UNDERTAKING This is to state that I, DR. HUZEIFA M. HUSEIN have completed this dissertation on MACULAR DISORDERS STUDIED WITH OPTICAL COHERENCE TOMOGRAPHY for the degree of M.Ch. in Ophthalmology, taking full responsibility for the originality of the material submitted & the methodology of presentation. The study was conducted with the help and guidance of, Dr. Ajay I. Dudani & Dr. M. S. Husein at their respective clinics. DR. HUZEIFA M. HUSEIN M.B.B.S., D.O.M.S, F.C.P.S., M.S. (USAIM)
DISSERTATION MACULAR DISORDERS STUDIED WITH OPTICAL COHERENCE TOMOGRAPHY University of Seychelles American Institute of Medicine (USAIM) M.Ch. Ophthalmology Batch: October 2008 Candidate: PG Guide: Dr. Huzeifa M. Husein Dr. Ajay I. Dudani 1
ABSTRACT / SCOPE The study was carried out to obtain diagnosis of Macular Disorders using Optical Coherence Tomography for the following conditions 1. Diabetic Maculopathy 2. Age Related Macular Degeneration (ARMD) 3. Central Serous Retinopathy 4. Macular Hole 5. Cystoid Macular Oedema 6. Epiretinal Membrane 7. Macular Scar 8. Idiopathic Polypoidal Choroidal Vasculopathy Diagnosis was aimed at providing conclusive evidence, for initial observations made by direct & indirect ophthalmoscopy. These OCT interpretations were helpful in providing an insight into the probable course of the disease, modality of treatment (Laser / Anti VEGF) required, effect of treatment (Eg: Decrease in CSME with focal laser) as also effective tool for patient counseling. Early lesions not visible by Direct / Indirect / 90D examinations or lesions without quantitative data (How big or how thick) even disputed lesions (Present or absent) can be distinguished out easily. 2
MATERIALS & METHODS 40 cases with Macular pathology were studied from April 2006 to September 2007. All consecutive cases with positive clinical findings on Direct Ophthalmoscope and Indirect Ophthalmoscope, showing pathological involvement of macula were selected. Age / Sex exclusion were not a criteria. On screening, those patients having macular lesions were subjected to detailed evaluation. History: Elicited from the patient onset, duration and vision reduction was noted. Symptoms: Macropsia Micropsia Metamorphopsia Positive Central Scotoma (inquired) Family History: Specific family history was asked (if related). Systemic History: History of Tuberculosis with treatment, Diabetes Mellitus, and Hypertension was ascertained. Past History: History of any ocular surgery or treatment was asked. History of any ocular laser was also asked. Drug History: Chlorpromazine Chloroquine AKT drugs 3
Examination: Distance Vision noted on Snellen s Chart Near Vision noted on Jagger s Colour Vision Ishihara s Chart External Examination on Slit Lamp: Cornea Anterior Chamber Iris Pattern Pupil Lens The above were examined to rule out any pathology. Intraocular pressure was noted with Keeler Pulsair (Intellipuff). AMSLER S GRID All the patients in the study were also subjected to Amsler s Grid. Each eye was tested separately, with reading glasses where prescribed. The central dot was to be focused. Any distortion, waviness of lines, burred areas or scotomas were questioned. After this, all the patients were examined with dilated pupils. Initially, Direct Ophthalmoscope. Subsequently, Indirect Ophthalmoscopy was done. Lastly, Slit Lamp +90D was used. The presence of Macular Lesion was noted as follows: o Size o Shape o Oedema o Hemorrhage o Exudate o Scarring 4
