Orbital Pseudotumor Research Articles

P23 A case of partial restoration of vision in a patient with Wegener's granulomatosis and acute proptosis without signs of pseudotumor of orbit

Case report - IntroductionOphthalmologic manifestations occur in half of the patients with Wegener's granulomatosis and about 8% result in a complete loss of vision. Proptosis is a specific manifestation of Wegener's granulomatosis and often leads to a complete loss of vision due to ischemia of the optic nerve. In the literature there are rare cases of proptosis in these patients which are not accompanied by pseudotumor development. We present the case of a patient with histologically confirmed Wegener's granulomatosis who developed acute proptosis with a complete vision loss over the course of a week and a partial restoration of vision during therapy.Case report - Case descriptionIn March 2020 a 60-year-old man was diagnosed with Wegener's granulomatosis (based on lung biopsy) with involvement of lung (infiltrates with a cavity of destruction), upper respiratory tract (ethmoiditis, sphenoiditis, bloody crusts in the nose), ANCA-MPO+. 40mg of prednisone was prescribedwith a gradual decrease to 10mg. In the summer of 2020, the patient had a saddle-shaped deformity of the nose. In October he first noted a gradual decrease and then a complete loss of vision in the right eye, accompanied by temporary proptosis and headaches. In November 2020 CT scan of the orbits showed no signs of pathology. Intravenous therapy with methylprednisolone 2250mg in total and cyclophosphamide 800mg was carried out, the dose of prednisolone per os was increased to 15mg.After 2 weeks, the patient developed an acute complete vision loss in the left eye, accompanied by proptosis, pain in the orbit, headaches and ophthalmoplegia. He was admitted to ophthalmological hospital; glaucoma was ruled out. A spectral domain optical coherence tomography of the left eye was performed: the thickness of the layer of nerve fibres was within normal limits, the macular profile, the differentiation of all layers of the retina was preserved. A week after a complete loss of vision, the patient was transferred to Nasonova Research Institute of Rheumatology where therapy with rituximab (1g + 1g with an interval of 2 weeks), intravenous therapy with methylprednisolone 2750mg in total, anticoagulant, ethylmethylhydroxypyridine succinate, eradication therapy were carried out. During treatment, the patient noted a gradual partial restoration of vision (the ability to see large and medium objects with the left eye), disappearance of pain, and a decrease of proptosis. On MRI 2 days after the start of therapy, signs of exudative ethmoidite and sphenoidite and chronical mastoidite were revealed.Case report - DiscussionProptosis is one of the most common and specific ophthalmic manifestations of Wegener's granulomatosis. Proptosis is most often caused by a pseudotumor of the orbit; however, in this patient the genesis of proptosis is not clear because no signs of pseudotumor were detected on CT before the start of therapy or on MRI 2 days after therapy. It is possible that this patient had retrobulbar cellulitis with compressive ischemia of the optic nerve, single descriptions of which are found in the literature. However, its bacterial genesis was doubtful because there wasn't a change for the worse after 7 days of absence of antibiotic therapy. Alternatively, this patient could have developed Tolosa—Hunt syndrome or cavernous sinus thrombosis against the background of exudative sinusitis which can also manifest with similar symptoms. However, the fact that the symptoms developed during therapy of hormones per os (the dose remained stable) is contradicted by the development of the Tolosa—Hunt syndrome. During decrease in hormones for the observation period (half a year) relapses of these symptoms weren’t noted. CT and MRI showed no signs of cavernous thrombosis or Tolosa—Hunt syndrome. However, all the above-mentioned arguments are just our speculation because, due to the urgency of the situation and technical reasons, we could not perform MRI of the orbit before the start of therapy. Despite the large number of questions regarding the cause of vision loss in this patient, we decided to describe this case, since the therapy performed in this patient was very effective and led to a significant restoration of vision, even despite the fact it began a week later from the occurrence of vision loss. Perhaps in the future, our experience could be useful for other doctors who come across a similar difficult diagnostic situation.Case report - Key learning pointsDespite the fact that this patient had an established reliable diagnosis of Wegener's granulomatosis, this situation has left many questions for both our rheumatologists and ophthalmologists. This patient had many risk factors for an acute vision loss, both depending on the underlying disease and the risks of developing concomitant pathology, so we can’t make a final decision on the genesis of his symptoms. However, such difficult patients can be found by every ophthalmologist or rheumatologist, thus it is important to accumulate knowledge about their treatment. In this regard, we would like to present this case and share this successful experience of treatment with our colleagues.

