study aimed to explore the association between body composition indices, such as fat mass (FM) and lean body mass (LBM), and blepharoptosis. METHODS: The study evaluated 12,168 Korean adults aged 40 years or older using data from the Korea National Health and Nutrition Examination Survey (2008-2011). FM index (FMI, kg/m2) and LBM index (LBMI, kg/m2) were used to correct for the effects of body size. Multivariable logistic regression analysis was performed to investigate the association between blepharoptosis and body composition indices such as FMI, LBMI, and fat percentage of whole body or head. Stratified analyses were also performed by levator function. RESULTS: Higher FMI and head fat percentage were significantly associated with blepharoptosis (adjusted odds ratio [aOR] = 1.35, 95% confidence interval [CI] = 1.10-1.65; and aOR = 1.32, 95% CI = 1.03-1.69 in the highest tertile compared with the lowest, respectively). In the subgroup with good levator function (≥8 mm), higher FMI, head fat percentage, and head lean mass were significantly associated with blepharoptosis (aOR for the right eye [aORr] = 1.42 and aOR for the left eye [aORl] = 1.36; aORr = 1.41 and aORl = 1.37; and aORr = 1.50 and aORl = 1.49 in the highest tertile compared with the lowest, respectively; all p < 0.05). CONCLUSIONS: Body compositions with high adiposity indices, such as high FMI and head fat percentage, were positively associated with blepharoptosis. Awareness of the potential correlation between obesity-associated fat deposition and blepharoptosis could improve management of the condition and contribute to understanding the pathogenesis of blepharoptosis.

Prognostic factors for relapse and survival among patients with ocular adnexal lymphoma: validation of the eighth edition of the American Joint Committee on Cancer (AJCC) TNM classification.

BACKGROUND/AIMS: To validate the prognostic performance of the American Joint Committee on Cancer (AJCC) eighth edition classification for ocular adnexal lymphoma (OAL). METHODS: We performed a retrospective review of 140 consecutive patients treated for primary OAL between March 2010 and September 2017. Associations between T/N/M categories at presentation and disease-related outcomes, including relapse, progression-free survival (PFS) and overall survival (OS) were evaluated. RESULTS: Seventy-nine women and 61 men (median age, 52 (range 20-84) years; median follow-up, 57 (range 7-131) months) were included. Histological subtypes included mucosa-associated lymphoid tissue lymphoma (92.1%, n=129), diffuse large B-cell lymphoma (5.0%, n=7), follicular lymphoma (1.4%, n=2) and mantle cell lymphoma (1.4%, n=2). Patients with ≥T2 disease had significantly higher risks of overall relapse (unadjusted HR)=4.32, p=0.016), decreased PFS (uHR=5.19, p=0.004) and decreased OS (uHR=9.21, p=0.047). Patients with ≥N1 disease had significantly higher risks of overall relapse (uHR=9.17, p<0.001) and decreased PFS (uHR=9.24, p<0.001). M1 disease was significantly associated with higher risks of overall relapse (uHR=3.62, p=0.036), decreased PFS (uHR=5.13, p=0.001) and decreased OS (uHR=9.24, p=0.013). On considering TNM categories as continuous data, the uHRs for per level increase in T, N and M categories were 1.77, 1.83 and 2.30 for overall relapse and 1.72, 1.87 and 2.78 for decreased PFS, respectively (p<0.05 for each comparison). CONCLUSION: The T, N and M categories of the AJCC eighth edition classification have prognostic value for relapse and survival among patients with primary OAL. Particularly, nodal/metastatic involvement at presentation indicated less favourable outcome.

Reconstruction of Large Upper Eyelid Defects Using the Reverse Hughes Flap Combined With a Sandwich Graft of an Acellular Dermal Matrix.

