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VISIPer fect N Jour nal of The Venu Institute & Research Cent re

Volume 18 Issue 1 July to September 2014

Visual Functions in Amblyopia: Effect of Treatment and Pg.2 prognostic significance

How to minimize complication in SICS Pg.4

Social & Environmental Aspects of Low Vision Pg.5

Retinoblastoma Pg.6

DCR in Paediatric Patient: Pearl and Pitfall Pg.10

Visual Outcome of Penetrating Keratoplasty Pg.12

Bilateral Endogenous in Enteric Fever: Case Pg.15 report on brief review Pg.18 Idiopathic Orbital Inflammatory Disorders

From The Editor’s Desk

Dear Readers . The incidence of congenital Greetings from the Venu Family. obstruction of naso- lacrimal duct ranges between 1.75% to 20% of all infants. We take immense pride in presenting this issue of Conservative methods remain the mainstay of “Vision” to commemorate the birth anniversary treatment. However, a better understanding of of our founder: Late Dr. R.K. Seth. He was a great surgical procedure of DCR in a child may visionary who believed in reaching out to people improve the success rate. in rural areas and providing cost effective . Corneal diseases are one of the major causes of in developing countries. We begin this edition with an article on the most Corneal blindness is treatable if corneal commonly performed ocular - transplant is successfully done. Despite the surgery. Small incision is a emergence of newer lamellar keratoplasty surgical techniques commonly used in developing techniques, specific indications for penetrating countries. It results in good visual outcome and is keratoplasty still exist. The indications and useful for high volume cataract surgery. outcome of penetrating keratoplasty have been highlighted in this issue. Visual impairment affects various aspects of our life. People with low vision are unable to read Endophthalmitis is a potentially blinding ocular even with refractive correction or surgical condition. It needs urgent attention and intervention. However, early rehabilitation and treatment. We present an interesting case report support can improve their quality of life. The of bilateral endogenous endophthalmitis in a article on Low vision tells you about the social patient of enteric fever. and environmental factors to be considered while planning rehabilitation. The article on idiopathic orbital inflammatory disorders which tells about the clinical Retinoblastoma (RB), is one of the few life presentation and management. threatening conditions encountered by an ophthalmologist. A timely diagnosis and effective Wishing you a very happy Dusshera and management could thus save the life of the child . Deepawali

Most of us deal with infants presenting with Editorial Team

Managing Editor : Ms. Tanuja Joshi

Editor : Dr. Suryakant Jha

Editorial board : Dr. S.C. Gupta : Dr. Abhishek B. Dagar

Editorial Support : Mr. Jitender Saroya

July - September 2013 Journal of the Venu Eye Institute & Research Centre Perfect Vision 3 How to minimize complications in SICS Dr. Sachin Agarwal, Junior Consultant Venu Eye Institute & Research Centre

good pre operative assessment of the patient and cases aspiration of cortical matter should be done Acareful intra-operative approach will help in carefully because of poor fundal glow. minimizing the complications of SICS. Intraoperative : Factors like age, sex, physical condition, other eye Proper Side port construction is important for a good status, DM, HT are helpful in understanding the surgery. Single plane entry should be avoided. amount and type of cataract. More is the age harder will be the cataract though it may appear deceptively During tunnel construction it should neither be close soft on slit lamp. or far away from the limbus. Frowned incisions are better than others even if they are large. It is always Females tend to have more mature cataract and poor better to have easy smooth delivery from large condition of the eye. incision than struggling with a smaller wound. While entering into the AC dipping the keratome almost Poor physical condition also results in deep set vertically into the results in good valvular and hard nucleus. action which prevents iris prolapse.

DM are always risky. Capsulo Rhexis or can opener should be adequate otherwise relaxing nicks can be given to Uncontrolled BP results in extensive upthurst. If the make nucleus prolapse easier. other eye has complicated surgery then risk is higher in the eye to be operated. should be done gently to avoid zonular dialysis. One should try to prolapse one pole Ocular factors to be assessed: of nucleus out of the bag during this step. it is an Cornea may appear better on slit lamp than on important step in hard cataracts with less dilated . Moderate or large pterygium pupil. Here it can be done gently under the iris and should be operated on before cataract. Cataracts with then in the centre to make nucleus free from capsular poor cornea can be made easy by doing can-opener adhesions. This prevents capsular dialysis while capsulotomy or if capsulo rhexis is done then relaxing prolapsing large nucleus from the bag. nicks can be given to make nucleus prolapse easier. Instead of hydrodissection, hydrodelineation should IOP should be checked during examination and be done in posterior polar cataract. Size of the tunnel digital tension checked on table before starting the should always be checked for easy and quick delivery case. of nucleus. Excessive force should always be avoided. AC depth should be checked. Shallow AC results in upthurst. I/A can be started from the tunnel for 9-10'O clock then rest of the cortex can be removed easily from Iris details like atrophy, PEX, poor dilatation should side port. After removing most of the cortex, Iol may be checked. Non/less dilating can be given NSAIDS be inserted and then cortical aspiration can be for 2-3 days before surgery for sustained dilatation complete. Good hydrodissection makes I/A easy. during the surgery. IOL centration is important during dialing. AC – risky cataracts like HMSC, MSC, intumescent formation is important at the end of surgery. /morgagnian cataract, brown/black, posterior polar, subluxated ones should be examined carefully. Concluding, it is impossible to have zero rate complication, still we can minimize them gradually Fundus should be examined. In asteriod hyaloid with experience.

4 Perfect Vision Journal of the Venu Eye Institute & Research Centre Social & Environmental Aspects of Low Vision Mr. Bhaskar Ghosh, Sr. Optometrist Venu Eye Institute & Research Centre

isual impairment in general affects four main integrated in a school for normally sighted children. Vfunctional areas: Basically the problems of those in residential schools · Orientation / mobility are related to growing up in a protected environment · Communication specially designed to meet the needs of their · Activities of daily life (ADL) and impairment: this same fact is also a positive factor. In · Sustained near vision tasks the local school there may be restricted knowledge Early intervention and special education can balance concerning the disability and limited special teaching the negative effects of visual impairment in many materials and techniques and thus the child cannot cases, hence, it is essential to consider these factors in learn at an optimal level. planning rehabilitation programmes and educational support. Environment Aspects The concept of visual space is naturally difficult to Social Aspects develop in cases of severe visual impairment. For The first year of life- Congenital visual impairment example, to some visually impaired people a square often causes poor development of eye contact which is room may appear round if the contrast of corners is important in early bonding between the parents and too low to be seen. Such a simple arrangement as the child. Inactivity because of reduced visual placing lamps in the four corners makes the room stimulation is a common problem, particularly look square to the visually impaired. Normally amongst blind children. This leads, in some cases, to sighted children use plenty of tactile experiences in self stimulation that becomes a disturbing mannerism learning about space. This type of play is even more which may later be socially more handicapping than important for the visually impaired child thus special the visual impairment itself. Motor delay makes the education should be available as early as possible and child function like a younger child and increases the preferably from the first year of life. risk of overprotection that is common in the families of visually impaired children. Spatial concepts are needed for orientation. Orientation exercises can be started on special play Pre School Years mats and by arranging the child's play area. Contrasts Visual communication (i.e., non-verbal) among are more important to a visually impaired than to a sighted peers may be difficult to understand since sighted person. The arrangement of the home many clues are so subtle than even normally sighted environment and the day care centre requires special adults do not always notice them. Mild visual knowledge in illumination and low vision. impairment is often not diagnosed in infancy and the child is thought to be clumsy and inattentive when in Educational Implications reality the child is hampered by lack of normal visual The recognition that children with severely impaired information. For example, a child may “recognize” vision can benefit from specific educational methods pictures but by memorizing the location of different and adapted learning materials has gained coloured dots on the page and not having proper momentum in developed countries over the last 30 visible details. Getting accustomed to play in a large years. A multidisciplinary approach involving group of children may be problematic to blind and medical, psychological and educational professionals near-blind children who, with restricted visual has led to assessment of the child's special information have to depend heavily on auditory cues educational needs in terms of access to print and in a cacophonic environment where multiple children visually presented learning materials as well as to are talking simultaneously. programmes emphasizing visual stimulation communication and coordination. Such School Age developments have shown that children with low The social environment of the visually impaired child vision need not, and should not be educated as if they varies a lot depending on whether the child is in a were totally blind. residential school for visually impaired children or

