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

Figure 8.27: Photomicrograph showing histiocytes with lymphocytes and plasma cells in Rosai-Dorfman disease ( x 40, Hematoxylin Eosin)

Figure 8.28: Photograph showing downward displacement of right eyeball, with proptosis in a patient with Juvenile Xanthogranuloma

with Rosai Dorfman syndrome (Figure 8.27). Patient was seen by oncologist who advised radiation to the orbit. Patient refused treatment and was lost to follow-up. She came back one and a half years later with double vision and massive proptosis. There was increased retrobulbar resistance. Disc edema was seen on fundus evaluation. In view of the huge size of the lesion and the high dose of radiation that would be required with its potential complications, it was decided to perform a surgical debulking. Intraoperatively, a firm grey tumor was seen infiltrating the inferior orbit. Inferior rectus was densely adherent to it. Patient was advised tapering dose of oral steroids. Patient was free of symptoms on 2 years of follow-up.

Case 9

A male child, age 1 year, presented with prominence of right eye since last 20 days. There was no pain, redness, fever or weight loss. On examination, there was downward displacement of right eyeball with 4 mm proptosis (Figure 8.28). Resistance to retropulsion was felt. Restriction of elevation and abduction was seen. Fundus examination revealed mild disc edema with dilated tortuous retinal veins. CT scan revealed a large slightly hyperdense retrobulbar mass with excavation of medial wall of orbit (Figure 8.29). The mass was removed piecemeal by anterior orbitotomy. Histopathological examination showed features of juvenile xanthogranuloma (Figure 8.30 ). He was started on tapering dose of

Figure 8.29: CT scan showing a large slightly hyperdense retrobulbar mass with excavation of medial wall in the patient with Juvenile

Xanthogranuloma

oral steroids. On 6 months followup, complete resolution of proptosis was seen. He was asymptomatic at follow-up of 3 years.

Case 10

A young female of age 35 years, presented with painless progressive protrusion of both eyes since five years. She had occasional double vision. She had been treated with oral steroids off and on with dramatic improvement in symptoms, and recurrence on stopping the same. She had received anti

tuberculosis therapy earlier. She also reported dryness of the mouth. On examination, her best corrected visual acuity was 6/36, N36 and 6/24, N6 in the right and left eye respectively.Axial proptosis was noted with firm palpable orbital masses in both orbits, most prominent in the superonasal orbit.Ocular movements were restricted in all gazes.There was no lagophthalmos (Figure 8.31).

Slit lamp examination revealed reduced tear meniscus, superficial punctuate keratopathy and posterior subcapsular cataract in both the eyes. Fundus examination revealed striae in the right eye and choroidal folds in the left eye. Bilateral parotid enlargement was noted on both sides. Thus the

possibility of Sjogren’s syndrome with underlying autoimmune disease was considered. CT scan of the orbit revealed bilateral diffuse illdefined extra and intraconal soft tissue with clumps of calcification within (Figures 8.32A and B). Transeptal orbital biopsy was done under general anesthesia. Histopathological examination suggested xanthogranulomatous inflammation. Medical oncology and dermatology opinion was sought. In view of side effect of long term steroids, patient was started on oral anti-inflammatory drugs and steroid sparing immunosuppressive therapy (Cyclophosphamide, Endoxan 50 mg daily), with biweekly monitoring of WBC and platelet counts. Surgical debulking was not

Figure 8.30: Photomicrograph shows numerous Toutan giant cells, (Hematoxylin Eosin × 40 ) suggestive of Xanthogranuloma

Figure 8.31: Photograph showing bilateral proptosis in a patient with Xanthogranuloma orbit

considered in view of potential risks. Oral antiinflammatory drugs had to be stopped after a month due to increase in dry eye symptoms. On last followup, patient was symptomatically better.The was considerable decrease in proptosis and improvement of extraocular motility. Patient was symptomatic due to dry eyes, but had much reduced proptosis and orbital symptoms.

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ACKNOWLEDGEMENTS

We are grateful to Dr J Biswas and Dr S Krishna Kumar, Ocular Pathology Service, Sankara Nethralaya for their help with the photomicrographs and Dr Veena Noronha, Radiology Service, Sankara Nethralaya for help in the interpretation of radiologic images. We are also grateful to Dr Nirmala Subramaniam, Emeritus Professor of Oculoplasty, Sankara Nethralaya for providing some of the photographs.

