Neck stiffness

Neck stiffness (‘meningism’) in an acutely unwell child is a sign of meningitis or, rarely, a subarachnoid haemorrhage. Meningism may occur with other infections such as tonsillitis. Neck stiffness may also indicate a subarachnoid tumour in the posterior cranial fossa, or an orthopaedic problem. It should be possible to flex their chin onto their chest, or to touch their knees with their nose.

In toddlers, getting them to look up or down following a toy is helpful. When formal examination is possible lay the child flat. Cradle the head in both your hands and gently flex the neck. It is said that having the head over the edge of the bed while you support it in your hands is helpful but few children trust you that much!

Movement and coordination

Children with Duchenne’s muscular dystrophy turn themselves prone, get up on to all fours then, climbing up their legs, rise to a standing position – Gower’s sign.

Limb examination

The key to this group of examinations is communicating to children what you want them to do. Use age-appropriate language and description. Demonstration helps.

Cranial nerve examination

III, IV and VI – Occulomotor, trochlear and abducens nerves

• Place one hand gently on the child’s head and ask the child to follow your finger through a range of movements. Look for conjugate eye movements and any nystagmus. Does the child have a squint (Figure 14.1)?

Figure 14.1 Testing the ocular muscles.

VIII – Auditory or vestibulocochlear nerve

• How well do the parents think their child can hear?

• Can the child hear your wrist watch, or fingers gently rubbing? Formal hearing tests require special equipment and expertise

• A tuning fork may help diagnose conductive hearing loss (Rinne and Weber’s test, Figure 14.2), but an age-appropriate hearing assessment and tympanometry (to look for normal movement of the tympanic membrane) is essential if there is any significant concen regarding hearing.

Figure 14.2 Rinne and Weber’s tests for hearing loss. (a) Weber’s test. (b) Rinne’s test positive: air conduction louder than bone conduction (normal).

Neurological investigations

All the following investigations may be alarming for children who, in any case, find it difficult to lie still. In babies and toddlers, sedation or anaesthetic may be needed. Careful preparation with parents and a play therapist is helpful.

Tension headache

Case 14.1 gives an example of tension headache which is the commonest cause of headache in children. The key features are:

The headache appears to arise secondarily to muscle tension in the neck, face and scalp which, in turn, relates to stress, anxiety and worry. The exact mechanism is unclear. Dental problems or short sight may contribute.

Teasing or bullying in school and family stress at home are common causes but children of an anxious disposition may suffer tension headache simply as a result of the stresses and strains of everyday life. With reassurance, explanation of the cause of the pain, and relief where possible of aggravating factors, spontaneous improvement is the norm.

Migraine

Migraine has been described in children as young as 2 years. The most widely accepted hypothesis is the trigemino-vascular theory. Migraineurs are hypothesized to have a hyperexcitable cerebral cortex. Focal neurological excitation is followed by cortical depression, with reflex changes in cortical blood flow; oligaemia is followed by reactive hyperaemia. Serotonin (5-HT) is a key neurotransmitter implicated in migraine pathogenesis. The migraine preventers, pizotifen and propranolol, block 5-HT₂ receptors, whereas triptans (e.g. sumatriptan), used in acute migraine treatment, are 5-HT₁ receptor agonists. The key features are:

The acute attack is best managed with rest and analgesia. Analgesia is most effective if taken very quickly after symptom onset. The earlier treatment is given, the more effective it is. In those with recurrent episodes, a food diary is helpful in identifying dietary triggers. A trigger is implicated in at least 20% of cases of chronic migraine. Triggers include prostaglandin E and the vasoactive amines, tyramine and phenylethylamine, found in chocolate, cheese and red wine. Caffeine and nitrates/nitrites (found in processed meats) are important triggers.

Medication, particularly the oral contraceptive pill, may also be a migraine trigger.

If attacks are frequent – more than once or twice a month – a prophylactic such as propranolol or pizotifen will usually reduce their frequency. Propranolol must not be used if there is a history of asthma. Treatment should be combined with avoidance of known triggers, and a good sleep routine.

In the acute situation, prompt use of simple analgesia (e.g. ibuprofen, paracetamol) at symptom onset is often effective. In those with severe symptoms, refractory to simple analgesia, triptans are appropriate.

