Ophthalmologists must recognize signs of shaken baby syndrome

Any ophthalmologist may be called on to document inflicted childhood neurotrauma, round table participants say. Part 1 of two parts.

Introduction:

At a round table convened at the American Association for Pediatric Ophthalmology and Strabismus meeting earlier this year, Ocular Surgery News Pediatrics/Strabismus Section members and guest participants discussed two important topics in pediatric ophthalmology. In this issue we present part 1 of the round table, the ophthalmologist’s role in diagnosing and documenting shaken baby syndrome.

Next issue will contain part 2, an update on amblyopia.

MODERATOR Robert S. Gold, MD the Pediatrics/ Strabismus Section Editor of the Ocular Surgery News Editorial Board, is in private group practice in Longwood, Fla.

Brian J. Forbes, MD, PhD is a member of the division of ophthalmology at Children’s Hospital of Philadelphia and an assistant professor of medicine at the University of Pennsylvania School of Medicine in Philadelphia.

Scott E. Olitsky, MD a member of the Pediatrics/Strabismus section of the Ocular Surgery News Editorial Board, is the ophthalmology section chief at Children’s Mercy Hospital in Kansas City, Mo.

Marc F. Greenberg, MD is in private group practice, specializing in pediatric ophthalmology, in Atlanta

Naval Sondhi, MD a member of the Pediatrics/Strabismus section of the Ocular Surgery News Editorial Board, is a clinical professor at Indiana University in Indianapolis.

Anthony P. Johnson, MD a member of the Pediatrics/Strabismus section of the Ocular Surgery News Editorial Board, is in private group practice in Greenville, S.C.

Robert S. Gold, MD: The topic we’re going to talk about today is shaken baby syndrome. The new term for this, now favored by the National Institutes of Health, is “inflicted childhood neurotrauma.”

We appreciate Brian Forbes, MD, PhD, being here today to add to our discussion because in the Journal of Pediatric Ophthalmology and Strabismus Dr. Forbes and his colleagues recently published a review article on inflicted childhood neurotrauma, and we look forward to his comments on this issue.

In that article, Dr. Forbes and coauthors state that homicide is the leading cause of death in infancy, and that half of the homicides occur in the first 4 months of life. Eighty percent of infant homicides are thought to represent child abuse, and these victims are usually younger than 3 years of age. And this was an incredible statistic in the article, that in 2001 there were more than 900,000 children who were abused and neglected in the United States.

We’d like to ask Dr. Forbes to begin by reviewing some of the common presentations of child abuse that ophthalmologists, not only pediatric ophthalmologists, might see in their offices. What would be the common presentations of a child with inflicted childhood neurotrauma?

Brian J. Forbes, MD, PhD: I think it depends on your institution. At the Children’s Hospital of Philadelphia we are always consulted and are seldom the primary caregiver in the case of nonaccidental trauma or inflicted childhood neurotrauma. We’d be consulted primarily to rule out retinal hemorrhages in a scenario where there was a suspicion of abuse.

At the Children’s Hospital, we have a suspected child abuse and neglect team that coordinates a number of services that is called on to evaluate the child to determine if they’ve been shaken or abused.

Dr. Gold: How many children do you get consulted for, and in how many cases is your diagnosis positive for inflicted childhood neurotrauma?

Dr. Forbes: We are consulted for about 15 cases monthly. For better or worse, I’d say only about 10% to 15% of those are positive.

Dr. Gold: I’m in practice in central Florida, and my partner and I are consulted approximately 100 to 150 times in a year, about two or three times a week. That’s a lot of consultations. I would say that between 25% and 30% of ours are positive. In our area, if there’s any question at all in their mind, the child protective team consults everyone, including ophthalmology.

Marc F. Greenberg, MD: Our numbers are probably similar to that. Maybe a little bit less because a lot of our consultations are negative. It’s difficult to say, but it seems like some months up to 80% of them are negative. Our neurosurgeons would be the primary surgeons taking care of the babies with suspicion of abuse. Maybe they have a better predictive value and have become good at knowing who needs to see us and who doesn’t.

