Conjunctivitis and chalazion

• Neonatal conjunctivitis may be sight-threatening and a threat to the newborn infant’s health. Immediate investigation with appropriate treatment is needed

• If a child does not have itch as a major symptom then allergic conjunctivitis is unlikely. Think of other causes of conjunctivitis in this situation

• Most chalazia are not infected and redness and swelling is the result of sterile inflammation. Consequently topical and oral antibiotics are of little use in treatment. Most chalazia resolve spontaneously

Ptosis, also called blepharoptosis, is a droopy upper eyelid and results from innervational or muscular defects of the levator superioris or Muller muscles. Innervational defects include third cranial nerve palsy, Horner syndrome (sympathetic nervous system) and myasthenia gravis. Congenital ptosis is the commonest muscle defect causing ptosis in children. Ptosis will cause visual defects when the lid is so low that it occludes the visual axis or if it induces astigmatism by altering the corneal curvature. Ptosis is also a cosmetic concern in that it may make an affected child look sleepy or dull. Surgical correction is possible in most cases.

Learning difficulties

Learning difficulties are common in school-age children and are the result of a neurobiological disorder, i.e. brain dysfunction. The majority of reading difficulties are the result of defects in phonological awareness. It is commonly assumed that there may be a visual abnormality that contributes to or even causes the learning difficulty. This assumption is ill founded and arises because vision is so obviously involved with activities such as reading and writing. Children with learning difficulties are no more or less likely to have visual problems than children without evidence of learning problems. Rather than expending effort on therapies for perceived ocular abnormalities, parents should be encouraged to take an educational approach to their child’s learning difficulties.

Visual handicap

Visual handicap in childhood may be the result of ocular and/or cortical visual abnormalities and may be associated with other abnormalities, e.g. deafness, motor defects and intellectual defects. Intervention and support for a particular child needs to be planned after a thorough assessment of the child’s visual and associated handicaps. From a purely visual point of view, interventions may include mobility training, low vision aids, such as magnifiers and closed circuit television, and training in alternative means of communication, such as ‘reading’ braille and using a computer to write.

The presence of additional handicap such as deafness or an intellectual deficit compounds the situation and necessitates skilled intervention over many years to achieve optimal outcomes.

Rare but important eye problems in childhood

These are mentioned briefly because prompt recogni­tion enables early treatment and optimal outcomes.

Poor vision in infancy

This first comes to attention when a child fails to achieve normal milestones of visual development (see Measurement of vision in children, above). If the cause of severe visual impairment is within the eye, sensory nystagmus will develop at about 3–4 months of age. This nystagmus is often slow and somewhat pendular rather than jerky in appearance. Severe visual loss secondary to CNS abnormality does not cause nystagmus. Causes of poor vision in infancy include:

• cataracts

• albinism

• retinal colobomas

• infantile glaucoma

• congenital retinal dystrophy

• retinoblastoma

• delayed visual maturation

• cortical visual impairment.

Prompt recognition is vital as there may be a treatable cause (e.g. cataracts) and, even if no treatment is possible, early and appropriate intervention minimizes the negative effects of severe visual impairment on general development.

Cataract

A cataract is any opacity within the lens. Bilateral congenital cataracts will often cause poor vision in infancy, while unilateral congenital cataract may go unrecognized, as one eye has normal vision. Both bilateral and unilateral congenital cataracts are treatable if diagnosed early. Cataracts are detected readily by inspection of the red reflex with the direct ophthalmoscope.

There are numerous causes of congenital cataracts, including: hereditary (dominant, recessive and X linked); metabolic (e.g. galactosaemia); association with systemic syndromes (e.g. Down syndrome), and congenital infection (e.g. rubella embryopathy). Many, especially unilateral cataracts, are idiopathic.

Retinoblastoma

This is a rare childhood cancer arising within the retina. Sporadic and hereditary forms are recognized. The sporadic form is the result of two separate mutations that negate the action of the retinoblastoma (Rb) gene within a single retinoblast cell, and thus is always unilateral. The hereditary form arises when the first of these two mutations occurs in one Rb gene within a germ cell (most often a sperm). The second mutation occurs within the retinoblast. As all retinoblasts descended from an affected germ cell have the first mutation, by chance more than one retinoblastoma will usually develop and hence the hereditary form is often, but not always, bilateral.

Retinoblastoma most often presents with leukocoria (white pupillary reflection: the white tumour is seen immediately behind the lens), strabismus, poor vision, or a known family history of retinoblastoma. Prompt recognition is vital as early treatment will increase the possibility of preserving vision and life. With current treatments the 5-year survival of this childhood cancer is about 98%.

Glaucoma

Glaucoma in infancy presents with a cloudy and enlarged cornea with associated epiphora (watery eye) and photophobia. It may be unilateral or bila­teral and is usually an isolated ocular abnormality. If unrecognized it will result in severe and untreatable visual loss over weeks to months. Prompt diagnosis allows surgical treatment, which controls the glaucoma in the majority of cases.

Colobomas

These defects result from failure of complete fusion of the embryonic fissure of the developing eye between the fourth and sixth week of gestation. If the optic nerve or macular area of the retina is involved then vision will be significantly affected. An iris coloboma may or may not be present in association with a visually more important posterior pole colobomas. Colobomas are not treatable.

• Learning difficulties are seldom the result of simple eye problems

• Examine the red reflexes of all infants suspected of having poor vision and all infants with strabismus. An abnormal red reflex in this situation may be due to retinoblastoma and urgent referral is mandatory

• Think glaucoma if a child has one eye that bigger than the other. Then look for co-existing clouding of the cornea and seek history of watery eye and photophobia

The following is a brief account of the common ocular features of some paediatric systemic diseases.