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 5 Retinoblastoma Dr. Bhumika Sharma, Vitreo Fellow Venu Eye Institute & Research Centre

Introduction vPoor vision 5% vAsymptomatic 3% etinoblastoma is the most common intraocular vOrbital cellulitis 3% Rmalignancy in children, with a reported vUnilateral 2% incidence ranging from 1 in 15,000 to 1 in 18,000 vHeterochromia iridis 1% live births. vHyphema 1% ?The average age at diagnosis is 18 months §unilateral cases being diagnosed at around 24 Moderately advanced lesions usually present with months leucocoria due to the reflection of light by the white §bilateral cases before 12 months mass in the fundus ?6% are familial while 94% are sporadic ?In 1971, Knudson proposed the two hit hypothesis. Three patterns of tumour growth are usually seen: He stated that for retinoblastoma to develop, two chromosomal mutations are needed. In hereditary ?Endophytic, in which the tumor grows into the retinoblastoma, the initial hit is a germinal vitreous cavity. A yellow white mass mutation, which is inherited and is found in all the progressively fills the entire vitreous cavity and cells. The second hit develops in the somatic vitreous seeds occur. The retinal vessels are not retinal cells leading to the development of seen on the tumor surface. retinoblastoma. Therefore, hereditary cases are predisposed to the development of nonocular ?Exophytic, in which the tumor grows towards the tumors such as osteosarcoma. In unilateral subretinal space. usually sporadic retinoblastoma, both the hits occur during occurs and retinal vessels are seen over the tumor. the development of the retina and are somatic mutations. Therefore, there is no risk of secondary ?Diffuse infiltrating tumor, in which the tumor nonocular tumors. diffusely involves the retina causing just a placoid thickness of the retina and not a mass.This is Presentation: generally seen in older children and usually there ? Common presenting features of retinoblastoma is a delay in the diagnosis. vLeucocoria 56% vStrabismus 20% ?Advanced tumors manifest with : vRed painful eye 7% ?Proptosis secondary to extension or orbital extension and systemic metastasis ?Retinoblastoma can spread through the optic nerve with relative ease especially once the lamina cribrosa is breached. Orbital extension may present with proptosis and is most likely to occur at the site of the scleral emissary veins. Systemic metastasis occurs to the brain, skull, distant bones and the lymph nodes

Management: Investigations: vClinical evaluation with careful attention to details, aided by B-scan ultrasonographyh elps in the diagnosis. Computed tomography and magnetic resonance imaging are generally reserved for cases with atypical manifestations

6 Perfect Vision Journal of the Venu Eye Institute & Research Centre and diagnostic dilemma and where extraocular or intracranial tumor extension is suspected Group C: Discrete local disease with minimal subretinal or vitreous seeding. vUltrasonography (B-scan) shows a rounded or • Tumour(s) are discrete. irregular intraocular mass with high internal • Subretinal fluid, present or past, without seeding reflectivity representing typical intralesional involving up to one fourth of the retina. calcification. • Local fine vitreous seeding may be present close to discrete tumour. vComputed tomography delineates extraocular • Local subretinal seeding less than 3 mm (2 DD) extension and can detect an associated from the tumour. pinealoblastoma. Group D: Diffuse disease with significant vitreous vMagnetic resonance imaging is specifically or subretinal seeding. indicated if optic nerve invasion or intracranial • Tumour(s) may be massive or diffuse. extension is suspected • Subretinal fluid present or past without seeding, involving up to total retinal detachment.

Small tumors (<3mm) outside Bigger tumors (>3mm) or any • Diffuse or massive vitreous disease may include Group A macula Group B tumor in macula or any tumor or with subretinal fluid “greasy” seeds or avascular tumour masses. • Diffuse subretinal seeding may include subretinal plaques or tumour nodules.

Group E: Presence of any one or more of these Localized seeds (subretinal or Diffuse seeds (subretinal or Group C Group D vitreous) Vitreous) poor prognosis features. • Tumour touching the lens. • Tumour anterior to anterior vitreous face involving ciliary body or anterior segment. • Diffuse infiltrating retinoblastoma. • Neovascular . • Opaque media from hemorrhage. • Tumour necrosis with asaseptic orbital cellulites.

International Classification of Retinoblastoma Classification: (Shields) International Classification of Intraocular Retinoblastoma (Murphree) Group A: Small tumor Group A: Small intraretinal tumours away from • Retinoblastoma <3 mm in size in basal foveola and disc. dimension/thickness • All tumours are 3 mm or smaller in greatest dimension, confined to the retina Group B: Larger tumor • All tumours are located further than 3 mm from • R e t i n o b l a s t o m a > 3 m m i n b a s a l the foveola and 1.5 mm from the optic disc dimension/thickness • Macular location (<3 mm to foveola) Group B: All remaining discrete tumours confined • Juxtapapillary location (<1.5 mm to disc) to the retina. • Clear subretinal fluid <3 mm from margin • All other tumours confined to the retina not in Group A. Group C: Focal seeds • Tumour-associated subretinal fluid less than 3 mm • C1 Subretinal seeds <3 mm from retinoblastoma from the tumour with no subretinal seeding.

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 7 • C2 Vitreous seeds <3 mm from retinoblastoma in diameter and height) located in visually • C3 Both subretinal and vitreous seeds <3 mm noncrucial areas from retinoblastoma ?Standard 6 cycle chemoreduction and sequential Group D: Diffuse seeds aggressive focal for larger tumors and • D1 Subretinal seeds >3 mm from retinoblastoma those located in visually crucial areas • D2 Vitreous seeds >3 mm from retinoblastoma • D3 Both subretinal and vitreous seeds >3 mm ?Defer focal therapy until 6 cycles for tumors from retinoblastoma located in the macular and juxtapapillary areas

Group E: Extensive retinoblastoma ?Transpupillary thermotherapy or plaque • Occupying >50% or brachytherapy for residual tumor in the macular • Neovascular glaucoma and juxtapapillary areas >6 cycles • Opaque media from hemorrhage in anterior chamber, vitreous, or subretinal space ?Focal therapy for small residual tumor, and plaque • Invasion of postlaminar optic nerve, choroid (>2 brachytherapy/ external beam radiotherapy (>12 mm), sclera, orbit, anterior chamber months age) for large residual tumor if bilateral, and enucleation if unilateral

