35 y/o female. Three weeks ago had headache, fever and chills. She went to local MD and was treated for a urinary tract infection but soon after this

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1 David S. George, MD

2 35 y/o female. Three weeks ago had headache, fever and chills. She went to local MD and was treated for a urinary tract infection but soon after this developed an infection in her lungs. One week ago she lost vision in her right eye and went to the ER. She was given another antibiotic for her lung infection, but she did not receive neuroimaging or any other work-up for her loss of vision.

3 Vision 20/CF at 12 inches OD, 20/20 OS Central VF loss OD only, OS VF WNL SLE unremarkable no cells in A/C Afferent pupillary defect on Right DFE Optic Nerve Edema Macular Edema Lipid deposition in retina

4

5 Neuroretinitis, also called stellate maculopathy due to lipid forming a star pattern in macula Differential Diagnosis includes: Immunocompromised individuals (HIV, chronic disease, Possible viral etiology in some cases Infectious neuroretinitis causes by Bartonella Henselae also called Cat Scratch Disease

6 Further questioning of the patient after retinal exam revealed the she had indeed been scratched by a wild cat she found a month ago. The scratch on her leg was still not entirely healed. Further exam revealed mild pre-auricular lymphadenopathy

7 The patient was told she had Cat Scratch Fever and she replied that I thought that was just a song. I assured her it was not the same problem Ted Nugent had.

8 A Bartonella Henselae titre obtained: Positive 1:20! The patient was placed on Doxyclycine 100mg PO bid and was also given a Medrol dose pack to reduce optic nerve swelling One week later we found the titre to be positive and her vision had improved to 20/100 and she reports no more headaches or fevor Two weeks later she was 20/70 with no visible edema, only residual lipid in retina 8 weeks later 20/40 and lipid continuing to show less lipid in retina, central MOCT 211 um OD

9 Cat Scratch Disease can take 7-14 days, and as long as 2 months, to become symptomatic after a cat scratch Often and there is local lymphadenopathy There may be a papule at the site of the initial infection Fever is common but other symptoms include headache, chills, backache, and abdominal pain. An acute encephalopathy of the brain may occur as well. This patient has mild confusion and foci of cortical hypointense T2 lesions in brain on MRI

10 Treatment - Azithromycin, Ciprofloxacin, Doxycycline Azithromycin is preferentially used in pregnancy to avoid the side-effects of doxycycline. Outcome is favorable despite the severity of the initial presentation.

11 12 y/o boy sent for referral by OD doe to poor vision OD and large change in refraction Patient is health, no medical problems other than seasonal allergies

12 Vision sc 20/60 with CL OD, 20/20 sc OS SLE corneas clear with Vogt s striea OD Fundus WNL MR BVA OD 20/40 over refract CL OD: Plano at 119 OD

13 Note KPI and K Prorbablity 0% and Normal IS ratio (<1.4)

14 Note KPI and Kprobability 100% and IS ratio is 13! (normal <1.4)

15 Keratoconus is usually bilateral The etiology is unkown, keratoconus likely arises from a number of different factors: genetic, environmental or cellular, any of which may form the trigger for the onset of the disease increased activity by proteases (or a decrease in protease inhibitors). Proteases are enzymes that break some of the collagen cross-linkages in the stroma.

16 Genetic predisposition, Autosomal Dominant, seen in some cases. Overall incidence is 1/2000 people. More common in Down s Syndrome patients Keratoconus has been associated with atopic diseases, which include asthma, allergies, and eczema. Iatrogenic, thin corneal bed after LASIK

17 Contact lenses typically RGP lenses Piggy Back contacts RGP over a soft CL Scleral Contact Lenses larger, less comfortable Cornea Intrastromal Ring Segments induce corneal flattening and facilitate contact lens fit Corneal Transplant has been standard last step treatment, risk of rejection, post of astigmatism with significant refractive error common

18 Collagen Cross-Linking (not yet FDA approved in US) stabilizes cornea to slow or stop keratoconus disease progression. USA clinical trial underway. DALK Deep Anterior Lamellar Keratoplasty; only the outermost epithelium and stroma are replaced (not Descement s membrane or the endothelium); it is also possible to transplant freeze-dried donor tissue (as do not need viable endothelium as in full transplant). The freezedrying process ensures this tissue is dead, so there is no chance of rejection.

19 Unilateral cases rare but asymmetry common perhaps this 12 y/o will exhibit evidence of keratoconus OS as he gets older.

20 Sudden decrease in vision OS in a 55 y/o male History of trauma OS years ago 1 month h/o sudden decrease in vision OS

21 Vision sc 20/40 OD, 20/60 OS BCVA 20/20 OD, 20/60 OS Anterior segment trace NSC OU anterior segment otherwise unremarkable DFE Paripapillary RPE disruption OU Dirty grey sub-retinal neovascular membrane (SRNVM) OS

22 Subretinal Neovascular Membrane

23 Area of hyperfluorescence around SRNVM but also a linear, paripapillary streaks of hyperfluorescence found on FA

24

25 Break in Bruch s Membrane can occur spontaneously or with trauma May bee seen in association with pseudoxathoma elasticum (PXE) as well as Paget s Disease (abnormal calcium deposition in bones), Sickle Cell Anemia, Ehler-Danlos Syndrome (inherited connective tissue disorder); but it can be Idiopathic. Histopathology reveals extensive calcification of Bruch s Membrane

26 Fibrovascular tissue can grow through the break in Bruch s Membrane, under the retina. This fibrovascular tissue can lead to subretinal bleeding, choroidal neovascularization and disciform scar.

27 ARMD, Exudative Choroidal Rupture Toxoplasmosis Lacquer cracks (pathologic myopia) Histoplasmosis (this case had initial appearance of POHS with peripapillary RPE disruption Fluoroscein Angiography is key in confirming the diagnosis. Angioid streaks appear hyperfluorescent on FA due to overlying retinal pigment epithelial (RPE) atrophy.

28 Asymptomatic Angioid Streaks do not require treatment Asymptomatic Angioid Streaks patients are more susceptible to choroidal rupture, particularly during participation in ball sports Choroidal neovascular membranes are usually treated with laser and/or intra-vitreal injections of anti-vegf medications.

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