 Alright everyone, I think since we've got two presenters this morning, we'll go on and get started. So our first is going to be Dr. Krista Kinard. She's one of our second year's soon-to-be chief residents. A lot of the people in the crowd already know her very well. So an extra interesting fact about her is that she likes to bench press with Tom Oberg on Saturday mornings. So a little extra information about Krista. So here she goes. Alright, good morning everyone and thank you all for being here. Thank you Dr. Kim also for giving me some time for this patient presentation. This is a neuro-ophthalmology patient presentation and we could maybe use some help still on ideas for the diagnosis. So the topic is whether or not this patient just had optic discodema with immacular star from the swelling or if they had neuro-retinitis. Our patient was a previously healthy 16-year-old girl who presented to Primary Children's Medical Center, ED, with complaints of blurry vision. On obtaining further history when she was seen in the neuro-ophthalmology clinic, we found out that she had had intermittent neck pain and stiffness as well as tinnitus of her ears starting kind of mid-February. And then in March she knows that she had just the slight blurry spots in her vision but it wasn't severe so she wasn't super worried about it. And then the first weekend in April she developed severe blurry vision as well as bad neck pain. So she ended up going into the ER at Primary Children's. On review of systems, I'll talk about what happened to her once she had the ER visit in just a little bit. On review of systems, she denied having headache, weight changes, changes in medication. She wasn't on any medications. Photophobia, rashes or skin lesions. She had noticed some dim-outs of her vision but this was more orthostatic. She'd noticed it when going rapidly from a sitting or laying down position to standing. But she'd had this intermittent knee pain for about four years when she developed the knee pain. She'd get swelling and redness of her knee joint and this would last about a month and then it would go away on its own. And she developed this knee pain of her left knee this time. It alternates about four days after she was seen in the emergency department. She'd also had an upper respiratory tract infection back in December of 2011 that did not require any treatment and she's not had any other sort of infections, GI or respiratory since then. In the emergency department, they did imaging. She had a normal brain MRI, a normal C-spine MRI and there was concern for thrombosis so they did an CT angio that was normal. Her laboratory studies, she had normal thyroid, normal inflammatory markers, CBC and chemistry. They did a lumbar puncture though and she had an abnormal white blood cell count but her glucose protein and the rest of her cell count was normal and her opening pressure was elevated at 50. At that time they were not concerned for an infectious etiology so other than the cell count they did not send it for the CSF for any other studies. So she was diagnosed with IIH and sent first to PZOP though and then to us. The remainder of her history was not extremely enlightening. She did not have any eye problems prior to this. She had a history of a heart murmur and interestingly every time her blood pressure had been taken in February, March and April had been high like the 140s but she didn't have a diagnosis of high blood pressure. Her only surgeries were the wisdom teeth extraction. She was on no medications initially. She's an athletic, full-time student. She plays soccer, throws a javelin. No pets in the home, no contact with pets really. No recent travel. She's never traveled outside of the US, not sexually active and no alcohol, tobacco or drugs. Her family history was non-contributory. On exam when we saw her in the neuro-ophthalmology clinic her blood pressure was relatively normal. She was a little tachycardic, normal weight and height. Her visual acuity was great. She was 20-20 in the right eye and 20-20 in the left. Near vision was good. Her intraocular pressures were normal. She had perfect color vision and almost perfect stereo vision. Her visual fields were full to confrontation. She didn't have any proptosis. Her eyes were straight. Her extracular movements were completely normal. Anterior segments were normal. But on her dilated fundoscopic exam she did have discodema nasally in both eyes as well as patent lines. And she had almost a complete macular star in the right eye and a partial macular star in the left eye. And some old vitreous cell on fibrils but no other evidence of inflammation. And with the patent's lines we knew she had had more discodema than this. But the notes from in the ER and the P's orthoclinic stated that she had 4 plus discodema. Her neurological exam was completely normal. So these were the photos. We might need to, if somebody could dim the lights it might show up better. But this was a color fundus of her right eye when we first saw her in the neuro-ophthalmology clinic on the second. You can see the nasal discodema and kind of see the outline of the patent's lines. And then the macular star right in here and here's the outline of the patent's lines. And the left eye it was a little bit less obvious. You can see the discodema again and then just a partial macular star. These were her autofluorescence photos and red free. And you can see the macular star in the right eye here. It doesn't really show up in the left eye here. And then on the OCT it's very subtle but in the right eye if you look carefully you can see that the external limiting membrane and kind of that photoreceptor layer is interrupted. But the left eye looks relatively normal. And so we had one of the retina fellows, Dr. Shakur take a look at this. And he agreed that that was abnormal in the right eye and that there had been some evidence of old inflammation in her right eye, not so much in her left eye. So this was when we first saw her. These were her visual fields and they were red as normal but she did have a slightly enlarged blind spot in both eyes a little bit more in the left than in the right. So our differential at this point was rather broad with the macular star it helped narrow it a little bit but we were still thinking infectious like an infectious neuro retinitis. Typical infections would be Bartonella, some of the