P37 Significance of a swollen eye in a child with Down syndrome

Case report - IntroductionDue to dysregulation of their immune system, children with Down syndrome are at increased risk of a number of conditions, including malignancy, infection and autoimmune disorders. This case highlights the importance of the MDT team working in order to fully explore possible diagnoses and reach the correct conclusion. We present a case of a relatively rare eye disorder, initially treated as peri-orbital cellulitis, that required a collaborative team approach to reach the final diagnosis.Case report - Case descriptionA 6-year-old-girl with Down syndrome was referred to the oncology and tertiary ophthalmology teams by her local paediatric team with a history of recurrent right-eye peri-orbital swelling. The impression had been of recurrent peri-orbital cellulitis which was treated with and responsive to antibiotics. However, the most recent presentation was unresponsive to antibiotics and had associated orbital symptoms of proptosis and restricted eye movement. An MRI-head showed a protruding right eye with an enhancing mass centred in the right-superior temporal-extraconal-orbit. The mass was infiltrating the extra-ocular muscles and there was low bone-marrow signal compared to the left-side. The MRI findings were discussed in the oncology MDT-meeting. Appearances were felt to be more in keeping with malignancy, rather than infection. An ophthalmology review confirmed no evidence of optic-nerve compression, so she was discharged on antibiotics with a plan made to admit for biopsy and bone marrow aspirate under general anaesthetic. Histology of the lesion was reported to show a mixed inflammatory-mass, angiocentric with no elements of neoplasia. Oncology-specific stains were negative. BMA was normal. The differential at this point was orbital-pseudo-tumour; if infection and systemic-auto-immune conditions could be excluded. Histochemical-staining of the biopsy excluded fungi, bacteria or mycobacteria. The granulomatous-nature of the infiltrate and the conspicuous presence of eosinophils were consistent with an autoimmune-condition. The little girl was discharged from the oncology team into the care of the ophthalmology and paediatric rheumatology teams for further work-up and management. She was commenced on prednisolone.On rheumatology-review, focussed-history and examination were unremarkable in terms of an underlying systemic-inflammatory condition. A full-blood-work-up was requested. Her pANCA and MPO were mildly raised, as were her inflammatory markers. In view of these results, a CT chest/abdomen angiogram was arranged which was normal. A diagnosis of orbital-pseudotumour was made. There was clinical improvement on prednisolone, so a tapering plan was provided.Case report - DiscussionFirst described by Birch-Hirschfield in 1905, orbital pseudo-tumour, also known as idiopathic orbital inflammatory syndrome (IOIS) is a benign, non-infective, inflammatory condition of the orbit without identifiable local or systemic causes. Post-treatment recurrence occurs in 37% of cases.In this case, as steroids were weaned, there was recurrence of eye symptoms with proptosis and redness of the eye. Repeat bloods were performed, and an MRI-head requested. The repeat ANCA-MPO was negative. Whilst awaiting her MRI, a further clinical review was carried out jointly by paediatric rheumatology and ophthalmology. At this appointment, there was notable proptosis which appeared to be getting worse. The impression was that there was recurrence of the inflammatory swelling, and that the sensible option would be to restart treatment with oral prednisolone. At this point, addition of a steroid-sparing DMARD was discussed. Methotrexate was chosen and commenced. The little girl continued to attend for joint rheumatology and ophthalmology reviews. She continued to have proptosis in the right eye but had excellent ocular motility and good visual-acuity. She was noted to develop a worsening in the lid swelling and possible proptosis with an intercurrent illness, perhaps suggesting that the underlying inflammatory process was still active. The plan was to continue methotrexate for 2 years and perform another orbital MRI before potentially reducing methotrexate. However, in this time the methotrexate dose was reduced due to deranged LFTs. She continued to have “flare ups” with increasing proptosis despite treatment with methotrexate. A repeat MRI after 2 years of treatment showed reduction in the size of the right supra-lateral orbital mass compared to the first scan. However, there was uniform enhancement following gadolinium injection with mild proptosis of the right-globe. The appearances were in keeping with an active granulomatous lesion. The current plan is to try and re-optimise the dose of methotrexate. If not tolerated, an alternative DMARD will need to be considered.Case report - Key learning pointsThe diagnosis of orbital pseudo-tumour is one of exclusion with evaluation directed to exclude neoplasms, infections, and systemic inflammatory disorders. It can manifest as a bilateral condition in 13% and with constitutional symptoms in 40%.Points for discussionDifferential of orbital pseudo-tumours in the paediatric populationPrognosis of pseudo-tumoursSuggested treatment pathway -- Is control to allow normal eye movement enough or should we be treating more aggressively?

