1 Department of Anaesthesiology and 2 Department of Obstetrics and Gynaecology, Pontificia Universidad Católica de Chile, Marcoleta 367, Santiago 833-0024, Chile. 3 Department of Anaesthesiology and 4 Department of Neuroradiology, Duke University Medical Center, Durham, North Carolina 27710, USA. 5 Department of Anaesthesia, Glasgow Royal Infirmary, 84 Castle Street, Glasgow G4 0SF, UK
* Corresponding author. E-mail: habib001{at}mc.duke.edu
Accepted for publication November 24, 2004.
![]() |
Abstract |
---|
![]() ![]() ![]() ![]() ![]() ![]() ![]() |
---|
Keywords: anaesthesia, obstetric ; anaesthetic techniques, subarachnoid ; complications, dural ectasia ; complications, Marfan's syndrome ; surgery, Caesarean section
![]() |
Introduction |
---|
![]() ![]() ![]() ![]() ![]() ![]() ![]() |
---|
![]() |
Case report 1 |
---|
![]() ![]() ![]() ![]() ![]() ![]() ![]() |
---|
On physical examination she had evident Marfan's syndrome body habitus: height 180 cm, weight 80 kg and arachnodactyly. Upon arrival at the operating theatre, arterial pressure was 140/80 mm Hg and heart rate was 60 beats min1. Airway examination was unremarkable. Her back revealed a scar extending from T1 to L3 from a previous kyphoscoliosis repair; she denied lumbar pain.
Continuous spinal anaesthesia was planned. Under standard non-invasive monitoring (ECG, arterial blood pressure and oxygen saturation), a 22 gauge spinal catheter over the needle (Spinocath; B. Braun, Melsungen, Germany) was inserted in the left lateral position at L4L5 interspace under sterile conditions (4 cm in the intrathecal space) by a midline approach. Aspiration of cerebrospinal fluid (CSF) was confirmed at all times. With the patient in the left lateral position, an initial bolus dose of plain bupivacaine 0.5%, 9 mg and fentanyl 20 µg was given, followed by plain bupivacaine 6 mg because of a bilateral low sensory level (T12). Twenty-five minutes later, after moving the patient from side to side in the supine wedged position with the knees flexed by a pillow, a third bolus dose of plain bupivacaine 6 mg was given. Despite the incremental dosing up to 21 mg of bupivacaine, the highest sensory level blocked was T10, as assessed by sharp pinprick. General anaesthesia was therefore induced using remifentanil 0.10.2 µg kg1 min1, propofol 2 mg kg1, succinylcholine and isoflurane in a mixture of oxygennitrous oxide. A male infant weighting 2120 g was delivered, with Apgar scores of 5, 7 and 9 at 1, 5 and 10 min respectively. Surgery lasted 50 min and the trachea was extubated at the end of the procedure, with excellent haemodynamic stability throughout the case, as previously described with a similar technique.2 3 On recovery, she had a sensory block at T12. She had no post dural puncture headache. A computed tomographic (CT) scan of her spine revealed an ectasic thecal sac (Fig. 1).
|
![]() |
Case report 2 |
---|
![]() ![]() ![]() ![]() ![]() ![]() ![]() |
---|
Continuous spinal anaesthesia technique was planned for the elective procedure. Preoperatively, left radial arterial and right internal jugular venous catheters were placed. An interventional neuroradiologist placed a spinal catheter at L5S1 in the left lateral decubitus position, using CT fluoroscopic guidance with an 18 G pencil-point epidural needle (Special Sprotte®; Pajunk, Geisingen, Germany). The catheter was left 5 cm in the subarachnoid space. The procedure was uneventful and the images showed dural ectasia and severe kyphoscoliosis (Fig. 2). Plain bupivacaine (0.5%) 10 mg was administered incrementally via the spinal catheter with the patient in the wedged supine position. A patchy spread was noted by sensory testing using sharp pinprick. A further 7.5 mg of hyperbaric bupivacaine 0.75%, administered over the next 30 min, combined with moving the patient from side to side, led to only marginal improvement. The block remained patchy with a sensory block to T7 on the left and L1 on the right. Aspiration of CSF was confirmed at all times.
