Search

Introduction: Rosai-Dorfman disease (RDD) is a sinus histiocytosis with massive lymphadenopathy. This rare condition is a benign disease of unknown etiology. Bilateral orbital localization of RDD is rare., Observation: The authors report the case of a 6-year-old child who presented with bilateral orbital-palpebral masses associated with chronic cervical lymphadenopathy. There were no laboratory signs of inflammation. Serological and tuberculosis screening tests were negative. Histopathological examination of a lymph node biopsy established a diagnosis of Rosai-Dorfman disease. The patient underwent surgical excision of the orbital lesions followed by long-term corticosteroid therapy. A favorable course was observed, with no sign of recurrence after one year of follow-up., Conclusion: Rosai-Dorfman disease is very rare in its bilateral orbital presentation. Histopathological diagnosis remains challenging. In Africa, the presence of chronic oculo-palpebral tumor associated with or without cervical lymphadenopathy must raise the suspicion of Rosai-Dorfman disease after ruling out tuberculosis and lymphoma., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Purpose: To describe a case series of patients investigated in internal medicine for orbital inflammation (OI) since the individualization of the clinical entity of the IgG4-related orbital disease (IgG4 ROD)., Patients and Methods: Thirty four patients were consecutively referred by a specialized center where orbital biopsy was performed when the lesion was surgically accessible. Fourteen patients were excluded in case of missing data or lymphoma, periocular xanthogranuloma or Graves' orbitopathy. Patients with systemic or auto-immune disease according to the international criteria, or presenting with idiopathic orbital inflammation syndrome (IOIS), were included. Knowing the histological similarities between IOIS and IgG4 ROD, immunohistochemical assessment of plasma cells for IgG4 positivity was performed for every patient with available biopsy. Clinical and biological characteristics, treatment and response to treatment of included patients are reported., Results: Among 22 included patients, 10 presented with orbital manifestation of a systemic or autoimmune disease including 2 sarcoidosis (9%) and 8 (36%) cases of non specific OI which were reclassified in IgG4 ROD. Finally, IOIS of various clinicopathological presentation was diagnosed for 12 patients including 10 with histological documentation. Whereas relapse and resistance were not found to be related to IgG4 positivity (50% in both IOIS and IgG4 ROD groups), another treatment in addition to corticosteroids was more often necessary in IgG4 ROD patients (50%) than in IOIS patients (25%)., Conclusion: After ruling out auto-immune orbital diseases, especially IgG4 ROD, IOIS should be discussed. Factors conditioning the corticosteroid response are yet to be determined., (Copyright © 2020. Published by Elsevier Masson SAS.)

Inflammatory orbitopathies relate to an inflammatory state originating within the orbit and its adnexes, except the inner ocular globe. Orbital inflammation (OI) may be either localized manifestation of a proven or like autoimmune disease, or local response from immune system against infectious, structural or tumoral antigens. We review the clinical manifestations of OI, which provide helpful clues to the diagnosis and describe the inflammatory, infectious and neoplastic conditions classically associated with OI. Autoimmune diseases are probably the most common causes of OI associated with a bilateral dacryoadenitis (e.g., sarcoidosis, granulomatosis with polyangiitis, IgG4-related disease). We focused on a major part of the IgG4-RD spectrum, the IgG4-related orbital disease which has been recently described and the idiopathic orbital inflammation syndrome that one should consider in patients 40 years of age or older with non specific inflammation OI on biopsy but without underlying local or systemic disease. An algorithm for the diagnostic approach of OI was proposed. If systemic explorations fail to diagnose an underlying disease, histopathologic control is required for distinguishing non-specific OI from other differential diagnosis, especially lymphoma. In the cases of pure myositic locations and posteriorly located tumours where biopsy could damage to the optic nerve, analysis of orbital lesions in T2W IRM sequence may be helpful to distinguish idiopathic OI (IOI) from lymphoma. When the diagnostic work-up fails, a corticosteroid trial could be used, but its beneficial effect has to be cautiously interpretated before definitively diagnosing IOI. Finally, treatments used in main infllammatory orbitopathies were also reviewed., (Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Purpose: To assess demographic, clinical, radiological, pathological features, treatment and follow-up of periocular or/and orbital amyloidosis., Patients and Methods: We conducted an observational retrospective monocentric study from January 2004 to April 2017 in patients diagnosed with histologically proven periocular