Validation of VISA classification for Thyroid Associated Orbitopathy

Transcription

1 25 Original Article Validation of VISA classification for Thyroid Associated Orbitopathy Dr M. Subrahmanyam 1, Dr P. Modini 2, Dr K.J. Sivacharan 3, Dr B. Kavya 4 Abstract Aim : To Validate the VISA classification in Thyroid Associated Orbitopathy (TAO) and evaluate the utility of its recommendations in clinical scenario. Material and methods: A total of 297 patients of TAO (case records of treatment received by 157 patients of TAO from Jan 2004 to December 2010 and another 140 patients of TAO who were treated based on the recommendations of VISA classification from January 2011 till December 2015) were included in this study. Their treatment and clinical response in comparison with the recommendations of VISA classification were analyzed. Results: 66 eyes of 41 patients underwent orbital decompression, 56 patients received systemic steroids, squint surgery was performed on 19 patients and lid surgery on 28 patients. Their treatment protocol as suggested by VISA classification has given very satisfactory results. Conclusions: VISA Classification is a very useful and practical classification for TAO and provides useful guidelines for management. Key words: Thyroid orbitopathy, VISA Classification, orbital decompression, systemic steroids, Introduction Thyroid Associated Orbitopathy (TAO) is very important since it is the most common cause of both unilateral (upto 50%) and bilateral (>85%) proptosis in people above the age of 15 years, across the globe 1. It has varied symptoms and presentations. It can have very trivial manifestations like mild lid retraction or can be very serious leading to blindness either due to corneal involvement or compressive optic neuropathy. It has varied presentations including pain, diplopia due to ocular motility restrictions, redness, defective vision or severe cosmetic blemish due either to severe lid retraction, proptosis or both, apart from squint. TAO was studied in detail and different classifications were proposed. The most widely used and popular classification was that suggested by Werner the NOSPECS classification in No signs or symptoms Only signs (limited to upper lid retraction and stare, with or without lid lag) Soft tissue involvement (oedema of conjunctiva and lids, conjunctival injection, etc.) Proptosis Extraocular muscle involvement (usually with diplopia) Corneal involvement (primarily due to lagophthalmos) Sight loss (due to optic nerve involvement). 1,3,4 Dept. of opthalmology, Maharajah s Institute of Medical Sciences, Nellimarla, Vizianagaram , Andhra Pradesh 2.Dept. of opthalmology, Govt. Medical College, Nizamabad. Telangana Unfortunately, the NOSPECS classification has some weaknesses and does not provide any inputs regarding clinical management of the disease at each stage. It also does not take into account the inflammation-component of TAO which is classified as inflammatory diseases of orbit. Thus it has very limited prognostic value. Dr Maatan Ph. Mourits et all observed that nearly a third of patients with TAO do no respond to immunosuppressive therapy. They made a prospective study on the response of TAO to systemic steroids in relation to the clinical activity and In 1989, introduced a Clinical Activity Score as to stage and grade the inflammatory phase of the disease 3. The 10 items of the Clinical Activity Score (CAS) Pain 1. Oppressive feeling behind the globe, during the last 4 weeks 2. Pain on attempted up, side or down gaze, during the last 4 weeks Redness 3. Redness of the eyelid (s) 4. Diffuse redness of the conjunctiva, covering at least one quadrant Swelling 5. Swelling of the eyelid (s) 6. Chemosis 7. Swollen Caruncle 8. Increase of proptosis by 2 mm during a period of 1-3 months

2 26 Impaired function 9. Decrease of eye movements in any direction 5 during a period of 1-3 months 10. Decrease of visual acuity of 1line(s) on the Snellen s chart (using a pinhole) during a period of 1-3 months Clinical Activity Score (CAS) - for each item present, 1 point is given. The sum of these points is the CAS. They are advised treatment with steroids if the inflammatory score was 4 or above. The CAS is a land mark study in TAO. But it has concentrated mostly on the inflammatory component of TAO. More over, it is difficult to make out the last two points at the initial visit.

