Tumors affecting the orbit include benign and malignant neoplasms arising from the various structures in the orbit including the blood vessels, nerves, bones, orbital fat and other soft tissues. The first step in the management of orbital tumors is to differentiate the malignant tumors from the benign ones. Complete surgical excision remains the primary treatment of choice for well-defined, clinically benign orbital tumors. On the other hand, for a suspected malignant tumor, an incision biopsy is the first step in the management to obtain a histopathologic diagnosis before a definitive therapy can be planned. Each orbitotomy technique is designed to provide the best surgical exposure to the lesion of interest, while causing least damage to the orbital structures, with a goal to achieve optimal functional and cosmetic outcome. The concept of minimally invasive surgery inherently includes hidden incisions and minimal dissection. There are variety of surgical approaches to the orbit. Having a thorough knowledge on the orbital anatomy and detailing out the size and extent of the lesion using pre-operative imaging can help the surgeon decide on the right approach.
Lecturer: Dr. Santosh G. Honavar, Director, Medical Services, Centre For Sight, Hyderabad, India
Dr. Santosh G. Honavar: Good evening, I’m Dr. Santosh Honavar. I am from Hyderabad, India. And I’ll be speaking to you today about logical approach to orbital tumors. Orbit is supposed to be Pandora’s Box, full of evil because 50% of orbital tumors are supposed to be malignant, and hence potentially life threatening. So, when we deal with orbital tumors, the need is to have a logical approach where oncological principles are respected. When you have a patient with orbital tumor, we have to ask these questions and answer ourselves.
What is it likely to be? Is it a benign tumor or a malignant tumor? Where is it located, Intraconal, extraconal. What are its relationship? Is it related to any of the crucial structures such as extraocular muscles or the optic nerve? Is it operable it all or not? If so, what approach? Would it be transconjunctival? Would it be lateral orbitotomy? What do I expect out of it? Do I expect to remove the entire tumor without causing any complications or would I just want to do any additional biopsy?
To answer these questions, we have to have very logical stepwise approach, starting with a good clinical history, followed by regional examination including the orbit. Then a targeted systemic evaluation followed by imaging. It could be ultrasound, CT scan or MRI as appropriate. Then specific systemic investigations followed by surgery. And last but not the least, histopathology because that guides further into treatment. I’m not going to go into the details of clinically evaluation of the orbit but just to emphasize on the logical approach, history should be very detailed.
These are the six piece of orbital proptosis as for example, including history of pain, progression, onset which could be acute in days, subacute in weeks, chronic in months to years, progression could be rapid, slow or indolent then you measure proptosis, measure displacement, palpate the orbit, look for pulsation and look for periorbital changes.
Assessment of proptosis extremely important, it should be objective by instruments such as Hertel Exophthalmometry, displacement has to be objectively measured because all these primary baselines help in further follow up of the patient. Ocular motility has to be very carefully assist and documented with photographs and also a diplopia charting. In Palpation, there are several factors that we need to enlist it. Tenderness, Location of the tumor, mobility if it is anterior, compressibility, Retropulsion, shape, surface, consistency, what about the orbital margin and do we feel any pulsation, a thrill or here a Bruit.
Not to forget the rest of the ocular examination including visual acuity, color vision, assessment of the pupil for Relative Afferent Pupillary Defect, Schirmers specifically for Lacrimal Gland Tumors, basic ocular examination, specific cranial nerve examination and if you think that the tumor is a secondary one, rising from the nasal cavity or Paranasal Sinuses then we have to do oral and nasal examination as well. This is followed by CT scan or MRI as appropriate. So, at the end offer careful clinical evaluation and radiological investigations, we come to a clinical radiological diagnosis.
Before I go into it, orbital tumors are primarily classified as benign and malignant primary tumors or secondary tumors. These are the primary tumors. There’s the list off primary tumors not in any particular order. Cystic Lesion are not tumors but they come in the realm of oncologists because they referred get referred to in ocular oncologist or Oculoplastics specialists. Vascular [indiscernible] [00:04:22] of tumors, optic nerve and meningeal tumors, Myogenic, Fibrous Connective-Tissue tumors, Osseous and Fibroosseous tumors, Histiocytic, primary Melanocytic and tumors of the lack of the Lacrimal gland.
Where, in whichever geographic location that you already heard a lot, epidemiology of tumors in that particular location because that affect your clinical diagnosis. If a particular type of tumor is more predominant in your given geographic location and that has to be built into your analysis of a differential diagnosis. This is in India-Asia where you find Lymphoproliferative, Vascular, secondary and cystic lesions are almost as common, 14%, about 14% followed by neurological – lesion, Inflammation, mesenchymal and Lacrimal gland tumor about 7% to 9%. So this is just an example, this year to know for your own geographic area.
In congenital lesion, if a child were to be born with proptosis or prominent of one of the eyes. There could be many reasons. If the lesion were to be located in inferior orbit without a visible eyeball or if the other eye want to have an Iris or [indiscernible] [00:05:35] use a spectacle of the Colobomatous Cyst. Here the most important first step of after [indiscernible] [00:05:42]. In Children, whenever we classified proptosis or [indiscernible] [00:05:50] diagnosis, it is based on the age of the individual and also the type of proposes.
In Children, the most common cause for Axial Proptosis is Optic Nerve Glioma. Optic Nerve Glioma is the most common cause of Axial Proptosis. In a Abaxial, we the classify into subacute and chronic. Subacute causes for the Abaxial Proptosis could be [indiscernible] [00:06:18], Leukemia, and [indiscernible] [00:06:20]. Chronic Abaxial Proptosis are caused by lymphangioma and orbital dermoid. This is a Unilateral Optic Nerve Glioma, you see despite a largish mask which is located intraconal posterior, the child has very certain problems. This children had asymptomatic, very shuttle, very slowly involving proptosis because the lesion is a very slowly evolving benign lesion.
It could even be bilateral. You see here a Bilateral Optic Nerve Glioma in a child with Neurofibromatosis. Example of Abaxial proptosis in a child which is subacute that [indiscernible] [00:07:04] located in the superior orbit extracoronally pushing the eye down and also causing closest because of the mechanical component and also because of the fact that the tumors is affecting the [indiscernible] [00:07:17]. And in the phonics, you find a fleshy lesion is about chart variant of [indiscernible] [00:07:23]. This is one more example of Abaxial Proptosis in a young child, subacute because of a Rhabdomyosarcoma in the inferior orbit with anterior [indiscernible] [00:07:33].
Example of Abaxial Proptosis in a six-year old child with subconjuctival hemorrhage and [indiscernible] [00:07:40], Granulocytic sarcoma, all you need to do here is a [indiscernible] [00:07:45] which confirms the diagnosis and the child goes through a pediatric [indiscernible] [00:07:49]. This is a PNET [indiscernible] [00:07:52] Clinical manifestation and biopsy is the only motility that can get [indiscernible] [00:08:00] Abaxial Proptosis in a young child, six-year-old child and all scan we find that the lesion is very close to the superior oblique muscle proptosis involving it, very well circumstance. And biopsy, excision biopsy, we found that [indiscernible] [00:08:20] but nevertheless of course for Abaxial Proptosis in a young child. Chronic Abaxial Proptosis could be because of orbital Lymphangioma, in fact this is the most common cause of chronic Abaxial Proptosis in a young child. Orbital Lymphangioma, depending on it’s location, it can cause Axial or Abaxial Proptosis.
This is a child with an anterior Lymphangioma with minor proptosis and displacement of the eye superiorly. Orbital Lymphangioma can also present with acute proptosis because in child hemorrhage which can be very sudden [indiscernible] [00:08:56]. This child was injured with very acute proptosis without any prior history of proptosis at all. And it looked like as if, there was a very acute exacerbation of lesion that was already there, but this patient designed with aspiration of the blood and integration lesion of bleomycin, [indiscernible] [00:09:17] acute bleed inside which was like asymptomatic for about six years before the child [indiscernible] [00:09:24].
Another cause for chronic Abaxial Proptosis would be orbital dermoid. If it’s anterior, of course, there will be no proptosis or displacement of the eye, what if the lesion is posterior or in the mid orbit, there could be displacement of the eye. On these anterior dermoid, whereas dermoid can also be in the mid orbit as you see here. This is in the mid orbit causing displacement of the eye and proptosis. And the lesion could be in the posterior orbit with an [indiscernible] [00:09:56] component in which situation there would be predominant proptosis with minor displacement. So, anterior dermoid will not cause proptosis or displacement, a mid orbital dermoid can cause displacement predominantly, where the posterior orbital dermoid can are can cause both.
