Ent, eye and dental disorders

22.1

B. Benjamin

Paediatric ear, nose and throat (ENT) disorders cover a wide field of congenital and acquired diseases of the ear, nose and paranasal sinuses, oral cavity, tongue, pharynx, larynx, tracheobronchial tree and oesophagus. They include craniofacial abnormalities, tumours and cysts of the head and neck, deafness and speech, language and communication problems. This section covers the common disorders.

The ear

The external ear

Congenital abnormalities

Differences in the shape of the pinna are common – small ears, large ears, accessory skin tags and unusual configuration of the helix and antihelix – but usually no treatment is needed except for unsightly protruding ears, which can be corrected surgically. Major abnormalities of the external canal such as stenosis or atresia are sometimes associated with small, malformed pinnae, abnormalities of the ossicles and possibly hypoplastic inner ear abnormalities with consequent major hearing problems. When these anomalies affect both ears, hearing aids or reconstructive surgical procedures may be necessary.

Otitis externa

Swimming in contaminated or heavily chlorinated water predisposes the delicate skin lining the ear canal to infection with bacteria or fungi. Otitis externa causes itch, soreness, discharge and partial deafness. Treatment includes removal of debris by swabbing and/or syringing followed by careful drying and regular administration of appropriate antibiotic/steroid drops. Water in the ear should be avoided.

Severe pain and exquisite tenderness indicate acute localized or acute diffuse infection requiring systemic antibiotics, especially when there is surrounding cellulitis, lymphadenitis or generalized toxicity. ENT referral and hospitalization may be required in severe or intractable cases.

Wax

Foreign bodies

Foreign bodies such as beads, pips, pieces of paper, insects, etc. sometimes become lodged in the external auditory canal where they cause discomfort, pain and partial deafness. Occasionally they are dis­covered by chance. If attempts at careful extraction using a syringe or small grasping forceps are unsuccessful in a young or fractious child, referral to a specialist is indicated for removal, if necessary under general anaesthesia. Rough or ill-judged attempts at removal may cause damage to the tympanic membrane or the middle ear ossicles.

Injury of the tympanic membrane

Indirect trauma such as a slap or blow to the ear, a blast injury or impact with water can compress the column of air in the ear canal and rupture the tympanic membrane. Direct trauma may be caused by a cotton bud, hairpin or an incorrect syringing technique. There is pain, bleeding, deafness and some initial unsteadiness. On inspection, bleeding or a tear may be visible. The ear should not be cleaned and no drops should be given. Antibiotics are usually given to prevent infection. Almost all traumatic injuries will heal within a month or two: if not, a graft may eventually be necessary.

The middle ear

This air-containing, irregularly shaped, bony cavity is lined by mucous membrane and includes the mastoid air cells posteriorly and the eustachian tube anteromedially. The latter opens and closes on swallowing, yawning and blowing the nose and has an active mucociliary lining to cleanse, ventilate and maintain air pressure in the middle ear. Motion of the tympanic membrane and the lever action of the three small, articulated ossicles create an efficient transducer mechanism to transfer sound energy at the air–water interface. As the footplate of the stapes moves rapidly in the oval window, vibrations in air become wave motion in the perilymph fluid of the inner ear.

Acute suppurative otitis media

Acute suppurative otitis media (ASOM) is due to infection of part or all of the mucoperiosteum that lines the spaces of the middle ear. The diagnosis can be verified only by examination of the tympanic membrane.

It is much more common in infants and children than in adults, with a peak incidence under 2 years of age and again between 5 and 7 years. It is more common in winter and where there is overcrowding and malnutrition. About 50% of children will have experienced an attack before the age of 2 years and about 75% by the age of 3 years. This high incidence is apparently due to an immature immune response and increased frequency of upper respiratory tract infections in this age group. The overall incidence is also much higher in Aboriginal children in Australia.

Many factors predispose to middle ear infection:

• pre-existing middle ear effusion or ‘glue ear’

• infants and small children have a short, wide, straight eustachian tube, the dynamic protective function of which is less effective in minimizing middle ear contamination from the nasopharynx than the mature adult eustachian tube

• nasopharyngeal disease, such as seen with acute or chronic upper respiratory tract infection, enlarged infected adenoids and (to a lesser extent) tonsils or rhinosinusitis can act as a focus of infection

• coexistent chronic middle ear disease such as chronic otitis media or a pre-existing tympanic membrane perforation

• cleft palate or repaired cleft palate or other rarer craniofacial structural abnormality affects the normal opening by the palate muscles and the normal closure by the spring action of the cartilaginous portion of the eustachian tube

• contamination of the nasopharynx in babies being bottlefed in the recumbent position or in infants who are vomiting

• attendance at preschool or kindergarten with exposure to pathogens

• parental cigarette smoking

• deficiency of surface-tension-lowering substance, surfactant, in the tube

• abnormality of mucociliary action affecting the normal cleansing mechanism

• immunodeficiency syndromes.

In ASOM, the pathological sequence of events in the air spaces and mucosa of the middle ear proceeds rapidly with oedema, hyperaemia and exudate into the middle ear, more often than not following a head cold or upper respiratory tract infection. Inflammatory swelling occludes the eustachian tube. The serous fluid becomes purulent after secondary bacterial infection and causes bulging of the pain-sensitive tympanic membrane. The body’s natural defences, with or without assistance from antibiotics, usually achieve resolution. If not, the tympanic membrane continues to bulge, forming an area of ischaemic necrosis that ultimately ruptures.

The microbiology of ASOM primarily involves bacteria but in 5–10% viruses may play a role, usually paving the way for secondary bacterial invasion. Common organisms include Streptococcus pneumoniae, Streptococcus pyogenes, Branhamella catarrhalis, Haemophilus influenzae (especially in younger children), Staphylococcus aureus and some Gram-negative or mixed infections. About 30–40% of aspirates will yield no pathogen.

The clinical features of ASOM vary with the age of the child, the efficiency of the host defence and the effectiveness of treatment. Severe, throbbing pain in one or both ears is the commonest feature. There may be minor earache for an hour or two or a fulminating febrile illness with acute pain. These symptoms are often worse in the evening or at night when the child is lying down. Infants may present with fever, attempts to pull at the affected ear, irritability, vomiting and abdominal pain. Rupture of the tympanic membrane, with bloodstained then purulent discharge, relieves the pain and allows a culture and sensitivity to be obtained. If perforation occurs it usually heals within a few weeks.

