[Management of delivery in patients with Marfan's syndrome presenting aortic dilatation]

Abstract

Objective: We report the anesthesic and obstetrical management of two pregnant patients with Marfan's syndrome. An important dilatation of the root of aorta was established at the beginning of the pregnancy. Based on a review of the literature and our experience, we searched for clues to identify the ideal term and the best mode of delivery, and which type of anesthesia may be the more appropriate in patients with aortic dilatation.

Results: No consensus can be found in the literature as far as anesthesia and obstetric management of these patients is concerned. Pregnancy must be continued as long as possible to ensure adequate fetal growth but fetal extraction should not be delayed if the diameter of the aorta enlarges too much. A diameter of 40 mm is probably the higher limit to accept for vaginal delivery. Beyond, cautious cesarean section would be advisable. In the absence of dural ectasia or a technical problem, neuraxial anesthesia is a good option.

Conclusion: According to the severity of the aortic dilatation and its evolution, specific management, based on good cooperation between obstetricians and anesthesiologists, is the key of a successful and safe childbirth.