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

Figure 5.7: Fungal filaments of Aspergillus species. Silver methanamine × 400

serological and/or cutaneous tests should be conducted for final diagnosis.

Cysticercosis

Cysticercosis of the orbit is very rare in the western literature, but Jacobiece and Font (1986) nath et al. (1977) documented nearly 24 cases in the literature. Sarada et al. (1981) found one case in the orbit among 50 cases of cysticercosis of the CNS. But in endemic areas orbital myocysticercosis is fairly common. They are usually managed with oral Albendazole and prednisolone. Only anteriorly located cysticercosis is excised and is available for histopathology (Figure 5.8). However, Murthy et al. (1990) reported on the use of ultrasound in preoperative diagnosis.

Cysticercus cellulosae, histologically, when sectioned shows fibrocellular, reaction with palisading histiocytes around the parasite. The worm may get calcified in later stages. The cellular infiltrate may be dominated by eosinophils.

Neoplastic Lesions

Classification of orbital tumors is given in Table 2 Benign tumors.

Figure 5.8: Cross-section of cysticercus. H and E × 400

Table 2: Classification of orbital tumors

Primary

I.Mesenchymal

a.Vascular: hemangioma, lymphangioma, hemangiopericytoma, hamangioendothelioma, Angiosarcoma, Kaposi’s sarcoma,

b.Lipoma, liposarcoma

c.Fibrous histiocytoma,

d.Fibrosarcoma

e.Rhabdomyosarcoma

f.Leiomyoma, sarcoma

g.Chondroma, chondrosarcoma

h.Giant cell tumor, osteosarcoma.

II.Neural

Glioma, neurilemmoma, neurofibroma, amputation

neuroma. III. Hemopoietic.

Lymphoma, leukemia, myeloma. IV. Lacrimal gland

Plemorphic adenoma, adenoid cystic carcinoma.

V.Miscellaneous

Meningioma, nonchromaffin paraganglioma, alveolar soft part sarcoma, malignant melanoma.

Secondary

I.Direct extension

a.Intraocular tumors retinoblastoma, melanoma

b.Eyelid tumors basal, squamous, sebaceous carcinoma, melanoma

c.Conjunctival squamous carcinoma, melanoma

d.Metastatic in children – neuroblastoma In adults–Lung Ca, Breast Ca.

BENIGN TUMORS

Cavernous Hemangioma

The most common benign intraorbital tumor in the young and middle aged is cavernous hemangioma that produces painless proptosis. It is common in females and may enlarge during pregnancy.

Despite the mention that cavernous hemangioma is the most common benign intraorbital tumor, there have not been many reports of a good series of this condition. Harris and Jacobiec (1979) could find 66 cases recorded during a 40 year period from three centers dealing specially with ophthalmic problems. Maroon and Kenerdell (1979) found five hemangiomas among 18 intraorbital tumors subjected to microsurgical treatment. The same authors reported 17 hemangiomas among 300 cases of orbital tumors seen from 1975 to 1982. Nath et al. (1977) found 12 cases among 120 cases of primary orbital tumors.

Being a benign slow growing tumor, it causes progressive often painless proptosis. As the lesion is usually situated behind the globe within the muscle cone, it produces axial proptosis.

Despite the prominent protrusion of the eyeball, vision is preserved and movements of the eye are spared till late in the course. About half of the patients in the series of Harris and Jacobiec(10979) had blurred vision and only three of the 66 had diplopia. Neither bruit nor pulsations were present in any of their cases.

Microscopically, the lesion is composed of dilated vascular channels. Some of the channels may contain prominent smooth muscles when the lesions are called venous hemangiomas (Figure 5.9).

Capillary Hemangioma

Capillary hemangioma is a benign tumor that manifests usually in first five years of life and tends to regress thereafter. It is generally single, bright red and smooth lesion. Histologically, capillaries of small size are closely packed with no smooth muscle in between (Figure 5.10).

