There are several reports regarding the anesthetic management of cesarean deliveries for patients with Marfan syndrome,1–3 and hemodynamic stability during the perioperative period is a major concern because of coexisting cardiovascular abnormalities. Lumbosacral dural ectasia is a clinical sign frequently seen in patients with Marfan syndrome and is one of the systemic features listed in the diagnostic criteria for Marfan syndrome.4,5 Although neuraxial anesthesia is often used in peripartum anesthetic management, few reports refer to the effects of dural ectasia on the spread and the duration of regional anesthesia.6–8 Herein, we present a case of a patient with Marfan syndrome and dural ectasia undergoing cesarean delivery under combined spinal–epidural anesthesia (CSEA), and in whom an unexpected clinical course probably due to dural ectasia occurred.
The patient’s permission was obtained to publish this case report.
A 32-year-old (gravida 1, para 1) woman was referred to our hospital at 27 weeks’ gestation. She had the typical body habitus of a patient with Marfan syndrome (height, 177 cm; weight, 59 kg; and nonpregnant state weight, 48 kg) as well as other clinical signs including a funnel chest, scoliosis, and bilateral thumb hypermobility. A transthoracic echocardiogram showed a dilated aortic root (39 mm) without aortic valvular regurgitation and mitral valve prolapse with mild regurgitation. Despite the patient’s many typical physical findings of Marfan syndrome, a definitive diagnosis by genetic testing had not been previously established. Four years before this admission, she had undergone cesarean delivery in another hospital. According to the previous anesthesia record, spinal anesthesia was maintained with 2.2 mL dibucaine, and the entire anesthetic management was uneventful.
Because progressive dilation of the aortic root was not observed and after discussions with obstetricians and cardiologists, elective cesarean delivery was scheduled to occur at 38 weeks’ gestation in accordance with our normal institutional practice. CSEA rather than general anesthesia was chosen to avoid hypertension during the perioperative period, especially during anesthetic induction, and to provide adequate postoperative pain control by epidural analgesia.
Under standard noninvasive monitoring and with the patient in the right lateral position, an 18-gauge Tuohy needle was inserted at the T12-L1 interspace via a paramedian approach, and an epidural catheter (Perifix®;B Braun Aesculap, Tokyo, Japan) was inserted. There was neither dural puncture nor complaint of paresthesia. Sequentially, a 25-gauge Quincke spinal needle was inserted at the L3-4 interspace via a median approach, and hyperbaric 0.5% bupivacaine 11 mg and fentanyl 10 µg were administered after confirmation of free-flowing cerebrospinal fluid (CSF). For these techniques, the interspaces were estimated using a landmark approach. Because the bilateral sensory block level assessed by a pinprick sensation was still below the level of L5 after 5 minutes with the patient in the head-down position, 10 mL normal saline was injected via the epidural catheter. Because the anesthetic level was still inadequate after the injection of normal saline, 10 mL of 2% lidocaine was administered twice epidurally to obtain a higher sensory block level. After a complete sensory block of the T11 level was confirmed by pinprick, the surgery was commenced. During the surgery, the patient was hemodynamically stable, and she received only 100 µg IV fentanyl after delivery and 4 mg ephedrine IV for the subsequent transient hypotension. At the end of the surgery, the sensory block was below the T6 level, and we started continuous epidural analgesia with 0.2% ropivacaine at 4 mL/h.
Approximately 7 hours after the spinal anesthetic had been injected, a sensory block below the T12 level remained along with a motor block below the L5 level only on the right side. The epidural catheter was removed for early ambulation during the next afternoon. Five hours after removal of the catheter and 32 hours after the spinal anesthesia, right-sided hypesthesia below the S1 level remained. In addition, she complained of typical symptoms of a post–lumbar puncture headache including neck pain, tinnitus, and imbalance after removal of the epidural catheter. These symptoms lasted for a week, and despite the use of nonsteroidal anti-inflammatory drugs and caffeine as well as hydration, she was not able to begin ambulating until postoperative day 8, and discharge was delayed until postoperative day 11.
After discharge, she underwent a magnetic resonance imaging (MRI) scan that showed lumbosacral dural ectasia consistent with that associated with Marfan Syndrome (Fig. 1).