The diagnosis is confirmed by the appearance of the tympanic membrane. However, many sick, irritable infants and smaller children are difficult, if not impossible, to examine, so in some cases a clinical diagnosis is made and treatment is commenced without visualization of the tympanic membrane.

The progression of ASOM can be divided into four stages:

1. Eustachian tube obstruction with a stuffy, blocked feeling of discomfort in the ear and a slightly retracted, pink tympanic membrane

2. Early infection with increasing earache, fever, and redness due to some mucoid or purulent material behind the tympanic membrane

3. Suppurative stage with severe local pain, constitutional symptoms and purulent exudate under pressure, leading to bulging of the tympanic membrane, which develops a yellowish colour with ischaemic necrosis prior to rupture

4. Resolution stage with dramatic lessening of pain and improvement of the tympanic membrane.

Treatment usually requires bed rest, adequate fluid intake, antipyretics and sufficient analgesic medication. Sometimes local warmth is helpful.

Although there is discussion about whether antibiotics are given too freely, many experienced physicians believe that antibiotics limit the disease, control pain and minimize possible complications. Others suggest that antibiotics should be withheld in non-severe cases pending further observation and given to those not recovering in 24–48 hours. It is generally agreed that children under 2 years of age should be treated with antibiotics rather than adopting a wait and see policy.

As a first-line treatment amoxicillin for 5–10 days is the drug of choice and is generally well tolerated. Erythromycin, sulfamethoxazole–trimethoprim or cefaclor are alternatives. Very occasionally a resistant or complicated infection requires myringotomy. Severe, otherwise uncontrolled infections require intravenous treatment in hospital.

There are no data to support the use of decongestants or antihistamines: in fact there is some evidence that they may be harmful. Topical antibiotic drops have no place in the treatment of acute suppurative otitis media.

The untoward sequelae of otitis media include:

• incomplete resolution with persistence of effusion (‘glue ear’)

• rarely, a ruptured tympanic membrane that will not heal. The chronic perforation will require grafting

• acute mastoiditis or its complications, which are still seen despite the use of antibiotics and usually present as a subperiosteal abscess behind the ear

• labyrinthitis with severe vertigo and vomiting

• intracranial complications, including lateral venous sinus thrombosis, extradural or subdural abscess, meningitis, cortical thrombophlebitis and intracerebral or intracerebellar abscess.

Remember that otitis media has not been ‘cured’ until both the appearance of the tympanic membrane and the hearing have returned to normal. The recently introduced universal pneumococcal immunization programme in Australia may be helpful in preventing recurrent ASOM, particularly for children attending day school.

Emma, aged 3 years 8 months, complained of a ‘sore’ ear late in the afternoon, refused her dinner and later developed distressing pain and a fever of 39.4°C. The family doctor found a crying, upset, vomiting child and an agitated mother. Otoscopy showed a red, bulging tympanic membrane on the left and a thickened, pink membrane on the right. The advice was bed rest, fluids as tolerated, paracetamol, amoxicillin and a progress examination next day.

Note that acute suppurative otitis media causes severe pain, often worse in the evening. Both ears and the upper respiratory tract must be examined.
Glue ear or otitis media with effusion

A confusion of names have been applied to ‘glue ear’ but otitis media with effusion (OME) or secretory otitis media are those used most often. It is a common cause of repeated earaches, fluctuating mild to moderate conduction deafness and educational impairment.

The aetiology is uncertain but the ventilation, drainage and clearing mechanism of the eustachian tube is abnormal. Organisms similar to those found in ASOM can be cultured in 30–50% of cases – the effusion apparently follows incomplete resolution of ASOM. A mucoid, non-purulent effusion, containing leukocytes, dead or live bacteria, serum protein and mucus, accumulates in the middle ear. The effusion may be thin, thick, gelatinous or, in advanced cases, even ‘rubbery’.

The middle ear mucosa becomes oedematous and granular in appearance. Microscopically, the goblet cells and mucous glands increase dramatically in number and small cysts filled with watery or inspissated mucus can be seen in the thickened subepithelial layer, which is infiltrated by chronic inflammatory cells. It is now believed that biofilm (a blanket of bacteria in a very low metabolic state and enclosed in a polymeric matrix) in the middle ear may be a contributing factor in otitis media with effusion.

The clinical features of OME are common up to 8–10 years of age, are usually seen first in winter and are sometimes variable and unpredictable. Symptoms often follow a viral upper respiratory tract infection or incompletely resolved ASOM. Because at first there may be no symptoms the condition may remain unrecognized. Earache and deafness are the two important features.

Earache presents in two ways. Firstly, as a flare-up during an acute respiratory infection there may be typical ASOM with severe pain. Secondly, repeated ‘small’ earaches, lasting for minutes rather than hours, may wake the child at night or occur at school and settle quickly.

Deafness is often suspected by the parents or teacher or may be discovered at a routine screening hearing test. School performance is often affected: ‘doesn’t pay attention’, ‘can do better’ or ‘not concentrating’ are frequent remarks. Non-specific symptoms include poor school achievement, decreased learning skills, interference with language development, an adverse affect on emotional growth, irritability and personality changes. The child may be at an educational disadvantage. A few children become clumsy if their balance is mildly affected. Infants may be irritable, crying or constantly unsettled at night.

Recognition of physical signs in the tympanic membrane is often difficult for the inexperienced: a good pneumatic otoscope with magnification is invaluable. The common physical signs include:

• yellow or amber appearance of the tympanic membrane

• vascular dilatation, which is easier to see with magnification

• thickening and dullness of the tympanic membrane

• indrawing of the tympanic membrane, giving a concave appearance so that the handle of the malleus appears short

• in advanced cases there may be atelectasis in the middle ear with atrophy and thinning of the tympanic membrane.

Sluggish or poor movement of the tympanic membrane detected using a pneumatic otoscope is a most important physical sign. The thicker the fluid, the less the ‘bounce’. In children old enough to perform a pure tone audiogram (Fig. 22.1.1) a mild to moderate conduction deafness will be detected. Impedance tympanometry tests the bounce of the tympanic membrane and typically shows a ‘flat’ curve.

