Sunday, September 22, 2013

Hot Seat #26: 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

You are seeing a 23 month old female who was well until 4 days prior to presentation. At that time, she began complaining of bilateral eye pain followed by 5 episodes of nonbloody nonbilious vomiting over 12 hours.

She slept all of the following day, at points becoming restless and awakening with eye pain. She also had decreased oral intake, but no longer vomiting. She was evaluated by her pediatrician and clinically deemed to have the flu.

Two days after symptom onset, parents noticed that she would preferentially sit with her head extended back while pulling her legs to her abdomen. Along with worsening eye pain, she also developed decreased urine output. This prompted parents to take her to an outside emergency department.

Parents denied any of the following during her illness course or the remote past: fever, sore throat, drooling, eye redness or drainage, trauma, rash, breathing difficulty, diarrhea or constipation, travel, developmental delay/regressions. Patient’s and Family’s past medical history were noncontributory.

First Question for You:

Her Care Moves Forward
A work up for intussusception was negative, including a CBC, BMP, 2 view AXR and limited abdominal US. Although the patient’s exam was largely unchanged, the parents declined the offer to admit for observation.

The following morning (the day of presentation), she had become less interactive and sleepier, though still arousable. Her preferred position of comfort was lying down, refusing to sit up or walk. Per parents, she had been maintaining her neck in more dramatic extension. Her complaint of eye pain persisted as well. She was therefore brought to the Peds ED

Pulse 111|BP 110/62| RR 24| SpO2 100%| Temp 37.9 °C| Wt 10.9 kg




  • Alert, awake, cooperative. But crying and sleepy, occasionally irritable with exam.
  • Preferentially lying in bed arching her back slightly with neck very extended. Very irritable when asked to move, but will stand while maintaining neck in extension
  • NCAT with no contusions or abrasions of scalp. TMs clear. Pupils equal, reactive, brisk, 4mm; no eye erythema nor discharge. Oropharynx clear with no tonsillar nor uvular edema/erythema/lesions.
  • Neck fixed in extension, will not allow active or passive flexion. No tenderness of c-spine or paraspinal muscles, allowing palpation without movement, no enlarged nodes nor masses.
  • RRR with no m/r/g, Clear breath sounds with no increased work of breathing.
  • Abdomen soft but uncomfortable with palpation diffusely, no masses, neg rebound/guarding/peritoneal.
  • Mental status normal responding to commands/parents’ questions/exam; Normal tone in extremities, 5/5 strength in distal upper and lower extremity with no overt sensory deficits. DTR 2+ in lower extremities and no clonus. Unable to cooperate for cranial nerve or cerebellar exam, but no gross deficits or lateralizing signs.


  • A couple more questions for you:


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    About the Hot Seat. Hot Seat cases are written by PEM fellows at Children's National, Inova Fairfax, and Johns Hopkins. The Hot Seat Attending is a selected faculty member who comments on the case without knowing the outcome. Emily Willner is the faculty mentor for the series but she is on a well-deserved leave. During July, August, and September guest faculty from Children's National, Inova Fairfax, and Johns Hopkins will serve as case mentors. Jennifer Anders, Johns Hopkins, edited this case.

    7 comments:

    1. i would argue, why is this not a brain tumor?

      the differential starts after you exclude a posterior fossa mass here. if using CT, good to scan the head and neck to best visualize that area. if you have a fancy MRI machine in your ED (cough cough, not us) then that's good too :)

      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.

      Now it does sound like she is in somewhat of a sniffing positioning so i would at least consider a space-occupying lesion in the neck, although my money would be on the head. If she had something on the anterior airway causing her to hyperextend her neck, i would expect stridor. and similarly if there was a deep neck mass in the retropharyngeal space, i would expect drooling and tripoding.

      CONSIDER LP: if her scans are negative, i'd pursue an infectious workup. she has an almost fever at 37.9 and i'm not sure if there are EOM defects (CN deficits) that she's compensating for by her neck positioning.

      You're not really describing anything that suggests a white matter lesion (ADEM, etc) so i don't know how helpful an MRI would be in the absence of an intracranial mass on CT....if you start with CT imaging.

      decreased urine output could be a sign of SIADH. or not drinking. if a tumor involves the vagus nerve, i think it can cause urinary incontinence....not sure about retention.

      scan please.

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      Replies
      1. [I love the coughing, Dave!]

        Priya and I were discussing treating pain in someone who is likely to have a neurological process or in whom a change in mental status or sleepiness is important to follow. Fentanyl is short acting and may be a good option if her pain needs to be treated.

        Delete
    2. I'd agree with Dave. Head CT first, to r/o mass or sub-arachnoid hemorrhage. If negative, I'd definitely LP. This child sounds like she has meningismus (eye pain is probably headache; vomiting; stiff neck held extended; and legs curled up).

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    3. Tumor until proven otherwise...although posterior fossa might not show up as well on CT. Hard to tease out 'eye pain' in someone <2, but it's an unusual complaint and in the context of the whole clinical picture is concerning for a CNS process, be it tumor vs. infection.

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    4. agree with above responses. 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). My money is on a mass or subarachnoid hemorrhage or Chiari malformation causing hydrocephalus. If CT negative, like Dewesh and Dave, next step would be an LP.

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    5. Agree with all the above... how exciting!
      Head CT to r/o mass 1st, with neck X-rays then Neck CT for a neck mass. 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. Unless it has been going on for a while, and now their is some muscle strain on top of the compensatory neck extension.
      That's all I have to add.

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    6. Joanna Cohen, Hot SeatSeptember 25, 2013 at 6:37 PM

      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 his 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 pain s, 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.

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    What's your opinion?