<< . .

( 87)

. . >>

Inadvertent dural
puncture at L3“4
120 cm L2“3 0.75% plain bupivacaine 21 ml T3“4 Waugaman, 1986 Transient paresthesia
48 kg with catheter
121 cm L2“3 0.5% plain bupivacaine 12 ml C5 Brimacombe, 1990 Difficulty threading
þ 11 ml saline
73 kg ?L3“4 catheter: i.v. difficulty
with 2nd attempt, but
111 cm L2“3 0.5% plain bupivacaine 5 ml T4 on the left Wardall, 1990
46 kg T6 on the right
119 cm T11“12 2% lidocaine 8 ml T5 Carstoniu, 1992
þ 1:200 000
61 kg
120 cm L3“4 2% lidocaine 13.5 ml T4 Morrow, 1998
þ 1:200 000
64 kg

Modified with permission from Carstoniu, J., Yee, I. & Halpern, S. Epidural anaesthesia for Caesarean section in an achondroplastic dwarf.
Can. J. Anaesth. 1992; 39: 708“11.

technique for this reason, since no dosage guidelines are available
Table 6.7 Epidural test doses in pregnant patients with for patients with short stature and abnormal spinal anatomy.
achondroplasia With a single-shot technique, fears of overdosage may lead to
inadequate anesthesia and intraoperative patient discomfort
Author Test dose
while excessive dosage can cause high spinal or epidural block.
Cohen, 198020 3 ml 3% 2-chloroprocaine Careful evaluation of the upper and lower level of epidural block
Brimacombe, 2 ml 0.5% plain bupivacaine should be made before incision to ensure adequate surgical
1990 anesthesia, with supplemental local anesthetic administered as
Wardall, 199071 3 ml 0.5% plain bupivacaine necessary. Because failed or high block can complicate regional
Carstoniu, 1992 1 ml 2% carbonated lidocaine with 1:200 000 anesthesia, preparations should always be made for management
epinephrine of a difficult airway including availability of all necessary
Morrow, 1998 1.5 ml 2% lidocaine with 1:200 000 epinephrine equipment.
Epidural test doses used in the reported cases have been quite
variable (see Table 6.7).20,21,68,71,74 While no guidelines exist for
the optimal test dose, the amount of local anesthetic injected
series eight achondroplastic dwarfs received six spinal or epidural
should be large enough to detect a subarachnoid catheter without
anesthetics without neurologic sequelae, although technical diffi-
inducing high or total spinal anesthesia.68 Since these patients
culty was frequently encountered.24 The details of five reports of
have abnormal spinal and epidural anatomy, the dose of epi-
continuous epidural anesthesia for C/S are outlined in Table 6.6.
nephrine and local anesthetic is not known for any one indivi-
Although some technical difficulties occurred, all patients had
dual. One patient received epinephrine, 5 mg, in the test dose.68
successful epidural anesthesia without neurologic seque-
lae.20,21,68,71,74,77 Some patients underwent multiple attempts at However, this may not be adequate to detect intravascular place-
ment compared to the more accepted test dose of 15 mg.18 Unlike
epidural placement: one patient had an inadvertent dural punc-
the proportionate short-statured patient, a dosage based on
ture, and one had an intravascular catheter, which was recognized
and then replaced with some difficulty.20,21,74 weight may not be appropriate, especially since the spinal cord
of an achondroplastic dwarf is larger in relation to their spinal
All but one of the patients presented in Table 6.6 required a
decreased dosage for appropriate surgical level of anesthesia
Single-shot spinal anesthesia for emergency C/S is mentioned in
compared with nondwarf individuals. A continuous regional
one report in which 1.3 ml of 0.5% hyperbaric bupivacaine and 10 mg
(spinal or epidural) technique is preferred to a single-shot spinal

2 Musculoskeletal disorders

Figure 6.3 Spondyloepiphyseal dysplasia (Patient F. L.) “
deformities of spine and limbs with contracture of the
pelvis and kyphoscoliosis. Reprinted with permission
from C. V. Mosby Co. from Tyson, J. E., Barnes, A. C.,
McKusick V. A. et al. Obstetric and gynecologic
considerations of dwarfism. Am. J. Obstet. Gynecol. 1970;
108: 688“703.

