Medically compromised children can be challenging to treat and can affect the provision of dental care. To treat medically compromised patients safely it is important to:

1. Obtain a relevant and thorough medical history

2. Understand the possible implications of the illness on dental treatment

3. Understand the possible implication of the condition on treatment planning

4. Understand the caries risk associated with the medical condition

With advances in medical treatment, significantly more children are surviving longer with more complex medical needs and these children will present to the general dentist for dental treatment.

Medical HistoryCardiovascular DisordersDental ManagementChallenges Involved In Treating A Child With CHDKey Points

All patients should have an accurate medical history recorded prior to any dental treatment and this should be updated at each visit.

These can be divided into two main groups – congenital heart defects (CHD) (a defect of the heart or great vessels present at birth) or acquired after birth (e.g. rheumatic fever). Congenital heart disease has an incidence of 7-8/1000 live births and may be cyanotic or acyanotic.

The acyanotic group of conditions are characterized by a communication between the systemic and pulmonary circulation without and shunting of blood (a pattern of blood flow in the heart that deviates from the normal pattern of the circulatory system. It may be described as left-to-right, right-to-left or bidirectional), or stenosis (narrowing) of a segment of the circulation.

The most common acyanotic lesions are:

1. Atrial Septal Defect

2. Ventricular Septal Defect

3. Patent Ductus Arteriosus

4. Coarctation of the aorta

5. Aortic stenosis

6. Pulmonary stenosis

Cyanotic conditions exhibit right-to-left shunting of desaturated blood. The most common cyanotic lesions are:

  1. Tetralogy of Fallot
  2. Transposition of the great vessels

Intensive prevention is imperative for this group of patients.

Prior to the publication of the NICE guidelines in 2008, many patients with heart defects required the prescription of antibiotics prior to invasive dental treatment. This aimed to reduce the risk of infective endocarditis (IE). However, NICE recommended that antibiotic cover should NOT be given to patients at increased risk of infective endocarditis in 2008.

This was because there was no evidence that antibiotics prevented infective endocarditis and each antibiotic exposure may trigger an anaphylactic reaction.

NICE have more recently revised the wording of the guidance to state,

“Antibiotic prophylaxis against infective endocarditis is not recommended routinely for people undergoing dental procedures”

For the dental team:

  • Lack of awareness of parent/carer
  • Low priority
  • Low attendance
  • Nutritional issues
  • Medication
  • Increased anxiety

(Balmer et al, 2003 – Stecken Blicks, 2004 – da Fonseca, 2009 – Hollis, 2014)

For the patient/parent:

  • Multiple hospital visits
  • Psychosocial issues
  • Dental team lacking in confidence
  • Lack of provision of appropriate care

(Parry and Khan, 2000 – Tasioula et al, 2008)

It is important to note that the caries risk for patients with CHD is controversial – some studies have shown increased caries risk and others have shown a similar level of caries to the general population. However, studies agree that the care index is low.

Patients with cyanotic cardiac defects may be at risk of increased bleeding.

1. Pulpotomy or pulpectomy is contraindicated in children with CHD due to increased risk of infective endocarditis.

2. There is no contraindication to the use of vasoconstrictors in local analgesia in children with CHD.

3. Electrosurgery, electronic pulp testers and ultrasonic cleaning should be avoided in children with cardiac pacemakers.

4. Ensure that patient is not at risk of prolonged bleeding due to thrombocytopenia (platelet count less than 150 x 10(9)/L and/or anticoagulant medication)

Bleeding Disorders

Haemophilia AHaemophilia BVon Willebrand DiseasePlatelet DisordersThrombocytopeniaPlatelet Function DisordersDental ManagementKey Points:

This is an X-linked recessive disorder which leads to a decrease in levels of clotting factor VIII. It is passed from mothers to sons and affects 1:10,000 live births. Daughters of a father with Haemophilia A are obligate carriers. The disease may be classified as mild, moderate or severe depending on circulating factor VIII levels

Severe – Plasma activity > 1 IU/dl

Moderate – Plasma activity 2-5 IU/dl

Mild – Plasma activity 6-40 IU/dl

This is also inherited as an X-linked recessive condition and results in low levels of the clotting factor IX.

This is the most common inherited bleeding disorder and is an autosomal dominant condition. It affects approximately 1:1000 and is characterised by defective platelets that do not adhere to each other and low levels of factor VIII. The most common symptoms are epistaxis and gingival bleeding.

Platelet disorders may be classified as quantitative (low numbers) or qualitative (sufficient numbers but defect of function). The most common oral symptoms are spontaneous gingival bleeding and bruising.