Extreme prematurity

Marked prematurity gives rise to eye problems by interfering with the orderly development of retinal blood vessels. This disorder is known as retinopathy of prematurity (ROP). Mild ROP is seen in 30–50% of infants weighing less than 1250  g at birth and then regresses without ill effect on vision. In some infants the ROP progresses and a fibrovascular proliferation develops within the eye that detaches the retina, with resultant loss of vision.

Excess oxygen administration to premature infants has been known to be a potent cause of severe ROP since the 1950s. Curtailment of oxygen use to amounts sufficient to limit respiratory and neurological sequelae has greatly reduced the incidence of blinding ROP but has not completely prevented it. In general it is the sicker and smaller infants that are still at risk of severe ROP.

Screening of at-risk infants (birth weight 1250  g) by an ophthalmologist enables detection of significant ROP before retinal detachment occurs. Retinal ablation with laser will then greatly reduce the risk of the development of retinal detachment.

Juvenile chronic arthritis

Childhood chronic arthritis gives rise to inflammation of the iris (iritis or anterior uveitis) in some affected children. Those at particular risk are young girls with oligoarticular juvenile chronic arthritis who are antinuclear antibody positive, although it also occurs in other presentations of juvenile arthritis also. The iritis that occurs in these children is painless and chronic and will, if untreated, often cause cataract and glaucoma. Periodic assessment by an ophthalmologist will detect early iritis and permit treatment to minimize the risk of visual loss.

Down syndrome

Down syndrome is associated with an approximately tenfold increase in the risk of developing eye problems during childhood when compared with the normal incidence. The eye problems are the same as for any child. An increased index of suspicion for eye problems should be maintained for individuals with Down syndrome.

Physical child abuse

Non-accidental injury may involve the eye. Direct trauma to the eye or eyelids will generally be obvious on inspection. Violent shaking of a small child is often associated with the development of retinal haemorrhages and a severe closed brain injury. Although not pathognomonic for child abuse, the presence of retinal haemorrhage is highly suggestive of abuse in cases of unexplained severe brain injury in a young child.

Diabetes mellitus

Diabetes mellitus is common in childhood. However, the duration of diabetes in children is often insufficient for there to be much risk of the development of eye complications during childhood itself. Screening for eye complications should begin at about puberty and occur 2-yearly thereafter if the examination is normal. Significant retinal abnormalities are seen in a small number of diabetic children in mid to late adolescence, particularly if the disease was of early onset and control has been poor.

22.3

Disorders of teeth and the oral cavity

N. Kilpatrick

The oral cavity can be considered the gateway to the body. It is the start of the alimentary tract and is integrally involved in the initial phases of digestion. The oral cavity consists of teeth sitting in sockets in the alveolar processes of the maxillary and mandibular bones supported by a fibrous sling known as the periodontal ligament. The oral cavity is lined by a combination of attached gingival tissue (gums) and more generalized mucous membranes. As with the rest of the body, the oral cavity is susceptible to both developmental and acquired disorders that can occur in isolation or as part of more general medical conditions or genetic syndromes.

It is becoming increasingly well recognized that oral health plays a significant role in maintaining good general health and wellbeing. This chapter will, therefore, summarize the key features of normal oral development and highlight the common disorders that affect both the teeth and their supporting structures. It will also identify the oral manifestations of some of the more common paediatric diseases.

Development

Teeth start to form from the 5th week in utero and may continue until the late teens or early 20s with the eruption of the third permanent molars (or wisdom teeth) (Table 22.3.1). The first tooth to erupt is usually the lower central incisor at around 7 months of age. By the age of 2.5 years most children will have a complete primary dentition. consisting of 20 teeth; 8 incisors, 4 canines, 8 molars. At around the age of 6, the primary incisors become mobile and fall out. Most people have 32 permanent teeth, the first of which to erupt is usually the lower first permanent molars at around 6 years of age. The period that follows, referred to as the mixed dentition phase, is highly variable.

Permanent upper incisors are usually more proclined than their predecessors, which allows the mandible to grow forward and encourages the development of what is described as a normal occlusion. This is known as a class I occlusion and while described as ‘normal’ is actually relatively uncommon, and the development of the permanent dentition is frequently neither well organized nor ideal. Variations of the norm are common, particularly in the anteroposterior dimension, which causes changes to the relationship between the upper and lower incisors. Cases where the maxilla is forward relative to the mandible and the upper incisors protrude creating an increase in ‘overjet’ are known as a class II malocclusion. Conversely those cases in which the mandible is relatively prognathic and the upper front teeth develop behind the lower ones the result is a class III malocclusion, or reverse overjet. These malocclusions may result from growth anomalies in either or both jaws and may be further complicated by the pattern of eruption of the dentition, the size of the teeth and other external influences such as thumb sucking. Recognizing malocclusions is not only important in determining the need for and nature of treatment but can also be important in diagnosing growth disorders and in syndrome identification, as jaw discrepancies are common in such conditions.
Practical points

• Eruption times vary widely

• Providing the sequence of eruption of the teeth is in order (central incisors before lateral incisors, etc.) delays per se are not a cause for concern

• Asymmetrical eruption, particularly of the permanent incisors, should be reviewed by a dentist in order to check that there is no obstruction (such as an extra tooth) to the eruption of the appropriate tooth

• The simultaneous presence of primary and permanent teeth during the mixed dentition phase is generally not a problem

• Premature loss of primary teeth can be a sign of underlying systemic disease and should be reviewed by a paediatric dentist

There are only a limited number of developmental anomalies that occur in the newborn or very young child. As it is unusual for infants to be seen by a dental health professional, it is important that the medical practitioner examine the oral cavity periodically and refer to a paediatric dentist as appropriate.