Pneumodescemetopexy With a Lower Concentration of Perfluoropropane (10% C3F8) in Descemet Membrane Detachment

A 75-year-old man with underlying left eye idiopathic orbital inflammatory disease and bilateral glaucoma suspect with cup disc ratio 0.7 underwent right eye phacoemulsification. Intraoperatively, Descemet tear and Descemet membrane detachment (DMD) occurred. Pneumodescemetopexy with air bubble was performed. His vision remained counting fingers and the cornea was oedematous three weeks after the operation. Anterior segment optical coherence tomography (ASOCT) showed extensive (80%) rhegmatogenous DMD with planar edge and a maximum of 460 micrometers separation from the stroma. Pneumodescemetopexy with low concentration perfluoropropane (10% C3F8) was performed together with postoperative positioning. On day five post-pneumodescemetopexy, his vision improved to 6/9, the cornea cleared with mild Descemet striae, and the gas bubble reduced to 30% fill. There was no DMD detected on ASOCT. His vision remained 6/9 and the residual gas bubble was 15% in the anterior chamber at two weeks post-pneumodescemetopexy. This case report suggests that pneumodescemetopexy with 10% C3F8 successfully reattached the large nonplanar rhegmatogenous DMD.

Single Institutional Experience on Orbital Inflammatory Pseudotumor: Diagnostic and Management Challenge

Aims: Orbital inflammatory pseudotumor is considered a non-neoplastic inflammatory process. The finding of clonality of B or T-cell receptors in cases pathologically diagnosed as orbital inflammatory pseudotumor has unknown clinicopathologic significance. We sought to investigate potential B and T-cell clonality and concomitant diseases in cases pathologically diagnosed as orbital inflammatory pseudotumor.Methods: Cases diagnosed as orbital inflammatory pseudotumor at our institution were retrospectively analyzed. Hematoxylin and eosin-stained slides, immunohistochemically stained slides and polymerase chain reactions on cell block material for the investigation of clonality of B and T-cell receptors were evaluated, to confirm the diagnosis and investigate the prevalence of concomitant diseases.Results: A total of 13 cases showing characteristic histopathologic features of orbital inflammatory pseudotumor were identified. CD138, IgG, and IgG4 showed varying numbers of plasma cells in each case, with 5 cases (5/13, 38%) exhibiting relative increase in the presence of IgG4 plasma cells. However, no cases showed diagnostic findings of IgG4-related disease (IgG4-RD). polymerase chain reactions analysis showed clonal B-cell populations in 2 cases (2/13, 15%). No cases showed anaplastic lymphoma kinase expression by immunohistochemistry. There were no clinical reports of progression to lymphoma or development of systemic IgG4-RD in any of the patients (average follow-up of 300 days), with 38% of patients showing systemic autoimmune conditions.Conclusion: A small but significant percentage of typical orbital inflammatory pseudotumor on histology showed B-cell clonality on polymerase chain reactions analysis of B-cell receptors, or features suggestive, but not diagnostic of IgG4-RD. Close follow-up of these patients to identify development of lymphoma, systemic IgG4-RD, or other rheumatologic conditions may be clinically warranted.

An Orbital Mass Partially Responsive to Steroid

A 68 year old woman presented with a progressively enlarging orbital mass. MRI of her brain and orbits was consistent with an orbital pseudotumor. Although there was some improvement in the patient’s pain and the size of the mass, it did not fully resolve.The patient had a biopsy demonstrate non-caseating granulomatous inflammation. A chest X-ray and CT chest demonstrated bilateral hilar lymphadenopathy. Further examination and interviewing of the patient revealed several months of joint paints and lower extremity nodules. The patient was diagnosed with orbital sarcoidosis and was started on methotrexate by a rheumatologist

Tolosa-Hunt Syndrome: A Review of Diagnostic Criteria and Unresolved Issues.

Purpose:To review the diagnostic criteria for Tolosa–Hunt syndrome (THS) and utility of recent modifications.Methods:We searched PubMed for keywords Tolosa Hunt and magnetic resonance imaging. We compared the three editions of International Classification of Headache Disorders and isolated case reports and case series with the assessment of cavernous internal carotid artery (ICA) caliber to find the prevalence of vascular anomalies. We also evaluated cases of THS with the involvement of extracavernous structures and the possible role of idiopathic hypertrophic pachymeningitis (HP). Cases diagnosed falsely as THS were also reviewed for the presence of atypical features and relevance of criterion D. We assessed nonconforming cases (those with normal neuroimaging benign THS) and idiopathic inflammatory orbital pseudotumor (IIPO).Results:Vascular abnormalities were found in 36.36% of THS cases. Benign THS may also show changes in ICA caliber. Evidence suggestive of idiopathic HP could be found in 57% of cases with the involvement of extracavernous structures, such as facial nerve and pituitary gland. Both THS and IIPO are steroid-responsive pathologies with similar clinical and radiological features. False-positive diagnosis of THS results from early labeling, based solely on clinical features and symptom resolution after steroid therapy.Conclusions:Benign THS may be a result of limitation of resolution of available neuroimaging technique or early testing. Early and late vascular changes can be seen in both THS and its benign variant; some of them are not innocuous. THS may be considered a type of focal idiopathic HP. IIPO may represent an anterior variant of THS. In the absence of histopathological diagnosis, steroid-induced resolution of symptoms should be confirmed radiologically and followed-up.

