Hot Seat #26 Denouement: Eye pain and neck extension

by Amy Boutsikaris, Inova Fairfax
with Joanna Cohen, Children’s National, on the Hot Seat
Case edited by Jennifer Anders, Johns Hopkins

The Case

This is a case of a 23 month old girl with bilateral eye pain and vomiting. She additionally was noted to have significant neck extension and abdominal pain. For a full case presentation, click here.

Clinical Pearls 
Thanks to Dave, Dewesh, Theresa, Sonny, Monika, and Rasha for your insights and Jo for putting it all together on the Hot Seat. Most people were concerned about an intracranial process. Dave used the available physical exam to try to localize the location of the abnormality. Dewesh, Monika, Dave, and Jo commented that they would likely perform and LP if head imaging was negative. Click here to read more of their clinical reasoning.

“You don't describe motor deficits, so i would think a cerebral mass is unlikely and the positioning doesn't support increased ICP, so my money points to the cerebellum.” –Dave Mathison, Children’s National

“This child sounds like she has meningismus (eye pain is probably headache; vomiting; stiff neck held extended; and legs curled up).” –Dewesh Agrawal, Children’s National

“Description of neck sounds like opisthotonic posturing which you could see with a brain mass or meningitis, among other things (e.g. metabolic disorders, various drugs, tetanus).” -Monika Goyal, Children’s National

“Posterior fossa might not show up as well on CT.” –Theresa Walls, Children’s National

“The fact that neck can't even be moved passively does make me concerned about a neck mass or space occupying lesion. As I would think that neck extension compensating for a head lesion should allow passive movement, even if with some pain.” –Rasha Sawaya, Children’s National

“Fentanyl is short acting and may be a good option if her pain needs to be treated.” –Sonny Tat, Children’s National

Here’s how you answered our questions  
1. At this time, which of the following is highest on your differential? (38 total responses) 

Most people were worried about a brain mass. No one thought it was likely a UTI.  

2. While pursuing your work up, how would you treat this patient’s discomfort? (35 total responses)

Most people chose acetaminophen for pain control in this case followed closely by ibuprofen and fentanyl. In addition, a few people said they would not treat this patients pain. Were there specific reasons for these specific choices?

3. Suppose she got better with acetaminophen and valium. What’s your next test? (36 total responses)

Most people wanted to get to get a head CT although some people were thinking about the neck as well. Interestingly, a few people wanted to do none of the options given. Were they being contrarian or we were missing something?

From the Hot Seat
by Joanna Cohen, Children's National

So here’s this 23 month old with persistent eye pain for four days, some emesis which seemed to have resolved, maybe some abdominal pain for 2 days (with a negative work up for intussusception the day before) and now sleepiness, irritability and dramatic neck extension. How do we tie all these complaints together?

Obviously we want to consider the big bad stuff, like meningitis and brain or neck mass, but the presentation is not typical. Eye pain is an unusual complaint for a toddler. I would want a good eye exam including flourescein to rule out a corneal abrasion, ulcer or foreign body, which are probably some of the most common reasons for eye pain in this age. Iritis can also causes eye pain, but we would need a slit lamp exam to look for it. If this is what she had, I would think of some sort of inflammatory condition like JIA or infection to tie it together. People with migraines often complain of eye pain, but that would be unusual in a toddler too.

How about the dramatic neck extension? This is a newer symptom so it makes me think an evolving process. Neck stiffness or extension in this age group is probably most commonly due to infection like a retropharyngeal abscess or meningitis, but this kid is afebrile and had a normal CBC yesterday making me think infection is less likely. There’s no history of trauma. She could have a spinal epidural hematoma, a posterior fossa tumor, or aneurysm rupture that could cause vomiting and neck stiffness, but I expect more CN palsies or vision deficiencies rather than actual eye pain. My number one thought is that the neck extension is a dystonic reaction. I would want to know if she got any medications, particularly phenergan, reglan, or compazine for nausea when she was seen the day before when the primary consideration was intussusception. If the parents were unsure, a dose of benadryl might clear this up.

What about the abdominal discomfort with palpation? Her abdomen is soft, but she does seem uncomfortable and she was drawing her knees to her chest and she seems sleepy. This all makes me think of intussusception which you can miss on US if the patient is not actively intussucepted. I would probably want to Guaiac the stools and keep an eye on the abdomen and if the patient began having abdominal pains, I would consider repeating the AUS. Of course eye pain is not a symptom I think of with intussusception.

So what to do now? I would make sure she got a thorough Ophtho exam. I would repeat the CBC and check inflammatory markers like an ESR or CRP. I would trial a dose of benadryl and motrin for the neck stiffness and get a CT of her neck and head, particularly if her sleepiness did not spontaneously improve. If her imaging is unremarkable and she remains altered with neck stiffness, I’d likely do an LP.

Denouement
by Amy Boutsikaris, Inova Fairfax

 

A head CT was done (representative image)

The patient went on to have a sedated brain MRI

 

 

The patient also had a sedated MRI antiography

This was a case of a 23m.o. female presenting with mild fever, transient emesis, vague abdominal pain, persistent eye pain, and worsening neck extension and irritability. Her symptoms were consistent with meningeal irritation by an intraventricular hemorrhage sourced by large bilateral intracranial AVMs. Her Head CT was significant for an intraventricular hemorrhage most prominent in the right lateral ventricle, occupying the body and atrium. Lesser amounts present in the left lateral ventricle and the fourth ventricle. The third ventricle appeared unremarkable. There was no overt hydrocephalus. Her MRI Brain & Angio was significant for very large unusually shaped AVM with dominant components in the lateral ventricles, thalami, and posterior limbs of internal capsules. A discrete area of a midline connection, immediately superior to the anterior commissure was noted. Collectively, the AVM measured 6.4 x 4.9 x 3.5 cm.

Pediatric neurosurgery was consulted with recommendation for decadron and an antiepileptic drug. The lesions were deemed inoperable from their standpoint. Neurointerventional radiology was also consulted with plan for dedicated cerebral angiography after the acute episode of her intraventricular hemorrhage subsided, with possible embolization. However, it was stressed to the parents that given the size and location of the AVMs, embolization of feeding vessels was extremely high risk for affecting truly perfusing branches.

The patient was admitted for 2 days over which her pain & PO improved. However, she did re-present 2 days following discharge with abdominal pain warranting a CT Angio of her abdomen and pelvis that was negative. Her pain subsided with aggressive constipation treatment, and she was discharged the next day. Her longterm prognosis is poor given the complexity and magnitude of her AVMs, but we have not seen her back in the ED since.

The Hot Seat is a recurrent online case series aimed at facilitating asynchronous sharing of PEM knowledge and experience. Faculty are placed on The Hot Seat and publish their opinions without knowing the case or its outcomes. Cases are written by PEM fellows at Children's National in DC, Johns Hopkins in MD, and INOVA Fairfax in VA. Emily Willner is the faculty advisor on case presentations but is on leave. This week's case was guest edited by Jennifer Anders, faculty at Johns Hopkins. We welcome your commentary.