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

Preventing Mishaps

1.Avoid extreme temperature changes, which can cause adhesive to fail.

2.Carry extra-adhesive and pre-packaged alcoholsoaked cotton balls in a small plastic bag.

3.Avoid placing the prosthesis in purses or pockets close to items such as ink pens and makeup that could stain it.

4.If adhesive are prescribed, be careful not to spill the adhesive bottle. To prevent evaporation, keep the lid tightened when not in use.

CONCLUSION

In majority of cases facial prosthesis restores the cosmetic appearance as well as self confidence (Figures 23.4A and B). However, the patient should be educated and motivated to use the prosthesis and maintain it properly. A cosmetic rehabilitation without the proper counselling is not effective. Overall a facial prosthesis remains an option in minority of cases.

Orbital Prosthesis 333

REFERENCES

1.Prince JH, A short history of the development of artificial eyes. In: Ocular prosthesis. E and S Livingstone Ltd, Edinburgh, 1946:6-7.

2.Jackson, IT, Tolaman, DE, Desjardins, RP,Branemark, PI: A new method for fixation of external prosthesis, Plast Reconst. Surg. 1986;77:668-72.

3.Raizada K, Murthy R, Honavar SG. Ocular prosthesis with lower lid augmentation for disfigured lids following chemical burns. Journal of Ophthalmic Prosthesis J Ophth. Prosth. Volume II PP 24-6.

4.Raizada K, D Deepa Rani, Naik M, Honavar SG, Journal of Facial and Somato Prosthesis. Post Enucleation Socket Syndrome: an ocularist View, Journal of Facial and Somatoprosthesis, 2005;1-12.

5.Yeatts RP. The esthetics of orbital exenteration.Am J Ophthalmol. 2005;139(1):152-3.

6.Bulbulian AH. Prosthetic reconstruction of the exenterated orbit. In: Facial Prosthetics. Charles C Thomas, Springfield, IL, 1973: 48-63.

7.Jahrling RC. Contracted socket following enucleation after multiple surgical procedures. Problems and treatment of enucleation, evisceration, exposure. Intercontinental Medical book corporation 1974:12;27-9.

8.Bulbian AH, Facial Prosthetics, method of retention of facial prosthesis 364.

Some of you may be surprised to know that nearly half the cases of proptosis can be managed without surgery. This is because of the advances in diagnostic and therapeutic interventions made in the past few decades which made medical managements more scientific, evidence based and safer. Many patients of proptosis obviously are to benefit from these advances. We are confident that most of the active orbital surgeons across the globe agree with this statement.

Many etiological factors involved in proptosis can now be safely managed medically. This chapter outlines the standard protocols and recent trends invading this arena. The role of medical management in thyroid orbitopathy, and the role of chemotherapy were dealt in detail in separate chapters and hence not included in this.

Let us consider the medical management in the following headings:

•Nonspecific inflammations of the orbit

•Specific inflammations of the orbit

•Vascular lesions

•Structural lesions

•Lymphoproliferative and other neoplastic lesions.1

NONSPECIFIC INFLAMMATIONS OF THE ORBIT (NSOIS)

Based upon the location of inflammation the nonspecific orbital inflammation or idiopathic orbital inflammation can present as five entities : Myositic,

lacrimal, anterior, diffuse and apical. Apart from clinical presentation, imaging is very helpful in diagnosis. NSOIS is usually acute or subacute in onset and painful. It is usually unilateral, and occasionally bilateral. Rarely it is recurrent. The visual symptoms include diplopia, and defective vision. Histologically NSOIS is characterized by polymorphous infiltrations.

Nonspecific Myositic Inflammation

This is the most common presentation in our experience. We manage patients presenting as a single muscle disease with nonsteroidal anti-inflammatory drugs and low dose corticosteroids. Recurrence is unlikely in them. Patients presenting with multiple muscle disease, are prone to recurrences. We treat them more aggressively with oral Prednisolone 2 mg/kg body weight tapered over 4-6 weeks or with intravenous methyl prednisolone2 pulse therapy. Patients with recalcitrant disease require immunosuppressives. Such patients were found to benefit from methotrexate in a dose of 15-25 mg per week3 usually marked clinical response is evident within a week. Those cases which fail to respond to the drugs warrant a biopsy to exclude a lymphoma.

Nonspecific Lacrimal Inflammation

Nonspecific dacryoadenitis should prompt a suspicion for systemic disease where a percutaneous biopsy is recommended first. Management includes moderate doses of steroids such as 1mg/kg oral prednisolone which can be tapered over 4-6 weeks. Majority of nonspecific dacryoadenitis resolves over 6-12 weeks.

