Welcome to our newest series, 'The Newport Catch' -- a collection of community emergency medicine cases from our local hospital in seaside Newport, RI.

It’s a no-room-at-the-inn kind of night in your community hospital ED. The rooms are full, the halls are full, the beds upstairs are full – and your waiting room is packed. 

The triage nurse approaches you. “I have an almost-two-year-old out here that I have to find room for.  She looks fine, but her parents say she just had an episode where she turned blue and lost consciousness, and they weren’t sure if she was breathing.  No CPR done. Vitals are all good now but – you know.”  She hands you a normal pediatric EKG and heads back to triage as you wonder how she managed to get that done -- because you can see the young family crammed into the triage room through the open door. The child is scampering about the tiny room, trying to climb the cabinets. 

You look at your computer.  There is literally nowhere to put this kid.  So you head to triage to scope it out.

The patient is a well-appearing, very busy toddler, accompanied by her parents. The mother does the talking, and she is visibly angry at her husband, whom she believes is at fault.

“He was tickling her, and he wouldn’t stop,” the mother said, “and then the poor baby was yelling ‘No Daddy!’ because it wasn’t fun anymore, and he still didn’t stop, because he thought it was funny. And then she started to cry, and then hold her breath because she was, you know, so upset – and then all of a sudden she was blue, and she went all limp! It looked like she died! “

You nod.  

“And then,” continues the mother, ”we could finally tell she was breathing, and in a few seconds she woke up totally fine, like nothing ever happened. And she’s seemed fine since then. And I know she looks fine now, too, but I swear, I thought she died.”  She glares at her husband again.

You ask a few more questions and do a quick (but good) physical. Here’s what you find:
-No PMH/PSH; normal growth; followed regularly by local pediatrician
-No medications; vaccines up to date
-No seizure activity or incontinence during this episode
-No family history of seizures, cardiac problems, or sudden death
-Normal exam with normal vital signs

Now what? Should you:

a) Return them to the waiting room and call them back for testing when a room opens?

b) Transfer them to the tertiary care children’s hospital for cardiac consultation?

c) Discharge them from triage with outpatient follow up?

What’s the diagnosis?  

Breath holding spells are common from infancy to age six; most children present with their first episode before the age of two. They are quite frightening for parents witnessing a first time episode. 

There are two types of spells: cyanotic and pallid.  Cyanotic spells are more common, and usually occur after a child becomes upset by a minor event, cries, and then holds their breath.  They quickly ‘turn blue,’ lose tone, and may progress to syncope, although many spells end before this point.  Most episodes are brief and self-limited, although children have been reported to display posturing or have generalized seizure activity with prolonged apnea. (It is theorized that children with airway abnormalities could be at risk for complications, but most anatomically normal children recover uneventfully.)

Pallid spells (also known as pallid infantile syncope) are much less common, and caused by cardiac bradycardia following minor trauma.  The child becomes pale and diaphoretic before loss of tone and consciousness. The child may even become incontinent and have a period of drowsiness after regaining consciousness; it may be difficult to differentiate these spells from seizures by history, necessitating further work up. 

Both types of spells tend to recur (some children have many spells each week) but have excellent prognoses, with normal neurologic development and complete remission usually by age 4-6. There is no diagnostic test for breath holding spells -- just a careful history. However, prolonged spells, spells without a classic trigger, or spells in patients with family history of prolonged QT or sudden death should have a more urgent syncope workup initiated.

If an otherwise healthy child has a classic history of a cyanotic breath holding spell with a clear trigger, full recovery after a brief episode, and no suspected seizure activity, they may be discharged with simple reassurance and outpatient follow up with their pediatrician. Some experts do advocate an EKG to screen for QT prolongation. Outpatient follow up may include a CBC and serum ferritin, as iron deficiency anemia is associated with breath holding spells. 

References:

Breath-holding spells. Breningstall GN. Pediatr Neurol. 1996;14(2):91.

Prospective study of children with cyanotic and pallid breath-holding spells.
AU DiMario FJ Jr . Pediatrics. 2001;107(2):265.

Nonepileptic Paroxysmal Disorders in Infancy, Nguyen TH et al.  Retrieved from UpToDate.com, accessed 4/18/2016.

Faculty Peer Reviewed by Chris Merritt, MD, MPH, FAAP
Pediatric Emergency Medicine
Hasbro Children's Hospital