o Pigmentation Fundus Fluorscein angiography was performed in few patients with suspect clinical findings. Lab Investigations Complete Blood Count, Haemoglobin, ESR, Blood Sugar Fasting & Post Parandial, HIV, VDRL, Torch Titers, X ray Chest After the above tests, the OCT was performed in all the patients. Procedure / Patient Experience The patient s experience is normally brief and comfortable. Each eye scan be done roughly in 5 7 minutes. The patient is seated in front of the OCT camera with chin on chin rest & forehead touching the headrest. The central ring is brought into focus, after which retinal vessels & optic disc are brought into focus. A laser beam, directed by the onboard computer scans various parts of the retina. The patient sees a rectangular field of red punctuated by green lights while the test is in progress. OBSERVATIONS & STATISTICS TABLE I: PATIENTS ACCORDING TO AGE Age in Years No. Of Patients % 1 20 4 10 20 40 10 25 40 60 16 40 >60 10 25 5
TABLE II: SEX DISTRIBUTION TOTAL MALE FEMALE 40 28 (70%) 12 (30%) TABLE III: DIFFERENT MACULAR DISORDERS (No. OF CASES) MACULAR DISORDER No. OF PATIENTS % Diabetic Maculopathy 11 27.5 Age Related Macular Degeneration 8 20 Central Serous Retinopathy 6 15 Macular Hole 5 12.5 Cystoid Macular Oedema 3 7.5 Epiretinal Membrane 3 7.5 Macular Scar 2 5 Idiopathic Polypoidal Choroidal Vasculopathy 2 5 TABLE IV: CASES WITH POSITIVE MACULAR SYMPTOMS SYMPTOMS No. OF CASES % MICROPSIA 2 5 MACROPSIA 4 10 METAMORPHOPSIA 13 32.5 POSITIVE CENTRAL SCOTOMA 2 5 6
TABLE V: CASES WITH DEFECTIVE COLOR VISION COLOR VISION No. OF CASES % NORMAL 25 62.5 DEFECTIVE 5 12.5 INDISTINCT 10 25 TABLE VI: CASES WITH AMSLER GRID CHANGES CHANGE No. OF CASES % NEGATIVE 25 62.5 POSITIVE 15 37.5 TABLE VII: MACULAR DISORDER & SYSTEMIC DISEASE SYSTEMIC DISEASE No. OF CASES % MALARIA TUBERCULOSIS 3 7.5 TOXOPLASMOSIS 1 2.5 DIABETES MELLITUS 14 35 TABLE VIII: CASES WITH ABNORMAL FLUORSCEIN ON ANGIOGRAM ANGIOGRAM No. OF CASES % HYPO FLUORESCENCE 3 7.5 HYPER FLUORESCENCE 17 42.5 ANGIOGRAM NOT DONE 20 50 7
DISCUSSION I. DIABETIC MACULOPATHY 11 cases (out of total 40) (27.5%) were of diabetic maculopathy. After routine examination, fundus photography, FFA was done to rule out Clinically Significant Macular Oedema (CSME). In all the cases, it was difficult to diagnose with certainty CSME. (By Indirect Ophthalmoscopy, fundus photograph) CSME being defined by the ETDRS study - Thickening of retina within 500μm from the center of fovea - Hard exudates at or within 500μm from the center of fovea, associated with adjacent retinal thickening - Retinal thickening of 1 Disk Diameter or more, any part of which maybe within 1 Disk Diameter from the center of fovea In our (above) patients with Diabetic Maculopathy, 4 cases (10%) showed focal leak and 3 cases (7.5%) diffuse oedema on FFA. OCT picture showed retinal thickening >500μm at the center of the fovea, corresponding to CSME. Focal grid laser was given to 4 (10%) of the patients with focal leaks and 3 (7.5%) with diffuse leaks were given grid laser. 4 (10%) remaining patients had retinal thickening with Neovascularization. I.e. Proliferative Diabetic Retinopathy (PDR), which was treated with Pan Retinal Photocoagulation and also grid laser. Good visual recovery was seen in 7 out of 11 patients. Repeat OCT could be done in only 2 patients due to poor patient follow up and cost factor. Repeat OCT in these patients showed that retinal thickening was decreased to <225μm with good visual acuity improvement. 8