In status migrainosus, in which severe migraine persists for more than 72 hours, treatment consists of rehydration and anti-emetics (e.g. parenteral metoclopramide). Triptans may be used in refractory cases.

Raised intracranial pressure

Once the skull sutures have fused at about 1 year of age, any obstruction to the flow of cerebrospinal fluid (CSF) will produce increased intracranial pressure (ICP) and consequent dilatation of the cerebral ventricles. The key features are:

In children over 1 year of age, the commonest cause is a tumour (as in Case 14.2). Brain tumours are collectively the second commonest malignancy in childhood, although still rare (see Chapter 7, pp. 72–74). Other intracranial space-occupying lesions may cause raised pressure, including head injury resulting in subdural bleeding or effusion, particularly in infants and young children, when non-accidental injury must be considered (see also Chapter 5). Infectious causes include postmeningitic hydrocephalus and brain abscess. Cerebral oedema may also cause acutely raised pressure, typically after severe head injury or encephalitis. These conditions frequently present with altered consciousness rather than headache. An example of raised ICP secondary to a medulloblastoma is shown in Figure 14.3.

Figure 14.3 MRI scan showing medulloblastoma. A scan of a 6-year-old child presenting with a 6-week history of headache and vomiting. There is a large tumour in the posterior fossa. Medulloblastoma was the most likely diagnosis (subsequently confirmed at surgery). The ventricular system is starting to enlarge as the intracranial pressure rises.

Untreated raised ICP leads to loss of vision due to retinal damage and eventually to unconsciousness, and finally to unstable vital signs as the mid-brain is pushed through the foramen magnum (coning). Children with headache and abnormal neurological signs require urgent intracranial imaging and, if necessary, neurosurgical referral.

In infants with open sutures, obstruction to CSF flow will cause rapid head growth. This prevents very high ICPs but brain injury may still result if this is left untreated. These children are too young to complain of headache. It is very important to monitor the head circumference, and consult an appropriate centile chart.

Epileptic and non-epileptic seizures

For management of the child with status epilepticus, see Appendix I, p. 291.

In Case 14.4 the diagnosis of day-dreaming was made because the turns were indistinct with a gradual onset and lasted a long time if the boy was not stimulated. The first-hand account is vital and can avoid the need for investigation.

Like Adam, most children presenting with seizures or turns do not have epilepsy. Indeed, children present with a rich variety of seizures and non-epileptic turns and making a diagnosis depends crucially on a careful history of the event.

Epilepsy

Epilepsy is defined by the tendency to suffer recurrent unprovoked seizures. Epilepsy is not one condition but a wide variety of clinical syndromes from the transient and benign to those characterized by intractable seizures with neurological deterioration.

Such a complex condition demands classification to define clinically important syndromes. The broadest classification is between generalized and partial epilepsies. Generalized epilepsies typically produce seizures such as absences, drop attacks and myoclonic jerks which are associated with a generalized disturbance of brainwave activity. Focal epilepsies on the other hand are characterized by an irritable focus in one part of the brain from which abnormal brainwaves may spread to produce secondary generalization. Either pattern of EEG abnormality can produce a tonic-clonic seizure. If the cause of the epilepsy is unknown, it is described as idiopathic, but if the cause can be defined, e.g. scarring from an old head injury, it is said to be a symptomatic epilepsy. Certain patterns of epilepsy have been defined within this basic classification. These epilepsy syndromes define the aetiology, natural history, EEG appearance and therapy for many childhood epilepsies (see Table 14.4).

Table 14.4 Important epilepsy syndromes

Infantile spasms (Case 14.5) are the clinical manifestation of a severe epileptic encephalopathy characterized by a grossly disordered EEG – hypsarrhythmia (Figure 14.4). The syndrome is treated with steroids, unless there is coexisting tuberous sclerosis (see p. 204), in which case vigabatrin is usually more effective. Lack of response indicates a poor neurodevelopmental outcome, as in this case. Infantile spasms may occur secondary to an underlying congenital or metabolic disorder.

Figure 14.4 EEG showing chaotic EEG (‘hypsarrhythmia’).

