has improved recently.38,39 Weiss reported neonatal survival ran-
toms.113,114,115 Magnetic resonance imaging was employed and
ging from 87â€“89% depending on the etiology of the hypertension,
typically revealed disc herniation with compression of neural
compared with maternal mortality rates ranging from 30â€“56%.39
elements. Most of the patients were treated conservatively at the
Parturients may present in right heart failure and cardiogenic
outset but were subsequently subjected to surgery due to intract-
shock during gestation; mortality attributable to such a presenta-
able pain or progression of neurological deficits. Regional
tion is unfortunately high and survival is not expected. Urgent C/S
anesthesia was employed for both the disc surgery as well as for
is likely necessary to achieve fetal and perhaps maternal salvage.
the subsequent labor and delivery without apparent sequelae.
The immediate goal of therapy in this setting should be to
A conservative approach is warranted when disc prolapse
decrease PVR, reducing RV afterload leading to an increase in
occurs in pregnancy; surgical intervention is reserved for
CO. This increased CO may improve systemic BP and reduce RV
women with bowel or bladder dysfunction. In the absence of
ischemia.106 Afterload reduction may be achieved by pulmonary
progressive or significant neurological compromise or intractable
vasodilation. Although sodium nitroprusside has been used in
pain, management is expectant.116 Pregnant women with radicu-
this setting, nitroglycerin is also an effective pulmonary vasodi-
lopathy but no sphincter dysfunction are managed in much the
lator with fewer effects on the systemic vasculature and on fetal
same way as those who do not have radiculopathy. Magnetic
and uterine activity.63 Unfortunately, systemic vasodilators also
resonance imaging is considered to be the imaging modality of
reverse hypoxic pulmonary vasoconstriction, which may worsen
choice and is not contraindicated during pregnancy. In the event
pulmonary V/Q matching and decrease diastolic pressure, result-
that surgery is indicated, regional anesthesia may be used. There
ing in decreased myocardial perfusion.107 Recently, the use of a
is limited obstetrical experience managing labor and delivery in
selective pulmonary vasodilator such as intravenous epoproste-
women with disc prolapse. As symptoms become more severe, it
nol or inhaled nitric oxide has been reported in decompensated
is likely that C/S will be used to decrease intrathecal pressures
parturients with resulting maternal survival.108,109 Vasopressors
and the risk of symptom exacerbation during labor and deliv-
directly increase arterial BP but increase afterload as well.
ery.112 Regional anesthesia is appropriate for the provision of
Norepinephrine is recommended to increase right coronary
labor analgesia and surgical anesthesia.
artery perfusion pressure and RV function and it is more effective
than phenylephrine.107 Contractility enhancing agents such as
phosphodiesterase inhibitors and calcium sensitizers may have
Osteoporosis of pregnancy
a role in the management of these patients but there are no
reports at this time regarding their use in this scenario. If the Osteoporosis associated with pregnancy, although still uncom-
mon, has been increasingly described in the recent literature.117
mother survives the acute event, it is advisable to maintain phar-
macologic support to allow RV afterload and volumes to decrease. There is clear evidence of decreased bone mineral density
Mortality remains high in the month after delivery and is during pregnancy. Prolactin results in osteopenia and circulating
presumed to be due to thromboembolic events, increased levels are increased in pregnancy. There are also increased
2 Musculoskeletal disorders
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MISCELLANEOUS SKELETAL AND CONNECTIVE TISSUE
8 DISORDERS IN PREGNANCY
Introduction suspected malignant ovarian tumors. General anesthesia was
induced with etomidate and succinylcholine and maintained
This chapter discusses a variety of miscellaneous conditions
with halothane, fentanyl, and pancuronium. The operation was
found during pregnancy, each with different degrees of rarity. It
uneventful except for hypertension, which occurred on handling
focuses on the pathophysiologic changes that occur with each
the tumor. The patient made a good recovery but delivered pre-
disease in order to highlight the impact on both anesthetic and
maturely at 27 weeks.
obstetric management. However, as some of the conditions
The second pregnant patient8 had uncontrolled hypertension
described have a wide and varied organ involvement, firm man-
despite medical management and required a second trimester
agement conclusions cannot be made. Each case should be
laparotomy for removal of a renin-secreting ovarian tumor.
assessed individually and the risk/benefit of any given anesthetic
Surgery was performed successfully under combined epidural
choice should be evaluated for each particular patient.
and general anesthesia. She had an uneventful delivery at term.
Both patients had mandibular cysts but intubation was unevent-
ful. The risks and benefits of either regional or GA will depend on
Gorlin syndrome (Gorlin-Golty syndrome
individual features of the patient (see Table 8.1). However, if a
or nevoid basal cell carcinoma syndrome)
renin-secreting tumor is suspected, it may be advantageous to
Gorlin syndrome was first described in 1960.1 The characteristic use regional anesthesia to prevent hypertensive surges.
features are multiple basal cell nevi, odontogenic keratocysts of
the mandible, and other congenital, mostly skeletal, abnormal-
ities.2 A variety of other neurological, genital, endocrine, and
Noonan syndrome was first described in 19639 and is a disorder
ophthalmic manifestations are also associated with this disease.