Congenital epulis

• a benign pedunculated soft tissue tumour on the alveolar ridge, present at birth

• composed of sheets of granular cells

• management is by careful surgical excision

• does not recur.

Oral alveolar developmental cysts (Bohn’s nodules)

• multiple 1–5  mm creamy nodules on the outer surface of the alveolar ridges (normally shed in utero)

• composed of epithelial remnants

• sometimes mistaken for prematurely erupting teeth

• similar nodules, found along the palatal midline, are known as Epstein’s pearls

• no treatment indicated as the contents discharge spontaneously by 3rd month.

Palatal odontogenic hamartoma

• unilateral or bilateral 3–5  mm dome like swellings adjacent to the midline of the palate behind the incisive papilla

• contain odontogenic epithelium or a developing tooth (demonstrated on occlusal radiograph)

• appear from 8–12 months of age

• managed by elective surgical excision (can be deferred to 12 months of age).

Eruption cyst

• a blue or clear swelling overlying the crown of an erupting tooth, most frequently in the incisor region of the maxilla

• eruption is slightly delayed and discomfort or pain may occur

• management is symptomatic only. Surgical intervention is contraindicated.

Melanotic neuroectodermal tumour of infancy (MNTI)

• very rare solid benign but locally invasive blue-black pigmented tumour

• cells of neural crest origin (two cell types: neuroblasts and black pigment cells)

• tumour cells associated with each primary tooth in one jaw quadrant

• commences in utero and always presents before 3 months age

• management by careful surgical excision, and removal of associated teeth; does not recur if completely removed

• computed tomography essential to locate all individual tumour deposits.

This tumour should not be confused with an eruption cyst or haematoma. It is the only oral lesion that can appear blue-black in this region at this age.

Natal and neonatal teeth

• present at 1 in 3000 births, or erupt in the neonatal period – usually in the mandibular incisor region

• in infants with cleft lip and palate commonly occur high in the cleft

• most are prematurely erupted normal primary teeth, but some may be ‘supernumerary’ or extra teeth

• can interfere with breast feeding (nipple trauma)

• removal is commonly indicated to alleviate parental anxiety and is simple (using topical local anaesthesia and a haemostat). Parents should be reminded that it is likely that there will be teeth missing in this region until the permanent dentition begins to erupt at around 6 years of age.

Teething

Teething is a normal process by which an infant begins to cut their first teeth (primary dentition). A variety of symptoms can accompany teething, including sensitive and painful gums, mouth ulceration, drooling, feeding difficulties, lack of sleep, fevers, diarrhoea and crying. The scientific evidence that any of these symptoms are directly related to tooth eruption is controversial. Nevertheless, they are com­monly reported and can cause significant distress to the child and anxious parent. Similarly there is no evidence base to support any particular management strategy. The use of chilled teething rings, hard, sugar-free rusk biscuits and finger pressure appears to help. Over-the-counter teething preparations are of limited use. Not only do many contain choline salicylate and significant amounts of ethanol and are contra-indicated in very young infants but also repeated use can cause ulceration of the gums. Some lidocaine (lignocaine)-based gels are thought to be slightly more effective and may be mildly antiseptic. Mild elevations in temperature can be managed with systemic oral medication but temperatures of 38°C and higher or other serious symptoms (e.g. convulsions) should not be ascribed to teething and should be assessed independently.

Developmental anomalies of the teeth

Teeth start forming from the 5th week in utero. Any disturbance in metabolism can cause damage to or even the death of the sensitive enamel-forming cells (the ameloblasts). Such a disturbance will leave a permanent developmental defect on the tooth surface that will appear as a loss of tooth substance (hypoplasia) or a deficiency in the quality of the enamel (hypo­mineralization) once the tooth erupts. By using published tables (or diagrams) of normal tooth development, enamel defects can frequently be related chronologically to:

• prenatal events (usually occurring between the 3rd and 7th month in utero), such as maternal rubella virus or cytomegalovirus (CMV) infection, maternal syphilis and pregnancy toxaemia

• perinatal events: may be prematurity, hypoxia and hyperbilirubinaemia

• postnatal events: measles virus infection, gastrointestinal disease, hypoparathyroidism and administration of tetracycline are some of the over 100 possible aetiological factors capable of inducing developmental defects of tooth enamel.

These defects will be found symmetrically and developmentally chronologically distributed on areas of the tooth crown that were at that particular developmental stage at the time of insult (Fig. 22.3.1). First permanent molars (the so called 6-year-old molars) are particularly susceptible to enamel defects as they are developing at the time of birth. Children with other health issues such as congenital heart disease or cerebral palsy are more likely to have developmental defects of their teeth as a result of systemic illness, fevers, periods of hypoxia, etc., in infancy and early childhood. When isolated defects occur in just one or two permanent teeth they may also be due to infection or trauma of the primary precursor tooth. Early identification of these defects is important, as the quality of the tooth enamel is compromised and the teeth may be sensitive, particularly to oral hygiene measures, and more likely to develop decay.

Miranda, aged 2 years, had been born normally at term with a normal birth weight. Since then she had demonstrated slow developmental milestones and mild hemiplegia with no obvious cause. Dental examination revealed a caries-free primary dentition, which, however, had chronologically distributed enamel hypoplastic (developmental) defects, affecting the teeth at the 4–7 months in utero stage of development. On questioning, her mother could not recall any major abnormal event during pregnancy; however, the grandmother had recorded in her diary the dates when her daughter had a severe viral infection and was in bed for several days (and she had gone to look after her).