International Staging System for Retinoblastoma Intraocular tumor,International Classification ?Stage 0: No enucleation (one or both eyes may Group D, Unilateral or Bilateral: have intraocular disease) ?Stage I: Enucleation, tumor completely resected ?High dose chemotherapy and sequential ?Stage II: Enucleation with microscopic residual aggressive focal therapy tumor ?Periocular carboplatin for vitreous seeds ?Stage III: Regional extension ?Consider primary enucleation if unilateral, sOvert orbital disease specially in eyes with no visual prognosis sPreauricular or cervical lymph node extension ?Stage IV: Metastatic disease Intraocular tumor, International Classification sHematogenous metastasis Group E, Unilateral or Bilateral: Single lesion Multiple lesions ?Primary enucleation sCNS Extension ?Evaluate histopathology for high risk factors Prechiasmatic lesion CNS mass High risk factors on histopathology, International Leptomeningeal disease Staging, Stage 2

Treatment: ?Baseline systemic evaluation for metastasis The primary goal of management of retinoblastoma ?Standard 6 cycle adjuvant chemotherapy is to save life. Salvage of the organ (eye) and function ?High dose adjuvant chemotherapy and orbital (vision) are the secondary and tertiary goals external beam radiotherapy in patients with respectively scleral infiltration, extraocular extension, and optic nerve extension to transected end. Intraocular tumor,International Classification Group A to C, Unilateral or Bilateral: Extraocular tumor, International Staging, Stage 3A: ?Focal therapy (cryotherapy or transpupillary ?Baseline systemic evaluation for metastasis thermotherapy) alone for smaller tumours (< 3mm ?High dose chemotherapy for 3-6 cycles, followed

8 Perfect Vision Journal of the Venu Eye Institute & Research Centre by enucleation or extended enucleation, external ?High-dose (3 weekly, 6-12 cycles): Vincristine beam radiotherapy, and continued high dose 0.025 mg/Kg, Etoposide 12mg/Kg, Carboplatin 28 chemotherapy for 12 cycles mg/Kg

Regional Lymph Node Metastasis, International Histopathologic high-risk factors predictive of Staging, Stage 3B: metastasis:

?Baseline evaluation for systemic metastasis ?Anterior chamber seeding ?Neck dissection, high dose chemotherapy for 6 ?Iris infiltration cycles, followed by external beam radiotherapy, ?Ciliary body infiltration and continued high dose chemotherapy for 12 ?Massive choroidal infiltration cycles ?Invasion of the optic nerve lamina cribrosa ?Retrolaminar optic nerve invasion Hematogenous or Central Nervous System ?Invasion of optic nerve transection Metastasis, International Staging, Stage 4 ?Scleral infiltration ?Extrascleral extension ?Palliative therapy in discussion with the family ?High dose chemotherapy with bone marrow rescue International Staging System for Retinoblastoma for hematogenous metastasis Stage 0 No enucleation (one or both eyes may ?High dose chemotherapy with intrathecal have intraocular disease chemotherapy for central nervous system metastasis Stage 1 Enucleation ,tumor completely rescted Stage 2 Enucleation with microscopic residual Special considerations for enucleation in tumor retinoblastoma: Regional extension ?Minimal manipulation A. Overt orbital disease ? Stage 3 Avoid perforation of the eye B . Preauricular or cervical lymph node ? Harvest long (> 15 mm) optic nerve stump extension ?Inspect the enucleated eye for macroscopic extraocular extension and optic nerve involvement Metastatic disease ?Harvest fresh tissue for genetic studies 1. Single lesion ?Place a primary implant ?Avoid biointegrated implant if postoperative 2. Multiple Lesions radiotherapy may be necessary Stage 4 B CNS Extension

Chemoreduction regimen and doses for 1. Prechiasmatic lesion intraocular retinoblastoma 2. CNS Mass ?Day 1: Vincristine + Etoposide + Carboplatin 3. Leptomeningeal disease ?Day 2: Etoposide ?Standard dose: (3 weekly, 6 cycles): Vincristine 1.5 mg/m2 (0.05 mg/kg for children < 36 months Conclusion of age and maximum dose < 2mg), Etoposide 150 Retinoblastoma is a rare childhood ocular tumour mg/m2 (5 mg/kg for children < 36 months of age), where early detection and appropriate management by Carboplatin 560 mg/m2(18.6 mg/kg for children < a specialized team of professionals can lead to salvage 36 months of age) of the eyeball and perhaps,vision.

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 9 DCR in the Paediatric Patient: Pearls and Pitfalls Dr. Vishal Nigam, Jr. Consultant Oculoplasty Venu Eye Institute & Research Centre

ongenital obstruction of the Naso-lacrimal duct DCR, special emphasis should be given to C(NLD) occurs in 1.75 to 20% of all infants. hemostasis in children due to their smaller blood Patients usually present with epiphora, matting of volume. eyelashes and purulent discharge.Conservative modes ?Risk of bleeding can be reduced by use of of treatment such as massage, topical antibiotics, hypotensive , local infiltration probing and silastic remain the mainstay in around the medial with local anaesthetic the management of congenital NLDO. However, in mixed with epinephrine and also by meticulous cases where multiple probings have failed, or when nasal packing. the child presents at a later age with chronic ?In rare cases where severe haemorrhage does dacryocystitis or mucocele, an External occur, transfusion may be required and this (DCR )is required. possibility should be discussed with the parents DCR in children if often considered an unpredictable preoperatively. procedure with a lower success rate than in adults. Reasons which are often cited for failure of DCR in Surgical Steps children include ?The surgery is performed under hypotensive · Poorly defined anatomy general anaesthesia · Rapidly changing anatomy ?Local infiltration is done with 2 ml of 2% · Tendency towards vigorous growth of scar Lignocaine mixed with 1:200,000 epinephrine. tissue ?Nasal packing is done with a moist ribbon gauze with the tip soaked in 2% Lignocaine mixed with However, with a better understanding of the subtle 1:200,000 epinephrine. modifications in surgical procedure which need to be ?Straight 8-10mm incision placed medial to the made while operating on a child, many studies have angular vessels. now proved that success rates of DCR in children are ?Orbicularis fibres are dissected by blunt dissection comparable to those in adults. to expose the medial palpebral ligament and the periosteum over the anterior lacrimal crest. In this presentation, we shall discuss the differences ?The is lateralized along with the which one should expect to encounter when operating overlying periosteum by carefully incising and on a child and special precautions which need to be lifting the periosteum over the anterior lacrimal taken for the same crest. ?The medial wall of the lacrimal fossa is punctured Differences in anatomy with a blunt dissector and the osteotomy is ?Lacimal fossa and anterior lacrimal crest are enlarged. poorly defined in children making it difficult to ?In children, it is important to carefully recognise recognise the proper site for initiating the the anatomic landmarks and not be overly osteotomy. The medial canthal tendon thus aggressive while enlarging the osteotomy. provides an important landmark to identify the ?The extent of the osteotomy can be roughly superior portion of the anterior lacrimal crest. described as follows ?The ethmoid sinuses are anteriorly placed and not wSuperiorly : Upto the medial palpebral prominent in children. ligament ?Though severe haemorrhage is uncommon during wInferiorly : Beginning of the nasolacrimal

