Hyphema/uveitis post cataract

[KHE]

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So I have this patient who is a long term diabetic who's not good about taking care of the diabetes or following up with appointments. I believe he's also a recovering alcoholic.

He's had treatment for PDR OU and injections. That of course caused a cataract which were removed about 4 months ago now. Vision after that was 20/40 OD and 20/30 OS. He did keep his one month follow up appointment after the surgery but I did see him after two weeks and he was doing well.

Today (Friday PM of course) he comes on an emergent basis complaing of loss of vision in the right eye for two weeks. Vision is light perception OD. 😱

So I look in and he's got a large uveitis and a grade 1 hyphema. IOP is 35. OS is quiet.

Gonioscopy showed some minor neovascularization but no obvious source of the hyphema.

The optic nerve looks similar to what it was at previous exams.

The patient then informs me that he had a toe amputated two weeks ago and has been taking morphine QID since then.

So based on clincal signs, it appears to be UGH syndroms but:

1) My understanding is that UGH syndrome usually occurs many months or even years after the cataract extraction. How common is it after 4 months?

2) My understanding is that in UGH syndrome, the IOP usually does not spike particularly high. This person obviously has a lot of damage to the TM.

3) I also thought that UGH does not normally cause such a substantial reduction in vision. What is the prognosis for any meaningful visual recovery?

Thoughts on this guy?

 

[Eye Doctor]

I'll give my 2 cents for what it's worth...still a resident though.

As for your questions:

1) I think it can happen any time after catarct surgery, especially if there are other risk factors (patient on coumadin, bad diabetic with fragile blood vessels, lens position, etc)

2) I think the increase IOP is probably related to a combination of the uveitis but probably more so due to the hyphema/bleeding and associated increase in IOP due to clogging of TM from the inflammatory cells/RBCs. You didn't mention if the patient's race - can the patient possibly have sickle cell disease/trait? - I would treat the pressure and IOP either way with steroids and IOP lowering meds.

3) I think UGH can decrease vision quite substantially especially if there are risk factors for large amount of bleeding. I had a patient with UGH and she was on coumadin (she was LP vision from vit heme as well as hyphema). I'm surprise you can even get a view to the nerve if the patient is LP vision. Your patient may also have macular edema/macular ischemia or combination of both. The visual potential probably will depend on his diabetic control and other factors if he does respond to conservative therapy. I think you should first get his IOP under control and get him back to his cataract surgeon - he may need an IOL exchange or removal.

 

[guttata]

Questions:

1. What type of lens was implanted? Posterior chamber in-the-bag, sulcus, or anterior chamber?
2. Was the lens dislocated?
3. Any findings on the DFE?
4. Any trauma?
5. Was there an APD?
6. Any possibility of sickle cell?
7. Is the patient on anticoagulants?

UGH syndrome is usually associated with older anterior chamber lenses rubbing against the iris. It is a pretty rare complication to have with a posterior chamber lens (happens if the lens is dislocated and chaffing the iris). It is fairly uncommon with the newer anterior chamber lens (haptic redesign).

Other things to consider: postsurgical hyphema (from the cataract incision site - vessels near the incision site can be fragile and bleed), PDR (and spillover from vitreous hemorrhage- very uncommon; or hyphema from the NVI), herpetic uveitis.

I would do a DFE and look for lens centration and PDR/vit heme.
I suspect he has a spontaneous hyphema from NVI.

Interesting case, please keep up updated.

 

[speyeder]

Questions:

1. What type of lens was implanted? Posterior chamber in-the-bag, sulcus, or anterior chamber?
2. Was the lens dislocated?
3. Any findings on the DFE?
4. Any trauma?
5. Was there an APD?
6. Any possibility of sickle cell?
7. Is the patient on anticoagulants?

UGH syndrome is usually associated with older anterior chamber lenses rubbing against the iris. It is a pretty rare complication to have with a posterior chamber lens (happens if the lens is dislocated and chaffing the iris). It is fairly uncommon with the newer anterior chamber lens (haptic redesign).

Other things to consider: postsurgical hyphema (from the cataract incision site - vessels near the incision site can be fragile and bleed), PDR (and spillover from vitreous hemorrhage- very uncommon; or hyphema from the NVI), herpetic uveitis.

I would do a DFE and look for lens centration and PDR/vit heme.
I suspect he had a spontaneous hyphema from NVI.

Interesting case, please keep up updated.

I agree with the above. UGH is very rare with PCIOLs. The key information you will want to obtain is:

1) What does the DFE show?
2) Is there an APD?

