An anaesthetist performs a nasotracheal intubation of a child.

Advanced airway management in patients with a known complex disease


This investigation looks into patient safety issues associated with airway management – the techniques used by healthcare professionals to help patients to get enough oxygen into their lungs, for example during surgery or a medical emergency.

The investigation focuses on the potential safety risks to people who may have a ‘difficult airway’ – that is, the anatomy of their mouth, throat or windpipe makes it difficult for health professionals to manage their airway. This can be the case for people with certain complex health conditions or diseases. In particular, it looks at the use of advanced airway management techniques including intubation, where a tube is passed through a patient’s mouth or nose, then down into their lower airway. The tube keeps the upper part of the airway open so that air can get through to the patient’s lungs.

There is no standardised definition of a ‘difficult airway’. However, it is generally referred to as a clinical situation in which a healthcare professional who is skilled at airway management encounters difficulty with one or more standard methods of airway management.

As an example, which is referred to as ‘the reference event’, the investigation considered the airway management of Ethan, a boy aged 12 who had a diagnosis of Hunter syndrome (a genetic condition that often affects the anatomy of the airway). Ethan was admitted to an emergency department after having a seizure at home. Attempts to intubate Ethan failed and he died.

The investigation’s findings, safety recommendations and safety observations aim to help healthcare professionals quickly recognise whether someone has a potentially difficult airway and may need advanced airway management techniques to keep their airway open. Some of the findings and conclusions may also be applicable to other health conditions.

The reference event

Ethan was taken to an emergency department (ED) by ambulance after his sister found him struggling to breathe and moving in a strange way. The ED staff thought that he was fitting and that this was likely caused by a lack of oxygen to his brain.

Ethan was given medicine in the ED to control his fitting. Basic airway management techniques, such as adjusting the position of his head, neck, and jaw, were used to help keep his airway open. He was prescribed and given additional oxygen. It was thought that Ethan may need to be intubated to help keep his airway open, but the procedure was predicted to be difficult because of his Hunter syndrome and severe obstructive sleep apnoea (a sleep disorder where the airway becomes blocked).

Ethan was monitored for several hours and the risks of intubation versus managing his airway using basic airway management techniques were continually assessed. Ongoing discussions took place with Ethan’s care team at the specialist (tertiary) hospital and with a patient retrieval service (the service that would be used if Ethan needed to be transferred to a hospital with a paediatric intensive care unit). Ethan began to show signs that his condition was deteriorating, and he was getting tired, so a decision was made to move to advanced airway management and specifically, intubation.

To ensure the best possible conditions for a potentially difficult intubation, Ethan was taken to one of the hospital’s operating theatres. This had more space and easier access to more specialised airway management equipment than the ED. Intubation using a camera (videolaryngoscopy) was attempted but was unsuccessful. Ethan was given oxygen between consecutive attempts at intubation. The difficult airway guidance was followed, and an emergency opening was created at the front of his neck so a tube could be inserted into his windpipe. This was also unsuccessful. An on-call ear, nose and throat (ENT) consultant was contacted as the team was unable to intubate Ethan. Attempts at creating an airway using surgical techniques were unsuccessful and Ethan died.

The investigation

A challenging airway is a well-recognised problem in people with a known complex disease, for example a metabolic disease like Hunter syndrome. The effectiveness of enzyme replacement therapies for people with metabolic disorders means their life expectancy is improving and therefore as they live longer, they may need surgical procedures for complications of their disease. During surgical procedures where a general anaesthetic is required, normally routine procedures such as tracheal intubation may be particularly difficult. Recognised rescue strategies should intubation fail (for example the creation of an emergency front of neck airway) may also be more difficult. Both procedures may be even more difficult if they are attempted in an emergency.

Risks associated with intubation include minor injury or damage to teeth, lips, mouth, or nose. Serious problems can occur when providing adequate oxygen is difficult or impossible and can lead to emergency front of neck airway procedures, airway trauma, unplanned admission to intensive care units, and can result in brain injury or death.

There are no standards for how an anticipated difficult airway is managed. In addition, healthcare professionals working in primary, secondary, and tertiary care (for example, GP practices, hospitals and specialist units), may not all have access to information about a patient’s anticipated difficult airway. These issues were explored as part of the investigation.


  • There is no nationally recognised system for sharing clinical information about people with a known difficult airway between primary, secondary, and tertiary care.
  • There is no standard process for documenting and sharing an individualised airway management plan for people with a complex disease to all health care professionals and services involved in their care.
  • Multidisciplinary team meetings to discuss the care of people with a complex disease and who have a known difficult airway are not happening consistently between primary, secondary, and tertiary care.
  • Existing guidance for healthcare professionals on how to care for people who have a complex disease and may have a difficult airway is not always co-ordinated and consistent.
  • There is currently no national standard for treating people with a known potentially ‘life threatening’ difficult airway who require advanced airway management.
  • The requirement for additional skills, for example a head and neck specialist or ear, nose, and throat (ENT) specialist, in emergency situations where a patient requires advanced airway management is challenging as 24-hour on-site ENT provision is not available in every hospital.
  • Training and competency assessment in videolaryngoscopy is not standardised and there is variability in how and when videolaryngoscopy is used.
  • Training and competency assessment for anaesthetists on airway rescue techniques such as emergency front of neck airway (eFONA) is variable.
  • The design of equipment to support advanced airway management does not consistently include robust user testing at a national level to help identify and understand risks.