PVFM is defined by recurrent closure of the vocal folds during inspiration. Remember to accurately breath, we need to normally have abducted vocal folds, cause otherwise there is no air passing distally.
Its etiology is not specifically known as a functional disorder, but some triggers are:
▶️Exercise
▶️Stress
▶️Airway Irritation via Trauma or Asthma
▶️Neurologic Disease
▶️GERD or Larygnopharyngeal Reflux
▶️Medications: EPS are associated (acute dystonia being a common link)
Symptoms of an Episode are mainly:
▶️Globus Sensation
▶️Throat TIghtness
▶️Air Hunger
As they tend to present in pediatric patients, children presenting often are brought in by worried parents, so addressing their worries might be the toughest aspect of this syndrome!
The approach to Dx is generally:
▶️PFT: flattened inspiratory loop is suggestive
▶️Laryngoscopy showing the above adduction is diagnostic
But like @rabihmgeha often mentions, in episodic conditions, you often see a patient post-episode and may not be able to diagnose it!
You can try to induce an episode with mannitol, histamine, or methacholine and then use the above methods, but there is not a lot of great evidence that these reliably induce an episode (goes back to multifactorial nature of this syndrome)
Differential Diagnosis: 80% of cases are misdiagnosed initially as asthma. The stridor here is high pitched, inspiratory, and nonresponsive to bronchodilators.
Also think laryngospasm, angioedema, and tracheal stenosis when you hear sudden onset stridor!
Acute Episode:
As commonly self limited, reassurance and CPAP are best during an episode. Interestingly, panting can relieve the stridor by initiating true vocal fold abduction
Some benefit shown with below treatments:
▶️Benzos: only acutely
▶️Heliox:
▶️Neb. Lido up to 4%
Long Term Management
▶️Gold Standard: Speech Therapy with abdominal breathing, pursed-lip breathing = opens the glottis
▶️Treating Comorbidities avoiding irritants is best
Long Term
▶️Ipratropium pre-exercise may prevent
▶️Botulinum toxin was effective in small case series
Summary:
PVFM is a multifactorial syndrome due to multiple triggers, presenting as acute recurrent episodes of stridor, air hunger, often self-resolving. Dx seeing adducted vocal cords and support during the episode, with chronic tx to keep the glottis unirritated and open!
Another new diagnosis I had never heard of until this pulm rotation: Ulcerative Bronchiolitis (yup, you saw that right). 🧵of an illness script of a rare condition!
Background: although called "Inflammatory Bowel Diseases", Ulcerative Colitis and Crohn's Disease do not spare other organs. Some examples:
▶️Rheum: Vasculitis, Arthritis
▶️Skin: Psoriasis, Erythema Nodosum...
▶️Neuro: Encephalitis
▶️Optho: Uveitis
and many more!
Lets focus on the Lung: UC in particular can affect a bunch of "aireas" (🥁):
▶️Upper Airway: Tracheal Stenosis (subglottic)
▶️Lower Airways: Most common = Bronchitis and Bronchiectasis, then rarely Bronchiolitis, Bullous Disease, OP, Vasculitis, and Eosinophilic Disease.
As someone not from this community, I don't wanna step on toes, but do want to ask a question.
Is this language on HIV associated HHV8 risk factors stigmatizing those identifying as homosexual or bisexual men, and a bit simplistic? no real discussion here.
Instead of comparing sexual orientation, sexual activtiy involving saliva among those with HIV (which yes is higher in MSM) is the proposed transmitting risk factor you might mean. "IV drug use" is an action that irrespective of sexual orientation.
The sources for why sexual activity with saliva is the risk factor for HHV-8 transmission and how # of sexual partners correlates.