Regional anesthesia in Marfan syndrome, not all dural ectasias are the same: a report of two cases
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- Baghirzada, L., Krings, T. & Carvalho, J.C.A. Can J Anesth/J Can Anesth (2012) 59: 1052. doi:10.1007/s12630-012-9778-5
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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.
A 31-yr-old G1P0 female with Marfan syndrome presented for elective Cesarean delivery at 353/7 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 cm2. 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.
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 cm2.
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.
Anesthésie régionale dans le syndrome de Marfan; toutes les ectasies de la dure-mère ne se ressemblent pas : une étude de deux cas
La prise en charge anesthésique de femmes ayant un syndrome de Marfan et une ectasie de la dure-mère au cours d’un accouchement par césarienne reste un sujet controversé. Nous présentons le cas de deux patientes ayant un syndrome de Marfan et une ectasie de la dure-mère chez qui une anesthésie neuraxiale a été réalisée avec succès.
Cas nº 1
Une femme de 31 ans, G1P0, ayant un syndrome de Marfan a été admise pour accouchement par césarienne programmé à 35 semaines et 3 jours de grossesse. L’imagerie par résonance magnétique de la colonne vertébrale a révélé une ectasie significative de la dure-mère dans la région lombosacrée avec une surface moyenne du sac dural lombaire de 4,71 cm2. L’administration intrathécale de 9 mg de bupivacaïne hyperbare à 0,75 % n’a procuré qu’une analgésie périnéale limitée. La dose du cathéter péridural a été titrée et 150 mg de bupivacaïne 0,5 % ont été nécessaires pour atteindre le niveau sensitif D4.
Cas nº 2
Une femme de 34 ans, G1P0, ayant un syndrome de Marfan a été admise pour accouchement par césarienne programmé à 37 semaines de grossesse. L’administration intrathécale de 13,5 mg de bupivacaïne hyperbare à 0,75 % a permis d’atteindre le niveau sensitif D5. Une IRM de la colonne vertébrale, effectuée avant le congé de la patiente a confirmé le diagnostic d’ectasie modérée de la dure-mère, avec une surface moyenne du sac dural lombaire de 3,61 cm2.
Les deux patientes décrites dans cet article ont répondu différemment à la rachianesthésie, le plus probablement en rapport avec la gravité de leur ectasie durale. Bien que l’imagerie par résonance magnétique préopératoire puisse aider à identifier les patientes à risque d’échec de la rachianesthésie, nous suggérons qu’une technique combinée de rachianesthésie et d’anesthésie péridurale soit envisagée dans les cas d’ectasie de la dure-mère.
Marfan syndrome is an autosomal dominant connective tissue disorder with a reported incidence of one in 3,000-5,000 individuals.1 It is caused by mutation in the fibrillin-1 gene located on chromosome 15.2 The cardinal features occur in the skeletal, ocular, and cardiovascular systems, but other organs can also be affected, including the skin, lungs, and dura.
The most important complication of Marfan syndrome is the progressive dilatation of the aortic root and ascending aorta, leading to aortic valve incompetence and aortic dissection.3 Another major feature in the diagnostic criteria4 is dural ectasia (DE), defined as the widening of the spinal canal, indentation of the vertebral body posteriorly, thinning of the cortex and laminae as a result of longstanding pressure, widening of the neural foraminae, or presence of meningocele.5 The prevalence of DE in patients with Marfan syndrome is estimated to be from 63-92%.6 Although DE can occur at different levels of the lumbar spine, it is most common at the L5-S1 level.7
The anesthetic management of these patients can be challenging, and the literature regarding the management of these cases is scarce. Spinal anesthesia may result in undesirable hemodynamic changes in patients with significant cardiovascular disease and may fail as a result of an increased volume of lumbar cerebrospinal fluid (CSF).8 While epidural anesthesia provides more stable hemodynamics, it may be difficult to perform due to scoliosis, and it has been associated with failure.1,9
We present two cases of patients with Marfan syndrome and associated DE. Both patients presented for elective Cesarean delivery where neuraxial anesthesia was used successfully but performed very differently. Both patients gave written informed consent for publication of this information.