The boy in Case 14.6 has childhood absence epilepsy – an idiopathic generalized epilepsy. Simple absence seizures develop between 5 and 10 years. The treatment of choice would be sodium valproate, lamotrigine or ethosuximide and the condition often resolves in later childhood.

The girl in Case 14.7 is having focal seizures with temporal lobe automatisms (semi-purposeful, repetitive movements like fumbling with buttons). Her awareness at the onset and subsequent tonic-clonic seizure illustrate the progression of the EEG abnormality across the brain. There is an epileptic EEG focus in the right temporal area and an MRI scan shows right-sided temporal lobe changes (mesial temporal sclerosis affecting the right hippocampus). If these seizures fail to respond promptly to anti-epilepsy medications then a referral for epilepsy surgery should be considered.

Introduction

Neurological disability affects 1% of children. These children can be divided into two groups – those who deteriorate with time and those who do not. Most of these children have an unchanging neurological problem such as cerebral palsy or learning difficulties. It is essential to distinguish those children with progressive disorders. Children generally get more able as time passes. Be alert to any suggestion of children losing skills. Key symptoms are:

Difficulties with movement

Difficulties in control of movement may arise in the brain (upper motor neurone) or peripheral nervous system (lower motor neurone) (Figure 14.6). These can usually be distinguished clinically (Table 14.5). In either case, deteriorating conditions must be detected by careful history taking and investigation if necessary.

Figure 14.5 EEG showing generalized 3 Hz ‘spike and wave’ activity.

Figure 14.6 Classification of motor disability.

Table 14.5 Clinical features of motor disorders

Abnormalities of central motor control – cerebral palsy

The motor control systems within the brain are complex and susceptible to malfunction caused by congenital brain abnormalities or acquired damage to the developing brain. The various patterns of movement that result are described as cerebral palsy. Cerebral palsy (CP) is defined as a persistent, although not unchanging, disorder of movement caused by abnormalities arising early in life affecting the motor control systems in the brain.

Cerebral palsy is classified by the type of motor abnormality observed (spasticity, chorea, athetosis, dystonia, ataxia) and, if relevant, the parts of the body involved, e.g. spastic diplegia (see Table 14.6).

Table 14.6 Classification of cerebral palsy

There are four major types of cerebral palsy as illustrated in Cases 14.8 to 14.11.

Hemiplegia (65% of CP) typically presents between 6 and 10 months. Birth history is usually normal – it is thought the unilateral brain abnormality develops in early pregnancy, perhaps as a result of a vascular occlusion. In Case 14.8, George will learn to walk – even if this is a little delayed. Associated problems, including learning difficulties, seizures and behaviour problems affect about a third of children and may result in complex disability.

Spastic quadriplegia (10% of CP), or whole-body cerebral palsy (Case 14.9), may result from birth events, although severe congenital brain anomalies cause a similar clinical picture. The associated problems of learning difficulties and epilepsy affect most children when the disability is profound. Long-term complications include a ‘windswept posture’, hip dislocation, scoliosis, gastro-oesophageal reflux and recurrent chest infections.

Spastic diplegia (20% of CP) is the classic abnormality after preterm delivery (Case 14.10), although most children with diplegia have an unknown cause. Inherited forms (X-linked and dominant) are recognized.

Choreoathetoid, or dystonic cerebral palsy (as in Case 14.11) (5% of CP), is rare. Neonatal bilirubin encephalopathy is the classic cause of this type of cerebral palsy but many cases are idiopathic and a few are familial. Deafness is a common association. Intelligence is often normal.

Abnormalities of peripheral motor control – the weak and floppy child

When the pathology of a movement disorder lies in the muscles, the peripheral nerves, the motor end plate or the anterior horn cell within the spinal cord, the resultant clinical picture is of weakness and low tone. The key clinical features in these cases are weakness, which is often progressive, and absence of limb reflexes. Many cases are inherited (see Figure 14.7).

Figure 14.7 Schematic illustration of site of peripheral motor abnormalities.

In Case 14.13 the diagnosis of infantile-onset spinal muscular atrophy, also known as Werdnig–Hoffman disease (a recessive condition), was made on DNA analysis. It is incurable and invariably fatal in the first 3 years of life, with the majority dying before their first birthday. Caitlyn quickly deteriorated and died less than a month later of respiratory failure. There may be a 1 in 4 recurrence risk, and genetic counselling is essential. Note that cervical fracture secondary to non-accidental injury may present similar clinical features.