If there is little to no view to the posterior pole, do a B-scan. Look for the presence of vitritis, RD, etc.

If there is a view, an FA might be helpful to look at macular perfusion.

Vision dropping from 20/30 to LP is concerning.

I would not expect that with a 1+ hyphema. Maybe with an 8-ball hyphema.

At the very least I would refer the patient back to the cataract surgeon, if not to a retina specialist. You may be dealing with something more ominous than uveitis/hyphema.

 

[JMK2005]

I've seen problems occur when the one piece AcrySof IQ lens is placed in the sulcus. From the history and poor DM control, NVI is the most likely. A fluorescein of the iris may show NVI that is not readily apparent at the slit lamp.

Also, LP vision with 1+hyphema doesn't make sense either. NVG/PDR/DME can cause the 1+hyphema, uveitis, and LP vision.

 

[Mirror Form]

Interesting case. You didn't mention that there were any issues regarding the cataract surgeries so I'm assuming the patient does not have an AC IOL. Therefore UGH syndrome would be unusual.

Uveitis plus hyphema in an alcoholic is suspicious for trauma. But, make sure you check for other causes of uveitis and signs of previous uveitis too (you never know with these patients).

The timing is also pretty concerning b/c if it really happened two weeks ago, you'd think it must have been a lot worse then unless he had a re-bleed. But then the post-traumatic iritis typically would have at least gotten mostly better.

 

[medduck]

This does not sound like UGH syndrome and is most likely neovascular glaucoma.

When you say uveitis, you are sure those are white cells in the anterior chamber and not RBC's associated with the nva/hyphema?

I bet that angle is zippered shut. The iop is 35 and not 70 because of hypoperfusion to the ciliary body. It certainly could have been higher than 35 for the last few weeks and his nerve may be toast- he does have an APD right? He must or cannot be LP. What does his nerve look like?

Assuming this is NVG, I would tap the AC to get the pressure down immediately, inject an anti-VegF into the vitreous and put on maximum medical therapy to keep the IOP down, also frequent steroid and cycloplegia. If the IOP stays elevated, you could try goniosynechiolysis (sounds like fresh PAS)- if that doesn't work then would need tube or cyclophotocoagulation. He also needs more PRP if not full. When you said he's had injections, was that anti-VegF or steroid?

Unfortunately after all this, he'll still likely be LP.

Good case.

 

[KHE]

This does not sound like UGH syndrome and is most likely neovascular glaucoma.

When you say uveitis, you are sure those are white cells in the anterior chamber and not RBC's associated with the nva/hyphema?

I bet that angle is zippered shut. The iop is 35 and not 70 because of hypoperfusion to the ciliary body. It certainly could have been higher than 35 for the last few weeks and his nerve may be toast- he does have an APD right? He must or cannot be LP. What does his nerve look like?

Assuming this is NVG, I would tap the AC to get the pressure down immediately, inject an anti-VegF into the vitreous and put on maximum medical therapy to keep the IOP down, also frequent steroid and cycloplegia. If the IOP stays elevated, you could try goniosynechiolysis (sounds like fresh PAS)- if that doesn't work then would need tube or cyclophotocoagulation. He also needs more PRP if not full. When you said he's had injections, was that anti-VegF or steroid?

Unfortunately after all this, he'll still likely be LP.

Good case.

Wow. So many questions....let's see if I can get them all.

1) APD - very hard to tell. Pupil never reacted well on either eye since I've seen him so watching for any dilation was not easy. Because of the LP vision, I too expected an APD and basically convinced myself that it was there but certainly not obvious.

2) Patient is Hispanic. No sickle cell or sickle cell trait. At least, he denied it and so did his daughter.

3) I tried to consult the cat surgeon but he was already gone for the day. (Jewish Holiday.) The two retina specialists were also gone because apparently a staff member in the practice was getting married. In the end, I made an appointment for Monday morning with the retina guys.

4) No trauma. Nothing slapping in the face with DFE other than the PRP marks. PCIOL is clear and appears in the bag.

5) The vision loss is definitely not from the hyphema. It's only grade 1.

6) As far as white cells vs red cells, it did not look like the RBCs from hyphema that I have seen before. It was more consistent with uveitis but this person has had a lot of treatment both anterior and posterior chamber so I'm not the most experienced guy at telling what's coming from where with this complex a case.

7) I did put a 4 mirror on but there was some corneal edema and poor cooperation from the patient. From the fleeting views I got, the angle did not appear "zippered shut" but I would not be confident in commenting on the presence of any neovascularization of the angle.

I cyclopleged him, started him on pred and Timolol 0.5, alphagan and azopt. An hour later, the IOP was down to 29.

