The case of a nine-year-old white female who presented initially with idiopathic inflammatory pseudotumor and was ultimately diagnosed (22 months later) as having Wegener's granulomatosis is presented. [ncbi.nlm.nih.gov]

• Soft Tissue Swelling

  Presenting symptoms/signs included soft tissue swelling in 92% of orbits, pain in 92%, proptosis in 85%, and extraocular muscle dysfunction or ptosis in 69%. Decreased visual acuity was seen in only 19% of orbits. [ncbi.nlm.nih.gov]

  Enhancing inflammatory soft tissue swelling with ragged, 'fluffy' margins is seen. When the extra-ocular muscles are involved, the tendinous insertions are not spared. [ajnr.org]

  ESR: 25 Figure 1 : Patient on presentation with ptosis, proptosis, periorobital erythema and edema Figure 2 : On slit lamp exam, the patient had 360 degrees of chemosis; pharmacologically dilated pupil Figure 3: Axial CT of the orbits showing soft tissue [webeye.ophth.uiowa.edu]

  This revealed only soft tissue swelling in the anterior right orbit. The sinuses appeared normal. On day 11, a biopsy of the mass was obtained. [healio.com]

  tissue swelling improvement, 82% with improvement in proptosis, 78% with improved ocular motility restriction and 75% had decreased pain. [eyewiki.aao.org]

• Hypoxemia

  Four months after his first visit, the patient's condition was complicated by fever, general fatigue, and hypoxemia. A cell marker study of the swollen lymph node showed monoclonality, indicating the development of non-Hodgkin's lymphoma. [ncbi.nlm.nih.gov]

• Persistent Cough

  Chronic nasal discharge, obstruction, epistaxis, sinusitis, hemoptysis, and persistent cough are the most frequent initial complaints.13 Fever, constitutional symptoms, arthralgia, skin lesions, and neurological symptoms are also frequent, though not [healio.com]

• Chronic Cough

  Wegener's granulomatosis should always be considered as a diagnosis in children with pseudotumor of the orbit, especially those with sinusitis, chronic cough, epistaxis, anemia, or fever. [healio.com]

• Ejection Murmur

  /VI systolic ejection murmur was noted. Laboratory results included: BUN 55 mg/dl and creatinine 3.2 mg/dl; the urine was dark with too numerous to count RBCs and 2 protein. [healio.com]

• Bilateral Proptosis

  A 39-year-old woman developed bilateral proptosis, photophobia, and pain with extraocular movements over the course of 5 days. [ncbi.nlm.nih.gov]

  Eye examination showed bilateral proptosis [Figure 1] ; 35 mm in left and 33 mm in the right eye as measured by Hertel's exopthalmometer, conjunctival injection, and mild chemosis with mild exposure keratitis. [ojoonline.org]

• Periorbital Ecchymosis

  A 75-year-old male suffered sudden onset of retroorbital pain, visual loss, periorbital ecchymosis, and double vision without preceding trauma, paranasal sinus surgery, or infectious signs. [ncbi.nlm.nih.gov]

• Conjunctival Pallor

  Conjunctival pallor was noted. Laboratory studies demonstrated a hematocrit of 14.2% with a hemoglobin of 4.6 mg/dl. Red blood cell indices were mildly hypochromic and microcytic. [healio.com]

• Unilateral Eye Pain

  In the acute form of the disease, patients present with unilateral eye pain, lid swelling, proptosis, and occasionally vision loss. In the chronic form, patients present with diplopia. [ajnr.org]

• Migratory Polyarthritis

  We report a child with persistent fevers, arthritis, and parvoviral infection who subsequently developed unilateral orbital pseudotumor, lytic bone lesions, bilateral anterior uveitis, band keratopathy, and migratory polyarthritis. [ncbi.nlm.nih.gov]

• Loss of Initiative

  Three male and three female patients, aged 27 to 74 years, presented with a variety of ophthalmologic problems, including orbital swelling, chemosis, proptosis, blepharoptosis, restricted eye motion, diplopia, and visual loss. [ncbi.nlm.nih.gov]

• Anuria

  Dabbage S, Chevalier RL, Sturgill BC: Prolonged anuria and aortic insufficiency in a child with Wegener's granulomatosis. Clin Nephrol 1982; 17:155-159. 29. [healio.com]