Scott E. Olitsky, MD: I think our numbers are similar. We probably see two to four a week, and I would say a rough estimate is that about 50% are positive.

Anthony P. Johnson, MD: I see probably 35 or 40 a year, and at least two-thirds of those are positive.

Naval Sondhi, MD: I probably see about 35 to 40 a year as well, and I’d say about 30% to 40% are positive.

I think the positive rate is going to be highly dependent on who has screened the child before they pick up the phone and call 911. If you’re really early on in the process, I’m sure the positive rate is going to be significantly lower. If they’ve been screened and multiple injuries have already been found, then I think the positive rate is going to be pretty high.

Dr. Greenberg: If they’ve already have an MRI scan identifying cerebral hemorrhage, that’s fairly definitive.

Dr. Johnson: I’m curious if the anecdotal observations of others match mine on a couple of issues.

First, in my experience, if patients don’t have a positive finding for an intracranial hemorrhage, I have not found any patients that have had retinal hemorrhages. But it has been published that, although rare, you can have retinal hemorrhages without intracranial hemorrhage. That was seen in a case where there was a confession for a shaking injury.

My understanding, prior to that publication, was that the threshold of retinal hemorrhages was probably greater than the threshold of intracranial hemorrhage. That was my understanding of why it is unlikely to see retinal hemorrhages in the absence of intracranial hemorrhaging. Have others had that experience?

Another thing that I’ve noticed anecdotally is, if I am consulted to see a patient who is doing well enough to be on the floor and not in the ICU, until about 2 years ago, my feeling was, I’m happy to come see them, but I’m not emotionally prepared to find evidence of shaking. I felt that, if they were doing that well, they will not have retinal hemorrhages. I’ve seen three or four patients in the past 2 years that have been well enough to be on the floor but have had retinal hemorrhages, and that surprised me.

Dr. Forbes: To address your first question, certainly I’ve seen a number of children who did not have intracranial bleeds at all but had retinal hemorrhages. But I would say that, in general, they are less severe retinal hemorrhages and less severe pathology.

I believe the literature says that 0% to 15% of kids can have retinal hemorrhages without having intracranial bleeds as a result of inflicted childhood neurotrauma.

And as for your second point, I agree. I’ve been surprised a number of times. So when I go to the floor for a consult, I make sure I do it promptly and I try not to bias my expectations.

Dr. Gold: I have a question about timing. The retinal hemorrhages that are present in an inflicted childhood neurotrauma patient, how long do they last, in general?

Dr. Forbes: It depends on exactly what the nature of the injury is. They probably clear, for the most part, by 2 weeks. Some of the preretinal hemorrhages can last as long as 4 to 6 weeks, and a small percentage can last for months. I think the run-of-the-mill retinal hemorrhage generally would clear by 1 month.

Dr. Sondhi: I agree entirely with what Dr. Forbes just said. From clinical practice, in following these children, those dates seem to be very realistic.

Dr. Johnson: I’ve had the same experience. The only ones that I’ve seen that have really lasted a long time are the ones that are so severe that the entire retinal architecture is almost indistinguishable at the time of the initial exam. I’ve seen some of those retinal hemorrhages last probably 2 or 3 months. A few of those have resulted in some macular scarring. But for the most part, in the patients that I see the intraretinal hemorrhages are gone very quickly.

Dr. Greenberg: I would agree that some of the more severe cases last longer than cases with macular folds. You see the blood in the retina for a longer period of time than with folds, but vitreous hemorrhages, in infants, last much longer than they would in older children or especially adults. There are some that are gelatinous, where the vitreous will hold blood for long periods of time.

Dr. Gold: As we go in for consultation, what else should ophthalmologists be looking for in these children?

Dr. Forbes: A lot of times I evaluate the CAT scan and the radiology reports, as well as much of the blood work, because I have an interest in those things. But I think really our role as ophthalmologists is to define what we see in the retina. The question earlier about whether or not there’s an intracranial bleed, I think that is important to know, but our role as ophthalmologists is to document what we see.

