He’s always sick: ENT infections and immunodeficiency

Cite this article as:
Alasdair Munro. He’s always sick: ENT infections and immunodeficiency, Don't Forget the Bubbles, 2019. Available at:
https://doi.org/10.31440/DFTB.20243

Otis is a 3yr old boy presenting to the emergency department with fever, and purulent discharge from his left ear. He otherwise looks well, however, his mother mentions this is his third ear infection since he was born, and he always seems to have a cough and a cold. She asks you if there could be a problem with his immune system?

Some children seem to have constant ear, nose or throat infections during childhood. We know that for a small, but important minority of children this may be the presenting feature of a primary immunodeficiency. Let’s look at how these may present, when to think of it, and what to do about it.

Primary immunodeficiency is rare

It’s worth stating from the outset, that the majority of children with recurrent ENT infections will not have a primary immunodeficiency. There is a relatively common phenomenon called “physiological immunodeficiency of infancy”, where-by there is a natural nadir in immunoglobulin levels as maternal immunoglobulin fades, and the child’s own immune system has only just become able to produce immunoglobulin for itself. This is at its lowest between 3-6 months and normally resolves by age 1. However, fully developed protection against encapsulated organisms doesn’t reach maturity until between 2-5 years, and IgA production doesn’t reach adult levels until adolescence. It can be completely normal for young children to suffer 4-11 respiratory infections a year (depending on exposure, e.g. siblings, nursery, etc.)

When should I suspect immunodeficiency?

When considering the characteristics of infections that should trigger suspicion for immunodeficiency, we should be thinking about:

More severe infections than is usual

Combined immunodeficiency disorders (affecting both cellular and humoral immunity), such as severe combined immunodeficiency (SCID), present in the first 3-6 months with severe, life-threatening infection. Unusually aggressive infections should prompt further investigation

Infections with unusual organisms

Infections with certain pathogens can point towards specific diagnoses, including respiratory infections with Pseudomonas aeruginosa (think cystic fibrosis or primary ciliary dyskinesia), oral/oesophageal candidiasis (think HIV or chronic granulomatous disease), upper respiratory infections with Pneumocystis carinii (think HIV or other T cell deficiencies) or recurrent otitis/sinusitis with Neisseria meningitidis (think complement deficiency).

Finally, to a lesser extent:

Frequency of infection

This is the least predictive of immunodeficiency, given the discussion above. Very frequent sinopulmonary infections in younger children with encapsulated bacteria can be the presenting feature of the rare condition X-linked agammaglobulinaemia (XLA: boys who produce no immunoglobulins). In late childhood and adolescence, the same presentation in a milder form may be a sign of combined, variable immunodeficiency (CVID), which is a heterogeneous group of disorders of antibody production.

Other, rare conditions include chronic granulomatous disease (CGD) which may present with deep abscesses of the outer ear or mastoid, or HIV presenting with recurrent otitis media (normally with other associated features)

 

When to refer

Some general guidelines have been produced by the Jeffrey Modell foundation for when to consider referral for immunodeficiency workup:

  • Four of more new ear infections within 1 year
  • Two or more serious sinus infections within 1 year
  • Two or more months on antibiotics with little effect
  • Two or more pneumonias within 1 year
  • Failure of an infant to gain weight or grow properly
  • Recurrent, deep skin or organ abscesses
  • Persistent thrush in the mouth or fungal infection on skin
  • Need for intravenous antibiotics to clear infections
  • Two or more deep-seated infections including septicaemia
  • A family history of primary immunodeficiency

Although having a low specificity, they provide a useful framework when thinking of children with more severe infections than usual.

 

Should I do some tests?

If considering referral, there are definitely some basic tests are useful to do first (if the child is severely unwell, don’t wait for tests to refer).

Full blood count

This is useful for ANY suspected immunodeficiency. Persistent lymphopaenia in a child <2yrs should prompt screening for SCID.

NB: It can be normal to have transient lymphopaenia or neutropaenia in isolation in young children following a viral illness. Incidental neutropaenia does not need repeat testing if there are no concerns about underlying immunodeficiency.

Immunoglobulins

IgG, IgM and IgA levels are useful to investigate children with recurrent ENT/airway infections.

It is also worth considering an HIV test if symptoms are consistent, but ensure you have a discussion with parents before testing.