The Lymphoproliferative diseases, including malignant lymphoma, are not a single disease entity but a collection of disorders ranging from benign reactive hyperplasia, atypical lymphocyte infiltrate to malignant lymphoma.1 Some consider idiopathic orbital inflammation (pseudotumor) as part of the lymphoproliferative disease spectrum.1 Approximately 75% of patients with purely orbital lymphoma will develop systemic disease, whilst 1-5% of those with systemic disease will have orbital involvement.1 Overall orbital lymphoma accounts for less than 1% of all lymphoma.2 Interestingly those patients with atypical orbital lymphocytic infiltrate are at an increased risk of systemic lymphoma.1

Various classifications of Lymphomas are in vogue. They include the following:

Revised European American Lymphoma Classification (REAL Classification)3

1.Leukemias and Lymphomas of B-cell Origin (Pan B CD 19,20+)

A.Indolent B-cell malignancies:

(i)Small lymphocytic lymphoma

(ii)Hairy cell leukemia

(iii)Follicular lymphomas

(iv)Lymphoplasmacytoid lymphoma

(v)Marginal zone lymphoma.

B.Aggressive B-cell malignancies:

(i)Diffuse large cell lymphoma

(ii)Follicular large cell lymphoma

(iii)Mantle cell lymphoma

(iv)Burkitt's lymphoma

(v)Plasmacytoma / Myeloma

2.Leukemias and Lymphomas of T-cell Origin (CD 2, 7+)

A.Indolent T-cell malignancies:

(i)T-CLL

(ii)Cutaneous T-cell lymphoma (Sezary syndrome)

B.Aggressive T-cell malignancies:

(i)Peripheral T-cell NHL

(ii)Angioimmunoblastic T-cell lymphoma

(iii)Intestinal T-cell lymphoma

(iv)Adult T-ALL

WHO Classification of NHL

1.B-cell Neoplasms

A.Precursor B-cell ALL

B.Mature B-cell malignancies:

(i)B-cell CLL

(ii)Plasmacytoma

(iii)Extranodal marginal B-cell lymphoma

(iv)Mantle cell lymphoma

(v)Follicular lymphoma

(vi)Diffuse large B-cell lymphoma

(vii)Burkitt's lymphoma

(viii)B-cell promyelocytic leukemia

(ix)Hairy cell leukemia

(x)Lymphoplasmocytic lymphoma

(xi)Monocytoid B-cell lymphoma.

2.T-cell Neoplasms

A.Precursor T-cell ALL

B.Mature T-cell malignancies:

(i)Mycosis fungicides

(ii)Adult T-cell lymphoma

(iii)Anaplastic or Null cell lymphoma

(iv)Peripheral T-cell lymphoma, not specified

(v)T-cell prolymphocytic leukemia

(vi)Aggresive NK cell leukemia

(vii)T-cell gruanular lymphocytic leukemia.

Modified Rye's Classification of Hodgkin's

Lymphoma4

1.CLASSIC HD:

A.Lymphocyte-predominance

B.Nodular sclerosis

C.Mixed cellularity

D.Lymphocyte depletion.

2.Nodular Lymphocyte-predominant HD

Clinically it is difficult to differentiate the malignant and non-malignant tumors since age, sex, presenting symptoms and radiographic findings are similar. Even utilising immunohistochemical and molecular biology techniques it is difficult to separate the conditions.1 Polymerase chain reaction is a method of amplifying target genetic material in tissue samples to identify a gene re-arrangement diagnostic of lymphoma. This is particularly useful when the microscopic morphology resembles chronic inflammation, but the clinical presentation is suggestive of lymphoma.5

Orbital lymphoma is typically a non-Hodgkins B-cell lymphoma arising from mucosa-associated lymphoid tissue (MALTOMA)1,6 and accounts for approximately 10-15%1,7 of all orbital masses. When only malignant masses are taken in to account, they account for 55% of the lesions.1 Other types of lymphoma identified in the orbit include extranodal marginal zone lymphoma (MZL), follicular (FL), diffuse large B-cell (DLBCL), mantle cell (MCL), B- cell chronic lymphocytic leukaemia (CLL)/small lymphocytic lymphoma, peripheral T-cell lymphoma (PTCL) and natural killer cell lymphoma (NKCL).8

Orbital lymphoma tends to be a bilateral disease and can affect the conjunctiva, lacrimal gland, be found in the nasolacrimal duct, as well as intraconal and extraconal space.1,9 The orbital lesions tend to present insidiously with painless proptosis in the sixth