Dermoid can also be done with dermoid or can have an extra orbital extension as you see here. Here the patient will come with a temporal for some mass along with proptosis. This is a patient where there is an extra axial extension, where we do a CT scan evaluate the location of the defect in the bone and tackle that during surgery. In adult, well circumscribed mass can be cavernous hemangioma, Neurilemoma, neurofibroma or fibrous histiocytoma. This is the most common differential diagnosis on well-circumscribe tumor in an adult.
The fifth would be a primary orbital melanoma. Cavernous hemangioma typically presents with very mild proptosis despite a large lesion. It’s a very slow growing lesion which grow at the expense of atrophy orbital fat and also accommodate themselves by scalloping the bone or widening on a face in the orbital wards. Especially, the last two ball, you see a large lesion causing very minimal proptosis, [indiscernible] [00:11:20] proptosis caused by a cavernous hemangioma.
One more example of a large cavernous hemangioma causing minimal axial proptosis begin at cavernous hemangioma. So, cavernous hemangioma is the most common cause for a well-circumscribed orbital tumor. Second comes Neurilemoma. Neurilemoma can rise from the inflow orbital nerves [indiscernible] [00:11:42] or any of the nerves in the orbit, if you can leave it arises from the super orbital nerve causing inferior displacement, the eye is [indiscernible] [00:11:50] proptosis, neurofibroma primary neurofibroma of the orbit with systemic neurofibromatosis is known as a patient with orbital neurofibroma. And a benign fibrous histiocytoma is a well-circumscribed lesion as a see here, very slowly growing causing displacement of the eye.
Causes for Axial proptosis in order of their occurance or cavernous hemangioma, Lymphangioma, optic nerve glioma with a late manifestation and Optic Nerve Sheath Meningioma. I already showed you examples of cavernous hemangioma and orbit lymphangioma. Not before and he want to come to you with a history of increasing vision or you find Relative Afferent Pupillary Defect with optociliary shunt vessels into as you see here, then you suspect optic nerve sheath meningioma. When you do a scan, you find that there is a tumor in the optic nerve with calcification of the optic nerve sheath or central calcification of the tumor indicating that there is optic nerve sheath Meningioma.
Examples of Abaxial Proptosis in adult, lymphoproliferative lesion, most common followed by Neurilemoma, lacrimal gland tumors and other structural lesion. This is a very well-circumscribed mass which is molding around the eye. Iso-dense or isointense on CT scan or MRI, causing Abaxial Proptosis turned out to be a non-Hodgkin’s lymphoma B Cell. Another patient with very well-circumscribed lesion molding around the, molding around the eye with anterior orbital extension, see here pink fleshy lesion in the superior phonics of B Cell lymphoma.
Neurilemmoma by, it caused by obviously now with the orbiting flow, orbital nerve and you can see that the eyeball is displaced upward with proptosis. Neurilemoma again caused by the infraorbital nerve in the left eye, you can see that the eyeball is displaced upwards with proptosis. Neurilemoma of the supraorbital nerve you can find on excision that the long stump of nerve has been excised along with the tumor to prevent [indiscernible] [00:14:11] you can find that the patient has a downward displacement of the eye along with proptosis. These are very beautiful neurilemoma which shows that neurilemoma can be multi-focal. This is along the same supraorbital nerve that we found two Neurilemoma. In fact you can find many small neurilemoma along the course of the nerve. So, it’s always a good idea to excise the tumor along with a long stump of the nerve. You can see this is the main tumor along with that you find small little neurilemoma in the course of the nerve.
Lacrimal gland tumors can be classified simply as a epithelial and non-epithelial. Among the non-epithelial Lymphoproliferative b disorders constitute tumor, rest of them constitute only inflammation. In the epithelial tumor, textbooks write that 50% of benign and 50% off malignant. And 50% of malignant tumors are very narcissistic carcinoma. Pleomorphic Adenoma of the lacrimal gland is the most common lacrimal gland Benign tumor in adults. It presents with a supratemporal orbital mass with downward and medial displacement of the eye. A very well-circumscribed lesion with fossa formation in the bone, very characteristic and it can simply be excised. Surgery is easy either by the literacy approach or anterior orbitotomy.
Very rarely, it can cause erosion of the bone because of pressure effect a longstanding lesion. But that should not be confused with a malignant lesion. Well-circumscribed lesion despite erosion of the bone, if it’s extradural if it’s not involved that, not destroyed can safely be excised. Pheolmorphic adenoma of the lacrimal gland is predominantly orbital loop tumor. However, in some patients there could be a palpable of extension. This nodular extension was corresponding with the palpable loop lesion that we could clinically and radiologically see. So, this was a combined Pleomorphic adenoma of the combine palpable loop as well as the orbital loop.
When a lacrimal gland tumor which causes displacement of the eye downwards and medially also extends beyond the midline, and if the patient has paresthesia and sensory symptoms, then you consider adenoid cystic carcinoma. Adenoid cystic carcinoma of the lacrimal gland can occur at any age, in fact, it can occur in teenagers well up to seventh or sixth or seventh decades of life. One more example of adenoid cystic carcinoma here, it can cause bone destruction which can be very irregular. As opposed to pleomorphic adenoma which causes very gentle scalloping of the bone are effacement of the bone. Adenoid cystic carcinoma causes abrupt bone destruction.
Causes for variable positional and pulsatile proposes. In fact these patients can present with enough thalamus as clinical manifestation. But when you ask them to bend or do [indiscernible] [00:17:08] maneuver, the lesion manifest. The eye comes forward and there could be a ballooning up of a lesion in the inferior or superior orbit, a very typical Orbital varices . But if a child were to come to you with or any anybody for that matter even if man did want to come to you with neurofibromatosis type 1 with [indiscernible] [00:17:26] proptosis, then you should look for a meningoencephalocele which is a cause for a pulsatile proptosis.
Going on to further differential diagnosis, a triradiate lesion. Triradiate lesion is typically has an epicenter in the sphenoid wing with orbital temporal fossa and intracranial extension. Five most common triradiate lesions are sphenoid wing meningioma, eosinophilic granuloma, osteosarcoma, ewing sarcoma and metastasis. This is adult patient who comes with cranial exposure. She also has proptosis downward displacement. But what is striking is temporal fossa index, that’s because the lesion has epicenter in the sphenoid wing with an orbital competent, temporal fossa component and an intracranial extension. That’s why it is called a triradiate lesion.
You look at the CT scan of a different patient with sphenoid wings. Meningioma, you find that the lesion is epicenter is here with an orbital intracranial and temporal fossa component causing proptosis with temporal fossa fullness and also displacement of the eye. Eosinophilic granuloma in most present similarly with a sphenoid wing center lesion may not have a predominant temporal fossa extension but surely it does have an intraorbital and then intracranial extension. This is also a triradiate lesion.
Osteosarcoma is a typical triradiate lesion. This a patient with who’s retinoblastoma survivor. In his third decade of life he presented with a triradiate lesion. Ewing sarcoma also causes triradiate lesion. And also metastasis, he just prostate. This patient has a sphenoid wing, metastasis from a prostate malignancy with a triradiate lesion. So, when you have triradiate lesion, you have very few differential diagnosis. There’s an unusual example for triradiate lesion. This a patient with, who’s a known case of multiple myeloma cured of or are in the phase of remission of multiple myeloma but suddenly presents with her proptosis with downward displacement.
When we look at this scan, we find lesion centered in the sphenoid wing with orbital intracranial and temporal fossa extension. And here we found that he had punched out lesion on skull X-ray. And all that we needed here was to do a finally last patient cytology to confirm the diagnosis that reports plasmacytoma. Now once we have a clinical radiological diagnosis, we have come step wise from history clinical evaluation. And we have done a clinical radiological diagnosis based on all the information that we have clinically and radiological. What’s the next step?
This step, we have to take a decision. Do we do surgery at all in this patient? Based on the clinical radiological diagnosis, in some patients it would be a clear no. You say no to surgery. You don’t want to do surgery because of the reasons I will explain in a while. In some patients you would say clear, yes to surgery. Of course you want to do surgery because that seems the only logical management options. In some patients you may want to do surgery, you may want to get into a discussion of the patient as two pros and cons of surgery versus nonsurgical approach. Know what are those.