Duchenne muscular dystrophy (Case 14.14) is a slowly and inexorably progressive condition. It is an X-linked recessive condition due to large mutations in the dystrophin gene and so only affects boys. Presentation is typically between 3 and 7 years of age, with lordosis, waddling gait and Gower’s sign. Calf muscle hypertrophy follows within 1–2 years. The majority of boys need a wheelchair by 12 years. About 1 in 3500 boys are affected, worldwide, with 20% of cases arising as de novo mutations. Diagnosis can be quickly obtained by measuring creatine kinase (CK) which is hugely elevated. Genetic confirmation and counselling is necessary for carrier females. Twenty per cent of boys have associated learning difficulties. Careful physical therapy and support is needed to prevent postural deformity and maintain function. Oral steroids have been shown to significantly slow the progression of muscle weakness, and maintain ambulation, on average by an additional 2–3 years.

Trials of novel gene therapies are in the very early stages.

Cardiac surveillance with annual echocardiography is needed, as progressive cardiomyopathy develops in the teenage years. ACE (angiotensin-converting enzyme) inhibitors are usually effective in relieving symptoms. Life expectancy is markedly shortened, with death usually in the third decade from cardiac or respiratory failure.

While some neuromuscular conditions can now be diagnosed by DNA analysis a muscle biopsy may still be necessary for accurate diagnosis. This procedure demands highly specialized pathological analysis and should be carried out in a specialist centre.

Myasthenia gravis (Case 14.15) is rare. Think of it when weakness is variable, with deterioration during the day, especially with intermittent diplopia and difficulty chewing or swallowing. It is often associated with other autoimmune diseases.

Acetylycholine receptor antibodies are present in 80–90%. Seronegative patients often have antibodies to muscle-specific kinase. Those with striated muscle antibodies are more likely to have an associated thymoma.

The mainstays of treatment are acetylcholinesterase inhibitors (most commonly pyridostigmine) and immunomodulatory therapy (prednisolone – usually combined with steroid sparing agents such as azathioprine or cyclosporin; intravenous immunoglobulin, anti-CD20 monoclonal antibody, plasmapheresis, thymectomy).

Infants born to affected mothers are at increased risk of postural deformities (arthrogryposis multiplex) and congenital myasthenia gravis.

Neurocutaneous syndromes

There are a number of neurological conditions associated with cutaneous manifestations. Two disorders account for the majority of presentations: neurofibromatosis and tuberous sclerosis. These conditions are dominant but show variable penetrance. The subtle diagnosis in a parent may not be made until a more severe clinical picture is diagnosed in the child.

In tuberous sclerosis, hamartomas occur throughout the brain and also commonly occur in the heart and kidney. Epilepsy and variable mental retardation may result. Infantile spasms (see p. 194) carry an especially poor neurodevelopmental prognosis. Cutaneous signs include small oval depigmented patches, ash-leaf macules, which fluoresce under ultraviolet light, hard leathery Shagreen patches in the lumbosacral region, and acne-like lesions, adenoma sebaceum on the face.

Two types of neurofibromatosis are recognized. Both are inherited in an autosomal dominant fashion.

Type 1 neurofibromatosis (NF1) results from mutations in the NF1 gene on chromosome 17 (>95%). The gene product, neurofibromin, is a tumour suppressor. Reduced levels of neurofibromin predispose to myriad different features. Mutations in SPRED1 also give rise to a phenotype of neurofibromatosis with café-au-lait patches and axillary or inguinal freckling, but other features do not develop. Incidence is estimated at about 1 in 3000 individuals, but milder cases are not recognized, and this is undoubtedly an underestimate.

NF1 is characterized by progressive development of café-au-lait patches (greater than 5 mm in children under 10 years, or >15 mm in children over 10 years, satisfies one of the major criteria for clinical diagnosis), axillary or inguinal freckling and cutaneous neurofibromas. Characteristically, children have relative macrocephaly. Learning difficulties (40%), hyperactivity and seizures may occur. There is remarkable phenotypic heterogeneity, even in the same family. Children with this condition are prone to tumours of the nervous system and other organs.