3 27 The VISA Classification : VISA classification system was proposed by Peter Dolman and Jack Rootman and is based on four disease end points:vision, inflammation, strabismus, and appearance/exposure 4. Each section records subjective and measurable objective inputs and plans ancillary testing. On the first visit, the date and rate of onset of both the systemic and orbital symptoms should be recorded, since this may help predict the ultimate severity of the inflammatory phase 4. Vision/Optic neuropathy: The primary goal of this section is to look for compressive optic neuropathy (CON). The history includes blurring of vision or color desaturation and the progress and duration of symptoms. Objective measures include best corrected visual acuity and color vision defects, relative afferent pupil defect (RAPD), and infrequently congestion or pallor of the optic disc. Ancillary testing includes coronal CT or MRI Scans to confirm crowding of the orbital apex, visual fields, and rarely VEP (visual evoked potentials) or optic nerve images. The severity of the neuropathy is reflected in the measurements of central vision & color vision. In the presence of Optic nerve compression, its decompression either Medical or Surgical is recommended. Inflammation: Symptoms of soft tissue inflammation include orbital aching at rest or with movement, eye lid and / or conjunctival swelling and redness. The VISA Inflammatory Score widens the grade for chemosis and lid edema from 0-2. Chemosis is graded as 1, if the conjunctiva lies behind the grey line of the lid and as 2, if it extends anterior to the grey line. Lid edema is graded as 1, if it is present but not causing overhanging of the tissues, and as 2, if it causes a roll in the lid skin including festoons in the lower lid. The worst scores from any of the four eyelids are recorded in the inflammatory score table on the far right section of the table. The pain score is based on the patient s report of deep orbit discomfort rather than ocular surface irritation (0 = no pain, 1 = pain with movement, 2 = pain at rest). An additional point is assigned for diurnal variation of symptoms, to reflect the variability in congestion typically seen during the active phase 5. Control of inflammation with systemic steroids is recommended if inflammatory score is 5 or above. Strabismus: The symptoms for strabismus include a progression from no diplopia, diplopia with horizontal or vertical gaze, intermittent diplopia in straight gaze, and constant diplopia in straight gaze,. Ocular ductions can be graded from 0 to 45 in four directions using the Hirschberg principle: the patient is asked to look as far as possible up, down, right and left while the observer points a bright light at the eyes and studies the light reflex on the surface of the eye. If the light reflex hits the edge of the pupil, the eye has moved 15, between the pupil edge and the limbus, 30 and at the limbus, 45. Strabismus can be measured objectively by prism bar cover test. Treatment suggested was either Fresnel s prisms or patching of one eye till the disease has stabilized and later squint surgery 3 months after Orbital decompression. Appearance/Exposure. Appearance concerns such as bulging of the eyes, eyelid retraction and fat pockets, and exposure complaints of foreign body sensation, glare, dryness, or secondary tearing 5. The treatment suggested includes surgical correction after the course of TAO. It can be orbital decompression, followed by lid surgery. In the active phase conservative management with Lubricants, or Inj. Botox for lid retraction are suggested. To the best of our knowledge this is the first study from India, Validating Visa *. A total of 297 consecutive cases of TAO from Jan 2004 to December 2015 were included in this study. Of these 157 patients from January 2004 to December 2010 were from their case records, in whom the treatment received was compared with the recommendations of VISA classification and for the remaining 140 patients (from January 2011 to December 2015) the treatment was followed as per the VISA protocol. The treatment modalities included orbital decompression for those with compressive optic neuropathy during the active phase of TAO and for those for cosmetic reasons after the disease quietened. The decompression involved 1 wall to 3 walls and always with fat excision ranging from 3 to 6 cc. Systemic steroids like I. V. Pulse steroids (I.V. Methyl Prednisolone) for those with active TAO and conservative management for those with inactive disease followed by surgery for soft tissue changes as per the recommendations of VISA classification. Squint surgery by way of recession was performed.

