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August 2008: Meningococcal disease warrants prompt referral

What are the key features of invasive meningococcal disease?

How should suspected cases be managed?

Has vaccination been effective?

What are the key features of invasive meningococcal disease?

How should suspected cases be managed?

Has vaccination been effective?

Meningococcal disease caused by Neisseria meningitidis continues to cause long-term morbidity and death in previously healthy children and young people. Combined figures from England, Scotland and Wales give an incidence of around 1,200-1,600 cases per year. The fatality rate is approximately 6%,1,2 with many of these deaths occurring in the first 24 hours of illness.

Cases can occur at any time of year, but are more common in the winter, when outbreaks of respiratory infections occur.

Although N. meningitidis causes meningitis, it is important to recognise that most of its devastating effects are due to the systemic inflammatory syndrome resulting from invasion of the organism into the circulation (septicaemia), rather than the consequences of meningeal inflammation.

There are at least 13 pathogenic serogroups of N. meningitidis, all of which can cause severe disease. Before the meningococcal C vaccine was introduced in 1999, groups B and C accounted for the majority of cases in the UK. Since then the number of cases has almost halved and most are group B. Group A is mainly found in sub-Saharan Africa and W135 has caused outbreaks associated with the annual hajj pilgrimage.

Recently, there has been a series of campaigns to increase public awareness of invasive meningococcal disease, including use of the glass test to recognise a purpuric rash. There have also been advances in intensive care management, which have improved the prognosis of those surviving long enough to access these services.

However, the most significant development has been the conjugate meningococcal C vaccine. This has now been followed by conjugate vaccines incorporating groups A, Y, W135 and C. Developing a vaccine for group B meningococcal disease has proved more problematic. However, over the past few years major strides have been made towards this goal.

It is possible, therefore, that the next generation of doctors will rarely see a case of invasive meningococcal disease in the UK. In the meantime, however, clinicians must remain alert to the possibility of invasive meningococcal disease, act swiftly when required but also remain confident in their clinical assessment of an unwell child.

Invasive meningococcal disease may present a diagnostic challenge as not all children present with the classic signs and, given the potential severity and rapidly progressive nature of the disease, early recognition and prompt, appropriate management is essential to optimise outcome.

A new guideline from SIGN on the management of invasive meningococcal disease in children and young people provides a framework for diagnosis and referral, see figure 1, opposite.3

Diagnosis

Occasionally, children can present with conjunctivitis, arthritis or chronic rash. However, the most common patterns of presentation are:

• Meningococcal septicaemia (20% of cases). The main features are fever, purpura and signs of sepsis
• Meningitis, which is clinically indistinguishable from meningitis caused by other bacteria
• A mixed picture, with features of both meningococcal septicaemia and meningitis.

A generalised petechial rash beyond the distribution of the superior vena cava or a purpuric rash in any location are strongly suggestive of meningococcal septicaemia. Sick children with these symptoms require urgent treatment and referral to secondary care. Obviously, rapid deterioration in the clinical condition of a child also warrants urgent referral.

Fine petechial rashes in the distribution of the superior vena cava can be caused by retching and vomiting. However, if the child is ill, even in these circumstances it is important not to discount the possibility of invasive meningococcal disease.

The following features in a sick child should raise the suspicion of invasive meningococcal disease:

• Petechial rash
• Altered mental state
• Cold hands and feet
• Extremity pain
• Fever
• Headache
• Neck stiffness
• Skin mottling.

The positive predictive value of any of these features in isolation has not been determined and may be low, but in combination they become more significant.

Infants with meningitis may not have the classical signs of fever, headache and neck stiffness but present with non-specific symptoms. Hence the guideline recommends that meningococcal disease should not be automatically ruled out in a young child presenting with fever, lethargy, poor feeding, nausea, vomiting and irritability or a non-blanching rash during the initial 4-6 hours of illness.

Invasive meningococcal disease rashes are vasculitic, so at first the lesions may blanch. However, they tend to have an irregular, moth-eaten appearance not typical of viral exanthemata.