Neurofibromas are less common in childhood, often appearing at adolescence. Plexiform neurofibromas are problematic, and may grow to huge proportions. If complete resection is feasible, this is the preferred option, but radical debulking is an alternative. Malignant peripheral nerve sheath tumours and neurosarcomas may also arise in teenagers. These are often very difficult to treat, but newer agents such as sorafenib which target the Ras pathway have shown promising results.

Children require annual opthalmological review in early childhood, as optic gliomas may affect vision, and are most likely to develop before age 6–7 years.

Annual blood pressure screening is also recommended, as hypertension may result from renal artery stenosis or phaeochromocytoma.

Orthopaedic abnormalities are common, including scoliosis, pseudo-arthroses and pathological fractures. Scoliosis is usually mild, but there is a subset of children with early-onset scoliosis in whom aggressive treatment is necessary.

Type 2 neurofibromatosis (NF2) is less common (about 1 in 35 000–40 000) and cutaneous manifestations such as café-au-lait patches and neurofibromas are unusual. Commonly, the diagnosis is not made until late teenage years or adulthood. The principal manifestation is with bilateral acoustic neuromas, and other brain and spinal cord tumours. Acoustic neuromas typically present with tinnitus, vestibular symptoms and hearing loss. In contrast to NF1, cataracts often occur. It arises from mutations in NF2 on chromosome 22. The gene product, merlin, is a tumour suppressor. Half of all cases arise de novo.

Sturge–Weber syndrome describes the association of a facial angioma (‘port-wine stain’) in the ophthalmic division of the trigeminal nerve with an ipsilateral leptomeningeal angioma, most commonly parietal or occipital, resulting in epilepsy (70–90%) and sometimes stroke-like episodes or permanent hemiplegia. Low-dose aspirin may reduce the frequency of stroke-like episodes. Congenital glaucoma (buphthalmos) affects over 30%.

An isolated port-wine stain without an underlying intracranial vascular malformation or glaucoma is not uncommon, particularly if the opthalmic division of the trigeminal nerve is spared. Mental retardation affects 50–75%. Although the lesions are static, the neurological sequelae may be progressive, with cerebral atrophy on the affected side. Effective seizure control improves outcome. Epilepsy surgery may be indicated.

In older children, migraine-type headaches are commonly reported (40–50%), and may be severe. Scoliosis and body asymmetry are commonly seen in older children.

Laser therapy may be used for port-wine stains, and is more effective when started at an early age (ideally in infancy). Skin camouflage is very effective in disguising lesions and may help self-confidence and self-esteem. The British Red Cross offer a free skin camouflage service.

Bell’s palsy

Children present with an acute unilateral lower motor neurone facial weakness – the onset is rapid, often over a few hours or overnight. The hallmark is unilateral lower motor neurone facial paralysis, affecting the entire side of the face. The involvement of the forehead distinguishes Bell’s palsy from upper motor neurone facial palsy, in which the forehead (frontalis) and eye (orbicularis oculi) are spared. The right side is affected in over 60% of cases. It is uncommon in children (10–15 per 100 000 per year). It may cause weeping of the eye due to lack of blinking, or dribbling due to failed lip closure. It is believed to arise from nerve injury arising from compression secondary to inflammation and oedema, as the nerve traverses the facial canal in the petrous temporal bone. There is growing evidence for an infectious aetiology, with herpes simplex and varicella most commonly implicated.

In the first instance, careful examination is required to exclude other neurological features. Swelling of the parotid may indicate inflammation or tumour affecting the facial nerve. The presence of contralateral limb weakness or diplopia may suggest a stroke or other central cause. Diabetes and hypertension are recognized associations. A history of tick bite may suggest Lyme disease, particularly if the facial palsy is bilateral.

Treatment with steroids (prednisolone 1 mg/kg, maximum 60 mg for 6 days) to reduce postulated facial nerve swelling is most effective if started within 3 days of onset. Evidence from randomized trials in adults suggests that use of antivirals (e.g. aciclovir) either alone, or in combination with steroids, is no better than placebo treatment in improving outcome. There is some evidence that specific facial exercises may be beneficial. It is important to protect the cornea by eye-patching at night and with the use of artificial tears. Eighty to ninety per cent make a spontaneous complete recovery within 6 weeks to 3 months. Patients not showing some recovery by 2 weeks should be referred for specialist evaluation.

