ࡱ> c jbjbSS %11+C]l l l l l l l | | | | | D | m        , DYl      l l     l  l   ~ l l l l  fl l  DN| | Part 22 ENT, EYE AND DENTAL DISORDERS 22.1 Ear, nose and throat, and head and neck surgery problems 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 auditory canal and the tympanic membrane are usually inspected (if necessary and if possible after removal of wax) using a handheld, battery-operated otoscope or a Seigle magnifying pneumatic speculum. An ENT specialist uses a slim, short telescope or a microscope for more detailed examination. 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 Black, brown, yellow or pale wax is a mixture of sebaceous material and ceruminous gland secretion combined with desquamated epithelium. Although normally removed by the self-cleaning chewing movement of the temporomandibular joint and evaporation, wax occasionally accumulates to occlude the canal, causing a hearing loss and a sensation of blockage. Obsessive parental attempts to clean normal wax with cotton buds or a matchstick or repeated daily insertion of a hearing aid mould may cause impaction of the wax, requiring removal by syringing or use of special blunt probes and suction by an ENT specialist. Sometimes, in obstinate cases, wax must be removed under general anaesthesia with the aid of an operating microscope. 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 airwater 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 bodys 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 510% 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 3040% 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 2448 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 510 days is the drug of choice and is generally well tolerated. Erythromycin, sulfamethoxazoletrimethoprim 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. Clinical example 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 39.4C. 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 3050% 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 810 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: doesnt 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 612 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 Johns 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. Practical points 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 babys 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 childs 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 mothers 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 Congenital conditions 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 3mm 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 710 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 Fracture or dislocation deformity of the external nose and nasal septum sometimes occurs during a difficult birth or after forceps delivery. The degree of displacement and nasal obstruction are occasionally severe enough to require correction in the neonatal period. Injuries of the nose, nasal bones and nasal septum occur commonly when toddlers fall during vigorous play and in older children during contact sports. Depressed, displaced nasal bones require correction within 10 days. Haematomas of the nasal septum should be drained and treated with antibiotics to minimize development of a septal abscess, which destroys cartilage and can lead to a saddle nose deformity. 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 (Littles 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, EpsteinBarr 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 36 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 1020% 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 mothers 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 2448 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 2448 hours: in fact treatment begun within 79 days of the onset is effective. Bear in mind that 1020% 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. Practical points 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. ArnoldChiari 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 68 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. 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. 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. 22.2 Eye disorders in childhood J. E. Elder A systematic approach to childrens eye disease allows rapid determination of the correct diagnosis or initiation of appropriate further investigation. Visual development A rapid sequence of anatomical and functional changes in the visual apparatus enables vision to develop from a very low level after birth to near adult levels by 1218 months of age. At birth an infant has visual acuity of approximately 6/120 and by 12 months this has improved to about 6/12. This rapid development is the result of retinal maturation, myelination of the visual pathways, the ability to accommodate (change the focal length of the eye) and maturation within the visual cortex. The first three of these processes (retinal maturation, myelination and accommodation) are complete by 46 months of age. The maturation of the visual cortex occurs more gradually, over a 68-year period, with the most rapid phase being in the first 2 years. Anything that interferes with this gradual cortical maturation may result in the development of amblyopia, which is one of the commonest ophthalmic abnormalities of childhood (see Amblyopia, below). Measurement of vision in children Measurement of visual acuity in preverbal children presents a challenge. Asking the parent Does your child see well? or How well do you think your child sees? often gives useful information about