LINEBURG


<< . .

 82
( 87)



. . >>

followed by atrophy and contractures. Changes usually occur ini- luteum and placenta during pregnancy) reduces skin thickening
tially in the hands and face but may be widespread, although the and improves motility in patients with moderate to severe diffuse
scleroderma.110 Otherwise, treatment should be directed at the
back and buttocks are usually spared. Dilated blood vessels (telan-
giectasias) commonly occur, particularly in the oral and nasal individual organs involved.
Life expectancy is significantly reduced in patients affected by
the disease, mostly as a result of renal failure and malignant
hypertension. Poorer prognosis is associated with greater skin
Table 23.11 Classification of scleroderma
involvement, visceral disease (particularly cardiac, pulmonary,
Limited cutaneous scleroderma
and renal), presence of antitopoisomerase I (anti-Scl-70), anemia,
Long history of Raynaud phenomenon
and elevated erythrocyte sedimentation rate.111,112 Patients with
Skin involvement limited to mostly hands (some on face, feet, and
the CREST variant (calcinosis, Raynaud phenomenon, esopha-
forearms)
geal dysmotility, sclerodactyly, and telangiectasia) are thought to
Nail-fold capillary dilatation
have a relatively benign disease and therefore a better prognosis.
10“15% late onset of pulmonary hypertension, telangiectasias, or
Increased risks of cancer, particularly lung tumors, are reported
interstitial lung disease
in patients with scleroderma.113
Renal involvement rare
May suffer from CREST syndrome (Calcinosis, Raynaud phenomenon,
Effect of pregnancy
Esophageal dysmotility, Sclerodactyly, and Telangiectasia)
Anticentromere antibodies (70“80%)
Most studies report that fertility is unaffected by scleroderma,
although some suggest that it may be reduced.114,115,116 The
Diffuse cutaneous scleroderma
impact of pregnancy on scleroderma is difficult to quantify as
Short history of Raynaud phenomenon before appearance of other
many symptoms of pregnancy are similar to those occurring with
skin changes
the disease (e.g. gastrointestinal reflux, edema, dyspnea). The
Widespread skin involvement particularly over proximal limbs and
most important determinants of adverse maternal and fetal out-
trunk
comes in parturients with scleroderma are the extent and severity
Nail-fold capillary dilatation
of organ involvement. Pregnancy is safest in those patients with-
Early and significant involvement of renal, pulmonary,
out significant cardiac, pulmonary, or renal disease. Steen117
gastrointestinal, and myocardial disease
prospectively reviewed 59 parturients (91 pregnancies) with
No anticentromere antibodies
scleroderma during a ten-year period. Symptoms remained
Anti DNA topoisomerase I (Scl-70) antibodies, anti RNA polymerase I,
unchanged during 63% of pregnancies, worsened in 18% (includ-
II, III antibodies
ing deterioration in esophageal reflux, cardiac dysrhythmias,
Scleroderma sine scleroderma (rare)
arthritis, skin thickening, and renal function), and improved
No skin involvement
(symptoms due to Raynaud phenomenon) in the remaining 20%
Visceral organs involved only (renal, pulmonary, gastrointestinal, and
of pregnancies.
myocardial disease)
Patients with hypertension or renal disease have a significantly
Raynaud phenomenon may be present
increased risk of preeclampsia (PET) and frequent antenatal mon-
Antinuclear antibodies may be present
itoring of BP is important.20 Due to diagnostic difficulties in