Dural sac dimensions by magnetic resonance imaging at L1-L5 levels
Antero-posterior diameter (cm)
Mediolateral diameter (cm)
Antero-posterior diameter (cm)
Mediolateral diameter (cm)
Anteroposterior diameter (cm) with 95% confidence interval ( )
1.4(1.2 to 1.7)
1.3(1.0 to 1.6)
1.3(0.8 to 1.6)
1.2(0.7 to 1.6)
1.1(0.6 to 1.6)
The remainder of the procedure was uneventful, and the patient delivered a live male infant with Apgar scores 9 and 9 at one and five minutes, respectively. Additional preservative-free morphine 2.5 mg was injected through the epidural catheter for postoperative pain control. The patient was monitored on the labour and delivery floor for 24 hr and then transferred to the ward. On the fifth postoperative day, she underwent surgery for aortic root repair and was discharged home 12 days later.
Our results confirm the successful use of neuraxial anesthesia in Marfan patients with DE; however, our results also suggest that the degree of DE may limit the efficacy of spinal anesthesia and require the use of epidural anesthesia.
The etiology of DE is unclear. The predominant presence of DE at the lowest vertebra supports the theory that the development of ectasia is influenced by hydrostatic pressure on a weakened dura.11 Patients are usually asymptomatic, but some may develop lower back pain, headache, proximal leg pain, weakness and numbness above and below the knee, and genital/rectal pain.7 Dural ectasia has also been reported in patients with neurofibromatosis type 1, Ehler-Danlos syndrome, ankylosing spondylitis, trauma, tumours, and scoliosis.12-14
The severity of DE can be evaluated by computed tomography or MRI using either quantitative (actual dural sac measurements) or qualitative (evaluating the prevalence of different features of DE, i.e., scalloping) criteria. There are no universally accepted quantitative criteria for diagnosing DE, and various methods have been used, including evaluation of dural sac ratio,15 assessment of dural sac diameter at S1 and L4 levels, nerve root sleeve diameter,16 and lumbar pedicle width.17 According to Fattori et al.,5 the degree of DE can be classified as mild, moderate, or severe based on radiologic imaging. The severity of this condition is suggested to be age related, which supports the theory that an ongoing pulsatile pressure of the cerebrospinal fluid on the lumbar and sacral canal causes progressive ballooning of a congenitally defective dural wall.18
Summary of published cases of neuraxial anesthesia for Cesarean delivery in parturients with Marfan syndrome and dural ectasia
Lacassie et al.8 (1st case)
Continuous spinal catheter inserted at L4-5 interspace by midline approach in left lateral position and left at 4 cm in the intrathecal space. Incremental top up of the catheter with 21 mg of 0.5% bupivacaine. Highest sensory block T10. Aspiration of CSF was confirmed at all times.
Intrathecal catheter top up was stopped for fear of potential neurologic injury. Fifty minutes later, the patient had a T12 sensory block. General anesthesia was induced for Cesarean delivery. A CT scan revealed ectatic dural sac. Patient had a history of T1-L3 scoliosis repair.
Lacassie et al.8 (2nd case)
Spinal catheter inserted in left lateral position under CT fluoroscopic guidance at L5-S1 level. The catheter was left at 5 cm in the subarachnoid space. Patchy sensory block to T7 on the left and L1 on the right after 10 mg of incremental top up of 0.5% bupivacaine and 7.5 mg of 0.75% bupivacaine. Aspiration of CSF was confirmed at all times.
General anesthesia was induced due to the patchy block. The images showed dural ectasia and severe kyphoscoliosis. Spinal catheter was used for postoperative analgesia and removed 18 hr after delivery. On day 3, patient complained of most likely a postdural puncture headache but refused the blood patch. The headache resolved on postoperative day 9.
Tan et al.9
Epidural catheter inserted at L3-4 level and failed to produce an adequate surgical block for Cesarean delivery after 15 ml of 2% lidocaine with epinephrine 1:200,000. Subarachnoid block at L3-4 level with 12 mg bupivacaine produced initial bilateral T4 block; however, it was patchy 10 min into the procedure.
