Orbital Pseudotumor Masquerading as Orbital Cellulitis

Anales de Pediatría

The purpose of this study was to review our clinical experience with 132 idiopathic orbital inflammation cases seen between 1971 and 1994. Clinical charts of the patients were evaluated retrospectively. Patient age ranged from 5 to 80 (mean 46.5) years. The follow-up period was between 6 to 172 (mean 42.8) months. Proptosis (82%), motility restriction (54%), visual acuity loss (38%) were the three more common presenting signs. The diagnosis was made by open biopsy in 96 cases and clinically in 36 cases. Radiologic patterns of involvement were available for 84 cases. Diffuse inflammatory disease (40 cases) was the most frequent radiologic pattern followed by myositis (21 cases) and dacryoadenitis (14 cases). Focal encapsulated mass (5 cases), Tolosa-Hunt syndrome (2 cases) perineuritis (1 case) and periscleritis (1 case) were the other types of orbital pseudotumors. The response to treatment was known for 60 of the 84 radiologically classified cases. High-dose oral corticosteroid treatment was successful in 35 out of 60 (58.3%) cases and radiotherapy in 9 out of 14 (64.3%) cases resistant to corticosteroids. One resistant case responded to cyclophosphamide and 2 cases with focal mass lesions were treated with orbitotomy. Four additional cases had spontaneous remission. Overall, 51 out of 64 patients (79.7%) had an eventual good outcome. In conclusion, we found open biopsy to be a safe and reliable procedure in orbital pseudotumors with the exclusion of acute phase cases. The success rate of treatment for this group of orbital disorders is high with the exception of certain disease patterns.

Asian Pacific journal of allergy and immunology / launched by the Allergy and Immunology Society of Thailand, 2007

To describe the clinical characteristics of orbital pseudotumor, a retrospective analysis was performed on patients with orbital pseudotumor at Siriraj Hospital for ten years. Forty-nine patients (24 males and 25 females; 62 eyes) with a mean age of 43.75 years were included (a mean follow-up of 25 months). Thirty-six patients (73.5%) had unilateral disease. The clinical features were proptosis (79.6%), ocular motor deficit (61.2%), pain (51%), lid swelling or lid mass (44.9%), ptosis (24.5%), and chemosis (18.4%). The most common presenting sign was proptosis (49%). All were treated with corticosteroids with clinical improvement in 40 (81.6%) patients. Ten (83.3%) of 12 patients with visual loss improved with mean recovery time of 10.3 days. Ocular motility recovered in 24 (80%) patients, occurring an average of 17.8 days after initiation of therapy. It is concluded that the clinical features of orbital pseudotumor are varied. Most patients were improved with corticosteroids treatm...

Clinical Radiology, 2011

Idiopathic orbital pseudotumour (IOP) is a benign inflammatory condition usually confined to the orbit. This may involve single or multiple intraorbital structures. Extraorbital extension can also occur. The imaging appearances often mimic other orbital diseases. Both computed tomography (CT) and magnetic resonance imaging (MRI) are frequently used to investigate orbital diseases, and it is important for radiologists to be aware of the variety of imaging appearances that occur in IOP. We present the imaging appearances in histopathologically confirmed cases of IOP and discuss the clinical features, natural history, and differential diagnosis of this condition.

