Ectopic orbital meningioma: Fact or fiction?

Primary intraorbital ectopic meningiomas are rare and their existence remains controversial. We present a 30-year-old female with painless, non-axial proptosis and a palpable superomedial mass. The MRI demonstrated that the mass had no optic nerve sheath or sphenoid wing involvement and was initially reported to have no intracranial extension. The patient was initially thought to have an ectopic orbital meningioma. Subsequent multidisciplinary team (MDT) consultation and further specialist review of the MRI revealed a subtle dural tail connecting to an enhancing mass in the olfactory groove. Biopsy revealed a WHO Grade 1 transitional meningioma with an infiltrative pattern. We argue that some previously reported cases of ectopic meningioma may lack the requisite imaging to discover the primary disease. Our report highlights the importance of MRI in this group of patients and the role of a skull-base MDT with specialist neuroradiology input to determine the true origin and extent of these extradural orbital meningiomas.     

Cognitive biases in orbital mass lesions – Lessons learned

Purpose

A patient’s presentation and clinical diagnosis can at times be clouded by their past medical history. Clinicians’ anchoring bias towards initial information, such as a history of cancer, may lead them astray when creating a differential diagnosis for a patient who presents with new signs and symptoms of a mass lesion, assuming metastatic disease without seeking tissue confirmation.

Periocular thelaziasis presenting as an orbital mass – a case report

Thelazia callipaeda is a rare parasitic infestation caused by spiruroid nematode of the genus Thelazia. We report a case of a 74-year-old gentleman who presented with a painless swelling of left lower lid since 15 days. Examination revealed a firm mobile mass along the inferior orbital rim. Magnetic Resonance Imaging showed a well-defined preseptal cystic lesion and Ultrasound screening revealed multiple mobile worms within. Patient underwent cyst excision in toto under local anesthesia. Four long refractile worms were isolated from within the cyst cavity. Species identification confirmed the parasite as Thelazia callipaeda. Periocular thelaziasis usually presents as free floating worms in the conjunctival sac, anterior chamber or vitreous cavity. It is important to be aware of this rare entity which should be considered as a differential diagnosis in endemic areas.     

Idiopathic orbital inflammation with sclerosing mesenteritis: a new association?

A 70-year-old man, who was recently diagnosed with sclerosing mesenteritis following an abdominal biopsy, presented with an acute onset of left upper eyelid swelling, moderate ptosis, mild chemosis and restriction of movements. A computed tomography scan showed an enlarged lateral rectus muscle with surrounding soft tissue changes. A diagnosis of orbital inflammation manifesting as myositis was made and the patient was commenced on high-dose prednisolone, which showed a rapid response. It is believed that this may be a new association similar to that previously reported with retroperitoneal fibrosis and orbital inflammation.     

Surgical repair of orbital fractures: how soon after trauma?

PURPOSE: To determine the results of surgical exploration soon after orbital trauma in comparison with surgery half a year or longer after trauma. METHODS: A retrospective study was done on 41 patients with motility disorders after trauma. Full ophthalmologic and orthoptic investigation was done in all patients. CT-scans and/or X-ray photographs were also made. The 41 patients were divided into two groups. One group of 13 patients did not need surgical repair. One group of 28 patients needed an operation. RESULTS: In 13 patients there was an orbital fracture but no surgical repair was needed. In 28 patients surgical repair was done. In 8 patients the operation was performed half a year or longer after trauma, 15 patients underwent surgery within two weeks after trauma, and 5 patients within two months. CONCLUSION: The best final results were obtained when the operation was done soon after trauma; however, the results in the patients who were operated late were fairly good too.     

“The influence of orbital vector on involutional entropion and ectropion”

Purpose: This study aimed to evaluate the effect of orbital vector and other biometric parameters (i.e. axial globe length, axial globe projection) on the development of involutional entropion or ectropion.

Methods: In this cross-sectional study, 167 eyes from 132 patients were included. Of these eyes, 128 had involutional entropion and 39 had involutional ectropion, all of lower lids. The axial globe projection was measured using Hertel exophthalmometry; axial globe length was assessed by A-mode ultrasound; and orbital vector was determined clinically. Patient-specific categorical variables and continuous variables were compared using the chi-square test and the two-sided t test, respectively. Correlations were derived using the Pearson correlation.

Results: The percentage of females was 59% and 33.3% in the entropion group and in the ectropion group, respectively. A significant association was found between the gender and type of eyelid malposition (p = 0.015). Exophthalmometry reading was greater in the ectropion group than in the entropion group (17.7 ± 2.5 versus 10.8 ± 3.7 mm, respectively; p < 0.001). There was no significant correlation between axial globe length and exophthalmometry reading. Positive orbital vectors were observed in 87.5% of eyelids with involutional entropion. Negative orbital vectors were observed in 92.3% of eyelids with involutional ectropion (p < 0.001). Patients with negative orbital vectors showed greater axial globe projection than patients with positive orbital vectors (18.0 ± 2.1 versus 10.6 ± 3.5 mm, respectively; p < 0.001).

Conclusions: There is an association between orbital vector measurement and involutional entropion and ectropion. Measuring the orbital vector may help predict the development of these lid malpositions.     