Antibodies to CMV were detected on testing. CMV was the presumptive cause of the enamel defects and possibly also of the mild neurological defect and hemiplegia. This diagnosis helped early planning for future assessment and care. These enamel defects can increase the risk of developing dental caries, as the surface of the teeth are often more porous and retain plaque, and they can be quite sensitive. Such teeth can be protected with a tooth-coloured adhesive material and the parents should be encouraged to assist Miranda with her oral hygiene and to maintain regular dental visits.
Acquired disorders of the teeth

Dental caries

Despite a decline in prevalence worldwide, dental caries (decay) remains one of the most common chronic diseases in childhood. As with many diseases there are considerable inequalities in terms of caries experience throughout the population. In Australia just over 60% of 5-year-old children are caries-free but, of those who do have caries, a mean of three teeth per child are affected. Furthermore 10% of the 4-year-old children who do have caries have more than nine affected teeth. In fact, 80% of all decay is experienced by just 20% of children. It is therefore important to identify children at high risk of developing decay and target them for proactive prevention. Given that very few pre-school-age children get taken to a dental health professional, the responsibility lies with medical and nursing health professionals to identify infants at risk of developing decay and to provide appropriate anticipatory preventive advice.

Dental caries (or decay) is an infectious disease caused by the presence of certain bacteria (predominantly mutans streptococci) in the oral cavity. The mutans streptococci (MS) metabolize sugars and starches to produced acids, which lower the pH of the oral cavity and promote loss of minerals from the tooth surface. Minerals in the oral cavity, including fluoride, are redeposited on the tooth surface once the neutral pH is restored (normally after about 20 minutes). This process is dynamic and as long as minerals are replaced the tooth surface remains sound and intact. If, however, the drop in pH is prolonged and/or frequent there will be a net loss of minerals, leading to a weakening and eventual breakdown (cavitation) of the tooth surface. The early sign of mineral loss is characterized by precavitated or ‘white spot’ lesions, usually around the necks of the teeth where the MS tend to accumulate in a biofilm (known as dental plaque) on the teeth. Early identification of these precavitated lesions is important as they signal the need for proactive preventive measures to encourage remineralization. Failure to change the oral environment to one that encourages remineralization will result in cavities. If this occurs, then restorations (fillings) are necessary.

Early childhood caries (ECC; historically also referred to as nursing bottle caries, baby bottle decay and many other terms) is a distinct form of dental caries affecting pre-school-age children. ECC is particularly virulent, causing massive destruction to the primary dentition in children as young as 18 months of age. At birth MS do not inhabit the oral cavity; however, the earlier colonization occurs the greater the risk of ECC. The most common source for transmission of MS has been shown to be the primary care giver, usually the mother. Poor maternal oral health coupled with inappropriate feeding behaviours such as prolonged on-demand feeding, particularly through the night, places an infant at high risk of developing ECC. Medical practitioners, paediatricians and maternal child health nurses are all in a strategically good position to identify individuals at risk of developing ECC (Table 22.3.2).

Prevention of dental caries

Strategies to prevent dental caries should start as soon as the first primary teeth erupt (Table 22.3.3).

Fluoride

Fluoride is the single most effective way to protect teeth from decay. It acts in two ways; it can enhance the ability of teeth to resist demineralization caused by intraoral acids and it can also inhibit oral bacterial enzymes to reduce the conversion of sugars in to acids. However, the latter effect is relatively small in comparison to its biochemical modification of the structure of tooth enamel.

Fluoride can be delivered both systemically and topically. Fluoridation of the water supplies allows for both effects. Water ingested during development of the teeth allows fluoride to be incorporated into the developing dental enamel. However, it is as a topical agent that water has its most beneficial effect, as low-dose fluoride comes in to frequent contact with the teeth before being ingested. As such, water fluoridation is considered a very cost-effective public health intervention. However, many homes in rural and remote areas do not enjoy ‘town water’ and so miss out on the advantages of water fluoridation.

The other common source of fluoride comes in the form of toothpaste. In Australia and New Zealand (and most parts of Europe) there are two common strengths of fluoride toothpaste; most adult toothpastes contain around 1000  ppm (parts per million) fluoride whilst junior toothpastes contain lower concentrations of fluoride, around 400  ppm. The early exposure of primary teeth to fluoridated toothpaste is very effective in preventing caries, as the newly erupted immature tooth surface is highly susceptible to the beneficial maturation effect of fluoride. For most infants a junior toothpaste will be appropriate; however, for those infants at high risk (see Table 23.3.2) exposure to an adult strength toothpaste may be more appropriate. Advice from a paediatric dentist should be sought. The use of additional topical fluoride supplements (tablets or drops) can be of benefit on an individual basis; however, the universal prescription of fluoride supplements is no longer recommended and advice should be sought from an appropriate dental professional.

In addition to encouraging optimal exposure to fluoride, providing advice on healthy dietary practices that reduce the length of time that the oral cavity spends with an acidic demineralizing pH will also reduce the risk of caries. At all ages, trying to reduce both the total amount and frequency of intake of sugary foods and drinks is important. For infants only milk, formula or water should be put in the nursing bottle and on-demand nocturnal feeding should be discouraged. In particular, children put to bed and allowed to sleep with a nursing bottle are likely to develop decay. As children get older, encouraging water drinking and limiting sugary snacks/drinks to meal times when salivary flow is optimal will optimize the buffering capacity of the oral cavity.

