Bone destruction of orbital wall in idiopathic orbital inflammatory pseudotumor:does it always imply malignancy?

AIM:To assess the clinical presentations and outcomes of idiopathic orbital inflammatory pseudotumor(IOIP)patients with orbital wall bone destruction(OWBD)and to propose an expanded classification system that includes bone destruction.METHODS:The study retrospectively reviewed clinical presentations,imaging findings,treatment modalities,and outcomes of six patients diagnosed histopathologically with IOIP and OWBD at the Beijing Tongren Hospital,Capital Medical University between October 2018 and June 2021.RESULTS:Over two years,6(10%)of 60 IOIP patients at our hospital exhibited OWBD,but this may overrepresent severe cases.The cohort consisted of three men and three women,aged 17 to 60y(mean 35.5±16.1y).Presenting symptoms included proptosis,eyelid swelling,decreased visual acuity with pain,and palpable mass.Imaging revealed multiple anatomical structures involved with the medial wall being the most common site of bone destruction.Histopathological examination showed classic type in five patients and sclerosing type in one patient.All patients underwent surgical resection followed by methylprednisolone treatment.Follow-up(mean 30.3±3.1mo)indicated three patients had no recurrence,while others had varying degrees of symptom persistence or recurrence.CONCLUSION:IOIP with bone destruction is a rare but significant subtype that mimics malignancy,leading to potential diagnostic and therapeutic challenges.Our findings suggest that complete surgical resection combined with adjunctive glucocorticoid therapy can yield favorable outcomes.However,larger-scale studies are needed to further optimize therapeutic approaches.

Relationship between dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor and paranasal sinusitis

AIM：To determine the clinical features of the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor and its relationship with paranasal sinusitis.· METHODS：A retrospective analysis of 46 patients who received surgical treatment at the Department of Ophthalmology,Beijing Tongren Hospital,Capital Medical University for the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor from October 2010 to December 2012.Each patient underwent magnetic resonance imaging（MRI） of the orbits and the 4paranasal sinuses.Disease status and the level of serum immunoglobulin G4（IgG4） was measured before and6 mo after surgery.· RESULTS：The initial clinical feature of the idiopathic dacryoadenitis type of orbital inflammatory pseudotumor was redness or swelling of the eyelids.Masses were palpated in the area of the lacrimal gland in some patients.Of the 46 patients,16 also suffered from sinusitis（34.8%）,with 14 cases of ethmoid sinusitis,8cases of maxillary sinusitis,9 cases of sphenoid sinusitis,and 8 cases of frontal sinusitis.Of the 16 patients with sinusitis,4 patients had a medical history of rhinitis（range：10mo to 15 y previously）,10 patients had occasional nasal congestion,and 2 patients had no nasal congestion.Thirteen of the 46 patients had elevated serum IgG4 levels.Nine of these 13 patients had MRI signs of sinusitis.All patients（n=46） received oral glucocorticoid treatment for approximately 3mo after surgery.No sign of recurrence was found in the orbital MRI 6mo after surgery.Of the 16 patients with sinusitis,9cases of elevated serum IgG4 levels improved after treatment with decreased serum IgG4 level and 7 cases of normal serum IgG4 levels remained unchanged.· CONCLUSION：Some patients with the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor may also suffer from paranasal sinusitis.The incidence of paranasal sinusitis was much higher in patients with IgG4-elevated dacryoadenitis subtype orbital inflammatory pseudotumor than in those with normal IgG4 levels.Dacryoadenitis subtype orbital inflammatory pseudotumor and paranasal sinusitis may both the clinical manifestations of IgG4-related disease involved in different locations.

Role of IgG4 serology in identifying common orbital lymphoproliferative disorders

AIM： To explore the role of IgG4 serology in identifying common orbital lymphoproliferative disorders, METHODS： Eighty -one patients with orbital lymphoproliferative diseases were treated in the Department of Ocular Oncology, Beijing Tongren Hospital, Capital Medical University between September 2010 and December 2012. Serum IgG4 levels were measured in 46 cases of idiopathic orbital inflammatory pseudotumor （IOIP）, 17 benign lymphoepithelial lesion （BLEL）, 12 cases of orbital mucosa-associated lymphoid tissue （MALT）, and 6 cases of diffuse large B-cell lymphoma （DLBL） using immuno-scatter turbidmetry （ISTM）.RESULTS： The frequency of elevated IgG4 levels in patients with IOIP, BLEL, MALT, and DLBL was 30.43% （14/46）, 76.47% （13/17）, 8.33% （1/12）, and 0.00 （0/6）, respectively. Among the patients with elevated serum IgG4 levels, all IgG-IOIP patients were male, and 92.31% of the IgG4-BLEL patients were female （12/13）. The mean serum IgG4 level of IgG4-1OIP patients was lower than that of individuals with IgG4-BLEL, but the variation in serum IgG4 levels was larger in IgG4-1OIP than IgG4- BLEL patients. Only one case of IgG4-MALT with elevated serum IgG4 levels had a medical history 〉10y, which was significantly longer than the MALT patients with normal serum IgG4 levels. There was no significant elevation of serum IgG4 levels in patients with DLBL. CONCLUSION： Detecting serum IgG4 levels plays an important role in the differential diagnosis of orbital lymphoproliferative diseases.