A predisposition to tumor formation is well documented; those characterized by abnormalities of the facial, cardiovascular, and
skeletal systems.10 Inheritance is usually autosomal dominant,
described include ovarian fibromas, central nervous system
(CNS) tumors (mostly medulloblastomas), skin, and, occasion- although most cases are due to new mutations (1:8000 births).
ally, cardiac tumors.3 Although this disorder has an autosomal The chromosomal abnormality is a mutation in the PTPN11 gene
on chromosome 12q24.1.11 Males and females may exhibit
dominant mode of inheritance, 50% arise from spontaneous
mutations. The causative gene mutation is located on chromo- Noonan syndrome, but males have cryptorchidism and hypo-
some 9q22.3, which is normally required for the function of a plastic testes (so are rarely fertile), whereas females are usually
transmembrane receptor involved in patterning and growth. fertile, although their menarche is often delayed. Maternal trans-
This Gorlin mutation reduces its ability to act as a tumor sup- mission of the gene is reported to occur three times as frequently
as paternal transmission.12 The syndrome is similar to Turner
pressor gene resulting in the increased incidence of cancers.
Males and females are equally affected and the disease preva- syndrome with respect to short stature, characteristic facies (pto-
lence is about 1 per 60 000.4 The condition has a variable pheno- sis, downward slanting eyes, hypertelorism, hooded eyelids,
typic expression with most cases reported in Caucasians. This broad flat nose, high arched palate, micrognathia, abnormal
may be due to the protective effect of melanotic pigmentation ears), shield-shaped chest deformity, and webbing (Ã¾/Ã€ fusion)
in black ethnic groups that prevents the formation of basal cell of the neck. However, Noonan syndrome differs in that patients
carcinomas.5 have a normal karyotype, a risk of mental retardation, coagulation
Southwick and Schwartz6 followed a group of 36 patients with defects, skeletal abnormalities, and associated heart lesions
(mostly right sided rather than left, as in Turner syndrome).13
Gorlin syndrome, three of whom had severe bradycardic/hypo-
tensive reactions to general anesthesia (GA). The authors postu- The most frequently reported cardiac abnormality is pulmonary
lated that the patients might have had an unusual response to stenosis (usually with a dysplastic valve), although a diverse range
of abnormalities have been described.14,15
thiopental. However, as there was minimal discussion of the
patientsâ€™ preoperative status and anesthetic details, there was Improving results from cardiac surgery in childhood mean that
little evidence to make this conclusion. There are no other similar more women with complex Noonan syndrome are becoming
reports in the literature. pregnant. Parturients are at risk of operative delivery due to
There are two reports of pregnant patients with Gorlin syn- their contracted â€˜â€˜maleâ€™â€™-type pelvis and increased risk of cepha-
drome associated with renin-secreting ovarian tumors.7,8 The lopelvic disproportion. These patients should be assessed early
first patient7 needed a laparotomy at 17 weeksâ€™ gestation, due to in the prenatal period to discuss anesthetic and obstetric
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
Table 8.1 Clinical features of Gorlin syndrome Table 8.2 Clinical features of Noonan syndrome
Organ Disease process implications Organ Disease process implications
CNS CNS tumors Raised ICP Airway High arched palate Possible difficult
Medulloblastomas (3â€“5%) intubation
Meningioma (1%) Short webbed neck
Congenital hydrocephalus Micrognathia
Mental retardation (mild) Consent/ Chest wall Pectus deformity Limited respiratory
issues â€˜â€˜Shield-shapedâ€™â€™ chest
Skin Basal cell carcinoma Nil Spine Kyphoscoliosis Regional techniques
Palmar and/or plantar pits Lumbar lordosis
(90%) Narrow spinal canal
Skeletal Short stature
Orofacial Odontogenic keratocysts Airway Cardiac Congenital heart Limited cardiac
(75%) management disease reserve
issues Mostly right sided Conduction defects
Maxillary hypoplasia PS/ASD (69%) Risk of endocarditis
Mandibular hyperplasia CNS Mental retardation Implication of
Cleft lip/palate consent and awake
Macrocephaly (40%) procedures
Frontal bossing (25%) Hematological Bleeding diatheses Possible
Hypertelorism (5%) contraindication
High arched palate (40%) to regional
Widened nasal bridge (60%) techniques
Spine Scoliosis Poor respiratory/ Deceased factors
CV function (e.g. XI, XII, VW)
Hemivertebrae Difficulty with Platelet dysfunction Perioperative
Spina bifida occulta Renal Congenital Possible renal
Fusion defects abnormalities dysfunction
Rib Bifid/hypoplastic (38â€“60%) Poor respiratory/ Dermis/ Lymphedema Difficult intravenous
CV function epidermis access
Chest wall deformities Redundant skin