His daughter refused to bring him in tomorrow to check the IOP so they have an appointment Monday with the retina specialist.

After his cat surgery in June, he did have some elevated IOP (29) at the one week visit. Combigan brought that down to 15 at the two week visit and at that time his steroid schedule from the surgeon indicated (IIRC) another week to 10 days of steroid treatment. I told him to continue taking the combigan as long as he was taking the steroid and we would see him in two weeks for the one month visit. He was lost to followup until today due to the toe amputation.

I'll post when the retina specialist calls on Monday.

 

[JMK2005]

So it appears the LP vision is still a mystery. My guess is a CRAO.

 

[medduck]

Sounds like a good treatment plan.

One thing going against CRAO is the subacute nature of the vision loss- you said the patient complained of vision loss for 2 weeks, not sudden loss of vision 2 weeks ago, correct?

 

[Mirror Form]

5) The vision loss is definitely not from the hyphema. It's only grade 1.

I wouldn't be so sure of that. A grade 1 hyphema means that the AC is packed with RBC's, and that can easily cause count fingers to HM vision. LP is still a stretch though.

You mentioned that you did a gonioscopy and did not see any obvious source of the hyphema and that the angle did not appear zipped shut. Maybe it's partially zipped shut in some quadrants? One thing that I thought of later was that he may have had a CRVO after cat surgery and is now presenting with his 90 day glaucoma.

 

[KHE]

Sounds like a good treatment plan.

One thing going against CRAO is the subacute nature of the vision loss- you said the patient complained of vision loss for 2 weeks, not sudden loss of vision 2 weeks ago, correct?

No, sorry. It WAS a sudden loss of vision. Not a gradual loss. So CRAO is a good bet.

 

[KHE]

I wouldn't be so sure of that. A grade 1 hyphema means that the AC is packed with RBC's, and that can easily cause count fingers to HM vision. LP is still a stretch though.

You mentioned that you did a gonioscopy and did not see any obvious source of the hyphema and that the angle did not appear zipped shut. Maybe it's partially zipped shut in some quadrants? One thing that I thought of later was that he may have had a CRVO after cat surgery and is now presenting with his 90 day glaucoma.

My understanding of grade 1 hyphema was that it related to how much blood was on the cornea. It was a small amount coming no where close to the pupil. Also, I was able to get a reasonably decent view of the optic nerve so I would think that if the vision was decreased to that extent from hyphema that I would have as much of a hard time seeing in as he was seeing out. Is that not accurate?

It may have been zippered shut in some quadrants. Like I said, it was a 4 mirror gonioscopy on an uncooperative patient with a fairly unclear cornea and fleeting views. I like to think I'm reasonably good at gonioscopy but this one was certainly not going to be my time to be the shining star. It was almost a leap of faith.

I too was thinking of a possible CRVO from elevated IOP. But given that the vision loss was two weeks ago I would think that it would have been the glaucoma first then the CRVO, not the other way around. For the CRVO to have occured first, would it not take longer for the pressure to rise?

 

[Mirror Form]

My understanding of grade 1 hyphema was that it related to how much blood was on the cornea. It was a small amount coming no where close to the pupil. Also, I was able to get a reasonably decent view of the optic nerve so I would think that if the vision was decreased to that extent from hyphema that I would have as much of a hard time seeing in as he was seeing out. Is that not accurate?

That is what a grade 1 hyphema is (less than 1/3 layering). And you usually can get a decent view of the posterior pole and optic disc. However, many of the grade 1 hyphemas that I've seen presented with very low visual acuity. In order to have layering, you have to have a lot of rbc's floating around in the AC.

Lets face it, spontaneous hyphemas are pretty rare. So he may just be an alcoholic who had blunt trauma when he mixed his whiskey with morphine and didn't remember it. If we take the patient's word that he didn't have trauma, then the most likely causes of spontaneous hyphema in this patient are very severe iritis or rubeosis iridis. Of course he may have some really rare blood dyscrasia, neoplasm, or vascular anomaly too.

I too was thinking of a possible CRVO from elevated IOP. But given that the vision loss was two weeks ago I would think that it would have been the glaucoma first then the CRVO, not the other way around. For the CRVO to have occured first, would it not take longer for the pressure to rise?

Well, once again, I have a hard time putting too much faith into the history provided by a patient who waited two weeks to come see you for "sudden vision loss." He has two good eyes, and may not have noticed his unilateral vision loss initially.