This is a patient with bilateral proptosis, a child who is just 18 months old. He has temporal fossa fullness with bilateral proptosis and this is a typical manifestations of Metastatic Wilms disease. So, no surgery here, just a systemic a evaluation and the child goes to an oncologist. Exactly similar situation, Bilateral proptosis with raccoon eye sight sphenoid wing center lesion. Typical case of metastatic neuroblastoma. Again, no surgery here, child goes to an oncologist.
Granulocytic sarcoma, patient with proptosis subconjuntival hemorrhage broad spots in the fundus. All you need to do here is peripheral blood smear, not an orbital Biopsy and the child goes to a Pediatric Hammett oncologist. This patient I showed you, plasmacytoma all that we need to diagnose here was a final last patients psychology. So those are the patients clearly are no to surgery. There are some patients where you say no to surgery. But you still want to intervene, non-surgical approach to orbital tumors.
Like this patient who’s an airline pilot has almost no proptosis. But he was failed in the recent medical exam because he had a visual field defect. And when we did an MRI here, we find that there is a cavernous hemangioma sitting exactly at the orbital epics and peeping into the superior orbital fissure causing the visual loss that he had. He had visual field defect caused by this chamber which was pressing on the optic nerve. Cavernous hemangioma of course is surgeon’s dialect. But what do you want to mangle in the area of orbital apex and superior orbital fissure in this patient, whose only known occupation is flying airplanes. It’s very difficult clinical situation because the patient may end up with for the loss of vision, for the loss of visual field or even certain diplopia which may preclude him from carrying out his normal activities. So in such patient we may not want to do surgery, we may not want to do surgery at all and consider other possible options such as stereotypic radiation.
We did that here. And consequently you can see that the tumor has reduced to about 30, 25 to 30% of its initial volume and you find that his visual field has completely returned to normal. And he was back to work. This is reported in the literature as well. This patient who has a small cavernous hemangioma peeping into the superior orbital fissure and at the optic nerve with consequent severe visual field loss was treated as for the literature by Gamma Knife radiation 2000 cGy. His tumor became smaller in size and which visual field defect became totally normal. So, there are nonsurgical options to even benign tumors by means off very creative use of radiotherapy, we can treat some of these tumors.
So, current understanding is that if even if it’s a benign tumor, if it is pear shaped impending on the superior orbital fissure and the optic nerve, you may not want to do surgical treatment. You may want to consider radiation. The next option of course for a orbital tumors is injections. Orbital lymphangioma is no longer treated with surgical excision. This young lady wants cosmosis, she has mild axial proptosis of the right eye and she wants cosmosis. She also has a recurring hemorrhage, mild hemorrhage and also sunconjunctival hemorrhage because of a longstanding orbital lymphangioma. She wants relief from that.
What if you were to do surgical intervention? You can do surgical intervention to double the tumor but it is all around the superior rector’s LPS complex, lateral rectors, middle rectors and the optic nerve. So, you might want to get into the lesion from superonasal and superotemporal orbit aspect. But the chances are that you may end up with a complication even diplopia, even [indiscernible] [00:24:44] for that matter and she doesn’t want any of that. How do you treat it? You can treat by interregional injections such as Picibanil that was used here. You can see nice clearance of the lesion in the intraconal space, nice clearance of the lesion in the extraconal space and she got the cosmosis that she wanted. Her axial proptosis is gone and she’s stable ever since.
So, this was treated by injection Picibanil. If there is a chocolate cyst or a macrocystic lymphangioma then you aspirate Chocolate cyst or microcystic competent of lymphangioma. The blood is aspirated either CT guided or if it’s palpable interiorly or you can get into it by radiological clues, even without a CT guarded, aspiration is fine as long as it’s extraconal. And then inject Bleomycin into the lesion. So, you hold the needle exactly where you were when you aspirated so that you don’t get displaced out of the microcystic competent of the lesion and you inject bleomycin. This patient was treated with injection bleomycin, somebody had attempted a biopsy before the patient was sent to us and it was lymphangioma. So, the residual component of the lesion was treated with injection bleomycin and the child did well.
Does the patient that I showed earlier where the child had an acute proptosis because of interracial hemorrhage? The hemorrhage was drained and the residual lesion was treated with bleomycin. One more patient with an anterior mid orbital lymphangioma treated with multiple injections of bleomycin and the lesion is gone. So, either Picibanil bleomycin works very well in orbital lymphangioma. Anterior lymphangioma again, you can see this lesion treated with injection bleomycin. Now what about eosinophilic granuloma? Eosinophilic granuloma is treated conventionally by curettage. What would you do Curettage in a situation where the Dura is involved, seemingly involved? It may not be anatomically completely involved but there is a possibility of CSF leak if you want do aggressive curettage here.
So, short of it, what we do here is biopsy from a safe zone and hold the child under anesthesia for about 15 minutes while our pathologist confirms the diagnosis by [indiscernible] [00:26:56] inject the lesion with intralesional triamcinolone. So, this patient was injected with triamcinolone and after three months the bone has partially remodeled after six months bone has completely remodeled without any residual lesion. So, using a eosinophilic granuloma can also be treated with injection.
One more recent patients where there was bone destruction with using Eosinophilic granuloma, here again we did an incision biopsy to confirm the diagnosis. Injected intralesional triamcinolone, lesion went away 90% of it has gone away and the residual was again treated with injection triamcinolone and we are awaiting final results. So, this works very well intralesional injection for use in eosinophilic granuloma.
Then the next category incisional biopsy. Incisional biopsy is specifically for suspected malignant tumors. Poorly localized and infiltrated lesion associated with crucial orbital structures where if you were to do complete excision, you may cause functional issues. Residual functional issues such as loss of vision, diplopia, squint, tosses et cetera. You may also want to do incisional biopsy and get an inter operating diagnosis and plan your for the surgery based on that. It is not for tumors which are well circumscribed and are not associated with crucial arbitrary structures. If a lesion were to be very well circumscribed and is away from all the crucial orbital structures and you’re unlikely to produce complications, then the best option would be exceptional biopsy.
I’d like to talk a bit about the way we do in incisional biopsy. By literature, it’s known that 8 to 20% of orbital biopsies result in nonspecific or misleadingly wrong diagnosis affecting the management and the final outcome. It could be because of unrepresentative sample, non-uniformed pathology within the lesion. Example is a lymphoproliferative lesion, part of it could be, lymphoma part of it could be benign or atypical lymph hyperplasia. So, if you hit a part of the lesion which just shows a typical Lymphoid hyperplasia, then you might miss the diagnosis.
Sometimes, we hit the tissue reaction which could be superficial tissue reaction which could be in the form off dense fibrosis, and the lesion is not properly diagnosed. There’s an example of a child with subacute proptosis. Lesion in the superior extraconal space. We always do a three layered or multi-layered biopsy, and we always analyze all the layers of biopsy. Here the superficial biopsy showed only inflammation and fibrosis. Example, if this were to be the lesion, if I were to be taking biopsy only from the superficial part, this being the epicenter, this would show only fibrosis and inflammation. So, if you were to do only superficial biopsy then you might misinterpret this particular lesion as an inflammatory lesion and treated with steroids.
What if we do our midzonal and the deep biopsy? There we find adrenal liposarcoma which shows all the typical immunohistochemical characteristics. Adult patient with bilateral proptosis with a paranasal sinus extension. Here superficial biopsy showed inflammation and fibro-osseous whereas deep biopsy short granulomatous inflammation with multi nuclear giant cells and GMS stain shows fungal filaments. So, it was fungal granuloma. Again here, just a superficial biopsy would have misled us. So, in all patients where we do an incisional biopsy, we do superficial, midzonal and deep biopsy. And the depth that we go to is judged by the CT scan. If this is a tumor, if there’s a center of the lesion, the area of biopsy you should involve multiple zone. So, superficial midzonal and deep, because sometimes it’s in the intermediate zone that you find the tumor and in some specific lesions, it’s the deep part of the lesion that shows classic characteristic or specific etiology.
