Kolkata Retina Conclave 2024 interesting cases

INTERSTING RETINA CASES

1. Retina in Systemic diseases Manmath Kumar DAS Consultant Vitreo -retinal SURGEON KIMS, Bhuabaneswar

2. Case 1 62 yr old female DOV since 1 yr Known case of T2 DM & Hypothyroidism, no other systemic issues Came to us for 2 nd opinion as she was advised for PPV +ILM peeling elsewhere

3. Past history Was diagnosed as NPDR with DME h/o one shot of Triamcinolone in right eye elsewhere h/o one shot of Avastin in both eyes In view of no response, was advised for PPV+ILM peeling + C3F8 suspecting ONH pit maculopathy

4. BCVA : 6/24, N18 & 4/60, N36 Ant. Segment : normal except trace cortical spokes

5. Funduscopy

6. OCT

7. FFA (done elsewhere)

8. Quick recap of history & Clinical findings DOV : 1yr , 62 yr, female Severe visual loss Otherwise systemically well Huge macular NSD with outer retinal schisis No response to either steroids/ Anti- VEGF

9. Differential diagnosis NPDR with compromised Renal function Paraproteinemias

10. Renal Function normal Urine Bence Jones Protein, Serum Protein Electrophoresis

11. On Systemic workup……..

12. Multiple myeloma

13. What happened to our patient ??? The pt. lost to follow up after 4 long years !!! BCVA : 3/60 ( both eyes) Finally , turned up in early 2024 for Follow-up

14. FFA ( follow-up)

15. FFA ( Follow-up)

16. OCT- after treatment for 5 years OD OS

17. THE IMMUNE SYSTEM Cellular Immune System  White Blood Cells: Granulocytes Lymphocytes: T cells Humoral Immune System  B cells and antibodies

18. A B BASIC STRUCTURE

19. T YPES OF A NTIBODIES o Types of Heavy Chains o o o o o IgG IgA IgM IgD IgE o Types of Light Chains o o Kappa Lambda

20. W HAT HAPPENS IN M ULTIPLE M YELOMA ?  Malignant plasma cell makes a clone of itself and these malignant plasma cells, or myeloma cells, accumulate in the bone marrow.  The malignant plasma cells secrete an antibody, or immunoglobulin, called the M- protein , or M- spike , or paraprotein or myeloma protein . It can be detected in the blood and/or the urine of most myeloma patients.

21.    P ROTEIN E LECTROPHORESIS AND I MMUNOFIXATION Protein electrophoresis separates the proteins in a blood or urine sample into several groups based on their size and electrical charge. In most patients with MM, large amounts of an abnormal immunoglobulin protein (M-spike) will appear as a large peak on the graph. Immunofixation is done to identify the specific type of protein that is being produced by the malignant plasma cells. The amount of protein produced may vary throughout the course of the disease, but the type generally will remain the same.

22. N ORMAL S ERUM P ROTEIN E LECTROPHORESIS P ATTERN Figure 1 Typical normal pattern for serum protein electrophoresis.

23. A BNORMAL S ERUM P ROTEIN E LECTROPHORESIS P ATTERN Figure 2 Abnormal serum protein electrophoresis pattern in a patient with multiple myeloma. Note the large spike in the gamma region.

24. S ERUM F REE L IGHT C HAINS (FLC) OR F REE L ITE A SSAY  Measures the amount of free light chains in the serum (blood).  In normal circumstances, plasma cells produce an excess of light chains compared to heavy chains. A small amount of these light chains will not become incorporated into intact immunoglobulins. These are “free ” light chains and are released into the blood.

25. S ERUM F REE L IGHT C HAINS ( CONT ’ D )  Most patients with Multiple Myeloma produce increased amounts of either kappa or lambda free light chains, which can be measured in the blood.  Consequently, the ratio of kappa to lambda light chains is abnormal in most patients and is a sensitive indicator for this disease.  This test may be used to monitor progression and/or treatment.

