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Purpose: The aim was to evaluate patient profiles of rhino-orbital-cerebral mucormycosis (ROCM) cases with central retinal artery occlusion (CRAO) postcoronavirus disease 2019., Design: A nonrandomized retrospective case-control study., Methods: The ROCM cases presenting with CRAO were compared with a control ROCM group without CRAO at a tertiary care center. Demography, systemic status, clinical features, histopathology, imaging, and blood profile were assessed for any specific risk factors., Results: A total of 12 patients were seen in the CRAO group and 16 in the non-CRAO group. The male-to-female ratio was 3:1 with a mean age of 49.5 years. In the CRAO group, 75% had diabetes mellitus with mean hemoglobin A1c of 9.03%, and 66.7% had received steroid treatment. All cases were histopathologically confirmed positive for mucor. There was a significant difference in mean D-dimer and serum ferritin between the 2 groups, with higher level in the CRAO group. All patients with CRAO had light perception-negative vision, with total ophthalmoplegia and proptosis seen in 66.7% of cases. Four patients had orbital apex involvement, 5 had cavernous sinus involvement, and 8 had intracranial involvement in the CRAO group., Conclusions: Inflammatory markers D-dimer and serum ferritin were significantly associated with CRAO, suggestive of hyperinflammatory and hypercoagulable state. A high index of suspicion should be maintained in cases with elevated markers and prophylactic anticoagulants can be started to prevent CRAO in a subset of patients., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 Asia-Pacific Academy of Ophthalmology. Published by Wolters Kluwer Health, Inc. on behalf of the Asia-Pacific Academy of Ophthalmology.)

Background: Idiopathic orbital inflammation (IOI) is a disfiguring and vision-threatening fibroinflammatory disorder. The pathogenesis of IOI has not been elucidated. We sought to clarify the regulatory T cell (Treg) distribution and function in patients with IOI., Methods: The frequency, phenotype and function of Tregs were identified by multicolor flow cytometry and in vitro cell functional assays. Plasma and tissue samples were obtained to investigate cytokines, chemokines and their receptors of interest by relative real-time polymerase chain reaction (PCR) and Luminex assays., Results: Compared with healthy subjects, patients with IOI exhibited obvious increases of Tregs in peripheral blood and affected orbital tissues. Circulating Tregs from patients with IOI were significantly more polarized to a Th17-like phenotype with defective regulatory function, whereas orbit-derived Tregs were polarized to a Th2-like phenotype. Furthermore, ST2 expression levels in circulating Tregs and interleukin (IL)-33 mRNA levels in orbital tissues were decreased in IOI. IL-33 restored the suppressive function of Tregs, reduced interferon (IFN)-γ production by Tregs and decreased the activation of orbital fibroblasts (OFs) cocultured with Tregs in IOI., Conclusion: Increased Tregs with proinflammatory and profibrotic polarization were first identified in IOI, suggesting that Treg plasticity and heterogeneity plays an essential role in IOI pathogenesis. Additionally, our study identified a regulatory effect of IL-33 on inflammation and fibrosis in IOI. Reversing the plastic Tregs via IL-33 might be a potential option for IOI patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Chen, Ye, Xiao, Mao, Ai, Chen, Lian, Shi, Wang, Bi, Yang, Ji, Zhang and Yang.)