or/and orbital amyloidosis., Results: Six patients were included (2 females, 4 males). Mean age was 76.8 years (range 66-88 years). Mean time between first ophthalmological symptoms and diagnosis was 27 months (range 11-36 months). The main symptoms were subconjunctival infiltration (6 patients; 100%), periocular pain or discomfort (4 patients; 66.6%) and subconjunctival hemorrhage (1 patient; 16.6%). Clinical findings included ptosis (4 patients; 66.6%), keratitis (3 patients; 50%) leading to corneal perforation in one patient, and proptosis (3 patients; 50%). One-half of the patients showed bilateral involvement. AL amyloidosis was identified on immunohistochemistry in 5 patients (83.3%). One case of B cell marginal zone orbital lymphoma was diagnosed. Systemic work-up was negative for all patients. Treatment consisted of simple monitoring (1 patient; 16.6%), surgical debulking (3 patients; 50%), ptosis surgery (1 patient; 16.6%), eyelid or eyelash malposition surgery (2 patients; 33.3%) and orbital radiation beam therapy (2 patients; 33.3%). Mean follow-up was 14.6 months (range 6-36 months), and no progression nor recurrence were noted., Conclusion: Periocular or/and orbital amyloidosis is rarely encountered. Diagnosis is based on pathological examination, and immunohistochemistry analysis should always be performed to guide systemic work-up. Orbital lymphoma and multiple myeloma should be ruled out if AL amyloidosis is diagnosed. Progression is slow, and surgery is the mainstay of treatment in symptomatic patients. Long-term multidisciplinary follow-up is advocated., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Diplopia is defined as "double vision" when looking at a single object. Monocular diplopia is related to an ocular disorder and must be differentiated from binocular diplopia which is secondary to ocular misalignment. The examination of the patient with binocular diplopia is often challenging for non-specialists. However, a careful and systematic clinical examination followed by targeted ancillary testing allows the clinician to localize the lesion along the oculomotor pathways. The lesion may involve the brainstem, the ocular motor nerves III, IV or VI, the neuromuscular junction, the extraocular ocular muscles, or the orbit. Causes of binocular diplopia are numerous and often include disorders typically managed by internal medicine such as inflammatory, infectious, neoplastic, endocrine, and metabolic disorders. In addition to treating the underlying disease, it is important not to leave diplopia uncorrected. Temporary occlusion of one eye by applying tape on one lens or patching one eye relieves the diplopia until more specific treatments are offered should the diplopia not fully resolve., (Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

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.)

Introduction: Neuromuscular hamartoma, also known as neuromuscular choristoma or benign triton tumor, is a rare benign tumor of well-differentiated striated muscle fibers mixed with peripheral nerve fibers. According to our research, this is the sixth case of benign triton tumor of the trigeminal nerve and the third case of isolated orbital location reported in the world literature., Purpose: To report a rare case of orbital neuromuscular hamartoma and discuss the role of corticosteroids in the treatment of these lesions for which surgical excision is often difficult., Observation: A 47-year-old woman, with a history of tuberculous lymphadenitis treated in 2006, presented with a clinical scenario of inflammatory orbitopathy without loss of visual acuity progressing over 20 days. MRI showed a lesion centered on the soft tissues of the infero-lateral right orbit. A biopsy was performed, showing neuromuscular hamartoma on histology. The patient was put on a tapering dose of corticosteroids with clear clinical and anatomical improvement. Orbital CT follow-up was obtained two months after discontinuation of treatment, confirming the disappearance of the tumor mass., Conclusion: Hamartoma of the orbit is a very rare entity and may clinically simulate malignant neoplasms; the diagnosis is histologic. Given the difficulties encountered in the resection of these tumors, we believe that corticosteroids might be proposed as an alternative treatment that could modulate inflammation and bring about regression or disappearance of the tumor., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Orbital inflammation is a generic term encompassing inflammatory pathologies affecting all structures within the orbit : anterior (involvement up to the posterior aspect of the globe), diffuse (involvement of intra- and/or extraconal fat), apical (involvement of the posterior orbit), myositis (involvement of only the extraocular muscles), dacryoadenitis (involvement of the lacrimal gland). We distinguish between specific inflammation and non-specific inflammation, commonly referred to as idiopathic inflammation. Specific orbital inflammation corresponds to a secondary localization of a "generalized" disease (systemic or auto-immune). Idiopathic