Delhi Journal of Ophthalmology Delhi Journal of Ophthalmology

Editor Rohit Saxena Managing Editor Rajesh Sinha

Editorial Committee Editorial Board Parijat Chandra Jitendra Jithani Rajvardhan Azad Vimla Menon Rasik B Vajpayee Tushar Agarwal M.Vanathi Atul Kumar Pradeep Sharma Rajinder Khanna Chandrashekhar Kumar Prakash Chand Agarwal Ashok K Grover V P Gupta Harbans Lal Shibal Bhartiya Swati Phuljhele Mahipal S Sachdev S. Bharti Amit Khosla Munish Dhawan Reena Sharma Lalit Verma Ashok Garg B Ghosh Kirti Singh Harinder Sethi Varun Gogia Sharad Lakhotia P K Pandey B P Guliani Raghav Gupta Sashwat Ray P V Chaddha Ramanjit Sihota S P Garg Ashish Kakkar Saptrishi Majumdar Dinesh Talwar Divender Sood Arun Baweja Rachana Meel Shraddha Puranik K P S Malik Rishi Mohan Sanjay Mishra Tanuj Dada Namrata Sharma Tarun Sharma General Information Delhi Journal of Ophthalmology (DJO), once called Visiscan, is a quarterly journal brought out by the Delhi Ophthalmological Society. The journal aims at providing a platform to its readers for free exchange of ideas and information in accordance with the rules laid out for such publication. The DJO aims to become an easily readable referenced journal which will provide the specialists with up to date data and the residents with articles providing expert opinions supported with references.

Contribution Methodology Author/Authors must have made significant contribution in carrying out the work and it should be original. It shouldbe accompanied by a letter of transmittal.The article can be sent by email to the Editor or a hard copy posted. Articles received will be sent to reviewers and their comments will be emailed to the author(s) within 4-6 weeks. The identity of the authors and the reviewers will not be revealed to each other by the editorial team. Detailed instructions to the contributors and for advertisement are included at the end of the journal.

Editorial Process The DJO has Dr Rohit Saxena as its Editor who is assisted by a team of renowned ophthalmologists and an illustrous editorial board. The reviewers,who are leaders in their respective fields, form the back bone of the journal by setting standards for the published work.

Disclaimer The journal does not take any responsibility for the articles published in the journal unless it is explicitly stated so. The views expressed in the articles and editorials are of the authors and do not in any way reflect the policy of the Delhi Journal of Ophthalmology. The journal does not endorse or guarantee the quality or efficacy of any product or service mentioned or advertised in the journal issues. Advertisements carried in this journal are expected to conform to internationally accepted medical, ethical and business standards.

Editorial Office Dr Rohit Saxena, Room No. 479, Dr R.P. Centre for Ophthalmic Sciences, AIIMS, New Delhi-110029 Ph +91-011-26593182, Email : [email protected]

Published by : Dr Rohit Saxena, Editor DJO, on behalf of Delhi Ophthalmological Society, Delhi

Editorial Assistant : Varun Kumar

Vol. 21, No. 1, July-September, 2010 DJO 1 Delhi Journal of Ophthalmology

DJO Vol. 21, No. 1, July-September, 2010 2 Delhi Journal of Ophthalmology Contents Editorial

5. The Ostrich Should be the National bird of India ...... Rohit Saxena Major Review

6. Orbital Space Occupying lesions in Children Mridula Mehta, Sumita Sethi, Neelam Pushker, Mandeep S. Bajaj, Seema Kashyap, Seema Sen, Bhavna Chawla, Mahesh Chandra, Supriyo Ghose 13. Peripheral Retinal Degenerations K Sudhamathi, Umah Venugopal, Ajay Sharma, Deependra V Singh 19. New Insights in Primary Angle Closure Glaucoma Tanuj Dada, Gaurav Kumar, Lalit Tejwani, Vishal Arora, Meenakshi Wadhwani, Anita Panda 26. Idiopathic Juxtafoveolar Retinal Telangiectasis Neha Goel, Bhanu Pratap Singh Pangtey, Anisha Seth, Usha Kaul Raina, Basudeb Ghosh 33. Contact lens fitting in Keratoconus Pooja Singh, Raghav Gupta, Jeewan S Titiyal, Rajesh Sinha Preferred Pratice Patterns

39. Cataract surgery in Fuch’s Corneal Dystrophy Shikha Gupta, Varun Gogia, Ritika Sachdev, Rajesh Sinha, Namrata Sharma Cases Reports

42. Morning Glory Syndrome Rashida Shabbir Tankiwala, Memuna Bahadur 44. An Unusual Case of Metastatic Tubercular Endophthalmitis Meenakshi Kabra, Sarita Beri, Rajniv Garg, Pamela d’souza, Rajesh Jain, Anita Nangia, Sanjay Kumar Mishra 46. Atypical Presentation Of Conjunctival Neoplasia Vandana Jain, S.Gupta, R.Matai, R.K. Srivastava 48. Isolated Strabismus as a Presenting Feature of Large Pituitary Macroadenoma Kamaljeet Singh, Prateek Gujar, Nida Usmani, Santosh Suman History of Ophthalmology

50. Amblyopia: A historical consideration Shibal Bhartiya, Sumita Sethi Instruments Scan

55. Multifocal Electroretinography Lalit Aalok, Swati Phuljhele Instructions to Authors

59. Instructions to Authors

Vol. 21, No. 1, July-September, 2010 DJO 3 Delhi Journal of Ophthalmology

DJO Vol. 21, No. 1, July-September, 2010 4 Delhi Journal of Ophthalmology

Editorial

The Ostrich Should be the National bird of India The Super bug problem

Dear friends,

We are all smarting under the insult the colonial minded British have heaped upon us. Just when our medical industry was getting set to reverse the flow of patients and break free, these jeal- ous British in their inscrutable way have accused us of producing the superbug: and scaring away our patients.

It took hours of prime time television and front page headings in national dailies to get back our confidence and to reassure all of us that the report is false and the ‘New Delhi’ Metalloproteinase 1 is just jealously and nothing else. After all isn’t it that many countries have reported similar multi drug resistant organisms. Also there is no concrete epidemiological data linking this NDMI to us.

But could there be just a little lesson for us to learn in it. Could it be that our medical prac- tices is helping to create such superbugs. Those that are resistant to increasingly newer antibiotics as we are pushed by the pharmaceutical industry to use the latest available antibiotics for ‘viral URI’s and just about anything else.

Could the easy availability of over the counter cutting edge drugs and their indiscriminate use even by the lay help to create such resistance?

Could we Ophthalmologists be a major contributors to this problem: we give the topical formulation of these latest antibiotics for just about anything: itching, surgical prophylaxis, mild congestion etc. and minute quantities of the antibiotic reaches the gut where the sub-MIC concentrations ensure that the bacteria get enough drug to adapt to them but not kill them.

Should we start working for a rational antibiotic use (especially topical ones) policy for hospitals, for a ban on OTC availability of life saving newer antibiotics, but then I don’t think this is necessary. We know that for all our problems there is always someone to blame: Mr. Kalmadi, the rain gods, the government, insensitive officials etc, we know this time it is the jealous British doctors worried about their loss of livelihood.

That is why I think that the Ostrich should be the National Bird of India.

Dr. Rohit Saxena

With due apologies to the peacock, the national bird of India and our esteemed forefathers who felt that the peacock truly represents the colours and diversity of India. “Hiding their head in the sand, like an ostrich” is an English metaphor which means to be foolishly ignoring their problem, while hoping it will magically vanish.

The Delhi Journal of Ophthalmology is now indexed at Index Copernicus. The editorial board is involved in the task of getting the journal indexed in other sites as well as improving the quality of articles and their presentation. This is only possible with the support of each and every DOS member.

In addition to the present heads, the DJO also publishes original research including thesis work of residents. We also welcome comments to articles and advise on how to improve the DJO. Any DOS member who has not received the previous four issues please contact DOS Secretariat [email protected], [email protected] or Editor, DJO editordjo@gmail. com. Some copies have come back due to incorrect addresses, so members are requested to please provide correct addresses and contact details to DOS Secretariat.

Vol. 21, No. 1, July-September, 2010 DJO 5 Delhi Journal of Ophthalmology

Major Review Orbital Space Occupying Lesions in Children

Mridula Mehta, Sumita Sethi, Neelam Pushker, Mandeep S. Bajaj, Seema Kashyap, Seema Sen, Bhavna Chawla, Mahesh Chandra, Supriyo Ghose Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi

Orbit is an anatomical Pandora’s box when it comes to lesions history, the ophthalmologist should also consider orbital that can occupy its domain. It is a complex cavity containing involvement secondary to systemic pathology. Past trauma various compactly arranged tissues; the globe, the extraocular and family history also may aid in the diagnosis. muscles, vessels, nerves, glandular and mesenchymal tissues. A thorough general physical examination with review of all A wide spectrum of pediatric orbital tumours can be seen in systems form the next step in evaluation which should be childhood; these space occupying lesions can be primarily followed by complete and elaborate evaluation of ocular and orbital pathology or secondary to some systemic disease. periocular area. Relative protrusion can be observed by simply These differ substantially from those in adult patients since standing behind a seated patient and gazing downward toward in children there is greater incidence of congenital lesions, the chin from the forehead to assess the displacement of one higher frequency of infection, and unique benign and globe as compared to the contralateral side. Decreased visual malignant tumours that involve the orbit. We in this article acuity (measured in a child by an age appropriate method), will primarily describe an approach to a child with proptosis change of refraction, and pupillary abnormalities should be and the commonly seen benign space occupying lesions in noted. Extraocular motility dysfunction and diplopia should children. Malignant space occupying lesions will be the main be carefully assessed and documented. One should carefully feature of part II of this review article. evaluate status of various cranial nerves in relation to the orbit. Palpation of the anterior orbit can assess associated Incidence and differential diagnosis of orbital tenderness, consistency, and mobility of the mass. In case space occupying lesions in children of proptosis, measurements should be made with Hertel Various studies have reported marked variation in incidence exophthalmometry and also one should note the displacement of various space occupying lesions in children.[1-7] Bullock of the eye in planes other than the anteroposterior dimension et al compared their findings to nine other published (eg, downward, lateral). Slit lamp examination and intraocular series and have reported cystic lesions of the orbit (mainly pressure measurement, should be done where possible. dermoids) to be the most common orbital masses in children Dilated funduscopic examination may reveal optic disc followed by vasculogenic lesions (capillary hemangiomas, edema or pallor, retinal detachment, choroidal folds, vascular lymphangiomas or cavernous hemangiomas)[8]. The engorgement or shunt vessels, or indentation of the posterior common malignant lesions of the orbit described in children pole. Functional evaluation of optic nerve should be done with were rhabdomyosarcoma, secondary malignant tumours/ color vision, visual fields and VER if possible. malignancies (including neuroblastoma, Ewing’s sarcoma, and orbital involvement in retinoblastoma), lymphomas and Cystic lesions leukemia. In a retrospective analysis of histopathological Dermoid Cysts records from our centre by Bajaj et al, contrary to western Dermoid cysts are benign developmental choristomas, thought reports, secondary orbital involvement of retinoblastoma was to be the most common space–occupying orbital lesion of the most common cause of proptosis[1-3, 9]. childhood.[2,4,8] These cysts are congenital arising from nests of primitive dermal elements that have been trapped in Approach to a child with Orbital space occupying bony suture lines at the time of fetal closure. This sequestered lesion tissue forms dermoid cyst which histopathologically is lined While evaluation of a child with orbital disease, a meticulous with keratinized epithelium and has dermal elements, such as history of the patient’s ocular and systemic systems is vital. hair follicles, sweat glands and sebaceous glands. The ophthalmic history should include the age of onset, Shields and colleagues have classified orbital dermoids into the duration and rate of progression of the proptosis. The juxtasutural, sutural and soft tissue types[10]. The juxtasutural informant or if possible the patient should be queried about type are adjacent but not obviously attached to the bony suture pain, change in visual acuity or refraction, diplopia, and line. These are the most common type in children and appear decreased fields of vision. While eliciting a thorough medical in the superotemporal and superonasal quadrants. A sutural

DJO Vol. 21, No. 1, July-September, 2010 6 Orbital Space Occupying lesions in Children Delhi Journal of Ophthalmology dermoid cyst extends into the bony suture line, forming a pit or Presenting at birth as a large orbital mass causing moderate hourglass configuration. Soft tissue or complicated dermoids to massive proptosis, the teratomatous lesion may be usually present at on older age. accompanied by conjunctival keratinization, exposure Clinically, the juxtasutural dermoid cyst in children presents as keratopathy and corneal ulceration. Teratomas may present a painless mass in the superotemporal area and is unattached as primary orbital, combined orbital and intracranial, or sino- to overlying skin. The mass is smooth, mobile, and nontender orbital masses[11]. with no visual symptoms. The soft tissue dermoid grows Massive teratomas traditionally have been treated by orbital slowly over a long period of time and often presents with exenteration especially when the eye is severely damaged. proptosis. Computed tomography (CT) scan preoperatively is Debulking surgery with sparing of globe is possible with even required to confirm the diagnosis and rule out any intracranial visual preservation in cases which present early for better extension (Figure 1). Deeper orbital lesions may show complete bony detects.

Figure 1 (A & B) Coronal computed tomographic scan of patient with right orbital dermoid, Photomicrograph showing cyst wall with keratinized squamous epithelium lining and multiple appendages including hair follicle [h] and sebaceous glands.

Management of dermoid cysts is surgical, aiming for complete excision. Introperatively, rupture of the cysts is to be avoided to limit lipogranulomatous inflammation and scarring. If the cysts is accidentally ruptures, copious irrigation of the site is performed. Excision of sutural and soft tissue cysts is more complicated. Sutural cysts often cannot be removed intact because of their communication into or through the bone. Care Figure 2 (A, B & C) Axial computed tomographic scan showing is taken to remove all remaining cyst lining, thereby limiting presence of well defined uni-ocular hydatid cyst in the retrobulbar the possibility of recurrence. Soft tissue cysts are removed by compartment in a child with proptosis, Gross section of excised way to a transconjunctival or lateral orbitotomy approach. cyst with the characteristic pearly white membrane and Photomicrograph showing the laminated membrane with the scolex Teratomas (arrow). Teratomas are rare congenital germ-cell tumours that may cosmetic and if possible visual rehablitation[12-14]. appear in the orbit. Arising from primordial germ cells, Hydatid cyst these tumours are characterized by the presence of all three Cystic hydatid disease is a zoonotic infection of humans germinal layers i.e ectoderm, mesoderm and endoderm. They caused by the larval stage of Echinococcus granulosus (Figure are benign growths and do not invade orbital bone, although 2). Man is the intermediate host while dog is the definitive orbital enlargement is often seen. host. The prevalence rate varies with endemicity and is 5-10%

Vol. 21, No. 1, July-September, 2010 DJO 7 Delhi Journal of Ophthalmology Orbital Space Occupying lesions in Children in Northern Kenya, which is among the highest worldwide. The most common site for the development of hydatid cyst is the liver and lungs with act as two natural filters during the process of migration of the larvae from the gut. Orbital hydatid disease is quite rare and represents <1% of the Echinococcus cases. The symptoms include progressive proptosis with or without pain, disturbance in ocular motility, visual deterioration and chemosis. A few cases with acute onset visual loss have been reported due to serous retinal detachment, secondary to local inflammation caused by liberation of toxins from semipermeable cyst wall. In long standing cases visual loss can occur due to secondary optic atrophy or exposure keratitis. Serological tests : The Casoni’s intradermal test shows an immediate hypersensitivity reaction with high sensitivity and low specificity; the counter immunoelectrophoresis test has been adapted to diagnose hydatid disease with high specificity and sensitivity. Imaging : USG and MRI are valuable diagnostic aid, for detection of the lesion. The characteristic ‘double wall sign’, as described by Betharia et al. from our centre is often diagnostic of hydatid cyst on ultrasonography.[15] MRI is superior for delineation of its relationship to the adjacent Figure 3 (A & B) Clinical photograph capillary hemangioma, ocular structures. Axial computed tomographic scan of a young child with extensive Complete surgical removal via orbitotomy and cryo-extraction capillary hemangioma involving lids, orbit and cavernous sinus. of the endocyst has been described as the gold standard in the management of isolated hydatid cyst. In the present era of the central nervous system causing neurocysticercosis and anti-heliminthic drugs, preoperative and post operative course the eye and adnexa causing ocular/orbital cysticercosis[16]. of oral albendazole is recommended. Ptosis may be an unusual presentation when the cyst resides in the levator causing mechanical restriction of its action. Orbital cysticercosis Intraocular cysticerci are easily diagnosed by ophthalmoscopy, Cysticercosis is the infestation of tissue by larval tapeworm however the diagnosis of orbital cysticercosis was largerly Taenia solium. Man is the definitive host while pigs are the speculative until the advent of advanced imaging modalities intermediate host of T.solium, but at times humans can be like ultrasonography, CT and MRI. High resolution infected by taking raw food contaminated with eggs of T.solium ultrasonography displays the characteristic picture of a or by consuming food or water contaminated with faecal matter sonolucent area with well-defined anterior and posterior containing ova. Human cysticercosis predominantly affects margins. The presence of a central echodense, curvilinear,

Table 1: Classification of vascular lesions

DJO Vol. 21, No. 1, July-September, 2010 8 Orbital Space Occupying lesions in Children Delhi Journal of Ophthalmology highly reflective structure within the cyst suggestive of scolex, enhance with contrast ,although bony remodeling may be helps to narrow the differential diagnosis to cysticercosis seen, no erosion of bone should be evident. as the aetiological cause. CT and MRI not only confirm the Treatment is indicated if there is compromise of the visual diagnosis but also helps to rule out neurocysticercosis, prior axis, high induced astigmatism, optic nerve compression, to starting of antihelminthic drugs from an ophthalmologist or exposure keratitis because of severe proptosis. Therapy side. Presence of a cystic lesion without a scolex with positive generally falls into medical, radiotherapeutic, or surgical ELISA for anticysticercal antibodies is also diagnostic. A treatment. combination of oral albendazole and corticosteroids is given Medical therapy involves the use of intralesional steroid in confirmed cases for minimum of 4 weeks, after intraocular injection, systemic steroids, or interferon. Combining long and intracranial cysticercosis are ruled out by indirect and short acting steroids (Triamcinolone+ Betamethasone) ophthalmoscopy and imaging respectively. Intraocular is common. Complications include eyelid necrosis[22], cysticercosis is associated with a poor prognosis for vision, hypopigmentation of the skin[23], localized fat atrophy and though sub-retinal cysts can be removed by an intravitreal or rarely central retinal artery occlusion. Interferon and systemic transscleral approach, the results are however not very good. corticosteroids are very effective, although systemic side effects are common. Surgical excision is reserved for well- Vasculogenic lesions circumscribed lesions or those causing severe functional In 1999, the Orbital society created a new classification of sequelae that are unresponsive to medical therapy. vascular lesions based on hemodynamic behavior (Table-I). This new system guides management and prevents high risk Lymphangiomas and unnecessary surgical intervention[17]. Lymphangiomas are benign hamartomatous malformations. They may be present in the conjunctiva, eyelids, or deep in Capillary Hemangioma the orbit. Classically they are viewed as separate from the Capillary hemangiomas are one of the most common benign vascular system, although some overlap is seen between orbital masses in pediatric patients. One-third of all orbital lymphangiomas and varices, or so called “combined capillary hemangiomas are diagnosed at birth, and more lesions”[24]. Histopathologically, lymphangiomas may or than 90% are visible by 6 months of age.[18] The lesion can may not be blood filled and characteristically have lymphoid present in one of three ways. Superficial involvement appears cell aggregates with or without germinal centers. as telangiectatic vessels in the skin, with time the lesion The majority of lymphangiomas appear in the first two becomes more raised and nodular, developing a strawberry decades of life and usually have a gradual course and undergo like appearance. Deeper lesions form raised, soft, purplish slow enlargement with increasing proptosis over many nodules. Deep orbital involvement may have no overlying years. These may suddenly undergo expansion secondary to skin changes and presents solely with proptosis,. The child intralesional hemorrhage (chocolate cyst) or due to expansion presenting with a typical capillary hemangioma will have of lymphoid elements during an acute upper respiratory a superficial, raised, red or deep blue lesion in the eyelid, infection and present with sudden proptosis. Hence, a careful conjunctiva or anterior orbit, which may enlarge with valsalva history may reveal sudden painful proptosis, facial trauma maneuvers or crying (Figure 3)[19]. or that the tumour or proptosis started right after a upper These lesions enlarge in size until the patient is about 2 to 3 respiratory infection. Physical examination may reveal bluish years old, and thereafter stabilize and then start involuting from discoloration of blood vessels within the eyelid skin; however four years of age and most doing so by the seventh year. Visual if the vessels extend under the conjunctiva, and are pale straw complications from capillary hemangiomas are common with colored they are called lymphangiectasias. Clinically, these amblyopia affecting 43% to 60% of patients having eyelid lesions can be differentiated from orbital varices by the lack or orbital involvement.[20] Other major complications of of enlargement on valsalva maneuver. Additional lesions capillary hemangiomas include superinfection, ulceration, may be found elsewhere in the body, including on the mucous hemorrhage and necrosis. Two rare complications are membranes of the mouth. Non-contiguous intracranial Kasabach-Merritt syndrome, characterized by sequestering of vascular anomalies associated with orbital lymphangiomas platelets and fibrinogen in the lesion resulting in coagulopathy have been described[24]. On CT scanning, these lesions can be and high output cardiac failure is seen in patients with large well-circumscribed or diffusely infiltrative because of a lack capillary hemangiomas. of encapsulation. Critical orbital tissues may be encased or For delineating large, diffuse and infilterating lesions, difficult to identify. The lesions show either no enhancement imaging modalities like CT scan, ultrasound, MR imaging or rim enhancement of the large cystic spaces, as in loculated and technetium-99m-labelled red blood cell scintigraphy are lesions. Phleloliths may be seen[25]. MR imaging may also be useful[21]. The masses are usually well circumscribed and useful in delineation of the lesion prior to attempted surgery.

