Friday, 22 April 2016

Refer All Patients (Easter egg - laryngomalacia)

Referral rates from Primary Care have risen over the past ten years yet children are having the same symptoms and illnesses.  Increased referral is sometimes because there are more treatments available and is therefore quite appropriate.  In the case of laryngomalacia, there are really only two treatments, time or surgery, so why should more children be seen by specialists?

I suspect that the answer is partly parental expectation and partly the undermining of the clinical independence of General Practice.  I believe that both of these can be affected by making sure that we know everything there is to know about such conditions.  This allows us to explain the problem confidently and manage it (where appropriate) ourselves.

Sometimes, clinicians in secondary care have anxieties about the ability of primary care to assess and manage a condition and they mitigate that by recommending that all are referred.  General Practice has been the place where uncomplicated laryngomalacia has traditionally been managed.  The case must then be made for that to change if necessary.

So, I will go through some things that you may or may not know about laryngomalacia.  Before I do that, I will give a quick overview.

Laryngomalacia is a condition caused by an abnormal laryngeal cartilage.  It is a dynamic problem that evolves from birth, partly to do with shape and partly to do with floppiness. The typical presentation is that of a child who starts making upper airways noises (video link here) especially when lying down.  Everyone know that upper airways problems are dangerous, yet at least 90% of laryngomalacia will resolve without causing significant problems.

In order to be confident in this we know a bit about it.  Here are the trade secrets.

1. Laryngomalacia is not truly congenital
Although the abnormality may be present or evolving at birth, it is not clinically apparent immediately.  There is something that happens to the larynx shortly after birth which completes the airway abnormality and so the typical clinical presentation occurs sometime in the first few weeks of life.  Stridor that is present immediately after birth is therefore a red flag.

2. Laryngomalacia has a sense of humour
Parents will present their children for assessment with anxiety and frustration in equal measures.  The anxiety is completely understandable.  Their baby makes a noise when it breathes!  The frustration comes from the child's apparent inability to perform during the consultation.  Often a description is all that is needed.  Smart phones make it possible for parents to bring recordings or alternatively you can show them the video link above for reference.  

3. There are pretenders
As always, there are conditions that present in a similar way to laryngomalacia.  Essentially any problem that causes chronic airways turbulence can cause a similar scenario of intermittent stridor.

Pretenders include:

  • Vascular rings (blood vessels that encircle the trachea)
  • Subglottic stenosis (e.g. due to endotracheal ventilation)
  • Cysts
  • Polyps
  • Webs
  • Haemangiomas

If the diagnosis of laryngomalacia is uncertain, it is best to refer so that the ENT specialists can do an endoscopy in an outpatient clinic.

4. Laryngomalacia has a  synergy with gastro-oesophageal reflux
If the laryngomalacia is problematic, the baby will compensate by increased work of breathing.  The greater negative pressures created to overcome the laryngomalacia then increase reflux of milk into the oesophagus.  This can in turn cause inflammation of the epiglottis, worsening the turbulence of the upper airway.  For this reason, anti-reflux medication is now often used is a baby has symptomatic laryngomalacia.

5. Most cases will follow a benign course
About 9 out of 10 babies with laryngomalacia will have no problems with feeding or breathing.  If there are signs of respiratory distress, or symptoms of feeding difficulties, I would normally refer for assessment by an Ear, Nose and Throat specialist.  Most of these children will still require no surgical intervention.

So, one approach would be to carefully assess whether the history and examination are consistent with laryngomalacia and that this is not adversely affecting the child.  If both of these are true then referral is not necessary for the clinician who is confident to provide good safety-netting advice and watchful waiting.

If you have any other strategies or thoughts on this subject please post a comment below.

Edward Snelson
Unknowingly uncertain but rarely indecisive

Disclaimer - If you ask ten doctors about this you will get nine different answers and one story that goes on indefinitely.

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