Dr. Sondhi: We also need to concentrate on at least the periocular region. Other injuries in that area need to be documented, and we can do a good job with documenting those.

Also, I’ve had a few patients that have subluxated and luxated lenses as a presenting sign. We looked and found hemorrhages, but we saw the lens profile in the pupil before we even looked in the posterior segment. That’s obviously a function of the severity of the shaking more than anything else.

Dr. Olitsky: It’s important not just to document the presence of hemorrhages but also to document very carefully where they are — in the periphery, in the posterior pole, what layers of the retina they occur in, whether there’s retinoschisis — because these things can become important later on, if these cases go to trial.

How to document

Dr. Gold: How do we document what we find? What do you put in your notes in the ICU, in the hospital?

Dr. Johnson: I’m glad that you asked that question, and I’m curious to hear the response from others. I have changed significantly in the past 10 years in my approach to this, mainly because I feel like I understand it better.

But also, since I’m the only one in our community to do this, I feel a responsibility. As much as I hate it when these things happen, I feel a real responsibility. I wonder sometimes if I’m being too aggressive, but I feel like we have the easy job. We just have to identify whether the findings are consistent with a need for further investigation, and then we have to communicate that to the child abuse team and let them do the investigation.

I look to see if bleeding studies have been done and to document that there’s no bleeding. I don’t try to do a total body evaluation. I feel that’s up to the pediatrician and the forensic evaluation. But if something is obvious on the face or the periocular area, I make a note of that.

I’m careful to document the absence or presence of intracranial hemorrhage. I don’t often look at this myself, but I look at the report and document that in my notes.

I’m careful to draw pictures of what I see. As Dr. Olitsky mentioned, I document the number of hemorrhages if they’re countable, or specifically note if they’re too numerous to count. I note if they go out as far as I can see in the periphery to the equator, or farther if I can see farther. I note the level of the hemorrhages — if they are intraretinal, if they are in the nerve fiber layer, or preretinal or vitreous hemorrhage. And I note if there are perimacular retinal folds, hemorrhagic retinoschisis cavities and other findings that Monte D. Mills, MD, and others have described. That has come up many times in my testimony.

I don’t feel that photography is the standard of care at this point. Photography is sometimes not available, and even though I try to get photographs every time, I don’t always offer them to the prosecutors, unless I feel that they really offer something that the rest of the testimony will not offer.

But at the conclusion I write almost the same thing each time, if it’s a suspicious case. I simply say that the presence of retinal hemorrhage coexisting with intracranial hemorrhage is not explained by whatever the history is — a 2-foot fall from the bed on the carpeted floor, for instance — and is extremely suspicious for nonaccidental head trauma and must be investigated as such. If there’s a perimacular raised image, I indicate that that is pathognomic for a shaking injury, and I leave it at that.

Dr. Forbes: It’s crucial, as others have said, to document the layers, the extent, the number of hemorrhages, because many times down the road those details will be needed. To just say either presence or absence and not document exact numbers, that can be a problem. At the Children’s Hospital, we have a separate retinal hemorrhage grading scale we use because we would like to evaluate the extent of retinal hemorrhages in relation to visual outcomes.

In terms of documentation on the chart, again, we have a child abuse team so many times we’re called upon solely for the ocular findings, and they want to know about the presence of retinal hemorrhages and whether it’s consistent with a concern for child abuse. If they’re not present it doesn’t necessarily rule abuse out as well.

Photography the standard?

Dr. Gold: Retinal photography has been mentioned a couple of times. Obviously, there are many places in the country that don’t have the ability to take the RetCam (Massie Research Laboratories) or sophisticated equipment into the hospital to take fundus photography. So, again, the precise, detailed observation of the pediatric ophthalmologist is critical, and I don’t think anybody will disagree with that.

Dr. Greenberg: Can the RetCam replace the ophthalmologist?