Vol. 21, No. 1, July-September, 2010 DJO 9 Delhi Journal of Ophthalmology Orbital Space Occupying lesions in Children Management requires mainly observation initially for growth colour vision defects, and field defects[29,32]. (clinical and radiographic studies) prior to considering The classification of orbital infections by Chandler et al. intervention. Treatment of lymphangioma is typically (Table-III) does not necessarily imply an order of disease indicated when it is associated with growth, optic nerve progression; however, it helps explain the physical signs compression, corneal exposure, glaucoma or evidence of and symptoms of the various infections and helps organize vision deterioration. Surgically, most patients require several treatment plans. Etiological agents implicated include debulking surgeries to relieve acute optic nerve compression Staphylococcus aureus, Streptococcus pneumoniae, or corneal exposure. In rarest of cases, orbital lymphangioma Hemophilus influenzae, anaerobes, Propionibacterium acnes, patients may require exenteration of the orbit. Pseudomonas, Pneumocystis, mixed infections and rarely parasites and fungi[33]. Leukocytosis and blood cultures Orbital varix are unreliable. CT scans with coronal sections are useful. It The orbital varix is a rare orbital vascular lesion, it is actually is important to note that computerized scans cannot predict a vascular hamartoma typified by plexus of low pressure, whether the mass represents a hematoma, an exudate or a low-flow, thin walled and distensible vessels that intermingle transudate[34]. MRI is frequently necessary for patients with normal orbital vessels. This lesion generally involves the superior ophthalmic vein; however other veins of the orbit can also be affected. Most patients with an orbital varix develop positional proptosis secondary to its connections to the systemic venous circulation. The proptosis is exacerbated when the patient assumes a prone position, bends over, as performs a valsalva maneuver. Rarely, the lesion can have an acute presentation with intracranial infections[34]. Ultrasonography is a useful with painful proptosis and decreased visual acuity, due to adjunct. the thrombosis or hemorrhage of the affected vein. CT and Aggressive parenteral antibiotics with judicious surgical MRI images show an irregular mass usually located in the intervention constitute the mainstay of treatment. All children posterior orbit. CT is superior to MRI in the demonstration of have to be admitted before initiating therapy, even if they phleboliths and to rule out orbital wall defects. lack orbital signs, because children are deficient in IgG2 and The treatment of orbital varix is difficult. Usually the anterior are predisposed to bacteremia. For orbital cellulitis, empiric portion of the lesion is excised surgically. Alternatively, antimicrobial therapy should be chosen to provide activity drainage of the blood clot and electrocauterization of the against S aureus, S pyogenes, and anaerobic bacteria of the vessel wall have been done. It is seldom possible to remove upper respiratory tract in addition to the usual pathogens the entire lesion, especially when it is located posteriorly in associated with acute sinusitis (ie, S pneumoniae, H influenzae, the orbit. Recently there have been studies indicating the role and M catarrhalis). Intravenous therapy is maintained until of percutaneously injected n-butyl cyanoacrylate (NBCA) the infected eye appears nearly normal. At that time, oral to embolize orbital varices followed by surgical resection, antibiotic therapy can be substituted to complete a 3-week as an aid in visualization and hemorrhage prevention during course of treatment. Nasal decongestants can be used to help surgical resection of symptomatic orbital varices[26]. drain the sinuses. Surgical drainage generally is not necessary for cellulitis; however, any patient of orbital abscess with Orbital abscess compromised vision, well-defined abscess, exposure Orbital abscess is a well delineated form of orbital cellulitis keratopathy or complete ophthalmoplegia and not responding or rather a complication there of characterized by collection to conservative treatment should receive surgery for drainage of pus within the orbital tissues[27]. Adjacent sinus disease and debridement. accounts for most of orbital cellulitis in children[28,29]. Ethmoidal sinusitis is the most common etiological factor, Optic nerve Glioma others being trauma, skin infections, dental infections, Gliomas, benign intrinsic tumours of the optic nerve or otitis media, intraorbital foreign bodies, endophthalmitis, visual pathway occur primarily in children, with a mean dacryoadenitis, squint surgery, retinal buckling procedures, age at presentation of 8.8 years. These tumours occur bacteremia & HIV[30,31]. isolated or may be associated with neurofibromatosis type-I. Orbital celllulitis / abscesses in children commonly present as Histopathologically, optic gliomas arise from the astrocytes of an acute febrile illness, with painful proptosis, lid swelling, the optic nerve. chemosis and impaired ocular motility. These can be Gliomas may occur anywhere along the visual pathways. The associated with visual deterioration, pupillary abnormalities, signs and symptoms at presentation are related to tumour

DJO Vol. 21, No. 1, July-September, 2010 10 Orbital Space Occupying lesions in Children Delhi Journal of Ophthalmology location and patient’s age. Optic nerve gliomas in preverbal Neurofibroma patients typically presents with axial proptosis (Figure 4). Neurofibroma is a benign peripheral nerve tumour that can Older children may note a decrease in vision or a change affect the orbit. It can be divided into localized, diffuse and in visual field. Other findings on examination include disc plexiform type[36]. The localized type is clinically and swelling, or optic atrophy and decreased motility. Gliomas radiographically similar to schwannoma and is associated with intracranial extension may come to the attention of the with neurofibromatosis in about 10% of cases. The diffuse clinician because of complaints of pain and headache. type has a variable association with neurofibromatosis On CT scan, gliomas appear as fusiform enlargement of the and the plexiform type is almost always seen in association optic nerve. Intracranial gliomas are better imaged with MR with neurofibromatosis. Localized neurofibroma, rare in imaging. Physiological tests can be used to determine the children, can manifest as proptosis, globe displacement, extent of lesions within the optic pathways. Surgery is the diplopia and optic nerve compression. Diffuse and treatment of choice for gliomas of the optic nerve (as opposed plexiform neurofibromatosis are very similar clinically and to gliomas of the chiasm), particularly when there is profound radiographically, but are classified separately because of visual loss and when there is significant proptosis. Radiation subtle histopathologic differences. They usually occur in therapy for treatment of optic nerve and chiasmal gliomas the first decade of life and show gradual progression, often remains controversial. Others recommend that radiation with involvement of other periocular and ocular tissues, therapy be given early in the course of the disease to minimize including the uveal tract, the diffuse poorly defined mass can the risk of visual deterioration[35]. Often chemotherapy is cause the classic S-shaped curve to the upper eyelid owing administered in an effort to defer the use of radiation therapy. to subcutaneous involvement by the tumour. The plexiform Newer treatments like proton therapy could spare more normal type can be very extensive with massive involvement of the tissue in children with optic pathway gliomas compared with orbit, eyelids and intraocular structures. In addition, patients 3D conformal radiation, particularly in larger tumours. As with neurofibromatosis can have congenital defects in the treatment has evolved over time from primary exenteration to sphenoid bone that can produce a characteristic pulsating proptosis similar to that seen with encephalocele On orbital CT and MRI, it appears as an irregular, ill defined mass, often with extensive periorbital involvement. While the suspected localized orbital neurofibroma have to be excised completely, the diffuse, unresectable type should be managed more conservatively.

References 1. Castillo BV Jr, Kaufman L. Pediatric tumours of the eye and orbit. Pediatr Clinic North Am 2003; 50: 149-172. 2. Shields JA, Bakewell B, Augsburger JJ, Donoso LA, Bernardino V. Space occupying orbital masses in children: areview of 250 consecutive biopsies. Ophthalmology 1986; 93:379-384. 3. Bullock JD. Space occupying orbital masses in children. Ophthalmology 1986; 93:384. 4. Youssefi B. Orbital tumours in children; a clinical study of 62 cases. J Pediatr Ophthalmol Strabismus 1969; 6: 177 -181. 5. Kodsi SR, Shetlar DJ, Campbell RJ. Garrity JA., Bartley JB. A review of 340 orbital tumours in children during a 60 year period. Am J Ophthalmol 1994; 117:177-182. 6. Gunalp I, Gunduz K. Pediatric orbital rumors in Turkey. Ophthal Plast Reconstr Surg 1995; 11: 193-199. Figure 4 (A & B) Clinical of child with Optic nerve glioma, CT scan 7. Shields JA, Bakewell B, Augsburger JJ, Flanagan JC. saggital view of child with Optic nerve glioma showing globular Classification and incidence of space-occupying lesions of enlargement of optic nerve. the orbit: a survey of 645 biopsies. Arch Ophthalmol 1984; 102:1606-1611. biopsy, postoperative radiation and chemotherapy, survival of 8. Bullock JD, Goldberg SH, Rakes SM: Orbital tumours in these patients has increased dramatically. children. Ophthal Plast Reconstr Surg 1989; 5:13-16.

Vol. 21, No. 1, July-September, 2010 DJO 11 Delhi Journal of Ophthalmology Orbital Space Occupying lesions in Children 9. Bajaj MS, Pushker N, Chaturvedi A, Betharia SM, Kashyap 23. Cogen MS, Elsas FJ: Eyelid depigmentation following S, Balasubramanya R, Sen S Orbital space-occupying lesions corticosteroid injection for infantile ocular adnexal in Indian children. J Pediatr Ophthalmol Strabismus. 2007 hemangioma. J Pediatr Ophthalmol Strabismus 26:35¬-38, Mar-Apr;44(2):106-11 1989. 10. Shields JA, Kaden IH, Eagle RC Jr, et al: Orbital dermoid 24. Katz SE, Rootman J, Vangveeravong S et al: Combined cysts: Clinicopathologic correlations, classification, and venous lymphatic malformations of the orbit in management. The 1997 Josephine E. Schueler Lecture. association with noncontigu¬ous intracranial vascular Ophthal Plast Reconstr Surg 13:265-276, 1997 anomalies. Ophthalmology 105:176-184,1998 11. Kivela T, Tarkkanen A: Orbital germ cell tumours revis¬ited: 25. Wright JE, Sullivan TJ, Garner A, et al: Orbital venous A clinicopathological approach to classification. Surv anomalies. Ophthalmology 104:905-913,1997 Ophthalmol 38:541-554, 1994 26. Couch SM, Garrity JA, Cameron JD, Cloft HJ. Embolization 12. Chang FO, Dallow RL, Walton OS: Congenital orbital of orbital varices with N-butyl cyanoacrylate as an aid teratoma: Report of a case with visual preservation. J Pediatr in surgical excision: results of 4 cases with histopathologic Ophthalmol Strabismus 17:88-95, 1980. examination. Am J Ophthalmol.2009 Oct;148(4):614-618. 13. Habal MB: Orbital teratomas: A new approach for surgical 27. Antonio A.V.Cruz, MD, Marisa M. Mussi Pinhata, MD, treatment. Journal of Craniofacial Surgery 1:8-14, 1990. Patrician M S Akaishi, MD, Luciana Cattebeke, MD, Jose 14. Orbital teratoma: a rare cause of congenital proptosis. Mehta Torranda Silva, MD, Jorge Elia Jr, MD, Neonatal Orbital M, Chandra M, Sen S, Bajaj MS, Pushker N, Meel R, Ghose S. Abscess Ophthalmology 108 : 2316-2320, 2001. Clin Experiment Ophthalmol. 2009 Aug; 37(6):626-8. 28. Acute inflammations of the orbit in children. Arch Ophthal 10 15. Betharia SM, Sharma V, Pushker N. Ultrasound findings in : 483-497, 1933. orbital hydatid cysts. Am J Ophthalmol.2003 Apr;135(4):568- 29. Jarrett WH II, Gutman FA. Ocular complications of infection 70. in the paranasal Sinuses. Arch Ophthal 81: 683 , 1969. 16. Pushker N, Bajaj MS, Betharia SM. Orbital and adnexal 30. Weiss A, Friendly D, Eglin K, Chang M, Gold B. Bacterial, cysticercosis. Clinical and experimental ophthalmology 2002; periorbital, orbital cellulitis in childhood .Ophthalmology Vol. 30 (5):322-33. 90 : 195-203, 1983. 17. Harris GJ. Orbital vascular malformations: a consensus 31. Haynes RE, Gramblett HG. Acute Ethmoiditis, its relationship statement on terminology and its clinical implications. Orbital to orbital cellulitis . Am J Disease Child 114: 261, 1967. society. Am J Ophthalmol 1999; 127: 453-455. 32. Sullivan T J, Aylward GW, Wright GE. Actinomycosis of 18. Haik BG, Jakobiec FA, Elsworth RM, et al: Capillary orbit. Br. J. Ophthal. Vol 1992; 76: 505-506. hemangioma of the lids and orbit: An analysis of the clinical 33. Kronish JW, Johnson TE, Gilberg SM, Corrett GF, McLeish features and therapeutic results in 101 cases. Ophthalmology WM, Scott KR. Orbital infections in patients with HIV 86:760-792,1979 infections. Ophthalmology Vol 103: 1483-1492,1996. 19. Margileth AM, Museles M: Cutaneous hemangiomas in 34. Younnis RT, Anand VK, Davidson B.The role of computed children. JAMA 194:135-138, 1965 tomography and magnetic resonace imaging in patients with 20. Stigmar G, Crawford JS, Ward CM, et al: Ophthalmic sequelae sinusitis with complications. Lanyngoscope 112 (2) 224 - 249 of infantile hemangiomas of the eyelids and orbit. Am J Feb 2002. Ophthalmol 85:806-813, 1978 35. Wong JY, Uhl V, Wara WM, Sheline GE. Optic gliomas. 21. Gdal M, Gelfand Y: Cryoextraction of orbital cap¬illary A reanalysis of the University of California, San Francisco hemangioma diagnosed by technetium-99m¬labeled red blood experience. Cancer 1987 Oct 15; 60(8):1847-55. cell scintigraphy. Ophthalmic Surg Lasers 28:954-956, 1997 36. Krochel GB, Rosenburg PN, Wright JE, et al. Localized orbital 22. Sutula FC, Glover AT: Eyelid necrosis following in tralesional neurofibromas.Am J Ophthalmol 1985; 100:458-464. corticosteroid injection for capillary hem angioma. Ophthalmic Surg :103-105, 1987

DJO Vol. 21, No. 1, July-September, 2010 12 Delhi Journal of Ophthalmology

Major Review Peripheral Retinal Degenerations K Sudhamathi, Umah Venugopal, Ajay Sharma, Deependra V Singh Eye-Q Superspeciality Eye Hospitals, Gurgaon

The vast array of peripheral retinal degenerations leave an ophthalmologist with numerous possible differentials and prognosticators when an individual presents to him. The following review is an aid in understanding which degenerations make retina susceptible to a possible retinal detachment in near future; and are possible indicators for prophylactic therapy at presentation and which of them do not predispose to any complications and thus can be left untreated.

Peripheral retina defined as an area anterior to equator of body begins. The border between the two is scalloped,with the eye is a site for potential pathologic lesions which may the scalloped teeth(dentate processes)facing anteriorly predispose to Rhegmatogenous Retinal detachment. ,alternating with oral bays between the teeth. Pars plana of This article focuses on differentiation of such lesions from ciliary body extends 3-4mm anteriorly forwards to the Pars benign peripheral retinal lesions, assessment of the risk of Plicata.Vitreous base spans the ora serrata and extends 1.5mm RD associated with them and decision making regarding anteriorly onto the ciliary body and 1.5-3mm posteriorly onto prophylactic treatment of these lesions. peripheral retina. Vitreous is tightly adherent to retina at optic disc, vitreous base, edges of peripheral degenerations and Relevant Anatomy along the retinal blood vessels. The attachment at vitreous A sound understanding of the anatomical location of the base is considered to be the strongest. peripheral retina structures and their relationship to one Peripheral retina is best visualized with either 3-mirror another is required. The peripheral retina is three to four disc Goldman lens or indirect ophthalmoscopy. diameters (3DD-4DD) wide. The primary reason for performing a dilated examination The vortex veins and ampullae serve as very good landmarks of the peripheral retina is to detect potential predisposing for an examiner. These vortex ampullae and veins represent lesions[1] that can lead to retinal detachment (RD). Retinal the equator of the eye. detachment constitutes separation of the sensory retina from Retina ends sharply at ora serrata,where parsplana of ciliary the underlying retinal pigment epithelium, (RPE) due to

Table-1 Features of Benign peripheral retinal degenerations (not predisposing to RD) Benign Lesions Demography Natural Course Appearance

Paving stone Degeneration 27% Rarely lead to o Atrophy or Absence of Outer Layers of Retina breaks o Loss of RPE o Absence of Chorio-capillaries o Lesions occur as Single or Confluent areas Congenital RPE hyper trophy Common, no Rarely o Large, Well Demarcated, Usually Black area estimate available. Causes RD o Apex pointing to the Optic Nerve- Bear Tracks Unilateral 1-2% o Histologically: Enlarged RPE cells, with Large bilateral, in o Spherical Melanin Granules, or Macromelanosomes these cases rule out Gardener’s syndrome RPE hyper plasia Reactive process Does not cause RD Posterior to the Ora Serrata along the Vitreous Base. sec. to Flat black pigmentation with irregular margins infection,trauma or uveitis Peripheral Cystoid 18% May lead to Cavitation of Outer plexiform & nuclear layers Degeneration Retinoschisis

White Without Pressure Areas 5%,in Ged 66% Rarely breaks Optical phenomena in which vitreous traction changes 23% in Myopia seen. Considered color of retina by some as precursor to GRT

Vol. 21, No. 1, July-September, 2010 DJO 13 Delhi Journal of Ophthalmology Peripheral Retinal Degenerations Table-2 Features of Lesions predisposing to Rhegmatogenous RD

Retinal Lesions Demogrophy Natural course Appearance

Lattice Degeneration 6-10% of General Present in 20-30% of Histology: discontinuity of the internal limiting population all RDs membrane of the retina, overlying pocket of 13-30% of Myopes liquefied vitreous, condensation and adherence of vitreous at margin of lesion, atrophy of inner retina. Typical ophthalmoscopic findings: white lines of Vogt, vascular sheathing, RPE hyperplasia, atrophic holes, tears along margin. Snail Track Degeneration Variant of lattice Small retinal holes at Similar to lattice Frost like appearance with degeneration. Risk same edge seen yellow spots as lattice

Snow Flake Degeneration Hereditary Can lead to breaks White spots in periphery involves inner retinal layers

Pigment Clumps & Pigment Seen in 20% of normal & Retinal hole maybe Proliferation and migration of RPE cells into Degeneration 17% of Myopes. seen sensory retina Developmental/Mechanica l or Inflammatory

Retinal Tuft 72% population 50% Cystic & zonular may Small retinal elevations caused by focal Bilateral predispose to retinal vitreoretinal traction. 3 types-noncystic, cystic tear/hole zonular

Meridonial Fold and Complex 26% males Occasional tear seen at Folds of redundant Retina at ora posterior margin

the accumulation of liquefied vitreous fluid in that potential to vitreous traction. It causes the retinal surface to have a space. Most RDs are the result of one or more retinal breaks thin whitish appearance which can even slightly change its that allow passage of fluid between the sensory retina and the location between examinations. It is not uncommon for a RPE. Since occurrence of RD is frequently associated with narrow zone of optically darker retina to be on the posterior moderate to severe loss of vision, it is always imperative to margin of W-W/O-P. Scleral depression can enhance the subject all the patients at high risk to periodical peripheral whitish appearance of the entity and the posterior margins. retinal examination. Also it is important to distinguish W-W/O-P is also associated with such retinal degenerations dangerous from benign peripheral retinal lesions[2]. as lattice degeneration and retinoschisis. White-with or -without-pressure is found to some extent in Peripheral retinal conditions can be classified as – over 30% of normal eyes, with a strong tendency toward Benign Peripheral retinal lesions not predisposing to RD. bilateralism. Individuals under 20 years of age have only 1) Paving stone/Cobble stone degeneration a 5% occurrence, while those over 70 years of age have 2) Peripheral cystoid degeneration approximately a 66% frequency. A study of myopes found 3) RPE Hyperplasia a prevalence of 0% in myopic eyes with the shortest axial 4) Congenital RPE hypertrophy ‘Bear Tracks’ length and 54% in eyes with axial lengths over 33 mm. In 5) White without pressure. patients of all ages, it is most frequently found in myopic patients at 22.8%. This lesion is rarely associated with retinal Rhegmatogenous Peripheral retinal lesions break formation. 1) Lattice degeneration 2) Retinal Tufts Lattice Degeneration 3) Meridional folds and Complexes Lattice degeneration is a Vitreoretinal pathology that produces 4) Snail Track Degeneration pockets of liquefied vitreous and retinal thinning, both of 5) Snow Flake degeneration which can result in retinal tear formation and subsequent 6) Pigmentary degeneration and pigment clumps detachment of neurosensory retina[5]. White-Without-Pressure Lattice lesions are usually hyperpigmented, mottled looking W-W/O-P is a fairly common retinal finding and is related and most often located in the far periphery of the retina. They

DJO Vol. 21, No. 1, July-September, 2010 14 Peripheral Retinal Degenerations Delhi Journal of Ophthalmology

Figure 1 Pigmented lattice with holes pre (1A & 1B) and post laser Figure 2 Old Rheg. RD from lattice with holes (2A) and lattice with (1C & 1D) large atrophic hole in fellow eye. Non pigmented lattice pre (2C) and post laser (2D).

Figure 3 Large Flap tear from PVD with laser marks 3A, Rare Figure 4 Juvenile retinoschisis with cart wheel macula (4A), Large retinal tear at the anterior margin of lattice 3B, Flap tear at the inner wall holes pre (4B,4C) and post laser delimitation (4D). posterior margin of lattice pre (3C) and post laser (3D).

Figure 5 Low/no risk peripheral degenerations; Fundus Figure 6 White without pressure. photographs showing Paving stone degenerations (5A & 5B), snowflake degeneration (5C) and Cystic tuft (5D).

Vol. 21, No. 1, July-September, 2010 DJO 15 Delhi Journal of Ophthalmology Peripheral Retinal Degenerations are elongated lesions with the long axis parallel with the ora detachment have a significant association; 20% to 41% of serrata and they can be singular or multiple in number. Lesions patients operated for a rhegmatogenous retinal detachment which are around blood vessels are known as perivascular have lattice degeneration present in the eye. This does not lattice degeneration. mean that all patients with lattice degeneration are likely to Lattice degeneration tends to be both fairly symmetric and develop a retinal detachment; in fact it has been estimated that bilateral, affecting both eyes 33.0%- 48.1% of the time. this occurs in only 0.3% to 0.5% of patients or 1 in 200-300 The number of lesions per eye can vary from 1 to 19 or patients with the disease. more and the average number per eye is approximately [2]. There is always fairly normal appearing retina between Decision Making lattice degeneration and the ora serrata and this is important Natural History of Precursors to Rhegmatogenous Retinal in differentiating prominent vitreous base from lattice. On Detachment occasion, only the posterior margin of a pigmented area in Precursors to retinal detachments are PVD and symptomatic the far periphery may be seen during ophthalmoscopy and retinal breaks. Nearly all patients with a symptomatic RRD it may gives rise to the presumptive diagnosis of lattice will progressively lose vision unless the detachment is degeneration. The pigmented area should be viewed under repaired, Prevention or early diagnosis is important because scleral depression so as to be able to view anterior to the the rate of successful reattachment is higher and the visual lesion and if the pigmented area is adjacent to the ora serrata results are better if detachment spares the macula. Successful than the diagnosis would most like be a prominent vitreous treatment allows patients to maintain their abilities to read, base, but if retina is found anterior to the lesion than the most work, drive, care for themselves, and enjoy a better quality likely diagnosis would be lattice degeneration. of life. White lines may display a crisscrossing pattern, the appearance that is responsible for the term lattice degeneration[5]. White Posterior Vitreous Detachment lines are not commonly seen in young patients, only being Posterior vitreous detachment is the cause of many retinal found in 3.3% in the 10 - 19 year old age group, but they breaks, which can then lead to retinal detachments. The do increase in frequency with advancing age, reaching symptoms of PVD include light flashes and floaters, and a maximum prevalence of 42.9% after age 50 years. patients with such symptoms are at significant risk for retinal Abnormal pigmentation is the most common finding in detachment[3,4]. lattice degeneration and occurs in 81.7% and 92% of lesions. Approximately 15% of patients with acute symptoms of This pigmentation found in the retina and choroid seems to PVD have a retinal tear at the time of the initial examination. increase with age. The second most common feature is tiny Patients with acute PVD who have no retinal breaks on white or yellow flecks, which are seen in 80% of lesions and presentation have a 2% to 5% chance of developing them in are located between the retinal surface and the vitreous cortex. the weeks that follow.In patients who present with substantial Lattice degeneration is associated with W-W/O-P, atrophic vitreous hemorrhage, 67% were found to have at least one holes, and linear and flap tears. Atrophic holes may form in break, with 31% having more than one break and 88% of the a lesion due to the loss of all of the sensory retinal tissue, and breaks occurring in the superior quadrants. occur in 18.2% to 28.7% of cases. The frequency of retinal detachment caused by atrophic holes in lattice degeneration Symptomatic Retinal Breaks is fairly low and has been reported to be 2.8% and 13.9%. A symptomatic retinal break is defined as one caused by Retinal tears have been reported in 2.4% in 125 autopsied vitreoretinal traction in a patient with a new PVD or a break eyes with lattice degeneration and another report found retina associated with a significant increase in flashes and floaters. tears in 1.5% of 289 patients with lattice lesions followed Approximately one half of untreated symptomatic retinal for 3-10 years. Other reports found retinal tears in 1.0% of breaks with persistent vitreoretinal traction (horseshoe or flap eyes with lattice degeneration. Lattice with tractional tears or tears) will cause a clinical retinal detachment unless treatment a mixture of breaks was found to be responsible for retinal is applied. detachments in 16% to 27% of all primary detachments. Two reports found that 55% to 70% of retinal detachments in eyes Risk Factors for Rhegmatogenous RD. with lattice degeneration were caused by tears at the posterior Aside from retinal breaks, risk factors for RRD include edge of the lattice lesions. Scleral depression enhances the myopia, lattice degeneration, cataract surgery, trauma, and a visualization of these lesions and for confirming the existence history of RRD in the other eye. Combinations of these factors of retinal breaks. in a single eye further increase the risk. Lattice degeneration is significant in that it is the most commonly associated retinal finding in rhegmatogenous Myopia RDs undergoing surgery. Lattice degeneration and retinal More than half of nontraumatic RRD occurs in myopic

DJO Vol. 21, No. 1, July-September, 2010 16 Peripheral Retinal Degenerations Delhi Journal of Ophthalmology eyes.As axial length increases, so does the risk of RRD[4]. frequently bilateral. A pseudophakic RRD is not necessarily As compared with that of emmetropes, low myopes (1 to 3 caused by cataract surgery alone. The fellow eye in a patient diopters) have a 4-fold risk and higher myopes (>3 diopters) with pseudophakic retinal detachment is also at higher risk have a 10-fold risk. The tendency to develop atrophic breaks, for developing a retinal detachment, whether the fellow eye is absence of Gel vitreous to plug holes and occurrence of early phakic or pseudophakic. Phakic fellow eyes in patients with and acute PVD are factors responsible for high risk of RD in pseudophakic retinal detachment have about a 7% risk of myopes. RRD, indicating that all the risk for developing RRD cannot be attributed to cataract surgery alone. Lattice Degeneration Lattice degeneration, increases the risk of retinal Early Detection and Prevention detachment[5]. Lattice degeneration is present in 6% to 8% There are no effective methods to preventing vitreous changes of the general population; an individual with this disease has that lead to RRD. If factors associated with an increased a high risk of RRD. risk of retinal detachment are discovered during a routine eye examination in an asympotomatic patient, a peripheral Cataract Surgery fundus examination is advisable. Patient at high risk should The overall risk of RRD after cataract surgery is approximately also be educated about the symptoms of PVD and retinal 1%. Two large studies found that the risk of RRD after cataract detachment as well as about the values of periodic follow-up surgery is 6 to 7 times greater than that of phakic control examination[12]. groups[7,8,9].The following have been reported to increase the risk of retinal detachment after cataract surgery: vitreous Diagnosis loss; increased axial length; lattice degeneration; Nd:YAG The initial evaluation of a patient with risk factors or symptoms laser capsulotomy; Caucasian race; and younger age. A recent includes all features of the comprehensive adult medical eye study has found the risk to be higher for as long as 20 years. evaluation, with particular attention to those aspects relevant to PVD, retinal breaks, and lattice[13] degeneration. Trauma Patients with blunt or penetrating ocular injuries that have History altered the structure of the vitreous or retina are at increased o Symptoms of PVD risk of RRD[10]. Although nearly all breaks caused by blunt o Family history OF Rheghmatogenous RD trauma occur at the time of the injury, the detachment may not o Prior eye trauma, including surgery be symptomatic for years because the younger age group at o Myopia risk for trauma has a formed vitreous. Trauma also accelerates o Examination of the vitreous for PVD , pigmented cells, the development of PVD. hemorrhage, and condensation o Peripheral fundus examination with scleral depression. Rhegmatogenous Retinal Detachment in the Fellow Eye There are no symptoms that can reliably distinguish PVD Patients with a history of non-traumatic detachment in one with an associated retinal break from PVD without an eye have about a 10% increased risk of developing RRD in the associated retinal break; therefore, a peripheral retinal fellow eye[11]. Because pathologic Vitreoretinal changes are examination is always required. The preferred method of Table 3 Recommendations for Management Type of Lesion Treatment Acute symptomatic horseshoe tears Treat promptly Acute symptomatic operculated tears Treatment can be considered Traumatic retinal breaks Usually treated Asymptomatic horseshoe tears Treatment should be considered Asymptomatic operculated tears Treatment is rarely recommended Asymptomatic atrophic round holes Treatment is rarely recommended Asymptomatic lattice degeneration without holes Not treated unless PVD causes a horseshoe tear Asymptomatic lattice degeneration with holes Can be treated if other risk factors are present Asymptomatic dialyses No consensus on treatment due to insufficient evidence to guide management, most surgeons treat Fellow eyes with atrophic holes, lattice degeneration, Treatment advocated by most surgeons. or asymptomatic horseshoe tears

Vol. 21, No. 1, July-September, 2010 DJO 17 Delhi Journal of Ophthalmology Peripheral Retinal Degenerations Table- 4 Suggested Follow-Up Lesions requiring close follow-up (2-4 weeks) Lesions that require 6 months follow up Symptomatic PVD with no retinal break Fellow eyes with atrophic holes, lattice, Asymptomatic horseshoe tears.

