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orbital pseudotumor

Andreu Fernández-Codina, Blanca Pinilla, Iago Pinal-Fernández, Cristina López, Guadalupe Fraile-Rodríguez, Eva Fonseca-Aizpuru, Iago Carballo, Pilar Brito-Zerón, Carlos Feijóo-Massó, Miguel López-Dupla, Maria Cinta Cid, Fernando Martínez-Valle
BACKGROUND: IgG4-related disease (IgG4-RD) is an autoimmune disease triggering an inflammatory cascade that leads to fibrosis. Outcome measures are limited and treatment options remain underexplored. OBJECTIVES: To assess the variation of the IgG4 responder index (IgG4-RI) in a cohort of IgG4-RD patients and to explore their treatments and outcomes. METHODS: We studied the clinical phenotype, severity of the disease and response to treatment in an ambispective multicenter cohort study including 14 different hospitals in Spain...
February 13, 2018: Joint, Bone, Spine: Revue du Rhumatisme
Anastasia Drobysheva, Julie Fuller, Cory M Pfeifer, Dinesh Rakheja
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...
January 1, 2018: International Journal of Surgical Pathology
Mitchell R Gore
Orbital inflammatory pseudotumor is an infrequent lesion that may mimic malignancy and may be associated with autoimmune disease. Orbital pseudotumor is in the differential in any patient with an orbital mass, especially in the setting of chronic infection or immunosuppression. This HIV-positive patient presented with chronic sinusitis and orbital pseudotumor.
November 2017: Clinical Case Reports
Hong Ki Min, Youn Soo Lee, Suk-Woo Yang, Jennifer Lee, Seung-Ki Kwok, Ji Hyeon Ju, Wan-Uk Kim, Sung-Hwan Park
Background/Aims: This study investigated the clinical and pathological features of immunoglobulin G4 (IgG4)-related ophthalmic disease. To clarify the features, we compared IgG4-related ophthalmic disease and orbital inflammatory pseudotumor. Methods: We retrospectively reviewed the medical records of 103 patients who were initially diagnosed with orbital inflammatory pseudotumor, and identified 16 cases in which the diagnosis was based on surgical biopsy and for which data in medical records were sufficient for analysis...
October 19, 2017: Korean Journal of Internal Medicine
William Phillip Pereira da Silva, Larissa Balbo Zavarez, Fernando Luiz Zanferrari, Juliana Lucena Schussel, Leonardo Pérez Faverani, Juliana Elizabeth Jung, Laurindo Moacir Sassi
Inflammatory myofibroblastic tumor, also known as inflammatory pseudotumor and plasma cell granuloma, is a tumor that occurs most often in the lungs, abdomen, skin, soft tissue, genital system, and mediastinal. Before surgery, the diagnosis is difficult to establish because of its diverse manifestations. In the head and neck, manifestation is rare and may occur in the upper respiratory tract, soft tissues, orbits, and skull base. This article aims to report a rare manifestation of the disease in the face, highlighting the importance of a correct diagnosis to determine the most appropriate form of treatment, in male patient, leucoderma, 22 years old, with complain of a painless unilateral growth in the left cheek, beginning 2 months before and with progressive growth...
November 2017: Journal of Craniofacial Surgery
W A Lagrèze, N Gross, J Biermann, L Joachimsen
BACKGROUND: Placement of a ventricular shunt is the primary surgical procedure for lowering intracranial pressure in pseudotumor cerebri syndrome; however, if ophthalmological symptoms prevail over neurological symptoms or if there are no neurological symptoms at all, optic nerve sheath fenestration may be a valuable option for relief of pressure on the retrobulbar optic nerve when papilledema caused by pseudotumor cerebri syndrome threatens vision despite previous conservative measures...
October 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
Makoto Hibino, Tetsuri Kondo
A 77-year-old Japanese woman presented to an ophthalmologist with an erythematous swollen upper eyelid and diplopia which was initially diagnosed to be idiopathic dacryoadenitis on the basis of a histological evaluation of an orbital mass that was in remission following a 3.5-month period of systemic corticosteroid therapy. She subsequently developed respiratory symptoms, and was finally diagnosed with systemic granulomatosis with polyangiitis (GPA) based on the clinical and histological features. She was successfully treated with corticosteroids and azathioprine...
October 1, 2017: Internal Medicine
Min-Wei Ren, Yi Du, Shan Ren, Cheng-Ye Tang, Jian-Feng He
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...
2017: International Journal of Ophthalmology
Chen Chen, Hongmin He
Extranodal NK/T cell lymphoma (ENKL) is a rare entity, associated with an aggressive clinical behavior and poor prognosis. The optimal treatment strategies, particularly for relapsed patients, have not been clearly determined. A 40-year-old female ENKL patient with orbital involvement as the first presentation was initially suspected to have an orbital pseudotumor. When the patient developed disease progression following treatment with methylprednisolone, biopsy of the orbital mass was performed. Histopathological examination of the biopsy specimen was consistent with the diagnosis of ENKL (Ann Arbor stage IE)...
October 2017: Molecular and Clinical Oncology
Evan S Marlin, David Dornbos, Daniel S Ikeda, Norman L Lehman, Ciarán J Powers
BACKGROUND: Diffuse intracranial aneurysmal vasculopathy is a rare condition, previously described in patients with human immunodeficiency virus infection. IgG4-related disease (IgG4-RD) is a recognized inflammatory disease of systemic organs, leading to fibrosis of connective tissues. It also has been linked to inflammatory dilating aortic aneurysms, coronary vascular disease, hypophysitis, orbital pseudotumor, and pachymeningitis. It has not yet been described as a cause of diffuse intracranial dilating vasculopathy...