Fungal lesion, again, the deep competent generally contains the fungus whereas in the periphery, there could be dense fibrosis simulating a non-specific inflammation. In orbital tuberculosis again, they would be deep part of the lesion which would reveal the infection. So, in orbital incisional biopsy, we recommend that we do a layered biopsy, superficial, intermediate and deep going to the epicenter of the lesion which is estimated in depth based on the CT scan and MRI.
Now, if you do have any doubt about where you are in the orbit, then you can use 3D localization techniques. 3D localization techniques or intra operative localization would be very useful if you were to have a lesion which is in the posterior orbit, you can use intra operative localization and make your instruments navigable. This is a patient where we’re doing a biopsy from a deep seated orbital lesion where the forceps itself or a probe that can be made navigable can be used to confirm where exactly you are in the orbit before we take a representative sample from the orbit. So, these techniques actually help us localized a lesion and localized area where we are doing biopsy from.
So, following incisional biopsy, some patients would need chemotherapy if they have a systemic manifestations, some patients may simply need radiation. For example, this is an outpatient with orbital lymphoma mantle cell, stage III simply treated with Bendamustine and Rituximab. So, without any functional or cosmetic blemish, his lesion is cured. So, incisional biopsy helps us confirmed the diagnosis, guides us towards specific systemic investigations based on which we decide what is the final management in consultation with the oncologist.
Now, what about excisional biopsy? Excisional biopsy is for benign or malignant lesions as judged by clinical radiological evaluation well circumscribed and localization. So, any tumor which is benign or malignant as judged by clinical radiological evaluation which is well localized and circumscribed, non-infiltrative, not associated with crucial orbital structures can be treated with excisional biopsy because these lesions which are not associated with crucial orbital structures have minimal risk of functional deficits. So of course there’s a risk but it is very minimal and that needs to be discussed with the patients.
There are several surgical approaches, neurosurgical endonasal and orbital. If a tumor were to be predominantly in the posterior orbit with cranial extents, such as optic nerve glioma with intracranial extension then that could be a neurosurgical approach. If a tumor work to be predominantly nasal or paranasal sinus with orbital extension, that could be an endonasal approach or done with in concurrence with any anti-surgeon. If a tumor is predominantly orbital then we have several approaches. Transconjunctival, anterior orbital, lateral orbital or a combined approach. No anterior orbitotomy can be done by lid cease incision, superior sub-brow incision is called benedict incision, you can also do a vertical lid split incision called the Byron Smith.
A lesion such as adult marked which is located in the anterior orbit can be excised with lid cease incision which is extremely forgiving in terms of cosmosis. For a supernal nasal lesion which is in the anterior or mid orbit you can excise with lid spilt approach, vertical lid spilt approach or a sub-brow incision for lacrimal gland malignancy. This was an arena customer. In Lacrimal gland malignancy, when we do biopsy we prefer a direct approach not a lid cease approach because that causes multiple layers of tumor infiltration. If it’s only an incision biopsy, we can see the tumor in multiple layers as planning and giving radiation becomes very, very difficult. So, [indiscernible] [00:35:13] like lacrimal gland malignancy, we approached the lesion directly by an incision right in the sub-brow lesion.
In lateral orbitotomy, there are several approaches. Kronlein, Berke-Reese were older ways of approaching the orbit. Currently, we used a modified Stallard-Wright which is not as acutely assess this but a very lazy as like that or eyelid crease incision these are approaches for lateral orbitotomy. But what is the current trend is to do a minimal access surgery, that means that for several lesions in fact about two thirds of lesions you can approach these lesions by our transconjunctival approach. Less is more is addictive. In transconjunctival orbitotomy, there are several incisions. You can do a medial or lateral perilimbal or paralimbal incision. But more popular are transcaruncular incision which caused by the name of Balch and Goldberg, inferior forniceal extension of the same lesion with the lateral canthotomy and the inferior cantholysis gives you a panoramic view into the orbit.
In fact, any tumor which is located in the medial, info medial, inferior, infra lateral and to a certain extent super lateral can all be approached by Transconjunctival incision. What cannot be approached by Transconjunctival incisions are the lesions that are located in the superior orbit because then you have to breach the forniceal attachment of liberator does having a risk of process. So, every other lesion except those located in the bank superior aspect can be approached by transconjunctival incisions. This is a child I showed you earlier with the orbital teratoma. Now this tumor is – this lesion is on both the sides of the optic nerve natural as well as medial with extraconal as well as intraconal extension.
Here we approached by a transconjunctival incision with lateral canthotomy and inferior cantholysis, that is a lateral cystic component of the lesion. And you can see this update now they’re on medial to it is a solid competent of the lesion. All this was excess under visualization using an operating microscope that was a grew in the tumor where the optic nerve passed and the optic nerve was very well preserved. There’s a final outcome after six weeks where the child has residual esotropia that needs to be corrected but there was no damage to the optic nerve at all because we could see everything. This was a very panoramic kind of an approach where the tumor was completely seen optic nerve was visualized before it was dissected free from the tumor.
This was a intraconal cavernous hemangioma where we do the transconjunctival approach. I’ll show you a video clip here. You start with a small lateral canthotomy, that’s the lateral canthotomy about 8 to 10 millimeter, followed by inferior cantholysis, this is optional but if it’s done then you get very lose lower eyelid and you can straight away go to the inferior phonics and be about 4 to 5 millimeter away from it. So, in fact the incision is about four millimeters from the lower edge of the tarsus, about four millimeters from the inferior phonics. Retract and approach the area of interest directly, that is the inferior optical margin. Make an incision in the periosteum very close to the inferior orbital margin not to damage the infra orbital nerve at this stage. Then dissect the periorbita until the end point is reached.
End point for me is a visualization of the effect, apex off the inferior orbital fissure or the inflow orbital nerve. As we approach the apex of the inferior orbital fissure, we stop the dissection and we cauterize the bleeders perforating vessels through the bone are gently cauterize using a fulmination mode of RF. And incision is made in the periorbital in the cordiant where the tumor is located. Orbital detractors are used to gently retract the orbital fact and the lesion is localized and it is dissected without using a sharp tipped instrument using blunder sectors. After about 50 to 75% of the lesion has dissected a [indiscernible] [00:39:31] used for gentle and to posterior attraction to get a purchase over the tumor and the tumor is very gently [indiscernible][00:39:37].
As the tumor comes out, we have to visualize if there’s a posterior feeding vessel, feeder vessel that has to be cauterize to minimize the risk of secondary hemorrhage. Then all that we do is to close the lateral canthal incision, reestablish the lateral canthal angle and conjunctival suturing. Conjunctival is simple close with interrupted 60 Vicryl sutures, a few of these sutures is all that is required and this patient generally do very well. The same patient after about two weeks after surgery without any morbidity that follows a large orbitotomy or bone cut orbitotomy. This patient do very well. There is scarless healing. So, Transconjunctival approach is extremely gratifying. This are some of the examples of patients who have received a similar surgery.
This was a cavernous hemangioma a younger, operated by a transconjunctival approach. This was a neurilemoma from the intra orbital loop by the transconjunctival approach. Fibrous histiocytoma by the transconjunctival approach. Now when we do like a lateral orbitotomy currently, we prefer that lateral orbitotomy without a bone cut. This is a patient with adenoid cystic carcinoma of the lacrimal gland with bone destruction. But again, we use multi-mobile treatment here so we don’t exercise the bone. But we exercise the entire tumor along with the periosteum that is affected and that is investing it.
So here, we have a direct approach not a lid crease approach. We identify the periosteum that is adherent to the tumor and make an incision aboard 10 millimeter away from the area of a lesion, that’s how we estimate the amount of periosteum that is adherent to the tumor and make incision about 10 millimeter away from the area of a lesion. Then the tumor is dissected using blunt dissectors and a combination of blunt and sharp dissection wherever there is fibrous lesion making sure that we’re not damaging any crucial structure. Then after having dissected the tumor to about 75%, [indiscernible] [00:41:49] posterior attraction. Vascular particles have cauterized and the lesion is delivered.
So, same patient following three weeks post-operative does that. So, this was without a bone cut at least in adenoid cystic carcinoma, it is advisable not do the bone cut. And even in patients who have other benign or malignant lesions in the orbit, we try not to do the bone but to minimize mobility. What next after surgery? Next after surgery is a very careful attention to histopathology. Pathologists has to give out the diagnosis, and you query the pathologist if you have any doubt at all, have a discussion with the pathologist, based on which they might do for the specific test such as immunohistochemistry and also gene rearrangement and other specific test that are available in advance histopathology of the orbital tumor.