Acute symptomatic horseshoe tears Asymptomatic horseshoe tears. Acute symptomatic operculated tears Asymptomatic operculated tears. Traumatic retinal breaks Asymptomatic atrophic round holes. Asymptomatic lattice degeneration with and without holes. Asymptomatic dialyses. evaluating peripheral vitreoretinal pathology is with indirect • Always explain to patient the symptoms of PVD ophthalmoscopy combined with scleral depression. and importance of prompt retinal examination after experiancing those symptoms. Diagnostic Tests A B-Scan maybe required in case of opaque media like References Cataract or Vitreous Hemorrhage secondary to retinal tear or 1. Rutnin U, Schepens CL. Fundus appearance in normal any other cause. eyes. III. Peripheral degenerations. Am J Ophthalmol 1967; 64:1040-62. Treatment 2. Glasgow BJ, Foos RY, Yoshizumi MO, Straatsma BR. Table 3 summarizes recommendations for management. Degenerative diseases of the peripheral retina. In: Tasman Treatment of peripheral horseshoe tears should be extended W,Jaeger EA, eds. Duane’s clinical ophthalmology, vol well into the vitreous base, even to the ora serrata. The surgeon Philadelphia: Harper & Row, 1993:1-30. should inform the patient of the relative risks, benefits, and 3. Boldrey EE. Risk of retinal tears in patients with vitreous alternatives to surgery. The surgeon has the responsibility for floaters. Am J Ophthalmol 1983;96:783-7. formulating a postoperative care plan and should inform the 4. Karlin BD, Curtin BJ. Peripheral chorioretinal lesions and axial patient of these arrangements[13]. length of the myopic eye. Am J Ophthalmol 1976; 81:625-35. 5. Yanoff M, Fine BS. Ocular pathology: a text and atlas, 3rd Follow-up ed.Philadelphia: JB Lippincott, 1989:404-7. 6. Benson WE, Morse PH. The prognosis of retinal detachment The guidelines in Table 4 are for routine follow-up in the due to lattice degeneration. Ann Ophthalmol 1978;10:1197- absence of additional symptoms. Patients with no positive 200. findings at the initial examination should be seen atthe 7. The Eye Disease Case-Control Study Group. Risk factors for intervals as recommended in the Table 4. All patients with idiopathic rhegmatogenous retinal detachment. Am J Epidemiol risk factors should be advised to contact the ophthalmologist 1993;137:749-57. promptly if new symptoms such as flashes, floaters, peripheral 8. Erie JC, Raecker MA, Baratz KH, et al. Risk of retinal visual field loss, or decreased visual acuity develop. detachment after cataract extraction, 1980-2004: a population- based study. Ophthalmology 2006;113:2026-32. Useful Tips 9. Russell M, Gaskin B, Russell D, Polkinghorne PJ. Pseudophakic Examination retinal detachment after phacoemulsification cataract surgery: • Whenever doubtful indent and depress Ten-year retrospective review. J Cataract Refract Surg . • Use both 28d and 20d lens 10. Tasman W. Peripheral retinal changes following blunt trauma. • Scan anterior to the lesion Trans Am Ophthalmol Soc 1972;70:190-8. • Change posture of the patient,supine is best but upright 11. Sharma MC, Chan P, Kim RU, Benson WE. Rhegmatogenous position gives best view retinal detachment in the fellow phakic eyes of patients with • Try to take fundus picture.if one can capture it, then one pseudophakic rhegmatogenous retinal detachment. Retina can easily barrage it by slitlamp laser photocoagulation. 2003;23:37-40. 12. Schepens CL. Diagnostic and prognostic factors as found Treatment in preoperative examination. Trans Am Acad Ophthalmol • Superior lesions more dangerous than inferior ones. Otolaryngol 1952;56:398-418. • Patients > 40 years of age without PVD to be treated in 13. American Academy of Ophthalmology Preferred Practice view of rick of PVD induced flap tear at the margin of Patterns Committee. Preferred Practice Pattern® Guidelines. lesion. Comprehensive Adult Medical Eye Evaluation. San Francisco, • Use more number of smaller spots than large intense CA: American Academy of Ophthalmology; 2005. ans burns. 06;32:442-5.

DJO Vol. 21, No. 1, July-September, 2010 18 Delhi Journal of Ophthalmology

Major Review New Insights in Primary Angle Closure Glaucoma Tanuj Dada, Gaurav Kumar, Lalit Tejwani, Vishal Arora, Meenakshi Wadhwani, Anita Panda Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi Primary angle closure glaucoma is one of the major causes for irreversible visual loss in Asia. In addition to pupillary block, lens induced mechanisms, plateau iris and supra-ciliary effusions are important contributing factors eluciadated by Ultrasound biomicroscopy. Treatment modalities for management of primary angle closure disease start with a laser iridotomy and include laser iridoplasty, lens extraction, combined procedures with goniosynechiolysis and glaucoma filtering surgery. This article is to give insight into newer concepts in treatment of angle closure based on the improvement in knowledge of pathophysiology and natural history of the disease.

According to the International Society of Geographical and PAC increases with age, peaking in incidence between 55 and Epidemiological Ophthalmology (ISGEO) Classification, 70 years of age[8] and is greater in females[9]. Population- primary angle closure glaucoma is subdivided according based studies have shown that most cases of PACG are to conceptual stages in natural history of angle closure of asymptomatic, whereas chronic PACG may develop after the glaucoma into primary angle-closure suspect(PACS), primary resolution or precede the occurrence of an acute attack of angle angle closure without optic neuropathy (PAC), and primary closure[10,11]. Data from India Vellore Eye Study (VES)[12] angle-closure glaucoma with neuropathy (PACG)[1]. and Andhra Pradesh Eye Disease Study (APEDS)[13] shows a prevalence of 4.32% and 0.71% for PACG. Hospital based • PACS- Irido trabecular contact (ITC) with normal optic disc data suggests an equal number of people with POAG and and visual field. IOP is normal and PAS is absent. PACG. Prevalence of primary angle closure disease (PACG • PAC- ITC + either raised IOP, PAS or typical symptom + PAC) in the rural population according to Chennai Eye • PACG-ITC + structural glaucomatous changes in Disease Incidence Study[14] is 1.58%. An additional 7% were optic nerve + Visual field loss. at risk of developing angle closure glaucoma.

These categories may clinically overlap or be potentially Mechanisms of Angle Closure Glaucoma related, and the natural history of the disease is such that the Most common mechanism of primary angle closure is pupillary patient may present to the clinician at any stage of the clinical block. The simultaneous activation of dilator and sphincter spectrum. The most important fact to remember about this pupillae muscles produces a resultant force whose vector lies disease, however, is that a significant reduction in morbidity more or less perpendicular to lens surface when the pupil is from this condition is possible as most cases can be detected in mid-dilated position. These pathologic mechanisms exist with available technology; and many cases treated with the because of primary anatomic variations in the size, position, timely single application of a simple, safe treatment. and relationship of the anterior segment structures (cornea, iris, ciliary body, lens). In plateau iris[15] there is anterior Epidemiology rotation of ciliary body pushing iris anteriorly crushing Of the estimated 67 million people worldwide thought to be peripheral shallow angle and deep central anterior, chamber. A affected with primary glaucoma, one-third to one half have large anteriorly displaced lens may also be an important factor primary angle closure glaucoma (PACG)[2]. One of the major contributing to primary angle closure glaucoma. factors determining susceptibility to primary angle closure is the ethnic background. In European and African populations Role of Lens in Pathogenesis primary open-angle glaucoma (POAG) is reported to be Eyes with primary angle closure have significant anatomic approximately five times more common than PACG; while in differences from normal eyes[16]. The most significant clinical the Chinese[3,4]. Mongolians[5] and Indians[6] the frequency hallmarks of an eye with angle-closure are the shallow AC of PACG may equal or be greater than POAG. In Eskimos/ and narrow angle. The mean anterior chamber depth (ACD) Inuit the prevalence of PACG has been found to be higher in PAC eyes is approximately 1.8 mm, which is 1 mm shorter than any other ethnic group[7]. than in normal eyes[16,17]. Angle closure becomes a rarity PACG is 2 to 3 times more likely to cause visual impairment when anterior chamber depth exceeds 2.5 mm[18]. Decreased than is primary open-angle glaucoma[3]. The incidence of AC volume[19], small corneal diameter[20], and short axial

Vol. 21, No. 1, July-September, 2010 DJO 19 Delhi Journal of Ophthalmology New Insights in Primary Angle Closure Glaucoma lengths]20] are all characteristic of eyes with PACG. The most with the help of newer investigations ultrasound biomicroscopy satisfactory explanations for the more shallow AC is the age (UBM) and anterior segment OCT (ASOCT, Visante) more related increase in lens thickness and more anterior position of knowledge of role of lens in angle closure glaucoma has been the lens[16,18,21] The axial lens thickness is greater than in found. Treatment of angle closure and prevention of further normal subjects[16,18], and the thicker lenses are significantly progression of the angle anomaly is therefore now based on a more anteriorly positioned than in normal eyes[18]. Lowe better understanding of the pathomechanism of angle closure. estimated that increased lens thickness causes 0.35 mm of AC shallowing, and forward lens position causes 0.65 mm Laser Peripheral Iridotomy (LPI) of shallowing, accounting for the total of 1 mm difference It eliminates the pressure differential between the anterior and in AC depth of the smaller eye compared to the normal eye. posterior chambers by providing alternative route aqueous Growth of the lens, with an increase in the number of lens trapped in posterior chamber to enter the anterior chamber. It fibers continuing throughout adult life, results in an increase is also a safe and effective prophylaxis in suspect eyes with in lens thickness and anterior curvature[19]. occludable angles secondary to pupillary block, including Lowe also developed an index of relative lens position fellow eyes of patients at risk for bilateral angle closure[26]. calculated as LPI has proven to be an effective treatment for APAC, resulting Relative lens position in widening of the filtration angle and reduction of elevated Anterior chamber depth + ½ (lens thickness) axial length. IOP, it may not protect against chronic angle closure[27,28]. A When the biometry of contralateral eyes of patients having study in Asian eyes with acute PAC that had undergone laser an (acute primary angle closure ) APAC were studied PI showed 58.1% continued to have elevated IOP and 32.7% and compared to population-based controls, unfavorable eventually required trabeculectomy[29]. PI does not always dimensions were found consisting of more shallow anterior provide long term protection, recurrent attack can cause PAS chambers and narrow angles, and thicker lenses. These formation. A study showed 32.2% of 59 eyes with acute differences were considered to explain in part the estimated PAC had progression of PAS following a successful laser 50% risk for APAC in these eyes[22]. Decreased ACD is iridotomy[30]. accelerated in women between the fourth and fifth decades, Therefore primary angle closure may progress to primary which may explain their greater propensity for PAC in angle closure glaucoma and primary angle closure glaucoma females[23]. may continue to deteriorate even after a patent iridotomy is Relative resistance to flow of aqueous from the posterior present. This is related to the persistent angle closure caused chamber (PC) into the anterior chamber (AC) increases greatly by presence of plateau iris or a large anteriorly displaced lens when the dimensions of the iris–lens channel are changed in with increasing lens thickness as the patient ages. Performing such a manner that flow of aqueous is more impeded[24]. This a laser iridotomy only relieves one mechanism of primary incremental pressure differential determines the iris contour. angle closure related to papillary block. As this pressure increment increases, the iris becomes more convex. Clinically significant pupillary block is present when Argon Laser Peripheral Iridoplasty the increased iris convexity brings the iris into apposition with ALPI induces immediate focal iris stromal contraction to pull the trabecular meshwork , with extreme anterior iris-bulging, the iris root away from the angle wall thus widening it. It is is known as iris bombe[25]. therefore an effective modality for reducing angle crowding in nanophthalmos[31], PAC[32], lens-induced angle closure, Management and plateau iris configuration. It acts by eliminating and The management of PACG requires repeated ocular reducing the amount of residual appositional angle closure examinations with special emphasis on the evaluation after LPI[33]. of the filtration angle to determine the mechanism of the angle-closure and the clinical stage of the disease. The first Trabeculectomy treatment of an acute attack is primarily symptomatic, but When more than three quadrant synechial closure is must be followed by care to prevent the development of present the management of PACG is not unlike POAG. chronic angle closure glaucoma. In patients with established However, trabeculectomy in chronic PACG is associated synechial closure and optic neuropathy the IOP must be with a higher risk of failure, postoperative anterior controlled aggressively with reassessment of the target IOP chamber shallowing, malignant glaucoma, and a significant at each stage. Surgical decisions for angle closure should be rate of cataract formation[34,35,36,37] as compared to taken after careful evaluation of anatomic and physiological POAG. In a retrospective case series of patients with factors, and individualized on a case to case basis. medically uncontrolled acute PAC who underwent urgent After better understanding the pathophysiology of the disease, trabeculectomy only 56.2% had successful long term IOP

DJO Vol. 21, No. 1, July-September, 2010 20 New Insights in Primary Angle Closure Glaucoma Delhi Journal of Ophthalmology control without antiglaucoma medication during the mean and angle crowding after LPI, and therefore is the definitive follow up of 22 months[38]. Due to low success rate and high procedure in both the treatment and prevention of acute and rate of complications trabeculectomy in PAC is becoming less chronic angle closure glaucoma. It relieves all 3 mechanisms popular compared to lens removal. Trabeculectomy should of primary angle closure related to pupillary block, plateau be performed first in eyes with advanced glaucomatous optic iris and the thick anteriorly displaced lens. Hayashi et al[39] neuropathy with central 10 degrees of visual field involvement demonstrated that anterior camber depth and angle width where IOP is not controlled despite topical medications (atleast in ACG eyes approximates that of POAG eye and control 3 topical medications including one prostaglandin ) post laser normal eyes. In a study done on chronic angle closure patients iridotomy. In such eyes trabeculectomy must be performed showed mean increase in ACD and angle width from 2.04 mm with 2 releasable sutures to prevent shallow anterior chamber to 3.44 mm this was due to exchange of the thickened lens in the post operative period. The patient must be counselled (5mm) for IOL (1mm)[40,41]. regarding the requirement for cataract surgery in the near future. Surgical Tips and Caveats With advancement and increased skill of cataract it is possible Role of Lens Removal to do safe and successful cataract surgery in angle closure Lens removal seeks to correct persistent pupillary-block patient. Surgical challenges in doing cataract surgery in angle

TABLE 1 Effect of lens removal in primary angle closure disease

Study (year) Lens Procedure Glaucoma Preop Gonioscopy Follow-up Preop/Postop Success % Type (# of eyes) (months) (mean IOP mmmHg) IOP<22mmHg Greve42 ECCE AACG (5) Near or complete Range 31 to 16 76% (1988) PCIOL closure 6-42 Gunning43 ECCE CACG (41) PAC Mean 22.6 to 15.6 65% (1991) PCIOL 14.3 Gunning44 ECCE CACG (22) PAS Mean 53 28 to 17 Overall success of 68% (1998) PCIOL 77%eyes reported in both groups vs Trabeculectomy Roberts45 PHACO AACG (18) 3600PAS (2) 36, 24 mos 39 to 17 67% (2 eyes) (2000) PCIOL Lai46 PHACO CACG (7) 3600PAS (7) Mean 9 33 to 13 100% (2001) PCIOL GSL DLPI Hayashi47 PHACO CACG (68) No data Mean 25 21 to 15 41% (2001) PCIOL Jacobi48 PHACO AACG (43) Partial closure (7) Mean 10 41 to 18 72% (2002) PCIOL CSI AACG (32) Partial closure (9) 40to 20 35% Kubota49 PHACO CACG(13) PAS >1800 (5) Mean 13 to 14 62% (2003) PCIOL 14 Nonaka50 PHACO PAC (13) 2 Q 3 19 to 15 No data (2005) PCIOL closed by UBM Lai51 PHACO CACG (21) >90 - 270 closed Mean 21 20 to 15.5 66.7% (2006) PCIOL Liu52 PHACO PACG (29) PAS 9 clock 3 15 to12 41% (2006) PCIOL PAC/suspect(28) hours (17%) 14 to 12 100% Imaizumi53 PHACO AACG (18) No data 6 49 to13 100% (2006) PCIOL AACG (2) Pachimkul54 PHACO PACG(56) 6 23.3 to 14.8 (2008) PCIOL 6

AACG acute angle-closure glaucoma; CACG chronic angle closure glaucoma; CD choroidal detachment; CSI conventional surgical iridectomy; ECCE extracapsular cataract extraction; GSL goniosynechialysis; IOP intraocular pressure; mos months; # number; PAC primary angle closure; PACG primary angle-closure glaucoma; PAS peripheral anterior synechia; PCIOL posterior chamber intraocular lens; PHACO phacoemulsification.

Vol. 21, No. 1, July-September, 2010 DJO 21 Delhi Journal of Ophthalmology New Insights in Primary Angle Closure Glaucoma closure patients are that there could be difficulty in access showing the effect of lensectomy in PAC patients (Table 1). (small palpebral fissure), inflammed eye, high IOP, diminished red reflex, corneal epithelial and stromal edema, reduced Goniosynechiolysis working space due to shallow chamber and small pupil, PAS, It can be effectively done in cases of recent PAS formation large size of the lens and an already compromised corneal (< 1 year), long-standing PAS are likely to be associated with endothelium. There are increased chances of iris prolapse, permanent trabecular damage[55]. It is done with the help problems during capsulorhexis, high capsular bag tension, lens of direct gonioscope, after entering the chamber viscoelastic subluxation, posterior capsular rupture, malignant glaucoma injected and synechiae released with the help of blunt tip and suprachoroidal hemorrhage. To avoid these problems spatula. Frequent complications include hyphema, fibrinous • IOP must be controlled preoperatively, intravenous mannitol reaction and synechial reclosure of the angle. Given that the can be used for this purpose. procedure does not address the underlying pathomechanism • Topical anesthesia is best if you are an expert surgeon, if for synechial angle closure, be it pupillary-block or angle- you need to use peri-bulbar block inject only 3-5 ml and crowding, its use is recommended as an adjunct to other give intermittent digital massage. procedures such as LPI[56], ALPI[57,58], or mainly lens • Atropine may be used for dilating the pupil. extraction[60,61]. • If IOP is found to be very high during surgery, a pars plana Razeghinejad MR has also shown that combined vitreous tap is an option using a 23/25G vitrectomy probe phacoemulsification and viscogoniosynechialysis seem to be • Gradual decompression of the anterior chamber is essential an effective surgical procedure in the treatment of patients to minimize suprachoroidal hemorrhage. with CACG and angle restoration whether controlled or • Intraoperative preservative free intracameral adrenaline can uncontrolled by medication[62]. be use to dilate pupil. • Use chilled BSS plus and Viscoat to coat and protect the Phacoemulsification vs Laser PI for APAC corneal endothelium. Lam DS et al[61] compared early phacoemulsification and • Meticulous wound construction to avoid iris prolapse. peripheral iridotomy in acute primary angle closure and found (avoid posterior scleral entry) that the prevalence of raised IOP at 18 months was 3.3% of • Use iris hooks or pupil ring expanders in small pupil. the cases in phaco group vs 46.7% in LPI group. Angle in • Straight phaco tip should be used as there is less room for phaco group was more open compared to LPI group (mean manipulation in the crowded AC. Shaffer gonio grading 2.10 ± 0.76 vs 0.73 ± 0.64). PAS was • Goniosynechiolysis may be done if the presence of PAS is more in LPI group 228.6 ± 89.2 compared to 101.3 ± 74.6 confirmed by direct gonioscopy. in phaco group. Requirement for topical antiglaucoma drugs • Increased post operative reaction may occur due to excessive was more in LPI group after 18 month followup. However, iris manipulation, intense topical steroid therapy with there were no statistically significant differences in visual cycloplegia may be requird in the post operative period. acuity and visual fields between the 2 groups at 18 months. • Put a drop of timolol at the end of cataract surgery to blunt The authors thus concluded that early phacoemulsification post operative IOP spikes, may add oral acetazolamide. is more effective in treatment of APAC as compared to laser • Must check digitally the IOP after you seal the wound and iridotomy. side port with BSS. Never leave a hard eye ball on the table In a prospective nonrandomized trial done in Japan[63],primary in such eyes and elevated IOP can be very detrimental to the phacoemulsification was compared with laser iridotomy in glaucomatous optic nerve head. patients with CACG and PAC. In IOL group, IOP decreased • Keep a close check on IOP in the post operative period. from 14.8± 4.2mm Hg to 10.8±1.6mm Hg (p <0.05), whereas Some patients may be steroid responders – switch to in PI group 15.5±4.1 mmHg to 14.7±4.7mmHg (p=0.76). In bromofenac or nepafenac. IOL group postoperative 6 months no glaucoma medication The chronicity and stage of the angle closure process determines was required, PI group 0.2±0.4 (p <0.05) medication was the surgical outcome. In cases in which there is early optic disc required. Safety in both procedures was comparable. cupping and mild visual field loss, lens extraction alone may be enough to achieve adequate IOP control; whereas eyes with Phacoemulsification vs Phacotrabeculectomy advanced glaucomatous optic neuropathy are more likely to Two RCTs[64,65] were conducted in Hongkong comparing have poor residual trabecular meshwork function as a result of phacoemulsification with phacotrabeculectomy in medically PAS which affect more than three quadrants or non-synechial controlled and medically uncontrolled chronic angle closure damage. In such cases, an additional filtration procedure may glaucoma. They showed lower intraocular pressures with be necessary for IOP control. phacotrabeculectomy than with phacoemulsification alone, In recent years many studies are done and data are available but the difference was marginal and mostly statistically