Recently new products have been developed that contain casein phosphopeptide–amorphous calcium phosphate (CPP-ACP). These products, either as a chewing gum (Recaldent, Cadbury Japan Limited, Adams Division) or as topical cream (Tooth Mousse, GC Corporation, Itabashi-ku, Tokyo, Japan) act as a reservoir for calcium phosphate, maintaining a state of supersaturation around the tooth with respect to calcium and phosphate thereby depressing the demineralization of tooth tissue and promoting its remineralization. These products can be used in conjunction with fluoride products such as toothpaste as they act synergistically to promote remineralization in the oral cavity. With the exception of being contraindicated in individuals with milk protein allergy, they are safe and effective. These products are currently only available through dental surgery outlets but are being increasingly used, particularly in individuals who continue to develop caries despite optimal fluoride exposure.

Fluorosis

High serum levels of fluoride can produce a developmental abnormality of enamel maturation known as fluorosis. Mild cases usually appear clinically, as a white flecking or linear opacity of the enamel. Mild fluorosis is sometimes difficult to distinguish from developmental defects of enamel arising from other causes. The use of low-fluoride (440  ppm fluoride) junior toothpastes and the very cautious use of systemic supplementation reduce the risks of fluorosis. When recommending fluoride strategies, the risks of developing fluorosis (which can potentially create relatively minor aesthetic challenges in the permanent dentition) need to be weighed against the risk of developing dental caries and all its potential sequelae. Discussion between a paediatric dentist and the family is important in order to optimize prevention but reduce unwanted side effects.

Dental abscesses

If dental caries is undiagnosed, untreated or treated inappropriately it can cause pain, systemic infection and abscesses. Once the bacterial acids have caused breakdown of the tooth surface, the cavity becomes colonized with a range of microorganisms. If left, these will penetrate through the tooth to the pulp (or nerve), prompting a characteristic inflammatory reaction, a component of which is pain. Initially, the inflammation is reversible, the pain is sporadic and occurs only in response to stimuli such as changes in temperature. However, as the process continues the inflammation becomes irreversible, the pulp necroses and becomes colonized by bacteria and an abscess forms.

In the primary dentition an abscess can be superficial, pointing beneath the gum usually on the cheek side of the tooth and is often relatively painless. Alternatively and more seriously, an infection from a necrosed tooth can spread in to the deeper soft tissue planes and lymph nodes. Cellulitis is accompanied by systemic illness, high fever, facial swelling and often limited mouth opening. In the upper jaw this can cause closure of the eye while in the lower jaw can compromise the airway as the submandibular and sublingual spaces become involved. This is more common with an abscessed permanent molar tooth as the roots are located deep in the alveolar process of the mandible. In most cases a simple clinical examination will identify the affected tooth; however, a panoral radiograph is useful to confirm the diagnosis. Once antibiotic control has been established the involved tooth should be extracted as soon as possible (often under general anaesthesia).
Practical point

• When presented with any facial swelling, consider a dental cause as part of the differential diagnosis

Up to one-third of children will experience some sort of injury to either their primary or permanent teeth. The peak ages are between 18 months and 3 years when infants are learning to walk and prone to falling and again in early teens when boys in particular are participating in more adventurous games and sports. Most injuries in children are accidental in nature, as opposed to adults, where fights and traffic accidents are not uncommon.

Dental injuries can affect the teeth, bone and soft tissues or any combination of the three, with injuries to the teeth and lacerations of the tongue and lips being the most common. Jaw fractures are relatively uncommon in children and are not covered further here.
Clinical example

Jimmy, aged 3 years and 6 months, presented with his mother because he had been crying when he ate or drank at the day-care centre. Recently his mother had noticed that he sometimes woke up at night crying because of the pain in his mouth. On being asked about his health his mother reported that he had seen a heart doctor at the children’s hospital because he had a ‘hole in the heart’ and she had been told that he would need an antibiotic if he ‘had to have his teeth out’. She also acknowledged that Jimmy still took a bottle of milk to bed at night and that she sometimes put a little of his favourite chocolate powder in the milk to help him sleep.

Oral examination confirmed the presence of early childhood dental caries, with extensive decay involving the incisor teeth in both upper and lower jaws as well as the upper first primary molars and some white areas of early enamel demineralization on the recently erupted second molars. In addition, Jimmy’s gums were slightly inflamed and there were widespread deposits of white, furry plaque over this teeth and gums.

From the history of spontaneous pain particularly at night it was apparent that the decay was well advanced and involved the nerve (pulp) of at least some of the teeth. The presence of generalized plaque deposits around the teeth and gums suggested that toothbrushing did not happen regularly, if at all. In addition to posing a risk of bacteraemia (and hence for endocarditis in Jimmy, who has a pre-existing cardiac history) the presence of gingivitis also suggested that Jimmy was not receiving the optimum benefits to be gained from regular topical fluoride (toothpaste) exposure.

Management was in two stages. First, the immediate relief of pain and elimination of infection was achieved through the removal of all pulpally involved teeth and restoration of non-pulpally involved teeth. This was completed under general anaesthesia (because of Jimmy’s age and the extent of the treatment to be carried out) with an appropriate antibiotic cover of amoxicillin given at the time of anaesthetic induction. Second, proactive preventive advice was provided at the follow-up outpatient appointment. This advice included recommending (and demonstrating) toothbrushing using a fluoridated junior toothpaste, to be done by Jimmy’s mother twice a day, at the same time ceasing immediately the night time use of the infant feeding bottle. Finally, information was provided to Jimmy’s mother on the particular importance of good oral health for people with congenital heart disease.