General anesthesia was induced due to the patchy block. Postpartum course was complicated with postdural puncture headache. The authors reported constant “loss of resistance” with increasing needle depth. No follow-up imaging reported to confirm suspected diagnosis of dural ectasia.
Kim et al.26
Epidural catheter was inserted at L3-4 interspace in lateral position. A total dose of 27 mL of 2% lidocaine with epinephrine 1:200,000 and 100 μg of fentanyl was injected incrementally to produce a T4 block bilaterally 20 min after injection.
Uneventful Cesarean delivery. Epidural catheter was used for postoperative analgesia for three days.
The presence of DE may explain variability in the volume of cerebrospinal fluid, and as a result, different response to spinal anesthesia. Greater than normal volume of cerebrospinal fluid in the lumbar theca is postulated to restrict the spread of intrathecally injected local anesthetics and cause a “failed” spinal.21 Good correlations have been observed between the volume of lumbar CSF, measured by MRI, and the intrathecal spread of drugs in non-pregnant patients.22,23
Our patients exhibited different degrees of DE, and their dural sac dimensions at various lumbar levels are presented in Table 1. The anteroposterior dural sac diameters were measured perpendicular to the long axis of the dural sac at the midcorpus and lower endplate level of L1 through S1. Reference values were obtained from Oosterhof et al., where the authors measured dural sac dimensions in 44 healthy volunteers.15 Although, the difference in the dosing of spinal bupivacaine in our two cases makes the comparison between the effect difficult, in our view, the reason for a “failed” spinal in Case 1 was the greater severity of the patient’s DE. Even a low dose of hyperbaric bupivacaine is expected to result in a T3-4 sensory block in healthy parturients.24
It is likely that the dimensions of the epidural space in patients with DE are also enlarged along with the dural sac. Despite the dural sac being a soft-tissue structure, it exerts sufficient pressure on the vertebra over time to cause bony changes of the spine.17 Therefore, an increased circumference of the bony canal may result in an enlarged epidural space, requiring a higher volume of local anesthetic to achieve sufficient levels, as was observed in Case 1. Further observations, however, are necessary to confirm this hypothesis.
Although DE is not an absolute contraindication for epidural anesthesia, caution has been advised in its use for patients with Marfan syndrome and associated DE because of the theoretical risk of accidental dural puncture20 and CSF leak; however, when performed carefully by an experienced anesthesiologist, many benefits of epidural anesthesia, including minimal hemodynamic fluctuations and excellent postoperative pain control, can outweigh the potential risk. There has been some suggestion in the literature to perform an epidural in the lateral decubitus position to minimize the chance of accidental dural puncture. In this position, the ectatic dural sac is thought to be less extended in the region of needle insertion.20 We postulated that the lateral position may hinder the performance of the neuraxial technique and elected to perform the procedure with both patients in the sitting position. Although we did not acknowledge abnormal “loss of resistance” due to structural changes in the ligamentum flavum in either patient, this phenomenon has been suggested as a likely cause of accidental dural puncture.9
Patients with Marfan syndrome and associated scoliosis and DE pose many challenges to the anesthesiologist; therefore, we suggest a few details of our technique that could be useful to optimize the neuraxial technique and reduce the risk of complications. This assumption, however, requires confirmation in future observations. A pre-procedural ultrasound assessment of the spine may correctly identify the target interspace, determine the best interspace and insertion point in patients with scoliosis, and determine the depth to the epidural space; thus, it can potentially minimize the risk of a dural puncture. Electrical stimulation of the epidural catheter may be particularly useful in testing the catheter for double-segment CSEs and also in ascertaining the correct positioning of the catheter in patients with scoliosis.25
In summary, we reported two cases of parturients with Marfan syndrome and DE undergoing elective Cesarean delivery. Each patient responded differently to spinal anesthesia, most likely based on the severity of their DE. Although a preoperative MRI may help to identify patients at risk for a failed spinal, we suggest considering a CSE technique in cases of DE.
Conflict of interest
None of the authors has a conflict of interest.