DJO Vol. 21, No. 1, July-September, 2010 22 New Insights in Primary Angle Closure Glaucoma Delhi Journal of Ophthalmology insignificant. More complication and more glaucomatous optic with shallow AC with a functional bleb and an already neuropathy deterioration was seen with phacotrabeculectomy compromised endothelium, there is decrease in visual acuity group. Tham CC et al have also shown in another recent study and all structural and functional tests of glaucoma become that combined phacotrabeculectomy resulted in significantly difficult to perform and interpret due to presence of lenticular more surgical complications than phacoemulsification alone opacification. There is also a surgeon factor as many glaucoma in CACG eyes with coexisting cataract. They found that there surgeons are excellent when it comes to trabeculectomy but was no difference in visual acuity or disease progression may not be the best of cataract surgeons and hence would between the 2 treatment groups[66]. not prefer phacoemulsification as the initial procedure. If on the other hand a lens extraction is done first (with IOP being At R.P. Centre we evaluated the effect of phacoemulsification controlled medically) – the patient’s vision improves, all and foldable intraocular lens (IOL) implantation on biometric investigations (visual fields, HRT,GDX, OCT etc) become determinants of the anterior chamber angle in primary angle easy to perform an interpret and it is much easier to perform closure glaucoma (PACG) using Ultrasound Biomicroscopy a trabeculectomy in a pseudophakic eye with a deep anterior (UBM). chamber. The only precaution that needs to be taken is that Forty six eyes of 46 patients with chronic PACG and IOP must be medically controlled at all times during the cataract having a patent laser iridotomy were included in follow up post cataract surgery. this prospective, interventional case series. Angle parameters So a two stage procedure seems to be the best option. were measured using UBM, before surgery and 3 months Stage 1-cataract surgery by temporal clear corneal after phacoemulsification with IOL implantation. Intraocular phacoemulsification. IOP monitored and if required managed ocular pressure (IOP) was measured by applanation with topical medical therapy. If IOP not controlled medically tonometer and records of glaucoma medication administered : Stage 2 –trabeculectomy with MMC after 3 months or more. were maintained. Main outcome measures were IOP, Central If a patient is not amenable to follow up, has a poor Anterior Chamber depth (ACD), trabecular iris angle (TIA), compliance with topical ocular hypotensive therapy, advanced angle opening distance at 250 and 500 microns from scleral glaucoma with central 10 degrees visual field involvement spur (AOD250 and AOD500). The mean age of study or 360 degrees synechial closure of the angle confirmed on participants was 56.5 + 9.9 years(range 44-75yrs).The indentation gonioscopy : a phacotrabeculectomy with MMC preoperative mean IOP was 25.0 ± 5.4 mm of Hg on maximum is the preferred option. antiglaucoma medication which reduced to 15.8 ± 3.8 mm Hg (p=0.0001) at 3 months. Number of antiglaucoma medications Clear lens also decreased from 2.4 ± 1.1 to 0.4 ± 1.1 (p=0.0001). There At this moment due to lack of scientific evidence coupled with was a significant widening of the anterior chamber angle with ethical concerns, clear lens removal cannot be advocated as an the TIA increasing significantly after phacoemulsification option for the management of PACG. A laser iridotomy should (p<0.001) with an associated increase in AOD250, AOD500 be performed first and if there is presence of residual angle and ACD (p<0.001). We found that Phacoemulsification in closure, ultrasound biomicroscopy should be performed to eyes with PACG results in significant widening of the anterior rule out plateau iris syndrome (treated by ALPI). The patient chamber angle. This results in better IOP control after surgery should be put on medical management with prostaglandin and decreases the need for glaucoma medications. These analogues being the first line therapy. If IOP is not controlled findings suggest that in eyes with primary angle closure on medical therapy, a trabeculectomy should be performed glaucoma – lens extraction should be performed as the first with mitomycin c and use of releasable sutures to prevent stage procedure as it may control the IOP in majority of early post operative shallowing of the anterior chamber. eyes with / without medical therapy. If IOP is not controlled medically, a trabeculectomy may be performed as a second Conclusion stage procedure after 3 months. The lens has an important role in the pathogenesis of primary So the big questions remains that in an eye with PACG with and secondary ACG, and clinical studies suggest that IOP not controlled medically post laser iridotomy, what is the lensectomy and PCIOL implantation for ACG patients may preferred practice pattern with and without cataract.? offer successful IOP control, with prevention of progression of the glaucomatous optic neuropathy. The mechanism for this is Cataract present elimination of pupillary block and widening of the angle, thus Conventionally trabeculectomy has been performed first reducing the iridotrabecular proximity. Medical management for IOP control in eyes with PACG. If trabeculectomy is and LPI remain the most common modes of treatment of performed first, it leads to development / progression of an acute attack but newer approaches including early lens cataract, phacoemulsification becomes difficult in an eyes removal are gaining popularity as a definitive treatment that

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Ophthalmology. 46. Lai JS, Tham CC, Lam DS. The efficacy and safety of combined 1999;106:669—7 phacoemulsification, intraocular lens implantation, and limited 61. Lam DS et al. Randomized trial of early phacoemulsification goniosynechialysis, followed by diode laser peripheral versus peripheral iridotomy to prevent intraocular pressure iridoplasty, in the treatment of chronic angle-closure glaucoma. rise after acute primary angle closure Ophthalmology J Glaucoma. 2001;10:309—15 2008;115:1134–1140. 47. Hayashi K, Hayashi H, Nakao F, Hayashi F. Effect of intraocular 62. Razeghinejad MR, Rahat F.Combined phacoemulsification and surgery on intraocular pressure control in glaucoma patients. J viscogoniosynechialysis in the management of patients with Cataract Refract Surg. 2001;27:1779—1786 chronic angle closure glaucoma.Int Ophthalmol. 2010 Feb 23. 48. Jacobi PC, Dietlein TS, Luke C. et al. Primary [Epub ahead of print] phacoemulsification and intraocular lens implantation for acute 63. Hiroko Hata, Shinta Yamane, So Hata, Hiroshi Shiota Preliminary angle closure glaucoma. Ophthalmology. 2002;109:1597—603 outcomes of primary phacoemulsification plus intraocular lens 49. Kubota T, Toguri I, Onizuka N, Matsuura T. Phacoemulsification implantation for primary angleclosure glaucoma. The Journal of and intraocular lens implantation for angle closure glaucoma Medical Investigation 2008;55. after the relief of pupillary block. Ophthalmologica. 64. Tham CC, Kwong YY, Leung DY, et al Phacoemulsification 2003;217:325-8 versus combined phacotrabeculectomy in medically 50. Nonaka A, Kondo T, Kikuchi M. et al. Cataract surgery uncontrolled chronic angle closure glaucoma with Cataracts. Ophthalmology 2009;116:725–731 for residual angle closure after peripheral laser iridotomy. 65. Tham CC, Kwong YY, Leung DY, et al. Phacoemulsification Ophthalmology. 2005;112:974-9 versus combined phacotrabeculectomy in medically controlled 51. Lai J, Tham C, Chan J. The clinical outcomes of cataract chronic angle closure glaucoma with cataract. Ophthalmology extraction by phacoemulsification in eyes with primary angle- 2008;115:2167–73. closure glaucoma and co-existing cataract: a prospective series. 66. Tham CC, Kwong YY, Leung DY, et al. Phacoemulsification vs J Glaucoma. 2006;15(1):47—52 phacotrabeculectomy in chronic angle-closure glaucoma with 52. Liu C, Cheng C, Wu C. et al. Factors predicting intraocular cataract complications.Arch Ophthalmol. 2010 Mar;128(3):303- pressure control after phacoemulsification in angle closure 11 glaucoma. Arch Ophthalmol. 2006;124:1390—4

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Major Review Idiopathic Juxtafoveolar Retinal Telangiectasis Neha Goel , Bhanu Pratap Singh Pangtey, Anisha Seth, Usha Kaul Raina, Basudeb Ghosh Vitreo Retina Services, Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi

Idiopathic juxtafoveolar retinal telangiectasis (IJRT), also known as idiopathic macular telangiectasia (IMT), refers to a heterogeneous group of well recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management. Classically, three groups of IJRT are identified. Group 1 is unilateral, easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group 2, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, minimal exudation, superficial retinal crystalline deposits, and right-angle venules along with characteristic and diagnostic angiographic and optical coherence tomography (OCT) features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularisation (SRNV) is common. Group 3 is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This article presents a current review of IJRT, including the classification, clinical features, pathogenesis, complications, differential diagnosis, and treatment modalities.

Introduction exudative and non-familial (groups 1A and lB), and those Idiopathic juxtafoveolar retinal telangiectasis (IJRT) is an that are primarily acquired, non-exudative, obstructive and uncommon cause of vision loss, which may be unilateral or occasionally familial (groups 2A, 2B, 3A and 3B). Group 2A bilateral. It comprises a group of retinal vascular anomalies is the most common subtype reported and its development has characterized by retinal vessel dilation and tortuosity, been summarized into five stages (Table 2)[3]. Group 1A is multiple aneurysm formations, varying degrees of vascular the second most common. leakage, incompetence and lipid exudate deposition. Visual In recent years, newly recognized manifestations have loss is related to intraretinal edema, foveal atrophy and/or the expanded and refined the clinical spectrum of these development of subretinal neovascularisation (SRNV)[1,2]. macular vasculopathies. Furthermore, the use of high speed Historically, there has been confusion differentiating Coats’ angiography and optical coherence tomography (OCT) have disease and IJRT. The term Coats’ disease is now reserved provided a better understanding of the nature of the vascular for congenital retinal telangiectasis associated with massive abnormalities. In 2006, Yannuzzi et al proposed a simplified exudation, retinal detachment and retinal degenerative classification termed idiopathic macular telangiectasia (IMT) changes. This differs from IJRT, whereby exudation or with 2 distinct types (type I, or aneurysmal telangiectasia, diffusion abnormalities from incompetent capillaries are equivalent to group 1A and B and type II, or perifoveal confined to the juxtafoveolar region and are either of telangiectasia, equivalent to group 2A)[5]. The third type, congenital or unknown origin[3]. occlusive telangiectasia, was omitted from the classification based on its rarity and presence of capillary nonperfusion Classification rather than macular telangiectasia as the primary abnormality. Gass[2], who originally described this entity in 1968 and Perifoveal telangiectasia was further classified in 2 stages: the later coined the term IJRT in 1982, classified the disease nonproliferative stage when there are exudative telangiectasia into several types on the basis on biomicroscopic and and foveal atrophy, and the proliferative stage with the advent angiographic findings[3]. In 1993, Gass and Blodi revised of SRNV. this classification and defined 3 distinct groups[4]. Group 1 patients have clinically visible retinal telangiectatic blood Pathogenesis vessels and retinal exudation; group 2 patients have occult Gass and Oyakawa[3] suggested that chronic venous stasis telangiectasis and minimal exudation; and group 3 patients due to obstruction of the retinal veins as they cross retinal have clinically visible telangiectasis, parafoveolar capillary arteries on both sides of the horizontal raphe may be a cause occlusion and minimal exudation. Subclassification of groups of group 2A IJRT. Low-grade nutritional damage induced (Table 1) include those which are predominantly congenital, by specific retinal circulatory disturbances affects the retinal

DJO Vol. 21, No. 1, July-September, 2010 26 Idiopathic Juxtafoveolar Retinal Telangiectasis Delhi Journal of Ophthalmology cells, particularly those at the level of the inner nuclear layer, cases with good vision, and does not necessarily indicate loss which includes the Müller cells, leading to degeneration and of the photoreceptor cells. Intraretinal RPE proliferation has atrophy of these cells and the connecting photoreceptor cells been explained by the loss of PR cells, which allows the RPE resulting in growth of vessels and the migration of retinal cells to migrate into the overlying retina, especially along the pigment epithelial (RPE) cells into the retina[4]. Abnormalities venules. All eyes exhibiting RPE proliferation and migration of glucose tolerance may be found in cases with Type 2A demonstrate disruption of the IS/OS PR junction[8]. IJRT[6]. This supports the hypothesis that bilateral disease Macular holes (MH) may occur as a sequel to chronic macular occurs in relation to a widespread metabolic disturbance in oedema. We would then expect macular holes to occur more the retina, whereas unilateral cases represent a local and truly frequently in association with IJRT. However, the rarity vascular defect. of MH in IJRT as well as the preservation of good visual Preliminary data using 2-wavelength autofluorescence acuity in patients with MH implies that the holes were the imaging indicate that macular pigment density (MPD) is result of lateral separation of the photoreceptors within the significantly reduced in the central retina. These recent fovea and that there could not have been profound atrophy findings have provided increasing evidence that group 2A of the photoreceptors. There is a loss of the structural aspects IJT is not a disease limited to the retinal vasculature but that afforded by Muller cells, particularly the Muller cell cone, in neurons are intrinsically involved as well[7]. the central macula in IJRT[12].

Clinical picture and Diagnosis Differential Diagnosis The diagnosis of IJRT rests on a combination of stereoscopic When IJRT is suspected, it must be differentiated from biomicroscopy, fundus fluorescein angiography (FA) and OCT venous occlusive disease, diabetic retinopathy, radiation (Figures 1 – 4). On FA, the telangiectatic vessels are easily retinopathy, Eales’ disease, carotid artery occlusion and sickle visible straddling the horizontal raphe and filling promptly cell retinopathy. Group 1 patients, in addition to that noted in both the superficial and deep juxtafoveolar capillary above, should be distinguished from those with Coats’ disease plexus. Central cystic or noncystic macular edema is evident which is defined by extensive peripheral retinal telangiectasis, angiographically as late intraretinal staining. Diagnostic exudative retinal detachment, relatively young age of onset dilemma commonly exists to differentiate IJRT from occult and male predilection. Group 2 patients, during the early SRNV or cystoid macular edema (CME) on FA. The merit of stage of the disease, may demonstrate foveolar atrophy that OCT is to provide information about the retinal structure and simulates lamellar macular hole formation, or may possess a thickness in IJRT, as well as provide diagnostic clues in cases yellow foveal lesion that may be mistaken for adult vitelliform which are equivocal on FA. Following are the OCT features dystrophy or Best’s disease. In the late stage, patients who in IJRT[8,9] exhibit macular stellate pigment plaques with SRNV may 1. Foveal cyst in the innermost retinal layers – most common be misdiagnosed as having age related macular degeneration finding (ARMD) or focal choroiditis. In differentiating patients 2. Internal limiting membrane (ILM) draping across the with IJRT associated with SRNV from those with exudative foveola related to an underlying loss of tissue ARMD, IJRT is rarely associated with pigment epithelial 3. Intraretinal hyperreflective lesions – second most common detachment and large neovascular complex formation. Group finding. They correspond to ophthalmoscopically visble 3 patients who demonstrate atrophy of the juxtafoveolar retina hyperpigmented lesions. with capillary occlusion and minimal exudation are most 4. Disruption of the inner segment/outer segment (IS/OS) similar to those with sickle cell retinopathy[13]. photoreceptor (PR) junction line 5. Foveal detachment Management 6. Blunting of the foveal pit/foveal flattening Macular edema and exudation are the main cause of visual 7. Foveolar thinning loss in group 1 IJRT; the amount of exudation, edema, and 8. SRNV subsequent visual acuity loss is variable[3,4]. Treatment 9. Lamellar or full thickness macular hole[10,11] options include laser, intravitreal steroids, or anti-vascular endothelial growth factor (VEGF) agents[15,16]. Laser Whenever there is absence of macular oedema on OCT in spite may not always be possible due to the close proximity of prominent leakage of fluorescein in the fovea, IJRT must be of the abnormal vessels to the fovea. Also, due to lack of suspected. Presence of foveal thinning despite occurrence of significant improvement in visual outcomes and increased foveal cysts/detachment indicates that there is some degree of risk of the development of SRNV following treatment, laser retinal atrophy and serves as a distinguishing feature of IJRT. photocoagulation for macular edema associated with IJRT is Disruption of the IS/OS line can be visualised even in early currently not recommended[14]. Intravitreal triamcinolone

Vol. 21, No. 1, July-September, 2010 DJO 27 Delhi Journal of Ophthalmology Idiopathic Juxtafoveolar Retinal Telangiectasis Table 1: Classification of IJRT

Group Mean age of onset of Predominance Typical area of involvement, Visual loss Systemic association symptoms clinical picture

1A 40 years Male, Telangiectasia and aneurysms Amount of exudation None Unilateral temporal to fovea, 2 Disc and visual loss variable Diameters 1B Middle age Male, Focal, limited to two clock hour Exudation and edema None Unilateral may or may not occur. 20/25 or better 2A Middle age Male=Female, Retinal thickening temporal to the Progressive Possibly diabetes and older Bilateral but fovea, right-angle venules, RPE asymmetric hyperplastic plaques, superficial crystalline deposits, SRNV 2B Juvenile Bilateral SRNV Visual loss due to None SRNV 3A Middle age Female, Minimal exudation, capillary Visual loss due to Polycythemia, or older Bilateral obstruction and occlusion capillary obstruction hypoglycemia, ulcerative colitis, multiple myeloma, chronic lymphatic leukemia 3B Middle age or older Male=Female Minimal exudation, capillary and occlusion With CNS obstruction and occlusion Visual loss due to vasculopathy capillary obstruction and occlusion

Table 2: Stages of Group 2A IJRT

Stage 1 Asymptomatic Difficult to detect clinically Abnormal capillaries seen with fluorescein angiography (occult staining)

Stage 2 Asymptomatic Mildly dilated perifoveolar capillaries Slight graying of the retina, mild loss of transparency Superficial refractile particles

Stage 3 Progressive decreased acuity Dilated right-angled venules

Stage 4 Progressive decreased acuity RPE hyperplasia clumped around the right-angled venules Pseudovitelliform lesion

Stage 5 Rapid and severe vision loss Intraretinal and subretinal neovascularization, exudation and hemorrhage

DJO Vol. 21, No. 1, July-September, 2010 28 Idiopathic Juxtafoveolar Retinal Telangiectasis Delhi Journal of Ophthalmology acetonide (IVTA) is beneficial in the treatment of macular treatment, treatment of nonproliferative IJRT 2A with VEGF edema by its anti-inflammatory effect, downregulation of antagonists appears questionable. VEGF production, and stabilization of the blood retinal Although rare, development of SRNV generally results in barrier[15]. Intravitreal injections of anti-VEGF agents, poor visual acuity if left untreated, with 80% (21 of 26) of namely Bevacizumab, have shown improved visual outcome eyes in 1 study having a final acuity of 20/200 or worse[14]. and significant and sustained decrease in leakage onFA Histopathologic studies show that while neovascular and macular edema on OCT. It is likely that patients with membranes in ARMD originate from the choroidal group 1 IJRT with pronounced macular edema from leaky vasculature, vessels in proliferative IJRT originate from the telangiectasis may benefit functionally and morphologically retinal vasculature and contain more vessels and less fibrous from anti-VEGF injections supposedly even at a lower tissue. Before the advent of VEGF antagonists, therapeutic treatment frequency than in other diseases[16]. options for SRNV in IJRT 2A included laser photocoagulation, When considering treatment for group 2 IJRT, therapeutic photodynamic therapy (PDT) with or without IVTA, attempts for nonproliferative IJRT and those for the SRNV of transpupillary thermotherapy (TTT), and surgical removal the proliferative stage must be distinguished. The angiographic of the CNV[18]. VEGF has been implicated as the major late intraretinal staining pattern in nonproliferative IJRT angiogenic stimulus responsible for neovascularization in 2A has prompted many ophthalmologists to interpret it as IJRT 2A. Given the risk of permanent RPE damage with macular edema secondary to retinal vascular leakage. Several PDT, coupled with the huge evidence of efficacy of VEGF treatment modalities have been tried to treat this “macular antagonists in the treatment of SRNV in various entities, the edema.” To start, laser photocoagulation is not effective in the anti-VEGF approach is a reasonable treatment alternative treatment of nonproliferative IJRT 2A. In addition, treatment for proliferative IJRT 2A. Anatomical peculiarities related to maybe associated with RPE changes, post-treatment retinal neovascular lesions in the setting of IJRT, such as the location hemorrhages, and increased retinal vascular distortion[17]. above the RPE and the presence of anastomotic retinal Given that OCT shows that the fluorescein leakage seen is not vascular connections may facilitate inflow and concentration associated with retinal thickening, the angiographic “leakage” of the drug in the neovascular complex. Both intravitreal is probably due to the staining of the extracellular matrix Bevacizumab (1.25 mg)[20,21] and Ranibizumab (0.5 mg) rather than extracellular leakage, and visual acuity correlates [22] have been used successfully in proliferative group 2A with photoreceptor layer disruption and not the degree of IJRT. Recently, primary treatment with combined intravitreal “leakage,” IVTA is likely to have a minor or no therapeutic Bevacizumab or Ranibizumab and PDT have been reported effect in nonproliferative IJRT 2A[18]. Recent publications anecdotally for proliferative IJRT 2A[23,24]. In both cases, on intravitreal anti-VEGF injections, namely Bevacizumab, the PDT was performed with a laser spot of the same size report on possible short term benefits in some cases of IJRT as the SRNV and followed by the intravitreal injection. Thus 2A[19,20]. Inhibition of VEGF may be useful before atrophic anti-VEGF therapy combined with or without PDT appears changes occur. VEGF plays a pathophysiological role in efficacious and should be considered as a treatment option for IJRT 2A, because the structural capillary changes described proliferative IJRT 2A. histopathologically lead to a disturbed exchange of oxygen and substrates between the vascular lumen and neurosensory Conclusion retina, which in turn may lead to a hypoxia induced IJRT comprises essentially three groups that differ in their increased VEGF release by retinal cells[19]. However, appearance, presumed pathogenesis and management. In group despite decreased leakage on FA, underlining the effect of 1, the unilateral telangiectasis is easily visible and vision loss Bevacizumab on vessel stability and permeability, small is a result of exudation in the macula. Intravitreal steroids or cystic changes seen on OCT and visual acuity may remain Bevacizumab is generally effective in controlling the macular unchanged, emphasizing that visual deterioration is caused edema. In group 2, the most common, the bilateral capillary by microcystic degeneration and progressive retinal atrophy telangiectasis is more difficult to detect biomicroscopically, and not by intraretinal edema, and therefore cannot be halted but the angiographic and OCT findings are characteristic with intravitreal anti-VEGF injections. Moreover, VEGF and diagnostic. Vision loss is progressive and primarily due plays a role in photoreceptor differentiation and survival, to retinal atrophy, not exudation or development of SRNV. and in maintaining retinal vascular homeostasis. Therefore, Treatment options for this group are still limited, and have blocking VEGF may accelerate apoptosis among ganglion shown effectiveness only for the neovascular component. This cells and photoreceptors in IJRT 2A[19]. Given the current is primarily because the pathogenesis of this telangiectasis lack of convincing evidence of efficacy, the concern about the remains an enigma and is possibly secondary to a retinal potential deleterious effects of repeated injections and cost of neuronal dysfunction. New imaging modalities and functional

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Figure 1 – (a) Colour fundus photograph of a patient with IJRT 2A showing a greyish ring around the foveal centre with numerous superficial retinal crystals and a blunted, right angled draining venule (arrow). (b) Corresponding fluorescein angiogram shows in the early phase clearly visible dilatation and telangiectasis of the perifoveal capillary network with confirmation of the right angled venule (arrow). These capillaries show late intraretinal staining (c). Horizontal (d) and vertical (e) OCT scans show foveal detachment (asterisk), subfoveal cysts (arrowhead) with partial loss of the highly reflective line considered as the boundary between photoreceptor inner segments and outer segments (arrows).

Figure 2 - (a) Colour fundus photograph of a patient with IJRT 2A showing a greyish ring around the foveal centre with early intraretinal pigment deposition in the vicinity of a right angled venule (black arrow). (b) Corresponding fluorescein angiogram shows late intraretinal staining. Vertical (c) OCT scan shows blunting of the foveal pit (arrowhead) with foveal thinning. Horizontal OCT scan shows the characteristic ILM drape (dashed arrow) with partial loss of the highly reflective line considered as the boundary between photoreceptor inner segments and outer segments (arrows).

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Figure 3 - (a, e) Colour fundus photograph of a patient with IJRT 2A showing stellate intraretinal pigment epithelial plaques, right angled draining venules and refractile retinal crystals (Stage 4). The disease is asymmetric, being more advanced in the left eye. (b, f) Corresponding fluorescein angiograms show blocked fluorescence due to the RPE hyperplasia with some intraretinal staining. Vertical (c) and horizontal (d) OCT scans of the right eye show blunting of the foveal pit, foveal thinning and a hyperreflective intraretinal lesion corresponding to the pigment (arrow). In the left eye, the flat pigmentary proliferation on the foveal surface masks the underlying retinal structure on OCT (g, h).

Figure 4 - (a) Colour fundus photograph of the right eye of the patient in Figure 2 showing temporal parafoveal retinal elevation with few crystalline retinal deposits, a right angled venule (arrow), nasal RPE alterations and subretinal blood characteristic of SRNV (IJRT 2A, Stage 5). (b) Corresponding fluorescein angiogram shows early hyperfluorescence with intense late leakage (c). Horizontal (d) and vertical (e) OCT scans show elevation of the juxtafoveal retina secondary to the presence of a hyperreflective fusiform complex lying at the outer retina/retinal pigment epithelium (RPE) level (extent marked by arrow) and minimal intraretinal fluid.