Orbital Lymphoma 147

decade,1,7,10 whilst visual impairment is relatively rare occurring in only 13% of patients.7 It can rarely present like acute orbital inflammation or cellulitis. MZL has the lowest risk of accompanying extraorbital disease and consequently, the lowest risk of lymphoma-associated death.3

Radiographically orbital lymphomas tend to be homogenious in nature and mould themselves around orbital structures such as the globe and optic nerve.1,9 Bone erosion is rare although bone destruction can occur with aggressive tumors.7

Radiotherapy is an effective treatment for orbital MALT lymphoma using doses in the order of 30Gy.11,12 The 5 and 10 years survival rates for MALT lymphoma is 100% and 88%.12

Orbital inflammatory disease include a wide spectrum of conditions ranging from idiopathic orbital inflammation to orbital involvement of specific systemic inflammatory disorders such as Wegener’s granulomatosis, sarcoidosis, systemic lupus erythematosus (SLE) or Tolosa Hunt syndrome.13 In the case of Wegener’s granulomatosis and sarcoidosis ocular involvement occurs in approximately 50% of affected subjects. The inflammation may affect multiple or localized orbital tissues and can involve the sclera.13 Investigations are therefore guided towards identifying these specific diagnoses. These include a battery of blood tests including full blood count (FBC), urea and electrolytes (U&E), C reactive protein (CRP) and/or erythrocyte sedimentation rate (ESR), autoantibody screen, anti-nuclear antibody screen (ANA), ANCA, rheumatoid factor (RhF), thyroid function tests (TFT), thyroid peroxidise antibody screen and serum ACE. Radiological tests include a chest X-ray, and orbital imaging (CT and/ or MRI). An orbital ultrasound scan can be beneficial.

Where no underlying cause can be identified the diagnosis of idiopathic orbital inflammation can be made.

When to Suspect Lymphoma

•Age of onset: more common in the elderly

•Insidious onset

•Bilateral disease with no evidence of indentation of the globe (choroidal folds)

•Lacrimal gland involvement, lesions are typically firm/rubbery on palpation

148 Surgical Atlas of Orbital Diseases

•Conjunctival involvement, lesions are typically salmon colored

•Orbital imaging will reveal the lesion to be moulded around the globe.

When to Suspect Idiopathic Orbital Inflammatory Disease

•Onset is usually acute

•Symptoms include; pain, erythema, proptosis, diplopia and blurred vision

•Involvement of the extraocular muscles and the sclera (scleritis)

•Differential diagnosis includes; orbital cellulitis, a systemic inflammatory disease such as Wegeners granulomatosis, sarcoidosis, polyarteritis nodosa and neoplasms such as lymphoma

•Investigations include; FBC, U & E, CRP, antibody screen, serum ACE, ANA, ANCA, RhF, thyroid function and thyroid peroxidase antibodies

•Imaging; orbital CT and/or MRI scan, ultrasound scan to rule out scleritis and a chest X-ray where sarcoidosis is suspected.

CASE ILLUSTRATIONS

Case 1

A 52 years old diabetic female presented to the orbit clinic with enlargement of the left lacrimal gland. Examination revealed proptosis of 5 mm on the left side, whilst oculomotility was full, there was no RAPD and funduscopy was unremarkable. Routine bloods were taken to rule out autoimmune conditions and inflammatory conditions such as Wegner's granulomatosis, systemic lupus erythematosus (SLE), Sjorgren's syndrome, and sarcoidosis. These were all negative. An urgent CT scan revealed a discrete soft tissue mass arising from the left lacrimal gland (Figure 9.1) extending in to the orbit and displacing the lateral rectus muscle. The right orbit was normal. The differential diagnosis included plemorphic adenoma and lymphoma.

Excision biopsy via a lateral orbitotomy approach was performed since pleomorphic adenoma was suspected.

Initial examination of the biopsy specimen identified a dense exudate of lymphoid cells forming prominent germinal centers. The appearance was

Figure 9.1: Left lacrimal gland enlargement

suggestive of reactive lymphoid change. Subsequent immunohistochemistry identified T and B cell proliferation and in one specimen kappa light chains could be identified. A low grade lymphoma was suspected. Molecular genetics confirmed the diagnosis of low grade B cell lymphoma.

The wounds settled well postoperatively and vision was maintained at 6/9 in the left eye and 6/5 in the right. A referral was made to the lymphoma service for a course of radiotherapy. There was no evidence of systemic lymphoma. 40 Gy was given to the right orbit in 20 fractions.