4 28 This study includes a total of 297 patients of whom 132 are males and 167 are females distributed at a ratio of 1.0 : Vision: Compressive Optic neuropathy (CON) as evidenced by defective vision, color vision defects, Relative Afferent Pupillary Defect (RAPD), Optic nerve edema, Visual field changes and apical compression on CT Scan was seen in 42 eyes of 24 patients, that is in 8.1% of patients. Fig.1: Front view of a patient with TAO. Note the significant proptosis of both the eye with mild lid retraction, lateral flare and periorbital swelling. She has very mild inflammation but has bilateral CON as evidenced by visual acuity of 6/12 right eye and 6/9 in left eye with color vision defects, apical compression and optic disc edema. Fig.3: Automated Visual field analysis showing Visual field defect in a patient of TAO with CON. (Fig.1-3) They underwent surgical Orbital decompression by swinging lower lid approach which is a very safe procedure and also said to improve ocular motility and reduce diplopia6,7 as early as possible. In the event of delay due to any reason, they received Systemic Pulse Steroid till the time of surgery. Majority of them had 2 wall decompression along with 4cc of fat excision (table1). Table 1 Fig.2: CT Scan orbit (Coronal section) showing apical compression of optic nerve by enlarged extra ocular muscles. Fig.2A: Axial section of CT Scan orbit showing very significant bilateral proptosis( note that both the eyeballs are in front of the line joining the anterior most part of the lateral walls of the orbit) with thickened medial Rectus ( Red aster) and Lateral rectus( Yellow quadrangle) with increase in fat volume ( Green arrow).

5 29 Inflammation: The inflammatory score (Infl. Score) as per the VISA classification was noted for all the cases. Based on the inflammatory score the patients were divided into 4 groups. Fig.6A : Front view of the same patient showing bilateral proptosis Fig.4: Front view of a patient with TAO having 0 inflammation. with lid retraction with scleral show (Black Aster), Lid erythema Note how quiet the eyes are. She has moderate lid retraction in the (Green arrow), lid edema (white arrow) caruncular edema (red arrow) left eye and mild in the right eye (note that you could see the limbus) and chemosis (Yellow arrow) with increase in Fat volume and mild enlargement of Inferior Rectus on CT Scan. Fig.7: Front view of patient with severe inflammation as characterized by severe chemosis of grade 2 (Blue arrow), severe Fig.5: Mild inflammation in a patient of TAO. Note lid retraction, caruncular edema (White arrow), and conjunctival congestion, lid Conjunctival congestion (Red Aster), Caruncular edema (Black erythema with edema (Red astrix). He had severe deep seated pain arrow) and lid swelling (White diamond). and also severe periorbital swelling with diurnal variations.he had exposure keratopathy in the right eye (Red arrow) (fig 4 to 7): Group A (no infl). Where the infl score is 0, Group B (Mild) with infla score of 1-4, Group C (Moderate) with infl. Score of 5-7 and Group D (severe) with infl. Score of Most of the patients belonged to the first 2 groups and the average infl.score is 1.6. (Table2) Table 2 Fig.6: Profile of the patient showing moderate inflammation with very severe proptosis. Note the severe lid retraction with scleral show (black aster), Lid erythema (white arrow), lid edema (black arrow) and grade 2 chemosis (Yellow arrow). Also note the conjunctival congestion. INFLAMMATORY SCORE

