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ESRA19-0304 When regional anesthesia isn’t an option – management of a pregnant patient with marfan syndrome
  1. R Pato1 and
  2. J Figueiredo2
  1. 1Anesthesiology Department of Hospitalar Universitary Center of Central Lisbon, Lisbon, Portugal
  2. 2Centro Hospitalar Universitário de Lisboa Central, Anesthesiology Department of Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal


Background and aims Marfan syndrome (MFS) is a genetic disorder caused by mutation in the FBN-1 is associated with developing structure and flexibility of the connective tissue which is characterized by cardiovascular, ocular and skeletal complications and features. The most common phenotypes involve aortic aneurisms, ectopia lens and severe scoliosis. The anaesthetic management for labor in parturients with MFS is a true challenge, not only for the cardiovascular risk but also because of difficulties associated with neuraxial approach, narrowing the security of the fetus.

Methods Female, 27 years old, proposed to caesarean section at 38 weeks of gestation. ASA II diagnosed with MFS in childhood. As surgical background, description of spinal instrumentation for correction of severe scoliosis from D4 till L3 at the age of 16. At physical exam, stands out surgical longitudinal scar along thoraco-lumbar spine. Physical examination revealed normal cardiopulmonary function and absence of difficult airway assessment predictors.

Results Considering orthopedist advisement of difficulties in neuraxial approach in consequence of spine corrective surgery, it was decided to apply general anaesthesia with orotracheal intubation after fast induction with succinylcholine and propofol, maintained with sevoflurane and rocuronium. The male newborn was extracted 5 minutes patient induction, with an Apgar Index of 9/10/10. There were no intraoperative complications with the patient and newborn. Postoperative pain was controlled with intravenous analgesia protocol.

Conclusions Although regional anaesthesia is the gold standard for caesarean section, parturients with MFS should have a careful anaesthetic plan, considering cardiovascular and skeletal abnormalities that could be a relative contraindication for neuraxial blockage.

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