Epidural Anesthesia for Cesarean Section in a Pregnant Woman with Marfan Syndrome and Dural Ectasia.

Franco Pepe,Agata Privitera,Elisabetta Sanalitro,Francesco De Luca,Puccio Scarpinati,Mariagrazia Stracquadanio

doi:10.1155/2017/2126310

Franco Pepe, Agata Privitera + Show 4 more

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https://doi.org/10.1155/2017/2126310

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Abstract

Marfan syndrome (MFS) is a genetic disorder of connective tissue, characterized by variable clinical features and multisystem complications. The anesthetic management during delivery is debated. Regional anesthesia has been used with success during cesarean delivery, but in some MFS patients there is a probability of erratic and inadequate spread of intrathecal local anesthetics as a result of dural ectasia. In these cases, epidural anesthesia may be a particularly useful technique during cesarean delivery because it allows an adequate spread and action of local anesthetic with a controlled onset of anesthesia, analgesia, and sympathetic block and a low risk of perioperative complications. We report the perioperative management of a patient with MFS and dural ectasia who successfully underwent cesarean section using epidural technique anesthesia. The previous pregnancy of this woman ended with cesarean section with a failed spinal anesthesia that was converted to general anesthesia due to unknown dural ectasia at that time.

Highlights

Marfan syndrome (MFS) is an autosomal dominant hereditary disorder of connective tissue; its incidence is estimated to be around 1 : 5.000, with no differences in gender or ethnic background
Some manifestations are evident since childhood, while others were at a later date, such as the lumbosacral dural ectasia; the main cause of morbidity and mortality is related to aortic dilation and acute aortic dissection
The purpose of this study is to present the case of a MFS pregnant woman at term with an extensive dural ectasia who had a successful cesarean section with epidural anesthesia during her second pregnancy

Summary

Introduction

Marfan syndrome (MFS) is an autosomal dominant hereditary disorder of connective tissue; its incidence is estimated to be around 1 : 5.000, with no differences in gender or ethnic background. Some manifestations are evident since childhood (such as ectopia lentis), while others were at a later date, such as the lumbosacral dural ectasia; the main cause of morbidity and mortality is related to aortic dilation and acute aortic dissection. The disease is not associated with a reduction in fertility; it is common to find a pregnant woman with the syndrome In such a case, it would be appropriate to have an accurate clinical evaluation before pregnancy, an echocardiography, to assess the size of the aortic root: a diameter greater than 40 mm puts the patient at risk of its rupture. The purpose of this study is to present the case of a MFS pregnant woman at term with an extensive dural ectasia who had a successful cesarean section with epidural anesthesia during her second pregnancy

Case Report

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