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Year : 2000  |  Volume : 10  |  Issue : 3  |  Page : 161-164
Case report: Sclerosing orbital inflammatory syndrome with intracranial extension


Dept of Ophthalmology and Radiology, Kamalnayan Bajaj Hospital, Marathwada Medical & Research Institute, Aurangabad, India

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Keywords: orbit, sclerosing orbital inflammatory syndrome, intracranial extension, CT

How to cite this article:
Nalgirkar A R, Bhagwat R B, Deshpande S R, Sharma R B, Ekbote S S. Case report: Sclerosing orbital inflammatory syndrome with intracranial extension. Indian J Radiol Imaging 2000;10:161-4

How to cite this URL:
Nalgirkar A R, Bhagwat R B, Deshpande S R, Sharma R B, Ekbote S S. Case report: Sclerosing orbital inflammatory syndrome with intracranial extension. Indian J Radiol Imaging [serial online] 2000 [cited 2020 Jan 25];10:161-4. Available from: http://www.ijri.org/text.asp?2000/10/3/161/30603
Orbital inflammatory syndrome termed commonly as pseudotumor is characterized by idiopathic inflammation of the orbital tissues, affecting adults as well as children and is unrelated to thyroid related orbithopathy or other systemic disorders. Patients with orbital inflammatory syndrome typically present with onset of orbital pain, restricted movement, diplopia, proptosis and impaired vision with optic nerve involvement. Headche, vomiting and fever are commonly seen in children [1].

However, not all patients with orbital inflammatory syndrome typically present with pain. Some may present late in the course of disease, without any proptosis with a totally or partially scarred lesion, which is termed as sclerosing orbital inflammatory syndrome'. The lesions of sclerosing orbital inflammatory syndrome are usually present within the retrobulbar fat pad or a single muscle[2]

We are presenting here a case of sclerosing orbital inflammatory syndrome with an intracranially extending lesion.


   Case Report Top


A thirty-five-years old man was admitted to the hospital in April 1993, with complaints of right-sided headache accompanied by nausea and vomiting since 1987. He had history of headaches on and off, without regularity, accompanied always by nausea and sometimes by vomiting.

In the developed collapse of the nasal bridge, spontaneously without any apparent cause. He underwent a bone grafting procedure, which was not successful in correcting the saddle nose. He had left hemiplegia, one month following this for which he was treated.

The headache started in the middle of 1987, initially around the right eyeball and around the temporal area, which later extended to the whole right side of the head. An ophthalmologist was consulted and he was prescribed glasses for constant use, the details of which were not available.

In 1988, he had another bone grafting procedure done for the saddle nose, which was unsuccessful in correcting the deformity. On getting repeated exacerbations of headache and vomiting he consulted a neurologist and was advised a CT of the brain, which was performed in March 1990. The scan reported an old right temporo­parietal infarct with no other abnormality. The CT of the brain was repeated in March 1991 and revealed the same findings.

The patient came for consultation to our hospital for severe right-sided headache and vomiting in April 1993 and was referred for an ophthalmic examination.

He had a vision of OD 20/20 (with +1.0 D cylinder in 120 degrees) OS 20/20, with normal anterior segment findings. Examination of the posterior segment under mydriasis revealed no abnormality. Examination of the external eye showed an enlarged interpalpebral fissure on the right side with a deep inferior lid sulcus. The right eye interpalpabral fissure was 16mm whereas the left eye was 12mm. The right eye lower lid margin was 4mm from the inferior limbus with the left eye's lower lid margin touching the inferior limbus. Exophthalmometry showed the distance of the corneal plane from the lateral orbital margin in the right eye to be 21 mm with a 2mm superior displacement of the eyeball and 19mm in the left eye. On palpation of the orbital rims no abnormality was noted. On laboratory investigations of the blood and cerebrospinal fluid, he had a positive VDRL and also mild hypochromic anemia. Other values were within normal limits.

A CT scan of the orbit was performed which showed an infiltrative lesion in the right anterior inferomedial orbit extending posteriorly to the parasellar region [Figure - 1]. The lesion enveloped the inferior oblique, inferior rectus, and medial rectus muscles and was in proximity to the optic nerve without involving it [Figure - 2]. The lesion enhanced with contrast injection. The other muscles and the sclero­uveal rim appeared well within normal limits. The left orbit and eyeball showed normal anatomy.