In many children OME will resolve without treatment over weeks or months. However, referral for specialist assessment is indicated:

• when infants and toddlers are persistently irritable, sleep poorly at night and rub or pull at their ears

• when older children have repeated earaches and/or persistent hearing loss.

There is no evidence that antibiotics, antihistamines, decongestants, mucolytics or antiallergy treatment have any significant beneficial effect.

In some cases, after adequate observation indicates that the effusion has been present for 3 months or more, myringotomy, suction removal of the fluid and insertion of tympanostomy tubes for middle ear ventilation/drainage is indicated. The tube remains in the tympanic membrane, functioning as a ‘temporary’ artificial eustachian tube providing sustained middle ear ventilation, discouraging recurrent effusions and promoting recovery, usually without any complications. The tube is extruded through natural mechanisms after 6–12 months but the effusion can recur. Insertion of the tubes may need to be repeated if the symptoms warrant it. In some children, predisposing causes such as infection and hypertrophy of the adenoids or upper respiratory tract mucosal disease or rhinosinusitis need treatment.

If untreated, the long-term irreversible complications of OME include atrophic thinning of the tympanic membrane, atelectasis of the middle ear space, damage to the middle ear mucosa, adhesive otitis media, retraction pockets, avascular necrosis of the incus and stapes, tympanosclerosis, cholesteatoma and cholesterol granuloma.

Clinical example

John’s mother was called to his school because he complained of earache and the teacher observed that he was not hearing well. She stated that her 6-year-old son had suffered four attacks of ‘ear abscess’ in the last 10 months. On examination, both tympanic membranes were dull and dilated vessels were seen to be running in a radial fashion. The tympanogram was ‘flat’. Referral to an ENT surgeon for treatment of glue ears was arranged. In fact, John had probably had otitis media with effusions (OME) for 10 months. Parents may be unaware of the hearing loss in OME. Variable deafness and ear infections almost always indicate OME.

Possible sequelae of acute suppurative otitis media are:

• Complete resolution

• Residual ‘glue ear’ and minor deafness

• Acute mastoiditis or a complication of mastoiditis

• ‘Masked mastoiditis’

• Open chronic perforation
Chronic suppurative otitis media

In this condition the tympanic membrane is perforated. The patient complains of partial deafness and painless, recurrent or persistent discharge.

There are two types of chronic suppurative otitis media (CSOM):

• mucoperiosteal disease with a central perforation from pinhole size up to complete destruction of the tympanic membrane. The basic middle ear disease is chronic, persistent or intermittent infection with a purulent, sometimes profuse, discharge due to infection of the mucoperiosteum of the middle ear space. For this reason it is often called tubotympanic disease. Because complications are rare this is known as a ‘safe’ ear. A clean, dry, chronic, central perforation, whether large or small, is suitable for tympanoplasty, using fascia or perichondrium, usually waiting until the child is 7–8 years old. Grafting is successful in about 95%, prevents recurrent infections causing further damage, restores hearing and allows swimming without the risk of infection

• bony disease with a small marginal perforation at the superior bony edge of the tympanic membrane is usually associated with cholesteatoma and chronic, often smelly discharge. Complications are likely; hence this is an ‘unsafe’ ear.

A cholesteatoma is not a tumour but is a very slowly enlarging, pearl-like pocket of misplaced squamous epithelium accompanied by enzymatic destruction of the surrounding bone and ossicles. It is sometimes called atticoantral disease because of its position high in the middle ear.

Mucoperiosteal infection is usually controlled by repeated dry mopping or suction cleaning of the ear and use of appropriate topical antibiotic drops. Water must not get into the ear. The common organisms are Pseudomonas aeruginosa, Bacillus proteus and Escherichia coli. Therefore a chronic or intermittently discharging ear must not be neglected. Cholesteatoma and bony disease usually requires surgical mastoidectomy to remove the disease, combined with tympanoplasty to repair the tympanic membrane and reconstruct the ossicular chain to preserve hearing.

The complications of cholesteatoma include erosion and destruction of the ossicles, osteitis, petrositis, mastoiditis, labyrinthitis, facial nerve paralysis, thrombosis of the sigmoid venous sinus, meningitis, extradural, subdural or cerebral abscess and septicaemia. Some of these complications are life threatening.

Deafness

The accurate assessment of the type, degree and cause of deafness is essential for optimum treatment.

The type of hearing loss is usually described according to the site of pathology:

• ‘Conductive’ deafness. Sound vibrations are not conducted normally via the ear canal (for example, obstructing wax), the tympanic membrane (for example, perforation) or the ossicles (e.g. congenital malformation). Conduction deafness is usually treatable

• ‘Sensorineural’ deafness. There is malfunction of the sensory (cochlear) components (for example, rubella deafness) or the neural (retrocochlear) components (e.g. acoustic neuroma). Sensorineural deafness is often congenital, may be acquired and cannot be cured. If severe enough, sensorineural deafness requires a hearing aid for amplification, or consideration for cochlear implant.

Universal newborn screening for deafness. Language and communication will ultimately be better when deafness is identified early so that a hearing aid and a support programme can be provided. However, it has been difficult to assess the hearing of small children with the traditional informal and special testing techniques (described below) which require a child to be old enough for some cooperation. It is now hoped that universal hearing screening tests which are being introduced for the newborn in Australia will detect most congenital deafness.

When a sound is heard by the ear a tiny corresponding ‘echo’ can be measured by a computer using a small probe placed in a baby’s ear canal – the basis for a non-invasive screening technique called oto-acoustic emissions (OAE) testing. Because the test takes only a few minutes and does not rely on the participation of the baby it is suitable for newborn hearing screening. If the initial result is unreliable, for example if the baby is restless or irritable, further evaluation is necessary. A small number of babies will need referral for auditory brainstem response tests (see below), which can give more reliable information about the hearing

Certain ‘at risk’ criteria can be used to select babies, all of whom should have hearing screening tests soon after birth, usually before leaving hospital, including those with a family history of deafness, craniofacial abnormalities, exposure to ototoxic drugs and those who needed more than 48 hours in a neonatal intensive care unit.