And finally the diagnosis is concluded. Based on that, you have a discussion with your oncologist, design what systemic evaluation that the patient needs and provide for the chemotherapy and all radiotherapy depending on the tumor, so that’s called adjuvant therapy. So, based on the histopathological diagnosis in a malignant tumor, for a microscopic residual tumor in the orbit, you’ll have to provide adjuvant radiation. If the patient has any risk for systemic metastasis, then we have to provide adjuvant chemotherapy.
Now, what about multimodal therapy? Multimodal therapy is nothing but a combination of surgery, radiation and chemotherapy at a specific sequence. This is a patient with sebaceous gland carcinoma of the eyelid with anterior orbital extension. This patient has undergone orbital exenteration. Orbital exenteration is a mutilating surgery done in specific conditions such as orbital retinoblastoma, rhabdomyosarcoma, adenoid cystic carcinoma, orbital sebaceous gland carcinoma, and orbital extension of [indiscernible] [00:43:52] interval squamous cell carcinoma. Is it effective? It’s a surgery which causes lot of mobility, mutilating but is it really effective?
Look at the literature and you find that it is not effective at all in terms of life salvage. Orbital RB has about 75% mortality, rhabdomyosarcoma managed primarily with orbital exenteration has up to 40% mortality. Adenoid cystic carcinoma, sebaceous gland carcinoma and squamous cell carcinoma, no different. They have high risk off systemic metastasis and mortality despite what we consider as the primary management modality that is orbital exenteration.
They will do multimodal treatment. In orbital retinoblastoma we begin with high dose neoadjuvant chemotherapy. This child has orbital retinoblastoma that’s the orbital component of the tumor. After three cycles of neoadjuvant chemotherapy, eye has gone into comfortable [indiscernible] [00:44:51] as you see here and the orbital component has completely disappeared. At this stage we do a enucleation with the long optic nerve stumps, treat the orbit with stereotactic radiation and provide adjuvant chemotherapy for 12 cycles. That is the protocol for orbital RB. Does it work for optic nerve extension? It does. As you see here, you find that the patient has an optic nerve extension with gross thickening of the optic nerve, following six cycles of neoadjuvant chemotherapy, optic nerve has returned to its normal caliber when we would do enucleation with the long optic nerve stumps and provide for the radiation and chemotherapy.
It also works for anterior intracranial extension of retinoblastoma. This patient has extension of the tumor right up to the cavernous sinus, following six cycles of neoadjuvant chemotherapy. There is residual tumor of the superior orbital fissure that can easily be treated with steroid tactic radiation. Of course, the eye needs Enucleation with a long optic nerve stumps and adjuvant chemotherapy as well. The benefit is that we don’t have to do orbital exoneration, children retain excellent cosmosis following enucleation, and there is a high success rate.
In rhabdomyosarcoma, smaller the residual tumor, better it is. You all understand that. But what if the tumor were to be located around crucial structures such as inferior rectus muscle. Suppose, I were to excise this entire tumor along with the inferior rectus, child would be left with refractive strabismus, which cannot easily be repaired and the child would go [indiscernible] [00:46:25]. So, then what we do is if this is the inferior rectus muscle, and the tumor is all around it, we leave a wedge of the tumor or a part of the tumor as you see here around the inferior rectus muscle, remove the residual tumor, rest of the tumor. So, residual tumor can easily be treated with stereotactic radiation and adjuvant chemotherapy, and these children typically do well. There’s a child with a large rhabdomyosarcoma where orbital exenteration is necessary. But to minimize intra-operative risk of bleeding, we can chemo reduce it and do a safe orbital exenteration.
In adenoid cystic carcinoma, again we follow the same principle where we chemo reduced the tumor by providing neoadjuvant chemotherapy, then we do en-bloc excision, then extended-field radiation including the superior orbital fissure and the cavernous sinus followed by adjuvant chemotherapy to minimize the risk of systemic metastasis. Example here, this is at the initial presentation, the tumor is reduced by about 30% of its initial volume following neoadjuvant chemotherapy at which point we did an excision and provided adjuvant radiation and adjuvant chemotherapy and that’s the final outcome of the patient five years later.
One more similar example of adenoid cystic carcinoma, and bone remodels. This patient has a born defect which is because of tumor infiltration. We don’t have to do orbitectomy in such patients. We simply remove the tumor after doing neoadjuvant chemotherapy, and the residual is treated with sterotactic radiation. You can find the nice remodeling of the bone after three years. A child with adenoid cystic carcinoma of the orbit, this call for primary orbital exeneration because it was very extensive, but by multimodal treatment we could treat it, and there is complete resolution of the lesion with complete re-modeling off the bone after on and half years.
So what if you have a recurrent adenoid cystic carcinoma with prior radiation? If the patient has had prior radiation we cannot give radiation again, despite having arbitral recurrence because full dose of radiation has already been given. There’s an innovative way of delivering radiation just to the bed of the tumor after doing primary excretion that is called after loading brachytherapy. So, we implant this silicon tubes right at the bed of the tumor after excising the tumor. So, the tumor is being excised and then we implant the silicon tubes, and the patient goes to a radiation oncologist where radiation is delivered by after loaded interstitial brachytherapy sing Iridium 192 sources.
With multimodal treatment for adenoid cystic carcinoma, there is excellent outcome. These are three groups of patients that we had historically, we had group one and group two where surgery and radiation was performed. And in group two, surgery, radiation and chemotherapy were performed. In group three, multiple, multimodal treatment was performed. Looking at the outcome, you can find that in group A and B there’s a much higher chance of local tumor recurrence, much lower eye salvage, much higher chance of orbital exenteration and much higher chance of systemic metastasis.
Where in the group that received multimodal treatment there’s only 10% chance of local tumor recurrence, 95% chance of eye salvage. Only one patient needed orbital exenteration, 80% retained useful vision and, only one out of 20 developed systemic metastasis. So, turn around in adenoid cystic carcinoma of the lymphoma gland by using multimodal treatment. This can be used for sebaceous gland carcinoma as well with orbital extension or regional lymph node metastasis. This lady has large sebaceous gland cyst with orbital extension and regional lymph node metastasis following neoadjuvant chemotherapy you can find that the orbital component has much reduced eyelid component has much reduced now amenable to excision or even orbital exenteration which is less extensive and the regional lymph node have settled on, thus she can avoid radical neck dissection and go for only external beam radiation.
This is magical response to neoadjuvant chemotherapy. This patient has a large sebaceous gland carcinoma, following one cycle of chemotherapy tumor is much reduced. Following three cycles on that is left is in the medial part of the lower eyelid, later half of the lower eyelid has become nearly normal. So, patient would need a excision of the tumor and a [indiscernible] [00:50:54] and reconstruction, whereas the primary presentation the patient needed extensive orbital exenteration. And multimodal therapy has much better survival, looking at the current literature and our own outcome, we find that multimodal treatment has definitely resulted in a turnaround in terms of patient survival. So, in conclusion I would say that logical approach to orbital tumors beginning with a good clinically evaluation and clinical radiological differential diagnosis followed by systemic evaluation, targeted systemic evaluation depending on the differential diagnosis that you arrived at that stage followed by planned surgical approach and histopathology guided systemic evaluation and adjuvant therapy helps optimize the outcome. Thank you so much.
In surgical interventions for optic nerve tumors, are we removing only the tumor or with the optic nerve or are we going for enucleation? It a depends on which optic nerve tumor that we’re talking about. For optic nerve glioma, of course removal of the tumor is not the choice at all unless we are trying to remove a progressive tumor that is very rapidly marching towards the [indiscernible] [00:52:05]. In which case there would be a risk of visual field defect in the cataract? That’s the only situation where I would advise excision of the optic nerve tumor, but not before trying systemic chemotherapy which is very useful on stereotactic radiation. So, optic nerve glioma, you may want to confirm the diagnosis and the grade of the tumor by an incisional biopsy from a safe zone, but no excision is advised unless in the situation that I mention.