orbital inflammation corresponds to uniquely orbital inflammation without generalized disease, and thus an unknown etiology. At the top of the differential diagnosis for specific or idiopathic orbital inflammation are malignant tumors, represented most commonly in the adult by lympho-proliferative syndromes and metastases. Treatment of specific orbital inflammation begins with treatment of the underlying disease. For idiopathic orbital inflammation, treatment (most often corticosteroids) is indicated above all in cases of visual loss due to optic neuropathy, in the presence of pain or oculomotor palsy., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Introduction: Observational study of the diagnoses and the treatment modalities used for orbital inflammatory pathologies discussed in multidisciplinary meetings (MDM) in Nantes University Medical Center over 4 years., Material and Method: A multidisciplinary meeting to discuss eyelid and orbital pathologies (excluding cancer) has been held in Nantes three times per year since October 2008. This retrospective study focuses on the cases of orbital inflammatory pathologies discussed at this meeting from October 2008 to October 2012 (49 cases included)., Results: Twenty-eight (57%) patients were diagnosed with orbital inflammation, 16 (33%) cases with isolated myositis and 5 (10%) with dacryoadenitis. In the diagnostic work-up, orbital biopsy was performed in 64% of orbital inflammation cases, 38% of myositis cases and 80% of dacryoadenitis cases. These specimens led to the diagnosis of 9 lymphomas, 2 histiocytoses and 1 metastasis. The internal medicine work-up allowed for the diagnosis of 3 granulomatoses with polyangiitis, one patient with sarcoidosis, and one patient with tuberculosis. Despite work-up, there remained 14 (29%) idiopathic orbital inflammatory syndromes (IOIS)., Discussion: The MDM of orbital pathology allows for the collective approach to the most difficult cases of management and is not concerned with stable orbitopathies or those presenting no diagnostic dilemma. Despite a multidisciplinary, rational etiologic work-up (frequently involving a biopsy), we find a higher rate of IOIS than reported in the literature., Conclusion: A multidisciplinary approach appears to be useful in improving management of orbital disease., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Acute myositis is the second most common component of non-specific orbital inflammation. We will describe its clinical features and natural history. This is a retrospective study of 10 cases. The diagnosis of acute myositis was based on clinical and imaging criteria. Our study includes five men and five women. The average age was 35.8 years (17-59 years). Clinical symptoms were: pain increased on eye movement (10/10), diplopia (4/10), proptosis (6/10), visual loss (3/10), lid edema (6/10), conjunctival hyperemia (7/10), anterior scleritis (2/10), episcleritis (2/10), chemosis (4/10), upper lid retraction (1/10), limitation of eye movement (3/10), fundus abnormalities (2/10). Imaging showed thickening of one or more extraocular muscles (10/10). Recovery was complete with anti-inflammatory therapy in six patients. Three patients experienced recurrence, and one patient had a clinical rebound upon tapering the treatment. Acute myositis can be defined by pain on eye movement, signs of inflammation, and extraocular muscle thickening on imaging. If the clinical presentation is typical, histopathological analysis can be deferred but remains necessary in cases of poor response to treatment, chronic duration or suspicion of tumor infiltration. The diagnosis of acute myositis may be suspected in the presence of consistent, well-defined clinical signs. Contiguous inflammation is often associated. Treatment is based on steroids or non-steroidal treatment anti-inflammatory therapy, administered alone or consecutively. Recurrences are frequent but do not alter the final prognosis., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Mucormycosis is an invasive fungal infection associated with high mortality. Orbital involvement was usually reported but was rarely isolated. We report the case of a 57-year-old patient who presented a proptosis associated to an ethmoid sinusitis. Inflammatory symptoms were late which was responsible for delayed diagnosis. Our patient died because of multi-organ failure., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Introduction: Mycetomas are pathological processes through which exogenous fungal or actinomycotic etiological agents produce grains. These etiological agents live in the soil and plants of endemic areas. They are introduced traumatically, primarily into the foot. The orbital location is rare., Observation: We report the case of a 17-year-old student admitted for progressive left proptosis over 2 years, following penetrating trauma by a fork in a rural setting. Examination revealed a heterogenous orbital mass with multiple fistulae, producing pus and black grains, and suggested, due to the color of the grains, a diagnosis of fungal mycetoma. MRI revealed a