In infants with deafness not detected by screening tests or those developing deafness after the newborn period hearing loss is commonly suspected by the child’s mother (Fig. 22.1.2). The degree of loss is assessed by informal and special audiological methods according to age:

• in infants days or weeks old, the normal but non-quantitative response to a sudden loud sound is the ‘blink’ or ‘startle’ reaction (Fig. 22.1.3). This test is best done with the infant lightly asleep. However, the universal screening programmes using OAE above will, when fully introduced, be a better form of testing

• in special units, computerized electrophysiological tests are available to record small electrical changes evoked in the inner ear during transmission of acoustic signals. An accurate measure of function in the brainstem and the ear can be provided by electrocochleography and auditory brainstem tests in infants and children, including those with behavioural problems or multiple handicaps

• in babies from 4 months of age, normal head turning responses (Fig. 22.1.4) are elicited towards the side of a sound stimulus, which can be varied from a soft whisper to the jingle of keys or the crumple of paper. An approximate, clinical quantitative estimate of hearing can be obtained

• older toddlers can be assessed in specialized paediatric units by behavioural methods, observing the child’s reaction to ambient sounds or by conditioning them to respond to a puppet or peep show – known as condition-oriented response (COR) audiometry

• children of 3 or 4 years of age or older can usually cooperate so that a quantitative pure tone threshold audiogram (Fig. 22.1.1) can be obtained for different frequencies for both air conduction and bone conduction.

The cause of deafness determines the need for treatment. The many causes of conduction deafness each need specific attention, e.g.:

• removal of wax

• operation for congenital external ear canal atresia

• myringoplasty for tympanic membrane perforation

• ventilating tube for persistent otitis media with effusion

• reconstruction of congenital ossicular chain abnormality.

Approximately 1 baby in 1000 is born with severe deafness, and approximately 1 in 1000 infants become deaf before they have developed speech. In many cases the cause of sensorineural deafness is difficult to determine. The known causes can be considered in four groups, as below.

Prenatal hereditary deafness

This is transmitted by a dominant gene in 10%. If one parent carries the gene, up to 50% of children will be affected. There is a recessive gene in 90%, and in this situation both parents must carry the gene and 25% of children will be affected. Consanguinity increases recessive transmission. There is, therefore, sometimes a positive family history of deafness. There are many hereditary syndromes with hearing loss as a feature. Some examples include:

• Waardenburg syndrome – epicanthic folds, different-coloured irises, white forelock

• Usher syndrome – retinitis pigmentosa, epilepsy

• Pendred syndrome – sporadic thyroid disease

• Alport syndrome – progressive renal disease

• Hurler syndrome – gargoylism

• Fanconi syndrome – anaemia, skin pigmentation, skeletal deformities, mental retardation.

Prenatal acquired deafness

Damage in the first trimester can affect the developing cochlea. Detailed radiological imaging will define the Michel deformity, which is total absence, and the Mondini deformity, which is partial maldevelopment of the bony cochlea. The Scheibe deformity has a normal bony cochlea but damaged hair cells in the organ of Corti. Causes of prenatal acquired deafness include maternal infection such as rubella, cytomegalovirus, toxoplasmosis, herpes and congenital syphilis. Drugs that are ototoxic to the embryo include aminoglycosides, loop diuretics, quinine and thalidomide.

Perinatal acquired deafness

Causes include prematurity, prolonged or difficult labour, hypoxia, Rhesus incompatibility, kernicterus, ototoxins, infectious diseases and others.

Deafness in infancy and childhood

The common causes of acquired deafness at this age are mumps, measles, meningitis, traumatic fracture of the petrous bone through the inner ear or acoustic nerve, and patent cochlear aqueduct. Fortunately in these cases, if only one ear is affected, the handicap is not as devastating as with bilateral deafness.

Treatment of sensorineural deafness

Most deaf children have normal intellectual capacity and some usable residual inner ear function. The diagnosis must be made early and the child fitted with hearing aids or be considered for a cochlear implant. He or she should receive early and con-tinued auditory training. Many such deaf children learn to understand the spoken word, to develop intelligible speech and play an active role in society. However, the hearing of speech sounds does not guarantee normal understanding, as amplification may be accompanied by distortion and decreased intelligibility. Thus general practitioners, paediatricians and otologists have an enormous responsibility to take the mother’s suspicion of deafness seriously and arrange prompt investigation and assessment. Early diagnosis is the key to optimal outcome.

A geneticist should give advice to parents who have had a deaf child and who want to know the chances of having another – is the problem dominant deafness or recessive? The ability to identify previously undiagnosed cases of hereditary deafness on the basis of specific genetic testing has improved. DNA-based testing is available for the diagnosis of mutations that cause deafness, often those in the GJB2 gene which encodes the protein connexin 26. Up to 50% of those with non-syndromic deafness have mutations in the gap junction protein beta-2 gene.

The nose

Many congenital anomalies affect the nasal structures:

• craniofacial and external nasal malformations

• cleft lip, palate and face clefts

• haemangioma and vascular malformations

• dermoid, encephalocele, nasolacrimal duct cyst and other rare masses

• bilateral congenital choanal atresia poses the greatest threat to life because neonates, being obligate nose breathers, develop increasing cyanosis and even fatal asphyxia when their nasal airways are completely obstructed. However, if the baby cries and takes a breath through the mouth the obstruction is momentarily relieved until the mouth closes. Choanal atresia can be confirmed by failure to pass a 3  mm diameter plastic catheter through the nose into the oropharynx. The airway can be maintained using a Guedel oral airway or an endotracheal tube pending computed tomography (CT) assessment and surgical correction

• unilateral choanal atresia presents as persistent glairy discharge later in life.

Acute rhinosinusitis

Acute infective rhinosinusitis presents with purulent nasal discharge, nasal obstruction, pain and tenderness over the involved sinuses, and general malaise. The acute episode often follows an upper respiratory tract infection, swimming or diving and usually affects the maxillary sinuses, and in older children the ethmoid sinuses. Plain X-rays and CT are required only in difficult cases. Treatment is symptomatic, using decongestant nasal drops or oral decongestants and paracetamol. Antibiotics are given in severe, persistent, recurrent or complicated cases. Amoxicillin for 7–10 days to cover the common upper respiratory tract pathogens is the drug of first choice. In penicillin-hypersensitive patients use cefaclor, erythromycin or doxycycline (not in children under 8 years).