Diabetic CSME has been described in four patterns by OCT i. Sponge Like Thickening Thickening of the retina like a sponge is seen here Treatment Focal Laser ii. Cystoid Macular Oedema Cyst like spaces are seen between the internal nuclear layer and outer plexiform layer Treatment Intravitreal Kenacort then, Focal Laser iii. Serous, Sub foveal Retinal Detachment An elevation is seen, corresponding to a Retinal Detachment just above the RPE chorio capillary layer Treatment Intravitreal Kenacort iv. Taut Posterior Hyaloid Membrane The outermost layer of the retina appears to be pulled taut by the hyaloid, giving rise to traction Treatment Para Planna Vitrectomy II. AGE RELATED MACULAR DEGENERATION (ARMD) Total 8 patients (20%) were diagnosed with ARMD. 5 had Dry ARMD (62.5%) and 3 had Wet ARMD (37.5%). The 3 patients with Wet ARMD underwent FFA and only one was seen to have classic Choroidal Neovascularization (CNV). On OCT imaging the Dry ARMD patients showed a) Local modulation of the RPE layer b) Thinning of neurosensory retina like a geographic atrophic patch On OCT imaging the Wet ARMD patients showed a) Classic CNV: Fusiform thickening and localized disruption of the RPE Chorio Capillary layer. Retinal oedema and sub retinal fluid also helped in diagnosis 9
b) Occult CNV: Seen as an area of increased Choroidal reflectivity due to increased penetration of the RPE c) Fibrovascular PED: Seen as elevated RPE layer above an area of mild back scattering region, corresponding to fibrovascular proliferation All the patients (3) of Wet ARMD had metamorphopsia. These patients were referred for PDT or TTT to a private practioner. The patients being poor didn t go there and thus had severe scarring with vision loss. Active Stage Highly reflective, many layered area was seen protruding into the sub retinal space. Intermediate Stage CNV reflectivity became stronger and its margin in the sub retinal space became smooth. Cicatricial Stage Moderately high reflective are covered by a dome shaped highly reflective area which corresponded to RPE was seen. III. CENTRAL SEROUS RETINOPATHY (CSR) CSR was seen in 6 patients (15%) in our study. 2 of these (33.3%) were females and 4 (66.6%) were males. Thus confirming that the incidence was more in males than females. 5 patients were in the age group of 40 50 years and one was between 30 40 years. All the patients were subjected to FFA first then OCT. 4 patients (66.6%) on FFA showed smoke stack appearance and 2 patients (33.3%) showed non specific leakage of dye. OCT was diagnostic in 2 of these non specific FFA patients. (8) 10
3 of the 6 patients (50%) had metamorphopsia with a positive Amsler Grid test. OCT imaging showed a characteristic elevation of the neurosensory retina. 3 of the 6 patients of CSR had Visual Acuity of 6/12 (Corrected by +1.5D). 3 of these patients followed up with us and had final visual acuity of 6/6 without any treatment. OCT was possible to be repeated in only one patient, it was seen as the characteristic settling of neurosensory retina. IV. MACULAR HOLE Macular holes were contributory by 5 cases (12.5%). From these 3 cases (60%) were diagnosed to be lamellar holes and 2 (40%) to be full thickness holes. (1 being post trauma). Of the total 5 cases, 3 patients had metamorphopsia with positive Amsler Grid. 2 patients with full thickness holes (50%) had positive central scotoma. Watzake (9) sign was positive in 2 patients with full thickness macular hole. In 3 of the patients with partial thickness holes, OCT was the ultimate diagnostic tool. Differentiation: Lamellar Hole: Diagnosed by evidence of perifoveal vitreous detachment with no loss of whole retinal tissue Full Thickness Hole: Diagnosed by the clear loss if retinal tissue at the fovea extending up o the RPE. Importance of OCT in Macular Hole - Surgical Planning & post surgery follow up - Screening & monitoring of other eye - Vitro retinal interface assessment. Demonstration of Posterior Vitreous Detachment (PVD), Vitreo macular traction & surrounding fluid cuff (2) 11