Postradiotherapy she suffered from a dry eye and was prescribed lubricants. A combination of radiation and diabetic retinopathy subsequently developed. However there was no evidence of recurrence at 6 years.

Case 2

A 31 years old male attended the eye casualty with a 3 weeks history of bilateral orbital inflammation (Figure 9.2) and right sided proptosis (Figure 9.3). Visual acuity was 6/5 in both eyes and IOPs were normal. Examination revealed bilateral conjunctival chemosis, associated with reduced upgaze and abduction. Fundoscopy was unremarkable. Routine bloods were taken to rule out inflammatory disorders. C-reactive protein and plasma viscosity were elevated as was the white cell count. An urgent MRI scan was arranged which revealed bilateral enlargement of the lacrimal glands (Figure 9.4) suggestive of lymphoma.

Urgent lacrimal gland biopsy was performed.

Histology confirmed the diagnosis of Hodgkin’s lymphoma and referral to the lymphoma service was made.

Figure 9.2: Bilateral orbital inflammation

Figure 9.3: Right sided proptosis

Figure 9.4: Bilateral lacrimal gland enlargement

Case 3

A 63 years old male was referred to the eye casualty with a 3 month history of diplopia and right sided proptosis. Onset had been gradual and visual acuity was 6/9 in both eyes. Examination revealed 3 mm of proptosis in the right eye which was associated with reduced ocularmotility in all directions of gaze. Intraocular pressure was normal and there was no evidence of an RAPD. Fundoscopy revealed evidence of choroidal folds on the right although both discs were healthy. There was no associated ocular pain or headache and there was no history of weight loss, fever or cough. He was an ex-smoker having stopped 6 years previously. There was no significant past medical history. Routine bloods were taken to rule out systemic causes. Urgent CT and MRI scans were performed, revealing orbital inflammation suggestive of lymphoma.

An urgent orbital biopsy was performed within 3 days and the patient started on a reducing dose of steroids postoperatively. Histology was inconclusive

Orbital Lymphoma 149

revealing only chronic inflammation. Lymphoma was still suspected, and after a discussion with the patient, a more extensive orbital biopsy was performed. Histology was once again inconclusive, revealing only scant lymphoid exudates. Subsequent immunohistochemistry identified B and T cells. No evidence of lymphoma was found.

Referral was made to the lymphoma team to rule out systemic lymphoma. None was identified. The patient continues to be followed up.

Case 4

A 26 years old female presented to eye casualty with a three day history of right orbital swelling. Visual acuity was 6/9 in the right eye and 6/6 in the left. There was marked chemosis (Figure 9.5) and proptosis of the right eye associated with and reduced upgaze and adduction. Fundoscopy was normal and there was no evidence of papilloedema. No RAPD was noted. The patient was apyrexial. A diagnosis of orbital cellulitis was made and appropriate treatment commenced. CT scan of the orbits revealed preseptal and orbital inflammation with evidence of mild proptosis. The extraocular muscles and optic nerve were normal and sinuses clear. There was no evidence of any abscess formation. An orbital ultrasound scan was normal.

Despite intravenous antibiotics no improvement in the symptoms was seen. Referral was made to the orbital service and idiopathic orbital inflammation diagnosed. Routine bloods were taken to rule out sarcoidosis, Wegeners granulomatosis, and syphilis, along with an auto-antibody screen, full blood count, thyroid function tests and C-reactive protein screen (CRP). All these tests were normal other than an elevated CRP. Systemic steroids were commenced (Prednisolone 1mg/kg) along with a histamine H2 receptor antagonist. Over the next few days the symptoms and eye movements started to resolve.

Figure 9.5: Right sided chemosis and lid edema

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The patient was discharged home on a reducing dose of steroids. Urgent immunology and rheumatology out patient appointments were arranged. No underlying immunological or rheumatological abnormalities were detected.

Three months following the initial episode, the patient returned to eye casualty with a flare up in the left eye, and then a further six months and two years later in the right eye. All episodes settled with a short course of systemic steroids. Biopsy is normally recommended for all suspected idiopathic orbital inflammation but in this particular patient the features were typical with no localising lesion so a biopsy wasn't performed.

Surgical Approach

Surgery is generally indicated to obtain tissue for histology and to aid diagnosis of suspicious lesions. Incisional biopsy is the treatment of choice.