Lymphangioma

Pathology of the Orbital Diseases 103

Figure 5.9: Histological section from a case of cavernous hemangioma, showing widely spaced vascular channels rimed by fibromuscular tissue. H and E × 400

Figure 5.10: Polypoidal tissue composed of proliferating vascular channels embedded in a inflammatory and edematous stroma from a case of capillary hemangioma, pyogenic type. H and E × 400

children and adolescents. Spontaneous hemorrhage into the cyst leads to abrupt proptosis and formation of “chocolate cysts”. Lymphangioma occurs in extraconal location and presents with pain and proptosis. Infiltration of muscles and nerves of orbit also occurs in this condition (Figure 5.11) .

Hemangiopericytoma

There are no lymphatics in the orbit. However, lymphangiomas do occur here and account for 0.5 to 3% of intraorbital tumors. It is commonly seen in

It is a rare lesion occurs in the fourth decade of life. It may be malignant in about 12% cases. The lesion consists of increased number of thin-walled vascular

Figure 5.11: Section showing dilated vascular channels with lymphoid aggregates in the mural compartment from a case of Lymphagioma. H and E × 400

channels with perivascular massing of pericytes separated by tumor cells in a network of extracellular material (Figure 5.12).

Meningiomas

Orbital meningiomas are of three types based on their origin—

1.Intracranial meningiomas which secondarily invade the orbit are the commonest. Meningiomas of the middle cranial fossa especially those of sphenoid ridge are notorious to cause proptosis. Meningiomas of the anterior cranial fossa may invade the orbital roof. In

Figure 5.12: Histological section showing thin vascular channels with oval to spindle cells oriented externally from a case of Hemangiopericytoma. H and E × 600

Figure 5.13: Section shows oval to polyhedral cells with vesicular nuclei and lightly acidophilic cytoplasm arranged in nests and whorls. There are scattered psammoma bodies, from a case of meningioma.

H and E × 400

addition ectopic meningiomas arising from, for example, the frontal sinus may encroach into the orbit.

2.Primary intraorbital meningiomas are rare tumors which arise from the optic nerve. This is the most difficult one to treat. The differentiation between optic nerve glioma and meningioma is an important clinical problem.

3.Those which have no apparent connection with the optic nerve are occasionally found in the orbit. These probably arise from ectopic arachnoid cell nests in the orbit.

Intraorbital meningiomas are more common in women than men. The average age of onset is about 30 years. About 40% occur below 20 years. The main clinical features are loss of vision and progressive exopthalmos. Neurofibromatosis may be associated within 16% of cases (Figure 5.13).

Malignant Tumors

Rhabdomyosarcoma

Rhabdomyosarcoma is the most common malignant mesenchymal orbital neoplasm and malignant orbital tumor in children. The average age of onset is six years and the lesion is rare after 25 years. The onset is rapid and the progression simulates cellulitis. Histologically, the tumors are of

three types (i) embryonal (ii) adult pleomorphic and (iii) alveolar.

Of these three types embryonal rhabdomyosarcoma is the most common type of malignant tumor. Structurally, tumors are composed of round, oval or stellate rhabdomyoblasts with mitosis in loose syncitium. Cells contain dense eosinophilic cytoplasm with striations.

Mortality rate is 40.6%. Prognosis is best in adult pleomorphic type and it is worst in alveolar type (Figures 5.14 and 5.15).

Figure 5.14: Cellular lesion showing spindle cells with acidophilic cytoplasm arranged in alveolar pattern in a case of alveolar rhabdomyosarcoma H and E × 400

Pathology of the Orbital Diseases 105

Adenoid Cystic Carcinoma

It occurs in adults of either sex in the fourth decade of life. The lesion is relentlessly progressive, invading the adjacent tissues with characteristic tendency to spread along perineural lymphatics (Figure 5.16).

