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Acute orbital pseudotumors are inflammatory lesions of unknown etiology that may affect part or, less often, all of the tissue within the orbit. A retrospective computed tomographic (CT) study of 16 patients demonstrated that these lesions occur in one of five specific anatomic patterns: anterior, posterior, diffuse, lacrimal, or myositic. The most common location was lacrimal followed by anterior pseudotumors. Posterior, most common location was lacrimal followed by anterior pseudotumors. Posterior, diffuse, and myositic pseudotumors were equally frequent. Localization on the basis of clinical features correlated with the CT localization. Illustrative cases of each of the five types are included. The role of CT in evaluating the therapeutic response is discussed.

CT scans of 9 patients with orbital pseudotumor (bilateral in 6 and unilateral in 3) showed findings distinct from those observed in Graves' ophthalmopathy. In bilateral involvement, they ranged from localized mass lesions to complete obliteration of normal orbital CT anatomical landmarks; diffuse or multifocal lesions involving the posterior globe and muscle insertions were most typical of the diagnosis. However, findings in unilateral pseudotumor may be indistinguishable from orbital mass lesions other than Graves' ophthalmopathy. Serial CT scans were used to show progression of disease and response to treatment.

Supervoltage radiotherapy was used in 21 orbits of 19 patients with idiopathic inflammatory orbital pseudotumor. Seventeen orbits (15 patients) were initially treated with systemic corticosteroids, but recurrence of orbital inflammation during dosage tapering was the most frequent indication for radiotherapy. Fifteen orbits (14 patients) responded favorably, as judged by reduced proptosis, decreased lid edema and conjunctival injection, improved ocular motility, and increased visual acuity. Six orbits (five patients) did not improve with radiotherapy. Patients who were successfully treated with radiotherapy have been free of recurrence for a mean follow-up period of 25.05 months; these patients have not required further corticosteroid treatment or additional radiotherapy. Low-dose (1,000 to 2,000 rad) supervoltage radiotherapy seems to have a definite role in the management of idiopathic orbital pseudotumor in the following instances: (1) when corticosteroids fail or systemic complications are unacceptable (2) when signs and symptoms recur during decreasing corticosteroid dosage, and (3) when systemic corticosteroids are medically contraindicated.

A patient with unilateral proptosis and goiter is presented who initially was thought to have Graves' disease. However, the histologic appearance of the orbital tissue and the results of thyroid function and serologic studies were most consistent with the diagnoses of orbital pseudotumor and Hashimoto's thyroiditis, respectively, as the cause of these abnormalities. Two previous instances of an association between orbital pseudotumor and thyroiditis have been reported. A review of the literature shows that the ophthalmologic signs in patients with a pseudotumor superficially resemble those observed in subjects with thyroid ophthalmopathy. However, a palpable supraorbital mass is often present and biopsy of the mass usually demonstrates the heavy lymphocytic infiltration typical of this disease. Since the implications of the diagnosis of pseudotumor differ from those of Graves' disease, this entity should be considered in the differential diagnosis of the patient presenting with unilateral proptosis.

In a retrospective study comprising 12 patients with the diagnosis or orbital myositis the authors present an account on the importance of standardized echography for the differential diagnosis and for the follow-up of the disease in relation to treatment. They describe the sonographic finding in affected oculomotor muscles and in myositis of the upper oblique muscle incl. the echographic finding of trochleitis. The authors discuss echographic signs of myositis (thickness and shape of muscle, reflectivity and internal structure) in image A and image B and their correlation with the histopathological finding. The authors discuss differential diagnostically important differences of the echographic picture of myositis, endocrine myopathy and others.

RESUMEN Introducción: La miositis orbitaria aguda es una afección inflamatoria de la musculatura extrínseca ocular muchas veces de causa desconocida. Forma parte de un grupo de afecciones inflamatorias orbitarias inespecíficas englobadas bajo el término de pseudo tumor orbitario. Afecta principalmente a las mujeres jóvenes. Objetivo: Describir el caso de una adolescente con miositis orbitaria aguda, afección inflamatoria poco frecuente en la edad pediátrica. Presentación del caso: Se trata de una adolescente de 14 años, mestiza, femenina, quien sufrió dengue por tres ocasiones poco tiempo antes de presentarse con dolor ocular del ojo izquierdo, fiebre elevada intermitente, protrusión ocular intermitente, vómitos, cefalea, decaimiento, pérdida de peso y de apetito. El ojo izquierdo presentaba edema del parpado superior y limitación a la abducción, musculatura ocular extrínseca: diplopía horizontal a mirada derecha a izquierda. El ultrasonido ocular informa presencia de imagen quística que no impresiona ser de contenido hemático. El resultado de la tomografía axial computarizada de órbita y cráneo simple fue compatible con diagnóstico de miositis orbitaria. Se indicó tratamiento inmediato con esteroides orales y se remitió al Instituto de Oftalmología “Pando Ferrer” para seguimiento. Conclusiones: La miositis orbitaria exige un alto índice de sospecha para un diagnóstico y tratamiento correcto. El dengue pudiera estar implicado en la etiología de la enfermedad.

We report a patient who developed an acute symmetrical polyarthropathy, orbital myositis and posterior scleritis following inoculation with the 1993 Fluvirin (inactivated influenza vaccine: surface antigen) vaccine. We believe that this represents a hypersensitivity reaction to the vaccine as extensive investigations revealed no underlying infective, inflammatory or systemic cause. We review the current indications for vaccination against the influenza virus and the known adverse reactions to the vaccine.

