Ординатура / Офтальмология / Английские материалы / Surgical Atlas of Orbital Diseases_Mallajosyula_2009

the morbidity and mortality of selected cases are reduced with prompt and appropriate antimicrobial therapy. Keeping in mind this fact we find it appropriate to present the following case reports:

CASE ILLUSTRATIONS

Case 1

A 25-year-old systemically healthy male patient presented with complaints of severe photophobia, redness, discharge, pain and severe swelling of the lids in the left eye, since 2 days. Past history was significant for a boil on the lower eyelid, 2 days ago. On examination, the best-corrected visual acuity was 6/6 and 6/9, in the right and left eyes respectively. Right eye examination was unremarkable. The left eye showed severe lid edema with scales on the skin and was diagnosed as having left sided periocular necrotising fascitis with associated keratoconjunctivitis (Figure 7.1A). The cornea showed multiple marginal infiltrates. Photographic documentation of the anterior segment condition was impossible because of the severe photophobia. Extraocular movements were full. A conjunctival swab and a periorbital skin swab were sent for culture and sensitivity. The corneal infiltrates were also cultured on Blood agar and Sabouraud's dextrose agar. The patient was seen by our infectious diseases expert and started on intravenous coamoxiclav 1 gram twice daily, intravenous ceftriaxone 1 gram twice daily and oral metronidazole 500 mg three times daily, pending sensitivity reports. Topical Lotepred eye drops every 3 hourly and ciprofloxacin (0.3%) eye drops 6 times a day were started, in the left eye.

On follow-up 2 days later, the patient was symptomatically much better. The skin scabs had fallen off, revealing violaceous, sub-epidermal necrosis. The conjunctival inflammation had reduced and the corneal marginal infiltrates had almost disappeared (Figure 7.1B). Culture and sensitivity results showed Staphylococcus aureus, sensitive to the administered medications. The culture plates for corneal infiltrates showed negative growth and were discarded after 3 weeks. The patient was seronegative for HIV.

Five days later, the skin lesions had healed and the conjunctivitis had resolved. Intravenous

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antibiotics were stopped and the patient was started on oral antibiotics for a week.

On final follow-up a month later, periocular skin discoloration was the only sequalae noted (Figure 7.2).

Discussion

Necrotising fascitis (NF) is a serious life threatening condition, with reported mortality of more than 20%. The limbs, perineum and abdomen are frequently involved with facial involvement being rarely involved. The organisms most closely linked to NF are Group A beta-hemolytic streptococci, though these bacteria are isolated in only a minority of the cases.20

Figure 7.1A: Periocular necrotising fasciitis: External photograph of patient at presentation, showing left sided severe lid edema, erythema and necrotic tissue with overlying skin scabs

Figure 7.1B: Periocular necrotising fasciitis: Slit lamp photograph of the left eye, in diffuse illumination (with upper eyelid retracted), showing periocular skin erythema and kerato-conjunctivitis

Figure 7.2: Periocular necrotising fasciitis: External photograph of patient, a month post presentation, showing healed skin lesions, with symmetrical palpebral apertures

Periocular NF is reported to have a better prognosis.22 Though reports of resolution of periocular NF postsurgical debridement are common, a detailed Medline and Embase search revealed only one case report which was reported to resolve with conservative management.23 Infections in the periocular region occur postsurgical procedures, post trauma, postfurunculosis or even without any antecedent cause.23

Ideally, a combination of intensive parenteral antimicrobial therapy and prompt surgical debridement of necrotic tissue should be performed. Intravenous pooled immunoglobulin and heparinisation may also have beneficial roles by neutralising super-antigen activity and aiding antibiotic perfusion.24 Mild cases especially those restricted to the eyelids alone may respond to medical therapy.23

We report the case of a 25-year-old male patient who presented with periocular NF associated with keratoconjunctivitis. We report this case to highlight the successful conservative management of peri-

ocular NF and the hitherto unreported anterior segment involvement.