6 30 Among 297 patients only 49 (16.5%) have active TAO and required Systemic Steroids.Rest of them had a quiet disease with very little/no inflammation but some presented with compressive optic neuropathy. The inflammatory component of TAO has responded very well to systemic steroids though in one case the inflammation subsided but the optic nerve compression worsened which responded to surgical decompression. Strabismus: Motility restriction leading to strabismus or squint and diplopia is not uncommon in TAO. This is because the extraocular muscles (EOMs) are primarily involved in TAO with enlargement and later fibrosis. The enlargement of the EOMs is responsible for proptosis, motility restriction, diplopia and compressive optic neuropathy (CON) due to apical compression.huge thickening of Medial Rectus is associated with CON. Motility restriction especially at extreme gaze is fairly common and was observed in 97 patients (32.7%) whereas diplopia was complained only by 22 patients (7.4%). Restrictive pathology of muscles as evidenced by Forced duction test and differential tonometry is the most common cause of motility restriction, even though myasthenia gravis may be rarely associated with TAO. Fig.8: Note restricted elevation of right eye (Blue Aster) as the patient is looking up, worsening of the lid retraction (Red aster). The patient had mild inflammation as evidenced by mild chemosis and conjunctival congestion (green arrow) Fig.9: A patient of TAO with associated Myasthenia gravis. Note bilateral restricted movements on attempted right gaze as she is not able to move the eyes. (fig 8-9) and can restrict ocular movements both in vertical and horizontal gaze 8. Strabismus surgery is usually delayed till the disease is burnt out and follows orbital decompression surgery since the squint can change after orbital decompression 6,7. Recessions of enlarged and fibrosed muscles are performed. Only 19 patients underwent strabismus surgery. Appearance : Appearance and Cosmetic blemishes are other important variables of TAO. Severe lid retraction, associated lid lag / lag ophthalmos, severe proptosis more so if unilateral or asymmetrical cause huge cosmetic problems and need to be addressed. Usually the permanent treatment is planned after the disease is quietened and the other problems like proptosis and squint are addressed. Lid retraction can be treated with Inj. Botox during the active phase of TAO to provide relief for the patient. The timing of the treatment is as per the guidelines of VISA classification. 28 patients (56 eyes) underwent Mullerectomy surgery for lid retraction Fig.(10). Fig. 10: The same patient as in fig.1 after bilateral orbital decompression and lid surgery. She has regained normal vision (6/ 6) and there is significant improvement in the lid retraction. Table:3: Management strategies in TAO Medical management with Steroids: 56 patients Conservative management with lubricants: Orbital decompression for CON... : 24 patients Orbital decompression for Appearance... : 17 patients Lid surgery... : 28 patients Squint Surgery...: 19 patients Inj. Botox... : 04 patients The treatment guidelines provided by VISA classification was compared to the actual treatment received by 157 patients who were treated prior to the adoption of the VISA classification and found to be very similar. Only 3 patients with inflammatory score of 4 and complaining of pain received systemic steroids which is contrary to the recommendations of the VISA classification. When we adopted the Classification for the patients from January 2011, there is not a single deviation from the recommendations of VISA and the results are very satisfactory.

7 31 VISA classification of TAO is very useful as it provides excellent guidelines for staging and also management of TAO. Its adoption has more advantages when compared to other classifications since it tells us what to do for every patient and at every stage of the disease. References : 1). Holds JB, Chang WJ, Daily RA et al., Thyroid Associated Orbitopathy in Orbit, Eyelids, and Lacrimal system, Basic and Clinical Science Course, American Academy of Ophthalmology, Singapore, 2008;7: ) Werner,S.C.: Modification of the classification of the eye changes of Graves Disease: Recommendations of the Ad Hoc Committee of the American Thyroid Association., Journal of Endocrinology and Metabolism, (1977), 44, ) Maarten Ph Mourits, Mark F. Prummel,Wilmar M. Wiersinga, L. Koornneef., : Clinical activity score as a guide in the management of patients with Graves Ophthalmopathy, Clinical Endocrinology (1997) 47: ) Dolman PJ, Rootman J. VISA Classification for Graves Orbitopathy. Ophthal Plast Reconstr Surg. 2006;22(5): ) Subrahmanyam M, Thyroid Associated Orbitopathy in Surgical Atlas of Orbital Diseases, Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, 2009 (1); ) Roncevic R, Sancovic Z, Roncevic D. : Results of Diplopia and strabismus in patients with severe thyroid ophthalmopathy after Orbital decompression, Indian J ophthalmol March; 62(3): ) Paridaens D, Lie A, Grootendorst RJ,Van den Bosch WA. Efficacy and side effects of swinging lower lid orbital decompression in Graves orbitopathy: a proposal for standardized evaluation of diplopia; Eye (Lond) Feb, 20(2): ) Chitra Selvan, Deep Dutta, Indira Maisnam, Anubhav Thukral et al. Thyroid associated Orbitopathy with ocular myasthenia in primary hypothyroidism: Keep those eyes Open. Indian j Endocrinol Metab Dec; 17 (suppl 3):S657-S659.