1404hrs: Pediatric Retina & Uveitis PANEL DISCUSSION

Some of this discussion was worked into the talks to accommodate visiting speakers flight times. Selected points are noted here. 

1410_Panel_Discussion

 

One comment though, neurologists don’t seem to believe that intermediate uveitis can potentially be associated with multiple sclerosis. Also, those with Toxoplasmosis should get neuro-imaging as they can have neurological involvement of disease.

 

1348hrs: Endophthalmitis (Amir Guerami)

Abbreviated talk due to running almost 1 hour behind.

1348_Guerami

 

Any intraocular surgery can lead to endophthalmitis. Generally pain presenting 1-7 days post-op with AC cells/flare/hypyon. Discusses ESCRS prophylactic Tx to prevent endophthalmitis. Intracameral cefuroxime vs topical treatment no significant difference. 

Glaucoma filtration risk thought to be about the same as following cataract surgery (but I don’t think he is including late onset cases that are at lifelong risk.)

Post-traumatic discussed as well with higher risk bacillus infections.

Endogenous endophthalmitis only about 5% of cases. Higher chance fungal with mortality as high as 29%

TASS Toxic Anterior Segment Syndrome usually occurs within the first day following surgery. Basically hyperacute vs typical acute endophthalmitis.  Vitiritis rarer in TASS. 

Preventive measures include povidone iodine, sterile speculum, pre and post op coverage with little solid evidence for any in particular this topical Tx.

Reviewed all the different treatment regimens depending on nature of organism. 

Endophthalmitis Vitrectomy Study also discussed with its finding of vitrectomy if LP or worse vision or chronic endophthalmitis or post trab or endogenous endophthalmitis.

Make correct diagnosis endophthalmitis vs TASS. Tap and Inject vs vitrectomy. Identify organism and use preventive measures.

1332hrs: Posterior Uveitis/Sympathetic Ophthalmia (Bernard Hurley)

Top 10 cases of posterior uveitis and what they have taught the speaker.

1332_Hurley

 

Case 1:

26 yo WM with blurry vision x 3 days. Granulomatous KPs, conj redness, 2+ cells, vitritis. Therefore a pan-uveitis.

Tb, Sarcoid, Syphilis, Lyme and others

All tests came back negative

Additional information: friend had lit a firecracker near his eye several weeks prior to uveitis presentation that did have exposed uvea other eye. Therefore, this patient had sympathetic ophthalmia! Bilat panuveitis develops 2-8 wks after initial injury. Used to be commonly made diagnosis around US civil war time. Much less seen now, likely mis-diagnosed in past. More aggressive surgery today with surgery that can expose uvea. Dalen-Fuchs nodules are the classic finding that’s been described in the fundus.

Therapy includes topical steroids and intravitreal and other immunosupressants. Is the trauma more of a red herring? 

Case 2:

25 yo WF poor vision OD, diagnosed by resident with macular hole. Colour photos show circular change both maculas but more diffuse retinal change is the pathology. Early hypofluorescence with later hyperfluorescence on angiogram. This is the pattern of inflammation as part of white dot syndromes including AMPPE, MEWDS, Birdshot, etc. - AMPPE

AMPPE multiple cream coloured lesions, equal prevalence male & female with good outcome. MEWDS tends to be more in myopic, female, younger and is uniocular with enlarged blindspot b/o papillitis. Birdshot, is a terrible form of uveitis that comes back and is difficult to control with strong HLA A29 correlation. Multifocal choroditis looks like Histo but has inflammation associated with it. POHS caused by an infectious agent. Serpigenous also very aggressive, starting peripillary or macular then spread.

Case 3:

24 yo WF shows the FA of another white dot syndrome unilateral in young female: MEWDS

Case 4:

16 yo F who noted large blind spot suddenly right eye, saw 2 ophthalmologists recently. Fullness to disc with margins not as distinct affected eye. Both right and left eye can tell myopic fundi based on the FA. On dilated fundus exam, can actually see lots of white dots, many near the disc. Another case of MEWDS.

Case 5:

15 you BF decreased VA x 2 days with VA 6/9 and 6/120. Scarred retina central burnt out area with creamy white infiltrate at all borders of the lesions. Other eye normal til dilate and look nasally were see active white fluffy border. Serpigenous Choroidopathy…a destructive, chronic condition. This does poorly. But, this seems young for serpigenous, so did limited workup done to check for Syphilis which showed +ve FTA-ABs.

Case 6:

37 yo hispanic male more of a vitritis, definite focii inflammation in the retina. Snowballs inferiorly in the better eye. Bad eye had a hypopyon. Systemically had penile rash. Diff Diag: Bechets’, Tb, Syph, Sarcoid, Lyme and turned out to be Syphilis. Afterwards did learn that he was knowingly HIV +ve as well.

Case 7:

Another case this time subtle subretinal lesion but once dilate see white dots and peripapillary atrophy more easy to appreciate. This is CNVM, young patient so consider POHS. No vitiritis with this condition. Know the organism.

Case 8:

6/24 vision with peripheral vasculitis with adjacent old scar. Fellow eye very healthy. This patient has toxoplasmosis treated with clindamycin and pred along with topical tx. Not all reactiviated from childhood, can be acquired.

Case 9:

46 yo animal control officer with swollen nerve one side. FA shows hot spot on optic disc. Sarcoid, syph, Tb, toxo. Eveolved to be more obvious as cat-scratch (Bartonella)

Case 10:

40 yo M worked up for PSCC and noted changes in fundus. White lesions along blood vessels; white lesions with haemmorhagic centres along blood vessels. CMV retinitis. Can treat with oral valganciclovir. Can get very bad pan-retinitis when immune system working again.