Oral health for children who have other healthcare needs is often not high on the list of parental priorities. Close cooperation is necessary between doctors, paediatricians and paediatric dentists to assist parents of such children in appreciating the importance of oral health for their child and providing advice on how to minimize potential future problems. It is never too early to start to talk about teeth.

Injuries to the primary dentition most commonly involve displacement of the upper primary incisors from their sockets with or without some soft tissue lacerations. In general infants are very resilient and parents are often more stressed than the affected child.

Injury to the primary teeth can, in some instances, affect the development of the underlying permanent successor. Primary teeth are never reimplanted as this too can have a detrimental affect on the permanent successor, however, parents can be reassured that early loss of primary incisors has no lasting effecting on speech, function or appearance.
Practical points

• Check that there are no other signs of injury such as limb fractures or head injury

• Refer to an appropriate dental professional

• If the displacement is mild and not affecting function (i.e. the child can bring their teeth together) regular monitoring may be appropriate

• If the tooth is more severely displaced, it should be removed, either under local or general anaesthesia depending on the age of the child and nature of the injury

• An avulsed primary is never repositioned

Injuries to the permanent dentition range from a small tooth fracture, to complete loss (avulsion) of multiple teeth and significant soft tissue lacerations. Injuries to the permanent dentition are often missed or incorrectly diagnosed. Appropriate emergency management of traumatized teeth impacts significantly on the ultimate outcome of the injury.

Unless replaced immediately the long-term prognosis for an avulsed permanent tooth is guarded. Given that this rarely occurs, the avulsed tooth should be handled as little as possible and stored in milk. The child should be referred immediately by telephone to an appropriate dental provider. Direct contact with the dentist will ensure that s/he is prepared to accept the child for treatment, thus avoiding further delays. In many instances this will be a paediatric dentist or hospital-based dental unit. Dental management of displaced teeth usually involves repositioning accompanied by placement of a thin wire splint bonded to the affected and some unaffected teeth to stabilize the injury for several days. In some cases further endodontic (root canal) therapy may be required and treatment can continue for many years after the initial injury. In many cases the prognosis for retaining traumatized teeth in the long term is very good. However, avulsion and severe intrusion injuries, particularly those with additional bony fractures, do not have a good prognosis in the long term. The aim of treatment in these cases is to retain a child’s natural teeth through adolescence so that once growth is complete a permanent prosthe-tic replacement of the compromised teeth can be considered.
Practical points

• All injuries should be carefully examined intraorally

• Ideally, an avulsed permanent tooth should be repositioned immediately

• Irrespective of the degree of seriousness of the injury, trauma to the permanent dentition should be referred promptly (by telephone) to a willing dental professional

Orofacial trauma is present in 50% of reported cases of child abuse. Bruising and laceration of lips and alveolar mucosa, damage to teeth, alveolar bone fractures, finger and bite marks on face and neck are all frequent signs of child abuse. These result from slapping, punching, hand over mouth, forcible feeding with spoon or fork and forcible intrusion or removal of a feeding bottle, dummy or toy from the mouth. Tears to the upper midline frenum in pre-ambulatory infants are highly suspicious and oral bruising and palatal contusion can be seen in cases of sexual abuse. Oral signs should not be neglected when considering the possibility of child abuse and an intraoral examination should form part of routine surveillance. (See also Ch. 3.9.)

Riga–Fédé ulceration

A Riga–Fédé ulcer is an ulcer that develops on the underside of the tongue. It is most common in infants with significant intellectual impairment or those with sensory deficits such as Riley–Day syndrome who continually rub the tongue backward and forward over the sharp edges of the lower incisor teeth. In some cases these ulcers interfere with feeding and become infected. Conservative management, covering the lower incisors with a small bonded splint, can be successful in allowing healing. If not then removal of the appropriate teeth is required.

Dental erosion

Dental erosion is defined as a chronic localized loss of dental hard tissue chemically etched away from the tooth surface without bacterial involvement (which differentiates it from caries, which is bacterial in aetiology). Many young children show wear of their primary incisors but more worrying is the fact that up to one-third of adolescents have significant wear of their permanent teeth as well. The appearance of erosive tooth wear is quite characteristic but possibly a little difficult for most non-dental professionals to diagnose. There is a general loss of lustre from the surface enamel, thinning and chipping of the upper incisors and there may be smooth exposed dentine, pulpal exposure and sensitivity.

As with dental caries the aetiology is multifactorial but is associated with an increase exposure of the teeth to acid (Table 22.3.4). These non-bacterial acids may be extrinsic (essentially dietary) or intrinsic (from the gastric tract).

Given the high prevalence of asthma, particularly in Australia and New Zealand, a suggested association between it and dental erosion is of note. This association appears to be more of a problem in adults than children, and one large study in children showed no such association. There are theoretical reasons that this might occur, e.g. chronic mouth breathing from associated allergic rhinitis leading to mouth dryness; higher incidence of gastro-oesophageal reflux, which may result in more acid in the oral cavity; an acidic base to some nebulizer solutions.

Children with dysphagia and other conditions associated with abnormal muscle tone tend to have more gastric reflux and are more likely to have dental erosion. In addition, those children with muscle spasticity tend to grind their teeth, which, superimposed upon the softening caused by acidic erosion, can lead to rapid tooth tissue loss.