Case 2

A 60-year-old systemically healthy female was referred with a 2-month history of irritation in the left eye associated with a mass in the lower lateral orbit. Her visual acuity was 20/20 in both eyes. The right eye was unremarkable. The left eye was 2 mm enophthalmic by Hertels exophthalmometry but showed no displacement (Figure 7.3A). Ocular motility was full in range. There was a nontender, hard, lobulated mass measuring about 30 mm × 10 mm in size in the inferior orbit, felt separate from the inferior orbital rim. The mass appeared to be contiguous with the globe. General physical examination including chest roentgenogram was unremarkable.

Computed tomography scan showed an illdefined extraconal anterior orbital mass located inferotemporal in the left orbit, with minimal contrast enhancement (Figure 7.3B). Differential diagnoses of scirrhous breast cancer metastasis or sclerosing orbital pseudotumor were considered and a biopsy was performed by the inferior conjunctival fornicial approach.

Histopathology revealed features of noncaseating granulomatous inflammation and fibrosis (Figure 7.4A). AFB was negative both by smear and culture. PCR for Mycobacterium tuberculosis DNA was positive (Figure 7.4B). Orbital tuberculosis was diagnosed and the patient received four-drug combination antitubercular therapy for 6 months. The orbital mass completely regressed and there was no local recurrence at two years.

Figures 7.4A and B: Orbital tuberculosis manifesting with enophthalmos: Histopathology of the orbital mass showing features of non-caseating granulomatous inflammation and fibrosis

(hematoxylin and eosin, x 500) (left) and polymerase chain reaction of the specimen positive for mycobacterium tuberculosis deoxyribonucleic acid (right)

Discussion

Orbital tuberculosis is rare, even in endemic countries.25 Erosion of a parenchymal pulmonary tuberculous focus in a blood or a lymph vessel may lead to dissemination of the organism; with systemic involvement.26 Orbital tuberculosis may involve orbital soft tissues, lacrimal gland, periosteum, and bones, and may extend to a contiguous paranasal sinus or intracranial cavity.25, 27 The disease course is generally slow and indolent.25,27 Clinical manifestations include orbital tuberculoma and cold abscess presenting with proptosis, and orbital osteomyelitis manifesting with discharging sinus and inflammation.25, 27 Orbital tuberculosis presents with proptosis and is not known to present with enophthalmos.28

Common manifestations of orbital tuberculosis are proptosis or discharging sinus. Our patient, however, presented with an orbital mass and enophthalmos. Orbital tuberculosis presents with proptosis and is not known to present with enophthalmos.25,27 Herein we report a case of orbital tuberculosis presenting with an orbital mass and paradoxical enophthalmos.

Case 3

A 55-year-old systemically normal female patient presented to us with right-sided sudden onset severe proptosis, pain and dimunition of vision, of 2 days duration. Past history was significant for a boil on the right upper eyelid, 2 days prior. The patient was on oral amoxicillin 750 mg three times daily, at presentation.

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On examination, the visual acuity was perception of light with inaccurate projection and 6/9 in the right and left eyes respectively. The right eye was severely proptotic, with greatly increased resistance to retropulsion. There was an upper lid wound, with purulent drainage. The bulbar conjunctiva was severely chemosed, making the remaining slitlamp examination difficult (Figure 7.5A). Extraocular movements were severely limited in all directions of gaze. Left eye examination was unremarkable.

The patient was mildly febrile. Remaining systemic examination was unremarkable. An orbital Computed Tomography (CT) scan, routine blood investigations and culture and urine examination and culture were ordered. The blood and urine examination reports were unremarkable, except for mild leucocytosis. The CT scan showed a superior orbital mass suggestive of an abscess. The paranasal sinuses were clear of any obvious infection. Tenting of the ocular contents was seen on axial CT, suggestive of increased orbital pressure.