associated neurologic deficits)78,79 was used. A continuous spinal
of fentanyl were administered via a 26-gauge needle to obtain a
bilateral block to T3.73 The patient had significant hypotension technique using a 20-gauge epidural catheter may be a good
minutes after the spinal injection, which responded to ephedrine alternative either electively or if inadvertent dural puncture
15 mg and a 250 ml fluid bolus with no subsequent episodes of occurs while attempting epidural catheterization.
hypotension. Combined spinal“epidural anesthesia was used by Postoperative pain management with neuraxial opioids was
Trikha et al. for vesico-vaginal fistula repair in a nonpregnant achon- mentioned in only one report, where epidural diamorphine in a
droplastic dwarf, and could be used for urgent C/S.75 However, the 2.5 mg dose was administered every 12 hours.21 There was no
disadvantages may be similar to single-dose spinal anesthesia with mention of postoperative monitoring in this report. Although a
regard to rapid onset of block and hypotension, and potential for single dose of epidural morphine is the usual practice in our
neurologic damage because of anatomic abnormalities. institution, a reduced dosage with increased postoperative
One brief report describes a successful continuous spinal anes- respiratory monitoring (such as hourly respiratory monitoring
thetic in a pregnant achondroplastic dwarf.72 A 32-gauge micro- and continuous pulse oximetry) seems appropriate. With this
spinal catheter (withdrawn from use in the United States by the proviso, epidural morphine can be used safely in parturients of
Food and Drug Administration because of concerns over short stature. Careful monitoring of neurologic function is also

Chapter 6

Figure 6.4 Patient with spondylometaphyseal dysplasia
two days postpartum. Note short stature, short neck.
Reprinted with permission from J. B. Lippincott Co. from
Benson, K. T., Dozier, N. J., Goto, J. et al. Anesthesia for
cesarean section in patient with spondylometepiphyseal
dysplasia. Anesthesiology 1985; 63: 548“50.

congenita65 developed massive pyramidal tract signs consisting
advisable in the postoperative period as the spinal stenosis and
decreased epidural space capacity may result in raised epidural of spastic tetraparesis, increased reflex activity, and intact sensa-
pressure and decreased spinal-cord perfusion. tion following direct laryngoscopy. This patient subsequently
died from complications related to surgical correction of the
Anesthesia for nonachondroplastic disproportionate cervical spine compression, leading some to advocate fiberoptic
parturients intubations for all patients with nonachondroplastic dispropor-
tionate dwarfism.76 Cardiac or pulmonary compromise may be
The anesthetic considerations for the nonachondroplastic dis-
proportionate parturient are similar to those with achondropla- present, depending on the presence of preexisting thoracic spinal
sia. Most of these patients will need a C/S for obstetric reasons. deformities and the degree of uterine impingement on intrathor-
Craniofacial deformities may be present, so the anesthesiologist acic structures. Cardiac anomalies may be present in some forms
should anticipate airway difficulties. Because problems may of dwarfism, thus any symptoms suggestive of cardiac disease
also result from atlantoaxial instability, preoperative flexion- should be investigated prior to delivery. As in achondroplasia,
extension cervical spine X-ray and possibly CT scan is warranted other spinal deformities can exist making regional anesthesia
in all patients.65 A patient with spondyloepiphyseal dysplasia more difficult.