338 Surgical Atlas of Orbital Diseases

Specific Inflammations of the Orbit

These are the most common etiological factors in proptosis for which medical management is commonly carried out. Therapeutic options in management of inflammations are ever expanding not only because biologically targeted agents are becoming increasingly available that can act on specific segments of inflammatory cascades but also because of advances in our understanding of etiopathogenesis.

Orbital Cellulitis

This is the most common cause of painful proptosis, acute in onset and most often unilateral. The principles of management of a case of orbital cellulitis are control of infection by the use of appropriate antibiotics, preventions of ocular as well as nonocular complications, surgical drainage when necessary and careful follow up. We insist on imaging on an emergency basis in every case of orbital cellulitis for two reasons : (1) To make sure that we are not missing other neoplastic lesions like Rhabdomyosarcoma, Retinoblastoma which clinically mimic orbital cellulitis (2) To plan the course of action: We prefer surgical drainage of orbital or sub-periosteal abscess. In the absence of abscess, medical management is preferred. The patient should be carefully monitored during the treatment for any threat of loss of vision, and clinical response to the drugs. From our experience and also from the literature we wish to emphasize that there is a great difference in the prognosis and hence management strategies of orbital cellulitis in children and adults. Usually in children under the age of 9 years the infection is by a single aerobic organism such as pneumococci4 and respond to medical management, the threat to affect vision is rare and surgery is rarely needed. In contrast adults harbor polymicrobial infection and the threat to vision is common and hence drainage is frequently required in addition to systemic antibiotics.5

Orbital cellulitis secondary to sinusitis is known to harbour organisms like Strep pneumoniae, H

influenzae, Bacteroids and anaerobic cocci. Recommended antibiotics include third generation cephalosporins like cefotaxime, ceftriaxone and cefuroxime. Alternatively piperacillin with taxobactum or ticarcillin with clavulanate can be used. Orbital cellulitis secondary to trauma or foreign-body

are known to be harbouring organisms like S aureus,

S epidermidis, Streptococci and anerobes. Recommended antibiotics for such cases include Vancomycin along with third generation cephalosporins or imipenem1 Imaging with a CT or a MRI can accurately monitor the progress and effect of treatment. Septic thrombosis of cavernous sinus either due to spread from contiguous structures or septicemia demands prompt recognition and treatment with broad spectrum antibiotics as discussed above for optimal clinical outcome.

Rhino-orbital Mucormycosis

This is one of the very harmful infections and can lead to death. It is almost always associated with uncontrolled diabetes mellitus and usually with ketoacidosis.6-8 Once the diagnosis is established based on clinical findings, microscopic fungal examination and culture, a multidisciplinary approach is commonly practiced. Diabetes should be managed simultaneously. Following wide excision of devitalized tissue, the area is daily irrigated with amphotericin. Systemic treatment with amphotericin is also recommended. Some believe in the usefulness of hyperbaric oxygen for such cases.9

Chronic Granulomatous Infections

Since the advent of AIDS, incidence of certain granulomatous infections of importance in proptosis like tuberculosis and syphilis is on a rise.

Orbital involvement in tuberculosis is usually by direct invasion from sinuses or hematological dissemination. Periostitis, cold abscess and orbital tuberculomas are well recognized lesions.10 In doubtful cases PCR is helpful. Orbital involvement is diagnosed with a high index of suspicion and aspiration biopsy. Systemic anti-tuberculous drugs are recommended in coordination with a chest physician.

Though literature says that syphilis is on a rise with immunosuppressive syndromes, we are yet to come across a case of syphilis with orbital involvement. Periostitis, acute and chronic inflammations are recognized lesions. Systemic antibiotic therapy usually with penicillins is useful in resolution of the disease.

Parasitic Infestations

Cysticercosis, echinococcosis and trichinosis are common parasitic etiological factors in the causation of proptosis.

Cysticercosis is caused by C.cellulosae, the larval form of tapeworm, Taenia solium. Though orbit is considered to be a rare site in the west, many reports in the literature suggests that orbital involvement is most frequent among Asians.11 Once diagnosis is established with the help of imaging and serology, systemic albendazole (15 mg/kg) along with steroids (prednisolone 1-2 mg/kg) for a period of four weeks is found to be effective11 If there is evidence of associated neurocysticercosis, treatment is with steroids (prednisolone 1mg/kg) and praziquantel 50 mg/kg in three divided doses for a period of 15 days. Association between orbital myocysticercosis and neurocysticercosis is not very common. We prefer to refer these patients to a neurologist for in-patient treatment since there is a rare possibility of generalized seizures. The response can be monitored and progress can be documented with imaging.