November 2017: World Neurosurgery
Davide Soloperto, Cristoforo Fabbris, Flavia DI Maro, Daniele Marchioni
No abstract text is available yet for this article.
June 22, 2017: Journal of Neurosurgical Sciences
Puneet S Braich, Robin K Kuriakose, Naveen S Khokhar, Jared C Donaldson, Timothy J McCulley
OBJECTIVE: To assess clinical characteristics of patients at risk for recurrent nonspecific orbital inflammation (NSOI). METHODS: This was a retrospective comparison study with a 24-month follow-up, in a clinic-based population in rural India. The medical records at two eye care centers were searched from March 2001-2014. A total of 153 patients with recurrent NSOI, 107 of whom had a single recurrence (Group 1) and 46 of whom had multiple recurrences (Group 2). Clinical and demographic data were compared between both groups...
June 20, 2017: International Ophthalmology
Abdulkadir Shehibo, Fisseha Admassu, Tiruzer Bekele, Martha Solomon, Liya Solomon, David M Gordon
Orbital pseudotumor is a rare, idiopathic, inflammatory process within the orbit. Early diagnosis is essential to prevent corneal, retinal and extraocular muscle damage. This article describes the presentation and clinical course of a 3-year-old male with bilateral orbital pseudotumor.
June 5, 2017: Journal of Tropical Pediatrics
M Colombi, C Dordoni, M Venturini, C Ciaccio, S Morlino, N Chiarelli, A Zanca, P Calzavara-Pinton, N Zoppi, M Castori, M Ritelli
Classical Ehlers-Danlos syndrome (cEDS) is characterized by marked cutaneous involvement, according to the Villefranche nosology and its 2017 revision. However, the diagnostic flow-chart that prompts molecular testing is still based on experts' opinion rather than systematic published data. Here we report on 62 molecularly characterized cEDS patients with focus on skin, mucosal, facial, and articular manifestations. The major and minor Villefranche criteria, additional 11 mucocutaneous signs and 15 facial dysmorphic traits were ascertained and feature rates compared by sex and age...
December 2017: Clinical Genetics
J Heichel, C Neumann, D Bethmann, U Siebolts, T Hammer, H-G Struck
Background Tumors of the lacrimal duct are rare. Inflammatory pseudotumors do not represent neoplasias as such. Chronic inflammation may lead to an angiomatous granulation tissue. Patients The first case presents a 53-year-old male patient suffering a mass of the mediocaudal orbit after an acute dacryocystitis has been cured. Using diagnostic dacryoendoscopy, a large dacryolith was detected. Removing the concrement, via external dacryocystorhinostomy, a polypous tissue appeared which was resected as well. The second case concerns a 29-year-old female patient...
May 3, 2017: Klinische Monatsblätter Für Augenheilkunde
Promil Jain, Rajeev Sen, Nisha Sharma, Shilpi Bhargava, Virender Singh
Tumefactive fibroinflammatory lesions (TFLs) are rare idiopathic benign fibrosclerosing lesions that clinically simulate a malignancy. TFLs are seen more frequently in males between 10 and 74 years of age. The usual site of involvement is the head and neck region, but rarely the extremities may be involved. Coexisting fibrosclerotic processes have been reported including retroperitoneal fibrosis, sclerosing cholangitis, sclerosing mediastinal fibrosis, and orbital pseudotumors. The etiology of this poorly understood entity remains unknown...
March 2017: Iranian Journal of Medical Sciences
A K Tanon, C Assouan, E Anzouan-Kacou, D N'guessan, A Salami, S Boni, E Konan
INTRODUCTION: The invasive fungal infection of the maxillary sinus is a rare and serious disease generally favored by immunosuppression. We report an exceptional case of pseudotumoral invasive fungal infection of the maxillary sinus in an immunocompetent patient. OBSERVATION: A 32-year-old patient consulted for labial and left temporal swelling associated with proptosis and chemosis that has been developing for 18 months. The scanner objectified a filling of the left maxillary sinus, and the ipsilateral orbital cavity, and the surrounding muscles...
March 20, 2017: Journal de Mycologie Médicale
Rachna Meel, Rebika Dhiman, Meenakshi Wadhwani, Seema Kashyap, Sanjay Sharma, Ajay Gogia
PURPOSE: Natural killer T-cell lymphoma (NKTL) is a highly malignant tumor that typically arises in the nose and/or paranasal sinuses. Isolated orbital involvement by NKTL is extremely rare, and only few case reports exist in the literature. Herein, we report a case of primary orbital NKTL in a young patient who showed very good response to chemotherapy with the SMILE regimen. METHODS: A 28-year-old healthy female presented to us with epiphora and fullness of the lower eyelid...
January 2017: Ocular Oncology and Pathology
Iman Aouidad, Pierre Schneider, Mathieu Zmuda, Jeremy Gottlieb, Manuelle Viguier
No abstract text is available yet for this article.
March 1, 2017: JAMA Dermatology
M Leo, F Maggi, G R Dottore, G Casini, P Mazzetti, M Pistello, S Sellari-Franceschini, M Nardi, P Vitti, C Marcocci, M Marinò
OBJECTIVE: One of the hypotheses on the pathogenesis of autoimmune diseases, including Graves' disease (GD) and Graves' orbitopathy (GO), involves bacterial or viral infections. Recently, Epstein-Barr virus (EBV) has been proposed to play a role in the pathogenesis of idiopathic orbital inflammatory pseudotumor (IOIP) in Asians. The aim of the present study was to investigate the possible association of GO with EBV infection/exposure, as compared with IOIP, using serum and tissue samples, as well as primary cultures of orbital fibroblasts...
May 2017: Journal of Endocrinological Investigation
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