The role of the medical practitioner in managing dental erosion is to be aware of the risk factors, to provide generic preventive advice (Table 22.3.5) and refer the child to a dentist for ongoing monitoring. If caught early enough, little active dental treatment will be required; however, if significant tooth tissue loss occurs in the young permanent dentition then pain and aesthetic considerations may mean that restorative treatment is required.

• Any child with a history of gastric reflux should be counselled as to the potential for erosive tooth tissue loss and advised to seek dental review

Bleeding gums

Gingivitis is the most common cause of bleeding gums. Gingivitis is a non-specific inflammation of the gingival tissues (gums) that reflects the bacterial challenge to the host when dental plaque accumulates. Dental plaque is a complex biofilm that starts to accumulate around the teeth immediately after toothbrushing. It consists of 70% microorganisms and 30% food debris and if brushing is ineffective the gums mount a subclinical inflammatory response within 2 days that will become clinically apparent by 10 days. The characteristic signs of chronic gingivitis include red inflamed gums that bleed when brushed and that can deteriorate to a point where there is bleeding on eating and, in some cases, even spontaneous bleeding. Gingivitis is relatively easily reversed with appropriate oral hygiene practices to remove the plaque.

If left untreated, gingivitis may progress to periodontitis, in which the inflammatory response becomes more destructive within the underlying supporting tissues. This can lead to significant bone loss around the teeth, which become mobile and may ultimately be lost. There is a high degree of individual susceptibility to periodontal disease, with certain subgroups, e.g. individuals with Down syndrome, being particularly prone to destructive periodontal disease. Fortunately, with the exception of some specific conditions, e.g. Ehlers–Danlos syndrome, and individuals with cyclic neutropenia children and adolescents do not experience significant periodontal disease.

Non-plaque-related causes of bleeding gums include; local trauma, exfoliating teeth, hormonal changes, e.g. puberty, underlying haematological conditions, e.g. leukaemia, and some drugs, e.g. cytotoxics and anticoagulants.

Oral ulceration

Ulceration of the oral cavity is often a sign of underlying systemic disease and if so treatment of the oral lesions will be essentially symptomatic whilst the underlying disorder should obviously be attended to appropriately.

A careful history and examination should be completed in order to assist with the diagnosis.

• Every child with a special health-care need should have a regular dentist familiar with the implications of their medical history

• Good communication between paediatricians and paediatric dentists will optimize health outcomes for individuals and their families

• The specific prescription for sugar-free alternatives should be part of routine clinical practice

• Parents of children with a congenital heart defect should be provided with written confirmation of their child’s cardiac status with respect to endocarditis

• Standard protocols for prophylactic antibiotic cover should be followed when a child with a significant congenital heart defect requires any invasive dental procedures. The protocols can be accessed at: http://www.rch.org.au/cardiology/health-info.cfm

The most common oral infection seen in children is primary herpetic gingivostomatitis. The signs are of a systemic viral infection with fever, lassitude, lack of appetite and very sore oral cavity with characteristic painful ulcerated gingival tissues as well as occasional ulcers on the tongue and buccal mucosa. Other viral infections can involve the oral cavity including hand, foot and mouth disease and chicken pox.

Treatment is symptomatic, involving fluids and analgesia and encouraging the parents to maintain oral hygiene although this can be difficult when the mouth is very sore (Ch. 14.1).

Trauma

Single ulcers located in specific sites around the oral cavity can be caused by both physical or chemical trauma. Use of a toothpick to relieve food packing between teeth can cause ulceration interdentally while the placement of an aspirin tablet directly on to the buccal gum in order to relieve toothache has been known to produce a nasty chemical ulcer. In most cases of a traumatic ulcer, the aetiology will be relatively clear; however, it is important to bear in mind the possibility of self-inflicted injury. Particularly in instances of apparently random and repeated episodes of traumatic ulceration, there may be an associated condition involving insensitivity to pain, such as Riley–Day syndrome.

The elimination of the irritant, e.g. a fractured filling, poorly contoured orthodontic appliance, or cessation of the injurious behaviour.

Aphthous ulcers

In many situations an obvious aetiology is not forthcoming. These are referred to as aphthous ulcers. Fortunately, they are relatively uncommon in children, as they are difficult to treat. They are non-infective, extremely painful ulcers occurring most commonly on the labial and buccal mucosa and tongue borders. A prodromal burning sensation precedes breakdown of an initially white papule to form an ulcer with a crateriform base that heals slowly over 8–10 days. Aphthous ulcers may be of minor or major type and are often associated with stress; however, they may also be associated with an underlying iron or folate deficiency. They may be recurrent and associated with menstruation in adolescent girls.

Aphthous ulcers are difficult to manage and treatment is rarely totally successful. Chlorhexidine gluconate or a tetracycline/nystatin combination mouthrinse along with diligent toothbrushing helps to relieve pain and control secondary infection. Other strategies for aphthae include topical an­­aesthetics, systemic analgesics and topical anti-inflammatories, including steroids. However, these medications will at best reduce the duration of the ulcer experience rather than cure it.

Haematological

Children suffering from an underlying haematological disorder, including neutropenia and leukaemia, can experience oral ulceration. Children with leukaemia may present initially with bleeding gums in the absence of poor oral hygiene. Ulceration accompanied by mucositis and secondary infection is also common as a sequel to chemotherapy. Those with cyclic neutropenia in particular experience cyclical bouts of severe gingivitis accompanied by significant periodontal bone loss resulting in premature loss of both primary and later permanent teeth. Children with this condition need regular review and management by a paediatric dentist or periodontist in order to try and retard the process.