Hypertrophic pachymeningitis is an infrequent condition which starts with a thickening of the dura mater and whose pathogenesis is unknown. We present two new cases of unknown aetiology.Case 1. A 53 year old man complained of occipital headache, tinnitus and deafness since February 1981. In October 1981 he was admitted to hospital with a worse headache, perio-orbital pain, dysgeusia and ipsilateral peripheral facial palsy. In December he had generalized tonic-clonic seizures and paralysis of the VII and XI right cranial nerves and IX, X and XII left cranial nerves. In February 1982 he developed right trigeminal neuralgia. He was readmitted in November 1983 with continuous headache, vomiting and a behavior disorder. On CT there was marked attenuation of the posterior dura mater, which the neurosurgical department considered unsuitable for biopsy. He died in March 1985. On necropsy there was hypertrophic pachymeningitis. Case 2. A 62 year old patient consulted in November 1995 complaining of right hypoacusia for the past six months, progressively accompanied by ipsilateral paralysis of the II, IV, VI, VII and VIII cranial nerves but with no other alterations on physical examination. Analytical and serological investigations were normal. Cranial MR showed an extraparenchymatous infiltrating lesion in the middle cranial fossa. Biopsy was decided on when no clinical improvement was seen with corticosteroid treatment. The pathologist reported hypertrophic pachymeningitis. Treatment was started with cyclophosphamide in monthly doses and the condition has remained stable to date.With these two cases we wish to establish a pathogenic relation between the Tolosa-Hunt syndrome and orbital pseudotumor and show the role played by immunosuppressive treatment in the control of hypertrophic pachymeningitis.

The orbital pseudo-tumor is an orbital inflammatory disease of unknown origin that can affect all the anatomical structures that make up the orbit. The diagnosis is based on the assessment of clinical symptoms, imaging tests and the exclusion of other possible causes. Glucocorticosteroids are used for treatment, but other immunosuppressants as well as biological treatments can be used. The aim of the study is to present, based on the literature review, the current state of knowledge about pathogenesis, symptoms, differential diagnosis, and treatment of the orbital pseudotumor.

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We have reported a case of acute orbital pseudotumor, an idiopathic inflammatory process that usually responds dramatically to corticosteroids. Untreated, severe proptosis and eventual optic neuropathy may result. Although its signs and symptoms may cause confusion with infectious, endocrinologic, traumatic, allergic, and neoplastic ophthalmopathies, the acute onset, rapid progression, and characteristic CT picture are nearly pathognomonic of orbital pseudotumor. When both the clinical and CT findings are compatible with this diagnosis, a therapeutic trial of oral corticosteroids can be undertaken. It is important for the primary physician to be aware of this disease to facilitate appropriate diagnosis and treatment, avoiding unnecessary surgical intervention and possible complications.

• A case of myasthenia gravis in association with benign orbital pseudotumor of lymphoid origin is described. To our knowledge, this is the second report of such a case.

• Fifty-five patients, suspected on clinical grounds of having an inflammatory orbital pseudotumor, were studied retrospectively. Clinical groups based on natural history or response to corticosteroid therapy were correlated with available histopathological findings. These findings were interpreted without knowledge of clinical grouping or previous pathology reports. Although dogmatic statements are not possible, several conclusions are warranted. (1) Bilaterality was not significantly related to response to steroids or development of systemic disease. (2) B-scan ultrasonography, while not diagnostic, provided a worthwhile investigative technique. (3) Germinal follicles are associated with a good prognosis and indicate a reactive lesion, while diffusely distributed lymphoblasts are associated with steroid unresponsiveness and a probable neoplastic lymphoid lesion. (4) Eosinophils are more common in reactive lesions than in presumed lymphomas. Other cellular components showed no preferential distribution in either neoplastic or nonneoplastic groups.

In Reply. —Dr Schechter is entirely correct in pointing out that our statement about pediatric orbital pseudotumor having a predilection to involve the left orbit is not statistically significant. At one time, it had been believed that the left eye is more often the site of metastatic carcinoma than the right, but a larger series disproved this contention 1 . We share with Dr Schechter an aversion to inoculating the ophthalmic literature with inadvertent half-truths or outright errors. We were encouraged to state that the left orbit appears to be more often involved in inflammatory pseudotumor than the right because we were aware of a similar trend that Henderson 2 discovered in his analysis of pseudotumor cases. He found 23 leftside and 11 right-side cases. By pooling our data and those of Henderson with the exclusion of bilateral cases, a two-tailed z test in the test of proportion yields a statistically

A patient with bilateral orbital pseudotumor and bilateral cavitary pulmonary lesions is reported. This patient was considered to have the limited form of Wegener's granulomatosis that has...

Objectives. To describe the clinical, laboratory, histopathological presentations and final diagnoses for children presenting to a tertiary paediatric rheumatology service with an inflammatory lesion of the orbit. Methods. This was a retrospective descriptive case series of children with an inflammatory lesion of the orbit presenting to a single paediatric rheumatology service between January 1999 and July 2010. Results. Ten patients, median age 11.5 (range 3.116.2) years at referral to the paediatric rheumatology department were identified; median duration of symptoms at referral was 9 (0.7517) months. Imaging was performed in 9/10 cases: orbital MRI (n = 4), orbital CT scan (n = 1), both MRI and CT scan (n = 4). All 10 patients had an orbital biopsy; 2 patients had repeat biopsies. The final diagnoses were granulomatosis with polyangiitis (Wegener’s) (n = 5; ANCA positive n = 4, ANCA negative n = 1), idiopathic orbital inflammation (n = 3), atypical mycobacterial infection (n = 1) and sarcoidosis (n = 1). Conclusion. Inflammatory mass lesion of the orbit is an unusual presentation in children. The differential diagnosis is wide and may evolve over time. Orbital biopsy and screening for systemic features is essential before treatment with CSs or other immunosuppressants to exclude malignancy, infection, vascular lesions, autoimmune conditions or other causes of orbital inflammation that can be associated with serious systemic manifestations.