Purpose: To investigate the factors associated with response to steroid treatment and recurrence in patients with IgG4-related ophthalmic disease (ROD)., Methods: Twenty-eight patients with biopsy-proven IgG4-ROD treated between March 2010 and January 2017 were included in this retrospective study. Clinical features, serum IgG4 levels, systemic involvement, treatments and treatment outcome, factors associated with response to treatment and recurrence were assessed., Result: Thirteen men and 15 women (mean age 50.8 years) were evaluated over mean follow-up period of 27.3 months. Elevated serum IgG4 levels (>1.35 g/L) and systemic disease were noted in 9 (32%) and 18 patients (64%), respectively. The lacrimal gland was involved in all patients, and 22 patients (78.6%) had bilateral involvement. Most patients (82%) responded well to systemic steroids, but 12 (43%) relapsed after the initial steroid treatment, requiring additional therapies to achieve remission. Complete response to initial steroid treatment was associated with elevated serum IgG4 levels before treatment (P=0.001) and bilateral orbital involvement (P=0.050). Recurrence was associated with elevated serum IgG4 levels before treatment (P=0.007), lower dose (P=0.057) and shorter duration of initial steroids (P=0.042). Patients with recurrence eventually required significantly more steroids than those without recurrence (P=0.011)., Conclusions: Patients with IgG4-ROD responded well to systemic steroid treatment, but recurrence was common, particularly among those with elevated serum IgG4 levels and shorter duration of initial steroid treatment. Low-dose maintenance treatment with systemic steroids should be considered to avoid recurrence in patients with elevated serum IgG4 levels., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Granulomatosis with polyangiitis is rare in children. We report a case of a 12-year-old male who presented with new symptoms of left eyelid swelling and ptosis. Magnetic resonance imaging showed an enhancing orbital mass suspicious for a neoplasm. Excisional biopsy was performed. Microscopic examination revealed fibrovascular tissue with dense collagenous fibrosis and mixed inflammatory infiltrate that included many plasma cells. Many small and medium-sized blood vessels showed granulomatous and necrotizing vasculitis with disruption of the vessel walls and fibrinoid necrosis. Immunostain for IgG highlighted the numerous plasma cells, approximately 50% of which were positive for IgG4 immunostain. A diagnosis of granulomatosis with polyangiitis was suggested, with recommendation of serologic testing for anti-neutrophil cytoplasmic antibodies. Serum anti-neutrophil cytoplasmic antibodies were borderline high with a cytoplasmic staining pattern. The patient improved with steroid and methotrexate therapy. Granulomatosis with polyangiitis can present as an orbital mass in up to 30% of children. It may be misdiagnosed as IgG4-related disease since the inflammatory background in both conditions may be rich in plasma cells with a high proportion of IgG4+ plasma cells, and accompanied by fibrosis and obliterated blood vessels. The differential diagnosis in this location should also include inflammatory pseudotumor and inflammatory myofibroblastic tumor. Knowledge of this unusual manifestation of granulomatosis with polyangiitis and its diagnostic pitfalls can facilitate early diagnosis and treatment.

Objectives: To measure the frequency of infraorbital nerve enlargement (IONE) on magnetic resonance imaging (MRI) in European patients suffering from an IgG4-related ophthalmic disease (IgG4-ROD) as compared to patients suffering from non-IgG4-related ophthalmic disease (non-IgG4-ROD)., Methods: From January 2006 through April 2015, 132 patients were admitted for non-lymphoma, non-thyroid-related orbital inflammation. Thirty-eight had both pre-therapeutic orbital MRI and histopathological IgG4 immunostaining. Fifteen patients were classified as cases of IgG4-ROD and 23 patients as cases of non-IgG4-ROD. Two readers performed blinded analyses of MRI images. The main criterion was the presence of an IONE, defined as the infraorbital nerve diameter being greater than the optic nerve diameter in the coronal section., Results: IONE was present in 53% (8/15) of IgG4-ROD cases whereas it was never present (0/23) in cases of non-IgG4-ROD (P < 0.0001). IONE was only present in cases where, on MRI, the inflammation of the inferior quadrant was present and in direct contact with the ION canal., Conclusions: In European patients suffering from orbital inflammation, the presence of IONE on an MRI is a specific sign of IgG4-ROD. Recognition of this pattern may facilitate the accurate diagnosis for clinicians and allow for the adequate management and appropriate care of their patients., Key Points: • IONE on an MRI is a specific sign of IgG4-ROD. • IONE recognition allows for a quicker diagnosis and appropriate management. • IONE appears when inflammation is in direct contact with the ION canal.