PURPOSE: To investigate the usefulness of the reverse Hughes flap procedure combined with a sandwich graft of an acellular dermal matrix for reconstruction of large full-thickness defects of upper eyelids after cancer excision. METHODS: Clinical data were obtained from patients who underwent upper eyelid reconstruction using a reverse Hughes flap combined with a sandwich graft of an acellular dermal matrix (AlloDerm) as a tarsal substitute. The tarsoconjunctival flap of the donor lower eyelid was mobilized to reconstruct the posterior lamella, and acellular dermal matrix was grafted onto the tarsoconjunctival flap. A skin-orbicularis muscle flap superior to the defect was advanced to cover the acellular dermal matrix graft, followed by application of lid crease formation sutures to prevent postoperative entropion. The tarsoconjunctival pedicle was divided 3-8 weeks after the surgery. RESULTS: Six patients with sebaceous carcinoma were included, and all had ≥70% full-thickness upper eyelid defects after tumor excision. After a median follow-up of 40 months (range 6-62 months), all 6 showed satisfactory functional and cosmetic outcomes. Tumor recurrence, exposure keratopathy, upper eyelid entropion, persistent use of bandage contact lenses, lower eyelid deformity, and dermal matrix-related complications were not observed by the last follow-up. CONCLUSIONS: Reverse Hughes flap combined with a sandwich graft of an acellular dermal matrix as a tarsal substitute was successful in reconstructing large upper eyelid defects. Acellular dermal matrix graft and lid crease formation sutures enhance marginal stability and prevent postoperative entropion of the reconstructed upper eyelid.

Alveolar soft part sarcoma of the lateral rectus muscle: Suture technique to prevent postoperative strabismus.

PURPOSE: We present a rare case of intramuscular alveolar soft part sarcoma (ASPS) of the lateral rectus (LR) muscle and the surgical technique used to maintain orthotropia after complete resection of the mass. OBSERVATIONS: A 5-year-old boy presented with progressive proptosis of the left eye due to an orbital tumor. The patient was previously diagnosed with ASPS of the left LR muscle from an incisional biopsy, and the tumor size increased despite 5 cycles of chemotherapy prior to presenting to our center. Magnetic resonance imaging showed a 28x19x15mm-sized contrast-enhancing intramuscular mass of the left LR muscle, and there was no evidence of nodal or distant metastasis. The mass was excised en bloc, along with the insertion and the posterior normal part of LR muscle. To maintain proper eye alignment after resecting LR muscle, a 4-0 Prolene® hang-back suture was placed between the scleral insertion and the periorbita of the posterior orbit and the left medial rectus muscle was injected with botulinum toxin. During the follow-up of 51 months after surgery, the patient had no evidence of recurrence or metastasis and remained orthotropic in primary gaze, with a good cosmetic result. CONCLUSIONS AND IMPORTANCE: ASPS of extraocular muscles is a rare tumor occurring mainly in children and young adults, and treatment may cause significant sequelae such as orbital exenteration, radiation-induced complications, and large-angle strabismus. Complete resection of tumor including the extraocular muscle is essential for treatment, and a subsequent reconstruction using a hang-back suture technique is useful to achieve proper eye alignment as well as a good cosmetic outcome.

Association of T and N Categories of the American Joint Commission on Cancer, 8th Edition, With Metastasis and Survival in Patients With Orbital Sarcoma.

