1. Grand Rounds Vitamin A Deficiency Amir R. Hajrasouliha, M.D. University of Louisville Department of Ophthalmology and Visual Sciences Friday, March 7th, 2014

2. Patient Presentation CC:”I am going blind” CC:”I am going blind” HPI: 65 y/o WM present to us with gradual decreased vision OU for the past 8-10 months. He claim his vision is worse at night and indoors. He had no pain or discomfort. HPI: 65 y/o WM present to us with gradual decreased vision OU for the past 8-10 months. He claim his vision is worse at night and indoors. He had no pain or discomfort. He was told by an outside ophthalmologist that he will eventually go blind and there is no treatment available for his condition. He came to our clinic for second opinion. He was told by an outside ophthalmologist that he will eventually go blind and there is no treatment available for his condition. He came to our clinic for second opinion.

3. Patient Presentation Past ocular history: Past ocular history: Foreign bodies in cornea removed (4 years ago) Foreign bodies in cornea removed (4 years ago) Past medical History: Past medical History: Gastric bypass 1998 Gastric bypass 1998 Kidney failure on Dialysis 2010 Kidney failure on Dialysis 2010 Diabetes from 2008 Diabetes from 2008 Hypertension Hypertension Social history: Social history: EtOH abuser, heavy smoker EtOH abuser, heavy smoker ROS: ROS: Kidney failure, Heart failure, Neuropathy Kidney failure, Heart failure, Neuropathy Allergy: Allergy: NKDA NKDA

4. Exam VA cc P T TP VA cc P T TP EOM: Full OU CVF: Full OU 20/200 4  2mm 16 17 (-) RAPD 20/200

5. ODOS E/L/Lwnlwnl Conjclearclear Kclearclear ACformedformed Iriswnlwnl Lens1+NS 1+NS Anterior exam DFE: DFE:

6. Red free

7. OCT OD OD Subfoveal deposit with no fluid

8. OCT OS OS Subfoveal deposit with no fluid

9. Fluorescence angiography

10. Visual field OD OS HVF 24-2: Paracentral scotoma OD Centeral scotoma OS

11. Assessment and plan 68 y/o WM with gradual vision loss OU and subfoveal deposits OU and history of gasteric resection. 68 y/o WM with gradual vision loss OU and subfoveal deposits OU and history of gasteric resection. Vitamin Deficiency Vitamin Deficiency Vitiliform maculopathy Vitiliform maculopathy Plan Plan Pt started on vitamins (Ocuvite) and ERG and EOG ordered Pt started on vitamins (Ocuvite) and ERG and EOG ordered

12. EOG shows an attenuated Arden Ratio OU. EOG shows an attenuated Arden Ratio OU. The full-field ERG reveals severe rod and cone dysfunction OU. The full-field ERG reveals severe rod and cone dysfunction OU. Arden Ratio Arden Ratio =1.5 OD =1.5 OD Arden Ratio Arden Ratio =1.1 OS =1.1 OS

13. Follow up Pt returned in 8 months with improved symptom and BCVA of 20/20 OD and Pt returned in 8 months with improved symptom and BCVA of 20/20 OD and 20/40 OS 20/40 OS

14. Follow up

15. Follow up OCT OD OD OS OS

16. Vitamin A Compounds with 20-carbon structure. Contain a methyl substituted cyclohexenyl ring (  -ionone ring), and an isoprenoid side chain with either a hydroxyl group, and aldehyde group, a carboxylic acid group, or an ester group (retinyl ester) at the terminal C15. All-trans-retinal 11-cis-retinal RetinolRetinoic Acid Can’t be reduced to retinol or retinal in the body.

17. Bitot spot Corynebacterium xerosis

18. Vitamin A Deficiency Xerophthalmia refers to the ocular manifestations associated with vitamin A deficiency. Xerophthalmia refers to the ocular manifestations associated with vitamin A deficiency. These include xerosis, keratomalacia, nyctalopia and Bitot’s spots. These include xerosis, keratomalacia, nyctalopia and Bitot’s spots.

20. Visual cycle Miyazono S et al. PNAS 2008;105:16051-16056 ©2008 by National Academy of Sciences

21. Case report A case report of 61 year old male with history of bariatric gastric surgery present with night blindness and fundus white spots. After treatment with oral Vitamin A for 2 months vision and ECG changes improved. A case report of 61 year old male with history of bariatric gastric surgery present with night blindness and fundus white spots. After treatment with oral Vitamin A for 2 months vision and ECG changes improved. Fishman et al. Fundus white spots and acquired night blindness due to vitamin A deficiency. Doc Ophthalmo 2009;119 UIC chicago

22. Full-Field electroretinogram (ERG) responses before and after therapy with Vitamin A in dark- adapted (A) and light adabted (B) conditions Full-Field electroretinogram (ERG) responses before and after therapy with Vitamin A in dark- adapted (A) and light adabted (B) conditions

23. Thank You