an infants visual function. If a parent expresses concern about an infants vision, take note, as this concern is often well founded. An understanding of normal visual behaviour is vital to estimating visual function in infancy. At birth, when alert, an infant should be able to fix on a face briefly. By 6 weeks of age most infants smile in a visually responsive fashion to a face. At this age the infant will also be able to follow a face or light through an arc of 90. By 6 months of age an infant can reach for a small object and can actively follow objects in the visual environment. At 12 months of age a child should be able to reach and pick up tiny objects such as hundreds-and-thousands (sprinkles). More formal assessment of visual acuity becomes possible with the development of language. Children with specific language delay or more general intellectual delay will have difficulty with these tests of visual acuity. Picture-naming tests can be done by children between 2 and 3 years of age. Single letter-matching tests are within the abilities of most 34-year-olds. The standard Snellen chart test is often not performed well until the child is between 5 and 6 years of age. The vision should be tested for each eye individually. As with all testing in children, patience and an encouraging manner are vital to obtain the best results. Repeat the test on another occasion if the test results seem inaccurate. The notation for documenting visual acuity is the Snellen fraction, e.g. 6/6. Most visual acuity tests use standard distances of 3 or 6m between subject and chart. The numerator of the Snellen fraction is the distance from the chart while the denominator indicates which line on the chart was the smallest to be seen. If the vision is poor the subject should be brought closer to the chart. The vision then may be recorded as 2/18 or 1/60, etc., depending on how close the subject is to the chart and which line is read. What level of vision is abnormal? (or, when to refer!) This depends on the age of the child. An infant who is not fixing and following by 34 months, or reaching for small objects and tracking objects in the visual environment by 812 months, deserves further examination and investigation. If the child is able to do a more formal test of acuity, a difference between the two eyes of two or more lines (that is 6/6 and 6/12) indicates the need for further assessment. In children less than 3 years of age vision of 6/18 or less in either eye should prompt referral and in children older than 3 years 6/12 is an acceptable cut-off for referral. Assessment of a child with a possible eye problem History Prematurity, perinatal difficulties (e.g. birth asphyxia), significant syndromes (e.g. Down syndrome) and other sensory impairment (e.g. deafness) are associated with an increased risk of eye disease. Developmental delay often interferes with assessment of visual acuity, especially if language or intellect is affected. Common childhood eye problems such as strabismus and refractive errors have a clearly identified familial tendency, although the precise genetics are not well understood. Finally, the parents perception of a childs visual function is important, particularly if there is concern that the vision is poor. Examination In keeping with paediatrics in general, observation without approaching or touching a child often supplies a great deal of information. By observation it is possible to rapidly determine an infants use of vision. Does the child smile at a face? Is the child looking around the room? If something moves, does the child look to it? If there is a noise, does the child look to the source of the noise? A blind child will become still and will often drop the head down while using hearing to further localize the source of the sound, but will not look towards this source. Most eyelid, eyelash and ocular surface abnormalities can be detected readily by simple observation. Many intraocular abnormalities can be detected by examination of the red reflex. This is the red to orange colour seen within the pupil when the line of illumination and observation are approximately coaxial (that is, the same). This situation is most easily obtained by observing the childs eye with a direct ophthalmoscope from a distance of about 1m. It is then easy to compare the reflexes for the two eyes and the child is not threatened by the examiner getting too close. A dull or absent red reflex indicates an opacity, such as a cataract, in the normally clear media of the eye. A white reflex results from an abnormally pale reflecting surface within the eye, such as a white retinal tumour (retinoblastoma). While these intraocular disorders are rare, they are important in terms of the severe effect on vision or threat to life. Misalignment of the eyes Strabismus or squint is common in childhood and accurate assessment to confirm or refute the presence of misalignment is an important skill for anyone who deals with children. Observation will confirm the presence of large-angle strabismus. However, a broad nasal bridge or prominent epicanthic folds will mimic milder degrees of strabismus, especially in younger infants. This situation is known as pseudostrabismus (Fig. 22.2.1). The epicanthic folds cover the sclera on the medial aspect of the globe while the lateral sclera is easily visible. This creates the appearance of misalignment, particularly when the child looks laterally. Examining the symmetry of corneal light reflections will help to avoid being misled by pseudostrabismus. The cover test is by far the most reliable method of detecting strabismus. The cover test is done by first getting the child