with Maybelle Kou, Inova Fairfax on the Hot Seat
The Case
3 year old female with history of asthma presents with wheezing, tachypnea and noisy breathing.
She was seen in the ED 5 days prior for similar symptoms. Per the chart, her exam had been notable for stridor at rest, generalized wheezing, and moderate accessory muscle use with tachypnea. No hypoxia or fever. She was treated with racemic epi x1 and dexamethasone and improved. She was diagnosed with croup, and was discharged with croup instructions and Q4 hour albuterol (given asthma history).
Since that visit, she improved over the following 2 days, but 3 days ago her noisy breathing worsened to the point that kids at school are calling her “Darth Vader”. She has had tachypnea, cough and wheeze which “come in waves” and improved on the way to the ED today. No rhinorrhea or fevers. On exam: T 37.6, HR 130, RR 32, BP 104/74, Pox 98% RA. Alert and playful. Voice is hoarse. OP clear, MMM. No rhinorrhea. RRR, nl S1/S2, No gallop, rub or murmur. Normal respiratory effort. No position preference. Loud upper airway noises. Faint generalized expiratory wheeze, No stridor, No retractions. Exam otherwise normal.
Questions for you (Click on "vote" to record your answers and see what others said)
You were pulled away to a trauma and the eager 1st year resident has taken the liberty of ordering a rapid strep and chest x-ray. Rapid strep is negative. The CXR is below.
The patient’s mother approaches the desk and states “her breathing is weird again”. On re-exam, you note stridor and hoarse voice (she really does sound like Darth Vader) while happily playing with her mother’s cell phone. HR 130, RR 24, Pox 98%. Consistent inspiratory and occasional expiratory stridor, generalized faint wheeze, symmetric breath sounds. Some accessory muscle use but no retractions or flaring.
You delve deeper into the history:
The patient remains unchanged after your intervention above. Imaging, if obtained, was read as “normal” by the radiology resident.
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it's all about the clinical exam......
ReplyDeletebiphasic stridor points to the subglottic area. hoarse voice points to the vocal cords, usually in the supra-glottic or laryngeal area. so kinda a contradictory exam.
good to think about some things that cause acquired stridor, such as vocal cord dysfunction (not right age group), laryngeal nerve palsy (associated with CNS lesions), airway hemangiomas (not right age group), laryngeal polyps, and of course foreign body.
it's hard to do an FFL (flexible laryngoscopy) in a 3yo, but it sounds like the kid needs one. if unable to do it, just bronch. look for signs of neurologic dysfunction because posterior fossa masses and chiari's can cause laryngeal nerve palsies. would be a good idea to get neck films to look at hypopharynx.
Excellent case for discussion!
ReplyDeleteThe questions that come to mind here first are:
1. Critical airway?
2. Acute or chronic?
3. Upper or lower airway? (or both?)
#1: She doesn't seem to be a critical airway in how comfortable she is with no tachypnea and mild accessory muscle use, but this has also progressed over the last 3-5 days and might be worsening. Stridor always worsens at night or sleep, so it might be helpful from a dispo standpoint to see the child sleeping (if it's evening, for instance, and ENT recommends discharge with a morning clinic visit the next day).
#2: Acute or chronic: Only 5 days of symptoms points to acute, which are almost always infectious outside of a hx of FB aspiration, trauma, allergic or post-surgical. However, no fever, no rhinorrhea, playful bounceback leans away from this. She also seems relatively comfortable at rest now for a kid with biphasic stridor that a slowly developing chronic cause seems more likely. Plus, she's sitting on the hot seat.
#3 - The lack of tachypnea, hypoxemia, and the presence of insp sx >> exp sx when she was looking worse on the second exam point to extra-thoracic upper airway. Voice changes can be caused from problems along this whole tract
DDx is broad but by category, in decreasing order of likelihood:
1. Infectious (croup, spasmodic croup); even though I think this is less likely, common things presenting uncommonly are more common than uncommon things...
2. Inflammatory non-infectious(reflux making croup worse, allergic)
3. Tumor (papilloma* on/near VC, hemangioma, rhabdo, mediastinal)
4. FB/TB
5. Neurogenic (laryngeal N compression from a/g)
Mgmt:
Try racemic epi and dex, but maybe wait until after ENT examintes them to do this as your exam might be back to 96 hours ago when she looked great after round 1. Lat neck xray will probably be insisted by ENT prior to evaluation which is OK but as in this case, can be false negative. As Dave said, probably this kid won't be able to tolerate DFL so probably will need to go to OR given the patient's age for a look there at the supraglottic/glottic area and if all normal, then bronch to look subglottic and into the bronchi to look for FB (although doesn't seem like FB given the intermittent sx picture). If ENT wants to do it tomorrow, do your racemic epi/steroids and sleep trial and if great, d/c home if parents reliable with PCP call and ENT appointment assured. Ask the ENT to send you pics too.
Thank you Ashley, these presentations are always interesting and tricky.
ReplyDeleteI thought I had smart things to say about this case, but all has been said above!! Thanks boys!
Pt seems stable, without a critical airway. There seems to be an acute on chronic problem since after her 1st episode of wheeze she had a voice change and now an acute worsening.
My differential would be something in the upper airway. Vocal Cord paralysis, Upper airway Foreign Body, Polyp or other VC/larygneal mass, and possibly an acute URI may be exacerbating this.
If she looks well, at this point, i see no benefit from further steroids or rac epi. I would consult ENT and will be okay if she is seen and scoped within the coming 24 hours either admitted, observed O/N for ENT to come in am or ENT clinic in am.