Infective rhinosinusitis in infants and children usually responds to medical treatment. Surgical drainage may be necessary for chronic disease or for acute complications such as subperiosteal abscess, periorbital cellulitis, osteomyelitis or intracranial spread.

Chronic rhinosinusitis

Chronic rhinitis often has an allergic basis, with secondary bacterial infection being common. In younger children it is aggravated by, or inseparable from, hypertrophy and infection of the adenoids. Swollen turbinates cause intermittent or persistent nasal stuffiness and catarrhal discharge. Therapy with antihistamines and pseudoephedrine may be helpful but symptoms are more often controlled with regular use of intranasal metered aerosol steroid spray. Nasal allergy, often with hay fever or asthma, may be traced to specific allergens, which should be avoided where possible. Desensitization can be considered in older children. In severe, persistent cases cautery, laser or surgical reduction of the inferior turbinates may provide substantial relief. Nasal polyps in children strongly suggest cystic fibrosis.

Nasopharyngeal tumours and cysts

Antrochoanal polyp, dermoid cyst, meningoencephalocele, glioma and chordoma are rare benign conditions. Nasopharyngeal angiofibroma is an uncommon locally destructive, non-metastasizing very vascular tumour occurring mostly in adolescent boys. It usually presents as frequent, often severe, epistaxes and nasal obstruction. Treatment is surgical removal after embolization of the feeding vessels, although some advocate radical radiotherapy. Rhabdomyosarcoma or lymphosarcoma are rare malignant nasopharyngeal tumours.

Trauma

Foreign bodies

Unilateral purulent, sometimes bloodstained nasal discharge in a young child suggests the presence of a foreign body, such as a bead, eraser, piece of vegetable or other material, until proven otherwise. Unreactive inorganic objects may remain undetected for months or years. The most dangerous foreign body is an alkaline battery, which emits a small current and leaks its caustic contents, rapidly causing local tissue necrosis; it must be treated as an emergency. Removal may require general anaesthesia.

Epistaxis

Bleeding from a prominent vessel on the anterior nasal septum (Little’s area) is common and is sometimes frightening for parents. It may be aggravated by accidental trauma, nose picking, nose blowing and infection. Occasionally epistaxis may be the presentation of a blood dyscrasia or a nasopharyngeal angiofibroma. First aid is to apply constant pressure to the side of the nose with a cold face cloth for at least 5 minutes. Packing with ribbon gauze controls many persistent cases. If the bleeding continues and the vessel can be identified, it can be thermally or chemically cauterized using topical anaesthesia in older cooperative children or under general anaesthesia in others.
Clinical example

Benjamin, aged 9, was struck on the nose by the seat of a swing while he was playing in the park. His nose bled, appeared ‘crooked’ and became swollen. He was taken to the casualty department, where the ENT registrar confirmed traumatic fracture of the nasal bones by clinical examination. Corrective surgery was arranged for later in the week.

Bruising and swelling will rapidly hide the deformity. Careful palpation usually confirms the displacement without the need for X-ray.
The oropharynx

Acute sore throat (AST) in children is a common problem. The illness can be due to viral, bacterial, fungal or other infectious microorganisms or of unknown aetiology. Most children have a cold and nothing more and are better in less than a week. A number of questions arise. Is the infection due to a respiratory virus or to a bacterial infection? Is a throat swab useful? Are antibiotics justified? Is the sore throat a manifestation of a more serious systemic disease? Is admission to hospital necessary?

Arbitrary division of the acute infectious process into the descriptive ‘diagnostic’ categories rhinitis, nasopharyngitis, stomatitis, pharyngitis and tonsillitis is somewhat unsatisfactory because the inflammation often extends to overlap nearby areas. Nevertheless, the majority of patients with an acute sore throat can be described as having either acute ‘pharyngitis’ or ‘tonsillitis’.

Inflammation is widespread in the mucous membrane in ‘pharyngitis’ which can be caused by one of many viruses, whereas in more localized ‘tonsillitis’ the pathogen is usually either group A beta-haemolytic streptococcus (S. pyogenes) or a virus – adenovirus, Epstein–Barr virus (EBV) or Coxsackie A virus. The differentiation of bacterial from viral infection on clinical grounds is far from easy – often no more than an educated guess – because, for the most part, the symptoms and local signs accompanying AST correlate poorly with the presumed (or later proven) aetiological microorganism. Most acute upper respiratory tract infections that cause sore throat, fever and swallowing discomfort last 3–6 days, are viral and are mild in severity. They seldom warrant antibiotic treatment.

In approximately 50% of patients with acute sore throat beta-haemolytic streptococci can be isolated by surface throat culture; it seems reasonable to assume a cause and effect relationship. In fact this organism is more likely to be pathogenic when there are also local clinical findings of intense cellulitis of the uvula and soft palate and haemorrhagic palatal petechiae. Remember that 10–20% of otherwise normal children may be carriers of beta-haemolytic streptococci.

What, then, is acute tonsillitis? Acute tonsillitis has been defined clinically as a condition in which not only is inflammation mostly confined to the tonsils but the clinical features also include acute sore throat, fever, difficulty in swallowing, enlarged tender regional cervical lymph nodes, halitosis and constitutional symptoms such as lethargy, nausea and vomiting. Sometimes there is abdominal pain. Examination can show various appearances in tonsillitis:

• red mucosa over the tonsils and oedematous, generalized inflammation (parenchymatous tonsillitis)

• yellowish-white exudate in the crypts of the tonsils (follicular tonsillitis)

• the crypts become filled with ‘debris’, an exudate of desquamated epithelium and pus

• coalescence of these follicles can form a thin, white, non-confluent, patchy membrane that peels away without bleeding (membranous tonsillitis)

• the typical redness, oedema and purulent secretion (exudative tonsillitis). Note that this appearance is not necessarily diagnostic of streptococcal infection.

