This is a 7-year-old girl who presented with a cataract and congenital aniridia. The cataract was extracted through a scleral incision and a 1 piece foldable IOL was implanted.
Surgery location: on-board the Orbis Flying Eye Hospital in Trujillo, Peru
Surgeon: Dr. Stephen Lane, University of Minnesota
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Dr. Stephen Lane: This is a 7-year-old little girl with congenital aniridia. You can see that she does have a little bit of an iris remnant here, a little bit here the strand but very little iris and I wasn’t able to gonioscope her in the clinic because she was very scared. So, one of the big problems with aniridia is that as the children get older, obviously, she’s got cataracts already, is vascularization and wipe out of their limbal stem cells. They can also have congenital optic nerve hypoplasia and macular hypoplasia. And so, it’s unclear at this point whether or not her decline in vision is due to the cataract or is due to one of those other problems that we’ve mentioned, until we take cataract out at least we can eliminate that as a possibility, so we’ll see if that occurs.
But importantly she is going to have to be watched long term for the development of limbal stem cell dropout and corneal vascularization and scleralization. The other thing that they can have are congenital problems that are systemic in nature. So we are going to stain this with trypan blue even though it’s a fairly good red reflex. Now unlike an adult I am going to do a scleral tunnell incision. But not going to do a large scleral tunnel, but more than just a clear corneal incision because I’m always concerned about children rubbing their eyes.
So I want to have a very secure wound. I go right behind the blue white junction here. We will be using a lens implant and so we’ll enter with the keratome. We could now take the trypan blue. I like this flat canula to use for the trypan blue because you can paint it onto the surface very nicely.
Ok now I’ll take some more Viscoat. And I’ll take the cystotome.
We’ll bend our needle and in children the thing that’s different about capsulorhexis is that, the capsule is much more elastic than it is in an adult. And so as a result, the size of the rhexis ends up always being larger than what you would have anticipated. So you can see how elastic it is and that’s why I like to use a capsulorhexis forceps now rather than a needle, because I can control the size better. So it’s important to go very slowly because these do tend to tear out. If necessary you can go back in with more viscoelastic to deepen and I’m not sure if you can appreciate how elastic this is but it’s almost like rubber.
Now we will hydrodissect and you don’t have to hydrodissect very much, the lens is going to be very soft. Now this is going to be mostly an aspiration type of procedure because the lens is so soft. As you can see there’s really no ultrasound being used here, it’s almost entirely being aspirated and I’m going to change to I&A very quickly.
So we’ll switch to I&A and get the rest of it that way.
Trainee: About the question you asked earlier, you asked about any other systemic problems that he may have. Dr. Lane: What was the answer?
Trainee: The answer is WAGR syndrome.
Dr. lane: Did you say wilms syndrome?
Dr. lane: That’s very good.
Trainee: The other one is the WAGR syndrome.
Dr. Lane: yes
In many respects removing a very soft lens is more difficult than removing one that’s very dense, because you can’t break it.
So there’s a little bit of cortex up above and again want to try and get this as clean as possible.
Trainee: Residents have a question. Why is the incision in the sclera and not in the cornea?
Dr. Lane: As I said before, in children because they have a greater tendency to rub their eye, I want to have a more secure incision than clear corneal.
So you’ll have a vantage point that you don’t usually have in most cataract surgery is that you’ll see the positioning of the haptics as they unfold.
Now because we’re working through the main wound we’re going to go ahead and hydrate the side port incisions because we aren’t going to need to get back to those very much. Now we will remove the OVD. So everything is a little smaller, the eyes a little smaller, so everything just has to be more careful.
So, I am going to put a safety suture in, and just basically need a single suture.
Trainee: Do you think there is some subconjunctival infiltration around the wound?
Dr. Lane: Just a little swelling from the fluid that we used to close the wound.
There we have it, at the end looks good.
July 28, 2017
2 thoughts on “Surgery: Phacoaspiration of a Congenital Cataract in an Eye with Aniridia”
1. Complete hydrodissection is always helpful in making the overall procedure easier and safer. Because these lenses are so often soft the lens nucleus during hydrodissection often pops up and out of the bag so frequent pushing of the lens posteriorly is a nice technique not so much to enhance the fluid wave but to “decompress” the bag to prevent forward movement of the lens/nucleus.
2. I would defend the use of a scleral incision in young patients even if they have aniridia and potential gimbal issues. The incision is small – 2.4mm in this case so significant disruption of the conjunctiva is minimal, cautery is light and the incision is more stable and watertight in the sclera than it is with a clear cornea incision in a young patient with low corneal/scleral rigidity. Scleral incisions also heal more quickly. These are all important factors in young patients who are more likely to rub their eyes and be involved in minor trauma around their eyes in the postoperative period. In adults I would have no qualms using a clear corneal incision.
3. I have found 7 year olds to be very cooperative at the slit lamp especially since the surgeon will have seen them on multiple occasions pre and post operatively. The child becomes very familiar and at ease with the doctor. In my experience PCO does not occur for several or more years and I have never had a difficult time with Yag. Because PCO can occur within weeks after surgery in younger patients (2 years of age and younger) and difficulty getting them to the Yag laser I do recommend the technique described or use of a posterior casulorhexis and prolapse of the optic behind the rhexis.
3 comments / differences:
1. A good 4 quadrant hydrodissection is useful in children / soft cataracts. Else, it takes forever to get the epinucleus and cortex out. I would especially target the superior sub incisional area during hydrodissection. Of course, you need to bear in mind that sometimes the nuclear material pops out of the bag in children but it is not a big concern. The technique of completing the hydrodissection by pushing the lens after the “wave” doesn’t work well in children.
2. I would prefer a clear corneal incision in aniridia. The use of cautery and suturing of scleral tunnel and conjunctiva creates more damage to the limbal area. The post-operative peri-limbal inflammation is higher with scleral tunnel. A sutured corneal incision will hold well in a 7 year old.
3. Aniridia is often associated with macular issues. photosensitivity and nystagmus. This makes Yag laser capsulotomy very difficult. I would have done a primary posterior capsulotomy with anterior vitrectomy in this case to eliminate the risk of PCO.