Ocular manifestations of inflammatory bowel diseases: an update for gastroenterologists.

Inflammatory bowel diseases (IBD), that includes ulcerative colitis and Crohn's disease, can affect not only the gastrointestinal tract but a wide spectrum of organs. The extra-intestinal manifestations (EIMs) are one of the most challenging aspect of IBD, playing a significant role for the lifetime care and the quality of life of patients. Ocular manifestations are the third most frequent EIMs, preceded by articular and dermatological ones. The aim of this narrative review is to describe the different types of ocular involvements, focusing on their clinical management. Uveitis and episcleritis are the most common ocular EIMs, differing for many aspects. Uveitis are unrelated with IBD activity and they even precede the onset of the intestinal disease, while episcleritis are common defined as a good mark of IBD activity. Pain is uncommon in most cases of episcleritis, while severe eye pain and photophobia are the most frequent onset of anterior uveitis. Less common but even more severe, are orbital pseudotumor or posterior segment involvement. Most of the ocular EIMs can be successfully treated with topic and oral steroids and the underlying therapy for IBD can reduce or cut out at all the recurrence of these manifestations. Symptoms are commonly not specific, in some cases being unnoticed for years leading to permanent ocular consequences. Cooperation between different specialists is crucial to avoid all the possible consequences of a non-treated EIMs, especially for ocular ones.

Orbital Pseudotumor: Diagnosis and Management in A Secondary Hospital

Objective: To increase the awareness in diagnosing orbital pseudotumor and its management, especially in a peripheral secondary hospital with limited facilities.Case presentation: A 47 years old woman came with left eye (LE) protrusion, blurred vision, retrobulbar pain, and severe headache at regular intervals. This condition begun 8 months ago, and was diagnosed with orbital cellulitis, however the symptoms persisted until now. Physical examination of the LE showed a decreased in visual acuity, protrusion, chemosis and conjunctiva injection, increased in IOP, limited ocular motility, ipsilateral afferent pupillary defect and optic disc oedema. Plain and contrast CT-Scan showed a hypertrophy of ocular muscles with soft tissue swelling. Patient then diagnosed with idiopathic orbital inflammation and immediately prescribed with high dose steroid for 5 days with observation with glucose level observation for toxicity. Afterwards, she was given an oral corticosteroid and was tapered off for the next few weeks. At two months follow up, there were improvement in protrusion, motility and visual acuity. Conclusion: A complete history taking, physical examination and appropriate additional examination are needed. In hospital or clinical setting with limited facilities, a thorough and comprehensive examination are needed in order to establish the diagnosis and management of orbital pseudotumor.

Поражение орбиты (псевдотумор и дакриоаденит) как манифестация полиорганного саркоидоза

Ocular manifestations of systemic sarcoidosis may emerge at any disease stage. Ocular sarcoidosis is typically characterized by uveitic presentations that predominantly involve the choroid of anterior eye segment. However, orbital pseudotumor, dacryoadenitis, and other rare presentations (optic neuritis, dacryocystitis etc.) also occur in sarcoidosis. Threatened vision loss and poor quality of life are the reasons to prescribe systemic steroids in generalized sarcoidosis. Chest CT is required to identify respiratory diseases when sarcoidosis manifests as uveitis or granulomatous orbital inflammation. Multidisciplinary diagnostic and treatment approach to suspected sarcoidosis provides timely verified diagnosis and management strategy. This paper addresses case report of systemic sarcoidosis in a 34-year-old man. The initial manifestation was vision loss and left orbital pseudotumor. The enlarged subclavian lymph node and its biopsy were suspicious of granulomatous inflammation. Chest CT identified disseminated pulmonary lesions and intrathoracic lymphadenopathy that are typical of sarcoidosis. The results of multidisciplinary examinations verified systemic sarcoidosis. Systemic steroids resulted in clinical and radiological improvement and medicinal regression of the disease. Keywords: sarcoidosis, orbital pseudotumor, dacryoadenitis, adenopathy, computed tomography, multidisciplinary approach. For citation: Burylova E.A., Mamaeva L.A., Berdnikova A.S., Fedorova O.K. Orbital lesion (pseudotumor and dacryoadenitis) is a manifestation of systemic sarcoidosis. Russian Journal of Clinical Ophthalmology. 2021;21(1):40–44. DOI: 10.32364/2311-7729-2021-21-1-40-44.