2 Musculoskeletal disorders

Four case reports describe the anesthetic management in miscarried at 19 weeks™ gestation. Her mother also had Marshall-
parturients with nonachondroplastic disproportionate dwarf- Smith syndrome. This condition is associated with laryngomala-
ism.4,19,62,63 Two describe patients with spondyloepiphyseal dyspla- cia and respiratory compromise, making GA especially difficult
sia congenita who had a C/S under epidural anesthesia. The first should patients with this disorder carry their pregnancies to
term.80 Successful pregnancies in patients with conditions
patient had cervical spine abnormalities and marked thoracic
kyphoscoliosis and lumbar lordosis (see Figure 6.3).63 After inad- thought to be incompatible with pregnancy continue to be
vertent i.v. placement of an epidural catheter, repeat placement was reported. One report of pregnancy in a patient with diastrophic
dwarfism exists.81 This patient had an extremely deformed ver-
uneventful; 8 ml of 2% lidocaine with 1:200 000 epinephrine was
administered for a surgical block to T463 The second patient under- tebral column and severe restrictive pulmonary disease. While
went in vitro fertilization, resulting in a twin pregnancy.4 While she details about the anesthetic and airway management are not
did not have any cervical spine abnormalities, she did have marked included, she had a C/S under GA at 26 weeks™ gestation. One
kyphoscoliosis and vertebral flattening. She underwent uneventful report of a successful pregnancy in a patient with Cockayne
epidural anesthesia with a total of 8 ml of 0.5% bupivacaine with syndrome describes the patient being delivered by C/S, but
anesthesia is not mentioned.82 There is also a report of two
1:200 000 epinephrine (sensory level of T5) and used i.v. patient-
patients with severe osteogenesis imperfecta.5 Both patients
controlled analgesia for postoperative pain management.
Two published reports exist describing the anesthetic manage- were delivered by C/S, one managed with epidural anesthesia
ment of parturients with spondylometaphyseal dysplasia.19,62 and the other with awake fiberoptic intubation and GA, due to
her inability to tolerate the supine position.5 There is also a report
The first involved an emergency C/S performed for vaginal
bleeding in a parturient with a marginal placenta previa (see of a patient with Larsen syndrome who underwent uneventful
Figure 6.4).19 This woman had limited neck and jaw mobility epidural anesthesia for C/S.83
and was thought to have ˜˜a very high anterior larynx.™™ She under- As individuals with different types of dwarfism marry and have
went awake direct laryngoscopy, which revealed the epiglottis children, mixed type dwarfism will become more common. We
and posterior arytenoids. The authors then performed a rapid encountered a patient with mixed achondroplastic and ateliotic
sequence induction with easy endotracheal intubation. The case dwarfism who was married to a dwarf with an unspecified type of
was complicated not only by blood loss, but also by the presence chondrodysplasia. She had a C/S at 37 weeks™ gestation under
of pulmonary edema, probably secondary to ritodrine adminis- uneventful epidural anesthesia, delivered a 7 lb 6 oz (3345 g)baby,
tration.19 However, no anesthetic complications occurred. and received 3 mg epidural morphine for postoperative pain with
The second report described a woman with spondylometaphy- no complications.
seal dysplasia who had a CSE for an elective C/S.62 The spinal
dose was 1.4 ml of 0.5% hyperbaric bupivacaine with fentanyl
10 mg, which yielded a T7 level after 10 minutes. She then received
1 ml of 0.5% bupivacaine through the epidural catheter, produ- Currently, relatively few reports are available in the literature to
cing a T4 level five minutes later. Epidural diamorphine 2.5 mg, guide the anesthetic management of parturients of short stature.
and diclofenac 100 mg rectally, were given for postoperative All pregnant dwarfs are at high risk for C/S so anesthesia will
analgesia, with 16 hours of postoperative respiratory monitoring be needed. The physiologic changes of pregnancy often exacer-
and no complications reported.62 bate the preexisting mechanical and physiologic abnormalities
present in dwarfs increasing the risk from anesthesia. Since
numerous forms of dwarfism exist, it is critical that each parturient
Neonatal considerations
be evaluated early in pregnancy and her individual needs assessed
Many types of dwarfism can be diagnosed immediately after birth. prior to anesthetic intervention. Accommodations for the logistics
The incidence of newborn dwarfism in this population greatly of short-stature must be considered for the patient and sometimes
depends on the parents™ type of dwarfism and the accuracy of a short-statured partner, including obtaining smaller chairs, step-
the diagnosis.1 The prognosis for each infant is dependent on the stools, and placing the patient™s bed at a lower level. The patient
type of dwarfism the infant exhibits. For example, infants who are with short-stature desires a compassionate and safe birth experi-
homozygous for the achondroplasia gene either die in utero or the ence and an effort must be made by the anesthesia team to
early neonatal period. Infants with other types of dwarfism may provide individualized care and communication.
have thoracic cage abnormalities with respiratory compromise
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Chapter 6

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1985; 63: 548“50. 50. McKusick, V. A. Heritable Disorders of Connective Tissue, 5th edn. St Louis:
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63. Rodney, G. E., Callander, C. C. & Harmer, M. Spondyloepiphyseal dysplasia 74. Morrow, M. J. & Black, I. H. Epidural anaesthesia for Caesarean section in an
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cesarean section in an achondroplastic dwarf. Anaesthesia 1998; 53: 1236“7. 2001; 92: 1266“7.