Acute sore throat is one of the commonest complaints seen in general practice. It is occasionally a manifestation of a serious systemic disease. A useful classification of causes includes:

• acute viral pharyngitis. Examples are coryza, influenza, parainfluenza, the viral exanthemas and infections with Coxsackie viruses (herpangina), the ECHO virus group and many others. Coryza (common cold, viral nasopharyngitis, viral catarrh) is usually caused by rhinoviruses and coronaviruses in winter and spring and by enteroviruses in summer and autumn and at other times by other viruses. Coryza is highly infectious. There is an initial burning sensation above the palate, then sore throat, which is generally not severe, minimal fever, stuffy nose, rhinorrhoea and conjunctivitis. It usually resolves in 4–5 days. Complications include acute sinusitis, otitis media and lower respiratory tract infections

• acute bacterial tonsillitis, often due to beta-haemolytic streptococci but occasionally to other bacterial organisms in immunocompromised patients. It seems also that adenoviruses and Epstein–Barr viruses may lie dormant in tonsils for years and can be activated by non-specific environmental factors such as fever, chilling and stress

• infectious mononucleosis (glandular fever), caused by the Epstein–Barr virus, has many manifestations. Acute sore throat is consistently a prominent feature

• thrush, due to Candida albicans and predisposed to by diabetes, general debility, immunosuppression, nutritional deficiency and disturbances in the normal flora due to prolonged administration of antimicrobial agents. There is a wide range of appearances but characteristically there are white, curd-like, adherent patches overlying inflamed mucosa on the gingival surfaces

• Vincent angina (also known as ulceromembranous gingivostomatitis), caused by a combination of the normal spirochaetes of the mouth and mixed anaerobic bacteria often in injured necrotic tissue of the gums, which provides the necessary anaerobic environment

• diphtheria is now uncommon yet it remains a potentially lethal acute infection, caused by Corynebacterium diphtheriae, which produces a powerful exotoxin. A sore throat is part of the much more serious systemic toxic illness

• aphthous stomatitis. The cause is unknown. These recurrent, non-infective, well demarcated, painful ulcers have an erythematous border and are usually in the anterior part of the oral cavity on the mucosa of the lips, mouth and gingivae and the borders of the tongue, but when they occur in the soft palate or the fauces the patient complains of sore throat

• patients with acute leukaemia, agranulocytosis, aplastic anaemia or HIV infection may present with an acute sore throat.

Approximately 50% of cases of acute sore throat are eventually proved to be bacterial. Pending the result of culture it would seem prudent to immediately treat with an antibiotic those children who are extremely ill or toxic, and to await the result of culture in other children.

Examination of the mouth and throat

Older children are examined sitting up in a chair or on a bed and younger children sitting on mother’s lap or lying on the bed. It may be helpful to have an assistant steady the head. A torch with a bright light and a wooden tongue depressor is usually used but, where available, a headlight and an angled metal tongue depressor will provide a better view of the oropharynx. The lips, buccal mucosa, teeth and gums, floor of the mouth and the tongue are examined and then the palate, tonsils and posterior pharyngeal wall.

In severely ill children, brief examination of the oropharynx will eliminate grossly enlarged tonsils, peritonsillar abscess, retropharyngeal abscess and the rare case of diphtheria. In suspected acute epiglottitis care must be taken not to worsen airway obstruction, and the oropharynx should only be examined under expert controlled conditions if epiglottitis is a significant possibility.

Throat swab and rapid antigen testing

Pharyngitis caused by adenoviruses or herpes sim­plex is generally indistinguishable from that caused by S. pyogenes. Viral studies are rarely helpful. The only way to confirm a bacterial infection and justify treatment is to identify the streptococcal organism using a rapid antigen test or to take a throat swab, if facilities for culture and laboratory identification are available. Although not diagnostic, at times a Gram stain for tentative identification would be a reasonable basis for the commencement of anti­biotic therapy. Identification of S. pyogenes allows logical use of an antibiotic for those few who are judged to be unduly ill or those ‘at risk’ (see below under Antibiotics) and as a valuable reference in a patient who may later be suspected of having rheumatic fever.

Although seldom used, rapid immunological tests employ antiserum against group A streptococcal antigen. Streptococci obtained from a throat swab by chemical or enzymatic extraction are tested for agglutination using antibodies to group A Streptococcus. Such tests are reasonably accurate but the false-negative rate can be up to 30% – then a throat swab may be necessary.

A throat swab should preferably be taken before any antibiotics are given. Technique is important – with adequate exposure and illumination, sterile cotton wool swabs are rubbed vigorously on the areas of inflammation or exudate on the tonsils and posterior pharyngeal wall and put directly into a sterile container. Because the throat is normally colonized by many organisms, laboratory tests taking 24–48 hours are done to single out and identify the group A beta-haemolytic streptococcus. Remember, if diphtheria is a possibility the laboratory should be contacted as special tests are necessary.

Antibiotics

The difficult dilemma is that, although it might often seem reasonable to give antibiotics to a sick child with a sore throat, available evidence does not support their administration whether the infection is streptococcal or viral. When laboratory confirmation of a bacterial cause is available it is rational to give antibiotics to those at risk of remote complications such as post-streptococcal rheumatic fever or, less likely, acute glomerulonephritis. In others, the risk of these complications is not increased by delay in giving antibiotics for 24–48 hours: in fact treatment begun within 7–9 days of the onset is effective. Bear in mind that 10–20% of otherwise normal children may be carriers of S. pyogenes and that con­siderably fewer than 50% of cases of AST are eventually proved to be bacterial. In many cases no pathogen is isolated.

Antibiotics confer minimal worthwhile benefit (symptoms may last for 1 day less) over purely symptomatic treatment (rest, paracetamol and fluids) and are seldom effective in preventing suppurative complications except in high-risk groups. Nevertheless, pending the result of a throat swab, it might be prudent to immediately treat those who are unduly ill or toxic or those at risk, including diabetics, immunocompromised individuals and Aboriginal, Torres Strait and Pacific Islander children.

There are many proprietary ‘remedies’ available for upper respiratory infections and sore throat but none can be recommended. Throat lozenges, including those containing antibiotic, are of no use and antihistamine/decongestant preparations can lead to troublesome side effects.