V. CYSTOID MACULAR OEDEMA (CME) 3 patients (7.5%) had poor postoperative vision after routine cataract surgery I.e. < 6/36. Fundus photograph showed a dull foveal reflex. On FFA there was absence of the characteristic flower petal appearance. On comparison of FFA with OCT sensitivity, in detecting CME is 96% & specificity being 100% (10) However in all these patients, OCT showed characteristic cyst like spaces in the retinal layer. Local NSAIDS were prescribed for 3 months period. VI. EPIRETINAL MEMBRANE (ERM) A total of 3 patients (7.5%) had ERM. 2 were following Retinal Detachment surgery (66.6%). 1 patient with no discernable cause (33.3%). All the 3 patients had metamorphopsia. OCT imaging showed thickened membrane with a cleavage point seen in all 3 patients. Using OCT it was possible to study characteristics of ERM - Membrane presence with thickness - Retinal fluid accumulation - Cystic Changes - Presence of focal verses global retinal surface adherence The above increased the quality of preoperative assessment, helping in giving a postoperative visual prognosis. (11) Advantage of OCT being Depending upon the site of cleavage point, the surgeon can enter the vitrectomy scissor for ERM peeling. VII. MACULAR SCAR In total 2 of the patients (5%) had a macular scar. 1 after toxoplasma and the other idiopathic. 12
VIII. IDIOPATHIC POLYPOIDAL CHOROIDAL VASCULOPATHY (IPCV) Idiopathic polypoidal choroidal vasculopathy (IPCV) was diagnosed in only 3 patients (5%). They were initially diagnosed, erroneously as CNV. Later on Indo Cyanine Green (ICG), they showed polypoidal mass with complete wash out after ICG. OCT in both patients showed serous PED, seen as RPE chorio capillary layer elevation. 13 patients (32.5%) showed metamorphopsia. Some patients who didn t have symptoms of metamorphopsia had a positive Amsler Grid test, as seen in 15 patients (37.5%). CONCLUSION Our study consisted of 40 cases of macular disorders who underwent OCT. The following conclusions were drawn 1. Diabetic Maculopathy the most common. 11 cases (27.5%) 2. a) 4 cases (36.3%) of the 11 cases of Diabetic Maculopathy had Proliferative Diabetic Retinopathy with Taut Posterior Hyaloid Membrane, in which cases par planna vitrectomy is indicated. b) 7 cases (63.7%) remaining, had focal as well as diffuse leaks on FFA, in whom focal or grid laser was given. 3. Age Related Macular Degeneration (ARMD) was seen in 8 cases (20%). 3 cases (37.5%) were of Wet ARMD & 5 cases (62.5%) were of Dry ARMD. OCT helped determine the depth & spread of Choroidal Neovascularization (CNV). 4. Central Serous Retinopathy (CSR) was seen in 6 cases (15%). FFA showed no characteristic appearance in 3 patients (50%) and was thus diagnosed on OCT by elevation of neurosensory retina. 13
5. Macular Holes were seen in 5 cases (12.5%). 3 being lamellar hole (60%) & 2 full thickness holes (40%). OCT determined the grade of macular hole as well as the line of treatment. 6. Cystoid Macular Oedema (CME) was present in 3 cases (7.5%), which was difficult to diagnose on FFA. OCT revealed characteristic Cystoid Spaces with increased thickness of the layers. 7. Epiretinal Membrane (ERM) present in 3 cases (7.5%). 2 cases (66.6%) were following Retinal Detachment Surgery and 1 was idiopathic (33.3%). In all 3 cases, cleavage line was seen, which was useful during surgery for ERM peeling. 8. In 3 cases of CSR (7.5%), 2 cases (5%) of Idiopathic Polypoidal Choroidal Vasculopathy (IPCV) and 3 cases of CME (7.5%), in which FFA was not characteristic, OCT gave ultimate diagnosis. Thus OCT was diagnostic in all these above cases. 14