Aims: The present study aimed to investigate the clinical implications of serum IgG 4 levels in patients with IgG 4 -related ophthalmic disease (ROD)., Methods: The medical records of 31 patients who met the diagnostic criteria for IgG 4 -ROD were retrospectively reviewed. Twenty-five patients whose serum IgG 4 levels could be identified were included. Clinical manifestations and serum IgG 4 levels before and after corticosteroid treatment were obtained. Factors associated with relapse were evaluated by comparing the features of patients with disease relapse with those of patients without relapse., Results: Twenty-four patients were 'definite' and one was 'probable' for IgG 4 -ROD according to the diagnostic criteria. Serum IgG 4 levels were higher in patients with systemic involvement (p=0.046). All patients improved clinically after corticosteroid treatment. Serum IgG 4 levels decreased after steroid treatment (p=0.005) and normalised in nine patients. In cases of relapse, serum IgG 4 levels increased along with the aggravation of symptoms (p=0.047). Serum IgG 4 levels that were still elevated (≥135 mg/dL) after steroid treatment (p=0.034) and cessation of steroid treatment during disease remission (p=0.043) were predictive factors for IgG 4 -ROD relapse., Conclusions: Serum IgG 4 level can be considered an adjunctive marker for treatment response in IgG 4 -ROD. Patients with serum IgG 4 levels that remain elevated after steroid treatment should be carefully observed for relapse. A continuing maintenance dose of oral steroid is recommended to prevent relapse, even when clinical remission is achieved., Competing Interests: Competing interests: None declared., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Thanks to detailed studies conducted in recent years, a new disease syndrome was identified in 2001. It is known as a IgG4-related disease and its differentiation is based on the analysis of IgG4 levels in the affected tissues. The IgG4-related disease is considered to be a generalized pathological process involving a wide spectrum of various disorders that may affect distant organs. Orbital IgG4-related disease is a recently reported issue that may prove important for the elucidation of the etiology of idiopathic, lymphoplasmacytic or fibrotic disorders of various organs, including the orbits. In this article, we are describing epidemiology and differential diagnostics of IgG4-related orbital disease with particular focus on pseudotumors, MALT lymphomas and lymphocyte/plasma cell infiltrations of the orbit. We are also discussing therapeutic possibilities currently available in the management of the disease., (Copyright © 2015 Institute of Pharmacology, Polish Academy of Sciences. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.)

Objective: To study the clinicopathologic features of IgG4-related ocular disease and differentiated diagnosis with non-IgG4-related ocular disorders., Methods: Forty-three cases were selected from the Department of Pathology, West China Hospital, Sichuan University from December 2008 to January 2015 with retrospective series case study. The clinical manifestation, laboratory examination, imaging findings, treatment and histopathological characteristics were analyzed. According to the diagnostic criteria for IgG4-related ocular disease in 2014 and relevant literatures, our cases were divided into two groups: IgG4-related ocular disease (23 samples from 18patients) and non-IgG4-related ocular disease (31 samples from 25 patients). The male-to-female ratio, the periductal fibrosis and the orbital lesion area was analyzed by Pearson chi-square. The Immunohistochemistry of IgG4+/HPF, IgG+/HPF and IgG4+/IgG+ ratio and age was analyzed by two sample t test. The involved structural tissue and the infiltration of eosinophils and lymphocytes and plasma cells was analyzed by signed-rank test., Results: The significant statistic differences were observed between these two groups in immunohistochemical staining of IgG4 (>40/HPF) (t=12.17, P<0.01), IgG (t=2.91, P<0.01) and IgG4+/IgG+ ratio (≥40%) (t=16.98, P<0.01), the lymphocytic and plasmacytic infiltration (F=3.62, P=0.03),the periductal fibrosis(F=4.71 , P=0.03), the involved region of orbit (t=1.11, P=0.01), but not in the mean age and male-to-female ratio (t=1.09, P=0.58). IgG4 ROD should be distinguished form the non-specific inflammation disorders, lymphoma and so on., Conclusions: IgG4-related ocular disease often arises from middle-old man, characterized by bilateral orbital painless swelling and abundant IgG4+ plasma cells infiltration. Nearly all orbital tissue could be involved by IgG4-related ocular disease, especially lacrimal gland. But the characteristic obliterating phlebitis were not seen. The immunohistochemical staining of IgG4 and IgG plays an important role in differentiating IgG4-related ocular disease from non-IgG4-related ocular disorders. Combining clinical manifestation, laboratory examination, imaging findings with histopathology is helpful for diagnosis and differential diagnosis. (Chin J Ophthalmol, 2016, 52: 256-262).