Also mentions PORN (progressive outer retinal necrosis)

Case 11:

Referred by optom with central serous chroid retinopathy. But, angiogram shows cystoid macular edema.

 

 

1316hrs: Retinopathy of Prematurity (Khaled Tawansy)

1316_Tawansy

 

History: 3 week old hispanic male born 29.5wks gestation and seen 32.5 weeks post conception. VA LP, trace TVL, dilated post-pole vessels with some communication between the vascular arcades, ghost vessels, finger-like projections of retinal vessels.

Diagnosis: ROP? FEVR? Oxygen Toxic Retinopathy? Trauma? Something else?

Additional history: can’t really talk to mom as intubated in ICU. Has lupus and stopped all her lupus meds. Ultimately died 3 months after delivery. Found immune complexes in mom’s vessels. Feel therefore an immune related disease on top of ROP for this baby.

Treatment: did not know about this lupus vasculitis at first, otherwise might have treated for that. Based on BEAT-ROP study, did inject Avastin 0.6mg OS.

Three ROP variants: Typical ROP that responds to the study protocols, an aggressive posterior form, and a smouldering form. This last form never gets to threshold and the detachment can occur much later on, eg as a teenager. 

Biphasic retinal vascular development: vasculogenesis and angiogenesis both explained.

Compared risk factors between regular ROP and the smouldering form. Odds ratio favours smouldering form if some CNS abnormality. These patients need to be followed beyond the expected 45 weeks for late development of the ROP complications.

Now in our Anti-VEGF era, many of these cases may need close follow-up for injections.Talks about the BEAT-ROP study that used avastin as a treatment for ROP. 

Lessons from the BEAT-ROP study:

 

  • laser vs avastin stage III zone I and IIIp
  • prospective randomized
  • FDA approved with many site visits
  • primary outcome recurrence stage III plus
  • vascularity goes way down within 24hrs post injection
  • avastin not nearly as effective to salvage eyes that already had laser as it is primarily
  • neovascularization at the ridge resolves at 1-2wks with peripheral vessels crossing the ridge
  • growth of intrinsic vessels is delayed making follow-up more critical as wait for peripheral perfusion
  • rate of recurrence lower with avastin and occurs at two sites: leading edge and the original fibro-vascular ridge
  • advantage avastin vs laser far better zone I eyes than zone II eyes
  • some eyes will vascularize to ora
  • when detachments occurs after avastin, typically more dry and fibrotic which may be easier to operate
  • myopia and VF loss may be reduced in Zone I cases

 

1300hrs: Pediatric Retinal Surgery (Khaled Tawansy)

Original training at University of Michigan, fellowship at UBC and two others since then. Now a leader in pediatric retinal surgery. Both Drs Merkur and Albiani did additional training under his guidance after their fellowships here.

1300_Tawansy

 

Two talks based on two patients. Juvenile retinoschisis is the first case.

This first one has to do with a 2 m.o. baby with eyes that started to wander upward. Fundus looks like has traingular areas of elevation with loops of vessels. FA helps show dome shaped elevation. This is Malignant Bullous Schisis. Usually presents before 18 m.o. with schisis that marches posteriorly to eventually often involve macula. Retina scrolls up into a fold. 

Think X-linked Schisis when see vitreous heme, strabismus, amblyopia. Retinoschisin the involved protein mutation. A transmembrane protein involved in cell to cell adhesion. 

Foveomacular schisis has several clinical presentations, one of which is the cartweel appearance at the fovea. If get foveal displacement, it does so nasally. 

OCT often helpful to define the schisis area and also can show peripheral areas that might otherwise be missed. The areas that split correspond with the distribution of the retinoschisin protein in the eye.

Surgical indications include: vit heme, intra-schsis heme, bullous cavitty, progression to macula. Sometimes hard to tell where schisis ends and traction begins if retina detaching.

Back to the 2 m.o. patient who has lost fixation form schisis cavity extension. What are our options? Observe, intravit inj’n, laser, buckle, PPV, something else.

For management, presents a 3 week old with schisis and family history of exon 4 deletion. Shows how the retina starts to scroll off to the macular area. Quoting his mentor, Dr Ross, he opts for conservative approaches first, starting with a buckle, drainage of fluid, silicone oil. Child now stable more than 5 years and one of a series now collected who all had same exon 4 mutation and had surgery in first 2 years of life. All have 6/12-6/30 final acuity. Oil removed in 44%.

Now, back to the 2 m.o patients, management opted for was the encircling band, external drainage fluid. Careful not to create OUTER retinal hole in this process. Lens-sparing vitrectomy. Child has regained central fixation. What about the left eye for this patient? Here retina so bullous it is touching in many areas. Opted for injecting Plasmin and keeping patient in prone position.

Spontaneous reversal of schisis is felt to be something that can occur. Can see a ‘high water line’ indicative of where the fluid line was in the schisis cavity. 

Has also seen some kids with thick exudates in their schisis cavitis. These have all been refractory to the standard surgical techniques already discussed. Requiring re-operations with relaxing retinal incisions and not as favourable in terms of visual prognosis.

Summary. Think of schisis in boys when see vit hem or vision changes. Many variations and may not appear in 1st year with Exons 1-3. With Exon-4 there are more malignant manifestations. There is also the Exudative variant that is particularly difficult.