Ocular Oncology and Pathology, 2020

Introduction: A masquerade syndrome is an atypical presentation of a neoplastic process that mimics an inflammatory condition. In this paper, we focus on orbital pseudocellulitis. Case Series: Our case series includes 5 retinoblastoma patients with orbital pseudocellulitis at presentation. In 3 patients the disease was bilateral, in 1 trilateral, and in 1 unilateral. The eyes with pseudocellulitis were enucleated, while the fellow eyes were treated conservatively, when affected. Four patients responded well to the therapy and showed remission of the tumor. The patient with trilateral retinoblastoma did not respond to therapy and died of disease. Discussion: Differential diagnosis with infectious orbital cellulitis is extremely important. Patients with orbital cellulitis present with fever, sinusitis, leukocytosis, and raised inflammatory markers, while ophthalmoscopic examination is negative and imaging studies show sinus involvement. On the contrary, patients with retinoblastoma do not show systemic inflammation, while ophthalmoscopic examination reveals leukocoria, buphthalmos, and an intraocular tumor mass associated with retinal detachment. Magnetic resonance imaging shows intralesional calcifications and soft tissue edema without sinus involvement. Histology confirms the diagnosis. Conclusions: Medical history, physical examination, and imaging studies are crucial in the diagnosis of retinoblastoma-associated orbital pseudocellulitis. Retinoblastoma should be excluded in all patients with signs of pre-septal orbital cellulitis through fundoscopy and/or imaging studies

2015

A twenty-two year old female patient presented with new onset bilateral hard orbital masses and progressively worse tear lake problems. Computed tomography of the orbits revealed poorly differentiated bilateral orbital masses. Laboratory investigation revealed ANCA positivity. Routine biochemical investigations were all within normal limits. CXR was also normal. Biopsy of the orbital masses revealed nonspecific histological findings. An initial diagnosis of Granulomatosis with Polyangitis (GPA) was postulated. Oral steroids were given followed by a rapid response to steroid therapy. The working diagnosis of GPA was abandoned and a diagnosis of idiopathic orbital inflammation (IOI), or orbital pseudotumour was made owing to the benign, non-infective, inflammatory pathology with no evident systemic or local cause. Tailoring off of steroids resulted in repeated flare ups, resulting in the initiation of methotrexate therapy. The patient is in remission and is currently on combined steroid and methotrexate treatment. IOI is a diagnosis of exclusion and a rapid response to steroids serves as a diagnostic aid but is not in itself diagnostic.

Otorhinolaryngology Clinics - An International Journal, 2014

Objective: To present management dilemma in a challenging case of a benign orbitomaxillary pseudotumor and its impor tance of inclusion in the differential diagnosis of orbital disorders.

Clinical and Refractive Optometry

The purpose of this paper is to provide an overview of the diagnosis and management of orbital pseudotumor. Orbital pseudotumor is a benign, non-infective, protean clinical syndrome of unknown etiology characterized by inflammation of the orbit. Patients with orbital pseudotumor often present with redness, edema, or pain. More serious manifestations such as proptosis, motility limitations, optic nerve compression, and even visual loss, suggest the presence of a spaceoccupying or infiltrative lesion. Orbital pseudotumor is considered a diagnosis of exclusion. A complete evaluation includes careful history taking, detailed physical examination, neuroimaging, biopsy, and hematologic testing. After an initial biopsy, when feasible, patients are first treated with non-steroidal anti-inflammatory drugs (NSAIDs) and/or corticosteroids or radiation. When this is inadequate, patients may consider other options such as chemotherapy, immunosuppressants, or surgery. The goal of treatment is to ensure proper symptom control and to optimally maintain orbital structure and function.

Case Reports in Ophthalmological Medicine

Background. Orbital lymphomas are primarily non-Hodgkin type and can originate from the eyelids, extraocular muscles, soft tissue orbital adnexa, conjunctiva, or lacrimal glands. Orbital malignancies often represent a diagnostic dilemma for clinicians given their varying and atypical presentations. Objective. To report a case of orbital lymphoma mimicking orbital cellulitis. Case. A 66-year-old male patient presented with sudden onset of painful proptosis with visual impairment in the left eye for 15 days. On ocular examination, best-corrected visual acuity was 6/12 in the right eye and 2/60 in the left eye, abaxial proptosis with hypertropia, swollen and erythematous eyelids, restricted extraocular movement in all cardinal position of gaze, conjunctival congestion with chemosis and tortuous vessels, sluggish pupillary reaction, and chorioretinal folds in the inferior quadrants. The case was diagnosed as left eye orbital cellulitis, and the patient was treated with broad-spectrum in...