Gastrointestinal

As the oral cavity is the start of the gastrointestinal tract it is unsurprising that disorders affecting the rest of the tract can affect the mouth. Individuals with inflammatory bowel diseases, Crohn disease and ulcerative colitis (Chs 20.2 and 20.3) may experience oral ulceration. In addition, those with Crohn disease can have other significant oral manifestations, often described as orofacial granulomatosis, including:

• marked lip enlargement, together with enlargement of the buccal mucosa

• mucosal erythematous granulomatosis in the anterior regions of the maxilla

• large, linear ulceration in the buccal sulci posteriorly.

These oral findings can occur on their own or together with anal fissuring, genital swelling and gastrointestinal changes.

In patients with coeliac disease, a higher incidence of recurrent aphthous ulceration has been suggested but not proved. Such patients may also have dental enamel defects in the permanent teeth.

Treatment

If there are any concerns about gastric symptoms, e.g. weight loss, blood in the stools, the child should be referred to a paediatric gastroenterologist and paediatric dentist for a combined evaluation.

Dermatological

Oral ulcers occur in many of the rarer dermatological conditions such as epidermolysis bullosa, pemphigus and pemphigoid. However, these conditions are usually diagnosed from their more general manifestations rather than from their oral symptoms.

Malignancy

Oral malignancy is rare in children; however, single persistent, large ulcers that have no obvious traumatic aetiology nor are associated with acute systemic illness should be viewed with suspicion and referred for investigation to a paediatric dentist/maxillofacial surgeon.

Gingival swelling

Swelling of the gingival tissues can be congenital or acquired; however, the former are relatively rare. Individuals with any of the mucopolysaccharidosis disorders may have very enlarged gums, often to the extent that teeth fail to erupt or are ectopically displaced. Similar disruption to the developing dentition can occur in individuals with vascular anomalies such as a lymphangioma, which may affect both soft and hard tissues of the jaws.

Of the acquired causes of gum enlargement, drugs are most commonly implicated, specifically the antiseizure medication phenytoin and the immunosuppressant cyclosporin A. The latter has been widely used in organ transplant protocols and in children has caused significant aesthetic and psychological problems as the gums enlarge in an unsightly fashion and teeth fail to erupt or are covered in excess gum tissue. The advent of tacrolimus as an alternative to cyclosporin A has improved the quality of life for these children significantly by reducing not only the gum overgrowth but also the hirsutism. Most of the enlargement resolves once the causative drug, be it phenytoin or cyclosporin A, is ceased. Other more localized swellings of the gingival tissues include giant cell granuloma, histiocytosis and lymphoma and, while rare, such swellings are of obvious clinical significance and should be reviewed by a paediatric dentist.

Children with special healthcare needs

Children with significant medical conditions, developmental disabilities and craniofacial disorders face multiple health issues, among which is often an increased risk of dental disease coupled with barriers in accessing appropriate dental services. Given that many dental problems are relatively simple to prevent and manage if diagnosed early, close collaboration between the general health-care professional (medical practitioner, paediatrician or nurse) and the dental profession is essential. Identifying an appropriate ‘dental home’ for children with special health-care needs is important so that these complex children have access to timely and appropriate oral health-care. Many general dentists are not comfortable managing children with special health-care needs, whereas paediatric dentists are trained specifically in this area. Establishing a close collaboration between medical and dental clinicians not only optimizes the child’s oral health but significantly improves their general health and wellbeing as well as reducing the need for extensive, costly and burdensome dental treatment.

Medications

Many children with special healthcare needs are on multiple medications for prolonged periods of time. With the exceptions of the anticonvulsant phenytoin, the immunosuppressant cyclosporin and nifedipine, all of which cause gingival overgrowth, very few drugs directly affect the oral tissues. However, there is an increased risk of developing caries associated with the long term use of sugar-based liquid medications. As many of the common liquid medications are over-the-counter formulations, parents should be warned to check that, where possible, they purchase a sugar-free brand of medication such as cough linctus or analgesic.

While the evidence regarding the impact of asthma, and its treatment, on oral health is weak (see Dental erosion, above), nevertheless, ensuring that all children are registered with a dentist who is familiar with their general health issues will optimize their general wellbeing.

Congenital heart disease

Parents of infants diagnosed with a congenital heart anomaly should be provided with clear information regarding the implications of oral health on the general health of their child. Parental information sheets exist in most paediatric units to provide information and advice (http://www.rch.org.au/emplibrary/cardiology/dentalheart.pdf). Despite a lack of convincing evidence that dental disease and/or its treatment actually does cause bacterial endocarditis, proactive prevention (to minimize disease experience) and prophylactic antibiotic cover for any dental procedure that is likely to cause bleeding is still considered best practice (Ch. 15.2). Many parents are confused as to whether their child has a significant structural heart anomaly and whether they really do need antibiotic cover. When taking their child to a dental professional, parents should be provided with written confirmation of their cardiac status together with a clear indication of the risk of endocarditis and the need for prophylaxis endorsed by their cardiologist. This will facilitate the smooth provision of appropriate dental care and minimize the unnecessary prescription of antibiotics.

• Every child with a special health-care need should have a regular dentist familiar with the implications of their medical history

• Good communication between paediatricians and paediatric dentists will optimize health outcomes for individuals and their families

• The specific prescription for sugar-free alternatives should be part of routine clinical practice

• Parents of children with a congenital heart defect should be provided with written confirmation of their child’s cardiac status with respect to endocarditis

• Standard protocols for prophylactic antibiotic cover should be followed when a child with a significant congenital heart defect requires any invasive dental procedures. The protocols can be accessed at: http://www.rch.org.au/cardiology/health-info.cfm