Brown’s, Horner’s syndromes: When to watch and when to work up

In the third and final excerpt from a round table held during the AAPOS meeting, members of the OSN Pediatrics/Strabismus Section discuss how to approach the diagnosis and treatment of Brown’s and Horner’s syndromes.

Robert S. Gold

Robert S. Gold, MD: One strabismus problem that is not uncommon in our practices is Brown’s syndrome. How do you manage a child with congenital Brown’s syndrome?

Brown’s syndrome

Kenneth P. Cheng

Kenneth P. Cheng, MD: First, I explain to the parents what Brown’s syndrome is. If it is not causing any abnormal head posturing and if the eyes are well-aligned in useful gaze positions, then I do not treat it. I tell the parents that the surgical treatment, a tenotomy, can be unpredictable, sometimes resulting in undercorrection, yielding little improvement, and sometimes resulting in overcorrection and inferior oblique overaction. If the misalignment is only present in contralateral upgaze and if the eyes are still straight in horizontal contralateral gaze, then I definitely want to be more conservative regarding surgery in those patients.

Dr. Gold: Consider a patient with a significant chin, head posturing and significant hypotropia. What techniques other than tenotomy do you use? Anyone use tendon expanders?

Roberto Warman

Roberto Warman, MD: I use the tendon expander in the few cases that need treatment. I see a lot of Brown’s syndrome, and I agree that it is a minority who need surgery. Even then, I try to wait until the child is 4 or 5 years of age to achieve as much stereopsis as possible. I even allow the chin position at the beginning because it is not crucial. When I do go in, however, I use a tendon expander, and I have had good, regular and bad results.

Scott E. Olitsky

Scott E. Olitsky, MD: There would have to be pretty significant head posture to operate on an infant. If the child is doing well and binocular vision is developing, I explain to the parents that waiting might be good, even knowing that some corrective measure will be needed. My approach is the same as Dr. Cheng’s. I do a temporal tenotomy. I just have not been happy with some of the expanders. We can probably pick any treatment and all say the same thing: good, not so good and bad. There is not one treatment that will make us all happy.

Dr. Gold: Especially when you rarely do surgery for this, it is crucial to discuss the options in great detail with the family. I normally use a tendon expander. That is just my preferred procedure. But, again, it does not always achieve a satisfactory result.

Dr. Warman: Because it is not seen too often, I am 100% conservative in cases of bilateral Brown’s syndrome. I have followed perhaps seven or eight cases over the years. These patients have compensated well enough, but they are hampered in the sense that they have to move their heads from side to side. Bilateral Brown’s syndrome is one case I just never wanted to do surgery on because of the unpredictability of the results.

Dr. Cheng: In the literature, there are reports of cases that have resolved spontaneously or improved significantly over the course of long-term follow-up. I have observed a number of children with Brown’s syndrome who have improved or resolved after long periods of time.

Dr. Gold: The longer I am in practice, the more I see that happening as well. I will tell the parents that, in a rare situation, we will see this go away. Don’t count on it, but we may.

Anthony P. Johnson

Anthony P. Johnson, MD: I draw a tic-tac-toe board to demonstrate to the parents all the different positions of the gaze. I point out that if the alignment is only abnormal in gaze up, then a “grand slam” result would be to eliminate any kind of misalignment in all of the tic-tac-toe boxes. However, what we could do is eliminate the problem where it is and create a problem in primary gaze. Most of the time, the parents will accept that. I point out that young children are always looking upward because they are not very tall. But as long as mom is standing on the correct side of the child to see the gaze up and to the contralateral side of the Brown’s, she is going to see it and it is like a knife through her side. I do not downplay their concern; I try to validate them. My treatment system spends more time talking about it than doing anything about it.

Dr. Gold: One thing that happens almost universally in Brown’s syndrome, let’s say in the left eye, is that the parents almost always say that there is a problem with the right eye. I instruct my technicians to always look in the other eye, because more than likely that is where the problem is. You have to look at the motility in both eyes.

Horner’s syndrome

Dr. Gold: Let’s talk about evaluating infants with Horner’s syndrome or anisocoria, starting with a case in which parents have been referred by their pediatrician because the infant has one pupil larger than the other. They are worried. If you think it is Horner’s syndrome, what would you do to work that up?

Dr. Johnson: At least 95% of the time just on clinical exam, you can tell that it is an isolated case of either ptosis or anisocoria. I see that a lot in the office, probably three or four times a week, more likely ptosis than anisocoria. The question is whether or not it is a case of Horner’s syndrome. If it is a case of Horner’s, is it congenital or acquired? We know that acquired Horner’s in the preschool age group is very uncommon, but we all have the fear of a neuroblastoma. In one experience that is indelibly etched in my mind, the mom called my office and said that her 2-year-old had new onset of ptosis that had started 8 days earlier. In this case, the child did indeed have a neuroblastoma of the thoracic sympathetic chain.

This is very frightening for families when it comes up, and it comes up frequently. On the one hand, we want to be reassuring; on the other hand, we cannot afford to miss the diagnosis. If I am concerned at all, I skip all of the intermediate steps of analyzing first-, second-, third-order neuron and that sort of thing with drops, and I go to MRI of the brain and chest X-ray. I go right for the meat of looking for something. I do not do that very often, but that is my work-up if I feel like there is any question at all.