Dr. Sondhi: That’s an interesting question. One of the institutions where I used to do these exams, for a time, they had personnel that were trained to take pictures on the RetCam and transmit them over regular phone lines to have a pediatric ophthalmologist evaluate them to document the absence or presence of hemorrhages consistent with nonaccidental trauma.

They went through this with the legal department, and they considered that to be enough documentation legally. I disagreed, and I did not want to participate. I think photography is good — it’s another tool, it’s documentation that you can show in addition to your own record — but it is not mandatory.

Dr. Greenberg: This is an unusual diagnosis for us because we’re not usually looking at treating these patients. We’re getting called in to make a diagnosis mostly for legal purposes, whereas for most of the other patients we see in the office the question is, “Can you help make this better?”

We’re often not in that situation, and sometimes it’s late at night, and some people have suggested using a retina camera in the busier nurseries, at least if you’re seeing as many consults as we are.

I think it might be possible that some centers could do that. If you saw a picture like the one on the March/April cover of the Journal of Pediatric Ophthalmology and Strabismus, that would be enough. It describes exactly what you need to document.

Differential diagnosis

Dr. Gold: Let’s go through some of the differential diagnoses that come up in these legal situations.

The most salient thing about retinal hemorrhages in shaken baby syndrome is that there are a lot of them in the great majority of cases, while in other differential diagnostic possibilities the hemorrhages are much fewer in number.

Dr. Greenberg: The problem we’re all faced with is that there are certain other rare conditions that can look like the retina of an abused child. They are rare, but of course the lawyers will pull that possibility into play. And as the ophthalmologist, you can do as much as you want, but you’re still not the investigator. You can never say what cause is consistent with abuse. You can say this is not consistent, as Dr. Johnson said, not consistent with the history given, which is very important.

The presence of retinal hemorrhages, the presence of hemorrhages at different levels, and certain types of macular pathology such as macular folds, are the pathologies that have been correlated in the literature with a high likelihood of trauma.

I’m not sure that it has been shown that the number of hemorrhages — 10 or 20 hemorrhages, more than 10 hemorrhages — is more of a sign of abuse.

So I document that multiple hemorrhages are there, that they are at multiple levels, and then if there’s a fold or not, because these things have been shown to be more predictive of traumatic origin.

Dr. Forbes: The differential diagnosis that is often most difficult is that of accidental trauma vs. nonaccidental trauma. Certainly, there are any number of bleeding disorders and other conditions that can cause retinal hemorrhages. But again, even with such syndromes, the number of retinal hemorrhages is almost never as extensive as in nonaccidental trauma.

Dr. Gold: One of the other things that comes up in discussions with the legal profession is things like cardiopulmonary resuscitation.

Dr. Forbes: CPR is a good example. The literature shows that you never get retinal hemorrhages to the same extent with CPR as in nonaccidental trauma. They’re confined to one area, they’re smaller in number.

In rare cases of very significant accidental trauma, you can get a picture where there are more retinal hemorrhages, but that is extremely unusual.

Public health questions

Wide-angle funduscopic photograph showing the ocular fundus of an infant with acute inflicted childhood neurotrauma. Subretinal, intraretinal, and preretinal hemorrhages and optic disc hemorrhages are visible. Image: Forbes B.

Dr. Olitsky: This discussion raises an important issue, the question of who needs to know this information. I would say every ophthalmologist does.

Whether you see children in your office, whether you are a pediatric ophthalmologist or not, you can be on call for an emergency room and be asked to see one of these children. So I think any ophthalmologist that has any chance to come in contact with a child should know the answers to these questions and how to detail their examination. I don’t think that there’s an ophthalmologist who can safely say he or she will never be put in a situation where he’s called to see one of these patients.

Dr. Sondhi: That pertains to our earlier discussion of the presence of retinal hemorrhages in the absence of the intracranial hemorrhage. I get the feeling that some of our colleagues in pediatrics and the pediatric intensive care subspecialties start off with a neuroimaging study, and if that is negative, their index of suspicion drops, and they may not always take the investigation on the entire path.

