Regional anesthesia in Marfan syndrome, not all dural ectasias are the same: a report of two cases

Abstract

Background The anesthetic management of women with Marfan syndrome and dural ectasia undergoing Cesarean delivery remains controversial. We present two cases of patients with Marfan syndrome and dural ectasia where neuraxial anesthesia was used successfully. Case 1 A 31-yr-old G1P0 female with Marfan syndrome presented for elective Cesarean delivery at 35 3/7 weeks' gestation. The magnetic resonance imaging of her spine revealed significant dural ectasia in the lumbosacral area with a mean lumbar dural sac area of 4.71 cm 2 . Intrathecal administration of 0.75% hyperbaric bupivacaine 9 mg produced only limited perineal analgesia. The epidural catheter was titrated, and 0.5% bupivacaine 150 mg were required to achieve a T4 sensory level. Case 2 A 34-yr-old G1P0 female with Marfan syndrome presented for elective Cesarean delivery at 37 weeks' gestation. The intrathecal administration of 0.75% hyperbaric bupivacaine 13.5 mg produced a T5 sensory level. Magnetic resonance imaging of her spine prior to discharge confirmed the diagnosis of moderate dural ectasia with a mean lumbar dural sac area of 3.61 cm 2 . Discussion The two patients described in this report responded differently to spinal anesthesia, most likely based on the severity of their dural ectasia. Although preoperative magnetic resonance imaging may help to identify patients at risk for a failed spinal, we suggest considering a combined spinal-epidural technique in cases of dural ectasia. RésuméContexte La prise en charge anesthe´sique de femmes ayant un syndrome de Marfan et une ectasie de la dure-me`re au cours d'un accouchement par ce´sarienne reste un sujet controverse´. Nous pre´sentons le cas de deux patientes ayant un syndrome de Marfan et une ectasie de la dure-me`re chez qui une anesthe´sie neuraxiale a e´te´re´alise´e avec succe`s. Cas n8 1 Une femme de 31 ans, G1P0, ayant un syndrome de Marfan a e´te´admise pour accouchement par ce´sarienne programme´a`35 semaines et 3 jours de grossesse. L'imagerie par re´sonance magne´tique de la colonne verte´brale a re´ve´le´une ectasie significative de la dure-me`re dans la re´gion lombosacre´e avec une surface moyenne du sac dural lombaire de 4,71 cm 2 . L'administration intrathe´cale de 9 mg de bupivacaı¨ne hyperbare a`0,75 % n'a procure´qu'une analge´sie pe´rine´ale limite´e. La dose du cathe´ter pe´ridural a e´te´titre´e et 150 mg de bupivacaı¨ne 0,5 % ont e´te´ne´cessaires pour atteindre le niveau sensitif D4. Cas n8 2 Une femme de 34 ans, G1P0, ayant un syndrome de Marfan a e´te´admise pour accouchement par ce´sarienne programme´a`37 semaines de grossesse. L'administration intrathe´cale de 13,5 mg de bupivacaı¨ne Author contributions Leyla Baghirzada and Jose C.A. Carvalho cared for patients, reviewed the literature, and wrote the manuscript. Jose C.A. Carvalho and Timo Krings revised the manuscript, and Leyla Baghirzada submitted the final version of the manuscript. Timo Krings interpreted the magnetic resonance imagings for both patients.

Dural ectasia

“…[6] As there is dilation of the dural sac in dural ectasia, dural puncture may result in excessive leakage of CSF leading to increased chances of post-dural puncture head-ache (PDPH). [7] We observed an early onset of head-ache after two successful dural punctures, which was relieved within 24 h. Also, dural ectasia is a relative contraindication of epidural anaesthesia because of the fear of puncturing of dilated dural sac. [89]…”

Anesthetic management of a patient with Marfan syndrome and severe aortic root dilatation undergoing cholecystectomy and partial hepatic resection

“…[1] The goals of the anesthetic management of Marfan syndrome patients are thus first to decrease aortic pressure and to monitor the signs with preparations for possible aortic dissection. [34] Our patient presented late with Marfan syndrome along with emergent condition (carcinoma gall bladder). Her medical management included reduction of arterial pressure with angiotensinogen receptor blocker (ramipril), beta-blockers and diuretics.…”

“…[34] However, scarcity of reports of major surgery in patients with Marfan syndrome stimulated us to report a case of cholecystectomy with liver resection in a patient with Marfan syndrome with severe aortic root dilation. The patient gave us a written consent in local language for publication of this information.…”

Spinal meningeal cyst in a child with Marfan syndrome: A potential cause for apparent dural puncture during caudal epidural block

“…It is a multisystem disorder with main manifestations in cardiovascular, skeletal, and ocular systems . Neuraxial anesthesia in patients with Marfan syndrome is so far not contraindicated, however, can be complicated in patients with dural ectasia . We describe a case of apparent accidental dural puncture during performance of a caudal block in a child with Marfan syndrome.…”