|
![]() |
Discussion |
---|
![]() ![]() ![]() ![]() ![]() ![]() ![]() |
---|
Marfan's syndrome is an autosomal dominant disease resulting from a mutation in the fibrillin-1 gene, which is located on chromosome 15. It is a disorder of fibrous connective tissue that shows striking pleiotropism (the control by a single gene of several distinct and seemingly unrelated phenotypic effects)1 and clinical variability that can affect both men and women of any race or ethnic origin. Its incidence is 1:5000 individuals and the clinical diagnosis relies on a combination of major and minor clinical signs.1 4
The cardinal clinical features occur in three systems: skeletal, ocular and cardiovascular. The most common skeletal alterations are increased height, disproportionately long limbs and digits, anterior chest deformity (pectus excavatum), mild to moderate joint laxity, vertebral column deformity (kyphoscoliosis and thoracic lordosis), and a narrow, highly arched palate with crowding of the teeth.5 The ocular changes include myopia, increased axial globe length, corneal flatness and subluxation of the lenses (ectopia lentis).5 Cardiovascular manifestations are most frequently mitral valve prolapse, mitral regurgitation, dilatation of the aortic root and aortic regurgitation, but the major life-threatening cardiovascular complications are aneurysm of the aorta and aortic dissection.5
Widening or ballooning of the lumbosacral dural sac, dural ectasia, can be found in 6392% of patients with Marfan's syndrome.6 7 It is considered one of the major manifestations of the syndrome, together with ectopia lentis and aortic dilatation/dissection.8 It can be associated with back pain, the intensity of which correlates with the dural volume.9 However, a high prevalence of dural ectasia (41%) exists even in patients with Marfan's syndrome without back pain.9
Regional anaesthesia has been used previously with success in patients with this syndrome, both for analgesia during labour10 and Caesarean section.1014 There are no reports, however, describing patchy, erratic and inadequate spread of intrathecal local anaesthetics in patients with this condition. Many factors that affect the extent of spread of spinal anaesthesia have been identified. One of the most important factors influencing block height in patients receiving spinal anaesthesia is the lumbosacral CSF volume, contributing to the variability in the spread of spinal block.15 16 The erratic spread of spinal anaesthesia in both of these cases was most likely the result of dural ectasia and the associated increase in CSF volume. In both cases confirmation of the diagnosis of dural ectasia was done with a CT scan. Another possible explanation for the patchy block was the severe degree of kyphoscoliosis in case 2. However, this seems unlikely since there are multiple reports of successful neuraxial anaesthesia performed on patients with spinal deformities13 1719 and patients with corrected kyphoscoliosis and Harrington rods.13 On the other hand, a caudal direction of intrathecal catheters has been identified as a cause for delayed onset of spinal block and a requirement for more local anaesthetic to establish the block.20 However, this seems unlikely to be the cause of failure since, in both cases, we waited for an adequate period of time, we rolled the patients from side to side trying to achieve an even spread of the local anaesthetics, and administered a relatively large dose of bupivacaine. Similar doses were reported to be effective in achieving at least a T4 surgical block in 90% of patients undergoing Caesarean section with a continuous spinal anaesthesia technique using plain bupivacaine.21 Furthermore, in case 2 the catheter was inserted under CT fluoroscopic guidance and a cranial direction of the catheter was evident. Finally, confirmation of intrathecal position of the catheter in both cases was assessed by frequent aspiration of CSF.
Plain bupivacaine is hypobaric22 and redistributes to non-dependent areas.23 24 The upright position during spinal injection of plain bupivacaine promotes cephalad spread of the block.23 Positional changes also play a major role in promoting cephalad redistribution of plain bupivacaine, probably due to CSF dynamics associated with caval compression and epidural venous engorgement.24 25 In the cases presented, the spinal injections were made in the lateral and supine wedged positions and the patients were turned from side to side. It is unlikely that administration of the spinal anaesthetic in the sitting position would have overcome the diluting effect of CSF resulting from dural ectasia.