Assessment

Assessment is based on taking a good history, bearing in mind the parents' concerns, and conducting a full examination. If there are clear signs of meningitis or septicaemia prompt action should be taken.

The guideline recommends that if the child is considered at low risk of having meningococcal disease the parents should call back if the child deteriorates.

The medical consultation, however thorough, provides only a snapshot of a child's illness. Any child with a fever may go on to develop septicaemia, although the risk in the absence of specific symptoms and signs is very small. Therefore, if there is diagnostic uncertainty the child should be reviewed 4-6 hours later. In this case it is important that robust arrangements are made to ensure the review takes place, particularly if the child is initially seen out of hours. Parents should seek further advice if the child deteriorates, for example the rash changes, before the planned reassessment.

Obviously, reassessment should not delay treatment and referral in children at high risk of having invasive meningococcal disease.

It is important to assess how well the parents are coping with the child's illness and if they are able to continue care, detect any change in the child's condition and act appropriately.

Referral

Children deemed at high risk of invasive meningococcal disease should be referred immediately to secondary care. See figure 1, attached, for guidance on risk assessment and management.

The threshold for referral should be lower if there are concerns that the parent may not be able to monitor the child and call for medical help appropriately. It may also be lower in remote and rural areas, where the response time once a call is made may be considerably longer than in an urban area.

Treatment

There is a paucity of good quality trial data to underpin any recommendations related to care outside the hospital setting. However, expert opinion regarding the standard of care is well established. Hence the guideline recommends that parenteral antibiotics (either benzylpenicillin or cefotaxime) should be given as soon as invasive meningococcal disease is suspected. Treatment should only be withheld if there is a clear history of true anaphylaxis to these antibiotics.

SIGN has adopted The Joint Royal Colleges Ambulance Liaison Committee and the Meningitis Research Foundation recommendations when treating children and young people with suspected invasive meningococcal disease before they reach hospital.

The guideline recommends:

On scene:

• Appropriate airway management
• Oxygen therapy (with assisted ventilation if required)
• Rapid transportation to the nearest appropriate hospital

En route:

• Administer iv or im benzylpenicillin (or cefotaxime)
• Treat shock with boluses of iv crystalloid
• Identify and treat hypoglycaemia
• Provide hospital alert message, including age of patient.

The referring doctor should do as much of this as possible until the ambulance arrives.

Follow up

Most children with meningococcal disease make a full recovery. However, long-term complications do occur, particularly in those who have had severe sepsis. Children should have been given an individual care plan on discharge from hospital, with a follow-up appointment aiming to identify any ongoing concerns. Complications include:

• Hearing loss
• Neurological sequelae, eg seizure disorders, motor deficits, neurodevelopmental delay and learning difficulties
• Bone and joint complications, particularly injury to growth plates, which may not become apparent for many years
• Post-necrotic scarring
• Renal impairment
• Psychiatric, psychosocial and behavioural problems.

It is also important to appreciate that family members and carers, as well as affected children, may develop post-traumatic stress disorder following this episode.

Parents and carers should know what to look out for and that support is available.

Key points Figure 1. Management of invasive meningococcal disease in children and young people Author

Dr Rosie Hague
MD MRCP FRCPCH
consultant in paediatric infectious diseases and immunology, Royal Hospital for Sick Children, Glasgow and member of the SIGN Guideline Development Group for the management of invasive meningococcal disease in children and young people

Meningococcal disease warrants prompt referral Useful information

The SIGN guideline and quick reference guide on the management of invasive meningococcal disease in children and young people are available to download free from the SIGN website
www.sign.ac.uk

The Meningitis Research Foundation offers support for patients and families affected by meningitis and septicaemia
24-hour helpline: 0808 8003344
www.meningitis.org

The Meningitis Trust provides information, counselling and home visits for families affected by meningitis and septicaemia
24-hour helpline: 0800 0281828
www.meningitis-trust.org

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