Vol. 21, No. 1, July-September, 2010 DJO 31 Delhi Journal of Ophthalmology Idiopathic Juxtafoveolar Retinal Telangiectasis tests will hopefully improve the understanding and treatment 2007;27:473-6. capabilities of this condition. Group 3 is a perifoveolar 13. Tasca J, Grogg JA. Idiopathic juxtafoveolar retinal telangiectasia: capillary occlusive condition and is poorly understood a review and case report. Clin Eye Vis Care 2000;12:79-87. because of the scarcity of cases reported. 14. Engelbrecht NE, Aaberg TM, Sung J, Lewis ML. Neovascular membranes associated with idiopathic juxtafoveolar References telangiectasis. Arch Ophthalmol 2002;120:320-4. 1. Yanoff M, Duker JS. et al, eds. Ophthalmology. 2nd ed. St. 15. Li KK, Goh TY, Parsons H, Chan WM, Lam DS. Use of Louis: Mosby; 2004:196 –203. intravitreal triamcinolone acetonide injection in unilateral 2. Gass JD. A fluorescein angiographic study of macular idiopathic juxtafoveal telangiectasis. Clin Experiment dysfunction secondary to retinal vascular disease, V: retinal Ophthalmol 2005;33:542-4. telangiectasis. Arch Ophthalmol 1968;80:592-605. 16. Gamulescu MA, Walter A, Sachs H, Helbig H. Bevacizumab in 3. Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal the treatment of idiopathic macular telangiectasia. Graefes Arch telangiectasis. Arch Ophthalmol 1982;100:769-780. Clin Exp Ophthalmol 2008;246:1189-93. 4. Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal 17. Park DW, Schatz H, McDonald HR, Johnson RN. Grid laser telangiectasis. Update of classification and follow-up study. photocoagulation for macular edema in bilateral juxtafoveal Ophthalmology 1993;100:1536 –1546. telangiectasis. Ophthalmology 1997;104:1838-46. 5. Yannuzzi LA, Bardal AMC, Bailey Freund A, Chen KJ, 18. Nowilaty SR, Al-Shamsi HN, Al-Khars W. Idiopathic Eandi CM, Blodi BA. Idiopathic macular telangiectasia. Arch juxtafoveolar retinal telangiectasis: a current review. Middle Ophthalmol 2006;124:450-460. East Afr J Ophthalmol 2010;17:224-41. 6. Millay RH, Klein ML, Handelman IL, Watzke RC. Abnormal 19. Charbel Issa P, Finger RP, Holz FG, Scholl HP. Eighteen-month glucose metabolism and parafoveal telangiectasia. Am J follow-up of intravitreal bevacizumab in type 2 idiopathic Ophthalmol 1986;102:363-70. macular telangiectasia. Br J Ophthalmol 2008;927:941-5. 7. Schmitz-Valckenberg S, Fan K, Nugent A, Rubin GS, Peto T, 20. Kovach JL, Rosenfeld PJ. Bevacizumab (avastin) therapy for Tufail A, Egan C, Bird AC, Fitzke FW. Correlation of functional idiopathic macular telangiectasia type II. Retina 2009;29:27-32. impairment and morphological alterations in patients with 21. Mandal S, Venkatesh P, Abbas Z, Vohra R, Garg S. Intravitreal group 2A idiopathic juxtafoveal retinal telangiectasia. Arch bevacizumab (Avastin) for subretinal neovascularization Ophthalmol 2008;126:330-5. secondary to type 2A idiopathic juxtafoveal telangiectasia. 8. Gaudric A, Ducos de Lahitte G, Cohen SY, Massin P, Graefes Arch Clin Exp Ophthalmol 2007;245:1825-9. Haouchine B. Optical coherence tomography in group 2A 22. Karagiannis D, Georgalas I, Ladas I, Eustratios P, Mitropoulos P. idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol A case of subretinal neovascularization treated with intravitreal 2006;124:1410-9. ranibizumab in a patient with idiopathic juxtafoveal retinal 9. Surguch V, Gamulescu MA, Gabel VP. Optical Coherence telangiectasis. Clin Interv Aging 2009;4:63-5. Tomography findings in idiopathic juxtafoveal retinal 23. Rishi P, Rishi E, Shroff D. Combined photodynamic therapy telangiectasias. Graefe’s Arch Clin Exp Ophthalmol and intravitreal bevacizumab as primary treatment for subretinal 2007;245:783–8. neovascularization associated with type 2 idiopathic macular 10. Patel B, Duvall J, Tullo AB. Lamellar macular hole associated telangiectasia. Indian J Ophthalmol 2009;57:241-2. with idiopathic juxtafoveolar telangiectasia. Br J Ophthalmol 24. Combined photodynamic therapy and intravitreal 1988;72:550–1. ranibizumab as primary treatment for subretinal neovascular 11. Olson JL, Mandava N. Macular hole formation associated with membrane (SRNVM) associated with type 2 idiopathic idiopathic parafoveal telangiectasia. Graefe’s Arch Clin Exp macular telangiectasia. Graefes Arch Clin Exp Ophthalmol Ophthalmol 2006;244:411–2. 2008;246:619-21. 12. Koizumi H, Slakter JS, Spaide RF. Full-thickness macular hole formation in idiopathic parafoveal telangiectasis. Retina

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Major Review Contact Lens Fitting in Keratoconus Pooja Singh, Raghav Gupta, Jeewan S Titiyal, Rajesh Sinha Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi Keratoconus is a progressive, non-inflammatory, asymmetric ectatic corneal disorder that results in high amount of myopia and astigmatic refractive error. Early cases may be managed by spectacles; however, the definitive management is use of contact lens. In the mild stage, soft toric contact lens may be used. In the moderate and advanced cases, rigid gas permeable contact lens is the mainstay of treatment of these cases. Rose-k contact lenses have now become the contact lens of choice in these cases; however, the SOPER design is also very useful for management in these cases. Some cases may require piggyback and scleral lenses for better stability of the lens, and improved visual acuity and comfort to the patient. Keratoconus is a progressive, non inflammatory disease of the Slit Lamp Findings: central cornea that causes thinning and ectasia which leads Stromal thinning to high amounts of myopia and irregular astigmatism. It is a Posterior stress lines (Vogt’s striae) (Figure 1) progressive disorder ultimately affecting both eyes, although only one eye may be affected at the initial encounter with the patient[1,2]. Patients who are suspects of keratoconus on the basis of corneal topography alone with normal slit lamp biomicroscopy are known as forme fruste keratoconus.

Clinical Manifestations of Keratoconus Symptoms of keratoconus People with early keratoconus typically notice a minor blurring of their vision and come to their clinician seeking corrective lenses for reading or driving. At early stages, the symptoms Figure 1 Vogt’s striae of keratoconus may be no different from those of any other refractive defect of the eye. As the disease progresses, Iron ring (Fleischer ring) vision deteriorates, sometimes rapidly and is usually not Scarring: Epithelial or Subepithelial correctable with spectacles. Visual acuity becomes impaired Retroillumination Signs: at all distances, and night vision is often quite poor. Some Scissoring on retinoscopy individuals have vision in one eye that is markedly worse than that in the other eye. Some develop photophobia, eye strain from squinting in order to read, or itching in the eye, but there is normally little or no sensation of pain. The classic symptom of keratoconus is the perception of multiple ‘ghost’ images, known as monocular polyopia. This effect is most clearly seen with a high contrast field, such as a point of light on a dark background. Instead of seeing just one point, a person with keratoconus sees many images of the point, spread out in a Figure 2 Oil droplet sign chaotic pattern. This pattern does not typically change from day to day, but over time it often takes on new forms. Patients Oil droplet sign (“Charleaux”) (Figure 2) also commonly notice streaking and flaring distortion around Photokeratoscopy signs: light sources. Compression of mires infer temporally (egg-shaped mires) Compression of mires inferiorly or centrally Signs of keratoconus Videokeratography signs: Localised increased surface power External signs Inferior superior dioptric asymmetry Munson’s sign Relative skewing of the steepest radial axes above and below Rizzuti phenomenon the Horizontal meridian

Vol. 21, No. 1, July-September, 2010 DJO 37 Delhi Journal of Ophthalmology Contact Lens Fitting in Keratoconus Classification of keratoconus: • If scarring in visual axis area it can affect the vision. On the basis of Radius of Curvature of anterior segment of cornea: Corneal Topography 1. Mild/early –8.00-7.00(mm) /42D-47D Videokeratography(3,4) Orbscan Pentacam 2. Moderate -6.90-6.50(mm)/47D-52D 1. In basic orbscan height map, a mean radius of curvature 3. Moderate/advanced-6.40-6.00 (mm)/53D- 56D of corneal map is calculated and relative height above or 4. Advanced/severe-<6.00(mm)/>57D below this ideal best fitting spherical surface is known. 2. Warm colors show areas higher than best fitting sphere Keratoconus cones can be classified as: and cooler color which are lower than best fitting sphere 1. Nipple: Small diameter (5mm); cone lies in the lower nasal quadrant Pachymetry 2. Oval: Larger (> 5mm); lies more commonly in the It provides additional information about corneal thickness inferno- temporal quadrant in diagnosing keratoconus. Ultrasound pachymetry is best 3. Globus: Largest diameter (>6mm); 75% of the cornea is accepted. affected Refraction Related conditions: Retinoscopy is difficult /scissoring reflex. Subjective Keratoconus has been found related with atopic conditions refraction is needed. In early keratoconus and mild cases can asthma and eye rubbing. be corrected with glasses. Monocular mild keratoconus is best It has also being linked to Leber’s congenital amaurosis, dealt with glasses. Down’s syndrome and mitral valve prolapse cases. Dry Eye Assessment Posterior keratoconus: Dry Eye is ruled out by asking for relevant symptoms and by It is the rare form of condition reported in some cases. Uniform doing schirmer’s test and tear breakup time. corneal steepning was observed in generalized posterior keratoconus and corneal steepning was observed in localized Contact Lens Options For Kertaoconus central and posterior keratoconus and corneal flattening was In cases of high irregular astigmatism and moderate/advanced observed in localized peripheral posterior keratoconus. keratoconus cases rigid contact lenses will be required effectively to provide new anterior surface to the eye giving Investigations good, clear vision. They are considered when good vision is not Slit lamp examination: attained by glasses less than 6/9 and shadowing and diplopia In early kc: persists with glasses and patient becomes symptomatic • thinning of epithelium due to cone progressing • extra cellular and intercellular accumulation in Rigid Gas Permeable Lenses epithelium at the periphery of the cone • First choice of correcting irregular astigmatism • A mild to high dk/t material is preferred providing In later stages: stability required for high powered lenses. • ruptures or folds in descement’s membrane • A balance is required between material which is deposit • eventually some form of corneal scarring is seen resistance especially in patients who are atopic and • small stromal scars are due to idiopathic breaks in providing sufficient oxygen flux.( Figure 4) bowman membrane • hydrops: marked stromal oedema occurs as a result of descement membrane rupturing allowing aqueous humor to enter the stroma. (Figure 3)

Figure 4 Contect lens fitting in Kerato conus

Fitting Philosphies a. Apical bearing: Lens bears heavily on corneal apex. It provides good vision Figure 3 Acute Hydrops but may result in corneal abrasions scarring. (Figure 5)

DJO Vol. 21, No. 1, July-September, 2010 38 Contact Lens Fitting in Keratoconus Delhi Journal of Ophthalmology Advanced keratoconus • Small diameter lenses • S-Lim (J Allen) • Dyna-intra limbal (No.7) • Scleral lenses /JUPITOR lenses • Gas permeable (innovative sclerals)

Figure 5 Apical Bearing Preliminary examination b. Apical clearance: Many patients with keratoconus will be in their late teens or The back surface of the lens vaults apex of the cone. There is early twenties. They will need information and reassurance. lesser risk of corneal scarring. It causes variable vision due to They may present with concerns about the speed with which uncorrected astigmatism. (Figure 6) their vision has deteriorated. • It is important to explain the reason why the spectacle prescription has been changing rapidly over the past 12- 24 months. • The nature of the corneal thinning disorder and the reasons for corneal distortion should be explained. The advantages of contact lenses over spectacles should be emphasized. Figure 6 Apical clearance • The progression of the condition and the prognosis should be discussed. c. Three Point Touch: • An information leaflet explaining the condition, and Lens rests lightly against the cone apex. Also supported on information about the local keratoconus support group, nasal and temporal zones by mid periphery of the back surface should be provided of the lens. It provides stable fitting and good vision. (Figure 7) Fitting Considerations • cone position shape and size • corneal radius (central and steepest) • corneal toricity • degree of myopia and corneal astigmatism

Fitting Algorithm 1) After taking full history and symptoms, the preliminary Figure 7 Three Point Touch examination should include age, occupation, and Type of RGP Lens Design : motivation. Any history of previous contact lens Early keratoconus intolerance or allergies should be noted. • Aspheric or multicurve lenses. This is the Preferred lens 2) Full slit lamp biomicroscopy is vital. fitting technique. 3) Examine the keratometer readings. The mires may be • Kera I and II (No.7)Acuity K much distorted; however they provide useful information • Rose K (David Thomas) at the initial stages. 4) Choose the correct base curve using corneal topography; Moderate keratoconus start with the base curve equivalent to the steeper of • Rose K 2(David Thomas) the two keratometer readings. Many variations on this • Woodward KC3 /SOPER LENSES philosophy exist as already discussed. 5) Allow the lens to settle for about 20minutes before • Kera II evaluating the fluoresce in pattern. • Quasar KNO7 6) Examine the central area, the mid peripheral area and the periphery. Moderate/Advanced keratoconus 7) Evaluate the lens in the central position. Once you have • Rose K 2/IC(David Thomas) judged the fit, alter the fit as necessary (for example • Kera II/III flattens, if pooling) until you obtain gentle apical touch • Profile K (JAllen) and the three-point-touch. Use the Guillon grading scale

Vol. 21, No. 1, July-September, 2010 DJO 39 Delhi Journal of Ophthalmology Contact Lens Fitting in Keratoconus for assessing the fluoresce in picture. There should be Edge lift can be varied with standard steep/flat optimum lift minimal bearing (touch) at the apex of the cone, as well It is a multi-spherical posterior design with aberration as an area of bearing between the periphery of the lens control aspheric optics across the back and front optic zone and the intermediate zone of the cornea. diameters. The design provides an easy-to-fit systematic 8) The lens should be ordered in mid-high Dk material approach with a flexible edge lift for the practitioner enabling after an over-refraction has been undertaken. A them to reduce their chair time and dispense the optimum collection appointment should be arranged. An aftercare fitting lens to the patient. The system (26-lens set) allows appointment should follow four weeks after the collection the practitioner to choose lens options based on a systematic appointment, when slight modifications may be necessary. fitting approach. The design starts with a standard 8.7 mm. diameter that incorporates a decreasing optic zone as the base Soper Contact Lens curve steepens coupled with an intrinsic, computer designed The Soper lens system uses bicurve lenses based on sagittal peripheral curve system. The lens is provided through depth. (Figure 8) A steeper lens is selected by maintaining Paragon Optics and is manufactured on a DAC lathe. The the same base curve but increasing the diameter. Three standard lift lenses should work approximately 70% of the fitting sets are available in these contact lenses. Mild (7.4mm time. Additional lens diameters are available when needed diameter, 6.0mm OZ), moderate (8.5mm diameter, 7.0mm (8.3, 9.0). Peripheral curves and even base curves can be OZ) and advanced (9.5mm diameter, 8.0mm OZ). The smaller configured in a toric design.[6-8]. (Figure 9) lenses are used for smaller, centrally located cones. The large diameters are used for oval cones. The peripheral curve of this system is constant. Peripheral alignment and apical clearance typify the soper contact lens design[5].

Figure 9 Rose - K Lens

Soft Lens /hydrogels/silicon Hydrogels Why Soft Contact Lenses? Figure 8 Soper Lens Clinical experience has demonstrated that small subset Rose-K Contact Lenses keratoconus patients may experience a corneal hypersensitivity Unique keratoconus lens design with computer generated in certain stages of their condition. This hypersensitivity may peripheral curves based on data collected by DR.Paul Rose of be due, in part, to a dilatation or stretching of the corneal nerve Hamilton, New Zealand fibers; in addition, minute ruptures in Bowman’s layer may The system incorporates triple peripheral curve system be a contributing factor. This subset of patients may find it standard flat and steep in order to achieve ideal edge lift of difficult, or even impossible, to tolerate well-fitted GP lenses. 0.8mm Available in base curves: 4.75-8.00mm, Diameter: 7.92- Soft Designs for Keratoconus 10.00mm A viable option is to use one of the many custom toric soft Toric surfaces are available on front and back and periphery contact lenses we currently have at our disposal. These have too. (Table 1) proven successful in the early stages of the condition or in form

Rose K2 Rose K2 IC Rose K2 Post graft Primary indication Nipple/Oval Keratoconus Pellucid Marginal Degeneration, For patients who have Keratoglobus(PMD), Lasik undergone penetrating Induced Ectasia keratoplasty

Secondary Indication Early PMD Oval/Nipple Keratoconus Oval/Nipple Keratoconus

Parameters Available BC:4.3- 8.6mm BC: 5.7-9.3 mm BC: 5.7- 9.3mm OD: 7.9-10.4mm OD: 9.4 – 12.0 mm OD: 9.4- 12.0mm

Table 1 Type of Rose - K Contact Lens

DJO Vol. 21, No. 1, July-September, 2010 40 Contact Lens Fitting in Keratoconus Delhi Journal of Ophthalmology fruste keratoconus.2 However, custom soft spherical lenses long-term. Ideally, try to have the patient use the same care may also prove greatly successful. These lenses incorporate a regime for the two lenses as this will make cleaning easier, or tricurve posterior lens design with increased central thickness alternatively consider a disposable soft lens. The cornea should to mask much of the regular and irregular astigmatism. You be observed carefully for dryness and neovascularisation. can custom design these lenses using a broad range of base Piggyback contact lenses can play a beneficial role for patients curve radii, powers, diameters and center thickness values. who experience comfort or wetting problems, yet need the optical properties of a GP lens. At the least, the ability to use Advantages high-oxygen transmissible lens materials makes piggyback 1) They afford higher levels of comfort and longer wearing lens fitting a technique certainly worthy of a second look[9]. times, especially in patients intolerant of RGP corneal lenses or in monocular keratoconus. Hybrid lens system 2) They are useful where the cone apex may be displaced, Hybrids refer to contact lenses that have a rigid GP central especially if it is very low. portion surrounded by a soft lens material skirt. The earliest 3) They are useful for certain groups of patients, for lenses in this category were introduced as Saturn contact example airline pilots. lenses by Preci-sion-Cosmet in the early to mid-1980s. 4) They are relatively simple to fit. This initial lens concept went through several generations of development and several changes of ownership before Disadvantages arriving at what is offered today as the Soft Perm lens from 1) Visual acuity may be variable in cases of very high CIBA Vision. Among the changes in the Soft Perm lens from minus lenses. the original Saturn design was the addition of peripheral 2) Low-powered diagnostic lenses may not provide an curves onto the GP portion and changes to the peripheral accurate guide to the fit of the final lens, which may be curves of the soft skirt. Activity in the area of hybrid contact extremely high powered. lenses increased with the formation of SynergEyes, Inc. The 3) There may be reduced oxygen transmissibility and the company introduced the SynergEyes A hybrid lens, which risk of neovascularisation if the lenses are over worn. became the first in a family of lenses that include SynergEyes 4) If the condition has progressed, it may be difficult to KC (for keratoconus), SynergEyes Multifocal (an annular, change to RGP’s at a later stage. center-near design), and SynergEyes PS (for post-surgical fittings). One advancement that these entire lenses offer is the Piggyback lenses option of choosing among different peripheral curves (either With the advent of silicone hydrogel oxygen concern with two or three options depending on the design) for the soft piggybacking have greatly lessened. Weiss man and ye (2006) lens skirt portion. This adds another fitting parameter apart have shown that the oxygen transmission of a system using from just the base curve of the GP central portion to allow both high-Dk GP lenses and silicone hydrogel lenses is only better refinement of the fit. Of recent note are a number of new slightly less than that of silicone hydrogels alone. It’s an developments from SynergEyes. These include: improvement over the GP lenses alone of a decade ago. The system using both high-Dk GP lenses and silicone hydrogel 1. Increased center thickness of the GP lens portion in the lenses also offers significantly more oxygen transmission than company’s Enhanced Profile (EP) version, available in each custom hydrogel toric lenses or available hybrid lenses other of the different SynergEyes lens designs. An EP lens provides options you might consider to provide a similar comfort and a center thickness that is 0.09mm thicker than that of the fitting benefit. While slightly inconvenient with two lenses and standard lens for each design. This is helpful in reducing some care systems per eye, patients have responded very positively flexure in highly astigmatic corneas. Due to the effect of the to piggyback contact lenses due to the comfort and wearing soft lens skirt, the increased GP thickness does not significantly time benefits. alter the fit, cent ration or comfort of the EP lenses. The system consists of a rigid lens fitted on top of a soft lens. The aim is to maintain the same level of visual acuity as with 2. Introduction of the Clear-Kone design for keratoconus, a single lens. which is designed to better vault advanced keratoconic The RGP lens should be fitted first. Good centration is corneas without bearing. This new option may allow better important and a slightly larger area of apical touch is usually success in some of the most challenging cases. acceptable as the RGP lens will be cushioned by a soft lens. A silicone hydro gel soft lens should be used where possible, 3 FDA approvals to begin clinical trials of hybrid contact with good movement and coverage/ cent ration as in a normal lenses utilizing a soft silicone hydrogel material for the skirt soft lens fitting. Caring for the two lenses can be difficult portion of the lens. SynergEyes hopes to launch products

Vol. 21, No. 1, July-September, 2010 DJO 41 Delhi Journal of Ophthalmology Contact Lens Fitting in Keratoconus with this new skirt material in the first half of 2010. Limiting References: limbal encroachment or neovascularization is very important 1. Rabinowitz YS, Nesburn AB, McDonnell PJ. Videokeratography in post-surgical cases or in eyes that may eventually need of the fellow eye corneal surgery. in unilateral keratoconus. Ophthalmology 1993; 100:181-186. The main disadvantages of hybrid lenses are frequent 2. Buxton JN. Contact lenses in keratoconus. Contact intraocular breakage of the lens, giant papillary conjunctivitis and lens Med J. 1978; 4:74 peripheral corneal neovascularisation.It should be noted that 3. Rabinowitz YS, Garbus JJ, Garbus C, et al. Contact lens the Soft perm lens was not designed for keratoconus, but for selection for keratoconus a normal cornea. As it provides the comfort of a soft lens and using a computer-assisted videophotokeratoscope. CLAO J. visual acuity of a rigid lens it has been adopted by keratoconic 1991; 17:88-93 patients who inevitably over-wear these lenses and end up 4. Rosenthal P,Cotter JM. Clinical performance of a spline-based with complications. apical vaulting keratoconus corneal contact lens design. CLAO J. Scleral lenses 1995; 21:42-46 Scleral lenses play a very significant role incases of advanced 5. Raber IM. Use of CAB Soper Cone contact lenses in keratoconus where corneal lenses do not work and corneal keratoconus. CLAO J. 1983 surgery is contra-indicated. Scleral lenses completely Jul-Sep; 9(3):237-240. neutralize any corneal irregularity and can help patients 6. Betts AM, Mitchell GL, Zadnik K. Visual performance and maintain a normal quality of life. A PMMA lens can be used comfort with the Rose K in cases of Scleral toricity. lens for keratoconus. Optom Vis Sci. 2002 Aug;79(8):493-501. PMMA scleral lenses are made by the impression method. 7. Jain AK, Sukhija J. Rose-K contact lens for keratoconus. Indian This practice is confined to the HES. An impression is taken J Ophthalmol. of the cornea, generally with alginate material (ortho print) 2007 Mar-Apr;55(2):121-125. and a clear shell is made frompoly-methyl metha cry late 8. Ozkurt YB, Sengor T, Kurna S, Evciman T, Acikgoz S, Haboğlu material. Optic curves are ground on to the shell. This can be M, Aki S. Rose K done in-house or the shell can be sent to Cantor & Nissel. The contact lens fitting for keratoconus. Int Ophthalmol. 2008 Dec; shell is fenestrated, adjusted, and ground until a desirable fit 28 (6):395-398. is obtained. Once an acceptable fit is obtained the lens can be 9. Yeung K, Egbahli F, Weissman BA. Clinical experience with sent for working to the required power. (Figure 10) piggyback contact lens systems on keratoconic eyes. J Am Optom Assoc. 1995; 66:539-543

Figure 10 Scleral Contact Lens

Advantages • Easy to use and remove • Any type of irregularity is removed • Easy to store and dry • Long life

Disadvantages • Much chair time is needed • A very specialized fitting technique

DJO Vol. 21, No. 1, July-September, 2010 42 Delhi Journal of Ophthalmology

Preferred Pratice Patterns Cataract Surgery in Fuch’s Corneal Dystrophy Shikha Gupta, Varun Gogia, Ritika Sachdev, Rajesh Sinha, Namrata Sharma Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi

Fuch’s endothelial corneal dystrophy is a progressive disorder of the endothelium wherein a gradual decline of functional endothelial cells over the years, manifesting with visual disturbance in late adulthood. The visual loss in these patients can be either due to cataract or due to corneal dystrophy itself. Determining which patient requires cataract surgery is imperative for an ophthalmologist to visually rehabilitate the patient. However cataract surgery could potentially decompensate an already diseased cornea with borderline endothelial cell reserve. This review will help the surgeons in proper evaluation and practice during surgery to provide the patient with maximal visual gain. Fuch’s dystrophy and cataract surgery the FCD as corneal edema increases at night while sleeping. Fuch’s corneal dystrophy (FCD) is bilateral progressive Several hours may elapse into the morning before vision endothelial dystrophy usually presenting with visual improves and documenting what time the vision clears in a disturbance at an age of 50- 60 years. Abnormal production of particular patient over time can become a measure of how collagenous material by the affected endothelial cells causes the corneal disease is progressing (stages 2 and 3). In the marked thickening of Descemet’s membrane, which becomes fourth stage, vision reduces to hand motions due to growth studded with excrescences (guttae). Eventually there is a of avascular subepithelial connective tissue and peripheral gradual decline in the number of functional ATP dependent corneal vascularisation[1]. Na K pumps resulting in corneal edema[1]. FCD is associated with a low endothelial cell count as Pain well as abnormal cell morphology (pleomorphism and It is usually seen during the third stage which is characterised polymegathism) (Figure 1), where in an unplanned cataract by epithelial edema. As epithelial cysts coalesce to form large surgery has the potential to cause early decompensation of bullae, their rupture results in severe pain and predisposition cornea incurred due to mechanical, hydrodynamic, thermal, to ocular infection[1]. chemical, or possibly free radical damage secondary to high- frequency ultrasound used during phacoemulsification[3]. Preoperative considerations: Patients with decreased vision due to FCD and cataract These include can present with a number of challenges to determine the a) Patients with increasing age, history of previous ocular best surgical option for restoring sight because intraocular surgery, history of ocular trauma, ocular infection, angle surgery may accelerate corneal endothelial cell loss. Finding closure glaucoma, and pseudoexfoliation syndrome, an unresolving corneal edema in an eye with an uneventful diabetes of more than 10 year duration and long-term phacoemulsification surgery is every surgeon’s nightmare. contact lens use possess a poor endothelial cell density Therefore proper preoperative assessment and thorough reserve or abnormal cell morphology[5]. planning is a prerequisite before actual surgery in these b) If the patient has glaucoma and is on topical carbonic patients and one must evaluate, whether cataract surgery alone anhydrase inhibitors, it is reasonable to stop their use or cataract surgery combined with full thickness or lamellar as they interfere with fluid pumping ability of the keratoplasty (triple procedure) is required for an individual. endothelium by blocking the active sodium potassium pump thus worsening the edema in such patients[5]. Symptomatology c) Consider for endothelial keratoplasty with Glare phacoemulsification if preoperative corneal edema or The debilitating symptom of glare due to confluent guttae central corneal thickness (CCT) is more than 650µ.These with pigment can occur even when there is little to no stromal are the cases which are most likely to decompensate after or epithelial edema and pachymetry is relatively normal (stage an uneventful cataract surgery[3]. 1) or may also occur secondary to stromal edema (stage 2)[4]. d) Werblin had found 9% endothelial cell loss at 1 year after phacoemulsification with posterior chamber lens Visual loss insertion, with 11.5% loss at 3 years[2]. However, in the This can result from the cataract, the dystrophy, or both. presence of a hard nucleus (LOCS grade 4 and above) Vision which is worse in the morning is likely attributable to or a low endothelial cell count (ECC) of less than 1500/

Vol. 21, No. 1, July-September, 2010 DJO 43 Delhi Journal of Ophthalmology Cataract Surgery in Fuch’s Corneal Dystrophy mm2, conventional cataract surgery like extracapsular cataract extraction (ECCE) or small incision cataract Viscoelastic considerations surgery (SICS) is preferable [6]. No significant difference ‘Arshinoff soft shell’ technique should be considered in postoperative ECC exists when comparing ECCE with whenever possible wherein a viscodispersive agent preferably intracapsular cataract extraction (ICCE) [2]. HPMC or chondroitin sulfate is injected intracamerally followed by the use of a viscocohesive agent like 1.4% sodium Operative considerations hyaluronate which thereby pushes the viscodispersive against Anesthesia the endothelium providing protection during the surgery. It is preferable to give peribulbar block. Avoid intracameral Sodium hyaluronate 2.3% used alone, being a viscoadaptive anesthesia as these drugs can cause endothelial toxicity. If agent serves the purpose of both the agents, and hence can required, preservative free 1% lignocaine can be used instead also be used[5]. of 5- 10% lignocaine, 0.5% bupivacaine or 0.5% proparacaine all of which are endothelio-toxic[2]. Phaco-technique During phacoemulsification, phaco parameters in cases of Drugs compromised endothelium should include low phaco power Povidone iodine 5- 10% when used for sterilisation should be along with high vacuum, or restricted use of energy by thoroughly washed after use as it leads to significant corneal using fractions of second pulses or bursts, and millisecond- toxicity if it accidentally penetrates the anterior chamber. level microburst. The OZil Torsional system (Infiniti, Alcon, Acetylcholine 1% is toxic; instead use of 0.001% carbachol is Fort Worth, TX) is a hardware and software upgrade which recommended if intraoperative miosis is desired. Vancomycin includes a dedicated handpiece that produces side-to-side in a concentration of >1 mg/ ml is not considered safe for the rotary oscillations of the phaco tip[10,11]. Comparing with the endothelium[2]. jackhammer motion in conventional longitudinal phaco, the improved OZil Torsional oscillation sheers the lens material Sterilization with virtually no repulsion, thereby dramatically reduces Avoid use of instruments dipped in 2 % glutaraldehyde. phaco energy required for lens removal without compromising Aqueous substitutes: Among the aqueous substitutes used efficiency. While in Torsional phaco, although the tip moves intraoperatively, 2.3% or 1.4% sodium hyaluronate and a at a lower frequency of 32 kHz than the 40 kHz in traditional combination of sodium hyaluronate and chondroitin sulfate phaco, the side-to-side tip movement sheers the lens material should be preferred. Avoid air injection and use of trypan blue with no repellent force, and cuts with both direction of the tip for capsular staining. Amongst the irrigating fluids, balanced movement, thus significantly improving emulsity efficiency. salt solution (BSS) Plus (containing reduced glutathione, Other modifications include include non ultrasonic energy bicorbonate and dextrose) is better than BSS alone followed such as sonic frequencies, NeoSoniX-generated tip rotation, by Ringer Lactate and least protection being offered by and pulse water-jet technology[10,11]. Use of specific physiological saline solution which leads to endothelial cell chopping techniques with endocapsular emulsification of damage and corneal swelling[2,5]. nucleus is recommended. Entering the anterior chamber Role of preservatives: Epinephrine containing sodium repeatedly with phaco tip or second instruments damages the bisulphate as a preservative leads to increased cell endothelium; therefore minimum manipulation is advisable. loss, hence use of preservative free epinephrine is Entry and exit should always be made in a formed anterior recommended. Hydroxypropylmethylcellulose (HPMC) chamber to avoid chamber fluctuations during the surgery; containing benzalkonium chloride is known to cause striate therefore, it is advisable to inject viscoelastic through side keratopathy[2]. port before taking out the phaco probe. In a recent study by Liu et al, microincision cataract surgery was not superior to Incisions a standard phacoemulsification in terms of endothelial cell Limbal or clear corneal incision leads to an increased loss and produced statiscally insignificant greater cell loss but endothelial cell loss. Amount of cell loss is directly allowed excellent visual results in his series of patients[12]. proportional to the length of incision. Therefore ECCE or ICCE incision leads to a greater amount of cell loss as Intraocular lens (IOL) compared to phacoemulsification wound. Superior incision During IOL insertion, coat the anterior surface of the lens with has been found to be better than temporal incisions in terms a viscodispersive agent. Hydrogel / acrylic/ silicon IOLs have of cell loss. Scleral tunnel incisions in comparison to clear been found to be safer for endothelium than PMMA IOLs. corneal incisions cause less endothelial damage[2]. Sleeve Furthermore, posterior chamber (PC) IOLs lead to lesser cell should be appropriate size and should not fit too tight in the loss when compared to anterior chamber (AC) IOLs. IOLs wound such that fluid should pass out easily, thus minimising with surface passivisation e.g. Teflon coated or heparin surface the thermal damage to the cornea[5].

DJO Vol. 21, No. 1, July-September, 2010 44 Cataract Surgery in Fuch’s Corneal Dystrophy Delhi Journal of Ophthalmology modified are strongly recommended. Corneal endothelial References cell loss over time has been found to be more in aphakes as 1. Krachmer JH, Mannis MJ, Holland EJ. Cornea. Fundamentals, compared to pseudophakes[2]. diagnoses and management. 2005, Second ed, Vol 1, Chapter 101. 2. Smolin’s and Thoft’s. The Cornea. Scientific Foundation and Clinical Practice. 2005, 4 ed, Part B, Chapter 47. 3. Eghrari AO, Daoud YJ, Gottsch JD. Cataract surgery in Fuchs corneal dystrophy. Curr Opin Ophthalmol 2010, 21:15–19. 4. Seitzman GD. Cataract surgery in Fuchs’ dystrophy. Curr Opin Ophthalmol 2005; 16:241-245. 5. Pineda R, Espaillat A, Perez VL, Rowe SG. The complicated cataract. The MEEI phacoemulsification Practice Handbook. 2001. 6. Bourne RR, Minassian DC, Dart JK, et al. Effect of cataract surgery on the corneal endothelium: modern Postoperative considerations: phacoemulsification compared with extracapsular cataract Certain postoperative factors like shallow anterior chamber, surgery. Ophthalmology 2004 Apr;111(4):679-85. peripheral anterior synechiae, postoperative inflammation, 7. Terry MA, Shamie N, Chen ES, et al. Endothelial keratoplasty raised intraocular pressure, vitreous corneal touch, for Fuchs’ dystrophy with cataract: complications and clinical pseudophacodonesis, IOL corneal touch all predispose to results with the new triple procedure. Ophthalmology 2009 increased cell loss over time and thus should be carefully Apr; 116(4):631-9. looked for in all such patients[5]. 8. Covert DJ, Koenig SB. New triple procedure: Descemet’s stripping and automated endothelial keratoplasty combined Cataract surgery with endothelial keratoplasty with phacoemulsification and intraocular lens implantation. DSAEK when done together with phacoemulsification and Ophthalmology 2007 Jul; 114(7):1272-7. IOL implantation is known as triple procedure. In patients 9. Lombardo M, Terry MA, Lombardo G, et al. Analysis of with FCD the view through the cornea may be acceptable to posterior donor corneal parameters 1 year after Descemet allow for a closed cataract extraction by phacoemulsification stripping automated endothelial keratoplasty (DSAEK) followed by DSAEK. When the view is obscured by significant triple procedure. Graefes Arch Clin Exp Ophthalmol. 2010 stromal and epithelial edema, the DSAEK may be performed Mar;248(3):421-7. first, waiting for the edema and view to improve followed 10. Rekas M, Montés-Micó R, Krix-Jachym K, et al. Comparison by cataract surgery. The use of viscoelastic material during of torsional and longitudinal modes using phacoemulsification cataract surgery has not been found to adversely affect graft parameters. J Cataract Refract Surg. 2009 Oct;35(10):1719- adherence after DSAEK. Performing a smaller capsulorhexis, 24. using a larger lens diameter, and constricting the pupil with 11. Miyoshi T, Yoshida H. Emulsification action of longitudinal miotics after intraocular lens implantation allows for the and torsional ultrasound tips and the effect on treatment of the insertion of the graft without increasing endothelial loss. nucleus during phacoemulsification. J Cataract Refract Surg. In a prospective study on triple procedure in 89 eyes by Terry 2010 Jul; 36(7):1201-6. et al[7], 32% cell loss was found in donor corneas at the end 12. Wang Y, Xia Y, Liu X, et al. Comparison of bimanual and of 12 months representing a mean endothelial cell density of micro-coaxial phacoemulsification with torsional ultrasound. 1979 cells/mm[2]. Acta Ophthalmol. 2010 Feb 16.

Conclusion Therefore patients presenting with FCD and cataract pose a special challenge to the ophthalmologist and should be dealt with a proper preoperative assessment and postoperative follow up to give the possible & desired visual outcomes without causing any inadvertent complications.

Vol. 21, No. 1, July-September, 2010 DJO 45 Delhi Journal of Ophthalmology

Cases Reports Morning Glory Syndrome Rashida Shabbir Tankiwala, Memuna Bahadur Dr Babasaheb Ambedkar Memorial Hospital, Central Railway, Mumbai

Morning glory syndrome is a congenital optic disc anomaly described by Kindler1 in 1970. It is a congenital funnel- shaped excavation of the posterior fundus that incorporates the optic disc, resembling the morning glory flower. Ocular complications may include strabismus, reduced visual acuity and retinal detachment and it may have systemic associations as in Aicardi’s syndrome. A patient with monocular morning glory syndrome and reduced visual acuity is reported. The pattern reversal visually evoked potential was reduced.

Introduction Macula appeared normal, no evidence of retinal detachment, We report this case of unilateral Morning Glory Syndrome holes or degenerative changes seen in the peripheral retina. with no associated systemic abnormalities which is rare in Left eye showed vision of 6/6, unaided with normal ocular occurrence, although, the exact incidence of its occurrence is examination. unknown. Special Investigations Case Report • Colour vision on Ishihara charts was normal. A 14 yr old girl was brought by her mother with complaints • Ocular ultrasonography confirmed the presence of of headache. On inquiry, her birth history and family history marked irregular deepening of the optic nerve head on the were unremarkable. She was found on examination to have right side with the presence of a density over the disc. No less vision in her right eye. evidence of retinal detachment and normal retrobulbar Detailed examination: Her facial features appeared normal. region was noted. Left eye revealed normal ultrasonic Ocular adnexa including bony orbital rim and soft tissue examination [Figure 2]. were normal. She was orthophoric for distance and near. Best corrected visual acuity with -2.75 DS was 6/18. Slit lamp examination of the anterior segment was within normal limits. Pupils were round and regular and the reflexes were normal to light and accommodation in both eyes with no relative afferent pupil defect. Intraocular pressure (IOP) checked digitally was normal in both eyes. Fundus examination revealed the presence of a funnel-shaped excavation containing an enlarged, somewhat indistinct, optic disc surrounded by a wide annulus of chorioretinal Figure 2 Ultrasonic imaging of the right eye done using 20MHz pigmentary disturbance. A white tuft of glial tissue was seen ophthalmic probe with a synchronized vector A-scan is showing an over the central portion of the disc and blood vessels seen irregular optic disc with a high echo density seen over it. emanating radially from the disc margin [Figure 1]. • Visually evoked potential revealed bilateral normal latency of P100. Marked reduction in amplitude on the right side suggestive of axonopathy. The left amplitude was normal.

ISCEV STANDARD PATTERN - REVERSAL VEP Right Eye Left Eye Normal P 100 Implict Time 106 103 90-110 (ms) Figure 1 The right eye of the patient with morning glory syndrome. Amplitute 1.7 6.5 4-14 (uv) The optic disc is enlarged and excavated. A tuft of whitish tissue is present in the centre of the disc. There are peripapillary pigmentary The P 100 difference between both the eyes is 3 ms (normal > 10 ms) changes and the retinal vessels emerge from under the central tissue There is normal latency of conduction in both the eyes eye. and run a straight course toward the periphery of the retina The amplitude of P 100 is normal on the left side.

DJO Vol. 21, No. 1, July-September, 2010 46 Morning Glory Syndrome Delhi Journal of Ophthalmology Congenital forebrain abnormalities including basal encephalocoele and endocrine disturbances[4,6], midline facial defects including hypertelorism, cleft lip or cleft palate,renal hypoplasia[8]. Only rarely is the MGS part of a multisystem genetic condition. One example is Aicardi’s syndrome[9], which is an X-linked dominantly inherited disorder, characterized by severe epilepsy, agenesis of the corpus callosum, typical chorioretinal lacunae and learning disabilities. The chorioretinal changes may include optic disc coloboma (50 per cent of cases), morning glory syndrome, optic nerve hypoplasia, iris and choroidal colobomata and retinal detachment. Second example is Moyamoya disease, an association between morning glory disk anomaly (MGDA) and intracranial vascular anomalies[7].

References Figure 3 Humphrey central 30-2 Threshold showing enlargement 1. Kindler P. Morning glory syndrome: Unusual congenital of the blind spot in the right eye. optic disk anomaly. Am J Ophthalmol 1970; 69: 376-384. • Using the Humphrey’s Field Analyser, 30-2 threshold 2. Rufina Tin-yan Chan, Henry Ho-lung Chan,H Barry showed [Figure 3]. Collin (Clin Exp Optom 2002; 85: 6: 383–388) 3. G cennamo, G liguori, A pezone. et all ‘British Journal of Right eye: Enlargement of the blind spot Ophthalmology, 1989, 73, 684-686 Left eye: Fields to be within the normal limits 4. Kaori Kinoshita1), Itsuro Kazukawa1), Yuji Hashimoto1. • There was no evidence of multi-organ involvement, et al. Clinical Pediatric Endocrinology Vol. 14 (2005), which had been investigated. Supplement24 pp.S24_97-S24_100 5. K. Rubinstein, British Journal of Ophthalmology Discussion: 2003;87:363-365 Vision is usually poor in patients with morning glory 6. M. Hope-Ross a; S. S. Johnston. et al. Ophthalmic syndrome[8]. This vision loss may be due to the presence Genetics, Volume 11, Issue 2 June 1990 , pages 147 – 153 of retinal abnormalities in the macula[12], or amblyopia 7. P. Lenhart, S. Lambert, N . Newman. et al. American secondary to anisometropia or strabismus[1], or due to the Journal of Ophthalmology, 142, (4), 644 - 650. developmental defect of the optic nerve head. 8. N Deb, R Das, IS Roy, Indian journal of Ophthalmology Usually unilateral[11], but a rare case of bilateral MGS have Year : 2003 | Volume : 51 | Issue : 2 | Page : 182-183 been reported[8]. 9. King AM, Bowen DI, Goulding P, Doran RML. Aicardi Other ocular findings commonly observed in the affected syndrome. Br J Ophthalmol 1998; 82: 457. eye with MGS include: strabismus[1], an afferent pupillary 10. Jackson W, Freed S. Ocular and systemic abnormalities defect[10], visual field defects consisting of blind spot associated with morning glory syndrome. Ophthalmic enlargement and/or dense central scotomata[13], mild Paediatr Genet 1985; 5: 111-115. to moderate myopia[1]. Ocular associations found in the 11. Pedler C. Unusual coloboma of the optic nerve entrance. affected eye with morning glory disc anomaly may include: Br J Ophthalmol 1961; 45: 803-807. non-rhegmatogenous retinal detachment[14], the presence 12. Debney S, Vingrys AJ. Case report: The morning glory of marked persistent hyperplastic primary vitreous[3], lens syndrome. Clin Exp Optom 1990; 73: 31-35. coloboma[3], ciliary body cyst, congenital cataract, lid 13. Giuffre G. Morning glory syndrome: Clinical and electro- haemangioma, vitreous cyst and preretinal gliosis[12]. functional study of three cases. Br J Ophthalmol 1986; Morning glory disc may be mistaken for disc swelling in the 70: 229-236 affected eye. It needs to be carefully evaluated to prevent 14. Cheng-Lien Ho1 and Li-Chen Wei1, International misdiagnosis[5]. Ophthalmology. 24 (1) ; 2001. Ocular abnormalities have also been reported in the fellow eye. These include: microphthalmos, anterior chamber cleavage syndrome, microcornea and Duane’s retraction syndrome[12]. Systemic associations in morning glory syndrome have been well-documented.

Vol. 21, No. 1, July-September, 2010 DJO 47 Delhi Journal of Ophthalmology

Cases Reports An Unusual Case of Metastatic Tubercular Endophthalmitis Meenakshi Kabra, Sarita Beri, Rajiv Garg, Pamela d’souza, Rajesh Jain, Anita Nangia, Sanjay Kumar Mishra Lady Hardinge Medical College, Shahid Bhagat Singh Marg, New Delhi -110001

Tuberculosis is prevalent in developing countries like India, however ocular tuberculosis is a diagnosis of exclusion. This unusual case is of an 18 year old patient of pulmonary Kochs who presented to the out patient department of our hospital with unilateral acute pain in right eye with the marked diminuition of vision of 3 days duration. On Examination his vision in Right eye was PL-negative. This was preceded by gradual painful loss of vision in the right eye for the past two months duration for which he was already taking medication. He was diagnosed as a case of endophthalmitis with secondary glaucoma and ciliary and intercalary staphyloma in R/E. His eye was eviscerated. On the basis of clinical presentation, radiological and histopathological findings diagnosis of Tuberculous endophthalmitis was therefore confirmed. Case report and hot fomentation in right eye. His systemic antitubercular The prevalence of tuberculosis in India is 30% and the annual treatment was continued. incidence of infection is 1-2%. India forms the highest TB Blood profile was Hb 12gm, TLC -5800/mm3, ESR was burden country in the world, with 1.8 million cases occurring 83 in 1st hour by Westergens method while random blood annually[1]. An 18 year old male patient presented to the out sugar, liver and kidney function tests were with in normal patient department with unilateral acute pain in right eye with limits. Chest x-ray showed, resolving right upper lobe and the marked diminuition of vision of 3 days duration. There left upper and lower lobe parenchymal infiltration suggestive was history of dull ocular pain with gradual diminuition of pulmonary Kochs responding to antitubercular treatment. of vision in the right eye for the past two months. He was Evisceration of right eye was done and the Ocular tissue sent diagnosed as panuveitis at another centre and was started on for histopathological examination. The histopathological topical steroid antibiotic combination, atropine and timolol. report showed focal areas of caseous necrosis (Figure-1A) He was on regular treatment for his eye condition, without any with multiple epitheloid cell granulomas and Langhans giant improvement, for the past two months. cells. Ziehl Neilsen stain showed acid fast bacilli (Figure- He presented to us with severe ocular pain of 3 days duration. 1B). Diagnosis of Tuberculous panuveitis leading on to He gave a past history of pulmonary kochs for which he was endophthalmitis was therefore confirmed. already on regular four drug antitubercular treatment for the past four months. On systemic examination of the patient, Discussion chest examination revealed bilateral crepts. On ophthalmic Mycobacterium tuberculosis, the etiologic agent of examination his vision in the Right eye was PL-negative and tuberculosis can cause infection in many organs including the in the left eye was 6/6. Right eye had ciliary congestion, with eye. Ocular tuberculosis can involve any part of the eye and a superior ciliary and intercalary staphyloma was present, can occur with or without evidence of a systemic focus of corneal haze, hyphema and exudates in the anterior chamber tuberculosis[2]. Digital tension was high. The details of iris and pupil were The patient probably developed tuberculous panuveitis not clear. The left eye on examination did not show any other leading to endophthalmitis. Only sporadic cases of metastatic abnormality except absent consensual pupillary reaction. tubercular endophthalmitis have been reported. Metastatic USG B scan of the right eye showed low to moderate endophthalmitis is twice as common in the right eye as in amplitude spikes suggestive of vitreous exudates. The intra the left, probably because of the shorter, more direct route ocular pressure was 29 in the right eye and 11 in left eye. of arterial blood flow from the internal carotid artery to the A provisional diagnosis of endophthalmitis with secondary eye on the right side[2,3]. The visual prognosis following glaucoma and ciliary and intercalary staphyloma of right diffuse posterior metastatic bacterial endophthalmitis is very eye was made and the treatment in the form of intravenous poor. Removal of the affected painful blind is the treatment Omnatax and Amikacin, oral acetazolamide, analgesics was of choice. There is no case in the literature where visual started. Topically he was put on gatifloxacin-dexamethasone recovery is seen after this type of infection regardless of type combination, antiglaucoma medication, lubricating agent of therapy[2].

DJO Vol. 21, No. 1, July-September, 2010 48 An Unusual Case of Metastatic Tubercular Endophthalmitis Delhi Journal of Ophthalmology There are two possible contributory factors responsible for References endophthalmitis in this patient. Firstly, from the pulmonary 1. World Health Organisation. Report 2008. Global Tuberculosis infection, a septic embolus occludes the central retinal artery Control Survillence. Planning, Financing. Geneva 2008. and embolic fragments are then disseminated peripherally, so 2. Thompson MJ, Albert DM. Ocular tuberculosis. Arch ischemia as well as infection may add to the poor prognosis. Ophthalmol 2005; 123:844-49. [2]. Secondly, In patients with bacteremia the blood-borne 3. Greenwald MJ, Wohl LG, Sell CH. Metastatic bacterial organisms permeate the blood-ocular barrier either by direct endophthalmitis: a contemporary reappraisal. Surv Ophthalmol. invasion or by changes in vascular endothelium caused by 1986;31:81. substrates released during infection. Destruction of intraocular 4. Helm CJ, Holland GN. Ocular tuberculosis. Surv Ophthalmol tissues may be due to direct invasion by the organism[2-4]. .1993; 38:229-56. 5. Sheu SJ, Shyu JS, Chen LM, Chen YY, Chirn SC, Wang JS. Ocular manifestations of tuberculosis. Ophthalmology. 2001;108:1580-85. 6. Raina UK, Tuli D, Arora R, Mehta DK, Taneja M. Tubercular endophthalmitis simulating retinoblastoma. Am J Ophthalmol. 2000;130:843-45. 7. Kratka WH. Isoniazid and ocular tuberculosis: an evaluation of experimental and clinical studies. Arch Ophthalmol. 1955;54:330-44.

Figure 1A section shows epitheloid cell granuloma with Langhans giant cell. Part of pigment layer is seen in periphery of granuloma.Haematoxylin and Eosin stain (400x magnification)

Figure 1B Ziehl Neelson stain (1000x magnification) arrow showing Beaded long M.tuberculosis identified.

Conclusion All patients of systemic tuberculosis presenting with ocular involvement, metastasis as an etiology should be considered as one of the differential diagnosis. Prompt diagnosis and appropriate management can prevent blindness.