Guillain–Barré syndrome

Guillain–Barré syndrome is an acute demyelinating polyradiculoneuropathy. In its classic form, it presents as an ascending flaccid paralysis of arms and legs with areflexia. However, a subset of patients (~30%) have increased reflexes. Pain and dysaesthesia are commonly reported, but objective sensory loss does not occur. Autonomic neuropathy may develop, with resultant tachycardia, and less commonly tachyarrhythmias. Urinary retention affects 10–15%. The majority of cases of Guillain–Barré syndrome are in boys (60–70%).

Since the demise of polio, Guillain–Barré syndrome is the commonest form of acute motor paralysis in children. It follows a respiratory or gastrointestinal infection in two-thirds. Mycoplasma pneumoniae and Campylobacter jejuni are most consistently associated with Guillain–Barré syndrome. Serology for C. jejuni is positive in 50% of paediatric cases. Immunization may be a trigger of Guillain–Barré syndrome, and has been reported with a range of vaccines, notably certain types of influenza vaccine used in Europe.

Guillain–Barré syndrome is believed to arise from aberrant T-cell targeting of neural antigens. C. jejuni is particularly strongly associated with the Miller–Fischer variant of Guillain–Barré syndrome in which cranial nerve palsies and ataxia also feature, and antibodies to GQ1b ganglioside are typically found.

Inflammation of spinal roots leads to disruption of the blood–brain barrier, with a resultant increase in CSF protein, used as a diagnostic test. CSF protein may be normal at onset, but is elevated in the great majority by 10 days into the illness. Lumbosacral MRI with gadolinium enhancement shows abnormal enhancement of the nerve roots. Electromyographic studies are similarly best left to the second week of illness, when they will be diagnostic. Antibody tests may be helpful in Guillain–Barré syndrome variants when diagnosis is more problematic.

Treatment consists of supportive measures, including ventilation if respiratory failure supervenes. Thromboprophylaxis is advisable for non-ambulant children. Children more commonly develop respiratory failure than adults, but respond better to immunomodulatory therapy with immunoglobulin infusion being the mainstay of treatment. Plasmapheresis may be used in refractory cases. Full recovery is the norm, but it is a lengthy process, taking 6–12 months.

Spinal cord tumours

Spinal cord tumours are mercifully rare, but difficult to diagnose. They arise from either the spinal cord itself (medullary tumours) or its sheath, the dura mater. Progressive sensory and motor dysfunction arises from spinal cord compression. Precise symptoms depend on the location of the tumour. Pain is a prominent feature due to venous engorgement in the region of the tumour. Medullary neoplasms (most commonly, astrocytomas, ependymomas or haemangioblastomas) account for 10% of paediatric CNS tumours. Lumbar puncture may be associated with severe deterioration, and should only be undertaken under expert guidance for specific diagnostic purposes. Surgery is necessary for diagnosis and treatment. Radiotherapy may be useful for metastatic or inoperable tumours.

Causes of permanent hearing loss

The baby

Universal screening of all newborn babies in the UK was implemented in March 2006. The screen uses oto-acoustic emission screening, with brain-stem audiometry used to confirm permanent sensorineural hearing impairment (PSNI). Treatment in the first instance is with hearing aiding, but cochlear implants are necessary if hearing loss is severe. Previous targeted screening missed 50% of cases, with resultant poor outcomes. Groups at high risk of PSNI include premature infants, particularly those who were given aminoglycoside antibiotics, infants with craniofacial abnormalities, and babies born to a family with a family history of hearing impairment.

The infant

Hearing loss in this age group is usually identified by the parent. The child may not turn to sounds, or, at an older age, respond to their name being called. Their language development is delayed. Since the advent of universal hearing screening, the health visitor distraction test (7–9 months of age) is no longer performed.

The child at school

Poor school performance or behaviour may indicate a problem. Speech delay is another common presentation. Screening at school entry picks up some cases. See Table 14.7 for assessment and diagnosis of hearing loss.

Table 14.7 Hearing loss and testing

Chronic secretory otitis media (glue ear)

Glue ear is the commonest cause of a conductive hearing loss in childhood. The child’s Eustachian tube is less vertical than in an adult, so it drains less readily. This causes a middle-ear effusion. If chronic, this fluid becomes thick and tenacious like glue.