One possibility is that he got severe rubeosis iridis from either a CRVO or poorly controlled DM (or both). That could lead to a spontaneous hyphema. And his IOP rise is could be completely due to the hyphema, and not related to a closed angle glaucoma. But then the severe uveitis is still a mystery . . .

Well this is just a great case! Please give us updates!

 

[orbitsurgMD]

So I have this patient who is a long term diabetic who's not good about taking care of the diabetes or following up with appointments. I believe he's also a recovering alcoholic.

He's had treatment for PDR OU and injections. That of course caused a cataract which were removed about 4 months ago now. Vision after that was 20/40 OD and 20/30 OS. He did keep his one month follow up appointment after the surgery but I did see him after two weeks and he was doing well.

Today (Friday PM of course) he comes on an emergent basis complaing of loss of vision in the right eye for two weeks. Vision is light perception OD. 😱

So I look in and he's got a large uveitis and a grade 1 hyphema. IOP is 35. OS is quiet.

Gonioscopy showed some minor neovascularization but no obvious source of the hyphema.

The optic nerve looks similar to what it was at previous exams.

The patient then informs me that he had a toe amputated two weeks ago and has been taking morphine QID since then.

So based on clincal signs, it appears to be UGH syndroms but:

1) My understanding is that UGH syndrome usually occurs many months or even years after the cataract extraction. How common is it after 4 months?

2) My understanding is that in UGH syndrome, the IOP usually does not spike particularly high. This person obviously has a lot of damage to the TM.

3) I also thought that UGH does not normally cause such a substantial reduction in vision. What is the prognosis for any meaningful visual recovery?

Thoughts on this guy?

Not likely UGH, unless there is a history of complicated cataract surgery, misplaced or sewn-in haptic, etc.

More likely this is a hemorrhage following iris neovascularization, itself following a vascular event, probably a CRVO. Alternatively, he may have PDR that was unrecognized, although my money is still on occlusion. He also probably has NVG as well, especially if you saw any NV in the angle.

BTW, there is no such thing as "minor neovascularization." All NV is a sign of ongoing ischemia.

It might be good to know if there is a new relative afferent pupillary defect. Past retinal disease could have already created this finding, however. If there is iris NV, the pupil may not react well in the hemorrhaging eye and you may have to examine the pupillary response in the fellow eye and compare direct with consensual response there.

 

[Visionary]

I agree that this does not sound like UGH. The high IOP and hyphema are likely the result of rubeosis.

I also agree that the vision may have been down longer than 2 weeks. In binocular patients, monocular vision loss can exist for weeks to months before being detected (or acknowledged). This is particularly true with non-compliant patients. Furthermore, badly-controlled diabetics have notoriously poor mentation.

Overall, this sounds most like NVG in the setting of CRAO. CRAO can also result in rubeosis and NVG, though it is less common than with CRVO. I've seen it twice in the past year, however. For heme from an ischemic CRVO to completely disappear in such a short period would be unusual. You said cataract surgery was only 4 months ago with 20/40 post-op BCVA. Now VA is LP with an unremarkable fundus exam, except for PRP scars. CRAO typically has little heme, and the associated retinal whitening can disappear within weeks. I suspect OCT will show severe macular thinning (atrophy), even if FA is relatively normal.

Patient will likely need intravitreal anti-VEGF, followed by fill-in PRP. Unfortunately, I've found CRAO-associated NVG to be much more difficult to control. CPC may be necessary, eventually.

 

[eyescold]

This sounds more acute/subacute, but I might wonder about ocular ischemic syndrome with the cluster of NVI, anterior uveitis, and LP vision, especially in a vasculopathic patient with diabetes.

 

[DiveMD]

I agree that UGH is unlikely. The history of PDR ou is suggestive of NVI/NVA as the likely culprit of the hyphema. The inflammation and IOP spike are probably secondary to the hyphema and questionable angle integrity. The dropped VA is probably the results of a hyphmea along with posterior pole pathology. (I.e. worsening CSME, vit heme). This patient probably has an "Avastin-deficiency". 😉

 

[OMD]

Also, the IOP was 35 with a soft, edematous cornea, so it's higher than measured. I'm betting on full PRP if not full yet or other ablating procedure, IVA, and eventual tube shunt.

 

[KHE]

Ok, so the report came back from the retina specialist with the following notes:

Vision was 6/400
IOP was 26 on steroids and glaucoma meds

Great delay in arteriole and venous transit times on FA. Overall ischemia of the retina. No frank evidence of rubeosis.

Diagnosis is listed as

New central retinal artery occlusion with combined ocular ischemic syndrome.

The plan is to do PRP on the right eye though the liklihood of regaining vision is negligible.