Discussion
Ehlers‒Danlos Syndrome (EDS) describes a group of inherited connective tissue disorders that affect collagen synthesis with different subtypes. EDS affects approximately 1 in 5,000 people worldwide4. The cardinal features include joint hypermobility, skin laxity, hyper-extensibility and tissue fragility. These features amount to significant risks of major post-operative complications including mortality1.
Surgical intervention in patients with Ehlers–Danlos syndrome is challenging. A high frequency of surgical complications have been described in the literature, especially for the vascular sub-type (EDS4)4,5 Patients with EDS4 are prone to spontaneous rupture of visceral organs, aneurysms and dissections with an increased mortality rate2-4. In addition, EDS patients pose a further anaesthetic risk of cervical atlantoaxial subluxation as a consequence of the laxity of the cervical ligaments, as well as temporomandibular dislocation during endotracheal intubation9.
In our experience with this patient, successful surgical outcome was achieved through meticulous preoperative preparation aimed at minimising the risks involved. In this report we detail the steps we have taken which may be useful for all clinicians dealing with these patients in the future. This is also the first report in the literature detailing the steps of successful outcome of laparoscopic cholecystectomy in a patient with EDS4.