If both FBC and immunoglobulins are normal in the setting of recurrent infections, it is perfectly acceptable to wait for 3 -6 months to see if the condition improves before referral.

 

Conclusions

  • Primary immunodeficiencies are rare but important, and ENT infections may be the presenting feature
  • The severity of infection and presence of opportunistic pathogens are a much stronger predictor than the frequency of infections
  • Basic tests such as FBC and Immunoglobulins should be performed in children prior to/pending referral if they are not severely unwell
  • Consider investigation and referral for primary immunodeficiency early in children with severe infections and failure to thrive, or those with family history

Further reading: https://www.entmasterclass.com/ENT_Journal_2019_Interactive.pdf page 9

Otitis media

Cite this article as:
James Miers. Otitis media, Don't Forget the Bubbles, 2013. Available at:
https://doi.org/10.31440/DFTB.3131

A 2-year-old presents to ED with pyrexia – he’s pulling at his ear constantly in the waiting room.  You know where this heading, but are you supposed to treat him with antibiotics or not?

Do you know the difference between acute otitis media (AOM) and otitis media with effusion ?

 

Bottom Line

 

What is it?

Otitis media is a general term that has been used to describe multiple disorders of middle ear inflammation

Acute otitis media (AOM)

Otitis media with effusion (OME)

Chronic otitis media (COM)

Establishing the diagnosis is important as antibiotic treatment is not recommended for OME

The definition of acute otitis media requires three equally important components, which must all be present.

  1. Acute onset (<48 hours) of signs and symptoms
  2. Middle ear effusion (MEE)
  3. Signs and symptoms of middle ear inflammation (at least one of fever, otalgia, irritability in an infant; red tympanic not due to crying or fever)

The normal tympanic membrane

Who gets it?

There is considerable overlap between presenting signs and symptoms of upper respiratory illnesses and acute otitis media, especially in the preverbal child.

The peak incidence of otitis media is between 6 and 18 months of age. Neonatal acute otitis media is uncommon. Most effusions of the middle ear in this age are sterile and develop in the in utero environment.

Environmental insults, such as tobacco smoke, lack of breastfeeding, and exposure to children in daycare who frequently receive antibiotics, may lead to episodes that would not otherwise occur.

Prevention of the disease burden is possible through the use of newer vaccines such as the contemporary pneumococcal vaccine that includes protection from seven serotypes of Streptococcus pneumoniae.

What are the most common bugs?

Bacteria from the nasopharynx are the most common cause of acute otitis media and can be isolated from middle ear fluid in a majority of cases. The most common pathogens in the post-pneumococcal vaccine era are:

  • Nontypeable Haemophilus influenzae (56%)
  • S. pneumoniae (31%)
  • Moraxella catarrhalis
  • Mycoplasma may cause a bullous myringitis

Neonates may be affected by gram negative organisms and Staph aureus.

The role of viruses is unclear.

These data were obtained in the post-pneumococcal vaccine era and represent a change from before this vaccination program was initiated. Of importance is a major change in the increased prevalence of β-lactamase organisms such as M. catarrhalis (almost 100%) and non-typeable H. influenzae (35% to 40%).

 

What is otitis media with effusion (OME)?

OME usually follows an episode of acute otitis media, and both are processes of the same disease continuum. OME may persist for weeks to months after an episode of acute otitis media.

Close to 90% of episodes of OME  resolve spontaneously after an acute otitis media episode is diagnosed.

Guidelines recommend watchful waiting without the immediate use of antibiotics for children with uncomplicated otitis media with effusion.

Children with permanent hearing loss, craniofacial anomalies, or underlying speech delays may receive immediate antibiotics or have close outpatient follow-up by the primary care clinician.

 

Should I treat it?

Consensus guidelines strongly recommend the treatment of pain associated with acute otitis media.

Prescribing only an antibiotic for the treatment of acute otitis media is inappropriate care, as antibiotics are not analgesic medications.

The primary systemic analgesics and antipyretics used to treat acute otitis media are ibuprofen and paracetamol. In one randomized trial comparing ibuprofen, acetaminophen, and placebo, only ibuprofen was found to be superior to placebo.

Opioid medications may be used at night during the sleeping hours, although no studies have demonstrated the effectiveness of this medication class for the treatment of acute otitis media.