This is defined as a platelet count <150 x 10(9)/L (normal range 150-400 x 10(9)/L).

This may be idiopathic (of unknown cause) or follow the administration of various drugs. Platelet count must be at lease 80 x 10(9)/L before dental extractions should be attempted.

These may be congenital or acquired and will present with the same symptoms as thrombocytopenia.

A comprehensive bleeding history is required prior to deciding on a treatment plan. If the child has a diagnosed bleeding disorder careful liaison with the child’s paediatric haematologist is required.

NEEDS TO BE REPLACEDTREATMENT OPTIONS 
Factor VII

Factor IX
Desmopressin (DDAVP) which stimulates the release of factor VIII
Anti-fibrinolytic agents (Epsilon-aminocaproic acid/ tranexamic
acid): prevents clot lysis
Fibrin Glue

1. Avoid mandibular block injections as they can cause haematoma formation in the pterygomandibular area leading to airway obstruction.

2. Avoid dental extractions where possible to reduce bleeding risk. Pulp therapy of restorable carious primary teeth is the treatment of choice.

3. Extractions will usually need to be managed in a hospital setting in conjunction with the haematology team.

4. Local measures such as direct pressure, placement of surgical and suturing should be employed when dental extractions have been completed.

Other Common Disorders

White Blood Cell Disorders: LeukaemiaDiabetes MellitusEpilepsyCerebral PalsyAutism Spectrum Disorder (ASD)Down SyndromeReferences

Leukaemia is a type of cancer of the blood or bone marrow characterised by an abnormal increase of immature white blood cells known as lymphoblasts. Acute lymphoblastic leukaemia (ALL) is the most common form of childhood cancer accounting for 75% of childhood leukaemia. Patients may present with oral mucosal/gingival bleeding, pallor, fever, spontaneous bruising and anorexia.

In the United Kingdom, there are approximately 1200 new cases of childhood cancer each year. The oral cavity frequently develops complications either as a direct result of malignancy or as an unwanted side effect of medication. Treatment includes chemotherapyradiotherapysurgery and haematopoietic stem cell transplantation. Dental prevention is a priority for this group of children.

Complications of Chemotherapy:

1. Oral mucositis

2. Thrombocytopenia

3. Anaemia

4. Viral infections such as herpes simplex

5. Taste dysfunction

6. Nausea and vomiting

Unlike the complications of chemotherapy, radiation damage is site-specific and toxicity is localized to irradiated tissues.

Complications of Radiotherapy:

1. Mucositis

2. Xerostomia due to salivary gland irradiation

3. Taste dysfunction

4. Replacement fibrosis of masticatory muscles leading to trismus

5. Osteoradionecrosis

6. Dental defects including hypodontia, microdontia, enamel hypoplasia, arrested root development, premature closure of apices.

Dental Management:

If a child with leukaemia does require dental treatment during chemotherapy, careful liaison with the child’s haematologist/oncologist is necessary to plan the timing of the treatment. The patient may need a platelet transfusion and/or antimicrobial prophylaxis prior to treatment depending on their blood counts. In the ideal situation, dental treatment would be deferred until the child is in remission.

Pulp therapy of primary teeth is contraindicated during chemotherapy due to the potential risk of infection.

Paediatric Oncology:

Pre-treatment:

Dental screening as soon as possible post diagnosis

Prior to chemotherapy starting:

  • Removal of infected teeth
  • Dressing of other carious teeth
  • Organise mouth care/mouthwashes etc

During treatment

Support and encourage maintained oral hygiene

Help/advise re management of specific oral problems.

Type 1 or insulin dependent diabetes mellitus is the most common endocrine disorder of childhood and affects approximately 2/1000 children. Peak age of presentation is 5-7 years and symptoms include polyuriapolydipsia and weight loss. The aim of treatment is to maintain blood glucose at a normal level, preventing hypoglycaemia and hyperglycaemia. Treatment includes the administration of insulin as well as dietary control.

Dental considerations:

1. Poor control leads to increased caries risk which may be due to altered salivary glucose levels

2. Poor control also leads to increased risk of periodontal disease which may be due to an altered response to infection

3. Increased risk of candida infection

4. Intensive prevention is a priority

5. Dental appointments should be made first thing in the morning just after the child has had breakfast and insulin/medication when their blood sugar is well controlled

6. Signs of hypoglycaemia such as sweating, trembling and aggression should be recognised before a child progresses to loss of consciousness.