Introduction: Orbital inflammatory syndromes include a wide variety of inflammatory intraorbital processes which are very different in terms of clinical presentation and prognosis. We currently prefer to differentiate so-called "specific" inflammations, for which an etiology is able to be identified, from idiopathic orbital inflammatory syndromes (IOIS), for which the etiology remains unknown and the histology is nonspecific., Purpose: To propose an efficient diagnostic approach for clinicians managing patients with non-Graves' orbital inflammations., Materials and Methods: This is a retrospective and prospective study concerning 61 patients managed by the medical team for non-Graves' orbital inflammations between May, 1999 and May, 2013 in the ophthalmology departments of Nice and Limoges university hospitals in France. Seventeen specific inflammations, 19 orbital lymphomas and 25 idiopathic orbital inflammatory syndromes were included. Patients were divided into two groups. Thirty-six patients (group 1) underwent primary biopsy, while for the other 25 (group 2), therapy was begun empirically without biopsy. We could therefore compare both approaches in terms of diagnostic efficiency and time until identification of a specific etiology., Results: Our statistical results show that an approach without primary biopsy leads to a number of specific diagnoses statistically much lower than that obtained by the approach with primary biopsy. Also, the risk of missing a specific inflammation (with as a consequence an inappropriate treatment and a risk of functional sequelae as well as a fatal risk of missing a lymphoproliferative pathology) is very clearly higher in the case of not performing primary biopsy. Finally, the average time elapsed between the initial consultation with the ophthalmologist and a specific diagnosis was one month in the case of the first approach, while this delay was almost three times higher with the second approach, with a mean of 2.91 months (P<0.01)., Discussion: Our study shows that biopsy should be the mainstay of diagnostic management. A trial of empiric treatment is only performed first in myositis or in locations where biopsy could jeopardize functional prognosis. It should only be done after biopsy in all other cases. Of course, in all cases of relapse or recurrence after treatment, biopsy should be performed or repeated., Conclusion: The diagnostic work-up of a patient with an orbital inflammatory process must of course include blood testing and orbital imaging, but also a systematic primary biopsy for histological examination in the vast majority of cases. It must be repeated at least in the case of any doubt about the diagnosis or in the case of any recurrence or resistance to treatment., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Objective: To determine the changes in IgE levels in diagnosis and postoperative monitoring of orbital fungal granuloma., Study Design: Descriptive analytical study., Place and Duration of Study: Al-Shifa Trust Eye Hospital, Rawalpindi, Pakistan, from July 2012 to June 2013., Methodology: Cases with clinically high index of suspicion for orbital fungal granuloma and 50 healthy volunteers were inducted as control subjects. Patients with recurrent cases of orbital fungal granuloma, previous orbital surgery, and those with very low clinical suspicion of fungal granuloma were excluded. Total serum IgE level (IU/ml), eosinophil counts and skin prick test were performed in all subjects. Independent t-test was used for comparison of healthy volunteers and patients with biopsy proven orbital fungal granuloma. Repeated measures ANOVA was used for comparing the preoperative and postoperative total serum IgE level of patients with biopsy proven fungal granuloma., Results: The mean total serum IgE level for the healthy volunteers was 208.82 ±41.43 IU/ml. The mean pre-operative IgE value of histologically confirmed cases of fungal granuloma was 1613.72 ±282.83 IU/ml. The total serum IgE level gradually declined after surgery and anti-fungal treatment. The mean serum IgE level 3, 6 and 9 months postoperatively were 1039.48 ±308.40, 568.77 ±162.01 and 224.92 ±51.55 IU/ml respectively. These tests showed that the drop in IgE level in cases of fungal granuloma with treatment was statistically significant (p < 0.001)., Conclusion: Total serum IgE level can be used as a reliable diagnostic and postoperative monitoring tool in orbital fungal granuloma.