• Cranial Nerve Involvement

  Specific factors to consider include the following: chronicity of illness, degree of pain, history of trauma or recent procedure, presence of visual changes, presence of proptosis, presence of fever, restriction of eye movement, cranial nerve involvement [emra.org]

This case report demonstrates the use of Ga-67 citrate imaging in the early diagnostic workup of this disorder. [ncbi.nlm.nih.gov]

The volume explores the impact of diagnostic workup, the role of surgery, the role of external beam radiation therapy, the use of radioactive plaques, photocoagulation, cryotherapy, and other less major surgical procedures. [books.google.it]

Otherwise, workup for sarcoidosis, Wegener's granulomatosis, neoplastic, and infectious causes was negative. Cases 3 and 4 developed ocular hypertension requiring topical treatment. [iovs.arvojournals.org]

• Anergy

  An anergy panel was unreactive. Serum immunoglobulins IgG, IgA, and IgE were slightly elevated. Chest x-ray demonstrated multiple peripheral nodules under three cm (ill-defined) without cavitation. [healio.com]

• Hyperglobulinemia

  Laboratory evidence of elevated erythrocyte sedimentation rate, anemia, leukocytosis, thrombocytosis, hyperglobulinemia, especially of the IgG and IgA is characteristic in Wegener's granulomatosis. [healio.com]

Treatment with steroids is usually successful early on, but long-term treatment-failure and recurrence rates are high. [ncbi.nlm.nih.gov]

For early diagnosis, better treatment, and good prognosis, it should be considered in patients presenting with ocular symptoms similar to the other vasculitides. [ncbi.nlm.nih.gov]

Idiopathic orbital inflammation (IOI), also known as orbital pseudotumor, is a nonspecific orbital inflammation of unknown etiology. [ncbi.nlm.nih.gov]

Epidemiology: No particular predilection for age, sex or race. Most likely seen in middle-aged individuals. [oculoplastic-eyelid-orbit-surgery.com]

EPIDEMIOLOGY Rare: No gender/ethnicity preferences SIGNS Conjunctivitis Uveitis (anterior/posterior) Episcleritis Scleritis (anterior/posterior) Cranial nerve palsies Ptosis Proptosis Periocular/lid/orbital edema Periorbital erythema Retrobulbar neuritis [webeye.ophth.uiowa.edu]

This review summarizes recent findings regarding the epidemiology, diagnosis, pathophysiology, and treatment of idiopathic orbital myositis. [ncbi.nlm.nih.gov]

References Mohd Hanafiah K, et al: Global epidemiology of hepatitis C virus infection: new estimates of age-specific antibody to HCV seroprevalence. Hepatology 2013;57: 1333–1342. Seeff LB: Natural history of chronic hepatitis C. [karger.com]

The pathophysiology of this entity was investigated using magnetic resonant imaging (MRI) and ultrasound biomicroscopy (UBM). Clinical features, anterior chamber angle configuration, and intraocular pressure. [ncbi.nlm.nih.gov]

Diagnosis may be confirmed with a biopsy referred to as an orbitotomy. 2 However, if suspicion is high enough, response to corticosteroids can also be used to confirm a diagnosis without the need for biopsy. 2 Pathophysiology Although the pathophysiology [emra.org]

In our opinion, a possible pathophysiological explanation of the observed findings may take origin from the compression of the loco-regional venous system, due to the meningeal extension of the pseudotumor, even if small, as demonstrated by the impaired [bmcneurol.biomedcentral.com]

Pathophysiology The etiology and pathogenesis of NSOI is currently unknown. Both infectious and immune-mediate etiologies have been implicated. [eyewiki.aao.org]

It can also be used to see if your treatments are successful at improving your vision or preventing further vision loss. [themedicaleyecenter.com]

Eye cancer specialists must reduce the steroid medication very slowly in order to prevent recurrence (return) of the disease. [eyecancer.com]

Electronic address: [email protected] 2 Casey Eye Institute, Oregon Health & Science University, Portland, OR 97239, USA; Department of Medicine, Oregon Health & Science University, Portland, OR 97239, USA; Department of Public Health and Preventive [ncbi.nlm.nih.gov]

Prompt recognition and treatment by an ophthalmologist is needed to prevent complications. [journals.aace.com]

IF not, and if his vision is stable at the stated 20/25 level, I would probably consider another observation along with another form of chemotherapy or anti-metabolite treatment to prevent future recurrence. [eyecancercure.com]