to fix on an object while the observer determines which eye appears to be misaligned. The eye that appears to be fixing on the object (and not misaligned) is then covered while the apparently misaligned eye is observed. If strabismus is present a corrective movement of the misaligned eye will be seen as this eye takes up fixation on the object of regard (Fig. 22.2.2). If no movement is seen then the eye is uncovered. The cover test is then repeated but the other eye is covered this time and the eye that is not covered is again observed for a corrective movement and, if present, strabismus is confirmed. The test can be repeated as many times as necessary. If no movement is seen following repeated covering of either eye, then no strabismus is present. Care must be taken to let the child fix with both eyes open before covering either eye, otherwise normal binocular control may be prevented and a small latent squint (phoria) may be detected. Latent squints are normal variants and are of no significance. Common eye problems in childhood Amblyopia Amblyopia is the cortical response to abnormal input from the eyes and is manifest as reduced visual acuity in one or both eyes. This abnormal input may result from a refractive (spectacle) error, a structural abnormality of the eye (e.g. cataract) or strabismus. Provided it is detected early enough, while the developing visual cortex is immature, amblyopia is treatable. Conversely, if the amblyopia is not treated before visual cortex maturation (about 7 years of age), it may not be reversible later in life. Detection of amblyopia is one of the major reasons for routine visual screening in childhood. Common causes of amblyopia are strabismus and refractive errors. Refractive errors cause a poorly focused image to form on the retina and thus a poor-quality image to be transmitted to the cortex. Such input does not stimulate normal cortical development and amblyopia results. Strabismic (misaligned) eyes are not necessarily out of focus; however, if the cortex paid attention to the image from each eye diplopia would result, as each eye is sending a different view of the world. In children, the immature visual cortex is capable of ignoring the image from one eye. If this situation is allowed to persist the cortex may completely ignore or suppress the input from a deviating eye and amblyopia will result. Treatment of amblyopia involves correcting any focusing errors with appropriate spectacles and forcing the brain to use the amblyopic eye by depriving the brain of clear input from the better-seeing eye, most commonly with a patch. Unfortunately, simply realigning strabismic eyes is not enough to overcome amblyopia secondary to strabismus. Strabismus A squint or misaligned eye is important to detect as it is frequently associated with amblyopia. Most childhood strabismus is the result of failure of binocular control at a cortical level within the central nervous system (CNS). Less commonly it is the result of cranial nerve lesions or extraocular muscle disease. In most children this CNS abnormality in eye movement control is an isolated abnormality with no other associated neurological or intellectual problems. However, children with widespread CNS abnormalities have an increased risk of developing strabismus. Down syndrome is a good example of this, with an approximately tenfold increase in the risk of developing strabismus. The diagnosis of strabismus is outlined above and detailed consideration of the therapy of strabismus is beyond the scope of this chapter. The following is a brief description of the commoner patterns of strabismus seen in childhood and an outline of management. Infantile esotropia This is a large-angle convergent squint seen before 6 months of age. Strabismic amblyopia is common in infantile esotropia but refractive errors are rare. Patching followed by surgery is the most common initial treatment. Children with infantile esotropia need to be followed up throughout childhood, as about one-third need more than one operation and amblyopia can occur following apparently successful initial treatment. Intermittent divergent strabismus This occurs from 18 months of age onwards. It is often more noticeable on distance fixation and may be associated with closure of the deviating eye, especially in bright light. Amblyopia is uncommon, as the deviation is intermittent and presumably when the eyes are straight normal visual development proceeds. In some cases the divergence becomes more constant and in such situations surgery may be undertaken to improve alignment. Accommodative esotropia This occurs in children who are excessively long-sighted (hypermetropic). To overcome hypermetropia and focus a clear image on the retina, accommodative effort is used. Accommodation consists of the combination of changing focal length of the lens and converging the eyes (so that both are directed at the nearer object of regard). Thus in children with excessive hypermetropia there is increased focusing and at times excessive convergence; that is, a convergent squint (esotropia) appears as a result of the increased accommodative effort used by these children. Accommodative esotropia can be completely or partially corrected by prescribing glasses that compensate for the appropriate amount of hypermetropia. Amblyopia is often seen in association with accommodative esotropia and requires treatment. If glasses only partly correct the esotropia, surgery may be indicated to obtain optimal alignment. Clinical example A 3-year-old girl presented with a history of a worsening inward turn of her left eye over four months. The cover test confirmed a left