Wagner: This session is on surgical management of Brown syndrome. I am going to describe what I consider to be a typical case of Brown syndrome that would end up being a surgical case. A 3-year-old child had unilateral Brown syndrome in the right eye with significant chin-up face position and left face turn. There was a right hypotropia of 6 prism diopters in primary gaze that increased significantly in gaze to the left. There was also an underaction of the elevation in the right eye in the adducted position, as you would expect. The parents believed that the problem was greater in the left eye because they saw an overelevation of the left eye when the child attempted to gaze to the left and up. How would you manage this case?
Dotan: Most cases of Brown syndrome do not need surgery. Only a minority of children have symptoms severe enough to require surgery. In my opinion, surgery is required for hypotropia in primary position, an anomalous head position, or severe down shoot in adduction. In the case you described, I would consider surgery.
Wagner: Do you think this is a straightforward surgical case?
Raab: Yes. If the child has a chin-up position, I expect to measure hypotropia in primary gaze. If there was no hypotropia, the child would just turn his head to look up.
Deutsch: Although studies have published good results with several techniques, it's still difficult to get a good result in these cases. I agree with Dr. Dotan that you should not operate unless you have to, but I also agree that this is a surgical case because the condition is going to limit his ability to live his life.
Wagner: Once you determine that this is a surgical case, what would be your initial plan for surgery?
Deutsch: I would do forced ductions in the operating room. I think it's important that I'm not able to pull the eye up before the surgery and that I can move it up after I've finished. I vary between doing a spacer technique that Wright and others have described and doing a chicken stitch with a polyester suture with a superior oblique tenotomy.
Raab: I am a disciple of Marshall Parks, and he would routinely weaken the inferior oblique in the same procedure as the superior oblique weakening. I don't use a chicken stitch, although I have no argument with those who do.You said that you wanted to describe a typical case, but there is such thing as pseudo-Brown syndrome. With a positive forced duction result, you have to watch out for something like the rare Johnson adherence syndrome. There is such a thing as inferior oblique palsy, but it's not usually neurogenic. For me, the clinical distinction is that all of the original cases of Brown syndrome described by Brown himself had a large divergence in up gaze. I think you only get that with true Brown syndrome.
Deutsch: I agree that you definitely don't see a large divergence in up gaze with an inferior oblique palsy because the inferior oblique tendon would pull out more in up gaze. However, the most classic Brown syndrome that I've seen was after a child was hit in the face with a soccer ball. It turned out to be an entrapped inferior rectus tendon and it did splay in up gaze.
Dotan: This condition is not a disease, it's a syndrome that encompasses several things. My preferred approach is the split tendon elongation and I do not routinely weaken the inferior oblique muscle.
Wagner: Do you use a spacer?
Dotan: No. I split the tendon and suture the pieces in a Z-shaped fashion with a polypropylene 8-0 suture.
Raab: I completely transect the tendon but not the surrounding tissues.
Wagner: Do you purposely remove a section of it or just cut it?
Raab: My equivalent of the chicken stitch is not to remove a section. I just transect it. I wouldn't say I always weaken the inferior oblique tendon, but most of the time I do.
Wagner: When I first used to just do the superior oblique tenectomy, I used to see inferior oblique overaction develop. It doesn't happen right away. It could take a year before you see it in its full-blown form. Perhaps that's less likely to occur with the procedure Dr. Dotan described or with a spacer.
Deutsch: Several reports in recent years did not find inferior oblique overaction after surgery. Marshall Parks really pushed that. I trained with Dr. Raab and that's what I did when I started out in practice, but more recently I have not.
Dotan: I explain to the parents that the child might develop inferior oblique overaction in the future, but I would rather correct that later than do both procedures initially.
Raab: It all goes back to you don't operate these cases if you don't have to.
Wagner: Exactly. I think that's the most important thing. Most of these patients have good binocularity. Of course those are the cases in which you have the most problems if they're not where they're supposed to be, especially once you do the superior oblique. When I presented the case, I did say that the parents noticed the opposite eye overelevating.
Raab: Every time.
Wagner: Have you ever had a reason to weaken the superior rectus tendon in the opposite eye?
Deutsch: Not yet.
Raab: If you're lucky enough to get rid of the chin-up position in primary gaze, which means you got rid of the hypotropia, all the patient will have is some leftover inability to get up in up gaze. You never get rid of all of it. But no one uses up gaze by directing the eyes up. You direct your eyes down to read, but when you're looking up, you are essentially in primary position looking up. So I wouldn't use that as a reason to do the superior rectus.
Dotan: I agree. You should explain to the parents why you are doing the surgery, which is to eliminate the head posture. You tell them you cannot get rid of the problem completely in adduction, so that they won't have an unrealistic expectation. They need to know that we are doing the surgery just to correct the head position.
Deutsch: I worry about creating a vertical deviation by going to the opposite superior rectus.
Wagner: I have seen at least one case of what I describe as transient or reversible Brown syndrome where there probably was a nodule in the superior oblique tendon, which was going through the trochlea and intermittently getting hung up. Have any of you seen that?
Raab: No. It's been described but I have not encountered it.
Dotan: I've seen such a case. A patient was admitted to hospital because of double vision. When I saw him, everything had resolved and he had completely normal eye movements. Then he went to get some coffee and he called me to come and see him again. He had complete elevation limitation in adduction, but it was intermittent.
Wagner: Did you eventually do surgery?
Dotan: Yes. We injected methylpredinisolone to the trochlea, which resolved his symptoms.
Wagner: Is that what they call the canine tooth syndrome? I think that's when you've had a trauma to the trochlear area.
Raab: It's usually trochlear trauma through the eyelid.
Wagner: That could then damage the movement of the superior oblique tendon through the trochlea.
Raab: Marshall Parks told us that when you're going to operate on any type of Brown syndrome you should explore all six muscles. You make a lateral inferior fornix incision and you get a hook under the lateral, a hook under the inferior oblique, and a hook under the inferior rectus. Through an upper nasal incision, you get the superior rectus, the superior oblique, and the medial, just to see what they look like. I've reported where the apparent Brown syndrome was caused by abnormal connections between the superior oblique tendon and the superior rectus capsule. That gave you, in effect, a short superior oblique tendon, which is what Brown syndrome is all about. Lysing those connections without anything on the superior oblique or the superior rectus got rid of everything. But that's rare.
Wagner: Yes, but it's a good point. I think most ophthalmologists prefer not to operate on these cases unless they have to. I agree that surgery does not result in what I would call a cure for this condition. Most patients have some residual, but if you get them in good face position, you've achieved the best result you can.
This Eye to Eye session was conducted on Monday, April 3, 2017, during the annual meeting of the American Association for Pediatric Ophthalmology and Strabismus in in Nashville, Tennessee.