Incisional Biopsy

The lateral 1/2 of the upper lid skin crease is marked with pen and extended down parallel to the lid margin, level with the lateral canthus, where it is extended in a horizontal plane just past the orbital rim. Local anesthetic is injected subcutaneously. A skin incision is made along line with a cutting diathermy. Both the palpebral and orbital parts of the lacrimal gland are identified and an incisional biopsy is made. Any other suspicious lesions are also biopsied and sent for histology.

The deep tissues are closed with 5.0 vicryl and skin closed with 6.0 prolene.

Suspicious visible conjunctival (bulbar and palpebral) and sub-conjunctival lesions should also be biopsied.

A reducing dose of steroids is given for 18 days along with a histamine H2 receptor antagonist such as ranitidine.

A head-light can be worn throughout the procedure to ensure adequate illumination of the operating field.

REFERENCES

1.Akansel G, Hendrix L, Erickson BA, et al. MRI patterns in orbital malignant lymphoma and atypical lymphocytic infiltrates. Eur J Radiol 2005;53(2):175-81.

2.Norton AJ. Monoclonal antibodies in the diagnosis of lymphoproliferative diseases of the orbit and orbital adnexae. Eye 2006;20(10):1186-8.

3.Jenkins C, Rose GE, Bunce C, et al. Histological features of ocular adnexal lymphoma (REAL classification) and their association with patient morbidity and survival. Br J Ophthalmol 2000;84(8):907-13.

4.Schnitzer B. Classification of lymphomas. CRC Crit Rev Clin Lab Sci. 1978;9(2):123-78. Review.

5.Coupland SE, Krause L, Delecluse HJ, et al. Lymphoproliferative lesions of the ocular adnexa. Analysis of 112 cases. Ophthalmology 1998;105(8):1430-41.

6.White WL, Ferry JA, Harris NL, Grove AS, Jr Ocular adnexal lymphoma. A clinicopathologic study with identification of lymphomas of mucosa-associated lymphoid tissue type. Ophthalmology 1995;102(12):1994-2006.

7.Selva D, Rootman J, Crompton J Orbital lymphoma mimicking optic nerve meningioma. Orbit 2004;23(2):11520.

8.McKelvie PA, McNab A, Francis IC, Fox R, O'Day J Ocular adnexal lymphoproliferative disease: a series of 73 cases. Clin Experiment Ophthalmol 2001;29(6):387-93.

9.Sullivan TJ, Valenzuela AA Imaging features of ocular adnexal lymphoproliferative disease. Eye 2006;20(10): 1189-95.

10.Demirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC Orbital tumors in the older adult population. Ophthalmology 2002;109(2):243-8.

11.Bhatia S, Paulino AC, Buatti JM, Mayr NA, Wen BC. Curative radiotherapy for primary orbital lymphoma. Int J Radiat Oncol Biol Phys 2002;54(3):818-23.

12.Hasegawa M, Kojima M, Shioya M, et al. Treatment results of radiotherapy for malignant lymphoma of the orbit and histopathologic review according to the WHO classification. Int J Radiat Oncol Biol Phys 2003;57(1):172-6.

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

To comprehend the vascular lesions of the orbit, it is very important to have embryological, pathological and clinical concepts very clear in mind. There have been numerous ways of classifying these lesions, the most common being to divide them into malformations, shunts and new growths.1 Many entities have been placed under these three headings which we will discuss in detail.

MALFORMATIONS

Malformations are present since the time of birth, though they may not manifest at that time. Flat endothelium lines their wall, and in contrast to neoplastic lesions, do not show any growth in-vitro. The orbital society has classified malformations as:2

enlarge during the upper respiratory infections probably due to the inflammatory response of the lymphoid tissue within the lesion.3

Superficial lymphangiomas are lesions of the lid or conjunctiva, readily visible on inspection as multiple serous or blood filled cysts. These are usually purely lymphatic in character. Indication for management is due to cosmetic reasons and can be removed easily.4

Deep Lymphangiomas have in addition venous connections and may cause slowly progressive proptosis. They may present with increase in size during upper respiratory infection. It can also present with sudden proptosis due to bleeding into its lumen causing a chocolate cyst. A significant number of these patients may present with signs of optic nerve

Varices.

c.Arterial flow malformations. For example: Cavernous hemangioma.

d.Other congenital malformations. For example: Phakomatosis.