A good visual recovery in a patient with central retinal artery occlusion associated with Churg-Strauss syndrome: case report

Background: Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis of unknown etiology that affects small-to-medium-sized vessels. A multisystem disease that mostly affects the lungs and the skin. In addition, it could affect the renal, cardiovascular, gastrointestinal, central, and peripheral nervous systems. Ocular involvement in the CSS is rare. The reported ocular manifestations include corneal ulcer, uveoscleritis, conjunctival granuloma, orbital inflammatory pseudotumor, amaurosis fugax, retinal artery occlusion, ischemic optic neuropathy, oculomotor nerve palsy, and trochlear nerve palsy. Case Presentation: A 53-year-old man was admitted with sudden visual loss. CSS was diagnosed earlier, based on asthma, eosinophilia, peripheral neuropathy symptoms, and pulmonary infiltrates. His vision was completely lost in the left eye. Unilateral central retinal artery occlusion (CRAO) was observed by fluorescence angiography. Results: The treatment modality used in our patient was steroid pulse therapy, and his vision improved 24 hours later. This outcome might emphasize the importance of steroid pulse therapy since it may be effective for CRAO in CSS patients. Conclusion: This case report described a CSS patient with associated CRAO, in whom steroid pulse therapy improved his visual acuity. Therefore, since intensive treatment should be administered as early as possible to improve the prognosis of vision, rheumatologists should be aware of the presence of CSS-associated CRAO, and they should refer those patients to ophthalmology as soon as possible.

Isolated Unilateral Ptosis Caused by Idiopathic Orbital Myositis

Idiopathic orbital myositis is considered as a subgroup of idiopathic orbital inflammatory disease. It is a non-infectious inflammatory disorder primarily affecting the extraocular muscles and causes various eye symptoms including pain, diplopia and limitation of extraocular movement. Cases of isolated ptosis by idiopathic orbital myositis have been very rarely described in the literature. We report a patient who developed unilateral painless ptosis caused by idiopathic orbital myositis. A 52-year-old man presented with drooping of the right eyelid for 3 days. There was no history of headache, double vision or any other complaints. Neurological examination revealed right ptosis without pupil and extraocular muscles involvement. Repetitive nerve stimulation test was normal. Ptosis did not improve after the neostigmine injection. Magnetic resonance imaging scan showed asymmetric enlargement of right superior rectus/levator palpebrae superioris muscle complex and medial rectus muscle. Ptosis resolved dramatically after oral corticosteroid therapy. Isolated unilateral ptosis can be caused by various etiologies. Idiopathic orbital myositis should be considered in the differential diagnosis of ptosis. Keywords: Orbital myositis, Blepharoptosis, Oculomotor muscles

Autoimmune Pancreatitis and IgG4-related Cholangitis: A Single-center Experience

Background: Autoimmune pancreatitis (AIP) and IgG4-related cholangitis (IRC) are newly-recognized diseases. Reports in Thailand are scarce. Objective: To evaluate clinical presentations, other organ involvement (OOI), serology, radiologic, endoscopic features and treatment of AIP and IRC in one institute. Materials and Methods: Patients with AIP and IRC, who were diagnosed and followed-up at Siriraj Hospital during 2005 to 2016 were retrospectively reviewed. Results: There were 15 patients (75%) with AIP, 7 patients (37%) with IRC (4 isolated IRC and 3 IRC with AIP) and 1 (5%) with IgG4-related disease without AIP or IRC (sialadenitis and retroperitoneal fibrosis). Male to female ratio was 1.9: 1. Median age of onset was 64 years. Among the 15 AIP, all were type 1. Initial presentations were jaundice (60%), abdominal pain (40%), OOI (20%; sialadenitis, orbital pseudotumor), and weight loss (7%). Clinical manifestations during the course were jaundice (80%), abdominal pain (67%), weight loss (47%), OOI (47%; retroperitoneal fibrosis, IRC, sialadenitis, orbital pseudotumor, periurethral mass, lung nodules) and steatorrhea (7%). Serum IgG4 was elevated in all patients. Radiography showed diffuse type in 8 cases (53%), focal type in 5 (33%), and unknown in 2 (13%). More than half were suspicious of AIP before diagnosis. Corticosteroid was required in 10 patients (67%). Maintenance therapy was offered in 5 patients (33%) with steroid and azathioprine. Surgery was performed before diagnosis in 7 patients (47%). Spontaneous remission occurred in 4 patients (27%). Among the 7 IRC, initial presentations were jaundice (57%), weight loss (29%), abdominal pain (14%), prolonged fever (14%), and cholangitis (14%). Clinical manifestations during the course were jaundice (57%), abdominal pain (57%), weight loss (29%), recurrent cholangitis (14%) and retroperitoneal fibrosis (14%). Serum IgG4 was elevated in 5 patients (71%). Prednisolone was prescribed in 6 cases (86%). Five patients (71%) required azathioprine. Surgery was performed before diagnosis in 4 patients (57%). Conclusion: AIP and IRC were uncommon. The most common presentation was jaundice. All AIP were type 1 and more than half were diffuse type. OOI were common. Half of IRC had AIP. Approximately half of AIP/IRC patients underwent surgery before diagnosis. Corticosteroid was mainstay treatment. Keywords: Autoimmune pancreatitis, IgG4, Immunoglobulin G4, Sclerosing cholangitis, Thailand  