10 Perfect Vision Journal of the Venu Eye Institute & Research Centre canal surgery is assessed by clinical observation of wPosterior : Posterior lacrimal crest reduction in epiphora and discharge wAnterior : Beyond the anterior lacrimal crest ?Patients are followed up after 1 week, 1 month, 3 months and then after 6 months. ?A 0-0 Bowman's probe is intubated through the superior canaliculus to aid in identification of the Conclusion lacrimal sac. Excellent results can be obtained with external ?Careful attention to direct visualisation of the sac paediatric DCR if appropriate evaluation is done and with the use of the probe, and to the nasal anatomy precautions are taken. with the use of a nasal speculum and periosteal elevator is of paramount importance. ?Anterior flaps from the sac and nasal mucosa are References fashioned. Nowinski TS, Flanagan JC, Mauriello J. Pediatric ?The flaps are sutured with 3 interrupted 6-0 Vicryl dacryocystorhinostomy. Arch Ophthalmol (Polyglactin 910) sutures, out of which the central 1985;103:1226–8. suture is taken last to include bites through the Orbicularis to allow 'tenting-up' of the flap. Barnes EA, Yassir Abou-Rayyah, Rose GE, Pediatric Dacryocystorhinostomy for Nasolacrimal Duct ?Subcutaneous tissue and skin are sutured with 6-0 Obstruction, September 2001;108: Vicryl (Polyglactin 910) 1562–64

Post Operative management Takahashi Y, Kakizaki H, Chan WO, Selva D. ?Topical and Oral antibiotics are prescribed. Management of congenital nasolacrimal duct ?Adequate analgesia is important. In most cases an obstruction. Acta Ophthalmol. 2010 Aug;88(5):506- oral analgesic and anti inflammatory is sufficient. 13. ?Cold compresses External Dacryocystorhinostomy for Paediatric ?Advise the child to sleep with the head raised to Nasolacrimal Duct Obstruction. Rajat Maheshwari. minimise bleeding. Asian J Ophthalmol. 2006;8:242-4 ?Nasal pack is removed on the first post operative day. Kirsten RC. Congenital Lacrimal Abnormalities. In: ?Saline irrigation is performed, whenever possible, Bosniak S, editor. Ophthalmic Plastic and to confirm patency. r e c o n s t r u c t i v e s u r g e r y . Vo l . 2 , W B ?When syringing is not possible, success of the SaundersCompany; 1996: 777-83.

Quotable Quotes

“Few people get weak eyes from looking on the bright side”

-Anonymous “Of all the senses, sight must be the most delightful”

-Helen Keller

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 11 Visual Outcome of Penetrating Keratoplasty Dr. Ajay Kumar, Dr. Jayeeta Bose, Dr Amit Budrukkar Venu Eye Institute & Research Centre

bstract responsible for at least 25% of all blindness.4 In India, A the proportion of corneal blindness is estimated to be Objectives: 0.89%-14.7% in various studies done in different (1) To identify common clinical indications for regions of the country.5 penetrating keratoplasty (PK). Despite the emergence of newer lamellar keratoplasty (2) To evaluate visual outcome after PK. techniques, specific indications for penetrating keratoplasty (PK) still exist. These indications Study design: include corneal scarring, adherent leukoma, active Tertiary care centre based, retrospective, non- infectious keratitis, aphakic or pseudophakic bullous comparative, observational, case-series study. keratopathy, corneal dystrophies and keratoconus. The prognosis for penetrating keratoplasty in the Material and Methods: industrialised world has improved greatly during the Data was obtained from records of patients operated past 20 years, reasons being the relative ease in for PK in Venu Eye Institute & Research Centre, New mastering the technique and the smaller learning Delhi from January 2011 to April 2014. Data extracted curve compared to various lamellar techniques. includes age, gender, type of corneal and However, relatively little is known about the final best corrected visual acuities. effectiveness and role of penetrating keratoplasty in countries of the developing world. As so much Results: emphasis has been placed on cataract surgery in 124 eyes of 124 patients suffering from corneal developing countries like India that program dealing blindness were operated with PK. Various clinical with other causes of blindness has been neglected.6 indications for PK were corneal scarring in less than 2 quadrants in 63 (51%), corneal scarring in more than 2 Corneal blindness is treatable if corneal transplant is quadrants in 51 (41%) eyes, bullous keratopathy in 6 successfully done and managed post-operatively. (5%) eyes, corneal dystrophy in 4 (3%) eyes. Corn Von Hipple in 1888 did the first successful corneal transplant in human beings while Zirm (1906) Conclusion: and Magitot (1911) described the fundamental Corneal scarring is the most common indication for principles of homograft and preservation of cornea.7 PK. Out of various clinical indications for PK, poorest visual outcome is seen in vascularised corneal The data available on the results of penetrating scarring. keratoplasty in developing countries is very limited. Also, none of the studies available show the Introduction: correlation between pre-operative corneal pathology Corneal blindness is second to cataract as a cause of and the post-operative visual outcome. We are 1 visual impairment. Major causes of corneal blindness presenting a case-series study of 124 cases with are corneal scarring due to inflammation, ocular various causes of corneal blindness, their treatment trauma, corneal ulceration, xerophthalmia, with penetrating keratoplasty and their results in ophthalmia neonatorium and over-the-counter eye terms of visual outcome. . Trachoma causes corneal blindness in 4.9 million while ocular trauma and corneal ulceration Surgical technique: add 1.5 to 2 million new cases worldwide every year.2 A standard technique by single surgeon was used The prevalence of corneal blindness varies from one throughout. The donor corneal button was trephined population to other, depending on many factors like from the endothelial surface of the corneoscleral availability and general standard of eye care. In the button. The diameter was 0.25 to 0.5 mm larger than UK, corneal blindness accounts for 2% of blind that of the recipient bed. The mean diameter of the registrations3, while in Africa corneal disease is donor corneal button was 8.0 mm (range, 7.5– 8.5

12 Perfect Vision Journal of the Venu Eye Institute & Research Centre mm), and the mean diameter of the recipient bed was Post operative BCVA at 3 months: (Table-2) 7.5 mm (range, 7.0–8.0 mm). Standard surgical technique was used, and the donor cornea was sutured Group A Group B Group C Group D in place with 10-0 nylon using interrupted sutures in PL-6/60 16 17 2 2 all 124 eyes (100%). Post-operative medications included systemic and topical steroids, topical >6/60-6/24 28 18 3 2 antibiotics, cycloplegics and anti glaucoma drugs in >6/24 19 16 1 0 selected cases. Daily ocular examination was done for one week and review examination done monthly for Total 63 51 6 4 one year, followed by 2 monthly examination for next 3 years. Optical clarity of cornea and refractive media, Percentage of patients with BCVA between 6/60- visual acuity and status were 6/24 in various groups were as follows: determined at each visit. Removal of sutures was done after 4 months. Refraction was first performed 6 weeks after suture removal. Spectacles were prescribed according to need and the patient's individual preference. Corneal transplant was considered to failure if cornea was hazy due to any reason.