Lymphangioma

These are benign vascular lesions seen usually in the early childhood and commonly confused with orbital venous anomalies and hemangiomas. Though they are hemodynamically isolated, they arborize the orbit and bleeding into their lumen causing chocolate cyst is not very uncommon. Lymphangiomas often

Imaging modalities used include Ultrasonography, CT or MRI. Of these MRI is the diagnostic modality of choice.1 USG demonstrates cystic masses in the retrobulbar space. CT shows low density masses in intra and extraconal compartments with minimal ring enhancement on contrast. No vascular component is noted on angiography. MRI is useful to delineate the lesions well and is also helpful in timing the chocolate cyst as being acute, subacute or chronic which may have clinical implications.5

Management is usually conservative since spontaneous regression of the cysts is common. Surgery though ungratifying because of incomplete

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removal and recurrences, should still be carried on in the presence of signs of optic nerve compression. Carbon dioxide and contact Nd:YAG lasers are useful surgical adjuncts.6

Other recent modalities that are gaining upperhand in the initial management includes the use of sclerosing agents. Many sclerosing agents in use include Picibanil (OK-432)7,8 percutaneous ethanol9 and bleomycin.10 Certain other agents like 5% sodium morrhuate11 and sodium tetradecyl sulfate,12 considered to be more effective by some for the management of low flow vascular lesions have been recommended as the first line therapy for lymphangiomas.11

Orbital Varices

Orbital varices are weakened, dilated segments of orbital venous system. Age at presentation varies from childhood to middle ages .Most of the cases are unilateral and upper nasal quadrant is the favoured site. Clinical signs include visible lesions in the eyelid or conjunctiva, or the patient may present with a non-pulsatile proptosis which is accentuated with increasing venous pressure like while straining, assuming a dependant posture like sitting with a head down position or by a valsalva maneuver. Since the orbital venous channels are devoid of valves, a reversible proptosis occurs.13 Rarely varices may threaten the vision by optic nerve compression due to acute hemorrhage or thrombosis. Chronic lesions may present as enophthalmos.14

Imaging modalities used include CT scan, Doppler ultrasonography and angiography. Doppler demonstrates the flow of blood. Rapid spiral CT during valsalva maneuver shows characteristic enlargement of the engorged varix. Uniform contrast enhancement is seen. Sometimes phleboliths may also be seen. Angiography shows connection of the lesion to the venous system and completely fills up following injection.1

Management is usually conservative. But in the presence of signs of optic nerve compression, surgical removal is attempted .Complete removal is usually not possible since the lesions are friable, unencapsulated and bleed easily. Embolization using coils through a distal vein is another method to diminish symptoms.13

Cavernous Hemangioma

Cavernous hemangioma is the most common benign orbital tumor in adults predominantly affecting middle aged females. Most frequently it develops in the intraconal space though it may also develop elsewhere in the orbit.15

The patients present with slowly progressive unilateral axial proptosis which may be associated with decreased visual acuity, hyperopia, optic nerve compression, optic disc edema, choroidal folds and gaze-evoked amarousis, raised intraocular pressure and strabismus. Bilateral cavernous hemangiomas have also been reported.16,17

Imaging modality used commonly is a CT scan which shows a well defined intraconal mass with smooth margins that enhances either homogenously or inhomogenously with intravenous contrast. Sometimes small areas of calcification are seen.18 On MRI the lesion is isointense and hyper intense to the muscle on T1 and T2 weighted images respectively. With Gadolinium contrast, the lesion fills up homogenously.19

Management is surgical excision Lateral orbitotomy is the most common approach if the lesion is intraconal. Anterior orbital approaches are useful for extraconal lesions. Surgical removal is much simpler, since cavernous hemangiomas are well encapsulated. Cryo is a very useful adjunct. In very large lesions, passing a suture through the lesion helps in two ways, for exsanguination of the tumor reducing its size and to hold the tumor.

Other Congenital Malformations

Many other congenital malformations commonly termed as phakomatosis and include 'Sturge-Weber Syndrome', 'Wyburn-Mason Syndrome', 'KlippelTrenaunay Syndrome', 'Osler-Weber-Rendu Syndrome' and many more rare malformations.1

Sturge-Weber Syndrome: It is also called as encephalofacial angiomatosis and is a sporadic phakomatosis that involves the leptomeninges, brain and eyes. Struge-Weber is characterized by a naevus flammeus or port wine stain over the area of trigeminal nerve distribution, ipsilateral leptomeningeal hemangiomas and contralateral seizures and hemiparesis. Ophthalmological features