I wonder if we need to be involved in educating our colleagues that will interact with these patients, or at least the gatekeepers for these patients.

Dr. Forbes: In relation to that, at our institution, 10 years ago the percentage of positive retinal hemorrhages dropped from somewhere on the order of 60% down to 10% to 15% now. I think that’s a step in the right direction, that more children be evaluated and there should be more negative examinations to ensure we ate less likely to miss positive findings.

Dr. Greenberg: We’ve discussed shaken baby syndrome as a public health issue with people at our hospital.

To some extent, this is a public health tragedy. In many cases I feel that the people inflicting the injury may not know what they’re doing. They may have shaken some friend in a fight, to the same level that they may have seen others shaken at a football game, for instance, and they don’t realize that a very young child’s brain and eyes are much more sensitive.

We have talked about coming up with a public health campaign just to let the community know. We have Children’s Hospital advertisements all over town. What if, in our public service announcements, we all said, “Don’t shake your baby.”

I think an effort like that might decrease the occurrence a lot. I think people just don’t realize that this can happen.

Dr. Forbes: Actually, in the state of Pennsylvania it’s now mandated that new parents sign off on a form that says “Don’t shake your children.” As the father of a 2.5 month old I know because I had to sign such a form, and I got a four-page brochure to educate me that a little bit of shaking goes a long way.

Dr. Olitsky: Similar legislation is currently being introduced in Missouri. One argument used against the law was that it would cost between $10 and $15 per patient born to prepare literature. That seems like a small amount of money when compared to how many children are potentially affected.

Dr. Greenberg: Is there any data that has shown a campaign like that actually decreased the numbers of cases of the hospital?

Dr. Johnson: The National Center on Shaken Baby Syndrome, in Ogden, Utah, has data relating to that. The organization puts on a workshop every other year. They have a lot of public health information that they try to get into hospitals. There’s a lot of information available from them, and our job might just be putting the right people in touch with them.

Dr. Greenberg: It’s important even to explain to people what will happen not only to the child but to them. The typical documents describing the legal ramifications don’t do this.

Dr. Gold: And so to wrap this up, I’d like to quote something that Dr. Forbes said in his article about reporting suspected child abuse.

He said that abused children may have no other advocate, and so we are part of that advocacy team. Hopefully, this article will bring that realization to some of the Ocular Surgery News readership, and we can move forward and hopefully help some children.

For Your Information:

• Robert S. Gold, MD, can be reached at 225 W. State Road 434, Suite 111, Longwood, FL 32750; 407-767-6411; fax: 407-767-8160; e-mail: rsgeye@aol.com.
• Naval Sondhi, MD, is a clinical professor at Indiana University. He can be reached at Midwest Eye Institute, 201 Pennsylvania Parkway, Indianapolis, IN 46280; 317-817-1333; fax: 317-817-1331.
• Anthony P. Johnson, MD, FACS, can be reached at 131 Commonwealth Drive, Suite 390, Greenville, SC 29615; 864-458-7956; fax: 864-458-8390.
• Scott E. Olitsky, MD, can be reached at Department of Ophthalmology, Children’s Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108; 816-983-6730; fax: 816-855-1793; seolitsky@cmh.edu.
• Marc F. Greenberg, MD, can be reached at 5445 Meridian Marks Road NE, Suite 220, Atlanta, GA 30342; 404-255-2419; fax: 404-255-3101.
• Brian J. Forbes, MD, PhD, can be reached at Children’s Hospital of Philadelphia, Philadelphia, PA 19104; 215-590-4315.

Reference:

• Mills M. Funduscopic lesions associated with mortality in shaken baby syndrome. J AAPOS. 1998;2(2):67-71.
• Forbes BJ, Christian CW, et al. Inflicted childhood neurotrauma (shaken baby syndrome): ophthalmic findings. J Pediatr Ophthalmol Strabismus. 2004;41(2):80-88.

• The National Center on Shaken Baby Syndrome Web site is www.dontshake.com.