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Anesthetic management of a parturient with Ehlers Danlos syndrome type IV

[L'anesthésie d'une parturiente atteinte du syndrome d'Ehlers Danlos de type IV]

From the Department of Anesthesiology, The Ottawa Hospital, Ottawa, Ontario, Canada.

Address correspondence to: Dr.Ola P. Rosaeg, Department of Anesthesiology, B3, The Ottawa Hospital – Civic Campus, 1053 Carling Avenue, Ottawa, Ontario K1Y 4E9, Canada. Phone 613-761-4169; Fax: 613-761-5209; E-mail: norse{at}cyberus.ca

Purpose: To describe the anesthetic management of a parturient with Ehlers Danlos syndrome (EDS) type IV.

Clinical features: A 29-yr-old pregnant woman with EDS type IV was seen in the Obstetric Anesthesia Pre-assessment Clinic at 30 weeks gestation. She had a history of vertebral artery dissection, resulting in a transient neurological deficit at 22 yr of age. She had a normal vaginal delivery with continuous epidural analgesia for the delivery of her first child at 27 yr of age, before the diagnosis of EDS was made. Recent fibroblast culture demonstrated the production of abnormal procollagen type III, which is pathognomonic for EDS type IV. The patient and obstetrician preferred a repeat vaginal birth with instrumental delivery in the second stage. Analgesia for labour and delivery was provided with a continuous epidural infusion of ropivacaine and fentanyl. She delivered a healthy female infant with the use of outlet forceps, without complications.

Conclusion: A pre-delivery, multidisciplinary, individualized management plan is required in patients with EDS, a rare disease with variable clinical features. In the case described, continuous epidural analgesia was effective and associated with excellent maternal and fetal outcomes.