Lymphoma

Lymphomas in adults are encountered in 10% biopsies of orbital tumors. They are usually found in the anterior orbit. About 25% of lymphoma presenting as NSOID may evolve into lymphomas. They are essentially B cell lymphomas. Orbital lymphomas may be the first manifestation of systemic lymphomas (Figure 5.17).

Histiocytoma

Malignant fibrous histiocytomas are rare, and more lethal lesions of orbit. These are common in older age group. The origin of the tumor is from the histiocytes,and the neoplastic histiocytes are bizarre with areas of hemorrhage,necrosis and frequent mitosis. Focal or diffuse storiform pattern characteristic of histiocytomas is discernible .

Metastasis

It accounts for 5% of orbital tumors. In children, neuroblastoma, ewing's and leukemia and in adults, carcinoma of bronchus and breast are the common

Figure 5.15: Cellular lesion showing spindle cells with acidophilic cytoplasm arranged in alveolar pattern in a case of alveolar rhabdomyosarcoma. H and E × 600

Figure 5.16: Large cellular islands of basaloid cells with focal cystic pattern embedded in fibro vascular tissue. From a case of adenoid cystic carcinoma of the orbit. H and E × 400

106 Surgical Atlas of Orbital Diseases

Figure 5.17: Cellular lesion composed of monomorphic round cells with dark nuclei and scant cytoplasm seen in nodular aggregates form a case of orbital lymphoma. H and E × 400

primary sites. In about 50% of the cases the primary remains unknown. Of all metastases 30% are orbital and 70% ocular. The main symptoms are pain, proptosis and limitation of extraocular movements. Undifferentiated carcinomas arising in a paranasal sinus account for 4-9% of unilateral exophthalmos. The carcinoma of ethmoid most commonly involves the orbit. Pain, proptosis, restricted ocular motility, with ptosis occurring rapidly is a feature of malignancy of the orbit.

Optic Glioma

Optic glioma are primary tumors of the optic nerve and/or chiasma. They are usually low grade pilocytic astrocytomas which may appear fibroblastic due to invasion of the leptomeninges (desmoplastic) or gelatinous with oligodendroglial component. Those of the optic nerve may be intraorbital or intracranial

(Robertson and Broson, 1980; Alvord and Lofton, 1988).

Grave’s Disease

It is one of the most common causes of proptosis which mostly affects females. Lid retraction is an early sign. These orbital signs may manifest at any stage of the endocrine dysfunction. Computed tomography and ultrasonography may reveal the characteristic thickening of extraocular muscles without evidence of mass lesion. The lesion is often

bilateral, though the patient may present when it is still confined to one orbit. Microscopic examination shows edema, lymphocytic infiltration sometimes forming follicles.

MISCELLANEOUS

Mucoceles

Mucoceles of the paranasal sinuses may require neurosurgical attention when they involve the orbit and/or cranial contents. The most frequent site is the frontal sinus.

Those involving the sphenoid and/or posterior ethmoidal sinuses are rare. Chen et al. (1986) and Nugent(1970) reviewed 63 cases in whom visual impairment was noted in 71% of the cases. Optic nerve damage may be caused by intracanalicular extension of the lesion with erosion of the canal walls.

Periorbital swelling, pain and displacement of globe are the frequent symptoms of frontal and ethmoidal lesions. There may be a swelling over the frontal sinus with crackling sensation on palpation due to thinning of its anterior wall. Sphenoidal sinus mucoceles cause headache, peri or retroorbital pain and ophthalmoplegias due to extension into the orbital apex and cavernous sinus (pompili et al. 1990).

Sellar involvement is seen frequently in sphenoidal mucocele. Surgical intervention and excision of the lesion is indicated to relieve pressure on the orbital contents.

The aetiology of mucoceles is multifactorial. The basic cause seems to be obstruction of the ostium of the sinus by a variety of causes such as inflammation, trauma, polyps, previous surgery, allergy and benign and malignant tumors. The exact precipitating factor may not be evident in many cases (Weber and Mikulis, 1987).

