Walking on a tight rope: Anaesthetic Management of a Patient with Vascular Ehlers Danlos Syndrome for an Elective Caesarean Section

 

Vascular Ehlers-Danlos Syndrome (EDS) is a rare autosomal dominant genetic disorder caused by structural abnormalities in type-III collagen.

Pregnancy is not advised due to significant maternal risks and mortality.

These patients present unique challenges due to their heightened risk of vascular fragility, bleeding, hyperextensibility and uterine rupture.

C A S E  R E P O R T

20 year old primiparous woman diagnosed with vEDS prenatally

History: Excessive bruising, prolonged bleeding from minor wounds.

Transferred Care: Referred at 19 weeks’ gestation

 

A N A E S T H E T I C P L A N

General anaesthesia based on patient preference and multidisciplinary counselling.

 

M A N A G E M E N T

Standard AAGBI with invasive blood pressure monitoring*

GA technique tailored to minimise airway trauma and haemodynamic instability.

Blood conservation strategies: Prophylactic Tranexamic Acid and Desmopressin along with Intraoperative cell salvage.

 

GA vs RA

Bleeding Risk, Increased blood loss, vascular dissection

Neuraxial Haematoma, dura fragility

Haemodynamic Stability

Controlled Blood pressure management

Neuraxial hypotension

 

O U T C O M E

Healthy female infant delivered at 34 weeks

Mother transferred to maternal high dependency unit for observation

 

D I S C U S S I O N

Anaesthetic management remains controversial

Consideration General Anaesthesia Regional Anaesthesia

Airway Management Trauma – due to tissue fragility Avoids airway instrumentation

A collaborative approach - careful risk benefit analysis

Individualised Decision-making

Multidisciplinary Team Involvement

Meticulously planning

 

 

References

1. Malfait F, Francomano C, Byers P, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017.

2. Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV. N Engl J Med. 2000.