Color Doppler imaging of the orbital vessels in Behçet's disease

OBJECTIVE: To investigate blood flow velocity changes, pulsatility and resistivity index values of orbital vessels in Beh?et's disease with or without ocular involvement (groups I and II) and healthy volunteers (group III). METHODS: Twenty-five eyes in the first group, 24 eyes in the second group and 24 eyes in the third group were examined by using color Doppler imaging. Peak systolic and end-diastolic blood flow velocity measurements, pulsatility and resistivity index values were measured in ophthalmic, central retinal, nasal and temporal ciliary arteries and the central retinal vein. RESULTS: The end-diastolic blood flow velocity measurements in all arteries were significantly higher in groups II and III than in group I, but resistivity and pulsatility indexes of all arteries were higher in group I than the other two groups. There was no significant difference in blood flow velocity of the central retinal vein in the three groups. CONCLUSION: Detection of increased vascular resistance of orbital vessels may predict the diagnosis of ocular involvement in Beh?et's disease.     

Bilateral orbital tumor formation and isolated facial palsy in Waldenstrom’s macroglobulinemia

A 32-year-old lady presented with bilateral painful proptosis and left infranuclear facial palsy. She was mildly anemic. Ocular examination revealed a firm non-tender mass in the super lateral quadrant of both orbits. Fine-needle aspiration cytology from the orbital mass showed the presence of lymphoplasmacytoid cells. Bone marrow aspiration also showed the presence of similar cells. An IgM monoclonal gammopathy was seen on serum protein electrophoresis. A diagnosis of Waldenstrom’s macroglobulinemia was made on the basis of these findings. Hyperviscosity retinopathy is the most commonly reported ocular abnormality in Waldenstrom’s macroglobulinemia. Orbital tumor formation and cranial nerve palsies are rarely reported in this condition. We describe the first case of Waldenstrom’s macroglobulinemia presenting as an isolated orbital mass and facial nerve palsy.     

【In Press】 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-17A in affected tissues were detected by immunohistochemistry. RESULT: 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-17A/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.     

Bilateral orbital isolated (solitary) neurofibroma in the absence of neurofibromatosis – A case report

Isolated neurofibroma is a slowly progressive tumor rarely found in the orbit accounting for less than 1% of the space occupying lesions of the orbit. It usually presents in the 2nd to 5th decades of life with proptosis, swelling, visual changes, ptosis, diplopia or pain. Almost all cases reported are unilateral. We report a 23-year-old female with no systemic features or family history of neurofibromatosis who presented with right upper eyelid swelling. Radiological studies revealed bilateral identical masses in the superior orbits. The patient underwent surgical excision of the lesion on the right side and it was proved to be neurofibroma. She did not have a recurrence with a follow up period of 2?years but developed sensory deficit. This is the fifth reported case of bilateral isolated neurofibroma.     

IgG4-related orbital disease masquerading as thyroid eye disease,vice versa,or both?

A 40 year-old male presented after one year of unilateral, progressive, steroid-responsive, orbital inflammatory disease causing proptosis, extraocular muscle (EOM) restriction, and compressive optic neuropathy. The development of anti-thyroidal antibodies prompted the diagnosis of thyroid eye disease (TED); however, the prolonged active phase, remarkable reversibility of ophthalmic features with high-dose corticosteroids, unilaterally of disease, uncharacteristic EOM involvement (including both obliques), and the absence of autoimmune thyroid disease provoked consideration of alternative diagnoses. Inferior oblique biopsy stained positive for IgG4 with histologic features atypical of TED. The patient received rituximab for presumed IgG4-related orbital disease (IgG4-ROD) with subsequent reversal of compressive optic neuropathy, near complete resolution of EOM restriction, and improved proptosis, the latter two of which are not routinely anticipated in advanced TED. The possible role for B-cell depletion in both TED and IgG4-ROD suggests a degree of overlap in the underlying immune-related pathophysiology that is yet to be defined.     

Heparanase-1 is downregulated in chemoradiotherapy orbital rhabdomyosarcoma and relates with tumor growth as well as angiogenesis

AIM:To determine the role of heparanase-1(HPSE-1)in orbital rhabdomyosarcoma(RMS),and to investigate the feasibility of HPSE-1 targeted therapy for RMS.METHODS:Immunohistochemistry was performed to analyze HPSE-1 expression in 51 cases of orbital RMS patients(including 28 cases of embryonal RMS and 23 cases of alveolar RMS),among whom there were 27 treated and 24 untreated with preoperative chemoradiotherapy.In vitro,studies were conducted to examine the effect of HPSE-1 silencing on RMS cell proliferation and tube formation of human umbilical vein endothelial cells(HUVECs).RD cells(an RMS cell line)and HUVECs were infected with HPSE-1 sh RNA lentivirus at a multiplicity of infection(MOI)of 10 and 30 separately.Real-time PCR and Western blot were applied to detect the m RNA and protein expression levels of HPSE-1.Cell viability of treated or control RD cells was evaluated by cell counting kit-8(CCK-8)assay.Matrigel tube formation assay was used to evaluate the effect of HPSE-1 RNAi on the tube formation of HUVECs.RESULTS:Immunohistochemistry showed that the expression rate of HPSE-1 protein was 92.9%in orbital embr yonal RMS and 91.3%in orbital alveolar RMS.Tissue from alveolar orbital RMS did not show relatively stronger staining than that from the embryonal orbital RMS.However,despite the types of RMS,comparing the cases treated chemoradiotherapy with those untreated,we have observed that chemoradiotherapy resulted in weaker staining in patients’tissues.The expression levels of HPSE-1 declined significantly in both the m RNA and protein levels in HPSE-1 sh RNA transfected RD cells.The CCK-8 assay showed that lentivirus-mediated HPSE-1 silencing resulted in significantly reduced RD cells viability in vitro.Silencing HPSE-1 expression also inhibited VEGF-induced tube formation of HUVECs in Matrigel.CONCLUSION:HPSE-1 silencing may be a promising therapy for the inhibition of orbital RMS progression.