In optic nerve sheath meningioma again there is no surgical excision, it’s stereotactic tradition that works very well for optic nerve sheath meningioma. So, in optic nerve tumors, we don’t try t remove the optic nerve unless it’s a malignant tumor where there is no other option. There might be a malignant tumor, a rare one where no chemotherapy or surgery may work, no chemotherapy or radiation may work, such patients may undergo excision of the optic nerve along with the tumor, otherwise we try to preserve vision as much as possible.
Could you again showed the slide showing aspiration? Well, I don’t think I can go back to the slide but aspiration is a very simple technique. If the lesion is posterior then you might want to do it under CT guided or intraoperative ultrasound guided with a palpable lesion or if it’s in the middle of it you can with of course CT guidance film or MRI guidance you can approach the lesion very safely. Extra correlations are safe to approach by aspiration.
How to ensure good amount of biopsy from an ill-defined of a [indiscernible] [00:53:41]. Well the amount of biopsy how deep you want to go into the tumor depends on the estimation of the size of the tumor on CT scan and MRI. If the tumor is say well beyond 15 millimeter in thickness, we always go to the area where it is maximally thick. Of course you are to take into consideration what would be the safe approach. If the safe approach is say super lateral and you can easily go up to the maximum depth of the lesion by that approach that would be the approach. That has always guided by a CT scan and the functional problems that you may cause by a particular weapon. So, a combination of factors matters when you do, approach for biopsy. So, you always have to take a least 50% of the depth of the tumor or the thickness of the tumor that is the epicenter. Epicenter is the center of the tumor. So, to approach the center and slightly beyond so if it’s a 15 millimeter thick orbital tumor, I would go up to 7.5 to 10 millimeter depth, so I will take superficial biopsy then go with zonal on then deep.
Indication for Enucleation and exenteration in orbital tumor. Well, in orbital tumor, there is no indication for Enucleation per se. Orbital tumors are not treated by a Enucleation, except in patients where there is optic nerve glioma where you want to approach the tumor from the orbital side and you cannot do without enucleation, [indiscernible] [00:55:15], you can do that. Exenteration of orbital tumors is for lesions which cannot be otherwise treated with multimodal treatment, or tumor is so extensive or if the multimodal treatment fails, then you approach it by orbital exenteration. Typically in a patient where there is, adenoid cystic carcinoma of the glaucoma gland which is very extensive and even on chemo reduction, there is insufficient chemo reduction, and you feel that the residual tumor is not amenable to stereotactic radiation, then you perform orbital exenteration or in rhabdomyosarcoma where chemotherapy has failed. If it’s a recurrent or a refractive tumor multi drug resistant tumor where chemotherapy radiation has failed then you do orbital exenteration. Otherwise there are very few indications for primary orbital exenteration for tumors left now.
What is the best way to dissect a tumor? I see that the dissection is [indiscernible] [00:56:13]. Well, I used a spatula which used to be called a lens spatula where you know earlier they will do intra-capsular cataract surgery. It was designed for possibly that but it’s a small spatula which is slightly larger than a periosteal elevator which has very nice rounded tip. That’s something that you can use. You can definitely use a cotton bird. Cryo is not for dissection cryo is to get a purchase over the tumor or hold over the tumor without using a forceps, without having to use the forceps and breaching the capsule of the tumor. If you were to hold the tumor very, very nicely and gently without breaching the capsule of the tumor, that will be cryoprobe. So, cryoprobe gives you a good hold over the tumor, and as you start lifting the tumor up, you will find the posterior structures that are adhering to the tumor, so everything is under visualization orbital surgery, most of the maneuver, if they’re performed under visualization then it becomes safe.
If you do blind dissection using your finger tip or instruments that are just sent, you know put into the orbital without visualizing where you are, what are you doing, and without monitoring the pupil then it becomes unsafe surgery. Scissors are generally avoided in dissection unless you’re sure that what you’re cutting is a non-crucial structures as fibrosis. Monopolar cautery is best avoided because it causes optic nerve trauma.
What do you do if tumor erodes orbital roof and during surgery we encounter this? Well, if the tumor erodes orbital roof you know already that it is eroding by the scan that you’ve done. Well Intra-operatively, if there is any addition of the tumor, to the Dura mater, the bone is eroded and you find that the tumor has adhered into Dura. Well, [indiscernible] [00:58:11], if you feel that the tumor is minimally adhered into the Dura, then you can try to dissect it. Dura is a tough structure. It is not as thin as even the periosteum, it’s a very tough structure. So, you can take liberty with Dura. If it’s a small area of addition and you can keep orbital fat and glue [indiscernible] [00:58:33] glue standby.
So, if there is a CSF leak you don’t have to worry, you simply plug in the area of the leak with a small bit of orbital fat, and glued it. Glue it using [indiscernible] [00:58:44] and that works very well. That’s what the neurosurgeons advice. So, you don’t have to worry about small CSF leak, and you can go ahead and decide of the Dura rather than leaving it there and risking local tumor recurrence. But if it’s a large area of Dura addition, well then you might need the help of a neurosurgeon.
How to differentiate the dacryocystitis from lacrimal gland tumor? I think it’s very simple dacryocystitis is of the lacrimal side and lacrimal gland tumor is super temporal. But if you’re meaning dacryoadenitis but dacryoadenitis sometimes can be simulating a lacrimal gland tumor. In dacryoadenitis, squamous is affected. So, if you do squamous and if it’s slow, and if the patients has subacute lesion with pain, etcetera and signs of inflammation, then you find it is dacryoadenitis, it can be bilateral. It can follow viral fever. So, there are clinical clues to the diagnosis of dacryoadenitis and squamous being affected favors the diagnosis of inflammation that is dacryoadenitis.
Books for reference, well there are a lot of books, [indiscernible] [00:59:50], these are nice books of orbital surgery. You can refer any of these. How to manage [indiscernible] [00:59:57]? Orbital varices are generally difficult to manage. Orbital varices are not lesions for surgical management. Orbital varices can be managed by an interventional neuro-radiologist, by embolization, gluing, ballooning, etcetera, but these are not for surgical intervention. You will have to take the help of interventional neuro-radiologist for varices.
What are the conditions that need biopsy to decide management plan? Well, if you have an infiltrated lesion, which is affecting crucial structures. If you go to excise that lesion appropriately then you might cause functional disturbance, loss of vision, etcetera. And if you find it’s an infiltrated lesion could be a malignant lesion, could be an unusual inflammation, then you do an incisional biopsy because based on that you proceed further. So, if it’s a well circumscribe lesion as I said earlier, well circumscribe lesion, which is away from the crucial orbital structures, then the treatment of choice is complete excision.
But if it’s an infiltrated lesion affecting crucial structures as an extraocular muscles [in the optic nerves, etcetera, then you might want to do incisional biopsy. If you’re suspecting adenoid cystic carcinoma of the lacrimal gland, you do an incisional biopsy because currently you do multimodal treatment. Similarly, for [indiscernible] [01:01:17] sarcoma if you’re suspecting a round cell tumor in a child, if you’re suspecting that’s very eminently amenable to chemotherapy that you would want to do incisional biopsy. Otherwise for poorly differentiated infiltrated lesions.
How do you approach extraconal tumor surgically? That depends on the location of the Duma. If it’s a medial extraconal lesion then [indiscernible] [01:01:40] incision. Inferior extraconal lesion definitely inferior forniceal incision. Lateral extraconal lesion again, lateral forniceal incision. Superior of course, then you have to go by skin incision which could be by [indiscernible] [01:01:54]. Differential diagnosis of bilateral lacrimal gland enlargement, most common cause for bilateral lacrimal gland enlargement is inflammation benign reactive lymph node hyperplasia, the pure adenitis caused by viral infection or benign reactive lymphoid hyperplasia, [indiscernible] [01:02:17] disease are common causes bilateral lacrimal gland enlargement.
What study do you prefer, CT or MRI? Well, that’s a question that, you know it’s individual preference. I would want to do both in certain cases such as a patient where there is say lacrimal gland malignancy, CT and MRI. MRI is definite choice for tumors which are of the nerve origin, optic nerve or tumors which are extending intracranially, whereas if it’s a born based lesion than CT scan is fine. For tumors such as optics nerve sheath meningioma or optic nerve glioma, definitely MRI, for a [indiscernible] [01:03:02] etcetera, CT scan is fine. But if you want to trace the course of the nerve entirely, supposed patient has a neurilemoma of the supraorbital nerve, now I want to trace the cause of the nerve entirely and want to identify if there are small [indiscernible] [01:03:21] supraorbital nerve, then I would need an MRI. So, it’s a combination of CT and MRI, depending on the clinical situation.