Our patient experienced inadequate spinal anesthesia and severe postdural puncture headache (PDPH), despite having previously undergone uneventful spinal anesthesia. We suggest that these complications were probably due to the presence of dural ectasia.
Dural ectasia in the lumbosacral spine is one of the systemic features in Marfan syndrome occurring in 63% to 92% of patients.4,9 Dural ectasia is thought to be caused by CSF pulsations, because the dura mater is fragile due to alterations in the connective tissue in patients with this disease. Subsequent dural sac bulging can cause scalloping of the vertebral bodies. When additional bulging of the dural sac protrudes through the neural foramina, radicular cysts, called lateral meningoceles, may develop. Excessive ectasia may compress the surrounding structures and result in radicular and urinary disturbances.10
There are only a few reports describing CSEA for cesarean deliveries in patients with Marfan syndrome, and which anesthetic technique is most suitable for these parturients remains a matter of debate.1–3 We propose that the unique lumbosacral anatomy in Marfan syndrome influences the effect and duration of the neuraxial anesthesia in several ways.
First, the spread of intrathecally administered local anesthetics may be limited because the capacity of the lumbosacral subarachnoid space and the corresponding CSF volume are large.11 Lacassie et al.6 reported 2 cases of inadequate continuous spinal anesthesia in parturients with Marfan syndrome, and Baghirzada et al.7 described 2 patients with Marfan syndrome with differing severities of dural ectasia undergoing cesarean deliveries under CSEA. In these latter 2 patients, the spread of spinal anesthetics was quite different between the 2 cases due to their varying degrees of dural ectasia. In our patient, despite using intrathecal hyperbaric bupivacaine with the operating table tilted so that the head of the patient was lowered and epidural saline administration, her sensory block level was still not adequate for the surgery.
Second, the duration of the administered local anesthetic effect might be prolonged because most of the anesthetic remains in the dilated subarachnoid space, especially in the lateral meningoceles. This could be one reason why our patient had hypesthesia below the S1 level for 32 hours after the spinal anesthesia. Because the hypesthesia was still seen 5 hours after the removal of the epidural catheter, we do not believe that it was due to the epidural local anesthetic. Although the duration of local anesthetics is related to their lipid solubility, protein binding, and vascular uptake, stagnation of the CSF circulation might have prevented the anesthetic metabolism.
Third, the histologically fragile dura may contribute to an increased risk for severe and prolonged PDPH. According to Davenport et al.,12 CSF leakage can occur from even minor trauma, unrecognized trauma, or both at the thoracic spine level without dural ectasia. They considered that continuously pulsating CSF was a major etiology of dural injury. Furthermore, it is speculated that the ectatic dura is more vulnerable and difficult to repair once injured. Actually, the severity of PDPH observed in our patient was classified as severe according to Lybecker et al.,13 and the duration was >1 week.
In patients with Marfan syndrome, dural ectasia often starts in their teens and worsens progressively with age.4 Therefore, previously successful spinal anesthesia does not always ensure the success of future anesthesia. This might explain why our patient had different responses to spinal anesthesia in her 2 separate cesarean deliveries.
Based on the above, in planning neuraxial anesthesia for a patient with Marfan syndrome, the possible consequences of lumbosacral dural ectasia should be considered. The severity of dural ectasia can be related to the spread of spinal anesthetics and be assessed by an MRI scan. However, in view of the fact that confirming the severity of dural ectasia does not guarantee the success of neuraxial anesthesia and that it may not be practical to perform an MRI scan before each surgery, when anesthetizing patients with Marfan syndrome it is reasonable to assume the presence of dural ectasia, which is frequently seen in these patients. Although epidural anesthesia appeared to work as predicted with the presence of dural ectasia, the dura is structurally fragile and prone to injury especially at the lower lumbar levels. Therefore, to avoid accidental dural puncture, it might be appropriate to perform epidural anesthesia at a higher lumbar or lower thoracic level rather than via the lower lumbar spine.
In summary, we used CSEA for a cesarean delivery in a patient with Marfan syndrome, who experienced an unexpected clinical course probably due to dural ectasia. Considering the prevalence of dural ectasia in patients with Marfan syndrome, its possible consequences should be considered when planning neuraxial anesthesia.
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