A superior orbitotomy for drainage of the abscess was performed, under general anesthesia. 2 millilitres of pus was drained and sent for culture and antibiotic sensitivity testing and Pulsed field Gel Electrophoresis (PFGE), which revealed CAMRSA, resistant to all antibiotics, except vancomycin, cotrimoxazole (trimethoprin/sulfamethoxazole combination) and amikacin. The patient was admitted and started on intravenous vancomycin 1 gram every 6 hours and intravenous amikacin 1 gram daily, after consultation with our internist. The patient started improving three days post therapy. Intravenous dexamethasone 4 mg every 8 hours was started to decrease associated inflammation. This was subsequently increased to

Figure 7.5A: CAMRSA orbital cellulitis: External photograph of patient, showing severe right sided proptosis, with conjunctival chemosis and draining wound of the right upper eyelid

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8 mg every 8 hours, 2 days later. Serum creatinine and urea were monitored every 72 hours. Seven days posttherapy, the vision had improved to 6/24 and the ocular movements had normalized almost completely. However, lagophthalmos due to the earlier lid wound persisted. The patient was discharged on oral cotrimoxazole 960 mg every 12 hours and oral steroids 60 mg every day. The antibiotic was stopped a week later and steroids gradually tapered.

At 3 months follow-up, the vision in the right eye was 6/9. The lid wound had completely healed (Figure 7.5B).

Discussion

Methicillin-resistant Staphylococcus aureus (MRSA) has been recognized as a cause of nosocomial infections since the 1960s. Recently, MRSA infections have been reported among patients with no history of hospitalization. These infections have affected prison inmates, athletic teams, military recruits, children attending day care, and patients within no identifiable risk group.29,30 Community acquired MRSA (CAMRSA) is no longer a pathogen unique to certain high-risk populations such as prison inmates. Most patients presenting in an outpatient setting with an MRSA soft-tissue infection are not linked to any distinct high-risk group.30 These CAMRSA strains have different genetic background and antibiotic susceptibility profiles than hospital strains. Despite their broader antibiotic susceptibility, in comparison to hospital-acquired strains, CAMRSA strains can cause severe infections, such as necrotizing pneumonias, large soft-tissue abscesses, and necrotizing fasciitis, in otherwise healthy patients.

Figure 7.5B: CAMRSA orbital cellulitis: External photograph, 3 months postorbitotomy, showing complete resolution of the condition and complete healing of the lid wound

CAMRSA causing orbital cellulitis is rare, but with an increasing incidence.31

CAMRSA is geographically widespread, as it has been reported in many regions of the US as well as Europe, Japan, and Australia.30, 31 Within the orbit; this infection is extremely virulent and being resistant to most antibiotics, causes severe damage.

We report a rapidly evolving orbital cellulitis caused by CAMRSA. A rapidly evolving orbital cellulitis with an abscess in an adult from any cause should undergo prompt surgical drainage and treatment with susceptible antibiotics. So in this respect a MRSA orbital cellulitis is no different. The real teaching point in this case is to include MRSA as a significant possible cause of the cellulitis and to start empiric therapy that includes coverage for MRSA, until antibiotic susceptibilities come back. It is further recommended to maintain suspicion for MRSA in community patients with possible staphylococcal infection, failing β-lactam class antibiotic therapy, to obtain culture and sensitivity studies in cases of severe ophthalmic infections, and to be informed about the rates of MRSA in the local community.

REFERENCES

1.Henning Bier, and Uwe Ganzer. Involvement of the orbit in diseases of the paranasal sinuses. Neurosurgical Review 1990;13: 109-12.

2.Ambati BK, Ambati J, Azar N. Periorbital and orbital cellulitis before and after the advent of Haemophilus influenzae type B vaccination. Ophthalmology 2000; 107: 1450-53.

3.Kronish JW, Jhonson TE, Gilberg SM, et al. Orbital infections in patients with human immunodeficiency virus infection. Ophthalmology 1996;103:1483-92.

4.Smith AF, Spencer JF. Orbital complications resulting from lesions of the sinuses. Ann Otol Rhinol Laryngol 1948; 57: 5-27.

5.Chandler JR, Langenbrunner DJ, Stevens ER: The pathogenesis of orbital complications in acute sinusitis. Laryngoscope 1970; 80: 1414-28.

6.Uzcategui N, Warman R, Smith A, Howard CW. Clinical practice guidelines for the management of orbital cellulitis. J Pediatr Ophthalmol Strabismus 1998;35:73-79.

7.Givner, Laurence B. Periorbital versus orbital cellulitis. Concise Reviews Of Pediatric Infectious Diseases. Pediatric Infectious Disease Journal 2002.21:1157-58.

8.Shovlin JP. Orbital infections and inflammations. Curr Opin Ophthalmol. 1998; 9: 41-48.