On revaluation of the orbit in the previous CT scans, which were primarily intended for the brain, an infiltrative lesion could be seen in the anterior inferomedial part of the orbit. The lesion appeared to be limited to the orbit without any evidence of intracranial extension of the lesion.

A syphilitic granuloma of the orbit was suspected and a regimen of procaine penicillin, 8 lakh units per day IM for 15 days was prescribed, with symptomatic treatment for headache and vomiting. This failed to show any response over two months.

A fine needle aspiration biopsy was done which showed fibrous tissue bits and was inconclusive. A biopsy was then done by the anterior transconjuctival approach. On gross examination the inferior and medial recti, inferior oblique and the inferomedial eyeball seemed to be enveloped in a fibrous membrane of uneven thickness. Biopsy was obtained from the anteroinferior and inferomedial aspects of the lesion. The biopsy showed extensive areas of fibrosis with infiltration by lymphocytes and plasma cells. A diagnosis of sclerosing orbital inflammatory syndrome was considered and intralesional, steroid injection was given which relieved the symptoms within a week. The patient then was then started on oral prednisolone 60mg/day and was followed up on a weekly basis with gradual tapering of the steroids. On a follow-up CT obtained two months after starting steroid therapy resolution of the extracranial part was seen. On tapering and stopping steroids over a six-month period, the patient had a relapse with reappearance of symptoms, and oral steroids were restarted, tapered and he was kept on the least alternate day dosage.

The patient was not willing for surgical removal after explanation of the risks involved and was continued on low dose steroids and monthly follow-up. He had another attack of left hemiparesis in April 1995 for which he was hospitalized. He underwent MR, which revealed resolution of a part of the orbital tumor and involvement of the right cavernous sinus with a block of the right carotid artery [Figure - 3].

He is presently on low dose steroids and followedup a monthly basis.


   Discussion Top


Pseudotumor is a restricted category of non-specific inflammation of the orbit without identifiable cause. This definition therefore excludes specific infections, granulomatous diseases and collagen-vascular disorders.

Thus inflammations caused by foreign bodies, amyloid, parasites, bacteria, fungi, ruptured dermoid cysts, Graves disease, sarcoid, tuberculosis, syphilis and well defined vasculitideas such as polyarteritis nodosa and Wegener's granulomatosis should be recognized as such and not included as part of the syndrome of idiopathic inflammatory pseudotumor.

The clinical picture of pseudotumor varies widely with single or combined signs of mass effect, inflammation and infiltration. The histopathological hallmark is a mixed inflammatory exudate with fibrosis of varying degrees. Contary an old belief, orbital pseudotumor is not related to orbital reactive lymphoid hyperplasia (pseudolymphoma) and is not a lymphoid tumor.

Atypical histopathological findings of orbital pseudotumor include dominant sclerosis, granulomatous inflammation, vasculities, and tissue eosinophilia. In the absence of systemic fibro-inflammatory, granulomatous, and vasculitic disease, these atypical histopathological patterns can be considered to represent subclasses of orbital pseudotumors rather than distinct entities. Clinical and prognostic characteristics of both histopathologically classical and atypical orbital pseudotumors appear to be heterogeneous.

On ultrasound the pseudotumors give an appearance like any lesion having closely packed cells in orbital tissue. On USG, lesions are diffuse with an ill-defined shape or can be nodular & localized to one area. These have a regular acoustic structure and a low internal reflectivity (10% to 40%) because of the absence of interfaces within the cellular infiltrates. Sound attenuation is minimal. Borders are well defined if the lesion is nodular and poorly defined it the lesion is diffuse.

On CT, the pseudotumor reveals a broad range of pathological changes. Although the findings may be suggestive of idiopathic pseudotumors, they are by no means specific. It may appear as an isolated discrete with well-defined borders resembling any neoplasm or specific granuloma. The inflammatory changes may remain localized to part of the region of the globe resulting in an apparent thickening of the scleral margin. In such cases ultrasound examination may demonstrate an effusion in Tenon's space. A similar picture is seen in lymphomas and differentiation between the two conditions may be impossible. Myositic pseudotumor can mimic Grave's disease but in pseudotumor the involvement is less regular and is unilateral.