Features associated with an acute sore throat which should alert a perceptive medical attendant to a possible serious condition:

• Undue toxicity – the child appears sicker than might be expected

• Signs of respiratory distress, stridor or restlessness

• Difficulty swallowing or drooling

• Dehydration

• Marked or generalized lymphadenopathy

• Unilateral swelling in the pharynx

• Bruising or bleeding

• An adherent or obstructive membrane

Clinical example

The family doctor saw Andrew, a 7-year-old boy who had had a sore throat, difficulty in swallowing and fever for 2 days. The appearance in the throat was described as ‘tonsillitis’. An antibiotic was prescribed but when he was no better 3 days later it was changed to a different antibiotic. Again, Andrew was no better and in addition was complaining of feeling weak, with aching in the muscles and headache. Careful examination 8 days after the illness had commenced revealed tender, enlarged lymph nodes in the neck, axilla and inguinal region. His spleen was enlarged and tender. Blood tests were diagnostic of infectious mononucleosis.

An atypical sore throat needs caution about the diagnosis. Infectious mononucleosis is a systemic illness whose principal features are sore throat, fever, cervical (often generalized) lymphadenopathy and a feeling of malaise. Many organs in the body can be affected with protean manifestations. Antibiotics have no therapeutic value.
Indications for tonsillectomy and adenoidectomy

There is now reliable information to prove that the frequency of throat infections is reduced in selected patients undergoing these procedures. Operation is clearly indicated in a small number of children. There may be disagreement about the operation in individual cases and at times a second opinion may be in the best interests of the patient.

Indications for tonsillectomy

The indications for tonsillectomy are:

• repeated attacks of acute tonsillitis: at least three documented attacks a year for 2 years or more, making a minimum of 6 attacks in 2 years

• acute or chronic upper airway obstruction caused by enlarged lymphoid tissue. There may be an obstructive sleep pattern, even apnoea. Some cases with severe obstruction develop cardiac changes and cor pulmonale

• chronic tonsillitis. This usually applies to older children and adults

• peritonsillar abscess (quinsy). Two or more attacks are a definite indication for tonsillectomy

• biopsy excision for suspected new growth.

Indications for adenoidectomy

The indications for adenoidectomy are:

• enlargement causing severe nasal obstruction and breathing discomfort

• persistent discharge of infected mucopurulent material caused by large and infected adenoids

• possible benefit in repeated acute or chronic ear disease.

The tonsils and adenoids are often removed in a single, combined operation but there are clear indications for tonsillectomy alone or adenoidectomy alone. There is now greater awareness of the incidence and severity of obstructive sleep problems, which occur in an age range from 6 months to 10 years of age.

Contraindications to tonsillectomy and adenoidectomy

Tonsillectomy and/or adenoidectomy should not be performed if there is:

• a lack of staff or facilities to recognize and manage the potential complications

• recent respiratory tract infection, within the previous 2 weeks

• a systemic disorder, such as poorly controlled diabetes

• a bleeding disorder

• pharyngeal insufficiency, such as repaired cleft palate, submucous cleft palate or paralysis or paresis of the palate and so called ‘short’ palate. Adenoidectomy may cause or worsen escape of air through the nose (hypernasality), making speech difficult to understand.

Injuries of the tongue and oropharynx

Children with, for example, a pencil in the mouth may fall and injure the soft palate, tonsils or pharyngeal wall. At other times it is not uncommon for the teeth to lacerate the tongue and cause considerable bleeding. It is usually necessary to suture only the most severe of these injuries.

The larynx and trachea

Features of upper airway disease include:

• stridor: a prominent, audible manifestation of upper airway obstruction caused by turbulent airflow through a narrowed airway, usually the larynx or sometimes the trachea. It is most often inspiratory, sometimes expiratory and occasionally both

• other signs of partial or severe airway obstruction: tachypnoea, chest retraction

• cyanotic or apnoeic attacks

• husky, weak or absent cry

• repeated aspiration

• recurrent or atypical croup

• features of weakness, compression or stenosis of the trachea and/or bronchi.

Laryngomalacia

This is a common cause of stridor in infants. It is also appropriately called ‘floppy larynx’, both names implying collapse of the supraglottic tissues during inspiration. The cause is unknown. The features are intermittent inspiratory stridor, signs of upper airway obstruction, a normal cry and general health that is usually (but not always) normal. The features are often alarming to parents. As the condition is usually self-limiting there is seldom any need for treatment once a certain diagnosis has been established to differentiate laryngomalacia from the many other causes of stridor in infants. Occasionally, severe cases warrant laser removal of part of the redundant, floppy, supraglottic tissues.
Clinical example

Simon, aged 2 years and 6 months, ran to his mother in great distress, gasping for breath, coughing and crying. She rushed him to the nearby emergency department. The respiratory distress had lessened but pulse oximetry showed only 90% saturation. There was a wheeze and decreased air entry on the right side. An expiratory chest X-ray showed air trapping on the same side with shift of the mediastinum to the left. Simon was observed in the high-dependency ward until an impacted peanut was removed from the right main bronchus under general anaesthesia 90 minutes later. He made an uneventful recovery thereafter.

With inhalation of foreign bodies into major airways, if death does not occur in the first few minutes after inhalation, the situation usually improves but the implications remain serious. In this case the peanut, acting as a ball valve in the right main bronchus, let air into that side, but as the bronchus narrowed during expiration air was trapped in the lung.
Congenital and acquired subglottic stenosis

The reported incidence of subglottic stenosis has increased, partly because of the improved survival of premature babies who have been treated by prolonged intubation and partly because of more accurate diagnosis. Severe cases require tracheotomy and later repair by rib graft laryngotracheoplasty or even cricotracheal resection.

Vocal cord paralysis

Unilateral paralysis causes few symptoms in infants and children. Bilateral vocal cord paralysis is the cause of stridor in about 10% of infants with airway obstruction and is associated with a central nervous system anomaly (e.g. Arnold–Chiari malformation) in many cases. Tracheotomy is usually, but not always, required for bilateral paralysis.

Other causes of stridor

Laryngeal web, laryngeal atresia and laryngeal cleft are uncommon anomalies. Cysts causing clinical features include retention cysts, congenital cysts and cystic hygroma. Subglottic haemangioma is the commonest laryngeal tumour in infants and presents with inspiratory stridor in the first 6–8 weeks. The clinical features of tracheal obstruction are caused by tracheomalacia, tracheal compression by a vascular ring or other anomalies or congenital tracheal stenosis.