Do you still biopsy [indiscernible] [01:03:34] that is subjected to stereo tactic tradition? No, if it’s a radiological certainty that it is cavernous hemangioma, there are clear guidelines as to how do you diagnose cavernous hemangioma on MRI scan, then possibly you won’t need a biopsy. If you need a biopsy for an orbital [indiscernible], then it has to be very, very safe biopsy. You can even do a CT guided biopsy in an orbital [indiscernible] [01:04:02] taking the help of a radiologist.
Can you use Bleomycin for lymphangioma? Of course, yes. Bleomycin is the current standard of care for lymphangioma. Do you [indiscernible][ [01:04:13] cavernous hemangioma to facilitate excision? Personally, I don’t prefer it but some surgeons do it to make it smaller. Well, that’s fine. What injection is Picibanil? Picibanil is a Japanese drug. It is not easily available. You have to import it from Japan. It’s a very nice drug. It’s caused by, which is derived by Streptomyces. It is a sclerosing agent, very useful, causes good regression of lymphangioma. Only reason why it’s not very extensively used is because it’s not easily available.
For lymphangioma, do you need to aspirate all content first before you inject Bleomycin? Well no, lymphangioma are three types. When there is micro cystic, micro cystic is predominantly filled with blood where we as spread almost everything. The second is a combined micro cystic and micro cystic, where partial aspiration of the micro cystic component. If it is predominantly micro cystic then there is no scope for aspiration. It’s a semi solid lesion where hardly any blood content would be there, fluid content will be there, there you don’t aspirate.
Have you had any complication with injection Bleomycin? Well, injection Bleomycin, if it is injected, you know you have multiple sessions to inject. You don’t have to inject a large amount of drug in the same session, so I would limit injection to about three cc of Bleomycin. I won’t inject too much in the intraconal space so that can help you avoid complications.
For use in [indiscernible] [01:05:53]? 40 mg is what we inject in the first injection and if there is a residual you can go back in and inject 20 or 40 more. Maximum I have needed is about two injections so far in a bunch of [indiscernible] [01:06:10] I have treated. What lesion would be treated with gamma knife? Gamma knife is ah mode of delivering radiation, so it’s a much better way of doing radiation than [indiscernible] [01:06:22] radiation can be given with gamma knife. If you want to be safe that means that you don’t have to have membrane penumbra of radiation without any collateral damage, then you prefer gamma knife. It’s expensive but better than [indiscernible] [01:06:443] radiation in terms of complications.
What orbital tumors requires sentinel lymph node biopsy? Well if you have a eyelid malignancy or a conjunctival malignancy which has extended into the arbitrage sebaceous gland carcinoma, melanoma of the eyelid, conjunctival melanoma and squamous cell carcinoma of the conjunctival, then sentinel lymph node biopsy is advocated.
What is the risk off vision loss due to sclerosis and injections in lymphedema and what is possible cause for vision lass? Well, risk is low. There are not many cases in the literature that have suffered a vision loss. It could be because of direct optic nerve toxicity because of the drug itself which is rare, but if you inject a huge amount of Bleomycin in the intraconal space that is a possibility. Second is inadvertent damage to the optic nerves by the process of injection itself, so you don’t get too much into the intraconal space to inject the drug. Drug can be injected in the most anterior part of the lesion, rest of the drug would percolate into the lesion because lesion is not a very solid one, so you don’t get into the depth of the lesion, so you can minimize the toxicity to the optic nerve.
Is there any role of systemic steroids in cavernous angioma? Not to my knowledge. How to constitute dilute Bleomycin and how to determine the dose that you inject? It is one international unit per ml is the dose that we use and the amount of aspirate, supposed the aspirate is 3 cc, you inject half the aspirate 1.5 cc. If it’s a micro cystic lymphangioma, then you estimate the volume of the lesion based on CT scan and inject half the volume of the lesion.
Any tips to minimize damage to optic nerve in retrobulbar intraconal tumor excision? Well you have to know which part of the tumor you’re dissecting at any given point in time everything has to be a individualization. I personally use an operating microscope which wide in the field with increased working distance for tumor dissection. I always keep a lookout for the pupil, the moment people start dilating that means that either directly or indirectly you are giving you know, traumatizing the optic nerve, so you have to stop, reevaluate exactly where you are in the orbit and then go further. So, if you don’t dissect the part of the tumor, which is towards the optic nerve, until you apply [indiscernible] [01:09:15], and look at the posterior aspect of the lesion can be possibly saved with optic nerve.
How to manage post-operative [indiscernible] [01:09:22]? Well you really don’t have to manage [indiscernible] [01:09:25] because it’s not going to be too much. But if you have say floor excavation because of a which is in the inferior orbit, then you can reconstruct the floor by using, put this polyethylene or bone graft or any such material. What about the risk off seeding incisional biopsy and suspected malignant lesions? Definitely, it is a possibility. That is exactly the reason why you don’t do a multi plainer incision. Example, lead crease incision is a multi-planner approach. You go by the skin incision then you do orbicularis incision, then you do sub-orbicularis, then you go interior to the symptom, then you get into the lesion. So, multiple layers of the lid can get involved, and it is impossible for planning radiation in such patients. So, if you do a direct approach right on top of the lesion then seeding is fine because that area is anyway covered by radiation that they’re going to give, so, seeding happens in incisional biopsy. It is not preventable but you have to have a direct approach.
Retrobulbar cavernous hemangioma and neurilemoma looks almost identical by operating? Well, no, not really so. Cavernous hemangioma is fury pink, it’s strawberry colored whereas neurilemoma looks white yellowish or even bluish depending on its cystic competent, so it’s easy to identifiable but a pre-operative CT would have already helped you. How to differentiate bone destruction erosion and remodeling? Well, bone destruction causes irregularity of the bone with thickening, irregular thickening. Erosion would cause thinning of the bone but not too much of irregularity. Bone remodeling is a very gentle scalloping off the bone. So, if on a bone window CT scan, it’s fairly easy to differentiate.
In transformational approach, four millimeter above forix, do you cut the retractor? Do you have entropion after this? Well, we have to go through the retractor but there is no entropion after this because tissues rejoined fairly well. What [indiscernible] [01:11:30] gives good results with minimal collateral damage? Well I have no commercial interest but I find [indiscernible] [01:11:37] very safe. Do you suture the eyelids primarily after that exenteration? Yes, I do. I do eyelid sparing exenteration, that partial eyelid sparing, I spare all the orbicularis that I can. I spared all the skin that I can, then I do a layered suture. I would suture the orbicularis very carefully because that gives blood supply to the skin otherwise skin can necrosis. So, once you have a good back of orbicularis, then we suture the skin without [indiscernible] [01:12:06].
If transformational approach is done, isn’t it you face [indiscernible] [01:12:12] in front of the retractor? No, you actually go under the orbicularis, retract and go beyond the inferior orbital margin with your retractor. If you have done a inferior forniceal incision with [indiscernible] [01:12:25] it’s very easy to retract the skin orbicularis. Go to the inferior orbital margin, then you identify the [indiscernible] [01:12:35] cut so there is no fact that is encountered unless you could transept.
Is there any effect of stereo tactic radiation on other than the eyelid? No, if you limit the field of radiation, then no. What are your views on usage of RF in orbital surgery post-COVID era, I sit safe? RF is safe. It does cause some amount of you know, vaporization. But if you have a suction going on and if you’re using PPE in all patients universally, then there is no other, there is no, see, actually there is no vaporization related transmission of COVID that has been documented, right? So, what we’re vaporizing here is tissue. So, tissue vapors will to my knowledge cause COVID infection if you’re using a PPE anyway.
[Indiscernible] [01:13:27] approach if the tumor is located intraconally? Well, if the tumor is located intraconally and [indiscernible] [01:13:36]. If the tumor is located is located intraconally, [indiscernible] [01:13:44]. Do we still use the EBRT for it etnoblastoma? Yes, we use it only as an adjuvant treatment for patients who have optic nerve extension to the level of transaction or orbital retinoblastoma. These patients generally are non-heritable, arbitrated neuroblastoma is that this is of older children. Generally this are non-inheritable but despite there is that we have of second [indiscernible] [01:14:14]. Our primary goal is to save the child, so we have to do radiation in these patients the adjuvant treatment.