convergent squint and the red reflex was normal in each eye. Subsequent assessment by an ophthalmologist confirmed the findings and her visual acuities were 3/3 in the right eye and 3/9 in the left with refraction showing she was long-sighted. When next seen 6 months later she was wearing glasses and had been patching her right eye 2 hours a day. Her eyes were straight to cover test and her parents reported that her left vision was slowly improving and that surgery was not going to be required in her case. Refractive problems Refractive problems are the result of defects in the focusing components of the eye. These defects include abnormality of corneal curvature (a frequent cause of astigmatism) and abnormalities of lens power and axial length of the eye (which may result in hypermetropia or myopia short-sightedness). Children will rarely complain of poor vision related to refractive error. Rather, they readily accept the vision they have and get on with life. Children with high myopia will often manifest myopic behaviour (they will go very close to objects to look at them). Different refraction in either eye will often result in amblyopia because one eye will generally have a clearer image than the other and thus enable better cortical development for that eye. Routine screening of visual acuity is the only reliable way of detecting the majority of refractive errors in children. In many countries there are both preschool and school entry tests of visual acuity for this reason. Such screening testing needs to be reliable, available to all of the target population and followed up with appropriate intervention when defects are identified. As cortical maturation of vision occurs at about 7 years of age, screening should ideally commence in 34-year-olds, before any amblyopia becomes difficult to reverse. If a refractive error is suspected in a young child because of strabismus or poor visual acuity, then accurate and objective testing with cycloplegic retinoscopy is required. If a child is prescribed glasses, these should be worn the majority of the time. Practical points Vision assessment and strabismus  The parents assessment of their infant child s vision is often very accurate; if they are concerned their child is not seeing you should be concerned also "  Always try to gain as much information as possible by observation before actively engaging a child in an examination. Red reflexes can be observed from a distance. Urgent referral is required if an abnormal red reflex is found "  Children under 7 years of age will rarely complain of visual difficulty relating to refractive errors (reduced visual acuity). A vision screening programme is the most reliable method of detecting reduced visual acuity in children of this age "  Strabismus is a common cause of amblyopia. All suspected or confirmed strabismus should be referred to an ophthalmologist for further assessment and management "  Cover testing is the most accurate clinical method of diagnosing strabismus. When doing a cover test always make sure that the child is looking at a interesting fixation target  a small toy is useful for near fixation and a picture or larger toy for distance fixation "  Amblyopia is generally reversible if it is detected early and appropriate management is initiated Watery and sticky eyes This occurs commonly in infancy as the result of congenital nasolacrimal duct obstruction. About 10% of newborn infants have obstructed nasolacrimal ducts. This will present as a watery and sticky eye in the first few weeks of life. Despite the persistent discharge the eye is generally not red or inflamed. An inflamed eye suggests an alternative diagnosis such as infective conjunctivitis. If the obstruction persists, the lower lid will often become red and sometimes slightly scaly as a result of the skin being constantly moist. The differential diagnosis includes trauma, conjunctivitis and infantile glaucoma. These conditions are all described below. Most congenital nasolacrimal duct obstructions resolve spontaneously. Approximately 95% of cases have resolved by the time of the first birthday, with most doing so in the first 6 months. In persistent cases, probing under a general anaesthetic is recommended after 1 year of age. Trauma Trauma to the eye can take many forms. Physical trauma to the eye and surrounding structures may be blunt or sharp. Trauma can also result from radiation (thermal and electromagnetic) and chemical agents. Direct blunt trauma to the eye may disrupt iris blood vessels, causing bleeding in the anterior chamber of the eye (hyphema), tear the iris, dislocate the lens, rupture the choroid and rarely rupture the eye wall (sclera) if the force is sufficient. Simple inspection of the eye will reveal most of these injuries and choroid and globe rupture may be suspected on the basis of the nature of the injury and associated poor vision. Referral to an ophthalmologist is necessary in these cases for confirmation of the injury and further management. Sharp trauma may be due to a range of causes, from tiny objects such as a subtarsal foreign body causing a corneal abrasion, to fingernail scratches through to penetration of the eye by sharp objects such as a scissors blade or dart. Surface trauma can be diagnosed easily with the help of fluorescein staining and a cobalt blue light. Areas of epithelial abrasion will fluoresce green. If a round ulcer and/or vertical linear abrasions are seen, suspect a subtarsal foreign body and the upper lid should be everted. If identified, most subtarsal foreign bodies can be removed with a moistened cotton bud. Superficial trauma is treated with antibiotic ointment