Objective: To determine whether thyroid-stimulating immunoglobulin (TSI) testing can predict the risk of development of Graves orbitopathy in newly diagnosed Graves thyroidopathy patients., Design: Retrospective cohort, from 2008 to 2013., Setting: The Thyroid Referral Center at California Pacific Medical Center., Participants: A retrospective cohort of newly diagnosed Graves thyroidopathy patients from the California Pacific Medical Center Thyroid Referral Center. Patients were included if they had TSIs drawn at or near the time of diagnosis of Graves thyroidopathy. Patients were excluded from the study if they had a long-standing diagnosis of Graves thyroidopathy, orbitopathy at time of diagnosis, no TSIs drawn, or follow up of less than 6 months., Main Outcome Measures: Patients were followed for the development of orbitopathy as determined by their endocrinologists. Results were adjusted for family history, smoking status, age, radioiodine ablation treatment, and race., Results: Thirty-three patients met inclusion criteria out of a screened population of 506 patients. Eight out of 33 patients (24%) developed orbitopathy. The mean time from diagnosis of Graves' thyroidopathy to development of orbitopathy was 11.6 months (median: 7.5 months, range: 1 to 20 months). The mean initial TSI value was 421.3 in those that developed orbitopathy compared to 245.9 in those who had at least 6 months of documented follow-up and did not develop orbitopathy (p = 0.04). Those in the top tercile of initial TSI values were 14 times as likely to develop orbitopathy (relative risk (RR) = 14.0, p = 0.02; multivariate adjusted RR = 13.08, p = 0.03). Family history, smoking status, age, radioiodine ablation, thyroid-stimulating hormone, and race were not statistically significant predictors., Conclusions: TSI level greater than 400 at time of presentation of Graves thyroidopathy may be a useful predictor of risk for development of orbitopathy. This information will help to identify patients likely to benefit from early referral to an ophthalmologist for possible preemptive therapy to prevent the development of orbitopathy. Prospective cohort studies are needed to definitively establish the metrics for TSI as a predictor of orbitopathy.

Purpose: To determine the relation of orbital xanthogranuloma with IgG4-related disease., Methods: Retrospective consecutive case series over a period of 25 years. We searched our charts for histologically confirmed orbital xanthogranuloma. Patient files were reviewed for clinical and follow up data including presence or absence of systemic non-ophthalmic manifestations of IgG4 related disease. Slides were re-examined and histopathological classification was re-assessed. Sixteen cases of orbital xanthogranuloma were evaluated. Immunohistochemical stains for IgG and IgG4 were performed. Positive immunohistochemical staining required increased IgG4-positive plasma cells in the involved tissues scored as >50 per high-power field, with IgG4/IgG ratio >0.40., Results: According to the criteria described above 8/16 (50%) cases showed increased numbers of IgG4-positive plasma cells in the specimens. Two of these patients may have had signs of systemic disease., Conclusion: Raised numbers of IgG4-positive plasma cells are a common finding in histopathological specimens of xanthogranulomatous disease of the orbit and are often not indicative for IgG4 related systemic disease.

A 46-year-old male was referred to the Ophthalmology Service for a 7-year history of bilateral proptosis and a presumptive diagnosis of thyroid eye disease. Past medical history was only significant for autoimmune pancreatitis. All laboratory testing including tests of thyroid function were within normal limits. The patient underwent orbital biopsy and was found to have plasma cells containing mainly IgG4 immunoglobulin that was consistent with IgG4-related disease. The patient was treated with oral prednisone and the proptosis resolved within 3 weeks.

Purpose: To describe the clinical and radiologic features of orbital involvement in craniofacial brown tumors and to compare the rate of brown tumors in primary and secondary hyperparathyroidism., Methods: A retrospective hospital-based study of 115 patients with chronic kidney disease and secondary hyperparathyroidism and 34 with primary hyperparathyroidism was conducted. Laboratory results such as serum levels of alkaline phosphatase, calcium, phosphorus, and parathyroid hormone were recorded. Demographic data (age, sex, duration of disease) and image findings (bone scan scintigraphy, skull and long bone x-rays, CT) were also obtained. The main outcome measures were analysis of clinical, biochemical, and radiologic findings of all patients., Results: Of the 115 patients with chronic kidney disease, 10 (8.7%) had brown tumors in different bones of the skeleton. Five patients had lesions in the craniofacial bones. The maxilla, mandible, maxillary sinus, and nasal cavity were the most affected sites. The orbit was involved in 2 patients with lesions arising in the maxillary and ethmoid sinuses. One patient had facial leontiasis. All patients with brown tumors had extremely high levels of parathyroid hormone (>1,000 pg/ml, normal values 10-69 pg/ml) and alkaline phosphatase (>400 U/l, normal values 65-300 U/l). The mean serum levels of phosphorus and calcium were not abnormal among the patients with brown tumors. Age and time of renal failure were similar for patients with and without brown tumors. Among the patients with primary hyperparathyroidism, only 2 (5.8%) had brown tumors, and in just 1, the lesion was localized in the craniofacial skeleton. A 2-tailed Z test applied to compare the proportion of occurrence of brown tumors in the 2 groups revealed that the difference at the 90% of confidence level was not significant., Conclusions: Brown tumors are equally found in secondary and primary hyperparathyroidism. Craniofacial brown tumors involve the orbit, usually because of the osteodystrophy process that involves the maxilla and paranasal sinuses. The lesions do not necessarily need to be excised and may regress spontaneously after the control of hyperparathyroidism.

