Anesthesia management for patients with congenital epidermolysis bullosa: A challenge case report for the anesthesiologist.

INTRODUCTION AND

IMPORTANCE

Congenital epidermolysis bullosa (EB) is a group of rare genetic conditions characterized by fragile skin that blisters easily. Anaesthesia management in these patients is complex due to mucocutaneous fragility, which can complicate surgical procedures and airway management. Our study aimed to report a case of successful general anaesthesia in two patients with congenital epidermolysis bullosa undergoing oesophagal dilatation.

CASE PRESENTATION: The case involved a brother and sister, aged 21 and 15 respectively, both of whom have congenital epidermolysis bullosa and presented with solid dysphagia due to double stenosis of the oesophagus. Anaesthesia management included meticulous preoperative planning, use of non-adhesive monitoring equipment, careful management with video laryngoscopy, and minimisation of skin trauma.

CLINICAL DISCUSSION: Both patients underwent successful esophageal dilation under general anaesthesia without perioperative complications. Airway management was achieved in the first attempt using video laryngoscopy. Analgesia was effectively treated with paracetamol and tramadol.

CONCLUSION

This case illustrates the complexities and necessary precautions for anaesthesia management in patients with epidermolysis bullosa. Detailed preoperative evaluation, careful monitoring, and specific handling techniques can mitigate perioperative risks and ensure patient safety.