IP Innovative Publication Pvt. Ltd., 2019

Introduction: Infection in the orbit and the periorbital tissues are particularly important subsets of inflammatory diseases not only because of frequency of presentation but also because of life threatening conditions which demand prompt, specific and therapeutic management. Of the orbital inflammation with infections most common aetiological factors encountered, are due to bacteria. Materials and Methods: All patients with orbital signs and symptoms of inflammatory diseases of infectious origin were selected. Progression, symptoms, history of associated symptoms such as headache, fever, allergy etc. asked for. History of trauma, immunization and systemic illness, endocrinological disorders, infections of any systems and any dermatological disorders were taken into account. Local examination, ENT examination, laboratory and radiological investigations were done in all cases. Results: Incidence of orbital infection is 0.34%. Male preponderance and maximum number of patients were adult. The maximum aetiological factor of orbital infection being due to infections of the ocular adnexae, sinusitis, dacryocystitis, post-surgical, and trauma. Raised IOP in 24%, proptosis in 34%, restricted ocular motility in 42%, mechanical ptosis in 70%, NLD block in 18%, Corneal oedema or opacity in 14% and inflammation of ocular adnexae in almost all cases. In majority of the cases the organism responsible is Pseudomonas aeuroginosa, then staphylococcus aureus. Conclusion: Orbital infection is sight threatening and the pathognomic features and virulence of the causative organism is unpredictable. Early presentation appropriate diagnosis and judicious protocol if followed at an early stage would halt the morbidity and fatality. Keywords: Clinicopathology, Infectious origin, Orbital cellulitis, Orbital inflammation, Preseptal cellulitis

Common Eye Infections, 2013

nent loss of vision to the delay in diagnosis and intervention. Further, there were 9 cases of intracranial orbital abscess extension that required either extended treatment with systemic antibiotics alone or in combination with neurosurgical intervention. Patients with orbital cellulitis may present with signs of eyelid swelling, conjunctival chemosis, diplopia and proptosis which may not be prominent in cases of preseptal cellulitis. [1], [8], These patients may present with corneal infections resulting from exposure keratopathy due to their inability to close their eyes. Many of these patients come with local symptoms in the form of eyelid edema, redness, chemosis, decreased ocular motility and proptosis (Figure ). Patients having superficial signs of swelling (preseptal cellulitis) should be differentiated from deeper infection resulting in orbital cellulitis, in which case, signs and symptoms resulting from inflammation may be helpful. In particular, external ophthalmoloplegia, proptosis and decreased visual acuity are associated with orbital cellulitis rather than preseptal cellulitis. [8], [9] Temperature greater than 37.5℃ and leukocytosis resulting in fever may be more prominent feature of the pediatric orbital cellulitis. [4], In children, external ophthalmoplegia and proptosis may be the most common features, while decreased visual acuity and chemosis may be less frequent signs in both the pediatric as well as in the adult patients. In cases of the optic nerve involvement, disc edema or neuritis with rapidly progressing atrophy resulting in blindness may occur. Mechanical pressure on the optic nerve and possibly compression of the central retinal and other feeding arteries results in optic nerve atrophy. [10] Also orbital inflammation itself may spread directly into the substance of the optic nerve causing small necrotic areas or abscesses. [2] Compression of the feeding vessels as well as inflammation may result in the infarction of the optic nerve, infarction of the sclera, choroids as well as the retina. Inflammation may result in septic uveitis, iridocyclitis or choroiditis with a cloudiness of the vitreous, including septic pan ophthalmitis. A less common complication of orbital cellulitis is glaucoma that can cause decreased vision, reduced visual field or even enlarged blind spot on presentation. On occasion, one may not find any fundus abnormalities. Among our patients presenting to a tertiary eye care center in the developing country, presenting signs of 218 patients with orbital cellulitis included, eyelid swelling in 71.5%, proptosis in 68.3%, motility restriction in 59.2%, pain in 52.3%, and decreased visual acuity in 14.2% of cases. [8] Some of the differential diagnosis for patients presenting with orbital cellulitis may include, allergic reaction to topical or systemic medication, edema from hypo-proteinemia due to variety of systemic causes, orbital wall infarction and subperiosteal hematoma due to unrecognized trauma or due to blood coagulation disorders. Differential diagnosis may also include orbital pseudotumor (Figure ), retinoblastoma, metastatic carcinoma and unilateral or Diagnosis and Management of Orbital Cellulitis