For many, glue ear is a self-limiting condition that requires no management, other than explanation and reassurance to the family. Steroid nasal sprays may be beneficial in improving symptoms. In children with a persistent hearing, ventilation tubes (‘grommets’) may be appropriate. Grommets are small tubes sited in the tympanic membrane. They equalize the pressure between the middle and outer ear. This allows aeration of the middle ear, and resolution of the effusion with resultant improvement in hearing. Adenoidectomy is often performed at the same time as the insertion of grommets. Adenoidal hypertrophy is common. Large adenoids narrow the posterior nasal space. This may affect the functioning of the Eustachian tube.

The grommets remain in situ until they fall out as the tympanic membrane grows. Children are able to go swimming with grommets, but should be discouraged from submerging their heads or diving. Ear plugs are useful for baths or showers, to prevent ingress of dirty or soapy water.

Deafness (as in Case 14.16) is classified according to the level of impairment (Table 14.8). The impact of this loss on a child is not always easily determined.

Table 14.8 Classification of deafness

Supporting deaf children

The goal of supporting the deaf child is to ensure they are functionally integrated into their family, school and the wider world. This requires an approach tailored to the individual needs of the child and family.

Hearing aids

Hearing aids help many deaf children. However, some children find them intrusive and are reluctant to wear them. Cochlear implants are increasingly used for severe PSNI.

School-age children struggle within the noisy environment of a classroom and special provision may be necessary. Specialist teachers working within a sensory support service are invaluable. Speech and language therapy input to assist communication is vital. Learning to sign is appropriate for some.

Refractive errors

Squint

Squints are common. They can be divided into paralytic and non-paralytic types.

Progressive loss of vision

Some children are born with normal visual acuity and lose it over time. The most devastating loss is a sudden one. Causes include trauma and retinoblastoma. Gradual losses can be accommodated gradually. The impact depends on the nature of loss – whether it is central or peripheral. Causes include the retinal dystrophies and Batten’s disease, which is also associated with progressive intellectual and neurological deterioration and fatal outcome.

The white eye

The red eye

Neonatal eye infection

In the neonatal period, consideration needs to be given to the possibility of herpes simplex, Gonococcus and Chlamydia as causative agents. Gonococcus in particular may cause rapid destruction of the eye and requires combined topical and parenteral antibiotics. It is a notifiable condition (ophthalmia neonatorum). ‘Sticky eyes’ are common in the neonatal period. If sticky eye persists for 3–4 days after birth, empirical treatment with topical antibiotics such as chloramphenicol may be indicated. Chlamydia swabs should be taken in an appropriate transport medium. Persistent sticky eyes may be due to inadequately treated infection, or more commonly, occlusion of the nasolacrimal duct. Treatment involves nasolacrimal massage and wiping away any debris. If necessary, the duct may be probed surgically under anaesthesia.

Glaucoma

Glaucoma in children is most commonly congenital. It typically presents in early childhood with the classic triad of lacrimation, photophobia and blepharospasm, all secondary to corneal irritation. The eye may enlarge – buphthalmos (bull’s eye). Treatment is surgical.

Orbital cellulitis

In periorbital cellulitis, there is erythema and tenderness around the eye, face and eyelid. It is imperative to exclude orbital cellulitis with an appropriate examination of the eye and eye movements. This may require ophthalmological advice. The cause is bacterial and antibiotics are necessary.

Orbital cellulitis presents with a painful eye, sometimes proptosis, and decreased eye movements. These children are systemically unwell. They may experience a visual loss. Assessment with a CT scan of the orbit and prompt intravenous antibiotics are essential.

Iritis

Inflammation of the anterior eye may accompany a range of connective tissue diseases, most commonly juvenile idiopathic arthritis (see Chapter 6, p. 55). Presentation may be acute with a painful red eye, photophobia and lacrimation, or chronic with insidious loss of vision. The pupil may appear irregular, and the anterior chamber may appear cloudy or contain debris on slit-lamp examination. Complications include cataract and glaucoma. Treatment is with steroid eye drops with or without antiviral or antibiotic treatment.