PIG Meeting: 8th April 2021

29-year-old lady for elective ganglion excision from her wrist.

Background

• Netherton syndrome – rare autosomal recessive disorder. Characteristic triad of congenital ichthyosiform erythroderma, a specific hair shaft abnormality termed trichorrhexis invaginata (“bamboo hair”), and an atopic diathesis.
• 2 syncopal episodes a few years ago thought to be due to acquired long QT
• Regular cardiology review with recent normal echo and holter.
• No current evidence of long QT, normal ECG at clinic

Issues

• Patient’s mother is requesting cardiac anaesthetist for procedure as per previous cardiologist
• Anaesthetist called new Cardiologist with mother in room, advised she does NOT need to have a cardiac anaesthetist
• Patient’s mother became distressed and left the consultation after extensive discussion that a cardiac anaesthetist wasn’t required for this procedure. She stated that she no longer wanted to proceed with surgery.

Discussion

• Difficult situation to navigate
• Anaesthetic consultant and the patient’s current cardiologist were both involved in the discussions
• The benefits of regional anaesthesia in peripheral surgery were discussed. This was unfortunately declined due to anxiety.
• Techniques to manage the expectations of the patient while still working within acceptable limits of time and clinical resources
• Examples of strategies from pain clinics outlined
• Elective procedure, option to delay surgery and allow family time to consider options
• Complex social and medical needs of family were recognised as significant stressors

Plan

• Follow-up nursing phone call with family revealed that patient has decided to proceed with surgery as planned
• Procedural anaesthetist informed of events