26. B ONE M ARROW A SPIRATE AND B IOPSY  Most laboratory tests for Multiple Myeloma provide indirect information about the amount of tumor present, by measuring proteins that are secreted by the tumor into the blood and/or the urine.  These tests do not provide the same information as looking at the tumor itself. The myeloma cells are usually only found inside the bone marrow.

27. B ONE M ARROW A SPIRATE AND ( CONT ’ D ) B IOPSY    Small fragments of bone marrow cells can be withdrawn with an aspirate needle, and a small core of bone and marrow is often removed at the same time with a different type of needle. Both tests are done to determine how much of the normal bone marrow is replaced by myeloma cells. Additional, specialized testing (cytogenetics and FISH) can be performed which can provide information about the biology of the tumor itself.

28. Conclusions Though rare, para-proteinemias should be suspected in middle age to elderly if macular NSDs does NOT have a corresponding features either on FFA or OCT Unilateral cases are sometimes difficult to diagnose

29. Case 2 : 27 year, female Known case of Takayasu arteritis IIb Gradual DOV (OU) – 6 months assoc. with Intermittent pain, redness, headache Systemic complaints - headache, dizziness, syncope, seizure, carotidynia , bilateral upper limb and jaw claudication , amarusois fugax

30. On Systemic examination Brachial and Radial pulses absent BP not recordable in upper limbs BP lower limb – 134/59 (R) & 140/82 mm Hg CT angio - >90% stenosis in B/L common carotid and Sub clavian A . With mild circumferential mural thickening of Ascending aorta, arch of aorta & Descending Aorta

31. FFA – at presentation

32. Treatment - (OU) – PRP laser 3 to 4 sittings done

33. FFA – after PRP

34. Final Diagnosis Takayasu Arteritis iib with Ocular Ischemia , cataract Over next 2 years, she developed NVG (both) Recently VA (OD) – PL+, (OS) – CF 3MT

35. CASE 3 : 51 yr., female, known Hypertensive, Rheumatoid Arthritis Sudden DOV (OU) – 3 DAYS Severe HEADACHE – 3 DAYS BP = 130/70 Drug History – for HTN, Tab. HCQ, Methotrexate, Folate etc. BCVA (OU) – CF@ ½ MT

36. Fundus at presentation OD OS

37. OCT at presentation OD OS

38. Diagnosis ??? Blood dyscrasias -------- anaemia , leukaemia

39. Systemic workup Hematology consultation – CBC/ P. SMEAR/ SERUM IRON PROFILE Rheumatology consultation – CONTINUED PREVIOUS rx Medicine CONSULTATION for htn – ncct Brain, LFT/KFT

40. Investigations – results Peripheral smear- mild to mod. degree of Fe deficiency anemia NCCT brain - NORMAL LFT/KFT – NORMAL The physician changed anti- HTN Rx in view of poor HTN control Oral hematinic started

41. Working Diagnosis Anemic retinopathy

42. Rx- 25g ppv for OD under LA

43. Post-op BCVA: 6/36- OD, 6/60-OS

44. Just 4 days later AROUND 4 : 30AM, she had Generalized Seizures and loss of consciousness Low GCS, BP 230/120 mmHg, Sp o2 was 85 % EVEN WITH O2 (15L/MIN) Coarse Crepitations on Chest Auscultation She was shifted to ICU and intubated Subsequently diagnosed to have Acute Subarachnoid hemorrhage with Coma 1month later, she passed away

45. Where did we go wrong ?? Did we miss any warning signs !!! She gave history of one episode of seizures about 1 month back- took some medications – but did not reveal it at the time of first examination H/o Severe headache – 3 days NCCT brain – NORMAL

46. Risk factors of SAH Middle age Hypertensive h/o Severe Headache since 3 days -- SAH 1 episode of seizures --- Sub acute SAH

47. Final Diagnosis Terson Syndrome

48. THANK YOU