Echinococcosis or hydatid cyst as it is commonly called is an intestinal infestation of dogs. Orbital cysts are seen in 1% of echinococcosis. Systemic albendazole has been found to be effective in resolving the cyst.15 Steroids are recommended for violent inflammatory reactions following rupture of the cyst during aspiration or attempted surgical removal16 Certain studies have shown high efficacy in disease resolution when combination of praziquantel with albendazole12-14 is used.

Trichinosis occurs as cysts in extraocular muscles, which may show evidence of calcification on imaging. Treatment recommended include systemic thiabendazole along with steroids to reduce inflammation. Personally we have no experience as we are yet to come across a case of trichinosis with orbital involvement.

Vasculitis

Vasculitis or angiitides as some may call it is a clinical syndrome that encompasses acute or chronic inflammation of vessels with vaso-obliterative signs and symptoms17 Most common vasculitis that involve the orbit include Wegener's granulomatosis and Polyarteritis nodosa. Diagnosis is usually established by imaging, biopsy with a histopathological examination and specially for wegener's a serological examination in the form of C-ANCA15 Dramatic improvement is noticed when systemic steroids are combined with an alkylating agent like cyclo-

Medical Management of Proptosis 339

phosphamide.21,22 Recent studies have suggested a role of anti-TNFs (tumor necrosis factors) like Infliximab and Etanercept. The development of novel approaches focusing on blockade of specific molecules including TNF alpha is awaited.22 Another novel approach that is showing promise in the management of refractory Wegener's and C-ANCA related vasculitis is the use of Rituximab, a chimeric antiCD20 monoclonal antibody.23,24

Tolosa-Hunt Syndrome

This nonspecific granulomatous inflammation though rare is nevertheless an important differential diagnosis of apical orbital inflammations. The clinical course is marked by remissions and recurrences. SPIR MRI (spectral presaturation with inversion recovery MRI) has been recommended for diagnosis1 Management includes a high dose of systemic steroids which often produces a dramatic clinical improvement. SPIR MRI before and after corticosteroids have been found to be useful in some studies for definite diagnosis and monitoring of the disease.1

Vascular Lesions Capillary Hemangioma

Treatment is indicated when vision is threatened by amblyopia as a result of anisometropia, ptosis or strabismus.

Intralesional injection of steroids is the most frequently used method. Usually 40-80 mg of triamcinolone with 25 mg of methylprednisolone is directly injected into the lesion.1 Alternatively Triamcinolone 40 mg in combination 4 mg betamethasone can be used. The tumor usually begins to regress in two weeks but if necessary injection may be repeated after about two months. Early recognition and prompt treatment with intralesional steroid prevents amblyopia exanopsia, but followup and management of refractive amblyopia with glasses and patching is necessary in the longer term. Potential complications include skin depigmentation, fat atrophy, eyelid necrosis and rarely central retinal artery occlusion.

Systemic steroids are indicated for extensive lesions specially if associated with visceral involvement. Recommended dosage used is 1.5 mg/kg to 2.5 mg/kg Prednisolone daily over a few weeks with titration downward depending on response.1

340 Surgical Atlas of Orbital Diseases

Though steroids are effective in large majority of patients, a recurrence is not infrequent. Recurrent or resistant cases are being treated with recombinant interferon alpha-2a and 2b with variable results16 Recent studies have demonstrated good efficacy of interferons when given subcutaneously in a dose of 3 million units/m2. During clinical follow-up diagnostic ultrasound evaluation ( the depth dimension) proved helpful. One report suggested high efficacy of treatment when a combination of interferon alpha-2a with a low dose of cyclophosphamide.17

In the presence of very large platelet-consuming lesions as seen with Kasabach-Meririt syndrome, systemic antifibrinolytics like aminocaproic acid or tranexemic acid are used.18

Structural Lesions

Acute Intraorbital Hemorrhage and Emphysema

Post-traumatic fractures, soft tissue injury, contusions and retrobulbar blocks may be associated with acute rise in intraorbital pressure as blood is trapped in confined spaces. The effects of such rapid rise in the pressure include optic nerve ischemia and retinal hypoperfusion. The optic nerve head may demonstrate arterial pulsations. Hence the importance of prompt recognition and early management cannot be overemphasized. Though severe visual threat is a surgical emergency, for moderate degrees of orbital tension, treatment includes 500 mg of acetazolamide i.v., and mannitol 1-2 ml/kg i.v over 30 minutes has been advocated.1,19

Orbital emphysema is another cause of acute orbital tension and is almost always secondary to trauma. This rarely requires decompression as the air tends to absorb rapidly. Though most of the patients are managed with antibiotics, prophylactic use is usually not required for clean wounds.20