Results: A total of 124 corneal transplants were performed. Amongst them 87 were male and 37 female. All patients were above 10 years of age, 43 patients were from 11-19 years, 38 patients from 20-39 years, 40 Discussion: patients from 40-59 years and 3 patients from 60 years Corneal blindness or diseases is the second most 1,2 of age or above. Indications for keratoplasty are given important cause of blindness in the world. Eighty in (Table-1). Post-operative visual acuity is shown in percent of patients with blindness live in developing (Table-2). Post operative visual acuity was 6/24 or world. The corneal blindness is mainly due to corneal better in 28 of 63 eyes (31.25%) with corneal scarring ulcer, trauma, nutritional deficiency and traditional less than 2 quadrants, 18 of 51 eyes (35.29%) with use of . In India, ocular trauma, infectious corneal scarring more than 2 quadrants, 3 of 6 eyes keratitis, corneal ulceration and post-infectious (50%) with bullous keratopathy and 2 of 4 eyes(50%) keratitis contribute significantly to pediatric ocular with corneal dystrophy (Graph-1). morbidity. Congenital corneal disorders are also an important cause of childhood blindness that usually Various clinical indications for penetrating result from hereditary dystrophies, congenital keratoplasty: (Table-1) glaucoma, Peter's anomaly and other mesenchymal dysgenesis, birth trauma and metabolic disorders. Group Clinical diagnosis Number of patients In adults, the major causes of corneal blindness A Corneal scarring with vascularization 63 include bacterial, fungal or viral keratitis, hereditary <2 quadrants corneal dystrophy and eye injuries.5 Many of these B Corneal scarring with vascularization 51 diseases are preventable, and prevention of causes >2 quadrants would be the preferred method. However, in those C Bullous keratopathy 6 suffering from corneal blindness, visual D Corneal dystrophy 4 rehabilitation with is the only TOTAL 124 hope. Our results differ slightly from previous studies

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 13 in Indian patients, where indications for corneal PK. In majority of patients post PK with interrupted transplant were reported as corneal scar (28.1%), suturing BCVA is >6/60-6/24. Poorest visual outcome failed corneal graft (17.1%), keratitis (12.2%), bullous is seen in vascularised corneal scarring. keratopathy (10.6%) and keratoconus (6%)8. References: The frequency of indications for corneal transplant in 1. Congdon NG, Friedman DS, Lietman T. Important our study were different from USA, UK and France.9 causes of visual impairment in the world today. In USA bullous keratopathy (27.2%), failed graft JAMA 2003; 290: 2057-60. (18.1%), keratoconus (15.4%), corneal scar (7.8%), 2. Whitcher JP, Srinivasan M, Upadhyay MP. Corneal and keratitis (2.9%) were the main indications for blindness: A global perspective. Bull World Health corneal transplant.10,11 Organ 2001; 79 (3): 214-21. 3. Foster A. Patterns of blindness. Duane's clinical ophthalmology. Philadelphia: Lippincott, 1991;5: In our study corneal scar (31.25%), bullous Chap 53. keratopathy (20%) and corneal degenerations (12.5%) 4. Bulletin of the World Health Organization, 2001, 79: were the main indications for penetrating keratoplasty. 214–221. Thus in developed countries the causes are mainly 5. Gupta N, Tandon R, Gupta SK, Sreenivas V, Vashist pseudophakic bullous keratopathy, keratoconus, P. Burden of corneal blindness in India. Indian J failed corneal graft and corneal dystrophy while in Community Med 2013;38:198-206 developing countries, corneal scar (following 6. Dandona L. Is current eye care policy focus almost inflammation or trauma) are still most common. exclusively on cataract adequate to deal with Corneal transplant is successful if well trained blindness in India? Lancet 1998; 351:1312-16. surgeons and nurses are available as well as modern 7. Roper Hall MJ. Corneal transplant history. Stallard operating rooms, good equipments, reliable eye bank eye surgery. 7th Ed. Wright; London. Boston, facilities, clinical services for long term follow up and Singapore, Sydney. 1987. Page 202. treatment of graft rejection and other postoperative 8. Dandona L, Ragu K, Janarthanan M, Naduvilath TJ, complication.12 Shenoy R, Rao GN. Indications for penetrating keratoplasty in India. Indian J Ophthalmol 1997; 45: In our set up all these facilities were available. The 163-68. outcome of keratoplasty is defined in two ways. From 9. Al-Yousuf N, Mavrikakis I, Mavrikakis K, Daya SM. surgeon point of view the graft clarity indicates Penetrating keratoplasty: indications over a 10-year technically a successful surgery. From patient point of period. Br J Ophthalmol 2004; 88: 998-17. view the recovery of useful vision is important. As the 10. Cosar CB, Sridhar MS, Cohen EJ, Held EL, Alvim PT, Rapuano CJ et al. Indications for penetrating recovery of vision depends on many factors like keratoplasty and associated procedures, 1996-2000. amblyopia, retinal and optic nerve pathology, the graft Cornea 2002; 21: 148-51. clarity is the suitable way of assessing the success of 11. Dobbins KR, Price FW, Jr & Whitson WE. Trends in surgery. A larger sample size is needed for valid the indications for penetrating keratoplasty in the comparative study applicable to general population. Midwestern United States. Cornea 2000; 19: 813-16. 12. D Yorstan, M Wood, A Foster et al. Penetrating Conclusion: keratoplasty in Africa. Graft survival and visual Corneal scarring is the most common indication for outcome. Br J Ophthalmol 1996; 80; 890-94.

“Behind every great man is a woman rolling her eyes”

- Jim Carey

14 Perfect Vision Journal of the Venu Eye Institute & Research Centre Bilateral Endogenous Endophthalmitis in Enteric fever: Case report and brief review Dr. Om Prakash Gupta Venu Eye Institute & Research Centre BSTRACT A On ophthalmic examination her vision was HMCF in Endogenous endophthalmitis is a potentially blinding RE and PL positive in LE with accurate PR in both ocular resulting from hematogenous spread eyes. Her RE showed ciliary congestion, fine KPs, from a remote primary source. We report a case of grade 2 cells and fibrinous exudates in AC. LE bilateral (B/L) endogenous endophthalmitis showed ciliary congestion, fine KPs, grade 3 cells secondary to enteric fever in a 23 year old with fibrinous exudates in AC. Iris & pupil details immunocompetent girl. She reported bilateral were not clearly visible. Distant direct blurring of vision 2 days following admission to showed yellow fundus glow and medical ward for enteric fever. Her visual loss details of fundus were not visible. However, there progressed to hand movement close to face (HMCF) was a doubtful area of infiltration in retina in right in right eye (RE) and perception of light (PL positive) eye. Intraocular pressure (IOP) by applanation in left eye (LE) over next 2 days. On the basis of tonometry was 3 mm Hg in RE and 1 mm Hg in LE. clinical presentation & blood investigations she was USG showed low to medium intensity spikes diagnosed as a case of bilateral endogenous suggestive of vitreous exudates. On the basis of endophthalmitis caused by Salmonella typhi . history & clinical findings, provisional diagnosis of B/L endogenous endophthalmitis was made. CASE REPORT Intravenous (IV) antibiotic was changed to Endogenous endophthalmitis is an intraocular combination of ceftriaxone (1gm twice a day) & infection resulting from hematogenous spread of the vancomycin (1gm twice a day). Topical antibiotic- micro-organism(s) from a focus of infection steroid combination and cycloplegic (atropine) were elsewhere in the body. Generally associated with started. Vitreous tap with was immunocompromised states, debilitating diseases or planned but patient did not give consent for any invasive procedures, it accounts for 2 to 8 percent of surgical intervention. all cases of endophthalmitis.1 Early diagnosis and prompt aggressive treatment are imperative if visual After changing the antibiotics, patient's fever loss is to be prevented. subsided within 72 hours and her vision in RE improved to finger counting (FC) 1m & LE FC 2 m A 23 year old immunocompetent girl suffering from with improvement in AC reaction. Repeat cultures of fever since 5 days was admitted in medical ward. On blood after 3 days showed no growth. After 72 hours, admission, blood samples were taken for routine intra venous 8 mg BD was given for 2 exam, culture, widal test and peripheral smear. She days. Intravenous antibiotics were continued for 2 was started on intravenous ampicillin along with weeks. After 2 weeks, on discharge, her vision in RE supportive therapy. The patient reported bilateral was FC 2m & in LE FC 4 m. Patient has completed blurring of vision 2 days following admission. On more than 1 year of follow up, her gain of vision is systemic examination mild hepato-splenomegaly was still maintained with no sign of any deterioration or present. Blood profile showed Hb 8 gm%, TLC- relapse. 9000/mm3 with 76 % neutrophils and ESR 25mm in 1st hr by Westergren's method. Fasting & PP blood DISCUSSION sugar were normal. LFT showed mild elevation of Endophthalmitis can be infectious or non-infectious.2 alkaline phosphatase & lactate dehydrogenase. Chest Bacteria are the most common microorganisms that x-ray & KFT were within normal limits. HIV was non- cause this inflammation. 3 Virtually any reactive. Echocardiography showed no vegetations. microorganism can cause endophthalmitis but gram- Blood culture was positive for S.typhi. Widal test in positive cocci and gram-negative bacilli are the most 2nd week showed O antigen positive in titre 1:160 common. Most cases of bacterial endophthalmitis are dilution. USG abdomen confirmed mild hepato- from an exogenous source.3 Candida is the most splenomegaly with no infective foci. common organism causing endogenous