A continuous spinal anaesthesia was chosen instead of a single shot subarachnoid block in both cases, with the aim of incrementally inducing a surgical block, thus avoiding excessive haemodynamic instability. Furthermore, continuous spinal anaesthesia has been shown to be highly predictable in terms of level and quality of anaesthesia.21 The presence of the catheter would also provide excellent postoperative analgesia. A disadvantage of this technique, however, is a higher risk of postdural puncture headache. Since microcatheters are not currently FDA-approved in the USA, we attempted to decrease the risk of headache by using the Special 18 G Sprotte needle with a 20 G catheter in patient 2, since this needle might be associated with a lower risk of postdural puncture headache compared with the Tuohy needle.26
We did not use an epidural technique because of concern about epidural space fibrosis in case 1 and concern about difficult insertion and position of the catheter in the presence of severe kyphoscoliosis in case two. In both cases we were reluctant to further increase the dose of bupivacaine for fear of potential neurological injury. While the incidence of transient radicular irritation (TRI) is strikingly more common with intrathecal lidocaine compared with bupivacaine (plain or hyperbaric),27 there are cases where the only aetiology for the TRI was bupivacaine.28 29
Anaesthetic management in patients with Marfan's syndrome with significant aortic root dilation or dissection includes ß-blocker therapy in order to minimize sheer stress and increase in aortic wall tension,19 and maintenance of haemodynamic stability by careful titration of regional or general anaesthesia. Despite failure of the regional technique in both cases, haemodynamic stability was maintained and the general anaesthetic was uneventful. As an alternative to inducing general anaesthesia after failure of the regional technique, we could have attempted to supplement the patchy block using local anaesthetic infiltration. However, we felt that intraoperative pain, with the associated tachycardia and hypertension, was undesirable in our patients with cardiovascular compromise. Our general anaesthetic technique was effective in blunting these responses.
In summary, we present two cases of Caesarean section in patients with Marfan's syndrome in whom continuous spinal anaesthesia failed to provide adequate anaesthetic block, most likely as a result of dural ectasia.
![]() |
Acknowledgments |
---|
![]() |
References |
---|
![]() ![]() ![]() ![]() ![]() ![]() ![]() |
---|
2 Bedard JM, Richardson MG, Wissler RN. General anesthesia with remifentanil for Cesarean section in a parturient with an acoustic neuroma. Can J Anaesth 1999; 46: 57680[Abstract]
3 Mertens E, Saldien V, Coppejans H, Bettens K, Vercauteren M. Target controlled infusion of remifentanil and propofol for cesarean section in a patient with multivalvular disease and severe pulmonary hypertension. Acta Anaesthesiol Belg 2001; 52: 2079[Medline]
4 Beighton P, de Paepe A, Danks D, et al. International nosology of heritable disorders of connective tissue, Berlin, 1986. Am J Med Genet 1988; 29: 58194[ISI][Medline]
5 McKusick VA. Marfan syndrome; MFS. Online Mendelian Inheritance in Man, OMIM. MIM No. 154700. Baltimore (MD): Johns Hopkins University, 2003
6 Pyeritz RE, Fishman EK, Bernhardt BA, Siegelman SS. Dural ectasia is a common feature of the Marfan syndrome. Am J Hum Genet 1988; 43: 72632[ISI][Medline]
7 Fattori R, Nienaber CA, Descovich B, et al. Importance of dural ectasia in phenotypic assessment of Marfan's syndrome. Lancet 1999; 354: 9103[CrossRef][ISI][Medline]
8 Beighton P, Sujansky E, Patzak B, Portele KA. Genetic skeletal dysplasias in the Museum of Pathological Anatomy, Vienna. Am J Med Genet 1993; 47: 8437[ISI][Medline]