Histological examination (HPE) reveals fragments of polypoid tissue lined by pseudostratified ciliated epithelium. The sub epithelial tissue is made up of loose stroma with pools of small blood vessels and diffuse infiltration of mononuclear cells and prominent collections of eosinophils embedded with calcific spicules.

BIBLIOGRAPHY

1.Bakhshi S, Sidhu T: Pediatric orbital and ocular lymphomas, Pediatr Blood Cancer. 2008;50(4):940-1.

2.Bernardini FP, Bazzan M: Lymphoproliferative disease of the orbit, Curr Opin Ophthalmol. 2007;18(5):398-401.

3.Biswas J, Roy Chowdhury B, Krishna Kumar S, Lily Therese K, Madhavan HN: Detection of Mycobacterium tuberculosis by polymerase chain reaction in a case of orbital tuberculosis, Orbit. 2001;20(1):69-74.

4.Butnor KJ, Cummings TJ. Pathologic quiz case: left eye proptosis, ptosis, and blindness. Hemangiopericytoma of the orbit. Arch Pathol Lab Med. 2002;126(12):1555-6.

5.Cruz AA, Constanzi M, de Castro FA, dos Santos AC: Apical involvement with fibrous dysplasia: implications for vision, Ophthal Plast Reconstr surg 2007;23(6):450-4.

6.Eddleman CS, Liu JK: Optic nerve sheath meningioma: current diagnosis and treatment, Neurosurg Focus. 2007;23(5):E4.

7.Goisis M, Biglioli F, Guareschi M, Frigerio A, Mortini P: Fibrous dysplasia of the orbital region: current clinical perspectives in ophthalmology and cranio-maxillofacial surgery, Ophthal Plast Reconstr Surg. 2006;22(5):383-7.

8.Gordon LK: Orbital inflammatory disease: a diagnostic and therapeutic challenge, Eye. 2006;20(10):1196-206.

9.Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: A clincopathological analysis of sixty-six cases. In: Ocular and adnexal tumors. Birmingham, AL: Aesculapius, 1978;741-81.

10.Honavar SG, Sekhar. G, Orbital Cysticercosis. Orbit 1998; 17(4)271-84.

11.Kaur A, Kant S, Bhasker SK: Periorbital tuberculosis, Orbit. 2007;26(1):39-42.

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12.Lee V, Ragge NK, Collin JR. Orbitotemporal neurofibromatosis. Clinical features and surgical management, Ophthalmology. 2004;111(2):382-8.

13.Lin B, Looi A: Orbital lymphoma, Ophthalmology. 2007;114(7):1423.

14.Malhotra R, Wormald PJ, Selva D. Bilateral dynamic proptosis due to frontoethmoidal sinus mucocele. Ophthal Plast Reconstr Surg. 2003;19(2):156-7.

15.Nugent RA, Rootman J, Robertson WD, et al. Acute orbital pseudotumors: AJNR 1981;2:431-6.

16.Perry SR, Rootman J, White VA. The clinical and pathological constellation of wegener's granulomatosis of the orbit. Ophthalmology 1997;104:683-94.

17.Pillai S, Malone TJ, Abad JC. Orbital tuberculosis. Ophthal Plast Reconstr Surg 1995;11:27-31.

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19.Rootman J: Diseases of the orbit; A multidisciplinary approach. Lippincott Williams and Wilkins, (2nd Ed): 455-506.