Investigations include X-rays, CT, contrast oesophagogram and ultrasound of the neck or mediastinum. Flexible laryngoscopy and direct laryngoscopy, bronchoscopy and possibly oesophagoscopy under general anaesthesia ultimately establish a firm diagnosis.

Acute inflammatory airway obstruction

Acute infectious diseases of the upper respiratory tract that cause airway obstruction fall into two groups:

• Oropharynx. Acute bacterial or viral infection with obstructive hypertrophy of the tonsils and adenoids; infectious mononucleosis causing obstructive enlarge­ment of the tonsils and adenoids; peritonsillar abscess; retropharyngeal or para­pharyngeal abscess and Ludwig’s angina

• Larynx and trachea. Acute laryngotracheobronchitis or croup; spasmodic croup; bacterial tra­cheitis; acute supraglottitis or epiglottitis (with a frighteningly rapid onset) and diphtheria. Some cases (especially patients with acute supraglottitis, epiglottitis or diphtheria) have critical, life-threatening airway obstruction, a situation that requires immediate recognition and transfer to a paediatric hospital for relief of airway obstruction, if necessary by endotracheal intubation or tracheotomy, and intensive care management.

Important advances in treatment of these diseases include more effective antibiotics, diphtheria immunization, diphtheria antitoxin, the use of racemic adrenaline (epinephrine) for croup, steroids for croup, Hib vaccine, which has dramatically reduced the incidence of H. influenzae supraglottitis and epiglottitis, and the use of intubation in place of tracheotomy.

Multiple respiratory papillomas

Papillomas are the most common benign growths in the larynx. Human papillomavirus (HPV) type 6, type 11 and occasionally type 16 cause papillomas in the respiratory tract, most often in the mucosa of the larynx. About two-thirds of patients are younger than 15 years and one-third older than 15 years, with the highest incidence before the age of 5 years. There is a tendency for recurrence after removal, although sometimes unexpected spontaneous improvement can occur. In infants, large obstructing masses may threaten life. There is a strong association between recurrent respiratory papillomas in infants and children and maternal condylomata acuminata or genital warts but transmission of HPV during passage through the birth canal is unlikely, as some infants have papillomas already in their larynx at birth.

Growth may be slow and persistent or irregular and unpredictable. The commonest presentation is a change in the cry or voice, sometimes with increasing airway obstruction, and often an erroneous diagnosis of asthma, laryngitis, bronchitis or croup has been made. Therefore, persistent or progressive huskiness in an infant or child should suggest the possibility of papillomas. The mainstay of treatment is repeated removal at microlaryngeal surgery under general anaesthesia, using forceps or the carbon dioxide laser, which is a precise modality attended by minimal bleeding, causing little pain and limited local scarring. Many adjunctive treatments have been tried because of frustration and recurrence of the tumour but none of these have proved beneficial over the long term. There is no tendency for regression or disappearance at puberty, as was formerly thought.

Ingested and inhaled foreign bodies

Foreign bodies in the pharynx and oesophagus

Children often swallow foreign bodies. Sharp objects such as fishbones can impact in the tonsils, base of the tongue or the pyriform fossa. More often objects such as coins, buttons, lumps of meat or vegetable, plastic or pins lodge somewhere in the oesophagus, usually at the upper end but occasionally at a site of pathological narrowing (stenosis). Some show on X-ray but, ultimately, if an impacted foreign body is suspected, oesophagoscopy is necessary. Remember most small smooth objects will pass through the oesophagus and the gastrointestinal tract and be recovered in the stools; however, ingestion of a small alkaline button battery is extremely destructive of surrounding tissue and must be treated as an acute emergency to prevent perforation of the oesophagus and mediastinitis.

Simon, aged 2 years and 6 months, ran to his mother in great distress, gasping for breath, coughing and crying. She rushed him to the nearby emergency department. The respiratory distress had lessened but pulse oximetry showed only 90% saturation. There was a wheeze and decreased air entry on the right side. An expiratory chest X-ray showed air trapping on the same side with shift of the mediastinum to the left. Simon was observed in the high-dependency ward until an impacted peanut was removed from the right main bronchus under general anaesthesia 90 minutes later. He made an uneventful recovery thereafter.

With inhalation of foreign bodies into major airways, if death does not occur in the first few minutes after inhalation, the situation usually improves but the implications remain serious. In this case the peanut, acting as a ball valve in the right main bronchus, let air into that side, but as the bronchus narrowed during expiration air was trapped in the lung.
Foreign bodies in the larynx and tracheobronchial tree

The highest incidence of inhaled foreign bodies is in the second and third years of life and about 60% of deaths occur in children less than 4 years of age. Occasionally they lodge in the larynx or subglottic region but more often in one or other main bronchus. Diagnosis is made by awareness of the possibility and from a history of inhalation of a foreign body (a history of possible inhalation is present in about 65% of cases).

The clinical presentation may be:

• immediate, with sudden coughing, choking, gasping, spasm and cyanosis. Fortunately, few deaths occur in this stage

• delayed, with wheeze, chronic cough, atypical pneumonia, and chest X-ray changes (but about 20% show no abnormality). The foreign body may be found days, weeks or even months later

• symptomless. Although most foreign bodies will ultimately cause symptoms, occasionally some are found by chance on a chest X-ray or at endoscopy.

Only 5–10% of ingested foreign bodies impact in the larynx or subglottic region and then stridor, laryngospasm, dyspnoea, a husky voice, inspiratory wheeze or repeated atypical croup dominate the clinical picture. If death does not occur in the first few minutes after the foreign body is inhaled, the prognosis is good if the patient is promptly transported by road or air ambulance to a major paediatric unit where experienced personnel and adequate instruments are available. Ill-advised attempts at bronchoscopic diagnosis or removal by inexperienced surgeons or anaesthetists often worsen the situation.

Two-thirds of inhaled foreign bodies are nuts. Parents should be made aware that children under 4 years of age should be denied access to nuts, especially peanuts, in the hope that aspiration accidents will be minimized.

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