9 Ahn NU, Sponseller PD, Ahn UM, Nallamshetty L, Kuszyk BS, Zinreich SJ. Dural ectasia is associated with back pain in Marfan syndrome. Spine 2000; 25: 15628[CrossRef][ISI][Medline]
10 Handa F, Ohnishi Y, Takauchi Y, Kuro M. [Anaesthetic management of parturients with Marfan syndrome]. Masui 2001; 50: 399404[Medline]
11 Brar HB. Anaesthetic management of a caesarean section in a patient with Marfan's syndrome and aortic dissection. Anaesth Intensive Care 2001; 29: 6770[ISI][Medline]
12 Ben Letaifa D, Slama A, Methamem M, Ben Jazia K, Jegham H. [Anesthesia for Caesarean section in a Marfan patient with complicated aortic dissection]. Ann Fr Anesth Reanim 2002; 21: 6725[ISI][Medline]
13 Kardash K, King BW, Datta S. Spinal anaesthesia for caesarean section after Harrington instrumentation. Can J Anaesth 1993; 40: 6679[Abstract]
14 Pascoe HF, Jennings GS, Marx GF. Successful spinal anesthesia after inadequate epidural block in a parturient with prior surgical correction of scoliosis. Reg Anesth 1993; 18: 1912[ISI][Medline]
15 Carpenter RL, Hogan QH, Liu SS, Crane B, Moore J. Lumbosacral cerebrospinal fluid volume is the primary determinant of sensory block extent and duration during spinal anesthesia. Anesthesiology 1998; 89: 249[ISI][Medline]
16 Higuchi H, Hirata J, Adachi Y, Kazama T. Influence of lumbosacral cerebrospinal fluid density, velocity, and volume on extent and duration of plain bupivacaine spinal anesthesia. Anesthesiology 2004; 100: 10614[CrossRef][ISI][Medline]
17 Goodarzi M. The advantages of intrathecal opioids for spinal fusion in children. Paediatr Anaesth 1998; 8: 1314[CrossRef][ISI][Medline]
18 Shaw BA, Watson TC, Merzel DI, Gerardi JA, Birek A. The safety of continuous epidural infusion for postoperative analgesia in pediatric spine surgery. J Pediatr Orthop 1996; 16: 3747[ISI][Medline]
19 Silva TS, Popat MT. Combined spinal-epidural anesthesia in parturient with Harrington rods. Reg Anesth 1994; 19: 360
20 Standl T, Beck H. Influence of the subarachnoid position of microcatheters on onset of analgesia and dose of plain bupivacaine 0.5% in continuous spinal anesthesia. Reg Anesth 1994; 19: 2316[ISI][Medline]
21 Kestin IG, Madden AP, Mulvein JT, Goodman NW. Comparison of incremental spinal anesthesia using a 32-gauge catheter with extradural anaesthesia for elective Caesarean section. Br J Anaesth 1991; 66: 2326[Abstract]
22 Richardson MG, Wissler RN. Densities of dextrose-free intrathecal local anesthetics, opioids, and combinations measured at 37 degrees C. Anesth Analg 1997; 84: 959[Abstract]
23 Richardson MG, Thakur R, Abramowicz JS, Wissler RN. Maternal posture influences the extent of sensory block produced by intrathecal dextrose-free bupivacaine with fentanyl for labor analgesia. Anesth Analg 1996; 83: 122933[Abstract]
24 Russell IF. Spinal anesthesia for cesarean delivery with dilute solutions of plain bupivacaine: the relationship between infused volume and spread. Reg Anesth 1991; 16: 1306[ISI][Medline]
25 Russell IF. Effect of posture during the induction of subarachnoid analgesia for caesarean section. Right v. left lateral. Br J Anaesth 1987; 59: 3426[Abstract]
26 Morley-Forster PK, Angle PJ, Littleford J, Halpern SH, Currin M. Post-dural puncture headache in a parturienta comparison of the Special Sprotte vs Tuohy needle. Anesthesiology 2001; 95: A1053
27 Pollock JE, Neal JM, Stephenson CA, Wiley CE. Prospective study of the incidence of transient radicular irritation in patients undergoing spinal anesthesia. Anesthesiology 1996; 84: 13617[ISI][Medline]
28 Tarkkila P, Huhtala J, Tuominen M, Lindgren L. Transient radicular irritation after bupivacaine spinal anesthesia. Reg Anesth 1996; 21: 269
29 Corbey MP, Bach AB. Transient radicular irritation (TRI) after spinal anaesthesia in day-care surgery. Acta Anaesthesiol Scand 1998; 42: 4259[ISI][Medline]