Background: We describe an episode of thyroid-associated orbitopathy (TAO) following sequential bilateral cataract surgery., Case: We report the case of an 80-year-old woman who developed TAO in 2000., Observations: After an episode of mild TAO, the patient's condition was stable and quiescent for 4 years. Following cataract surgery on her right eye in 2004, there was rapid worsening of the TAO bilaterally that lasted approximately 12 months. Her condition then stabilized for 2 years. In 2007, she had cataract surgery on the left eye and the TAO again worsened bilaterally., Conclusion: Cataract surgery may lead to recrudescence of TAO in predisposed individuals.

Unlabelled: Radioiodine (131I) treatment of patients with Graves-Basedow disease may cause or aggravate the course of orbitopathy (GO) in some 15% of patients; while only 3% of patients treated with methimazole develop GO. The aim of this study was to evaluate the frequency of GO progression in patients with mild GO treated with 131I, compared to a control group., Materials and Methods: The studied group consisted of 21 hyperthyroid patients (mean age 49 +/- 12.8 years) with mild orbitopathy (CAS < 3 pts, NOSPECS < 4 pts). The control group included 18 hyperthyroid patients with Graves-Basedow disease (mean age 50 +/- 9.9 years) with no GO symptoms (CAS = 0 pts, NOSPECS < 1 pts). All patients were treated with 131I. Patients with GO underwent treatment with oral methyloprednisolone (MP) over 30 days in decreasing doses, commencing with a dose of 16 mg/day. TSH, FT4 and hTRAb serum concentrations were measured prior to, and 14, 30, 60 days and 12 months after administration of 131I, always accompanied by an ophthalmic evaluation., Results: In the studied group, mean TSH and FT4 concentrations prior to treatment were 0.05 +/- 0.08 microU/ml and 23.7 +/- 10.7 pmol/l, respectively. Mean 131I activity applied in this group was 605.0 +/- 89.0 MBq. No significant differences were stated between values of respective parameters in the studied and control groups. Prior to treatment, median hTRAb concentrations in the studied and control groups were 6.8 U/l (max 53.8, min 0.1) and 8.9 U/l (max 57.1, min 4.2), respectively, and did not differ significantly. After 14 days post commencing MP treatment the median hTRAb concentration in the studied group decreased (4.5 U/l, max 51.1, min 0.1) with respect to the control group (7.5 U/l, max 50.0, min 2.9). After 60 days and 12 months, median hTRAb concentrations in the studied group were 8.3 U/l (max 16.9, min 0.7) and 8.5 U/l (max 9.8, min 3.0) respectively, being higher than those in the control group and also higher than the initial value in studied group. Cured were 16/21 patients in the studied group and 16/18 patients in the control group. Within 12 months observation, progression of GO symptoms in 2 patients (9%) of the studied group was noted and exophthalmos observed in 3 patients (17%) of the control group., Conclusions: In patients with mild GO treated with methyloprednisolone 131I administration is effective and does not lead to aggravation of GO symptoms, compared with the control group. Long-term elevation of hTRAb concentration in studied and control group of patients with Graves' disease treated with 131I1 was found.

Background: Controversy exists about the optimal management of subperiosteal orbital abscesses (SPOAs) in pediatric patients. Some otolaryngologists advocate immediate surgical drainage while others recommend medical management initially and reserve surgery for nonresponders. We hypothesized that patients who can be managed without surgery have identifiable features on presentation that may aid in predicting their response to medical therapy., Methods: A retrospective chart review was performed on all patients < or =18 years of age who were admitted to the University of Iowa between 1984 and 2001 with findings consistent with an SPOA on computed tomography imaging. Patients were divided into two groups: group I received medical treatment only while group II underwent surgical drainage of the abscess. Presenting features were compared between the two groups., Results: Forty-two patients were identified with 17 group I patients and 25 group II patients. All patients had resolution of their SPOA and favorable outcomes. The following variables attained significance: group I patients were younger than group II patients (5.1 years versus 11 years; p < 0.0001), had less restriction of ocular motility (-1.0 versus -2.3), and were hospitalized for fewer days (6.5 days versus 9.6 days; p = 0.011). The following clinical variables did not vary significantly between the groups: gender, side of abscess, temperature, total white blood cell count, neutrophil count, chemosis, visual acuity, and proptosis. Culture results predominantly showed growth of anaerobic organisms (7/23). With increasing age, there was an increase in the number of organisms cultured (p = 0.005)., Conclusion: A subset of patients with SPOAs can be managed medically. These patients tend to be younger and present with minimal restriction of ocular motility.

