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To do or not to do? Neuraxial analgesia in Hereditary hemorrhagic telangiectasias
Abstract Number: 238
Abstract Type: Case Report/Case Series
Hereditary Hemorrhagic telangiectasia (HHT) or Osler-Weber -Rendu syndrome is a common genetic condition that affects 1:5-8000 Caucasians. It presents as recurrent episodes of epistaxis and some muco-cutaneous lesions. The triad of mucocutaneous telangiectasias and epistaxis, visceral arterio venous malformations (AVMs) and a diagnosis in a first degree relative confirms the diagnosis of HHT. The purpose of this case is to highlight the risks from HHT and its implications to an obstetric anesthesiologist.
Case Summary: A 36 year old G 1 P 0 parturient at 39 weeks presented to labor and delivery with spontaneous rupture of membranes. During anesthesia evaluation the patient mentioned she usually experienced multiple episodes of epistaxis. She also had a pulmonary AVM. A cerebral MRI obtained earlier in the pregnancy did not reveal any cerebral AVMs and a decision had been made by the obstetrician to let the patient labor. Information regarding the size of the pulmonary AVM was unavailable and the patient denied any episodes of hemoptysis or undue shortness of breath. There had been no work up to evaluate for spinal AVMs.
The patient expressed a strong desire to receive an epidural analgesia and was becoming progressively uncomfortable. After a thorough discussion of risks of neuraxial procedures a decision was made to obtain an urgent MRI. An urgent MRI of thoracic and lumbar spine was obtained without using gadolinium. The radiologist could not confirm the absence of an AVM without contrast but concluded there were no abnormalities.
With this information the anesthesia team placed a lumbar epidural catheter for labor analgesia. The placement was uneventful except for the epistaxis that coincided with it. The epidural catheter provided satisfactory analgesia. The plan was to avoid a prolonged or strenuous second stage as there were concerns of the effect of valsalva maneuvers on the pulmonary AVM.Despite augmentation with oxytocin, there was an arrest of cervical dilatation and a cesarean delivery was performed. The epidural provided satisfactory anesthesia and there were no complications.
Being unaware of the diagnosis of HHT is catastrophic. There is risk of cerebral hemorrhage,hemoptysis during second stage and true risk of spinal cord infarct with regional anesthesia. General anesthesia in a parturient carries a higher risk of the risk of hemoptysis.In conclusion the 1-2% risk of a co-exispinal AVM necenecessitates imaging before epidural