Saudi Journal of Ophthalmology, 2012

Idiopathic orbital inflammation, also known as orbital pseudotumor, describes a spectrum of idiopathic, non-neoplastic, non-infectious, space-occupying orbital lesions without identifiable local or systemic cause. The condition occurs mainly in young adults who may present with acute pain, proptosis, chemosis and limitations of extraocular movements. Decreased vision due to optic nerve infiltration and macular edema as a result of scleritis is less common sequel of orbital pseudotumor. Herein, we present a case of unilateral orbital pseudotumor in a young male who presented with chief complaints of painful decreased vision which was attributed to optic neuritis and macular edema as a result of scleritis. Imaging studies were helpful in the establishment of the correct diagnosis and patient's complaints improved with administration of systemic corticosteroids.

Survey of ophthalmology

We have reviewed the literature in order to delineate the clinicopathologic definition of orbital pseudotumor, also called idiopathic nonspecific orbital inflammation. The clinical picture of orbital pseudotumor varies widely, with signs of mass effect, inflammation and/or infiltration. On computed tomography, orbital pseudotumor presents as a unilateral focal or diffuse mass. The histopathologic hallmark of orbital pseudotumor is a mixed inflammatory infiltrate with fibrosis of varying degree. Contrary to an old belief, orbital pseudotumor is not related to orbital reactive lymphoid hyperplasia (pseudolymphoma) and is not a lymphoid tumor. Atypical histopathologic findings of orbital pseudotumor include dominant sclerosis, granulomatous inflammation, vasculitis, and tissue eosinophilia. In the absence of systemic fibroinflammatory, granulomatous, and vasculitic disease, these atypical histopathologic patterns can be considered to represent subclasses of orbital pseudotumors rather ...

International Journal of Research in Medical Sciences

Orbital pseudotumor is a non-granulomatous inflammation of orbital soft tissue for unknown etiology. In the first case, a 35-year-old female presented with bilateral painless upper eyelid swelling since 3years.No proptosis, no extra ocular movements restriction of both eyes was noted. Best corrected visual acuity both eye: 6/6, N6. USG B-Scan and CECT revealed bilateral lacrimal gland enlargement with bulky muscles and tendons. USG guided FNAC confirmed pseudotumor which showed prompt response to steroids but recurred after 1month of stopping of steroid therapy. In the second Case, a 15year old female presented with unilateral proptosis left eye for 3 months with painful extraocular movements and restricted in all gazes. Best corrected visual acuity in L/E - 6/12, N6, R/E-6/9, N6. USG B-scan and CECT revealed soft tissue lesion encasing Optic nerve. CEMR revealed bulky lacrimal gland, muscles and lesion extending up to orbital apex. Prompt response to steroid with no recurrence till...

British Journal of Ophthalmology, 2001

American Journal of Ophthalmology Case Reports, 2019

Differentiating idiopathic sclerosing orbital inflammation from orbital inflammation secondary to neoplasia may be challenging, as both processes can present similarly. Neoplasms in the orbit may induce inflammation with accompanying fibrosis. Limited sections of histopathological specimens may demonstrate nonspecific inflammation and lead to an inaccurate diagnosis. Observations: The authors present a case of infiltrating adenocarcinoma of the orbit with mucinous features which was misdiagnosed as idiopathic sclerosing orbital inflammation due to three separate benign biopsy specimens. Conclusions and Importance: The ophthalmologist must remain suspicious of malignancy in cases of suspected idiopathic orbital inflammation with an atypical clinical course, regardless of apparently benign biopsy results.