destructive process at the level of the lamina papracea of the ethmoid and the orbital floor. Anatomopathological examination confirmed the fungal nature of the infection, while culture in Sabouraud's medium was inconclusive. The outcome was favorable after exenteration and debridementof the ipsilateral maxillary sinus and nasal cavities, along with 4 months of ketoconazole. No recurrence has been observed for 14 months after surgery., Discussion: Mycetomas are endemic to northwest Africa. Most frequently located in the foot, they are seldom seen in the orbit. The color of the grains provides a clue as to the etiology. Black-grain mycetomas are always fungal and are treated surgically--essentially like cancer--as the persistence of a single grain will cause a recurrence., Conclusion: The orbital location of a mycetoma is rare. In the present case report, the concept of port of entry, the clinical appearance, and the color of the grains guided the diagnosis. The histological examination of the surgical specimen confirmed the diagnosis., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Introduction: Granulomatosis with polyangeitis (Wegener's granulomatosis) (GPA) is a granulomatous vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA). Affected organs usually include upper and lower respiratory tracts, and kidneys. Limited forms of GPA may affect the central nervous system (vasculitis, hypertrophic pachymeningitis, encephalitis), a location in which diagnosis is often uneasy., Case Report: We report a 74-year-old woman who presented with a limited form of GPA affecting the cavernous sinus. Diagnosis was considered in view of a retrospectively suggestive clinical presentation, compatible cerebral MRI and temporal artery biopsy, despite the absence of ANCA. It was supported by a favourable outcome with cyclophosphamide administration., Conclusion: GPA presenting as a cavernous sinus syndrome is rare. Three co-existing pathogenic mechanisms may be involved in GPA affecting the central nervous system: contiguous invasion from nasal or orbitary granulomatous sites, vasculitis, or primary intra-cerebral granulomatous inflammation. Lack of biopsy evidence of affected tissues and ANCA negativity should not delay diagnosis and appropriate therapeutic management in central nervous system GPA., (Copyright © 2012 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

We report an atypical case of cysticercosis of the left levator-superior rectus complex, mimicking a painful partial third nerve palsy. The patient presented with painful diplopia, with limitation of elevation, depression and adduction of the left eye, associated with a ptosis of the upper left eyelid. Magnetic resonance imaging revealed two cysts, located within the left levator-superior rectus complex, and nine parenchymal cysts located in both hemispheres of the brain. Antibody titers directed against Cysticercus cellulosae were elevated as confirmed by Elisa and Immunoblot. Diplopia and local inflammation resolved after albendazole and systemic steroid treatment. Surgical resection of the cysts was unnecessary., (Copyright © 2012. Published by Elsevier Masson SAS.)

Introduction: Paranasal sinuses mucoceles are extremely rare in children and adolescents. The objective of this study was to assess their clinical presentations and the contribution of endoscopic surgery in their management., Material and Methods: we retrospectively reviewed 11 children with paranasal sinus mucoceles who were managed during a period of 15 years (1995-2009)., Results: The mean age was 11.8 years with a sex ratio of 2.66. One patient had a history of nasal polyposis surgery. The symptomatology was dominated by ophthalmological complains. The ethmoïd sinus was the main localization. Orbital extension was found in eight cases, pyomucocele in one case. The surgical management was endoscopic (nine cases), and by an external approach (two cases). Two cases of recurrence were assessed. The mean follow-up was 22.3 months., Discussion: Paranasal sinuses mucocele is exceptional in children. Cystic fibrosis is the main etiologic factor. The symptoms depend on the location and size of the mucocele. The treatment is surgical and most authors prefer the endoscopic approach., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Evaluation of a patient with orbital disease needs a multidisciplinary approach. Clinical examination should seek to glean information regarding pathophysiologic effect and location and to evaluate consequences of this disease on cosmetic, vision and ocular motility. Procedures of imaging are based on the implied complementary exams that should be done according to the suspected clinical location and characters help to characterize the underlying process. Imaging can define the location, size, limits and relationship of the disease to other tissues and especially to the vascular system. It may be completed by blood test. The final diagnosis is based on several arguments and can appeal for a diagnostic biopsy in the most difficult cases., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)