Importance: No previous studies to date have validated the American Joint Committee on Cancer (AJCC) 8th edition of the TNM classification for orbital sarcoma. Objectives: To determine the prognostic performance of the most recent TNM classification for orbital sarcoma and to identify other prognostic factors for local recurrence, lymph node metastasis, distant metastasis, and death due to disease. Design, Setting, and Participants: This single-center retrospective cohort study included 73 consecutive patients treated for orbital sarcoma from March 1, 2003, through June 30, 2018. Data were analyzed from November 1 to December 31, 2018. Main Outcomes and Measures: T and N categories at presentation and disease-related outcomes, including local recurrence, lymph node metastasis, distant metastasis (DM), and death due to disease (DD). Results: The 73 participants included 43 men (59%), and the median age was 21 (range, 0-77) years. The common histologic types were rhabdomyosarcoma (RMS) (35 [48%]), solitary fibrous tumor/hemangiopericytoma (10 [14%]), and Ewing sarcoma (8 [11%]). The most common TNM designations were T2 N0 M0 (26 [36%]) and T4 N0 M0 (24 [33%]). T category was associated with the risk of all disease-related outcomes, including local recurrence (hazard ratio [HR] for T2 vs T4, 0.22 [95% CI, 0.06-0.81]; HR for T3 vs T4, 0.59 [95% CI, 0.13-2.65]; P = .03), lymph node metastasis by the last follow-up (T1, 1 [14%]; T2, 0; T3, 0; T4, 12 [35%]; P = .001), DM (HR for T2 vs T4, 0.29 [95% CI, 0.08-1.07]; P = .04), and DD (HR of T2 vs T4, 0.16 [95% CI, 0.04-0.73]; HR of T3 vs T4, 0.30 [95% CI, 0.04-2.34]; P = .02). Higher risk of DM and higher risk of DD were associated with disease category of at least T3 (HR for DM, 3.24 [95% CI, 0.89-11.72; P = .06]; HR for DD, 6.32 [95% CI, 1.43-27.95; P = .005]), N1 disease (HR for DM, 13.33 [95% CI, 4.07-43.65; P < .001]; HR for DD, 7.07 [95% CI, 2.45-20.44; P < .001]), tumor size larger than 3 cm (HR for DM, 2.72 [95% CI, 0.92-8.05; P = .06]; HR for DD, 5.79 [95% CI, 1.85-18.14; P < .001]), and age of patient with RMS younger than 1 year or 10 years or older (HR for DM, 6.85 [95% CI, 0.83-56.53; P = .04]; HR for DD, 7.03 [95% CI, 0.85-57.83; P = .04]). Higher risk of local recurrence was associated with disease category of at least T3 (HR for3 cm, 0.27 [95% CI, 0.09-0.77]; P = .009). Higher risk of lymph node metastasis was associated with disease category of at least T3 (odds ratio [OR], 13.33 [95% CI, 1.77-602.30]; P = .004), alveolar RMS (OR, 9.98 [95% CI, 2.13-51.55]; P = .001), and age of patient with RMS younger than 1 year or 10 years or older (OR, 9.20 [95% CI, 1.01-458.29] P = .03). Conclusions and Relevance: In patients with orbital sarcoma, T and N categories at presentation (defined by the AJCC 8th edition classification) correlate with metastasis and survival. These findings appear to support consideration of strict surveillance testing for regional nodal and systemic metastases in patients with orbital sarcoma with disease category of at least T3 and/or N1 disease.

Clinicopathologic features of biopsied lacrimal gland masses in 95 Korean patients.

PURPOSE: To investigate the clinicopathologic features of lacrimal gland masses biopsied in a tertiary referral hospital in Korea. METHODS: Records from 95 Korean patients who underwent lacrimal gland mass biopsy were retrospectively reviewed. Data included demographics, clinical presentation, imaging findings, histopathologic diagnosis, and associated systemic disease. RESULTS: The median age was 52.0 years (range, 16-76 years), and 51 patients (53.7%) were female. Thirty-three patients (34.7%) had bilateral disease. The histopathologic diagnoses were as follows: chronic dacryoadenitis (52.6%, n = 50;29 non-specific and 21 immunoglobulin G4-related disease (IgG4-RD)), lymphoproliferative disease (25.5%, n = 24; 18 lymphoma and six lymphoid hyperplasia), benign epithelial tumour (13.7%, 13 pleomorphic adenoma), malignant epithelial tumour (3.2%, three adenoid cystic carcinoma), dacryops (3.2%, n = 3), solitary fibrous tumour (1.1%, n = 1), and xanthogranulomatous inflammation (1.1%, n = 1). Patients with chronic dacryoadenitis were significantly more likely to be younger (mean 47.5 years), have bilateral involvement (52.0%), and have a longer symptom period (mean 15.6 months) than those with lymphoproliferative disease (60.0 years, 25.0%, and 6.7 months, respectively; p < 0.05, each comparison). Patients with IgG4-related dacryoadenitis were significantly more likely to have bilateral involvement (85.7%) and have associated systemic involvement (52.4%) than those with non-specific dacryoadenitis (37.9 and 0%, respectively; p < 0.05, each comparison). Sixteen patients (16.8%) had associated systemic involvement: 11 with IgG4-RD and 5 with lymphoma. CONCLUSIONS: Chronic dacryoadenitis and lymphoproliferative disease were the most common causes of lacrimal gland masses in our cohort. Younger patients with bilateral involvement and a longer symptom period were more likely to have chronic dacryoadenitis than lymphoproliferative disease. Associated systemic involvement was not rare in patients with IgG4-RD or lymphoma. Our results suggest that biopsy of chronic lacrimal gland masses should be performed for proper evaluation and management.