The Efficiency of Pulse Therapy with Glucocorticoids in Inflammatory Orbital Disorders: Case Report

Abstract Orbital pseudotumor represents a benign inflammatory disorder of the orbit, with unspecified etiology, whose clinical presentation can mimic the ocular pathology of Basedow disease, namely Graves’ ophthalmopathy, the two of them representing two of the most common orbital conditions. Imagistic methods and laboratory analyses can establish the diagnosis through orbital MRI images specific for the orbital pseudotumor, especially by dosage of the TSH receptor antibodies (TRAb) which will have increased values in Basedow disease with ocular damage. In both situations, the treatment is represented as a first therapeutic line by administration of corticotherapy in high dosage orally or intravenously, with favorably evolution, which is also observable in the case of our patient who was diagnosed with orbital pseudotumor based on MRI images, with associated thyroid dysfunction with negative TRAb, in which the pulse therapy with Solumedrol has determined the improvement of ocular symptoms and signs.

Clinical Analysis of Castleman's Disease of the Lacrimal Gland.

Objective To explore the clinical manifestations, imaging characteristics, and pathological characteristics of Castleman's disease of the lacrimal gland, enhance the knowledge of the disease, and improve the level of its diagnosis and treatment. Methods In the retrospective study, the data of 5 patients diagnosed with Castleman's disease of the lacrimal gland in Tianjin Medical University Eye Hospital from 2014 to 2018 were analyzed, and the relevant literature was reviewed. Results All the 5 patients were confirmed by pathological examination. Clinical manifestations were characterized by mass occupying lesions in the lacrimal gland area, without obvious pain, accompanied by eyelid swelling and ptosis, as well as space-occupying symptoms. Imaging examination showed that there was a soft tissue mass in the enlarged lacrimal gland area, and the mass was rich in blood flows while showing no obvious specificity, which could invade the surrounding muscles. All patients underwent surgical resection. Pathological results showed that 1 case was of the hyaline-vascular type, 3 cases were of the plasma cell type, and 1 case showed malignant transformation to plasma cell tumor. Conclusion Castleman's disease of the lacrimal gland is a rare orbital lymphoproliferative disease lacking specificity in clinical manifestations and imaging examination. As there are difficulties in differentiating the disease from orbital inflammatory pseudotumor and orbital lymphoma, its diagnosis still depends on pathological examination. The disease is mainly treated with surgical resection, and the pathological type is determined postoperatively.

Bilateral idiopathic orbital pseudotumour in a child: a case report

BackgroundIdiopathic orbital pseudotumour is rare in children. We report a case of bilateral paediatric idiopathic orbital pseudotumour and review the characteristics of this case.Case presentationA 14-year-old female patient presented at our Department of Pulmonary and Critical Care Medicine (PCCM) with complaints of recurrent severe cold and cough for 3 weeks, which had been treated with an intravenous antibiotic. Meanwhile, the patient developed swelling of both eyelids during the period of cold and cough, but her symptoms did not improve after the ocular administration of tobramycin dexamethasone eye drops. The patient was referred from the respiratory medicine ward to our department because of gradually worsening ocular pain, visual deterioration, increased intraocular pressure and serious nausea/vomiting within 24 h of hospitalization. The diagnosis of bilateral idiopathic orbital pseudotumour was made ultimately because of the course of the disease, exclusion of diagnoses such as bacterial ocular infection or malignant tumours and subsequent evidence from orbital magnetic resonance imaging (MRI). Favourable progress in the ocular tension and eyelid swelling was achieved through treatment with intravenous dexamethasone. The binocular intraocular pressure gradually declined to approximately 15 mmHg, and there was favourable progression in the patient’s vision to 20/40 in both eyes on the ninth day of hospitalization.ConclusionsOur patient developed rapidly progressive acute orbital signs and symptoms and anterior inflammation, such as pain, proptosis, limited ductions, periorbital oedema, chemosis, vision loss and high intraocular pressure. This case highlights that idiopathic orbital pseudotumour is an uncommon but important cause of acute orbital syndrome in children.