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 15 endophthalmitis.4 Risk factors for infection with this both chambers before institution of antibiotic therapy organism include intravenous drug use, surgery, give the highest yield for isolating the pathogen. malignancies, intravenous hyperalimentation, endovascular lines, diabetes, and the use TREATMENT o f b r o a d - s p e c t r u m a n t i b i o t i c s a n d Prompt administration of antibiotic therapy is the key immunosuppressive medications, especially to the management of acute endogenous corticosteroids.5 endophthalmitis. This condition is particularly responsive to intravenous antibiotics while in Extensive literature search could find only a few cases exogenous endophthalmitis, systemic antibiotics are of Endogenous endophthalmitis due to Salmonella. not actually necessary.12 Systemic antibiotics also Two reported cases were reported in 1-year-old treat distant foci of infection and prevent continued children,6,7 one case has also been reported in a HIV bacteremia thereby reducing chances of invasion of positive patient8 and another in a patient of Chronic the unaffected eye. Empiric broad-spectrum Myeloid Leukemia with sepsis.9 One case of bilateral antibiotic therapy with vancomycin and an panophthalmitis by Salmonella typhi has also been aminoglycoside or a third-generation cephalosporin reported.10 is warranted. In cases of severe typhoid fever (delirium, obtundation stupor or coma or septic shock Endogenous endophthalmitis can occur at any age and & positive culture for S. typhi or S. paratyphi A), has no sexual predilection. The right eye is involved dexamethasone treatment should be considered. A twice as often as the left eye because of the more single dose of dexamethasone 3mg/kg followed by 8 proximal and direct blood flow to the right carotid doses of 1mg/kg given every 6 hours decreased the artery.11 Bilateral involvement is seen in fewer than mortality rate from 56% to 10% in a patient of enteric 25% of cases.1,2 fever.6

At least 2 of the following 3 criteria must be met for a Intravitreal antibiotic injections have revolutionized diagnosis of endogenous bacterial endophthalmitis: the treatment of exogenous endophthalmitis but their positive blood culture(s), isolation of the same usefulness in endogenous cases is controversial. bacteria from vitreous or aqueous fluid as from blood Similarly, benefits of surgical intervention (i.e. cultures and posterior and/or anterior ocular ) in endogenous endophthalmitis have inflammation for which no cause other than infection been debated.11 The outcome of endogenous has been identified.1 Common clinical features of endophthalmitis (compared with that of exogenous endogenous bacterial endophthalmitis include endophthalmitis) is disappointing. The three main decreased visual acuity, pain, hypopion, fever, chills, factors that result in a poor prognosis include more headache and conjunctival hyperemia.1,2 Systemic virulent organisms, compromised host conditions and symptoms and ocular pain may be absent on initial delay in diagnosis. Even with aggressive treatment, presentation. Slit lamp examination and ocular vision is preserved in only about 40 percent of ultrasonography should be performed to look for patients (i.e. ability to count fingers or better).13 anterior vitreous haze/ cells and vitreous exudates/ However our patient became visually independent retinochoroidal thickening respectively. following treatment.

In addition to initial diagnostic laboratory tests, Figure 1: (a) testing for human immunodeficiency virus (HIV) infection should be considered in otherwise healthy persons with endophthalmitis. Routine radiographs RE On presentation may reveal a primary pulmonary infection. showing areas of Echocardiography is also warranted to assess the infiltration seen through hazy media possibility of endocarditis. Other tests may be necessary, depending on the clinical presentation. Blood cultures and intraocular cultures obtained from

16 Perfect Vision Journal of the Venu Eye Institute & Research Centre Figure 1: (b)

Severe vitreous exudation with cob web appearance Fig 3:(a&b) Appearance of right and left eye after 1 year showing healing with scarring

References 1. Okada AA, Johnson RP, Liles WC, D'Amico DJ, Baker Figure 1: (c) AS. Endogenous bacterial endophthalmitis. Report of a ten-year retrospective study. Ophthalmology USG showing 1994;101:832-8. low to moderate 2. Pflugfelder SC, Flynn HW Jr. Infectious intensity spikes endophthalmitis. Infect Dis Clin North Am suggestive of 1992;6:859-73. vitreous 3. Endophthalmitis Vitrectomy Study Group. exudates Microbiologic factors and visual outcome in the endophthalmitis vitrectomy study. Am J Ophthalmol. Dec 1996;122(6):830-46. 4. Brod RD, Flynn HW Jr. Endophthalmitis: current approaches to diagnosis and therapy. Curr Opin Infect Dis 1993;6:628-37. 5. Fraser VJ, Jones M, Dunkel J, Storfer S, Medoff G, Figure 1: (d) Dunagan WC. Candidemia in a tertiary care : epidemiology, risk factors, and predictors of mortality. LE on Clin Infect Dis 1992;15:414-21. presentation 6. Endogenous endophthalmitis due to Salmonella showing hazy typhimurium. Shohet, . Davidson, . Boichis, . media Rubinstein. Annals of ophthalmology Apr 1, 1983 7. Endophthalmitis due to Salmonella typhimurium. Appel, I.; Landman, I.; Savir, H.; Journal of and MEDLINE Abstracts 8. Endogenous endophthalmitis due to Salmonella choleraesuis in an HIV-positive patient. Yodprom, Rapeeporn; Pathanapitoon, Kessara; Kunavisarut, Paradee; Ausayakhun, Somsanguan; Wattananikorn, Sopa; Rothova, Aniki Ocul Immunol Inflamm. ;15 (2):135-8. 9. Metastatic endophthalmitis due to Salmonella typhimurium. Weinstein, J. M.; Elliott, J.; Tilford, R. H. Arch Ophthalmol. 1982 Feb ;100 (2):293-5 . 10. Bilateral endogenous panophthalmitis caused by Salmonella typhi: first case Fig 2: (a & b) RE & LE respectively after 48 hours, report. R Arora, S Das, D Chauhan,S showing clearing media Daraius, R Narula, R Sachdev Orbit. 2008 Jun ;27 (2):1157 .

