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.