CSME 15
PATIENTS ACCORDING TO AGE >60 Age in Years 40-60 20-40 1-20 0 2 4 6 8 10 12 14 16 18 No. of Patients No. of Patients DIFFERENT MACULAR DISORDERS (No. OF CASES) Central Serous Retinopathy Diabetic Maculopathy Cystoid Macular Edema Macular Scar Age Related Macular Degeneration Macular hole Epiretinal Membrane Idiopathic Polypoidal Choroidal Vasculopathy 11 5 8 3 3 6 2 2 16
BIBLIOGRAPHY 1. Hee MR, Izatt JA, Swanson EA, et al. Optical coherence tomography of the human retina. Arch Ophthalmol 113: 325 332,1995. 2. Puliafito CA, Hee MR, Lin CP, Reichel E, Schuman JS, et al. Imaging of macular diseases with optical coherence tomography. Ophthalmology 102:217 229,1995. 3. Swanson EA, Huang D, Fujimoto JG, Puliafito CA. Method & apparatus for optical imaging with means for controlling longitudinal range of sample vs. patent. 14 June 1994, #3321, 501. 4. Youngquist RL, Carr S, Danies Den. Optical coherence domain reflectometery. A new optical evaluation technique. Opt. Lit. 1987; 12: 158 160. 5. Jakada K, Yokohoma I, Chida K, Noda J. New measurement system for fault location in optical wave guide devices, based on interferometric technique; Appl. Opt 1987; 26: 1603 6. 6. Werner W, Fercher AF, Mengednot K. Eye length measurement by interferometery with partially coherent light. Opt. Lit. 1988; 13: 1867 9 7. Takahashi K, Ida H, Fukuchi T, Mallumura M. Staging of CNV by OCT. Schepens retina associates, Boston, MA 02114, USA. 8. Puliafito CA, Hee MR et al. OCT of Central Serous Retinopathy. Am J Ophthalmol 1995; 120 (1): 65 74. 9. Drener West, De Bistros, Guyner DR. Natural History of Idiopathic Macular Hole & Cysts. Arch Ophthal. 110: 1264 1268; 1992 10. Stanford MR, Anticliff RJ et al. Comparison between OCT & FFA for detection of Cystoid Macular Oedema. Ophthal. 2000: 107 (3): 593 599. 11. Puliafito CA, Wilkins JR et al. Characterization of Epiretinal Membranes using OCT. Ophthalmology 1996; 103 (12): 2142 2151. 12. Abrams GW, Blumenkraz MS, Lewis H, Campo RV. Vitrectomy for Diabetic Macular Traction and Oedema associated with Posterior Hyaloid Traction. Ophthalmology 1992; 99 (5): 753 759. 13. Palestine AG, Davis MD, Nussenblatt RB, Kuafman SC, Ferris FL. Macular Thickening & Visual Acuity Measurement in patients with Cystoid Macular Oedema. Ophthalmology 1987; 94 (9): 1134 1139. 14. Reichel E, Puliafito C, George A, Sulkes D, Rivellese M. optical Cherence Tomography after Laser Photo Coagulation for Clinically Significant Macular Oedema. Ophthal Surg Lasers 2000;31 (3): 192 197. 17
MASTER CHART Sr. No. OPD Age Sex V/A Symptoms Amsler Systemic Fundus Provisional FFA No. in History Diagnosis Yrs Metamorphopsia Micropsia Positive Scotoma 1 5205/06 58 M 6/24 - - - - - Hard Drusen Dry ARMD Not 2 7299/06 65 F 6/36 - - - - - Drusen Dry ARMD Not 3 2180/06 54 F 6/24 - - - - DM Hard Exudates/ Diabetic Focal CSME Maculopathy Leak CSR 4 1470/06 42 M 6/18 + - - + - Diffuse Elevation of Macula 5 2523/06 13 M FC12-14ft - - - - Toxoplasma CR Atrophic Scar at Macula Macular Scar Not 6 9321/06 48 M 6/60 - - - - DM FR Dull? CME No Flower Petal Pattern 7 7205/06 71 M FC6-8ft + - - + TB Drusen + Haemorrhage Wet ARMD 8 6101/06 14 M 6/36 + - - + - FR