IgG4 and IgE co-positive group found in idiopathic orbital inflammatory disease

AIM： To reveal the cytokines involved in idiopathic orbital inflammatory disease（IOID） and the relationship between Th17 cells, IgE and IOID pathogenesis.METHODS： Whole blood samples were processed immediately after collection and serological IgG4, IgG, and IgE antibodies were tested using ELISA. IOID and orbital cavernous hemangioma（CH） tissue samples underwent Bio-Plex multiplex cytokine detection. Hematoxylin-Eosin（HE） staining of all paraffin samples suggested the histological features of IOIDs, and expressions of IgG4 and IL-17 A in affected tissues were detected by immunohistochemistry. RESULTS： Among 40 IOID plasma samples, 52.5%（21/40） were positive for IgG4 and 25%（10/40） were positive for IgE. Overlapped IgG4 or IgE positive samples accounted for 22.5%（9/40）. Therefore, IOID samples were separated into three groups. The IgE＋/IgG4＋ group had a relevantly lower level of pro-inflammatory cytokine expression. IL-4（Th2 cell related）, IL-10 and TGF-β1（Treg cell immunity related） were elevated in all three groups. Some of the Th17 cell related cytokines（i.e. IL-17 A/F, IL-25, IL-23, and IL-33） displayed higher expression levels in the IgE-/IgG4-group compared to the other two groups.CONCLUSION： We discovered an IgG4-IgE co-positive group as well as Th17 cell immune involvement in IgG4-IgE co-negative subgtroup in IOID for the first time. The pathogenesis of IOID could differ from different subgroups according to the IgG4 and IgE detection. Therefore, we recommend that, Treatment stratagy should be made according to the clinical assessment of IgG4-IgE and Th17 profile detection.

Epstein-Barr virus-encoded small RNAs in idiopathic orbital inflammatory pseudotumor tissues: a comparative case series

AIM： To investigate the positive rate and types of cells that express Epstein-Barr virus-encoded small RNAs （EBERs） and to determine the distribution of EBER-expressing cells in idiopathic orbital inflammatory pseudotumor （IOIP） tissues. METHODS： We retrospectively examined 40 archived paraffin specimens from two teaching hospitals in Southern China between January 2007 and January 2015 that were pathologically determined to exhibit IOIP. Eleven concurrent paraffin specimens of thyroid-associated ophthalmopathy （TAO） composed the control group. In situ hybridization was performed to detect EBERs. Immunohistochemistry was employed to detect CD3, CD20, Vimentin, and smooth muscle actin （SMA）, and the positive rate, types of positive cells, and distribution and location of EBERs were evaluated. RESULTS： The positive expression rate of EBERs was 47.5% （19/40） in the IOIP group, which was significantly higher than that in the TAO group [0 （0/11）, P=-0.011]. in the IOIP group, the lymphocyte infiltrative subtype, fibrotic subtype, and mixed subtype exhibited EBER-positive rates of 57.1% （12121）, 12.5% （118）, and 54.5% （6/11）, respectively, and no significant differences were found between these subtypes （P=0.085）. Positive signals of EBERs were mainly present in medium-small lymphocytes between or around follicles and in the nuclei of activated immunoblasts （14/19）. CONCLUSION： The positive rate, types, and distribution of EBER-expressing cells in IOIP have been documented.These findings are conducive for a better understanding of the underlying mechanisms of Epstein-Barr virus infection in IOIP pathogenesis.

MRI manifestations of enlarged superior ophthalmic vein

Objective:To assess MRI in the evaluation of enlarged superior ophthalmic vein (SOV). Methods: MRI manifestations and etiology of forty-six patients with enlarged SOV were analyzed. Results: SOV enlargement was noted to occur in carotid-cavernous fistula, ophthalmic Graves'disease, Tolosa-Hunt syndrome, inflammation at the apex of the orbit, orbital pseudotumor and thrombosis of cavernous sinus. The dilated vein appeared as signal void tubular shadows on both T1 and T2 weighted images. The diameter of the enlarged vein was 3.5-6.0 mm. Extraocular muscle enlargement, orbital pathologies, enlarged carotid cavernous sinus etc were also revealed by MRI. Conclusion: The dilated SOV may be well demonstrated by MRI. The etiological diagnosis of enlarged SOV can be made in combination with the associated findings.

Orbital apex syndrome due to trauma in a 3-year-old boy

Orbital apex syndrome is a disastrous ophthalmic disease which usually leads to visual loss and immobility in the injured eyes. Up to now, no effective treatment has been achieved. So as much as possible attention should be paid to prevent such tragedies.