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 17 Idiopathic Orbital Inflammatory Disorders Dr. Vishal Nigam Venu Eye Institute & Research Centre

diopathic orbital inflammatory disorders (IOID) 'inflammatory orbital pseudotumour' by Birch- Ialso referred to as Orbital Pseudotumour is a Hirschfeld to describe a clinical mass clinically heterogeneous group of disorders characterized by mistaken for a neoplasm but histologically proven to orbital inflammation without any identifiable local or be inflammatory. However, the term 'Pseudotumour' systemic cause. It is a well recognized pathological was widely criticized at the time due to its negative identity which remains mostly confined to the orbit. implication. It referred to what the mass was not, Though extensive research has been done on this instead of referring to what it truly was. Hogan and group of disorders, the diagnosis of Pseudotumour Zimmerman coined the term 'inflammatory still remains a diagnosis of exclusion. Due to the nonneoplastic orbital pseudotumour'. The term variable nature of presentation, both in terms of 'idiopathic orbital inflammation' was proposed by severity and extent, no standardized classification Jacobiec and associates. system has been universally accepted. It usually presents as an orbital mass with infiltration of soft However, despite the initial objections, the term tissues by inflammatory cells and fibrous tissue in the pseudotumour has now detached itself from its initial absence of any identifiable cause. However the negative definition and has evolved to indicate a diagnosis may often be finally made after noting the process of idiopathic, nonspecific orbital clinical response to systemic steroids. inflammation. It is still the most widely accepted terminology for this group of disorders.

ETIOLOGY Thought the term itself signifies that the cause of the disorder is unknown, various theories have been postulated. None, however has been verified.

Initial reports of orbital myositis following upper Young female patient Pseudotumour of the respiratory tract or infectious sinusitis presenting with orbit presenting as suggested an infectious etiology for the illness. Cell eccentric proptosis of fullness of the left upper wall-deficient bacteria identified in orbital leucocytes the right eye lid were noted in tissue biopsies of some patients.

Birch-Hirschfeld in 1905 was the first to describe this An autoimmune pathogenesis was suggested by disorder as a mysterious orbital syndrome with a Easton and Smith which corresponds with the clinical impression of orbital benign or malignant occasional presence of coexistent autoimmune neoplasm in which, at the time of surgical exploration, disorders such as diabetes mellitus, rheumatoid only inflammatory tissue was found. However, for a arthritis and systemic lupus erythematosus. Recently, long time this diagnosis was inadvertently given to a circulating antibodies against extraocular muscle lot of disorders where the cause was not identifiable at proteins have been detected in patients with orbital the time. The most common disorders often confused pseudotumour. with Pseudotumour include orbital tumours, thyroid and systemic causes of inflammatory Barrett initially proposed that a pseudotumour may be mass lesions. With improved diagnostic and imaging a result of a fibroproliferative disorder, similar in techniques, the diagnosis of Pseudotumour is now etiology to idiopathic mediastinal fibrosclerosis. The made a lot more prudently. proposed pathogenesis is an aberrant immune- mediated exaggerated proliferation of fibroblasts and HISTORICAL ASPECT deposition of extracellular matrix. The initial term 'pseudoplasm malin' (malignant pseudoplasm) given by Panas was changed to Though all the mentioned theories merit discussion,

18 Perfect Vision Journal of the Venu Eye Institute & Research Centre none have been substantiated. The response to ?perioptic nerve inflammation corticosteroid and immunosuppressive therapy ?trochleitis suggests an immunological process but disputes the ?an inflammatory process restricted to the vicinity theory of an infectious origin. Similarly, the fact that of superior orbital fissure and cavernous sinus the disorder is most often unilateral goes against the (Tolosa – Hunt Syndrome) immunological theory. The exact nature of the ?a diffuse anterior soft tissue inflammation disorder and its origin continue to remain a mystery. ?sclerosing orbital inflammatory pseudotumor

CLINICAL PRESENTATION The presenting complaints of the patient depend Due to the varied presentation it has been difficult to upon the extent of the lesion and its anatomic location lay down strict guidelines for the clinical diagnosis of and also on the extent of fibrotic component in the a pseudotumour. Though it can occur at any age and in lesion. The clinical picture may be that of slowly either sex, it has been found to occur more commonly progressive proptosis with restriction of ocular within the age group of 40-60 years and occurs more movements and optic nerve compression. In contrast, frequently in male patients. It is almost always it may present with severe pain, congestion and unilateral, except in myositic pseudotumour. edema resembling an acute inflammatory episode.

On the basis of progression of symptoms, the disease process may be classified as an acute pseudotumour, where symptoms develop over days, as subacute where symptoms take weeks to develop, or as chronic where the disease progresses slowly and insidiously over a period of months.

IMAGING A CT scan is often the modality of choice for imaging an orbital pseudotumour. Though the presentation on CT scan may be as variable as on clinical examination, all lesions, irrespective of location, show 55 year old male patient presented with diplopia on up-gaze and i r r e g u l a r m a r g i n s w i t h swelling of the right upper lid since 4 weeks. CT scan showed an ill- enhancement on contrast defined heterogeneously enhancing soft tissue lesion involving administration. Though the superolateral quadrant of right orbit, suggestive of Orbital lesion in the specific region may Pseudotumour. Patient was treated with a short course of parenteral mimic other disorders, the fact steroids followed by tapering dose of oral steroids, following which that often more than one site is the patient improved both clinically and subjectively. involved in pseudotumour may give a clue towards the diagnosis. Some of the forms in which a pseudotumour may present include : In the lacrimal gland, the lesion is limited by the ?dacryoadenitis tough capsule of the gland, giving it a well ?myositis circumscribed appearance. It may suggest a ?sclerotenonitis neoplasm of the lacrimal gland, but is often

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 19 accompanied by inflammatory features surrounding to pink in colour, firm and rubbery in consistency and the gland. can affect any structure in the orbit. Features on histopathology may be described under four In the , the inflammation is headings: the cellular infiltrate, the stromal limited by the muscle sheath but not as strongly as in component, the vascular changes and the changes in the lacrimal gland. The lesion spreads linearly, which involved orbital structures. causes swelling of the muscles but not as severely as in Graves disease. Also, the involvement of the lateral The cellular infiltrate resembles features suggestive rectus along with the tendinous attachment of the extra of chronic inflammation, with predominantly small, ocular muscles help differentiate it from thyroid well defined mature lymphocytes admixed with related orbitopathy. plasma cells, neutrophil and eosinophil granulocytes and occasionally histiocytes and macrophages. Lesions near the apex of the orbit may be harder to diagnose and are often mistaken for a meningioma. The stromal changes range from edema, proliferative Unlike a meningioma, however, the forward border of fibrosis to sclerosis to finally hyalinization. the lesion is irregular and spreads forward in a fan-like manner. Orbital pseudotumours are richly vascular due to capillary proliferation. Common vascular changes include perivasculitis with lymphocyte infiltration in the capillary adventitia. Endothelial cells are swollen, hypertrophic and increased in number. The collagen of the adventitia is thickened.