Arterial spin labeling and diffusion-weighted MR imaging: Utility in differentiating idiopathic orbital inflammatory pseudotumor from orbital lymphoma

PurposeTo assess arterial spin-labeling (ASL) and diffusion-weighted imaging (DWI) and in combination for differentiating between idiopathic orbital inflammatory pseudotumor (IOIP) and orbital lymphoma. Material and methodsA retrospective study was done on 37 untreated patients with orbital masses, suspected to be IOIP or orbital lymphoma that underwent ASL and DWI of the orbit. Quantitative measurement of tumor blood flow (TBF) and apparent diffusion coefficient (ADC) of the orbital lesion was done. ResultsThere was a significant difference (P = 0.001) in TBF between patients with IOIP (n = 21) (38.1 ± 6.2, 40.3 ± 7.1 ml/100 g/min) and orbital lymphoma (n = 16) (55.5 ± 7.1, 56.8 ± 7.9 ml/100 g/min) for both observers respectively. Thresholds of TBF used for differentiating IOIP from orbital lymphoma were 48, 46 ml/100 g/min revealed area under the curve (AUC) of (0.958 and 0.921), and accuracy of (86% and 83%) for both observers respectively. There was a significant difference (P = 0.001) in ADC between patients with IOIP (1.04 ± 0.19, 1.12 ± 0.23 × 10−3 mm2/s) and orbital lymphoma (0.69 ± 0.10, 0.72 ± 0.11 × 10−3 mm2/s) for both observers respectively. Thresholds of ADC used for differentiating IOIP from orbital lymphoma were 0.84 and 0.86 × 10−3 mm2/s with AUC of (0.933 and 0.920), and accuracy of 89% and 90% for both observers respectively. The combined TBF and ADC used for differentiating IOIP from orbital lymphoma had AUC of (0.973 and 0.970) and accuracy of (91% and 89%) for both observers respectively. ConclusionTBF and ADC alone and in combination are useful for differentiating IOIP from orbital lymphoma.

Role of Quantitative Spectral CT Analysis for Differentiation of Orbital Lymphoma and Other Orbital Lymphoproliferative Disease

PurposeTo investigate the value of quantitative parameters from spectral computed tomography for the differentiation of orbital lymphoma from other lymphoproliferative disease, including idiopathic orbital inflammatory disease (IOID) and IgG4-related disease (IgG4-RD). MethodsPatients with orbital masses who underwent pre-treatment contrast-enhanced spectral CT were enrolled in this retrospective study. The subjects were divided into lymphoma and other orbital lymphoproliferative disease groups. Qualitative imaging features (margin, location, enhancement pattern, cranial nerves, soft tissue, and bone involvement) were reviewed. Quantitative parameters (iodine density and spectral attenuation curve slope) derived from spectral CT were measured. ResultsEleven patients had orbital lymphoma and 11 had other orbital lymphoproliferative diseases (idiopathic orbital inflammatory disease (IOID), n = 5; IgG4-related disease (IgG4-RD), n = 6). Qualitative analysis showed no significant difference between the two groups. There was significantly higher iodine density in orbital lymphoma (1.24 ± 0.24 mg/ml) than in IOID/IgG4-RD (0.83 ± 0.23 mg/ml; P = 0.001). An iodine density threshold of 1.0 mg/ml gave sensitivity, specificity, and accuracy of 81.8%, with an area under the curve of 0.876 (P = 0.0003). Orbital lymphoma had a significantly higher iodine spectral attenuation curve slope (2.44 ± 0.51 HU/keV) than IOID/IgG4-RD (1.66 ± 0.47 HU/keV; P = 0.001). A threshold of 1.99 HU/keV for the spectral attenuation curve slope of 40–70 keV gave sensitivity, specificity, and accuracy of 81.8%, with an area under the curve of 0.884 (P = 0.0002). ConclusionsQuantitative spectral CT parameters can help differentiate orbital lymphoma from other orbital lymphoproliferative disease, with lymphoma having a significantly higher iodine density value and spectral attenuation curve slope than IOID/IgG4-RD.