and a patch and daily review until any epithelial defect (ulcer or abrasion) is healed. If the wall of the eye (cornea or sclera) has been penetrated, intraocular contents may prolapse out through the wound, the iris and pupil may appear distorted or the anterior chamber may be shallower than normal. Any suspected penetration of the eye must be referred to an ophthalmologist for further investigation and management. The eye should be protected with a cone that does not exert any pressure on the eye. If vomiting is likely or occurs an anti-emetic should be given to prevent further prolapse of intraocular tissue. Thermal injuries to the eye itself are rare, as in most burn situations the eyelids are firmly closed and thus protect the eye. Facial burns may cause scarring that interferes with lid function, leading to exposure and drying of the eyes surface. If a primary thermal injury to the eye is suspected, fluorescein dye should be used to detect any ulceration. If ulceration is found, treatment is with antibiotic ointment and a patch. Radiation injuries to the eye are rare in childhood and most are the result of intentional irradiation as part of medical therapy for facial and ocular neoplasia. Typical injuries are cataract, dry eye syndrome, radiation retinopathy and optic neuropathy. These changes are seen some considerable time after the irradiation. Chemical burns to the eye are unusual in childhood but potentially are very serious, especially if the chemical is alkaline. Many domestic cleaning agents are alkaline. Strong alkali will denature and dissolve protein and penetrate deeply into the surface of the eye. Acids tend to coagulate surface structures and this often prevents deeper penetration of the acidic chemical into the eye. Immediate first aid should consist of copious irrigation with water at the site of the accident and this should be continued for at least 10 minutes. Following adequate irrigation all chemical burns of the eye should be referred to an ophthalmologist. Practical points Blocked tear ducts and eye injury "  Most blocked tear ducts resolve spontaneously by 1 year of age "  Simple inspection with the addition of fluorescein staining will enable the diagnosis of most physical trauma to the eye "  All chemical injuries to the eye should be regarded as serious. Copious irrigation is the cornerstone of immediate management of all chemical injuries to the eye Conjunctivitis Conjunctivitis may result from infective, allergic or chemical agents interacting with the conjunctiva. Symptoms are itch, pain and irritation or a gritty sensation. Signs are epiphora (watering), discharge and erythema of conjunctiva and lids. The relative prominence of different symptoms and signs varies with the cause of the conjunctivitis (Table 22.2.1). Conjunctivitis occurring in the first few weeks of life is generally bacterial and frequently acquired from the birth canal. Neisseria gonorrhoeae and Chlamydia trachomatis both cause a conjunctivitis with copious discharge and marked erythema in the neonatal period, termed ophthalmia neonatorum. Gonococcal conjunctivitis is serious because of the risk of spontaneous perforation of the cornea and resultant loss of vision and also the risk of more generalized sepsis. Chlamydial conjunctivitis is significant because of the risk of more generalized chlamydial sepsis. For accurate and prompt diagnosis of these infections, microbiological diagnosis and systemic as well as topical antibiotic therapy is needed. For culture, conjunctival swabs should be directly inoculated onto culture medium plates and conjunctival scrapings for Gram staining and immunofluorescent staining should be taken. Bacterial conjunctivitis occurring outside the first few weeks of life in children is usually the result of relatively innocuous organisms (e.g. Staphylococcus spp. and Haemophilus spp.) Microbiological investigation is not usually indicated and a broad-spectrum topical antibiotic should be prescribed (such as neomycin/polymyxin or chloramphenicol). Concern has been raised about topical chloramphenicol preparations because of a perceived risk of secondary agranulocytosis. It is the authors belief that this risk is extremely low but does exist and should not stop short-term use of topical chloramphenicol. Viral conjunctivitis is relatively common at all ages and clinically may be very difficult to differentiate from bacterial conjunctivitis. The discharge may be somewhat less with viral conjunctivitis. Viral conjunctivitis is frequently associated with upper respiratory tract infection symptoms. If the aetiology is uncertain, topical antibiotics as for bacterial conjunctivitis should be used. Allergic conjunctivitis is common in children of all age groups and has itch as its most prominent symptom. House dust mite, grass and other plant pollens are common allergens that precipitate allergic conjunctivitis. Therapy depends on the severity of the symptoms. If mild, cold compresses may be all that is needed. For more severe symptoms soothing topical astringent agents that include a topical antihistamine are helpful. In more persistent and severe cases topical sodium cromoglycate and steroid preparations may be indicated. Topical steroid should be used with the supervision of an ophthalmologist because of the risk of significant side effects, including cataract and glaucoma. Clinical example A 4-year-old boy presented with a 12-hour history of a red and watery eye. He complained of pain and his parents had not observed any discharge. Examination revealed a red eye with no obvious trauma or foreign body on the surface of the eye. Fluorescein staining demonstrated