Vol. 21, No. 1, July-September, 2010 DJO 49 Delhi Journal of Ophthalmology

Cases Reports Atypical Presentation of Conjunctival Neoplasia

Vandana Jain, S.Gupta, R.Matai, R.K. Srivastava ESI Hospital, Indore This communication delineates variable presentations in two cases of conjunctival neoplasia , histopathology of the same was suggestive of squamous neoplasia.Though incidence of conjunctival neoplasia is low, any case presenting as non resolving chronic conjunctivitis or recurrent pterigium should undergo cytological examination to rule out malignancy.

Case 1 This patient was treated with superficial keratectomy with A 46 year old male presented with redness , photophobia, Amniotic membrane transplant & topical Cyclosporin A blurred vision and membranous growth in the right eye drops. since 1 year. Subsequently he had progressive diminution of vision. He gave history of blunt trauma to affected eye Case 2 preceding the redness (by a wire) for which he was treated A 60 year old female presented with painless conjunctival conservatively elsewhere. On local examination of the growth of right eye of 21/2 months duration. Local right eye invasion of conjunctival tissue over cornea was examination revealed a sessile firm growth which had no present for 360 degree with papillary process extending fluctuation, pulsation and was nontender. Dimensions of the up to optical zone. Grossly it had gelatinous appearance with growth were 6mm X 4 mm. It was located at temporal limbus superficial vascularisation. (Figure1) with a 2 mm corneal invasion. (Figure 3)

Visual acuity was 6/12 with normal fundus examination His best corrected visual acuity was 2/60. Fundus examination bilaterally. Systemic examination was non contributory revealed faint red glow. Systemic examination was non .A provisional diagnosis of conjunctival melanoma with contributory. Serology for HIV was negative. A provisional differential diagnosis of sessile papilloma and squamous cell diagnosis of keratitis (D/d : Chronic conjunctivitis or Limbal carcinoma were considered. Subsequently total excisional stem cell deficiency) was made and he was initiated on biopsy of the mass was done with 2mm clear margins of topical antibiotic-steroid combination. As he didnot respond, conjunctiva & cornea. conjunctival scraping was done. Histopathology of the same Histopathology was suggestive of well differentiated revealed plenty of atypical cells and dysplasia consistent squamous cell carcinoma with stromal invasion. (Figure 4) with moderate grade of squamous intra epithelial neoplasia. (Figure 2).

DJO Vol. 21, No. 1, July-September, 2010 50 Atypical Presentation of Conjunctival Neoplasia Delhi Journal of Ophthalmology Discussion: References We present atypical presentation of conjunctival neoplasia 1. Intraepithelial and Invasive Squamous cell carcinoma observed in 2 cases. In case1 360 degree corneal involvement of the conjunctiva: analysis of 60 cases. Murat Tunc was noted as opposed to a single quadrant location which is ,Devron H Char,Brooks Crawford,TheodoreMiller.Br J more commonly described in literature. In addition the patient Ophtalmol 1999:83:98-103 was young male which is extremely uncommon. The second 2. Lauer SA , MalterJS ,Meier R.Human Papilloma case was an elderly female which is the more common age Virustype – 18 in conjunctival neoplasia. Am J of presentation. In both the cases there were no predisposing Ophthalmol 1990;110:23-7 factors. Both the cases continue to be on regular follow up as 3. Kearsley JH, Fitchew RS, Taylor RGS .Adjunctive recurrences are known and need to be picked up early. Few radiotherapy with Strontium 90 in the treatment of studies have given trial of 5-Flurouracil (5 FU) and/or 0.4% conjunctival squamous cell carcinoma. Int J Radiat oncol Mitomycin-C (MMC) and/or Cyclosporin A and/or Interferon Biol Phys 1987; 14: 435-43. with non conclusive results. 4. Bajaj MS , PandaA, Pushker N , Balsubramanya R .Amniotic membrane Transplantation .Br J Ophthalmol Conclusion 2002; 86: 1460 Though conjunctival neoplasias are uncommon, in cases of 5. Char DH. Conjunctival malignancies. In: Char DH. recurrent pterygium or chronic conjunctivitis (> 3 months) Clinical Ocular Oncology. 2nded.Philadelphia: cytological examination is must for early diagnosis. A high Lippincott-Raven index of suspicion is must to pick up these cases early and 6. Tabin G, Levin S, Snibson G et al. Late recurrences prompt and complete excision with a 2mm disease free margin and the necessity for longterm followup in corneal & may help in complete resolution of the disease. Additional conjunctival Intraepithelial neoplasia . Ophthalmology topical treatment has been tried in with unclear benefit. Close 1997.104: 485-92. follow up of all cases is necessary for early identification of recurrences.

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Cases Reports Isolated Strabismus as a Presenting Feature of Large Pituitary Macroadenoma Kamaljeet Singh, Prateek Gujar, Nida Usmani, Santosh Suman Department of Ophthalmology, Moti Lal Nehru Medical College, Allahabad

Pituitary adenomas usually present with visual field defects and headache. Large tumors may cause diplopia and ophthalmoparesis by cranial nerve compression in cavernous sinus. However, all such cases almost always have poor visual acuity also. We present a rare case of a 35-year-old male with a large pituitary adenoma who presented with headache, ptosis and diplopia but had normal vision in both eyes. He had supero-temporal quadrantic visual field defect in the right eye and an atypical visual field defect involving the temporal quadrants in the left eye. The relevant literature is reviewed in the discussion.

Introduction paresis of right lateral rectus and left superior, inferior rectus Pituitary tumours comprise 12 - 15 % of all intracranial and inferior oblique muscles. Fundus examination was within tumours. A majority of these are histologically benign [1]. normal limits. Physical examination revealed no definite Pituitary adenoma is a relatively common intracranial tumor clinical signs of endocrinological dysfunction. that presents to ophthalmologists with vision loss and field Computerized tomography showed a large expansile cuts due to its location below the optic chiasma [2, 3]. It can also present with hormonal disturbances- hyperpituitarism (functioning adenomas) or hypopituitarism (from compression of normal hypothalamic pituitary axis). Clinically, pituitary adenomas present as secretory or non-secretory tumours; visual manifestations are more common amongst non- functional adenomas [4]. Large tumor also compresses cranial nerves in the cavernous sinus. When it does so, some degree of vision loss/temporal field cut is almost always present. The prevalence of field defects in pituitary adenomas in general Figure 1- Figure A& B demonstrates the position of the eye in has been reported in various studies as 37 to 96 % [5]. primary gaze. Notable is the out and down looking left eye with It is distinctly uncommon for pituitary adenomas to present ptosis Figure C-H demonstrates the extraocular motility in the only with features of extraocular palsy in absence of different diagnostic position of gazes. Notable is the lack of vision loss [6,7,8]. We report a 34-year-old man with non- adduction and dextroelevation in the left eye (C,D,E). There is functioning pituitary macroadenoma who presented to us only limitation range of abduction in the right eye (D). with complaints of strabismus in absence of vision loss. intrasellar mass with small suprasellar and left parasellar Case report extension, with extension of mass in peripontine cistern. A 35 year old male came with the complains of headache On the left side it was indenting on pons and compressing for the past six months. Two months back he woke up one optic chiasma with possible invasion of left cavernous sinus morning and found he could not open his left eye. He also and internal carotid artery. We suspected it to be a pituitary suffered from double vision when he forced the left eye to macro adenoma. On magnetic resonance imaging (figure 2), open. On admission, visual acuity was 6/6 right eye and a large (41× 40× 38 mm) defined soft tissue lesion was seen 6/6 left eye. Vision was assessed using Snellen’s optotype expanding in sella turcica extending superiorly into supra- on self illuminated vision drum for distance. On testing the sellar cistern and compressing the optic chiasma , laterally near visual acuity patient had a vision of N/6. Neurological having left cavernous sinus invasion and posteriorly invading examination revealed complete left oculomotor nerve paresis the prepontine cistern. Levels of prolactin, cortisol and TSH and right abducens nerve paresis. Left eye on examination measured are normal. The visual field examination revealed revealed fixed abductant ocular position and absence of light a supero- temporal quadrant defect in the right eye and an reflex in the left eye. On testing the uniocular movements (in atypical defect involving the temporal quadrants in the left the four directions), in the left eye patient had restriction of eye. movement in adduction, elevation and depression. On testing We referred the patient to the department of neurosurgery for in the right eye, he had restriction of abduction. On testing gamma knife surgery for further management. The patient is for binocular movements (Figure 1), our findings revealed in our follow up.

DJO Vol. 21, No. 1, July-September, 2010 52 Isolated Strabismus as a Presenting Feature of Large Pituitary Macroadenoma Delhi Journal of Ophthalmology quantifying treatment results. Review of relevant literature reveals that it is distinctly rare for such tumors to attain large sizes, cause extraocular nerve palsy and yet have no symptom/sign of optic nerve compression (not even on fundus examination). Visual evoked response (VEP) may reveal subtle optic nerve compression features in these patients. Pituitary adenomas are responsible for a few cases of strabismus in adults, but these patients have other ocular/ neurological findings as well. Pituitary adenoma as a cause of isolated strabismus is very rare and ophthalmologists must be aware of this unusual presentation[6,7,8]. The other similar cases that have been reported are pituitary macroadenoma with isolated partial oculomotor nerve palsy in the setting of apoplexy [6], a case of haemorrhagic non-functioning pituitary adenoma presenting with abducens nerve palsy[8] and another case of acute third nerve palsy as the sole presenting sign [7]. Though these tumors are usually treated by neurosurgeons with micro neurosurgery or gamma knife, Figure 2- MRI (T1 and T2 sequence) of the brain showing a large they frequently present initially to ophthalmologists with defined soft tissue lesion expanding in sella turcica extending visual/ocular complaints. Ophthalmologists must therefore be superiorly into supra-sellar cistern and compressing the optic aware of all presentations of these tumors so that diagnosis is chiasma , laterally having left cavernous sinus invasion and made early and timely intervention/referral done. posteriorly invading the prepontine cistern References Discussion 1. Miller JD. Northfield’s surgery of the central nervous system. Pituitary adenomas constitute 10-15% of intracranial tumors 2nd edition. Oxford: Blackwell Scientific publications; 1987. [2]. Hormone secreting adenomas present early due to pp. 325-30. characteristic adenomas. Pituitary macroadenomas classically 2. Pollack IF. Brain tumors in children. N Eng J Med 1994; presents with asymmetrical bitemporal hemianopia, although 331:1500-1507. other patterns of visual dysfunction commonly occur 3. Black PM. Nonsecretory Pituitary Adenomas. In: Wilkins RH depending upon the size of tumor, direction of growth, and Rengachary SS, editors. Neurosurgery. 2nd Ed. New York: anatomic configuration of chiasma (prefixed, normal or McGraw-Hill; 1996. pp. 1321-1327. postfixed) and chronicity of the process. 4. Miller NR, Newman NJ, Biousse V, Kerrison JB, editors. Nonfunctioning adenomas are usually detected after they Walsh and Hoyt’s Clinical Neuro-ophthalmology. vol 3, 4th ed. attain large sizes, and present with vision loss (70-90 %), Baltimore: Williams and Wilkins; 1988. pp.1447. headache (40%), hormonal deficiency (15-40%), extraocular 5. Thomas R, Shenoy K, Seshadri Mandalam S, Muliyil J, Rao nerve involvement (1-4%), involvement of other cranial A, Paul P. Visual field defects in non functioning pituitary verves in the middle fossa skull base (2-4%) and seizures adenomas. Indian J Ophthalmol 2002;50:127-130. (4%) [2,9,10]. 6. Rossitch E Jr, Carrazana EJ, Black PM. Isolated oculomotor Diplopia or extraocular nerve weakness is rare, and should nerve palsy following apoplexy of a pituitary adenoma. J raise the possibility of metastatic tumor/pituitary apoplexy/ Neurosurg Sci. 1992;36(2):103-5. other diagnosis rather than routine non-secreting pituitary 7. Varma D, Tesha P, George N. Acute painful third nerve macroadenoma no matter how so ever large it may be [2,10]. Of palsy: the sole presenting sign of a pituitary adenoma: Eye patients presenting with extraocular nerve palsy, characteristic 2002;16(6):792-3. vision changes (bitemporal hemianopia/ blindness) is almost 8. Tanioka D, Abe T, Kunii N, Izumiyama H. A case of a always present, which help in clinical localization of the hemorrhagic non-functioning pituitary adenoma presenting lesion [2]. with abducens nerve palsy: No Shinkei Geka 2005;33(5):473-9. Non-functioning pituitary adenomas constitute 25 - 30% of all 9. Ebersold MJ, Quast LM, Laws ER Jr, Scheithauer B, Randall pituitary tumours and present with predominantly ophthalmic RV. Long term results in transsphenoidal removal of pituitary features; field defects being the most common [1]. Patients adenomas. J Neurosurg 1986;64:713-719. with pituitary macroadenomas may not have symptoms of 10. Nielsen EH, Lindholm J, Laurberg P, Bjerre P, Christiansen JS, visual disturbance, yet may have field defects consistent with Hagen C et al. Nonfunctioning pituitary adenoma: incidence, compression of visual pathways. It is therefore important causes of death and quality of life in relation to pituitary to perform field testing on patients with pituitary adenomas function. Pituitary. 2007;10:67-73. even if they have no visual complaints. Automated perimetry is a sensitive method for detecting visual field damage and

Vol. 21, No. 1, July-September, 2010 DJO 53 Delhi Journal of Ophthalmology

History of Ophthalmology Amblyopia A Historical Consideration Shibal Bhartiya, Sumita Sethi Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi

Figure 1: Albrecht von Graefe (1828-1870). “Even Grafe’s mistakes, and which mortal is free from mistakes, arose from his virtues…….his restlessness, which did not permit him to spend a single day away from the affected, was a direct consequence of his enthusiastic desire to heal.” (Hirschberg)

“To an unbiased observer the amblyopia treatment domain of its seat within the retino-cortical pathway. Von Graefe could appear to be a sort of privileged enclosure exempt from defined it as the condition in which the observer sees nothing, the obligation to apply the methodological rules universally and the patients very little. It is the functional counterpart adopted in clinical research concerning treatment of other of the neurophysiologic and neuroanatomic aberrations that diseases.” result from abnormal visual experiences in early childhood. Chavasses concept of amblyopia of arrest states that the visual Paliaga acuity remains at the level of development present at the time A historical consideration of amblyopia treatment would of onset of strabismus, which if persistent would result in undoubtedly lead the reader to conclude that this topic is superimposition of suppression amblyopia called amblyopia fraught with empirical innovation, yet lacking in critical of extinction[1]. evaluation, and appearing to be insufficiently evidence based. If the history of amblyopia were to be classified on Experimental Amblyopia this basis of particular study designs within its hierarchy, A major breakthrough in this field occurred in the early 1960’s defining the relative weight given to research findings when when Wiesel and Hubel published their now classic papers treatment decisions come to be made, then pictorially this on the effect of visual deprivation induced by lid suture in would resemble a somewhat bottom-heavy pyramid with visually immature kittens, on the physiology of the visual perhaps just a few systematic review at its apex.This article cortex, and on the histology of the lateral geniculate nucleus does not attempt to elucidate the extraordinary complexity (LGN).They established that unilateral lid closure during the of amblyopia, nor does it attempt to define the current scope first 12 weeks of life severely reduces the number of cortical of our knowledge. It just endeavours to summarize certain neurons which can be stimulated through the deprived eye major milestone as regard to development and treatment of as well as the number connected to both eyes (binocular amblyopia in animal models and in humans.Our understanding neurons) and functional anomalies are accompanied by of amblyopia is based on studies of experimentally produced histologic changes in the LGN layers receiving input from the strabismic, anisometropic and stimulus deprivation amblyopia deprived eye. Following the pioneering work of Hubel and in animal models. Extensive studies of clinical amblyopia Wiesel, and of Ikeda and Wright[2-9], there has been a virtual have been made using psychological, electrophysiologic and cascade of information on changes in the visual cortex and behavioral methods. The results of these investigations have the lateral geniculate nucleus (LGN) related to amblyopia, revealed a complex syndrome of sensory and motor anomalies, in both animal[10-14] and human models[15,16]. Due to the of which reduced visual activity is the most prominent part anatomic and functional differences in the organization of the of the overall disturbance clinically. Inherent limitations in visual system in the various species used, the data from later clinical research methods, however, have precluded precise studies on several different animals cannot be employed to analysis of the amblyogenic mechanisms and identification explain the mechanism of amblyopia in humans, strabismic or

DJO Vol. 21, No. 1, July-September, 2010 54 Amblyopia A Historical Consideration Delhi Journal of Ophthalmology anisometropic amblyopia. Paradoxically the world literature that do not implies that amblyopia does not improve of its is completely devoid of any histologic studies of the visual own accord. system of a human amblyope. However, indirect evidence Hubel and Wiesel coined the term ‘critical period’: a period emphasizes the similarity between clinical and experimental of time in early life, during which the visual system shows amblyopia, as the behavioral, cortical and histologic anomalies lability of deprivation and ability for reversal of the effect of can be atleast partially and in some cases completely reversed deprivation. Hardman Lea et al defined the sensitive period through enforced use of the amblyopic eye by suturing the as that passage of time during which the development of sound eye of kittens and infant monkeys within the sensitive the immature visual system may be altered by change in period. the quality, quantity or balance of the visual input via the 2 eyes[20,21]. Mechanism of Amblyopia Jastrzebsik et al devised a model of amblyopia, which describes Von Noorden et. al. summarized the extraordinary complex sensitivity, plasticity and elasticity (SPE) in relation to its mechanisms of amblyopia. They established that the visual response to occlusion[22]. Sensitivity indicating a propensity system of certain laboratory animals is exquisitely sensitive for the patched eye to worsen and for the amblyopic eye to to abnormal or decreased visual input from birth to 12 weeks improve and, among the sensitive eyes, some are distinguished of age, and even brief periods of abnormal visual stimulation in the model as elastic and some as plastic. Elasticity implying could cause a predictable set of behavioral, physiologic, reversibility, after occlusion is discontinued, of improvement and histologic anomalies in the visual system of different in the amblyopic eye, and of damage in the patched eye and species. For this pattern of anomalies, they coined the term plasticity implying a permanent change in both the eyes. A visual deprivation syndrome[17]. The similarity between study done by Oster et al suggests that younger age may be the manifestations of the visual deprivation syndrome in associated with both greater sensitivity and elasticity[23]. experimental amblyopias of apparently different etiology Their study indicates that stability i.e. loss of elasticity and suggested that each type is caused by common mechanisms. presence of sensitivity and plasticity becomes evident between They concluded that form vision deprivation is common to the third and fourth birthdays. all types of amblyopia and that the old and often maligned Recent randomised, controlled treatment trials, together passive concept of disuse implied in the term ex anopsia may with reviews of patients who have not been compliant with perhaps deserve a revival. treatment, indicate that the natural history of amblyopia is However, animal experiments have provided evidence that not that of spontaneous recovery. Intervention is required to the deleterious effect of form vision deprivation on geniculate maximize potential visual acuity in the affected eye. The age cell growth can be inhibited if binocular interaction is blocked at which that intervention will still be effective has not been by experimentally inactivating the cells in the adjacent confirmed and is the subject of on-going studies. non-deprived LGN laminae[18,19]. That is, under normal Priestly observed that after occlusion therapy some patients conditions there is a balance of interaction between the may show a change in fixation preferences without an terminals from corresponding retinal points in the LGN which improvement in visual acuity, where as others may improve takes place either in the LGN, via translaminar connections, in vision without a change in fixation[24]. The possibility of or in the visual cortex. When visual experience is abnormal angle of deviation being influenced by occlusion has drawn early in life, the deprived cells are at a disadvantage in the little attention. Swan in 1947 noted a significant increase in competition and their growth is inhibited[18,19]. Inhibiting the angle of esotropia in four out of one thousand patients interocular visual processes are known to occur even in normal following occlusion therapy. Pine and Shipman observed that binocular vision, as exemplified by retinal rivalry or the occlusion therapy as a treatment of amblyopia whether full- extinction phenomenon of Aulhorn. Binocular interaction has time or part-time carries a very small risk (4%) of increasing an even more profound effect in amblyopia, and there is ample a preexisting esodeviation by five prism diopters or greater so clinical evidence to suggest that the function of an amblyopic as to become cosmetically unacceptable[24]. eye is subject to inhibitory factors elicited by stimulation- of the normal eye.Thus a dual concept of amblyopiogenic factors Treatment modalities has evolved from all these investigations. Occlusion therapy Since 1722, where Saint Yves first described occlusion of Treatment of Amblyopia the dominant eye to promote use of the squinting eye, it The studies of early treatment intervention regimes allow has remained the mainstay of amblyopia therapy. Worth better understanding of the natural history of amblyopia. The noted that the age at which a squint developed and the age finding that those populations that undergo early intervention at which treatment began were important in establishing and treatment have lower prevalence of amblyopia than those prognosis[25]. From his results developed Worth’s fraction:

Vol. 21, No. 1, July-September, 2010 DJO 55 Delhi Journal of Ophthalmology Amblyopia A Historical Consideration age in months when permanent turn become apparent / age in exercises with Haidinger brushes on Cuppers coordinator. The months at which training began and it was used for prognosis. latter device uses the property of the fovea to polarize light. Occlusion provides the amblyopic eye a preferential chance of Inverse occlusion is continued till central fixation is achieved, development as the dominant eye is withheld from binocular after which direct occlusion is started. participation. The success rate of occlusion recedes with age, Both these methods, however, are time consuming, requires, good compliance and age less than 6-7 years ensures a success elaborate instrumentation and a regular follow up. It requires rate of almost 100%[26-28]. the cooperation of an intelligent patient (therefore useful for Rutstein et al showed that patients with strabismic or older than 5 years old) and the duration of therapy required is anisometropic amblyopia show a better and faster gain in longer. visual acuity when less than 7 years or less[28]. Most of the visual acuity improvement occurs within the first three months Medical Treatment of treatment. Epelbaum et al studied 407 patients of strabismic There is evidence that plasticity of the visual system during amblyopia and noted that recovery of acuity of the amblyopic the sensitive period is dependent on inputs from noradrenergic eye was maximum when the occlusion was initiated before 3 neurons, and is subject to pharmacological manipulations. years of age, decreased as a function of age and was almost In 1871, Nagel[39] used strychinine for the treatment of nil by the time the patient was 12 years of age[29]. Assaf, amblyopia, while Bieth attempted its use with oxygen. in a retrospective study involving 1904 patients of strabismic Barany and Hallden used alcohol as a inhibition mechanisms amblyopia noted that the period of maximum sensitivity to involved in amblyopia are known to involve synaptic short periods of occlusion extended to 18 months, declining neurotransmitters[40]. Pettigrew and Kasamatsu[41,42] to about 30 months of age in terms of transfer of fixation[30]. used neither activation of neither central nor epinephrine Bangerter recommend the occlusion of the amblyopic eye system for enhancing neuronal plasticity, while Kasamatsu to treat eccentric fixation[31].This inverse occlusion was used beta-blockers like propranol[41-44]. Duffy proved supposed to interrupt the subnormal fixation behavior. Its use that bicuculline, a GABA receptor blocker, which causes has now been discontinued as its much less effective than catecholamine depletion, reversed visual deprivation[45,46]. conventional occlusion[32]. Kasamatsu used beta-blockers like propranolol, and together Penalisation with Pettigrew, also used central norepinephrine system to Penalization has been described as an unpleasant neologism enhance neuronal plasticity[41-43]. Exogenous NGF (nerve used for defining methods of treating amblyopia that growth factor) prevents the effects of deprivation in rats; selectively fogs the image of the sound eye[33,34]. Maffei et al predicted that loss of competition for deprived In spite of better acceptance due to binocular stimulation, its eye is due to lack of neurotropic factor, and replenishing it only useful in unilateral amblyopia, requires prior solution may prevent amblyopia[47]. Visual deprivation is known of squint, anisometropia, and aniseikonia. It is also known to decrease retinal dopamine concentration in children and to carry the risk of occlusion amblyopia[35] and therefore, monkeys. Catecholamines and other neurotransmitter like its use is limited to non-strabismic, mild amblyopia and for GABA, glutamate and acetylcholine are involved in neuronal maintenance therapy. plasticity in deprivation amblyopia and can restore partial visual acuity[48]. Bodis Wallner and Yahr reported that the Pleoptics Therapy cortical visual patterns evoked by a stimulus pattern might be In 1936, Comberg started active stimulation of the macula altered in latency and waveform in Parkinson’s disease, which to treat eccentric fixation. Bargerter, who coined the term, characterized by a pathological deficiency of the dopaminergic propagated its use with inverse occlusion[36,37]. This method system[49,50]. Demenici L et al showed that dopaminergic involves dazzling of the eccentrically fixating area with bright drugs affect the visual performance of normal subjects, lights while protecting the fovea with a disc projected on to the producing an improvement in contrast sensitivity[51]. Gottlob fundus, followed by intermittent stimulation of the macula. observed that the administration of dopamine in normal Treatment is given under direct observation using a modified subjects increases the ERG b wave, selectively changes the Gullstrained ophthalmoscope (Pleoptophor). amplitude of oscillatory potentials and reduces implicit time Cuppers used a modified ophthalmoscope (Euthryscope), of the pattern VEP and pattern ERG[52]. which has discs of various sizes to create a central after image, apart from dazzling the eccentric point[38]. He also used Gottlob et al investigated the short-term effect of a large single the attenuate flashing of room illumination (alternascope) dose (200µg) of levodopa on contrast sensitivity and binocular to perpetuate after images, and devised the visuoscope. The suppression in adult amblyopes, in a cross over, double afterimage is projected on the space coordinator where the masked study[53]. They reported an increase in the contrast hand-eye coordinator is releases. This is then followed by sensitivity and a decrease in the fixation point scotoma, and