Edward T. Crosby

Introduction resulting from the conditions outlined in Table 7.1. Reduced
spinal mobility is characteristic of the structural curves as is
Symptoms relating to the musculoskeletal system are among the
asymmetry in lateral flexibility, which is best appreciated on
most common complaints registered by pregnant women. The
left- and right-bending x-ray films. Structural curves are asso-
maternal axial skeleton is subjected to considerable gestational
ciated with a fixed prominence, the rib hump, on the convex
changes and stresses and skeletal anomalies, both congenital
side of the curve. This prominence is best demonstrated in the
and acquired, may impact on the process and outcome of gesta-
forward-bend position. Kyphoscoliosis, a combination of kypho-
tion and labor. Among the most important of these anomalies is
sis and scoliosis, is uncommon in parturients. It is usually a
scoliosis, arising either as the idiopathic form or as a result of an
congenital disorder, although it may be related to progressive
underlying neuromuscular disorder.
infantile scoliosis or paralytic forms of scoliosis.
Other syndromes are less commonly encountered: these
The curve of scoliosis is typically described by its angle, loca-
include disc prolapse, osteoporosis of pregnancy, and spondylolysis
tion, and direction. The curve angle is determined by the Cobb
and spondylolisthesis.
method, in which the upper and lower end vertebrae of the curves
are identified. Lines are drawn through the end points of these
vertebrae and the Cobb angle is formed by the intersection of
perpendiculars to these lines (see Figures 7.1 and 7.2). The Cobb
Moderate to severe scoliotic curves are not common in women of angle is used to follow progression of the curve and to determine
childbearing age. The major reason for this is that screening the requirement for, and nature of, an intervention. The anatomic
programs identify many at-risk women early in the disease pro- area of the spine in which the apex of the curve is situated
cess, resulting in timely intervention and curve correction. determines the location of the curve. Thoracolumbar curves are
Additionally, primary neuromuscular diseases that result in sco- seen most commonly, followed in frequency by those affecting
liosis are relatively rare and may themselves limit a woman™s only the thoracic or lumbar spines.2 The lower limit of curves
reproductive potential. Despite the fact that women with moder- involving the lumbar spine is usually L3 or L4, although relatively
ate to severe scoliosis constitute a small population of obstetric few curves extend this far caudad. The direction assigned to the
patients, pregnancy within this population is common. The pro- curve is determined by the convexity. Right thoracic curves are
cess and outcome of pregnancy, labor, and delivery are often the most common curves described in idiopathic scoliosis.
similar in women with scoliosis compared with the general popu-
lation. However, if the disease process is advanced, pregnancy
Etiological considerations
and labor may not only be complicated, but also life-threatening.
In this small subpopulation, a detailed understanding of the The cause of the majority (85“90%) of cases of scoliosis is
pathophysiology of advanced scoliosis and the interaction with unknown and these are characterized as idiopathic. Idiopathic
pregnancy is necessary to provide effective maternal care. scoliosis is divided into three types: infantile, juvenile, and ado-
lescent.1 The majority of infantile scoliosis do not progress beyond
308 and resolve spontaneously. Less commonly, and usually in
Definition and description
males, the infantile curves are ˜˜progressive™™ resulting in severe
Scoliosis is defined as an appreciable lateral deviation in the deformity early in life. Juvenile scoliosis has its onset in the four-
normally straight vertical axis of the spine. Scoliosis can be clas- to nine-year age group, is less common than the adolescent form,
sified according to its cause and by a description of the curve, and is not as well defined. Adolescent idiopathic scoliosis (AIS)
including the magnitude, location, and direction.1 Scoliosis is has its onset between age ten and the age of skeletal maturity
divided into structural and nonstructural types, on the basis of and represents the most common form of idiopathic scoliosis.
spinal flexibility. Nonstructural curves are those seen in postural It usually occurs in an otherwise healthy child, often in asso-
scoliosis or those related to sciatica or leg length discrepancies. ciation with a family history of the disease. The inheritance
Nonstructural curves are occasionally seen in parturients, devel- pattern is consistent with a dominant inheritance with reduced
oping with progression of the gestation and resolving after
delivery. They do not affect the mobility of the spine; they are Less common causes of scoliosis are listed in Table 7.1. Of these
nonprogressive and resolve with attention to the underlying rarer forms, the most common in parturients are deformities
cause. Structural curves are those of idiopathic scoliosis or caused by neurological and myopathic conditions that result in

Obstetric Anesthesia and Uncommon Disorders, eds. David R. Gambling, M. Joanne Douglas and Robert S. F. McKay. Published by Cambridge University Press.
# Cambridge University Press 2008.
2 Musculoskeletal disorders

occurring as a consequence of pregnancy-associated osteoporo-
Table 7.1 Conditions associated with scoliosis sis, but reports of this syndrome are rare.5
Congenital (vertebral) anomalies
Incidence and prevalence

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