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Anesthetic Management of Cesarean Section in a Rare Variant of Ehlers- Danlos Syndrome
Abstract Number: SUN-46
Abstract Type: Case Report/Case Series
Ehlers-Danlos Syndrome is a clinically and genetically heterogeneous group of inherited connective tissue disorders. It is characterized mainly by skin,vascular and tissue fragility and joint hypermobility but clinical presentations vary. Six major subtypes have been described with several additional, rare EDS variants. We present the first report describing management of cesarean section in a parturient with a rare variant of EDS type VI (kyphoscoliotic).
A 25 yo G1,PO with EDS was scheduled for elective cesarean section at 37 wks. PMH included congenital hip dysplasia requiring hip replacement, repair of pectus excavatum, and multiple spine surgeries including Harrington rod placement for severe kyphoscoliosis. She was first sent for genetic testing for suspected EDS during this pregnancy. Testing revealed a rare variant in the FKB14 gene, which follows an autosomal recessive inheritance pattern. This variant is associated with kyphoscoliosis, myopathy and hearing loss and a variable constellation of signs due to connective tissue impairment affecting ocular, musculocutaneous, skeletal and cardiovascular systems.
Physical exam was significant for soft,stretchy skin,hyperextendible fingers and scoliosis. Echocardiogram showed good LV function with mild pulmonary HTN. PFTs were normal. Imaging of head, neck, hepatic and splenic arteries was done to rule out aneurysms, but the Harrington rods precluded imaging of the aorta.
Because of limited hip mobility after surgery, as well as the risks of uterine or vascular rupture, she was not considered a candidate for vaginal delivery. Regional anesthesia was ruled out due to her extensive back surgeries and persistent scoliosis.
After preoxygenation and induction with propofol,ketamine,lidocaine and succinylcholine, an atraumatic rapid sequence intubation was successful. A healthy boy, APGARS 8/9 was delivered. Anesthesia was maintained with sevoflurane, fentanyl, midazolam. Surgery was uneventful, EBL 700 ml. The patient was extubated and had a smooth post-operative course.
Literature regarding obstetric anesthetic management of EDS is mostly in the form of case reports. Though many symptoms overlap, clinical presentation and management varies depending on subtype. In the rare subtype seen in our patient, considerations included: preoperative cardiac workup to exclude valvular and conduction defects; pulmonary testing to rule out dysfunction due to scoliosis; hypermobile joints necessitating careful intubation to avoid atlanto-axial subluxation or TMJ dislocation; possible traumatic intubation due to friable mucosa; careful positioning of arms to avoid hyperextension; padding of extremities to avoid tissue damage; low ETT cuff inflation pressures; low tidal volumes to avoid pneumothorax and preparation for potential hemorrhage. Had regional been considered, there may be increased risk of epidural hematoma or PDPH.
J Obst Anaesth Crit Care 2014;4:35-37