a round ulcer on the upper part of the cornea. On everting the upper eyelid a small foreign body was found and was removed with a moistened cotton bud. The ulcer was treated with antibiotic ointment and a pad and healed in 1 day. Lid infections These are common in children and most arise in the skin appendages of the eyelids (lash follicles and meibomian glands). Infection of a lash follicle is called a stye (or hordeolum externum) and acute infection of a meibomian gland is known as hordeolum internum. Unless there is significant secondary erythema of the surrounding lid, topical and systemic antibiotics are not indicated. Occasionally, severe preseptal cellulitis will follow a focal lid infection and systemic (often intravenous) antibiotics will then be needed for treatment. More chronic inflammation of a meibomian gland is known as a chalazion. This is generally the result of sterile chemical inflammation rather than infection and occurs when the contents of a meibomian gland escape into the lid following blockage of the opening of the gland at the lid margin. A chalazion will appear as a lump in the substance of the lid and is often not particularly inflamed in appearance. Topical antibiotics seldom hasten resolution. Warm compresses may give symptomatic relief and help drainage. Chalazia may persist for many months. Some will discharge through the conjunctiva or the skin. On occasions surgical drainage is indicated for a persistently inflamed and large chalazion. Practical points 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 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 childs 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 childs 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 34 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. Practical points Further important considerations "  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 eye in paediatric systemic disease 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 3050% of infants weighing less than 1250g 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 <1250g) 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 Developmental anomalies of the soft tissues 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 5mm 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 5mm 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 38C 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. Clinical example 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 47 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 1000ppm (parts per million) fluoride whilst junior toothpastes contain lower concentrations of fluoride, around 400ppm. 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. Diet 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. Remineralization products Recently new products have been developed that contain casein phosphopeptideamorphous 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 (440ppm 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 Trauma 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 childrens 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, Jimmys 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 Jimmys 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 Jimmys mother twice a day, at the same time ceasing immediately the night time use of the infant feeding bottle. Finally, information was provided to Jimmys 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. Primary dentition 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 Permanent dentition 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 childs 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 Non-accidental injury 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.) RigaFd ulceration A RigaFd 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 RileyDay 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. Practical point  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 Acquired disorders of the soft tissues 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. EhlersDanlos 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. Practical points  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 Infections 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 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 RileyDay syndrome. Treatment 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 810 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. Treatment 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 childs 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. Practical points "  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 Fig. 22.1.1 Pure tone audiometry at 3 4 years of age. The child drops a coloured bead into the box when a sound is heard. A reasonably reliable pure tone audiogram can be obtained. Fig. 22.1.2 Parents often suspect deafness if the baby consistently fails to respond to loud sounds and  sleeps peacefully . Their suspicion of deafness should be investigated. Fig. 22.1.3 There are a number of tests for screening the hearing of newborn babies. The normal response to a sudden loud sound (80 90dB) is a  blink or  startle reaction. This test is best done with the infant lightly asleep. Fig. 22.1.4 Most babies over the age of 4 5 months are able to turn to a noise from an unseen source, so that each ear can be tested. The intensity of the sound may be varied to estimate the level at which response occurs. Fig. 22.2.1 This infant has prominent epicanthic folds, giving rise to the appearance of misaligned eyes. This is pseudostrabismus. Note that the corneal light reflections are symmetrical. Cover testing