masquerades, Lyme, Lupus or Sarcoid can rarely cause this but it's a possibility, diabetes, neoplastic process. We thought about tubular interstitial nephritis and uveitis with her history of high blood pressure. Not common but like an antifospholipid antibiotic syndrome or leavers neuro retinitis or acute idiopathic blind spot enlargement. So we ordered a bunch of tests. After she was seen in the emergency department at primary children's and had the opening pressure of 50 she was started on diamox 500 milligrams twice a day and like I said the optic discudema improved in the pediatric ortho clinic it was about 4 plus and 1 plus when we saw her. She did not develop any pallor we saw her back on the 23rd of May as well and on the 23rd of May her vision was still 2020 in the right eye in 2015 in the left and her stereo vision was now perfect. The laboratory's results were all negative except for a positive EBV antibody to the viral capsid antigen. I went over these results with the infectious disease department and they agreed this is evidence of past infection. We talked to them about whether or not it could have been as current as February and they did not think that this was the case and they recommended maybe checking for like coxie well that was really their only recommendation and then repeating the lumbar puncture to send CSF studies. She had also had a chest x-ray back in February. I forget why she had that it was some sort of injury and that was completely normal. So on the 23rd this was her OCT. She still has the macular star in the right eye and the interruption of the photoreceptor layer there and the left eye again looks relatively normal. So just to talk about neuro retinitis versus optic disc swelling that can cause exudates in the macular and give you a macular star. This area gets a little bit gray looking at the literature. Some of the papers group these entities on a continuum and others try to lump or put it into more specific categories with infectious neuro retinitis, non-infectious and recurrent neuro retinitis and then an entity called Odoms. The main difference that I could come up with to try to make a black and white between the two is that with neuro retinitis there is inflammation of the retina whereas if you just have optic disc swelling and a macular star there's no retinal inflammation and they'll call this Odoms sometimes. And the visual loss is pretty moderate in both. You have discidema in both and this can be diffuse or focal discidema. The macular star seems to develop a little bit sooner in neuro retinitis than in just discidema with the macular star. But typically in both this is followed by a serous detachment which is quite common and our patient did not have this. Vitreous cell is common in neuro retinitis. It's not just with discidema. Age group is the same. There's no gender bias. Typically no pain with neuro retinitis and there may or may not be pain if you just have disc swelling. And the visual field defects are typically central or secocentral with the neuro retinitis because of the maculopathy whereas they're more variable with discidema. And the visual loss that does occur in neuro retinitis is due to the maculopathy whereas in optic discidema it's due to an optic neuropathy. But both of these recover vision and they have a good prognosis with 90% or greater having good visual recovery. So in this article it was from the Journal of Neuro Ophthalmology their differential for optic discidema with a macular star. So of course neuro retinitis, a hypertensive retinopathy would be more bilateral. You'd see more hemorrhages. Our patient didn't have that. Papillodema can cause it. Our patient was rather young for an AION. She didn't have diabetes. Tumors you would see on exam. She didn't have any medications that she was on or any toxic exposures. And on review of systems it didn't suggest anything like polyarditis, no-dosa or inflammatory bowel disease. And then the etiologies of neuro retinitis are long. You can have bacterial infections, protozoa, viruses, nematodes, fungus. And then you can have a neuro retinitis from post-vaccination, non-infectious, and that's where the sarcoid comes in, and then uncertain etiologies. So basically there's still a lot about this that we just don't know or understand. The treatment guidelines, these are variable. It depends on how severe the vision loss is or the visual field defects. If it's idiopathic you can consider steroids for Bartonella. You can consider antibiotics, but this isn't necessary most of the time. And then with a recurrent neuro retinitis you can consider steroids, but you're also worried about more of a systemic disease and you consider immunosuppression. But again, most of the time patients are not treated. Treatment is only considered if there's severe vision loss or severe visual field defects. Just some of the highlights that I found in reading through this information. The macular star pathophysiology that you get just with optic discidema is not due to any problems with the retina, gas, and somebody, I forgot the name of the other researcher, they did studies and showed that it's vasculitis of the laminar and pre-laminar part of the optic nerve head. There's fluid leakage from the optic disc capillaries and based on the way Henley's lair lays, fluid flows into there and then you get the macular star pattern. Bartonella is the most common cause of neuro retinitis and typically patients are not treated. And if patients have optic discidema with this macular star, there's no increased incidence of multiple sclerosis. They've looked at this, MS targets myelin and it doesn't target the optic disc vasculature or the retina. So back to our patient, we'll continue to follow her neuro ophthalmology. We still don't know completely what she has and she's been referred to Dr. Vitale and believe her appointment is in June but I'd have to double check on that. And these are my resources. Any questions? I think that it's, while I'm looking at it, it seems like it's hard to draw hard lines between them. It seems like it's a little bit more of a continuum to me but I don't know what Dr. Warner and Dr. DeGray feel. True. It typically, at least in the reading that I did, it typically resolves. It can take up to a year. It takes longer than the discidema to resolve and then you're left occasionally with just some pigment changes there in the macula.