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Introduction

Thyroid-associated orbitopathy (also known as Graves’ Orbitopathy, Graves’ Ophthalmopathy, Thyroid Eye Disease) is an immune-mediated inflammatory disorder causing enlargement of the orbital muscles and fat (Figure 6.1).1,2 Its clinical spectrum ranges from eyelid retraction and proptosis with exposure complaints (Figure 6.2A) to more serious problems such as orbital soft tissue inflammation (with discomfort, eyelid and conjunctival congestion and edema), extraocular motility restriction, and loss of vision from compressive optic neuropathy (Figure 6.3A).3

Severe cases may result in lasting cosmetic disfigurement and functional visual impairment. Quality of life studies have shown that it may have

Figure 6.2A: Mild TAO in a young female with bilateral upper lid retraction (worse on the left side) and secondary ocular irritation and epiphora

Figure 6.1: Axial CT scan of patient with asymmetric TAO demonstrating right proptosis from marked enlargement of the right orbital muscles and mild enlargement of the fat compartment. Compare the affected right orbit with the normal left orbit

Figure 6.2B: Same patient at the time of surgery following a posterior graded lowering of both upper eyelids

112 Surgical Atlas of Orbital Diseases

Figure 6.3A: Patient with severe manifestations of TAO: VISA Score: V = 1/1 (bilateral optic neuropathy with vision reduced to 20/100, 20/80); I = 9/10 (pain at rest and movement, +2 chemosis, +1 eyelid edema, +1 caruncular edema, +1 conjunctival redness, +1 eyelid redness); S = 3/3 (motility restricted to less than 15° upgaze bilaterally);

A = 3/3 (corneal ulceration, Hertel 26 mm bilaterally)

Figure 6.3B: Same patient following intravenous pulsed corticosteroids, orbital decompression, and upper lid posterior lowering. VISA Score: V = 0/1 (no optic neuropathy); I = 0/10; S = 1/ 3 (eyes aligned but restricted to 30° motility); A = 1/3, mild (some residual upper eyelid deformity, Hertel 21 mm bilaterally)

more significant lifestyle consequences than chronic lung disease or diabetes mellitus.4

Incidence and Epidemiology

Thyroid-associated orbitopathy (TAO) is the most common orbital disease in the Americas and Europe, with an annual incidence in females of approximately 14 per 100,000 and approximately one-fifth that for males.5 Anecdotally, it may be less prevalent (or possibly causes fewer severe complications) in Africa and South Asia. However, it does occur in all races and ages, and is most common between the second and sixth decades.6

Between 25-50% of patients with immune thyroid diseases develop orbital involvement, and of those, 5-10% may develop more severe consequences such as severe inflammation and congestion, impaired motility, or compressive optic neuropathy.7 A smaller percentage may develop Graves’ lower limb dermopathy (pretibial myxedema, with deposition of subdermal hyaluronic acid), usually 1-2 years following the onset of thyroid gland dysfunction and shortly following severe orbitopathy, and a smaller subset may develop acropachy (clubbing of the fingers).8

Risk Factors, Predictive Variables for Disease Severity and Associated Immune Disorders

Risk factors for developing TAO include smoking, life stressors, poorly controlled hypothyroidism following radioactive iodine, and a positive family history of orbitopathy.9,10

Predictive variables for developing more serious consequences of TAO include male gender, increasing age, smoking, and a rapid onset of orbitopathy.11

Cigarette smoking has been shown by numerous studies to be correlated strongly with the development of TAO and a progressively higher incidence of smoking is seen with more severe disease.11-13

Patients with TAO have an increased probability of developing associated immune diseases, including superior limbic keratitis (SLK), myasthenia gravis, diabetes mellitus, alopecia and vitiligo.14 Psychiatric conditions such as bipolar affective disorder and anxiety occur more frequently in patients with thyroid dysfunction and with TAO.

Pathogenesis

The pathologic hallmark of TAO is a lymphocytic infiltration of orbital muscle and fat with expansion of these tissues from edema and deposition of hyaluronic acid and other glycosaminoglycans.2 Patients exhibiting fat expansion alone may present with proptosis and lid retraction. Those with muscle enlargement and more inflammatory features may develop proptosis, periocular inflammatory features, and possible restriction of motility or strabismus if fibrosis develops (Figure 6.4). In a limited number