failed to reveal misalignment of either eye. Fig. 22.2.2 Cover test. First the child s attention is attracted with a toy (top). Then the eye that appears to be looking directly at the toy is covered and the other eye is observed for a refixation movement (bottom). If there is a convergent squint there will be an outward movement of the uncovered eye (pictured) and if there is a divergent squint there will be an inward movement of the eye. If no movement is detected, the test should be repeated but covering the other eye first. Fig. 22.3.1 Developmental defects of primary tooth enamel at 4 months in utero (iu), 7 months in utero, and birth stages of tooth development. Table 22.2.1 Signs and symptoms of conjunctivitis Cause of conjunctivitis Symptoms Signs Viral Moderate discomfort Moderate epiphora Mild discharge Mild to moderate erythema Bacterial Moderate to severe discomfort Moderate epiphora Copious discharge Moderate to severe erythema Allergic Itch often prominent Mild to moderate epiphora Stringy discharge Mild erythema Chemical Pain intense Severe epiphora Mild discharge Moderate to severe erythema The descriptions in this table are intended to be a guide; there may be considerable variation and overlap in the signs and symptoms of conjunctivitis due to different causes. Table 22.3.1 A summary of the eruption times for primary and permanent teeth Primary dentition (months after birth) Central incisors Lateral incisors Canines First molars Second molars 6 12 9 16 16 23 13 19 23 33 Permanent dentition (years of age) Central Lateral First Second First Second Third incisors incisors Canines premolars premolars molars molars molars 6 8 6.5 8.5 9 13 9.5 11.5 10 13 5.5 7.0 11 13 17+ Table 22.3.2 The common risk factors for dental caries Risk factor Influence Fluoride exposure Exposure to fluoridated water source and the regular use of fluoridated toothpaste are two key factors that reduce caries risk Sugar exposure Infant feeding habits are very important with frequency of exposure being most relevant. High risk associated with prolonged on-demand night-time feeds Family oral health history Poor parental oral health places child at risk of decay as cariogenic bacteria can be transmitted to infants from their primary care giver (usually the mother) Social and family practices Poor, indigenous, ethnic and migrant groups have higher levels of dental disease Medical history Medically compromised children are more at risk of dental decay the impact of which, on their general health, can be considerable. They are also less likely to receive appropriate treatment Saliva flow Children with reduced salivary flow are at significant risk of developing caries as the acids in the oral cavity cannot be diluted, buffered and cleared effectively. Examples of such children are those on certain medications (e.g. anti-depressants, anticholinergics), exposed to radiotherapy or with certain conditions, e.g. Prader Willi and Velocardiofacial Syndrome. Table 22.3.3 A summary of caries preventive strategies Fluoride A smear of toothpaste should be applied regularly to an infant s teeth within 6 months of their  eruption For most individuals a junior toothpaste (400ppm fluoride) will be adequate until about the age  of 5 6. However, for children with additional risk factors, an adult toothpaste may be more  appropriate Teeth should be brushed twice a day with nothing to eat or drink after the night-time brushing Parents should supervise toothbrushing until around 8 years of age The use of additional topical fluoride supplements (tablets or drops) can be of benefit to a few  individuals but should be prescribed by an appropriate dental professional Diet Reduce the total amount and frequency of intake of sugary foods and drinks Avoid on-demand feeding through the night Limit sugary snacks to meal times, when salivary flow is optimal Avoid sugary snacks close to bedtime Increase water intake Dental attendance Parents should be encouraged to take their infant to a dental professional within 6 months of  the eruption of their first teeth Regular monitoring by a dental professional should continue in to adulthood Remineralizing products Products containing calcium phosphopeptides, e.g. Tooth Mousse (GC Corporation, Itabashi-ku,  Tokyo, Japan), are available through dental practitioners. These promote remineralization of  early carious lesions Table 22.3.4 The potential sources of acid responsible for dental erosion Extrinsic Intrinsic Dietary Gastric reflux Citrus fruits and juice Cerebral palsy Carbonated drinks (including Dysphagia   diet products) Gastro-oesophageal Sports drinks  reflux Wine Environmental/occupational Eating disorders Swimming (poorly regulated Bulimia nervosa  pool water) Nutrition regimes for elite athletes Lifestyle Rumination Recreational drugs Medications Topical effect aspirin, vitamin C, phenylketonuria supplements, nebulized asthma medications (although this is controversial, and probably a minor problem more likely to affect adults) Systemic effect on saliva anti-depressants, anticholinergics Some mouthwashes Table 22.3.5 Strategies to prevent dental erosion Reducing acid exposure Inform patients of types of foods and drinks that have greatest erosive potential Consumption of still/non-carbonated drinks as an alternative Limiting the intake of acidic foods/drinks to meal times Advocate consumption of a neutral food, e.g. cheese, immediately after a meal Rinsing mouth out after acid exposure, i.e. after an episode of vomiting, but delaying  brushing teeth immediately after the exposure as this increases wear of tooth tissue. 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