A seizure represents the clinical expression of abnormal, excessive, synchronous discharge of neurons, residing primarily in the cerebral cortex. This abnormal activity is intermittent and self-limiting. An individual is considered to have epilepsy when seizures recur over a period without obvious precipitants. Epilepsy affects approximately 1:103 people in the UK or 0.97%.

Classification:

Seizures can be classified into focal and generalised.

1. Focal seizures initially originate from one hemisphere of the brain and can be classified as aware and impaired awareness.

Aware – consciousness is fully maintained and may cause unusual movement such as jerking movements of the arm, foot or face.

Impaired awareness – consciousness impaired and patient may display automatism such as lip smacking.

2. Generalised seizures originate from both cerebral hemispheres from the beginning of the seizure and can be classified as Motor and Non-Motor.

Motor – A tonic-clonic seizure presents with loss of consciousness, muscle stiffening and jerking movements.

Non-Motor – Absence seizure (previously known as petit mal). An absence seizures presents with the patient “blanking out” or staring into space for a short time.

Dental Management:

Patients with epilepsy may take anti-convulsive medication and it is important to be aware of the possible side effects of these medicines.

1. Phenytoin (Dilantin) – gingival hypertrophy and thrombocytopenia

2. Carbamazepine (Tegretol) – thrombocytopenia

3. Sodium Valproate (Epilim) – caries (the syrup formulation contains sugar) and thrombocytopenia

4. Levetiracetam (Keppra) – thrombocytopenia

Optimal oral hygiene is required to reduce the risk of developing gingival enlargement for patients taking phenytoin. Consider the risk of thrombocytopenic bleeding if extractions are planned and liaise carefully with the patient’s paediatrician/consider a full blood count prior to treatment.

If a child is taking the sugary Epilim syrup, discuss with their paediatrician the possibility of a change to the sugar-free Epilim solution. Patients are at risk of dental trauma during epileptic seizures, particularly if they have an increased overjet and orthodontic treatment should be considered in these cases.

If an epileptic child requires a removable appliance, it should be well retained with clasps to minimize the risk of inhalation during a seizure. Stressful situations may precipitate a seizure in some patients and treatment under inhalation sedation may need to be considered in these cases.

This is the name for a group of life long conditions that affect movement and coordination, caused by a problem with the brain that occurs before, during or after birth. Symptoms aren’t usually obvious at birth but become noticeable during the first two or three years of a child’s life.

Symptoms of Cerebral palsy:

  • Delay in reaching milestones – not sitting by 8 months or not walking by 18 months
  • Seeming too stiff or too floppy
  • Weak arms or legs
  • Jerky or clumsy movements
  • Random, uncontrolled movements
  • Toe walking
  • Swallowing difficulties
  • Delayed speech
  • Vision problems
  • Learning difficulties

Classification: there are four main types of cerebral palsy:

Spastic (70%) – increased muscle tone

Ataxic (10%) – problems with balance, movement, gait and coordination

Dyskinetic (10%) – muscle toneis mixed–it may be too lax or hypotonic at times and too tight or hypertonic at others

Mixed (10%) – patient has symptoms of more than one of the types above and different parts of the body can be affected:

  • Hemiplegia – one side of the body is affected
  • Diplegia – two limbs affected
  • Monoplegia – one limb affected
  • Quadriplegia – all four limbs affected

Dental Problems:

  • Poor oral hygiene due to mouth breathing and reduced manual dexterity
  • Increased tooth wear due to bruxism/gastro-oesophageal reflux
  • Drooling due to oral muscular hypotonia
  • Similar caries risk to unaffected children
  • Increased overjet due to incompetent lips and hypotonia of oral musculature
  • Tongue thrusting habit
  • Increased gag reflex

Medical problems:

  • Cognitive impairment
  • Epilepsy 20-40%
  • Joint contractures
  • Speech difficulty
  • Hearing impairment

It may be necessary to treat the patient in a more upright position if they have an enhanced gag reflex and positioning on the chair may be difficult if they have contractures.

Autism spectrum disorders (ASD) are a group of lifelong neuro-developmental disorders characterised by impairment of social reciprocitycommunication and behaviour. Prevalence is 1:88 and unrelated to race or socioeconomic status. Patients with ASD can show a broad range of behaviours from seeming withdrawn and avoiding eye contact to making eye contact, hugging and smiling.

It is important to note that this interaction is usually on the child’s own terms and not easily elicited by others. Intellect ranges from severe learning disability to above average intelligence although intelligence quotient (IQ) is not a defining feature of ASD.