Infiltrative changes in the adjacent fat, associated periocular involvement or a discrete separate mass together with thickened muscles favors the diagnosis of pseudotumor. Varying degrees of contrast enhancement of the abnormal soft tissue is seen in most cases of pseudotumors.

On MRI, these lesions are usually hypointense on T2W images and shows homogeneous contrast enhancement. The lack of mobile protons due to the fibrotic background and high cellularity of the lesions may be the reason for their hypointensity and weaker enhancement on MR images.

Systemic corticosteroids are an established mode of treatment for pseudotumors, but recurrent and refractory tumors have been noted. In a study by Mombaerts et al [3] they found a moderate response, high recurrence and low cure rate in patients with orbital pseudotumors after treatment with systemic steroids, but noticed better prognosis in patients with pseudotumors involving the optic nerve. Low dose radiation and immunosupressants are of some value in refractory cases and may be utilized prior to surgical interventions in cases with extensive involvement.

In this case the initial suspicion of syphilitic granuloma was based on the strongly positive VDRL tests of the blood and CSF, appearance of the infiltrative lesion on CT which was enhancing with contrast injection, and the evidence of gumma formation elsewhere in the body (saddle nose). Furthermore evidence of a temporo-parietal infarct in a 32-year-old was suggestive of a pathology which retrospectively could be inferred to be syphilitic endarteritis. Though syphilitic granuloma of the orbit is rare, due to the strong clinical evidence, the patient was started on penicillin. Syphilitic granulomas and symptomatic neuro-syphilitic patients are known to respond rapidly to penicillin [4]. As there was no improvement in the patient symptomatically or clinically in the two months period after the penicillin course, the possibilities of sclerosing orbital inflammatory syndrome and orbital fibromatosis were considered. The patient was given an intralesional steroid injection and systemic oral steroids to which the patient responded objectively as well as symptomatically within a month of starting the therapy. Fibromatosis may present in a similar manner [4,5] and the diagnosis, necessarily histopathological is not possible without an excisional biopsy. Such a dramatic response to steroids is rarely if ever, seen in fibromatosis.

The infiltration and the extensively fibrotic lesion suggest that the inflammatory process can be limited to a focus of the lesion when other parts of the lesion have healed with a process of extensive fibrosis [6]. In such a case incisional or fine needle aspiration biopsy might reach the lesion, but may not demonstrate the typical appearance of orbital Inflammatory syndrome and even the histopathological diagnosis might become difficult.

Exclusion of other etiologies with systemic and radiological investigations, appearance of the lesion on CT, evidence of extensive fibrosis on histopathology and response to steroids [7] remain the prime criteria for arriving at the diagnosis of a sclerosing orbital inflammatory syndrome in which the lesion can extend intracranially.


   Acknowledgements Top


This report has been supported by Kamalnayan Bajaj Hospital, Marathwada Medical & Research Institute, Dr. Rajendra Prasad Marg, Aurangabad.

 
   References Top

1.Charvis RM, Garner A, Wright JEL. Inflammatory orbital Pseudotumour. Arch Ophthalmology 1978; 96:1817-1822.  Back to cited text no. 1    
2.Holmes KK. Spirocheatal diseases. In: Petersdorf RG, Harrisons Principles Of Internal Medicine, Vol-1. New York: McGraw Hill, 1983: 1134.  Back to cited text no. 2    
3.Mombaerts I, Schlingemann RO; Goldschmeding R; Koornneef L. Are systemic corticosteroids useful in the management of orbital pseudotumours? Ophthalmology, 1996; 103: 521-8  Back to cited text no. 3    
4.Henderson JW, Fatrow GM. Orbital tumours, 2 ndsub ed. Philadelphia : W B Saunders, 1980.  Back to cited text no. 4    
5.Idose 0 et al. Penicillin in the treatment of syphilis, Bulletin WHO: 47 (supplement). 1972: 1.  Back to cited text no. 5    
6.Leone CR, Lloyd WC. Treatment protocol for orbital inflammatory disease. Ophthalmology 1985; 9213-25.  Back to cited text no. 6    
7.Kennerdell JS, Dresner SC. The nonspecific orbital inflammatory syndromes. Surv.Ophthalmology 1984; 2993-103.  Back to cited text no. 7    

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Correspondence Address:
A R Nalgirkar
8, Adarsh Nagar, Aurangabad 431 005
India
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[Figure - 1], [Figure - 2], [Figure - 3]



 

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