Difficulties in the differential diagnosis of granulomatosis with polyangiitis and scarring pemphigoid

The article presents a clinical case of a rare pathology. The patient for several years visited various specialists. In connection with a similar clinical picture, multi-organ damage, differential diagnosis was performed between systemic vasculitis (granulomatosis with polyangiitis) and scarring pemphigoid.The mucous membrane of the mouth and nose is involved in the pathological process with granulomatosis with polyangiitis in the form of ulcerative defects, which subsequently lead to deformation of the nose. The danger with this systemic vasculitis is renal damage with the development of nephritis, renal failure. With scarring pemphigoid, eye damage is typical. In granulomatosis with polyangiitis, damage to the organ of vision is also sometimes possible, but mainly in the form of an orbit pseudotumor. Despite the fact that treatment for both forms of pathology involves the use of glucocorticoids and cytostatics, with a scarring pemphigoid, the main care is provided by dermatologists and ophthalmologists, while the diagnosis and treatment of systemic vasculitis is the task of rheumatologists. One of the criteria for a scarring pemphigoid is loss of vision. However, in this case, it was possible to establish a diagnosis, obtain the first positive results of therapy before the patient shows signs of disability. So, there is a hope for the possibility of preserving vision and a favorable outcome. The rarity of the disease and its poor knowledge, difficulties in diagnosis and the absence of certain standards of therapy, this diagnosis requires more attention from the specialists.

Minimally Invasive 360-Degree Approach to Intraconal Orbital Tumors

To evaluate clinically the efficacy of 360-degree minimally invasive endoscopic surgery in patients with orbital intraconal tumors. Retrospective interventional case series. A series of consecutive patients with orbital intraconal tumors were treated with minimally invasive surgery using an endonasal endoscopic approach or an anterior endoscopic orbitotomy over a 5-year period between January 2015 and January 2020 at the National Institute of Oncology and Radiobiology of Cuba. In 22 patients, the mean age was 52.3 ± 11 years (range 18-71 years) with a female to male ratio of 1.8:1. Visual loss was present in 3 patients and proptosis was present in 19 patients. The most common diagnosis was cavernous hemangioma (72.7%) followed by orbital sheet meningioma (9.1%). The lower-inner (type II) location was the most usual, followed by the lower-outer (type IV). A gross total resection was achieved in 95.5% of patients. In only 1 case with an orbital pseudotumor was the resection subtotal because of the fibrous consistency, irregularity, and adherences to the medial rectus muscle. All patients with visual loss improved to normal visual function after surgery at follow-up and orbital proptosis recovered in all patients with these symptoms. Both immediate and long-term morphologic and aesthetic results were good in all patients. The minimally invasive 360-degree surgical approach with a full endoscopic visualization can be safe and efficient in patients with select orbital intraconal tumors. Optic nerve location constitutes the main aspect in the surgical approach selection. A large case series is necessary to standardize this surgical philosophy.

Vertical restrictive strabismus associated with proptosis: Similar clinical signs, different etiopathogenetic causes. A report of three patients.

To report the different uncommon pathogenesis of three cases of severe vertical restrictive strabismus associated with progressive unilateral proptosis with similar clinical features. Case series of three patients who presented to the Orbit Outpatient Service of Policlinico Gemelli with a history of left progressive unilateral proptosis, slowly worsening vertical strabismus and the left eye fixed in downward position. A thorough hematologic work up was performed. All patients underwent complete abdomen ultrasonography, orbital contrast enhanced magnetic resonance imaging, forced duction test under general anesthesia, and orbital biopsy. Patients were 30, 60, and 46 years old respectively. MRI showed left inferior rectus enlargement in two cases and superior rectus enlargement in one case, with contrast enhanced combined muscle belly and tendon enlargement in all cases. Patients underwent forced duction test, muscle weakening (in two cases), and muscle biopsy with histopathologic examination. The superior rectus appeared infiltrated by an undifferentiated high-grade pleomorphic sarcoma, whereas the two inferior recti were positive for idiopathic orbital inflammatory disease with fibrosis areas and neuromuscular choristoma, respectively. Although proptosis and acquired vertical restrictive strabismus are most commonly associated with thyroid associated orbitopathy (TAO), they can also be a manifestation of many other conditions and the differential diagnosis can be particularly challenging. The three reported cases presented indeed with similar clinical features but had three distinct underlying orbital etiologies, two of which were extremely uncommon.