Lymphoproliferative and Other Neoplastic Lesions

These disorders encompasses a wide range of clinical syndromes. The advent of immunodiagnostics and molecular techniques had a profound effect on better understanding of pathogenesis and therapeutic

advances. Among the lymphoproliferative lesions, reactive lymphoid hyperplasia appears to be steroid sensitive as it responds to moderate doses of Prednisolone. Failure to respond to steroids can be managed by cytotoxic agents and low dose radiotherapy.1

Another clinicopathological entity; the indeterminate lymphoproliferative lesions are steroid resistant and may require treatment with immunosupressives or radiotherapy.1 The widespread use of chemotherapy for lympho-proliferative lesions and other neoplastic conditions is being dealt in detail in a separate chapter.

CASE ILLUSTRATIONS

Case 1

Mrs.K, female 54 years presented with acute, painful proptosis of left eye of 5 days duration. She had severe pain, nausea and mild fever. There was a very severe edema of the lids and periorbital edema (Figure 24.1A). The upper lid had complete ptosis. On everting the upper lid, the globe was found to be proptosed. The conjunctiva was congested and chemosed. Ocular motility was restricted (Figures 24.1B and C).CTscan showed orbital cellulitis without any abscess. She was given intravenous (Amoxicillin and clavilanic acid) and Metronidazole, with which she showed a marked improvement within 5 days. She was relieved from pain, proptosis reduced, and the ptosis improved markedly( Figure 24.1D) The conjunctival chemosis and congestion improved and the ocular motility restored to normal (Figures 24.1E and F).

Figure 24.1 A: Female 54 years presented with acute, painful proptosis of left eye. Note the severe edema of the lid and periorbital edema, and the gross ptosis

Case 2

Female 17 years, presented with proptosis of right eye associated with mid pain since 1 month. There was no history of trauma, defective vision or diplopia. Examination revealed mild proptosis of the right eye with fullness of right upper lid in the supero-temporal region (Figure 24.2A). CTscan of

the orbit revealed enlarged lacrimal gland molding to globe (Figure 24.2 B). The possibility of lymphoma was thought off. FNAC and immunohistochemistry were negative for lymphoma. Hence, a diagnosis of nonspecific orbital inflammation, involving the lacrimal gland was made; the girl was treated with systemic steroids to which she responded well (Figure 24.2C).

Figure 24.2 A: Note the fullness at the supero-temporal region of the right upper lid, with mild displacement of the globe and minimal ptosis

Figure 24.2B: Coronal section of CT scan of orbit showing enlarged lacrimal gland molding to the globe

Figure 24.3A: Male 28 years, presented with severe ptosis of right upper eye lid and mild proptosis of right eye

Figure 24.3B: Note the restricted adduction in the right eye

Figure 24.3C: Note the restricted depression in the right eye

Case 3

Male 28 years presented with proptosis of right eye, subacute in onset and associated with mild pain and defective vision( Figure 24.3A) On examination, he had mild proptosis associated with ptosis, and restricted ocular motility (Figures 24.3B to D).

Figure 24.3D: Note the restricted abduction in the right eye

E

F

Figures 24.3E and F: CT scan, Axial and sagital sections of the orbit show a hyperdense lesion abutting the optic nerve at the orbital apex. Its margins are indistinct. Axial section of the CT shows enlarged superior ophthalmic fissure

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Medical Management of Proptosis 343

8.Nithyanandam S, Jacob MS, Battu RR, Thomas RK, Correa MA, D'Souza O. Rhino-orbito-cerebral mucormycosis. A retrospective analysis of clinical features and treatment outcomes Indian J Ophthalmol. 2003;51(3): 231-6.

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16.Nolle B, Coners H, Duncker G. ANCA in ocular inflammatory disorders. Adv Exp Med Biol 1993;336: 305-7.

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18.Neidhart JA, Roach RW. Successful treatment of skeletal hemangioma and Kasabach-Merritt syndrome with aminocaproic acid. Am J Med 1982; 73: 434-8.

19.Rootman J, Stewart B, Goldberg RA. Orbital Surgery: A conceptual approach. Philadelphia: Lippincott-Raven, 1995.

20.Fleishman JA, Beck RW, Hoffman RO. Orbital emphysema as an ophthalmologic emergency. Ophthalmology 1984; 91:1389-91.

21.Selamet U, Kovaliv YB, Savage CO, Harper L. ANCAassociated vasculitis: new options beyond steroids and cytotoxic drugs. Expert Opin Investig Drugs. 2007;16(5): 689-703.

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