Voila! That's all I have to say today.
Biphasic stridor and hoarse voice points to an upper airway obstruction. H/o of onset of "asthma" 3 months ago when doing crafts suggests upper airway foreign body, and misdiagnosis by PCP as asthma. We physicians often fail to remember the dictum that "all that wheezes is not asthma" and also fail to differentiate between homophynous vs. heterophynous wheezes. Homophynous (sounds the same all over the lung fields) wheezing suggests large airway (tracheal) obstruction. Heterophynous (sounds differently in different lung segments) wheezing suggests lower airway disease/obstruction (that is different in different parts from bronchoconstriction and/or mucous plugging) such as with asthma. I've seen on numerous occasional "recent new diagnoses of asthma" that were really missed airway foreign bodies. I'd bet something is lodged around the larynx/upper trachea. Soft-tissue neck films would be good start. ENT will only do bedside flexible scopes above the vocal cords, but would agree to starting there (non-emergently) before going to the OR for anesthesia and rigid scope.
ReplyDeleteInteresting case of toddler with intermittent stridor. I think if I saw this patient for the first time, with common things being common, I would have done the same as the first clinician and treated for presumed croup with Dex and Rac Epi. But if the child returns 3 days later with the same symptoms, I would feel the need to broaden my differential. Run of the mill croup should have resolved by the 2nd presentation. Highest on my differential would be a possible foreign body (although the intermittent nature is a little odd). The patient does not appear toxic and is not drooling making things like epiglottitis or RPA less likely. The "darth vader" voice description makes me think of a lesion near or on the vocal cords. A finding like a vocal cord polyp could be intermittent in nature. CXR shown does not show a steeple sign or abnormal narrowing of trachea. Next step to get ENT to scope to take a look. Because the patient is in distress would want this sooner rather than later. -Jamil
ReplyDeleteInteresting case. I agree that this child's presentation is less likely solely infectious (but could be acute infx worsening a chronic, underlying condition) and is not likely a critical airway. This kid needs ENT!
ReplyDeleteLooking forward to the case resolution,
Asha
Given the patient’s “normal respiratory effort, no position preference and loud upper airway noises”, I am reassured at this minute that this child does not have an acute infectious process with imminent respiratory threat. The illness today is not convincingly croup-like. There is no known “allergen” exposure.
ReplyDeleteHoarseness is described, but the history does not support a pharyngeal abscess with this otherwise well appearing child, there is no torticollis, dysphagia or fever. The mouth exam rules out kissing tonsils or tonsillar abscess. The child is immunized, so we don’t need to worry about H flu so much, or diphtheria either. Likewise, her unremarkable body position does not support a fixed mass (such as a huge sublingual mass pressing on the trachea). No rhinorrhea, but was the NP looked at carefully? Must not forget the NP exam after all. I recall my colleague BDC’s story of a child with upper respiratory symptoms similar to the above, who ended up having a long foreign body in his posterior nasopharynx that produced intermittent respiratory symptoms by sliding up and down. Then there was the six month old that was fed plastic stickers by her toddler brother, that adhered to the roof of her mouth and caused intermittent choking when it slid backwards.
Added to the history is a previous “wheezing” event (within 3 months), my thoughts turn to a sub-acute process involving the trachea. The spasmodic nature of the cough could also be suggestive of a foreign body that might be mobile (such as a small piece of plastic). The progressive nature could point towards an enlarging vascular entity, either vascular ring or tracheal hemangioma. One would not expect these to respond verily to bronchodilator treatments. Laryngomalacia should have presented earlier in life. One would also expect to be able to elicit the finding of an extra-laryngeal mass on physical exam in a small child, however, a deep structure might be missed.
Assessment: In short: this is a 3 year old with intermittent respiratory distress with history suggestive of upper airway obstruction, with stable VS and clinical status S/P intervention. Her “wheezing” symptoms are somewhat responsive to inhaled treatments, pointing at inflammation that could be a secondary process.
Management:
Give an additional trial of bronchodilators and steroids to address the non-zebras.
I would forgive the intern for obtaining an XR. If my eyes do not deceive me, I do not see evidence of tracheal deviation, (so no ring or sling) or obvious foreign body (no ball-valve effect or hyperinflation) and it may just be the projection but the cardiac silhouette may appear a trifle on the large side.
The intern and I might later on have a calm discussion about test utilization, and CENTOR criteria, let alone strep tests in general.
MY PLAN:
Admit for observation and general surgery consultation for rigid bronchoscopy. She should have a PPD placed, and also have a repeat CXR/swallow study at some point.
Crumbs for thought
1) if you have nasopharyngeal endoscopy and are comfortable using it on a toddler, and the patient can tolerate the test, by all means take a look. Regardless of nasal endoscopy, you can’t see well below the vocal cords, and this child should have a rigid bronchoscopy anyway.
2) In the community setting, most general ENTs/surgeons may will not be comfortable with the age of the child, which would mean the headache of finding one that does, or transferring to a facility for children.
3) There is also the argument that this child has not responded to any treatment, therefore could be stable to be discharged with urgent follow up. Medical/legally, this would require contacting the specialist to arrange the visit for the child. Then there is the issue of where in the airway you think you have a problem. A three year old may not be amenable for an invasive test as an outpatient.
I just had a patient with the “Darth Vader breathing” chief complaint just last week. There must be an ICD-10 code coming.
-Maybelle Kou
Fellowship Director
Inova Children's Hospital