9.Mannor G. Unusual orbital infections: Oculoplastic and orbital surgery. Current Opinion in Ophthalmology 2000;11: 357-60.

10.Shuttleworth GN. (Clinical review)Orbital trauma.do not blow your nose. BMJ 1999;318:1054-55.

11.Wald ER. Periorbital and Orbital Infections. Pediatrics in Review 2004; 25: 312-20.

12.Jeyaratnam D, Reid C, Kearns A, Klein J. Community associated MRSA: an alert to paediatricians. Arch Dis Child 2006; 91: 511-12.

13.Hendrickson RG, Olshaker J, Duckett O. Rhinocerebral mucormycosis: a case of a rare, but deadly disease. J Emerg Med 1999;17:641-45.

14.Ferry AP, Abedi S. Diagnosis and management of rhinoorbitalcerebral mucormycosis. A report of 16 personally observed cases. Ophthalmology 1983;90: 1096-104.

15.Mortada A. Orbital pseudo-tumors and parasitic infections. Bull Ophthalmol Soc Egypt 1968; 6: 393-9.

16.Mun-Wai L, Kee-Siew F, Li-Yang H, Wee-Kiak L. Optic nerve toxoplasmosis and orbital inflammation as initial presentation of AIDS. Graefe's Archive for Clinical and Experimental Ophthalmology 2006;244:1542-44.

17.Luis Gorospe, Aránzazu Royo, Teresa Berrocal, GarciaRaya P, Moreno P, Abelairas J. Imaging of orbital disorders in pediatric patients. European Radiology 2003;13:201226.

18.Wendy L. Wright MS. Viral or Acute Bacterial Rhinosinusitis? Determining the Difference. The Nurse Practitioner: The American Journal of Primary Health Care 2005;30:30-41.

19.D B Jones and P G Steinkuller. Strategies for the initial management of acute preseptal and orbital cellulitis. Trans Am Ophthalmol Soc. 1988;86:94-112.

20.Urschel JD. Necrotizing soft tissue infections. Postgrad Med J 1999;75:645-49.

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21.Donahue SP, Schwartz G. Preseptal and orbital cellulitis in childhood. A changing microbiologic spectrum. Ophthalmology 1998; 105:1905-06.

22.Kronish JW, McLeish WM. Eyelid necrosis and periorbital necrotizing fasciitis. Report of a case and review of the literature. Ophthalmology 1991; 98; 92-8.

23.Luksich JA, Holds JB, Hartstein ME. Conservative management of necrotizing fasciitis of the eyelids. Ophthalmology 2002; 109; 2118-22.

24.Seal DV. Necrotizing fasciitis. Curr Opin Infect Dis 2001; 14: 127-132.

25.Aggarwal D, Suri A, Mahapatra AK. Orbital tuberculosis with abscess. J Neuro-Ophthalmol 2002; 22: 208-10.

26.Biswas J, Shome D. Choroidal tubercles in disseminated tuberculosis diagnosed by the polymerase chain reaction of aqueous humor: a case report and review of the literature. Ocul Immunol Inflamm 2002;10: 293-98.

27.Sen DK. Tuberculosis of the orbit and lacrimal gland: a clinical study of 14 cases. J Pediatr Ophthalmol Strabismus 1980;17:232-38.

28.Shome D, Honavar SG, Vemuganti GK, Joseph J. Orbital tuberculosis manifesting with enophthalmos and causing a diagnostic dilemma. Ophthal Plast Reconstr Surg 2006; 22: 219-21.

29.Weber JT. Community-associated methicillin-resistant Staphylococcus aureus. Clin Infect Dis 2005; 41: S269 - 72.

30.Rutar T, Chambers HF, Crawford JB, Perdreau-Remington F, Zwick OM, Karr M, Diehn JJ, Cockerham KP. Ophthalmic manifestations of infections caused by the USA 300 clone of community-associated methicillin-resistant Staphylococcus aureus. Ophthalmology 2006;113:1455-62.

31.Rutar T, Zwick OM, Cockerham KP, Horton JC. Bilateral blindness from orbital cellulitis caused by communityacquired methicillin-resistant Staphylococcus aureus. Am J Ophthalmol 2005;140:740-2.