Following exeneration for an orbital tumor, how do you manage a patient with presence with osteomyelitis of the frontal bone in the socket? Well, if that has happened then you have to get a diagnosis for osteomyelitis, what is the infectious of infecting organism, curate the bone which is in mold give antibiotics systemically as well as locally. Possibly that’s how I’ll manage it.
Can you comment on hemangioma pericytoma in terms of its Pathogenesis and how it can be clinically distinguish from cavernous hemangioma? Clinically, sometimes it’s very difficult to differentiate. Clinically, if it looks on a MRI like a cavernous hemangioma but has extreme degree of vascularity, and also if the ocular motility is affected and it’s rapidly growing proctorships disproportionate to the size of the lesion then you may consider hemangioma pericytoma as a possibility in your clinical radiological differential diagnosis.
Can one do surgery after radiation? Well, yes you can, and healing is likely prolong but not difficult. What do you think about endoscopic external orbital approach? Yes, it’s a very good approach. Some surgeons actually use an endoscopic external orbital approach to visualize the lesion. Some even use a combination of endoscopic [indiscernible] [01:15:41] approach. These are innovative ways of doing orbital surgery if you have these facilities, use most [indiscernible] [01:15:46] tumors I don’t use most, but I use intra operative frozen section.
Conjunctival map biopsy. Well, how do you do Conjunctival map biopsy? Although it is not related to this lecture, I’ll tell you. Conjunctival map biopsy biopsy is to map out the entire conjunctiva. You draw the entire ocular surface on a filter paper, then you take bits of conjunctiva from 369 and 12 o’clock para limbal inferior forniceal, superior forniceal, inferior palpebral conjunctiva, superior palpebral conjunctiva and [indiscernible] [01:16:09]. So that is a bunch of biopsy that is laid nicely on a filter paper each biopsies numbered and send to the pathologist, so that pathologist will tell you exactly which ones are involved. Then you can map out the area extent of [indiscernible] [01:16:31]
When [indiscernible] [01:16:37] present scenario? Well, I would cut the bone only if my dissection becomes very difficult. It means that if it’s a very [indiscernible] [01:16:46] almost a hard lesion in the intraconal space which is non-accommodating, [indiscernible] [01:16:52] are fairly accommodating lesions. They come out with a small incision. But if it’s a firm lesion for example or if there is a lot of bleeding during surgery, not able to visualize the entire extent of dissection or if we have difficulty in dissecting, you want more visualization, you want to go from the lateral side and dissect the posterior aspect of lesion, then you would bring in a bony cut otherwise generally not.
Well, how common you see posterior orbital [indiscernible] [01:17:22] in pediatric age two? Very, very uncommon, I have just seen two or three, so far. Do you consider intra-arterial chemotherapy for adenoid cystic carcinoma? Yes, I won’t do that myself. I do, the reason we do systemic chemotherapy is this that you know, look at the literature. Many patients 50%, 75% has a mortality with adenoid cystic carcinoma.
Why do they die? And when they have metastasis, they don’t have a recurrence in the orbit. They have metastasis. Well, they do have metastasis when the local tumor is under control. That’s because micro metastasis has already taken place by the time you have gotten to diagnose that tumor and treated, so systemic chemotherapy is given not just to chemo reduce the tumor, but also take care of the theoretical risk of micrometastasis, but it’s a really risk. But if you do intra-arterial chemotherapy, it shrinks the tumor, no doubt as they would say has shown very elegantly. But it doesn’t cause any affect, it doesn’t have any effect on systemic micrometastasis. So, I would prefer systemic chemotherapy.
What about optic nerve meningioma, optic nerve meningioma with extension till orbital apex but not optic canal extension? Optic nerve meningioma is treated very easily by stereo tactic radiation. It stabilizes the lesion. So, there is no role for primary surgery in optic nerve sheath meningioma. In fact it is a surgical disease where there is no surgical approach necessary unless you want to diagnose it, so, when you want to diagnose it, you go biopsy the most nodular thickest part of the tumor, not damaging the optic nerve, tiny bit of biopsy just to confirm the diagnosis. But if it’s radiological fairly confirmatory of your clinical diagnosis then you really don’t have to do a biopsy and state against your tactic radiation.
What chemotherapy drugs have given in patients with sebaceous gland carcinoma for chemo reduction? Well, we give five [indiscernible] [01:19:34] in combination. To repeat [indiscernible] [01:19:37], yes. If the tumor is around more than one muscle do you first do chemo? That depends on the type of the tumor. If the tumor is amenable to chemo reduction then certainly you want to do chemo reduction. But if the tumor is not amenable to chemo reduction then you might want to just do an incisional biopsy.
How do you approach intraconal lymphangioma for injection of [indiscernible] [01:20:01]? Intraconal lymphangioma without any anterior extension is best approached by CT guided injection. How do you treat conjunctival lymphangiectasia? It may not be treated but if you have a small lymphangiectasia and patient is symptomatic because of that or if it has led to sub-conjunctival hemorrhage et cetera, then you can exercise it locally. You can do Cryotherapy, it works well. You can do cauterization, all these modalities worked well to control a small conjunctival lymphangiectasia.
Is there a role for chemo reduction in [indiscernible] [01:20:40]? Very, very positive yes. Yes, we can use topical mitomycin. You can use topical fluorouracil or immunotherapy with Interferon Alpha-2B, all these help in chemo or immune reduction for extensive [indiscernible] [01:20:56]. But if you’re talking about an orbital extension of [indiscernible] [01:21:00], well you can still use chemotherapy, [indiscernible] [01:21:04] growth factor innovator, epidermal growth factor innovator that also helps reduced [indiscernible] [01:21:16], that’s a target therapy [indiscernible] [01:21:18].
How do you manage Cysticercus? Cysticercus is best managed medically, Albendazole 15 Mg per Kg body weight for four to six weeks along with concurrent steroid [indiscernible] [01:21:27] four to six weeks. Symptomatic [indiscernible] [01:21:30] embolization prior to excision? Yes embolization can actually help before you even plan excision. In fact we don’t do excision. [Indiscernible] [01:21:40], you do en-bloc excision or Enucleation along with complete removal of the tumor. That’s one option. Second option is to excise the tumor and flush with [indiscernible] [01:21:57], that means you don’t damage [indiscernible] [01:21:59] and disturb the tectonic integrity of the eye. But treat the residual tumor with [indiscernible] [01:22:04] therapy that has been very successful. In fact we’re able to say about 90% of eyes with flush excision [indiscernible] [01:22:12].
Proptosis is a sign of tumor? Well, of course it is a sign of tumor. It could be a sign of inflammation or infection also, so it’s calls for a clinical radiological diagnosis. Then if cryo is available, is not available, what are the instrument can be safely used to extract the tumor? Cryo should be available, otherwise you have to get hold of it, if you’re a serious orbital surgeon. Otherwise some surgeons use a large forceps four by three to, forceps with the blunt tooth and leave some tissue on top of the tumor and hold that tissue not the tumor capsular itself. Those are options but I would suggest that you get hold of a Cryo.
How do you cover a non-lid sparing exenteration, skin infiltration is very extensive, well, if it is a non-lid exenteration sparing if you want to cover then you have to bring in a tissue flap from the forehead or from the surrounding areas or size of the cryo provides three millimeter no, that’s what I use. What are next in recurrent tumors which earlier aggressive chemotherapy and radiation? Well if the tumor is recurrent and it was earlier addressed with chemo and radiation, it depends on the type of the tumor. If it’s around cell tumor, then it can be second line of chemotherapy can be given that is salvage chemotherapy.
If it is a type of tumor where chemotherapy is not possible then you have to do surgical excision or exenteration. Big breaking therapy with silicon for [indiscernible] [01:23:36]? Well it’s not a silicon. Silicon tube is used but it is Iridium seeds that are sent across into the orbit, that is the caused. That is the source of radiation. What is your approach to [indiscernible] [01:23:49] extraconaly, intraconaly? Well, multiloculated orbital [indiscernible] [01:23:57], you don’t have to specify but well [indiscernible] [01:24:10] if you have 1.5 that is also fine. In MRI, you don’t have to specify slice thickness, in CT scan, of course you have to specify size slice thickness as one to two millimeters, what is preferred.