High-dose amoxicillin, 80 to 90 mg/kg PO per day divided into two daily doses for 5 to 7 days, is the first-line recommended antibiotic for the treatment of uncomplicated acute otitis media.

The higher dose achieves concentrations in the middle ear that exceed the minimum inhibitory concentration for highly resistant forms of S. pneumoniae, the most common bacteria found in acute otitis media.

M. catarrhalis and non-typeable H. influenzae, although the addition of clavulanic acid increases the likelihood of vomiting and diarrhoea.

IM ceftriaxone for three daily doses may be considered if children cannot tolerate oral medications. Children with a known allergy to the penicillin class may consider the use of a macrolide agent, such as azithromycin.

A 10-day course of antimicrobials has been recommended for decades without any evidence to support an exact duration of therapy. Shortened treatment regimens (5 to 7 days) may reduce resistance to antibiotics and reduce side effects by decreasing total drug exposure.

What is the evidence for treatment?

Two randomized double-blinded placebo-controlled trials of young children diagnosed with AOM

Hoberman et al, University of Pittsburgh

Tahtinen et al, Turku University Hospital in Turku, Finland

In children between 6 months and 2 years of age with certain AOM, the use of antibiotics result in faster recovery and less treatment failure (reduced by 42-62% in the severe group)

Although a majority of patients receiving placebo also recovered spontaneously (53% in Hoberman et al, 55% in Tahtinen et al.), a physician cannot predict at the onset of illness which patients will improve without antibiotics.

Even in patients with non-severe illness, antibiotics were associated with a decrease rate of treatment failure.

Summary of Treatment

Antibiotics are advised for:

  • All infants under 6 months
  • Age 6 months – 2 years with certain diagnosis or uncertain diagnosis and severe illness
  • Age 2 years and over with certain diagnosis and moderate/severe illness

Observation:

Age 6 months onwards with uncertain diagnosis and follow up assured observation without antibiotics is an option for mild illness (no systemic features) with re-evaluation at 48 hours & antibiotics if no improvement

Summary of Antibiotic Choice

  • Antibiotic in the studies was amoxicillin-clavulanate
  • 1st line treatment is high dose amoxicillin, due to reduced side effects & drug exposure
  • Whether amoxicillin alone will yield the same benefit is unclear, although in previous studies  80% were successfully treated with amoxicillin
  • In severe disease, amoxicillin-clavulanate is the preferred first line

 

What are the complications?

Perforation

As in the photo below, there can still be a perforation that does not heal within a few weeks.  This is considered to be a ‘natural myringotomy’ and usually heals well.  Only GP follow-up is needed.

 

Small perforation

A small tympanic perforation taken from the Hawke Library

 

Facial Palsy

The facial nerve descends over the posterior wall (mastoid) of the middle ear and can be affected by OM

 

 

Mastoiditis

The infection can extend into the mastoid (posterior to the middle ear).  It can cause bone destruction, brain abscess, meningitis, VI and VII nerve palsies, lateral venous sinus thrombosis.  It is very unlikely if the ear exam is normal as it is linked to OM.

A CT is advised along for IV antibiotics (usually ampicillin)

 

Acute mastoiditis

 

Cholesteatoma

Cystic cholesterol lesions are linked to chronic OM and can erode local structures.

 

Cholesteatoma

Sepsis

Bacteraemia occurs in 3%.

 

Deafness

Conductive deafness can follow a persistent effusion.

 

Resolution and Follow-Up

Fever and ear pain should be expected for 24 to 48 hours after an ED evaluation. If symptoms persist 72 hours after antibiotic therapy has been initiated, reevaluation is needed. Routine, scheduled visits are not recommended for uncomplicated acute otitis media if symptoms have resolved.

Improvement should be evident by 2-3 days. Treatment failure may indicate incorrect diagnosis, development of a complication or presence of a resistant organism. If the initial treatment was amoxicillin, change to amoxicillin/clavulanate.

All children should be reviewed by the local doctor at 2 weeks for the presence of a persistent effusion (glue ear) or healing of the tympanic membrane (if ruptured).

ENT consultation is advised for children with persistent hearing loss or speech delay, chronic effusion for more than 3 months, frequent episodes of otitis media or underlying pathologies such as cleft palate or adenoidal hypertrophy.

 

References

Best Bets – Otitis Media

Hawke Library – otitis media guide