The orbits represent a microcosm of the body in terms of tissues present-muscle, adipose tissue, blood vessels, nerves, skin and bone as also the eyeball with its unique architecture, histology and spectrum of diseases affecting it. Inflammatory conditions of the orbit represent the commonest afflictions of the orbits and thyroid associated orbitopathy and infections constitute the bulk of these.

Excluding the above causes of orbital inflammation, the causative entities range from vasculitis, like Wegener’s granulomatosis to granulomatous conditions like sarcoidosis and the entity of idiopathic orbital inflammation, earlier popularly labelled as pseudotumor. As evident from the diversitry of underlying causes, the clinical picture, natural history, treatment and outcome of these conditions vary greatly.

The age profile of patients with orbital inflammation also varies greatly, ranging from the paediatric age group for juvenile xanthogranuloma to adulthood, mostly the 3rd-5th decades of life for the vast majority of inflammatory conditions. Orbital inflammatory disorders are less common in the elderly, and must be diagnosed only after ruling out metastatic disease and infections, by tissue diagnosis if needed.

Orbital inflammation affects both sexes and all races across continents.

Broadly speaking, all patients with orbital inflammation present with one or more of the inflammatory symptoms of pain, swelling around the eye, proptosis, double vision or reduced vision, redness or watering. Pain is a common symptom and

is variably described as dull, aching or throbbing and is poorly localized, with headache being a common complaint. Periocular swelling and puffiness, more pronounced in the mornings after rising from sleep is not uncommon. While double vision is reported by some of these patients, it is a primary complaint only in a small proportion of the patients and at times elicited only on examination.

Orbital disease, particularly resulting from inflammatory conditions is one of the few remaining areas in the practice of ophthalmology, where detailed and meticulous history taking still has an important role and bearing on arriving at a diagnosis. Details of symptoms, associated systemic features, response to medications and side effects of treatment are important areas which need to be probed carefully. For example, merely knowing that a patient’s orbital symptoms improved on oral steroids may be inadequate to arrive at a diagnosis of idiopathic orbital inflammation. If the details are sought, and suggest rapid relief starting within hours, and near total resolution, one would be more definite in making the diagnosis.

Examination of a patient with orbital inflammatory disease needs to be done with meticulous attention to detail. Careful documentation including photographic documentation is invaluable in studying the natural history of an individual patient’s condition as also in prognostication and in titrating medical therapy.

Close co-ordination with other specialists treating a patient is clearly essential in the management of patients with orbital inflammatory

conditions. In addition to primary care givers, like rheumatologists in managing a patient with a vasculitic condition, other specialists often need to be involved in care as the disease progresses or complications develop as a result of medical therapy, as in the need for orthopaedic care for aseptic necrosis of neck of femur developing from prolonged systemic steroid therapy.

Overall, the disease entities covered in this chapter are diverse and may have little in common except the propensity to produce orbital inflammation. Achieving a precise diagnosis, by obtaining orbital tissue for diagnosis if needed, is vital. With present day techniques and instrumentation, obtaining an adequate specimen for histopathological and immunohistochemical diagnosis is safe. Except for cases where the disease is localized to a relatively inaccessible region of the orbit like the orbital apex, or resolves fully on medical therapy based on presumptive diagnosis, an incisional biopsy is essential to achieve diagnosis and rule out other causes for orbital inflammation like masquerade syndrome related to malignant tumor. A fine needle aspiration biopsy is often inadequate for the purpose.

The treatment of orbital inflammations remains centred on the control of inflammation and prevention of the sequelae of persistent and prolonged inflammatory reaction. A host of immunosuppressive drugs like cyclosporine, methotrexate and cyclophosphamide are used in addition to intravenous methylprednisolone and the widely used oral steroid medications.

Surgery in patients with orbital inflammatory disease is primarily to obtain tissue diagnosis. Surgical debulking of the involved orbital tissue is rarely needed, and the use of destructive operations like orbital exenteration is only in extremely disfigured orbits with no visual potential and severely troubling symptoms. Radiation therapy has limited role.