Dull Lamellar Hole 9 5152/06 39 M 6/60 + - - + - Irreg. ERM Membrane on Macula 10 8320/06 18 F 6/36 - - - - DM CSME Diabetic Maculopathy 11 3389/06 63 M FC16-18ft + - - + - FR Dull Lamellar Hole 12 4591/06 33 F 6/18 - - - - - Elevation of Macula 13 1172/06 49 F 6/24 - - - - - Elevation of Macula CSR IPCV Smoke Stack Irreg. Leak Not Not Focal Leak Hypofluores cence Smoke Stack ICG done OCT Localized Disruption of RPE Localized Disruption of RPE Sponge Like Thickening Elevation of Neurosensory Retina Thinning of Retina Cystic Spaces in Retina Disruption of RPE- CC Layer No Loss of Whole Retinal Tissue Cleavage Line Seen Sponge Like Thickening No Loss of Whole Retinal Tissue Elevation of Neurosensory Retina Polypoidal Elevation, Serous PED
MASTER CHART Sr. No. OPD No. Age Yrs Sex V/A Symptoms Amsler Systemic History Fundus Provisional Diagnosis Metamorphopsia Micropsia Positive Scotoma 14 1494/07 48 F FC1-2ft + - + + - FR Dull Full Thickness Macular Hole FFA 15 2357/07 17 M <6/60 - - - - - FR Dull CME Diffuse Leak 16 4710/07 62 M 6/60 - - - - - Hard Drusen Dry ARMD Not 17 9021/07 34 F 6/60 - - - - DM Exudate / Dot Diabetic Diffuse Haemorrhage Maculopathy Leak 18 7420/07 74 M FC10-12ft - - - - DM NVD Diabetic Not Maculopathy 19 5252/07 39 M 6/60 + - - + DM CSME Diabetic Diffuse Maculopathy Leak 20 6001/07 31 M 6/24 - - - - - Elevation of CSR Smoke Macula Stack 21 1151/07 71 M 6/36p - - - - DM NVE + Inferior Vitreous Haemorrhage Diabetic Maculopathy (PDR) 22 7386/07 39 M 6/24 - - - - DM CSME Diabetic Maculopathy 23 4513/07 45 M 6/60 + - + + - FR Dull Full Thickness Macula Hole 24 7697/07 39 M 6/12 - - - - - Serous Elevation of Macula 25 6319/07 51 F 6/24p + + - + - Cellophane Maculopathy 26 4413/07 78 F FC14-16ft + + - + - Irreg. Membrane over Macula CSR ERM Wet ARMD NVE+ Hypofluoresc ence Focal Leak Hypofluoresc ence Ink Blot Appear ance Not Not OCT Loss of Retinal Tissue Cystic Spaces in Retina Thickening of RPE Sponge Like Thickening Sponge Like Thickening Sponge Like Thickening Elevation of Neurosensory Retina Taut Post. Hyaloid Memb. Sponge Like Thickening Loss of Retinal Tissue Elevation of Neurosensory retina Elevated Retina Localized Disruption of RPE
MASTER CHART Sr. No. OPD No. Age Yrs Sex V/A Symptoms Amsler Systemic History Fundus Metamorphopsia Micropsia Positive Scotoma 27 0395/07 54 M FC8-10ft - - + + TB Haemorrhage Below Fovea 28 2701/07 44 F 6/12 + + - + - Elevation of Retina Provisional Diagnosis Wet ARMD 29 5439/07 37 M 6/24 - - - - DM FR Dull Lamellar Hole CSR FFA Not Diffuse Leak Hypofluoresc ence ICG 30 1099/07 62 M 6/24 - - - - - Irreg. Membrane over Macula IPCV 32 9365/07 59 F 6/36 - - - - - Hard Drusen Dry ARMD Not 33 0641/07 55 M 6/18 - - - - - Subretinal CSR No e/o Precipitates Smoke 34 7706/07 49 M FC 4-6ft - + - + DM NVD + NVE Diabetic Maculopathy with PDR 35 8360/07 26 M 6/60 + - + + - Epiretinal Membrane + Cryo marks ERM (Post RD Sx) 36 4259/07 51 M 6/24 - - - - DM FR Dull + Drusen Dry ARMD Not 37 2166/07 57 M 6/24 - - - - DM CSME Diabetic Diffuse Maculopathy Leak 38 9943/07 46 M 6/36 - - - - DM CSME Diabetic Diffuse Maculopathy Leak 39 8601/07 52 M 6/60 - - - - DM / TB NVD + Diabetic Not Maculopathy + PDR 40 4849/07 68 M FC14-16ft Stack Not Not - - - - - FR Dull CME Not OCT Disruption of RPE Elevation of Neurosensory Retina Full Thickness Retinal Break Absent Serous PED Localized Disruption of RPE Elevation of Neurosensory Retina Taut Post. Hyaloid Memb. Elevation over Retina Thickening of RPE Thickening of Retina Sponge Like Thickening Taut Post. Hyaloid Memb. Cystoid Spaces