DJO Vol. 21, No. 1, July-September, 2010 56 Amblyopia A Historical Consideration Delhi Journal of Ophthalmology an increase in visual acuity by a half line in 2 of 9 patients with moderate amblyopia. About 1 in 5 achieved visual acuity tested (22%). Leguire et al confirmed these results in 8-12 of 20/25 or better in the amblyopic eye. years old amblyopes using 400mg of levodopa[54]. In order to determine the tolerance and efficacy of levodopa-carbidopa References with part time occlusion therapy for childhood amblyopia, 1. Wiesel TN and Hubel OH. Effects of visual deprivation Leguire and coworkers carried out a double masked placebo on morphology and physiology of cells in the cat’s lateral controlled randomized longitudinal study in 10 amblyopic geniculate body. J Neurophysiol 1963; 26: 978-993. children between 6 and 14 years of age[55]. Subjects received 2. Hubel DH and Wiesel TN. Receptive fields of cells in striate on average, 0.48/0.12mg/kg body weight levodopa/carbidopa cortex of very young, visually inexperienced kittens. J three times per day combined with part-time occlusion of the Neurophysiol 1963; 26: 994 -1002. dominant eye (3 hrs/day) over a 3 weeks period. At the end of 3. Wiesel TN and Hubel DH. Single-cell responses in striate cortex the dosing regime the levodopa/carbidopa group significantly of kittens deprived of vision in one eye. J Neurophysiol 1963; improved in visual acuity by 2.7 lines and in mean contrast 26: 1003- 1017. sensitivity by 70% in the amblyopic eye. The placebo group 4. Wiesel TN and Hubel DH. Comparison of effects of unilateral improved in visual acuity by 1.6 lines in the amblyopic eye. and bilateral eye closure on cortical unit responses in kittens. J Tolerance and occlusion compliance was similar between Neurophysiol 1965; 28: 1029-1040. groups. One month after termination of the treatment the 5. Hubel DH and Wiesel TN. Binocular interaction in striate levodopa/carbidopa group maintained a significant 1.2 lines cortex of kittens reared in artificial squint. J Neurophysiol 1965; improvement in visual acuity and 70% improvement in 28: 1041-1059. contrast sensitivity in the amblyopic eyes. The placebo group 6. Wiesel TN and Hubel DH. Extent of recovery from the effects did not maintain an improvement in visual acuity between the of visual deprivation in kittens. J Neurophysiol 1965; 28: 1,060- eyes. They concluded that levodopa/carbidopa, at an average 1072. of 0.48/0.12 mg/kg. Body weight is well tolerated, and when 7. Hubel DH and Wiesel TH. The period of susceptibility to the combined with part time occlusion, is efficacious in improving physiological effects of unilateral eye closure in kittens. J visual functions in amblyopic children. Physiol (London) 1970; 206: 419-436. Citicoline (Cytidine–5 diphosphocholine) has been used 8. Ikeda H and Wright MJ: Is amblyopia due to inappropriate clinically for head injury and Parkinsonism. In a dose of 1000 stimulation of the sustained pathway during development? Br J mg I.M.for 15 days, without any amblyopia therapy to patients Ophthalmol 1974; 58: 165-75. aged 9-37 years (mean 16.6years), it caused a temporary 9. Hubel DH, Wiesel TN and LeVay S. Plasticity of ocular improvement in visual acuity without any side effects. dominance columns in monkey striate cortex. Phil Trans R Soc London, Series B 1977; 278: 377-409. Future : PEDIG studies 10. Wiesel TN. Postnatal development of the visual cortex and the The Pediatric Eye Disease Investigator Group (PEDIG), the influence of environment. Nature 1982; 299: 583-591. network of university-based and community-based pediatric 11. Noorden GK von. Histological studies of the visual system eye care practitioners conducting multiple clinical research in monkeyswith experimental amblyopia. Invest Ophthalmol studies, conducted the Congenital Esotropia Observational 1973:12: 727. Study, which assessed the early course of esotropia in infants, 12. Noorden GK von and Middleditch PR. Histology of the and the Amblyopia Treatment Studies, a series of randomized monkey lateral geniculate nucleus after unilateral lid closure trials, the first of which compared atropine and patching for and experimental strabismus: further observations. Invest treatment of moderate amblyopia in children 3 to <7 years Ophthalmol 1975; 14: 674. old. In a randomized, multicenter clinical trial, 193 children 13. Crawford MU and Noorden GK von. The effects of short- with amblyopia were assigned to receive weekend atropine or term experimental strabismus on the visual system in Macaca patching of the sound eye 2 hours per day. At 17 weeks, visual mullata. Invest Ophthalmol Vis Sci 1979; 18: 496. acuity had improved from baseline by an average of 7.6 letters 14. Noorden GK von, Crawford MU and Levaey RA. The lateral in the atropine group and 8.6 letters in the patching group. geniculate nucleus in human anisometropic amblyopia. Invest The mean difference between groups (patching - atropine) Ophthalmol Vis Sci 1983; 24: 788- 789. adjusted for baseline acuity was 1.2 letters. Visual acuity in 15. Noorden GK von and Crawford MU. The lateral geniculate the amblyopic eye was 20/25 or better in 15 participants in nucleus in human strabismic amblyopia. Invest Ophthalmol Vis the atropine group (17%) and 20 in the patching group (24%; Sci 2729-2732. difference, 7%; 95% confidence interval, -3% to 17%). They 16. Adler’s Physiology of the Eye: clinical application edited by concluded that treatment with atropine or patching led to William M. -Hart, Jr. 9th ed. p 811, Mosby -Yearbook, 1992. similar degrees of improvement among 7- to 12-year-olds 17. Von Noorden, G.K.,and Crawford, M.L.J.: Forn deprivation

Vol. 21, No. 1, July-September, 2010 DJO 57 Delhi Journal of Ophthalmology Amblyopia A Historical Consideration without light deprivation produces the visual deprivation 38. Cuppers C. Modern schielbehandlung. Kun. Monatsp., syndrome, Brain Res. 1977. Augenheikd 1956; 129: 579. 18. Guillery, R.W., and Stelzner, D.J.: The differential effect 39. Nagel A. Die Behandlung der amaurosen und amblupien mit of unilateral lid closure upon the monocular and binocular strychnin. Klin Monatsol Augenheikd 1871; 9: 264-269. segments of the dorsal lateral geniculate nucleus of the cat. 40. Barany EH and Hallden U. Experiments aiming (It the treatment J.Comp. Neurol. 139 : 413, 1970. of squint amblyopia with medicaments. Acta Ophthalmol 1949; 19. Von Noorden, G.K., Crawford, M.L.J, Middleditch, P.R..: The 27: 138- 145. effects of monocular visual deprivation : disuse or binocular 41. Pettigrew JD. Pharmacologic control of cortical plasticity. interaction ? Brain Res. 111:277, 916. Retina 1982; 2: 360. 20. Hardman Lea SJ, Loades J, and Rubinstein MP. The sensitive 42. Pettigrew JD and Karamatsu T. Local perfusion of noradrenaline period for anisometropic amblyopia. Eye 1989; 3: 783- 790. maintains visual cortical plasticity. Nature 1978; 271: 761-764. 21. Jastrzebski GB, Hoytes and Marg E. Stimulus deprivation 43. Kasamatsu T. Enhancement of neuronal plasticity by activating amblyopia in children. Sensitivity, Plasticity, and Elasticity the norepinephrine system in the brain: A remedy for amblyopia. (SPE). Arch Ophthalmol 1984; 102: 1030-4. Hum Neurobiol 1982; 1: 49-54. 22. Oster JG, Simon JW and Jenkins P. When is it safe to stop 44. Kasamatsu T and Pettigrew JD. Depletion of brain patching? Br J Ophthalmol1990; 74: 709- 71 I. catecholamines: failure of ocular dominance shift after 23. Noorden GK von. Classification of amblyopia. Am J Ophthalmol monocular occlusion in kittens. Science 1976; 194: 206-209. 1967; 63: 238. 45. Duffy FH, Burchfield JL and Snodgrass SR. The pharmacology 24. Pine L, Shipmann S. The influence of occlusion therapy on of amblyopia. Ophthalmology 1978; 85: 189-195. esodeviation. Am Orthop J. 1982; 32: 61-65. 46. Duffy FH, Snodgrass Sr, Burchfield JL and Conway JL. 25. Worth C. Squint. 1st ed. London 1903; 76. Bicuculline reversal of deprivation amblyopia in the cat. Nature 26. Fulton AB and Mayers DL. Esotropic children with amblyopia: 1976; 260: 256-257. effects of patching on acuity. Graefes. Arch Clin Exp Ophthalmol 47. Domemici L, Cellerino A and Maffei L. Monocular deprivation 1988; 226: 309-312. effects in the rat visual cortex and lateral geniculate nucleus are 27. Lithander J and Sjostrand J. Anisometropic and strabismic prevented by nerve growth factor (NGF) II. Lateral geniculate amblyopia in the age groups 2 years and above: a prospective nucleus. Proc R Soc Lond Series B 1993; 25: 25-31. study of the result of treatment. Br J Ophthalmol 1991; 75: 111- 48. Bear MF, Singer W. Modulation of visual cortical plasticity by 116. acetylcholine and noradrenaline. Nature 1980; 320: 172. 28. Rutstein RP and Fuhr PS. Efficacy and stability of antiamblyopia 49. Bodis-Wollner land Yahr MD. Measurements of visual evoked therapy. Optom Vis Sci 1992; 69 (10): 747-754. potentials in Parkinson’s disease. Brain 1978; 101: 661-671. 29. Epelbaum M, Melleut, Buisseret P, Dufier JL. The sensitive 50. Bodis- Wollner I, Yahr MD, Mylin Land Thornton J. period for strabismic amblyopia in humans. Ophthalmol 1993; Dopaminergic deficiency and delayed visual evoked potentials 100 (3): 323-327. in humans. Ann Neurol 1982; 11: 478-483. 30. Ahmed AA. The sensitive period: Transfer of fixation after 51. Dominici L, Trimarchi C, Piccolino M, Fiorentini A and occlusion for strabismic amblyopia. Br J Ophthalmol 1982; 66: Maffei L. Dopaminergic drugs improve human visual contrast 64-70. sensitivity. Human Neurobiol 1985; 4: 195-197. 31. Bangerter A. Ambyopie behandung Basel : 5 Karger AG 1953; 52. Gottlob I, Weghaupt H, Vass C and Auff E. Effects of levodopa 123. in human pattern electroretinogram and pattern visual evoked 32. Parks MM and Friendly DS. Treatment of eccentric fixation in potentials. Graefe’s Arch Clin Exp Ophthalmol 1989; 277: 421- children under 4 years of age. Am J Ophthalmol 1960; 61: 395. 427. 33. Campos EC. Future directions in the treatment of amblyopia. 53. Gottlob I and Strangler-Zuschrott E. Effect of levodopa on Lancet 1997; 349: 1190. contrast sensitivity and scotomas in human amblyopia. Invest 34. Noorden GK von and Milani JB. Penalization in the treatment Ophthalmol Vis Sci 1990; 31 ( 4 ): 716-780. of amblyopia. Am J Ophthalmol 1979; 55 : 511-18. 54. Leguire LE, Rogers GL, Bremer DL, Walson P and 35. Jampolsky A. Unequal visual inputs and strabismus management: Hadjiconstantinou- Neff M. Levodopa and childhood A comparison of human and animal strabismus. In: Symposium amblyopia. J Pediatr Ophthalmol Strabismus 1992; 29:290-298. on strabismus. Transactions of the New Orleans Academy of 55. sLeguire LE, Walson PD, Rogers GL, Bremer DL, and Ophthalmology. St. Louis: CY Mosby, 1978; 358-492. mcGregor ML. Longitudinal study of levodopa/carbidopa for 36. Bangerter A. Behandlung der Amblyopie, Ophthalmologica childhood amblyopia. J Pediatr Ophthalmol Strabismus 1993; 1946; 111: 220, 1946. 30: 354-360 37. Bangerter A: Amblyopiebehandlung, ed.2, Basel, 1955; S. Karger A.G.

DJO Vol. 21, No. 1, July-September, 2010 58 Delhi Journal of Ophthalmology

Instruments Scan Multifocal Electroretinography Lalit Aalok1, Swati Phuljhele2 Vitreoretina Consultant, Perfect Vision Eye Hospital Faridabad1, Senior Research Associate, Dr RP Centre2

Visual electrophysiological tests provide information about the functional integrity of the pigment epithelium (Electrooculogram or EOG), the photoreceptors and inner layers of the retina (electroretinogram or ERG), and the optic nerve and occipital cortex (Visual Evoked Potential or VEP). The information is objective and independent from the voluntary response of the patient and thus is especially useful for non-cooperative patients and infants. The multifocal ERG technique provides reliable parameters for the accurate evaluations of diseases and their different treatment modalities existing and emerging.

Introduction out. Wide-field mfERG permits assessment of retinal function The full-field or Ganzfeld ERG (ffERG) provides a mass from the central 90 degrees of the retina. response from the retina, and thus will not be altered substantially by localised lesions of the macula, which Methodology contributes only 10% to the ERG response. Therefore its Patient preparation greatest value lies in disorders that result in diffuse functional The pupils must be fully dilated prior to mfERG testing as impairment of the retina. On the other hand, a diffuse retinal changes in pupil size alter the mfERG responses significantly. disease with macular sparing will have abnormal ERGs but Patients must be light adapted for at least 15 minutes in room normal central visual acuity. The multifocal ERG technique light for obtaining the cone driven responses, and recording (mfERG), developed by Sutter and Tran in 1992, provides is done with the room light on. Patients need to have a visual spatial maps of ERG responses from multiple areas of the acuity permitting fixation upon the target. To help patients macula and would be a very useful technique to assess macular having poor vision maintain an accurate fixation upon the status in such situations. Multiple local ERG responses, presented target, as well as to promote results of the best typically 61, are recorded from the cone-driven retina under quality, an optical correction appropriate for viewing distance light-adapted conditions. Although multifocal ERG recording must be used at all times. The near infra red image sensor of rod function is possible, maintaining dark adaptation during incorporated in the optoelectronic stimulator enables checking testing is difficult in an actual clinical setting. Focal ERG uses for stable central fixation during mfERG testing. “Large a focal light stimulus to elicit a local ERG response, a process field” eye glasses should be preferred to avoid masking of which would be excessively time consuming if multiple areas the peripheral parts of the stimulation display. Recording may were to be tested in succession as in mfERG. While mfERG be done monocularly (preferable), or binocularly to aid in provides useful information about the photoreceptor status, it fixation at the target in instances of low subject visual acuity. requires preparation of the patient and the output is complex, requiring expertise in interpretation; its use is therefore Recording electrodes mainly limited to specialist centres where research is carried These may be of contact lens or non-contact lens types. Table 1: Comparison of different ERG techniques

Focal ERG Full-field/Ganzfeld ERG Multifocal ERG (ffERG) Test duration Time consuming Time for dilatation and dark Time for dilatation and light adaptation-30 minutes adaptation is around 20 Time for actual test is around minutes 30 minutes Time for actual test is less than 10 minutes Identification of disease Localised disease can be better Unable to detect localised Localised disease can be better detected retinal disease; examines detected; more sensitive; entire retina examines the central retina Interpretation Requires familiarity Requires experience Output requires expertise in interpretation

Vol. 21, No. 1, July-September, 2010 DJO 59 Delhi Journal of Ophthalmology Multifocal Electroretinography Contact lens type electrodes are optically clear and lie retina, namely the photoreceptors and the bipolar cells. The in contact with the cornea, contact being aided by use of standard mfERG mainly measures the cone function and the either topical anaesthetic drops or artificial eye drops. response components are technically known as kernels. The Repeated use should be avoided to prevent the risk of disease most commonly analysed responses are the first-order and transmission or disturbance of their transparency. Corneal the second-order kernel components. First order kernels are electrodes provide responses of larger amplitude and with obtained by adding all the records following the presentation of fewer artefacts. Examples of contact lens electrodes are Jet a flash in that hexagon and subtracting all the records following electrode (unipolar), Dorian Gold lens (bipolar) and Burian- a dark frame. (Figure 3) shows the typical waveforms in the Allen electrode (unipolar or bipolar). Burian-Allen electrodes first-order kernel mfERG electrical response. The waveform make use of an incorporated speculum to hold the lids apart is biphasic with an initial negative deflection followed by a and prevent blinking. positive peak. There may be a second negative deflection after Non-contact lens electrodes may lie in contact with the cornea the positive peak. These peaks are labelled as N1, P1, and N2, or the bulbar conjunctiva. Examples of non-contact lens type respectively. The N1response amplitude is measured from the electrodes are gold-foil electrodes, H-K loop and DTL fiber. starting baseline to the base of the N1 trough; the P1 response The active electrode lies in the inferior fornix or on the cornea, amplitude is measured from the N1 trough to the P1 peak. The the reference electrode lies lateral to the lateral canthus (if peak implicit times are measured from the stimulus onset. using a unipolar electrode). The ground electrode is placed in The second order kernel represents the temporal non linearity the centre of the forehead. (Figure 1: Placement of electrodes) of the local responses of the retina or in other words is a measure

Recording technique The stimulus source may be any one of Cathode Ray Tube (CRT), Light emitting diode (LED), Liquid Crystal Display (LCD) screen or Scanning Laser Ophthalmoscope (SLO). The advantage of the scanning laser ophthalmoscope (SLO) is that it shows the exact anatomical location of the stimulus relative to the fundus of eye. The recording technique describes the one utilised in the MetroVision system (Perenchies, France). A field of ±300 horizontally and ±240 vertically centred on the fovea is Figure 1: Placement of electrodes stimulated at a viewing distance of 30 cm with a stimulus consisting of an array of usually 61 squares or hexagons (scaled or of uniform size) on a high resolution colour monitor. (Figure 2) Increasing the number of stimulated hexagons to 103 or 241 or higher improves the spatial resolution but progressively increases the testing time. The sizes of the hexagons are scaled (increased in size) with retinal eccentricity from the fovea to the periphery to elicit approximately equal amplitude responses from all locations. This is because the concentration of the cones decreases with increasing distance from the fovea. A red fixation target is presented within the central hexagon. The luminance of each hexagon is independently alternated between black and white (93% contrast) according to a pseudorandom binary ‘m-sequence’ at a frame rate of 75 Hz i.e every 13.33 seconds. The mean luminance of the stimulus is 100 cd/m2 with the stimulus screen being surrounded by an uniformly illuminated background cover with the luminance set at 30 cd/m2 to eliminate the rod responses. At a stimulus frequency of 18 Hz, 5000 responses are acquired over a period of 5 minutes for each eye.

Understanding the mfERG response The human mfERG is dominated by the cells of the outer Figure 2: Metrovision monitor screen with scaled hexagon stimuli

DJO Vol. 21, No. 1, July-September, 2010 60 Multifocal Electroretinography Delhi Journal of Ophthalmology

Figure 3: The multifocal ERG waveforms

Figure 7: Display output of results

Figure 4: Trace array display

Figure 8: Macular sparing with a foveal peak on multifocal ERG in a patient with retinitis pigmentosa.

Table 2: P¬1 wave characteristics according to damage to different retinal layers Figure 5: Three dimensional topographic display showing foveal peak and the blind spot Damage to P1 wave of mfERG Amplitude Implicit time Cone receptor Smaller Moderate delay or normal Outer plexiform layer Normal or larger Large delay Inner plexiform layer Normal Small delay On-bipolar cells Smaller Moderate delay Off-bipolar cells Larger ? slightly faster Ganglion cells Normal Normal

of how the mfERG response is influenced by the adaptation to successive flashes. Many authors claim that temporal non linearities arise from the inner retina and therefore abnormal second order kernel may indicate abnormal processing or Figure 6: Group average by rings adaptation at the level of the inner retina. It has an initial

Vol. 21, No. 1, July-September, 2010 DJO 61 Delhi Journal of Ophthalmology Multifocal Electroretinography

positive peak followed by a negative trough, called P1 and in response amplitudes had not occurred, laying stress N1, respectively. on the measurement of implicit times over amplitude The mfERG response output can be in several forms. parameters. These changes are more prominent in the 1. Trace array: The individual electrical trace response from peripheral regions and wide-field mfERG may be more each stimulated zone is displayed in an array from the useful in detecting affectation of the more peripheral entire tested area. (Figure 4) retinal areas. (Figure 8)A study by the author in 87 eyes 2. Topographic color map displays of amplitude and implicit with retinitis pigmentosa revealed highly significant times: These may be 2 or 3 dimensional displays. (Figure 5) correlation between the visual acuity and the P1 and N1 3. Group averages: Grouping of the electrical responses may response amplitudes in patients with retinitis pigmentosa. be done by concentric ring zones, quadrants or by user 5. Patients with Stargardt’s disease have decreased defined zones. (Figure 6) The most commonly reported amplitude in the region of central macula with minimal result is response density, in which the responses from the implicit time delays. elements in each ring are summed and then divided by the 6. Retinal vascular occlusions (RVO): There is reduction area of these elements. in the amplitude and increase in the latency of mfERG The average response per zone is displayed in nV/deg2 in responses in vascular occlusions. The alterations on graphic form, or as a histogram. (Figure 7) The amplitudes and mfERG are found to correlate with the areas of visual field implicit times for the different waveforms are also displayed changes in both branch arterial and venous occlusions. for each zone. The RMS analysis (root mean square) identifies Branch arterial occlusion causes damage to the inner the presence of a response when noise is present. The RMS 2/3rd of the retinal layers and thus the mfERG changes measurement of the bar in color, which represents the signal are more prominent in the second-order kernels than column, is compared to the RMS measurement of the black the first-order kernels. Laser treatment is followed by bar, which represents the noise column. The multifocal map implicit time delays and amplitude reduction in DR and can also be superimposed on the image of the patient’s eye vascular occlusions after 8-12 weeks, with the implicit fundus. Each laboratory must develop its own normative data time alterations being more prominent. which preferably should be age-adjusted. 7. Glaucoma: Though significant reductions in both first- Test guidelines for mfERG testing have been published by the order and second-order kernel response amplitudes International Society for Clinical Electrophysiology of Vision are seen in glaucoma, correlation with the visual field (ISCEV: http://www.iscev.org), to allow for further research changes are inconsistent and lacking and mfERG may not before standards are set. As is expected, the stimulus and be a reliable indicator of functional loss in glaucoma. recording parameters, and the adaptive state of the eye strongly 8. The mfERG technique can be used to follow the effects affect the electrophysiological responses, necessitating of clinical intervention, for example after PDT therapy standardisation for purposeful scientific exchange of test for AMD, before and after retinal detachment surgery or results. macular hole surgery, after treatment of macular edema in Indications diabetic retinopathy using different treatment modalities, 1. The mfERG can be used to differentiate diseases that after retinal transplant procedures or in patients with affect the outer retina from those that affect the ganglion retinitis pigmentosa after stem cell therapy. cells or optic nerve. (Table 2) References 2. Age-related macular degeneration (AMD): A significant 1. Sutter EE, Tran D. The field topography of ERG reduction in the foveal P1 amplitude and delay in N1 components in man--I. The photopic luminance response. implicit time is seen in early AMD. More severe alterations Vision Res. 1992;32:433-446. are seen in more severe disease. It has been suggested that 2. Hood DC, Bach M, Brigell M, Keating D, Kondo M, rod-mediated mfERG may be more sensitive in detecting Lyons JS, Palmowski-Wolfe AM. ISCEV guidelines for retinal dysfunction in early AMD. clinical multifocal electroretinography (2007 edition). 3. Diabetic retinopathy (DR): The implicit time measures Doc Ophthalmol. 2008;116:1-11. are more sensitive as compared to the amplitude changes, 3. Hood DC, Frishman LJ, Saszik S, Viswanathan S. Retinal which are reduced, in detecting retinal dysfunction origins of the primate multifocal ERG: implications in DR. The foveal thickness measurement on Optical for the human response. Invest Ophthalmol Vis Sci Coherence Tomography (OCT) has been found to 2002;43:1673-1685. correlate significantly with the mfERG amplitudes and 4. Hood DC. Assessing retinal function with the multifocal implicit times. technique. Prog Retin Eye Res 2000;19:607-646. 4. Retinitis pigmentosa: The amplitude is decreased 5. Lai TYY, Chan W, Lai RYK,Ngai JWS, Li H, Lam DSC. typically along with delays in implicit times. Implicit The clinical applications of multifocal electroretinography: time alterations have been found at times when changes a systematic review. Surv Ophthalmol 2007;61-96.

DJO Vol. 21, No. 1, July-September, 2010 62 Delhi Journal of Ophthalmology Instruction to Authors

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