Treatment of orbital inflammatory disease needs to be tailored and titrated to the individual disease entity and patient. Since inflammation often tends to relapse and recur, long term follow-up is needed as also monitoring to assess for complications of orbital disease like ocular motility restriction or complications of therapy, like steroid induced cataract.

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The prognosis for patients with orbital inflammatory disease is variable and depends on the underlying disease and its severity.

ORBITAL AMYLOIDOSIS

Amyloidosis refers to a heterogenous group of disorders of protein metabolism characterized by the extracellular deposition of abnormal insoluble protein fibrils. Deposition of amyloid in the eye and its adnexal structures may occur as part of systemic amyloidosis or as a localised form. Local orbital amyloidosis is a rare condition, comprising only 4% of cases of local amyloidosis seen in the head and neck regions.1

Ocular findings in primary generalised amyloidosis include purpura of the eyelids, which can frequently be the presenting sign; bilateral, symmetrical, small amyloid papules of the skin of the eyelids, nodules in the lids, ptosis; proptosis, globe displacement with or without visual impairment, ophthalmoplegias or amyloid neuropathy affecting pupillary function or both and subconjunctival hemorrhages.

Histologically the specimen shows fibrous connective tissue and massive amyloid deposit infiltrated with lymphocytes, plasma cells, and foreign body giant cells. Amyloid deposits are identified histologically by congo red staining (Figure 8.1) and viewing under polarized light where amyloid deposits produce a distinctive ‘apple green birefringence’. The pathogenic mechanisms leading to local tissue deposition of amyloid are not clear. The universal constituent of amyloid is the amyloid P component (AP). It is derived from normal

Figure 8.1: Congo red staining showing amyloid (Congo red x 200)

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circulating plasma protein, serum amyloid P component (SAP). The isolated pure human SAP radiolabelled with I 123 is a highly specific tracer for all types of amyloidosis.2

The best method of orbital imaging is CT scan, for its ability to detect calcification which differentiates it from other lesions. Radionuclide SAP scans help in anatomical localisation of amyloid deposits.

Management of orbital amyloidosis is difficult. Standard treatment aims to reduce reproduction of the monoclonal immunoglobulin precursor via chemotherapy or radiotherapy or surgery of the localized lesion. Total excision is usually difficult and surgery is aimed at debulking the mass with preservation of palpebral gland of the lacrimal gland, levator and rectii muscles.

REFERENCES

1.Gean-Marton AD, Kirsh CFE, Vezina LG, et al. Focal amyloidosis of the head and neck: evaluation with CT and MR imaging. Radiology 1991;181:521-5.

2.Murdoch IE,Sullivan TJ, Moseley I, Hawkins PN, Pepys MB, Tan SY, Gamer A, Wright JE. Primary localised amyloidosis of the orbit. Br J Ophthalmol 1996;80:1083-6.

SARCOIDOSIS

Sarcoid (from the Greek ‘sark’ and ‘oid’ meaning “flesh-like”) or Besnier-Boeck disease or Schaumann’s syndrome.

Sarcoidosis is an idiopathic chronic non necrotizing granulomatous multi-systemic disease that affects skin, brain, eyes, lungs, spleen, thyroid, and liver. It commonly affects young adults, who frequently present with hilar lymphadeno pathy, pulmonary infiltration, ocular and cutaneous lesions.

Ocular involvement manifests in 25-60% of patients with systemic sarcoidosis.1 The most common ocular manifestation in sarcoidosis is uveitis and the most common orbital manifestation is dacryoadenitis, which is frequently bilateral.2 Other manifestations include eyelid swelling and palpable eyelid masses, conjunctival nodules, retrobulbar masses, proptosis, optic nerve, chiasma or sheath involvement, optic radiation infiltration, bone destruction and rarely extraocular muscle involvement.

It is mainly a diagnosis of exclusion based on clinical picture, laboratory tests, biopsy and radiologic evidence. Up to 90 percent of patients with ocular sarcoid have abnormal chest radiographs. Hilar lymphadenopathy is seen (Figures 8.2A and B). Lung biopsy by tracheobronchial fiber optic techniques is 90 percent accurate. Biopsy of an enlarged, potentially infiltrated lacrimal gland or conjunctival granuloma is an acceptable alternative.