Medical problems:

  • Iron deficiency anaemia
  • Epilepsy – 22-fold increased risk when compared with the general population
  • Abnormal sleeping pattern
  • Constipation
  • Macrocephaly

Dental problems:

  • Similar caries levels to general population
  • Poor oral hygiene which is attributed to poor manual dexterity
  • Increased risk of dental erosion due to bruxism and gastro-oesophageal reflux
  • Increased risk of dental trauma
  • Children with ASD may have difficulty expressing pain
  • Negative behaviour may be a barrier to accessing dental care
  • Busy waiting rooms may represent a problem for children with ASD
  • Children with ASD may not like bright lights, loud noise, dental smells or having their head touched
  • Children with ASD may communicate via sign language/ Makaton rather than verbal language.

Treatment Tips:

  • Do not keep patients with ASD waiting
  • Ask parents if the patient is likely to allow a dental examination/ to wear protective glasses/ allow a mirror in mouth
  • Children with ASD may not like light touch such as having their head touched but some are reassured by deep touch. Placing a lead apron over the patient during treatment may reassure them.
  • Routine is important to patients with ASD and seeing the same dentist and dental nurse at each visit will facilitate acclimatisation.
  • Children with ASD may not tolerate flavoured toothpaste – OraNurse, which is flavour free and comes in 1400 ppm fluoride is available online may be recommended.
  • Patients with ASD may not tolerate a dental examination but may open if given a hand mirror. This will allow the dentist to stand in front of the patient and he/she may have a brief look intra orally.
  • If a child with ASD will not sit on the dental chair, they may allow a dental examination sitting on a regular chair at a table.

Down syndrome (DS) is a genetic condition caused by a chromosomal abnormality resulting in a learning difficulty and a characteristic appearance. It is caused by a trisomy of chromosome 21 with an incidence of 1:700.

Characteristic Appearance:

  • Small at birth
  • Hypotonia
  • Growth defect–short, broad stature
  • Brachycephaly (flattenedback of skull)
  • Short arms/legs
  • Clinidactyly (curved finger most commonly fifth finger)•Slanting eyes
  • Epicanthic folds
  • Single palmar crease
  • “Sandal toe” deformity
  • Mid face hypoplasia

Medical Problems:

  • Cardiac defect – 40% (most commonly AVSD)
  • Neurological – atlanto-axial instability (10-15%), ASD (5-7%) and learning disability
  • Immunology – impaired cell mediated immunity with infection of the skin/GI tract/respiratory tract common
  • Increased risk of leukaemia
  • Endocrine – hypothyroidism and decreased growth hormone
  • Respiratory –recurrent respiratory tract infection, sleep apnoea and Subglottic stenosis
  • Obesity
  • Rheumatology – arthritis x 6 more common in children with DS

Dental problems:

  • Open mouth posture (due to muscle hypotonia)
  • Class III malocclusion
  • Anterior/posterior crossbite
  • Relative macroglossia
  • Fissured tongue
  • Hypodontia
  • Microdontia
  • Hypocalcification
  • Short roots
  • Taurodontism
  • Bruxism
  • Increased risk of periodontal disease
  • Decreased caries risk
  • Delayed eruption
  • Delayed exfoliation
  • Increased risk of cleft lip/palate

Why do patients with DS have a decreased caries risk?

  • Higher pH of saliva
  • Higher bicarbonate levels in saliva
  • Low streptococcus mutans levels in saliva
  • Hypodontia
  • Microdontia
  • Spaced dentition
  • Delayed eruption
  • Shallow fissures

Why do patients with DS have an increased risk of periodontal disease?

  • Impaired cell mediated immunity with reduced chemotaxis, diminished phagocytic activity and deficient T cell function
  • Abnormal bacteriocidal activity of polymorphonuclear leukocytes
  • Poor manual dexterity
  • Open mouth posture
  • Mouth breathing

Management tips:

  • Tell-show-do, distraction and positive reinforcement techniques can be beneficial
  • GA may be necessary for dental treatment
  • Due to risk of atlanto-axial instability – head is taped for stability during treatment under GA.

Little, J.W., Falace, D., Miller, C. and Rhodus, N.L., 2017. Dental Management of the Medically Compromised Patient-E-Book. Elsevier Health Sciences.

Seymour, R.A., 2003. Dentistry and the medically compromised patient. The Surgeon1(4), pp.207-214.

Cameron, A.C. and Widmer, R.P., 2013. Handbook of Pediatric Dentistry E-Book. Elsevier Health Sciences.

Barry, S.M., 2012. Improving access and reducing barriers to dental care for children with autism spectrum disorder. University of Leeds.

See also Safeguarding