Dr. Cheng: When the clinical scenario indicates acquired Horner’s, then we would all agree that a work-up is required, not only MRI and chest X-ray, but also urinalysis. The cases that are problematic for me are when the baby has Horner’s, but it is unclear whether the case is congenital or acquired. Excluding history of birth trauma, the overwhelming majority of these are going to be congenital lesions. Although the baby is young and the mom says it has been present since birth — could it still be an infantile neuroblastoma? The chances are exceedingly small. In Pennsylvania, babies undergo perinatal screening or newborn screening that should theoretically pick up a lesion. Yet, I am still faced with reports in the literature saying there is a need to work up all these babies. The problem is that, basically, the stem-to-stern MRI requires extensive sedation and is very expensive, and the yield is going to be infinitesimally small. I have to come to grips with this. If the history goes back to birth, then I am comfortable watching those patients.

Dr. Warman: The first thing, the crucial thing, is to make sure it is Horner’s. Most cases that come in are not, which should be reasonably clear by clinical diagnosis. If you are not absolutely sure, sometimes it is better to have the patient come back in 2 weeks when the child may be more quiet or cooperative. You do not lose anything by waiting a couple of weeks. But the diagnosis is a clinical diagnosis, and pharmacological testing is not very accurate or reliable in infants. We almost never have cocaine drops on hand, and even when you use them, it is very difficult to put the exact same amount on each eye in an infant. So I do not use them. Either I am convinced or I am not convinced that it is Horner’s. Once I am convinced that it is Horner’s and I reasonably determine that it is congenital and not worsening, then I do not think the child needs any treatment. I agree that data are showing that neuroblastoma is rare, so when the case is clearly acquired, also look at the neck for dissection of the aorta. Even in young children, maybe not infants but slightly older, aortic dissection is much more common than neuroblastoma, and you really do not want to miss that because there may be no other symptoms.

In a study by Smith and colleagues published in Archives of Ophthalmology, the researchers report seeing zero cases of neuroblastoma in a population-based study of pediatric Horner’s syndrome. The conclusion of this paper is to be very selective on the work-up rather than doing MRI of head, chest and abdomen in all cases. If we order such a work-up once every couple of years, that is probably reasonable.

Dr. Olitsky: If clinically I am convinced that the child has Horner’s syndrome, then I order imaging. We cannot say for sure if it is congenital or maybe congenital infantile neuroblastoma. So I image all of them.

Dr. Cheng: What if the mom says, “I’ve noticed this since birth,” and the patient is now 9 months old. Would you still image that patient?

Dr. Olitsky: What if the patient is 7 months old? The point is that I do not know what the cutoff is, so I image all of them.

Rudolph S. Wagner

Rudolph S. Wagner, MD: The dilemma that I have is when a baby has anisocoria as the main finding, but on examination you do not find ptosis. You turn out the lights and observe a dilatation lag. My question is, are you concerned from the clinical examination in a child with anisocoria but no ptosis that this could possibly be Horner’s syndrome? How do you prove the diagnosis in a baby with these findings?

Dr. Cheng: It is very difficult in some patients to determine whether dilatation lag is present, especially when the iris is a very dark brown. I do use cocaine testing to try to confirm Horner’s if I am not sure. It is difficult to get, and I write a prescription for the drops, so the parents have to acquire the drops. I tell them, “This isn’t going to do any good on the street. It’s not good for anything else.” The parents have to go to the hospital to have the formula made or order it through a compounding pharmacy. Iopidine (apraclonidine 0.5%) is great for older patients, but it cannot be used in a baby younger than 6 months of age.

Dr. Warman: Getting back to the case of anisocoria but no ptosis in an infant, I have the patient come back. It is difficult to see in a little baby, so if everything else is fine, I have them come back in 1 month. But to do further testing, you have to find some ptosis there; if not, we would be doing a lot of tests on a lot of patients.

Reference:

• Smith SJ, Diehl N, Leavitt JA, Mohney BG. Incidence of pediatric Horner syndrome and the risk of neuroblastoma: a population-based study. Arch Ophthalmol. 2010;128(3):324-329.

• Kenneth P. Cheng, MD, can be reached at 1000 Stonewood Drive, Suite 310, Wexford, PA 15090; 724-934-3333; e-mail: kpc123@verizon.net.
• Robert S. Gold, MD, can be reached at 790 Concourse Parkway South, Suite 200, Maitland, FL 32751; 407-767-6411; fax: 407- 767-8160; e-mail: rsgeye@aol.com.
• Anthony P. Johnson, MD, FACS, can be reached at Jervey Eye Group, 601 Halton Road, Greenville, SC 29607; 864-458-7956; fax: 864-458-8390; e-mail: apj@jervey.com.
• Scott E. Olitsky, MD, can be reached at Children’s Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108; 816-234-3000; fax; 816-346-1375; e-mail: seolitsky@cmh.edu.
• Rudolph S. Wagner, MD, can be reached at Children’s Eye Care Center, 1 Clara Maass Drive, Belleville, NJ 07109; 973-751-1702; fax: 908-665-8482; e-mail: wagdoc@comcast.net.
• Roberto Warman, MD, can be reached at Miami Children’s Hospital, 3200 SW 60th Court, Suite 103, Miami, FL 33155-4072; 305-662-8390; fax: 305-661-7862; e-mail: rwarman@eyes4kids.com.
• Disclosure: No products or companies are mentioned that would require financial disclosure.