A

B

Figures 8.2A and B: CT chest showing hilar lymphadenopathy in sarcoidosis

Typical histologic findings from biopsy include accumulation of T lymphocytes and mononuclear phagocytes, diffuse non-caseating epitheloid granulomas and derangements of the normal tissue architecture. Infectious etiologies need to be excluded by culture and/or staining.

Corticosteroids are the mainstay of treatment in symptomatic cases. Severe symptoms are generally treated with steroids. In cases of steroid intolerance or resistance, steroid sparing agents such as azathioprine and methotrexate are often used. Cyclophosphamide has also been used. As the granulomas are caused by collections of immune system cells, particularly T cells, there has been some early indication of success using immunosuppressants, interleukin 2 inhibitors or anti tumor necrosis factor-alpha treatment (such as infliximab).

Lofgren’s syndrome represents an acute presentation with erythema nodosum, bilateral hilar lymphadenopathy and polyarthralgia. This entity has a relatively good prognosis.

The combination of anterior uveitis, parotitis and fever is called uveoparotid fever and in association with cranial nerve palsies is referred to as HeerfordtWaldenstrom syndrome.

REFERENCES

1.Hunter DG, Foster CS. Ocular manifestations of sarcoidosis. In: Albert DM, Jakobiec FA, eds. Principles and practice of ophthalmology. Philadelphia: WB Saunders, 1994; 443-50.

2.Jakobiec F, Bilyk JR, Font RL Non infectious orbital inflammations. In: Spencer WH, editor. Ophthalmic pathology - An Atlas and Textbook. WB Saunders, Philadelphia, 1996; 2810-58.

NONSPECIFIC ORBITAL INFLAMMATORY SYNDROME (NSOIS)

Nonspecific orbital inflammatory syndrome (NSOIS), commonly referred to as Idiopathic Orbital Inflammation, Orbital Pseudotumor is defined as a benign, non infective clinical syndrome characterized by features of nonspecific inflammatory conditions of the orbit without identifiable local or systemic causes. Idiopathic orbital inflammation is the third most common non infectious orbital disease, following Graves’s orbitopathy and lymphoproliferative diseases. It accounts for 4.7 to 6.3% of orbital disorders.1

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Idiopathic orbital inflammation has highly variable clinical features, from a diffuse to very focal process targeting specific orbital tissues, such as the lacrimal gland, extraocular muscles, optic nerve and orbital fat. Presentations vary according to the specific location and the degree of inflammation, fibrosis, and mass effect. Ptosis, chemosis, motility dysfunction, and optic neuropathy may also be found. Entrapment, compression, and destruction of orbital tissues may occur in patients with extensive sclerosis. Unilateral presentation is typical, but bilateral presentations are not uncommon.

Radiological imaging studies allow tissue characterization and localization without surgical intervention and thereby have become invaluable diagnostic tools. Computed tomography is the preferred mode of imaging. Idiopathic orbital inflammation is typically seen on CT scans as a focal or diffuse mass, usually poorly demarcated and enhancing with contrast. Common CT findings include enhancement with contrast medium, infiltration of retrobulbar fat, proptosis, extraocular muscle enlargement, muscle tendon or sheath enlargement, apical fat edema, optic nerve thickening, uveal-scleral thickening, edema of the Tenon capsule, and lacrimal gland infiltration. Tendons of the extraocular muscles may be involved or spared.

The histopathological spectrum of idiopathic orbital inflammation is typically non diagnostic, wide, and diverse, ranging from the typical diffuse polymorphous infiltrate to the atypical granulomatous inflammation, tissue eosinophilia, and infiltrative sclerosis. In the absence of systemic fibro inflammatory, granulomatous, and vasculitic disease, these atypical presentations are considered to be subclasses of idiopathic orbital inflammation.2

NSOIS respond rapidly to high dose steroid therapy in tapering doses but recurrences are common. In such cases, chemotherapy (e.g. methotrexate, cyclosporine) and low-dose radiation (e.g. 1500-2500 cGy EBRT) may be needed to control the inflammation.3

REFERENCES

1.Henderson JW: Orbital tumors, Newyork, Ravin press, 1994, (3rd ed), pp 13-14; 47;317-411.