Changes in involved orbital tissue vary with the location of the lesion. Orbital fat, when involved 67 year old male patient presented with axial proptosis of shows features of mixed inflammatory infiltrate the left eye. Patient was on anti-thyroid medications over with increased fibrous tissue. The delicate the last six years. Ultrasound B-scan revealed thickening interlobular septa are thickened and become of the SR-LPS complex of both eyes with all other confluent. Lipogranuloma formation may occur. muscles being of normal thickness. CT scan revealed Within the lacrimal gland, lymphocytic and bilateral intraconal heterogeneously enhancing mixed plasmocytic infiltration occurs with periductal and density lesion (Left > Right) which was pushing the periacinar fibrosis. Interlobular septa become globe outwards. A diagnosis of bilateral pseudotumour thickened with fibrosis. In cases with myositis, was made and patient was treated with parenteral steroids followed by tapering dose of oral steroids. infiltration with lymphocytes and plasma cells occurs in a diffuse or multifocal pattern. The normal ROLE OF TISSUE BIOPSY striations are lost and the fibers degenerate. A fine needle aspiration biopsy is not helpful in the diagnosis of orbital pseudotumour as even an MANAGEMENT AND COURSE underlying malignancy may present with areas of Orbital pseudotumour is believed to be a self limiting non-specific inflammation. A tissue biopsy should be disorder. Although benign, it may run an aggressive attempted, except in the myositic form of the disease course when the eye, extraocular muscles and optic where damage to the extraocular muscles may affect nerve may be at risk. Therapy during this phase is ocular motility. Also a biopsy may be avoided in directed towards relieving patient discomfort and to posteriorly located lesions near the optic nerve for fear prevent acute as well as long term damage to the optic of damaging the nerve. nerve, muscles.

HISTOPATHOLOGY Treatment options have been listed in the following Macroscopically, a pseudotumour is yellowish-gray anagram

20 Perfect Vision Journal of the Venu Eye Institute & Research Centre Stage 1 : Lesion seen on CT scan

Oral steroids, Topical steroids,

Good response Unresponsive: Stage 2

Taper steroids Intravenous Dexamethasone

Unresponsive: Stage 3 Good response

Histopathological confirmation Taper with oral steroids

Intralesional Triamcinolone

Taper steroids

Good response Unresponsive: Stage 4

Taper with oral steroids Orbital Irradiation

Unresponsive: Stage 5

Immunosuppressants

Good response Unresponsive

Taper with oral steroids Repeat tissue biopsy

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 21 CONCLUSION Though pseudotumour is a convenient diagnosis Mombaerts I, Schlingemann RO, Goldschmeding R: which is hard to disprove, care should be taken before Idiopathic granulomatous orbital inflammation, reaching this diagnosis. After a thorough ocular as Ophthalmology;103:2135, 1996 well as systemic evaluation, once the diagnosis is confirmed a stepwise and meticulous approach to Rootman J, Nugent R. The classification and management is prudent. Care should be taken to management of acute orbital explain to the patient about the risk of recurrence and pseudotumours. Ophthalmology 1982;89:1040–8 the need for prolonged therapy. At the same time, as a one must not forget to not be too Chavis RM, Garner A, Wright JE: Inflammatory aggressivey, as a lot of these patients require long term orbital pseudotumour: A clinicopathological study. medications. Arch Ophthalmol; 96:1817, 1978.

Bibliography Jabs DA. Improving the reporting of clinical case Henderson JW: Orbital Tumors, New York Thieme; series. Am J Ophthalmol Stratton; 1980 2005;139:900–5

Jacobiec FA, Jones IS: Orbital Inflammations, Zborowska B, Ghabrial R, Selva D, et al. Idiopathic Clinical Ophthalmology; vol.2. Hagers Town, MD orbital inflammation with Harper and Row; 1989: p1-75. extraorbital extension: case series and review. Eye 2006;20:107–13 Jacobs D, Galetta S. Diagnosis and management of orbital pseudotumour. Curr B N Swamy, P McCluskey, A Nemet, R Crouch, P Opin Ophthalmol 2002;13:347–51 Martin, R Benger, R Ghabriel, D Wakefield. Idiopathic orbital inflammatory syndrome: Clinical Mombaerts I, Goldschmeding R, Schlingemann RO, features Koornneef L. What is orbital and treatment outcomes. Br J Ophthalmol pseudotumour? Surv Ophthalmol 1996;41:66–78 2007;91:1667–1670

COMMUNITY OPHTHALMOLOGY SOCIETY OF INDIA

8th Annual Symposium November 22nd, 2014 at Venu Eye Institute & Research Centre 1/31, Sheikh Sarai, Press Enclave Marg New Delhi - 110017 , Tel.: 011-29251155

22 Perfect Vision Journal of the Venu Eye Institute & Research Centre VENU EYE INSTITUTE & RESEARCH CENTRE, NEW DELHI TEACHING & TRAINING PROGRAMMES - INFORMATION SHEET FOR INDIA, AFRICA AND SAARC COUNTRIES S. No. Programme Duration No. of Course Fee seats per candidate A. MEDICAL TEACHING PROGRAMMES 1. DNB(Primary) 3 years (July each year) Two 40,000/ year ` 2. DNB (Tutor) 2 years (July each year) Two 40,000/ year ` B. MEDICAL TRAINING – LONG TERM 1. Intensive IOL Fellowship 1½year (Every 4 – 6 months) Six - 2. Oculoplasty 1 year Two - 3. Glaucoma 2 year Two - 4. Cornea 2 year (when slot vacant) Two - 5. Vitreo-retina Fellowship 2 ½ year (when slot vacant) Two - 6. Comprehensive 2 years (Every 4 – 6 months) Two - Ophthalmology Fellowship MEDICAL TRAINING – SHORT TERM 1. ECCE with IOL Implantation 1 month Two 25,000 ` 2. SICS 1 month One 30,000 ` 3. 1 month One 40,000 ` 4. Advanced 15 days / 1 month One 30,000/ ` Phacoemulsification 60,000 ` 5 Medical Glaucoma 2 month One 20,000 ` 6. Medical Retina 2months One 20,000 ` WORKSHOP ( PERIODICAL) 1. Contact Lens Five days workshop 12 5,500 ` (June / December ) 4 Days 12 5,000 LVW -1 Basic clinical low vision ` ( 1st week of January & July) PARAMEDICAL TEACHING PROGRAMME 1. B.Sc. (H) in & 4 Years (3+1) 15 23,800 Per ` Ophthalmic Techniques 4th Year- Internship Year PARAMEDICAL TRAINING –SHORT TERM 1. Eye Bank Technician’s Three months 02 No fee Training Course (Basic) Jan. / April /July /Oct. 2. Eye Donation counsellor’s One month 02 No fee Training Program 3. Instrument Maintenance Six weeks 2 5,000 ` Course OBSERVERSHIP 1. Eye Bank Observership for One week One per No fee Medical Directors (3rd week of every month) course 2. Eye Bank Observership for One week One per No fee Eye Bank Managers (3rd week of every month) course CUSTOMIZED / TAILOR MADE COURSES 1. Refresher course for 2 weeks 5 5,000 ` Optometrists 2. Foreign graduate 1 month 2 US$ 250 observation programmes

For further queries, kindly contact us at [email protected] or write to The Incharge, Teaching & Training Department, Venu Eye Institute & Research Centre, 1/31, Sheikh Sarai –II, New Delhi -110017, Ph. No. 91-11-29251155 /56, Fax No. 91-11-29252370

July - September 2014 Journal of the Venu Eye Institute & Research Centre Perfect Vision 23 Perfect Vision VolumeVolume 17 18 issue issue 1 1 July - September 2014