Validation Study of the AJCC Cancer Staging Manual, Eighth Edition, Staging System for Eyelid and Periocular Squamous Cell Carcinoma.

Importance: To our knowledge, there are no validation studies to date of the prognostic value of the AJCC Cancer Staging Manual, eighth edition (AJCC 8), criteria for eyelid and periocular squamous cell carcinoma. Objective: To determine the association of tumor (T) category in AJCC 8 with local recurrence, nodal metastasis, distant metastasis, and disease-specific survival (DSS) for eyelid and periocular squamous cell carcinoma. Design, Setting, and Participants: In this retrospective, single-center cohort study, 109 consecutive patients with eyelid and periocular squamous cell carcinoma treated from January 1999 to April 2018 were included. Patients with secondary involvement of the periocular region were excluded. Main Outcomes and Measures: Local recurrence, nodal metastasis, distance metastasis, and DSS. Results: Of the 109 included patients, 81 (74.3%) were male, and the median (range) age was 66 (40-91) years. At presentation, 43 patients (39.4%) had recurrent tumor, 4 (3.7%) had nodal metastasis, and 1 (0.9%) had distant metastasis. The median (range) follow-up was 23 (1-161) months. During follow-up, 11 patients (10.1%) developed local recurrence, 7 (6.4%) developed nodal metastasis, 2 (1.8%) developed distant metastasis, and 9 (8.3%) died of disease. The 5-year DSS rate was 87.7% (95% CI, 79.5-96.9). Chronic immunosuppression (hazard ratio, 47.24; 95% CI, 7.33-304.30; P < .001) and presentation with recurrent squamous cell carcinoma (hazard ratio, 5.22; 95% CI, 1.12-24.31; P = .04) were associated with local recurrence during follow-up. Of the 11 patients with local recurrence during follow-up, 7 (64%) had perineural invasion. T category was associated with nodal metastasis; clinical stage of T2c or worse at presentation was associated with higher risk of nodal metastasis and death of disease but not with a higher risk of local recurrence. Distant metastasis was associated with nodal metastasis at presentation (hazard ratio, 32.50; 95% CI, 1.97-536.40; P = .02) and during follow-up. A total of 33 patients (30.3%) had different T categories depending on whether disease was staged according to the seventh or eighth edition of the AJCC Cancer Staging Manual. Compared with AJCC 7, AJCC 8 showed a better predictive value in terms of local recurrence (T3, 17% vs 14%; T4, 11% vs 16%) and DSS. Conclusions and Relevance: These findings suggest that T category in AJCC 8 is associated with nodal metastasis and DSS. Immunosuppression and presentation with recurrent disease are associated with increased risk of future local recurrence. Patients with tumors of clinical stage T2c or worse at presentation are at increased risk of nodal metastasis and worse DSS and should undergo surveillance for nodal metastasis. Future studies, ideally prospective in design, could provide greater confidence in these findings.