2.Root man J: The classification and management of acute orbital pseudotumors: Ophthalmology 1982, 89;1040-48.

3.Leone C: Treatment protocol for orbital inflammatory diseases; Ophthalmology 1985, 92; 1325-31.

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KIMURA’S DISEASE

Kimura’s disease is a chronic inflammatory disorder of uncertain etiology which typically presents as multiple cutaneous nodules in the head and neck region particularly the preauricular regions. It was first described in China in 1937 by Kim and Szeto as eosinophilic lymphogranuloma.1

Kimura’s disease is most commonly seen in patients between 20-40 years of age with a striking male predominance and is endemic among the oriental population. It is characterized by a triad of insidious onset of painless subcutaneous nodules in the head and neck region, blood and tissue eosinophilia and markedly elevated serum immunoglobulin levels.2 In the orbit, presentation is in the form of proptosis, upper lid swelling and orbital masses usually in the lacrimal gland.

The histomorphology of Kimura’s nodule is characterized by intense infiltration of lymphocytes, vascular proliferation and plasma cells with a variable number of lymphoid follicles with germinal centers. Typically, there is a moderate to intense eosinophilic infiltration mainly in a perivascular pattern. Immuohistochemical stains would typically show IgE reticular network in the germinal centers.3

Following initial presentation, surgical excision and biopsy with debulking is the preferred mode of treatment, but recurrence is common. Other treatment options include radiation, systemic corticosteroids, cyclosporine and pentoxyfylline.

REFERENCES

1.Kim HT, Szeto C. Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz’s disease. Chin med J. 1937 23:699-700.

2.Hui PK, Chan JK, Ng CS, Kung IT, Gwi E. Lymphadenopathy of Kimura’s disease. Am J Surg Pathol. 1989; 13:177-86.

3.Motoi M, Wahid S, Horie Y, Akagi T. Kimura’s disease: clinical, histological, and immunohistochemical studies. Acta Med Okayama.1992;46:449-55.

WEGENER’S GRANULOMATOSIS

Wegener’s granulomatosis (WG) is a fulminant systemic disease of unknown aetiology consisting of necrotizing granulomatous vasculitis of the upper and

lower respiratory tracts, focal necrotizing glomerulonephritis, and systemic small vessel vasculitis involving multiple organ systems.

Incidence of ocular involvement in WG varies from 29 to 79%.1, 2 Ocular involvement can be either due to an extension from the adjacent paranasal sinuses (contiguous) or as a result of focal vasculitis (noncontiguous).1,2 Presentation can be in the form of proptosis, dacryocystitis, scleritis with peripheral keratopathy, kerato-conjunctivitis sicca, uveitis, retinitis, retinal vascular occlusions, exudative retinal detachments, and optic neuritis.

Laboratory findings support or confirm the diagnosis of WG. In the active stage of the disease raised ESR and leucocytosis are seen. Chest X-ray and computerised tomography detect pulmonary involvement. Routine urine analysis detects renal involvement. Serum antibodies against the cytoplasmic component of neutrophils and monocytes (cANCA) form a useful adjunct in the diagnosis of WG. Indirect immunofluorescence is currently the standard test for ANCA screening.3 Between 80% and 95% of all ANCA found in WG is cANCA.

Diagnosis is established by biopsy in orbital and paranasal sinus involvement. The characteristic histopathologic picture is that of necrotising vasculitis of the blood vessels, usually with granuloma formation in the surrounding infiltrates.

Management of WG requires a multisystem approach. Oral corticosteroids along with a cytotoxic agent, of which cyclophosphamide is the most efficacious, is the treatment of choice. Early treatment with cyclophosphamide and corticosteroids reduces both ocular and systemic morbidity.

Exacerbations are common in the first two years after diagnosis. In cases of remission, azathioprine, cyclosporine A and methotrexate may be used.

REFERENCES

1.Straatsma BR. Ocular manifestations of Wegener’s granulomatosis. Am J Ophthalmol 1957; 144:789-99.

2.Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener’s granulomatosis. Ophthalmology 1983;90:279-90

3.Harman LE, Margo CE. Wegener’s granulomatosis. Survey of Ophthalmology 1998; 42:458-80.