Purpose: To describe a case of orbital Wegener granulomatosis without systemic disease., Method: Case report., Results: A 69-year-old patient with bilateral inflammatory lacrimal gland masses underwent multiple biopsies that showed a nonspecific lymphoplasmacytic infiltrate consistent with orbital pseudotumor. After unsuccessful treatment with systemic corticosteroids and radiation, severe orbital disease rapidly progressed and the patient underwent unilateral enucleation. The enucleated specimen showed multifocal vasculitis, tissue necrosis, and granulomas consistent with Wegener granulomatosis (WG). Elevated antineutrophil cytoplasmic antibody titers supported the diagnosis of WG. The patient did not have any extraocular signs of WG and continues to be disease-free systemically., Conclusion: The authors believe this is the first report of bilateral lacrimal gland masses presenting as a localized form of WG in the total absence of systemic disease.

Objective: To measure the frequency with which lumbar puncture (LP) was performed in children with periorbital and orbital cellulitis in the years 1979 through 1991, and to examine its value as a diagnostic test in these patients., Design: Retrospective review of both computerized discharge diagnoses and medical records., Setting: Tertiary care hospital., Patients: Two hundred fourteen children younger than 18 years of age discharged between Jan. 1, 1979, and Dec. 31, 1991, with a final diagnosis of periorbital or orbital cellulitis., Main Outcome Measures: Rates of LP and of bacterial meningitis., Results: Of the 214 patients, 96 (45%) had LPs. Two children (1%) had meningitis; in both, the diagnosis was strongly suspected before the LP was performed. No other subject had a discharge diagnosis of meningitis. During the 13 years of study, the proportion of patients in whom LP was performed increased from 14% in the first 6 years (1979 through 1984) to 62% from 1985 through 1991 (p < 0.0001). Those older than 2 months who were afebrile on arrival in the emergency department were 12.4 times more likely to have an LP in 1985 through 1991 than in 1979 through 1984 (p = 0.005)., Conclusions: The increase in the number of LPs performed from 1985 through 1991 in children with periorbital or orbital cellulitis was not accompanied by an increase in the diagnosis of early or unsuspected meningitis. These data suggest the need for more judicious use of LP in children with periorbital swelling.

A case of orbital hematic cyst is reported. The patient was a 3-year-old boy in whom swelling of the left upper lid and left exophthalmos appeared after blunt trauma in the region of the left eye. A hematoma was thought to be present from upper lid to the orbit. The hematoma was absorbed but the swelling of the eyelid and exophthalmos increased though more than seven days elapsed after the trauma. The existence of the cyst located from the upper lid to the orbital deep portion and collection of blood were confirmed during operation. Histopathologically the cyst consisted of thickened fibrogranulomatous tissue and the inner wall lacked endothelial or epithelial lining. The blood collected in the cyst hardly coagulated and was examined hematologically. Extreme delay of activated partial thromboplastin-time and prothrombin time, extreme decrease of fibrinogen and abnormal increase of fibrin degradation products were recognized. This indicated that the blood in the cyst had been in a localized accelerated fibrinolytic state. The results support the hypothesis of the mechanism leading to the enlargement of hematic cyst proposed by Pearson et al.

Standardized sonography and CT scanning have distinct advantages and disadvantages in the evaluation of patients with orbital diseases. Echography provides an efficient screening examination in patients presenting with signs and symptoms of orbital pathology. It allows the detection, localization, measurement and in almost 80% differentiation of orbital lesions. High resolution CT scanning gives an excellent topographic display of masses in the orbit. In visualization of intracranial causes of orbital processes and demonstration of changes in the posterior third of the orbit, in the orbital bones and the periorbital sinuses it is superior to ultrasonography.