Anaesthetic management of parturients with Ehlers Danlos Syndrome:a case series
Abstract Number: 256
Abstract Type: Case Report/Case Series
Introduction:Ehlers Danlos syndrome(EDS) is a group of inherited connective tissue disorders caused by a defect in collagen which reduces tissue elasticity and can affect the connective tissues in skin, ligament, muscle, bone, vessel and visceral organs.Depending on the mutation inherited there are six major types with symptoms which may be mild to life threatening with a variable incidence depending on the subtype.Anaesthetic concerns include difficult airway,easy bruising, arterial fragility, joint dislocations and osteoarthritis requiring careful positioining,pre existing nerve entrapments,dysautonomia,valvular heart disorders,post partum hemorrhage.
Case Series:We retrospectively reviewed the case notes of all Ehlers Danlos parturients admitted to our maternity centre between 2008-2010.We recorded the subtypes of EDS and with their relative pre -pregnant medical history.We also recorded number of admissions pre-delivery, gestation at delivery, delivery mode, and anaesthetic input in the peripartum period.We reviewed maternal and fetal outcomes, and any complications.
Six parturients presented to the unit during the review period, with an average age at delivery of 28yrs, and a mean gestational age of 38+6 weeks.Mode of delivery is listed in table as follows.Two labour epidurals were resited due to inadequate block.Maternal complications included three post partum haemorrhage with one patient requiring blood transfusion.One baby (born to a patient with preeclampsia) required admission to the special care baby unit(SCBU) for observation.
Discussion:Since EDS comprises of a spectrum of disease there is no standardised guidance for the care of these patients in pregnancy. Most of the literature comprises of single case reports.Most of the forms are mitis(Type II)although it is less reported.Uterine and arterial rupture has been reported resulting in high maternal mortality especially with Type I(gravis) and Type IV(ecchymotic.Tyson(1972) suggested that elective caesarean section be recommended for all(1)but increasing numbers of subsequent reports of successful vaginal delivery indicate that this is not necessary, or necessarily safer. There is no consensus regarding mode and timing of delivery for EDS in pregnancy.The frequent prevalence of cardiac involvement and the subtype will guide the anaesthetic and obstetric birth plan.It is not known whether there is a higher incidence of dural tap during epidural insertion or local anaesthetic resistance(2).
Conclusion:Management of Ehlers Danlos syndrome is challenging but with multispecialty level of care and expertise safer maternal and perinatal outcome is possible in these women who wish to conceive.
1.Taylor DJ,Wilcox I. Ehlers Danlos syndrome during pregnancy. A case report and review of literature.Obstetrical & Gynaecological Surv.1981;36(6):277-81.
2.Glynn JC,Yentis SM. Epidural analagesia in a parturient with classic type Ehlers-Danlos syndrome.Int J Obstet Anaesth 2005;14:78-9.