416
Chapter 23


distinguishing between scleroderma-induced renal crises, PET,
Table 23.12 Clinical features and anesthetic implications
and hemolytic uremic syndrome, it is unclear whether there is an
of scleroderma
elevated risk of sclerodermal renal crises in patients affected by
the disease. However, in those developing renal deterioration
Organ Disease problem Anesthetic implications
during pregnancy, there is significant morbidity.118 Although
angiotensin-converting enzyme inhibitors are generally contra- Skin Nonpitting edema Difficulty with
indicated in pregnancy due to associated fetal renal insufficiency, cannulation
the benefits of these medications may outweigh their risks in the Hidebound skin Easy insertion of re-
treatment of scleroderma-related hypertensive and renal gional needles, due to
crises.119 Renal crises are the most common cause of maternal sparing of lumbar area
death in parturients with scleroderma. Steen117 suggested that Difficult intubation
those with early diffuse scleroderma should consider avoiding (poor mouth opening)
pregnancy until their disease is stabilized in order to diminish CVS Raynaud phenomenon Caution with arterial
the risk of renal complications. cannulation as may
Many studies suggest that there is an increased risk of sponta- result in digital
neous abortion in parturients with scleroderma,120 with higher ischemia
rates of loss seen in patients with diffuse rather than localized Myocardial ischemia Reduced cardiac reserve
cutaneous disease. There may be an increased rate of premature Chronic pericardial
and ˜˜small for gestational age™™ births but these studies are unclear effusion
about the influence of medications (particularly immunosuppres- Conduction Risk of dysrhythmias
sive agents) and other risk factors for prematurity.115,121 disturbances
Hypertension Maintain cardiovascular
stability
Obstetric management
Respiratory Interstitial fibrosis Limited respiratory
Parturients should be evaluated fully and followed closely through- Pleural effusion reserve
out pregnancy with input from cardiologists, nephrologists, and Chest wall restriction
chest physicians. Close fetal surveillance is also important with Pulmonary Avoid precipitants pro-
attention given to the possible fetal effects of medications (see hypertension ducing increased PVR
Table 23.3). (hypoxia, hypercap-
Vaginal delivery is the preferred method of delivery. However, nia, acidosis, pain,
ineffective uterine contractions or cervical dystocia during hypothermia, high
labor may result from uterine and cervical wall thickening.115 positive end expira-
Augmentation of labor with oxytocics should be used cautiously tory pressure)
as considerable hemodynamic changes can occur. These changes GIT Hypomotility
may be detrimental in scleroderma patients with cardiac disease Reflux esophagitis Risk of aspiration
or pulmonary or systemic hypertension. Careful positioning of Diarrhea/ Dehydration
patients during delivery is important due to associated contrac- malabsorption Coagulopathy (vitamin
tures and restrictive skin changes. Patients should be kept warm K deficiency)
in order to reduce symptoms of the Raynaud phenomenon. Renal Renal Proteinuria Confusion with
function should be monitored in patients with renal insufficiency. diagnosis of
Moore and coworkers122 reported an interesting case of obstruc- preeclampsia
tive uropathy resulting from uterine enlargement within a non- Renal insufficiency Altered drug handling
compliant abdomen. Malignant Risk of end-organ
Elective C/S may be required if fibrosis of perineum and cervix hypertension damage
prevents vaginal delivery.123 Wound healing may be problematic NS Peripheral/cranial Full neurological
in patients with advanced disease and in those on corticosteroids, neuropathy evaluation needed
and a careful operative technique to promote healing is impor- prior to regional
tant. Patients with significant cardiovascular, pulmonary, or renal anesthesia
compromise would benefit from observation in a high depen- MS Arthritis Adequate padding/
dency setting in the postpartum period. Myopathy careful patient
Contractures positioning

Anesthetic management CVS ¼ cardiovascular; GIT ¼ gastrointestinal tract; PVR ¼ pulmonary
vascular resistance; NS ¼ nervous system; MS ¼ musculoskeletal
Patients should be reviewed early in pregnancy so that organ
system
involvement can be evaluated and anesthetic strategies formu-
lated. Anesthetic implications and problems of scleroderma are



417
5 Other disorders


Antiphospholipid syndrome
described in Table 23.12. Venous access may be difficult due to
cutaneous changes and central venous catheterization may be
Antiphospholipid syndrome (APS) is an autoimmune disorder char-
necessary. Noninvasive BP monitoring is the preferred technique,
acterized by medium to high titers of antibodies directed against
as arterial cannulation may induce vasospasm and distal necro-
phospholipid binding plasma proteins, together with typical clinical
sis. Brachial arterial catheterization may be superior to radial
manifestations of thrombosis or fetal loss.132 However, due to vari-
arterial catheterization in patients with the Raynaud phenom-
able organ involvement, clinical features are diverse (see
enon due to diminished ischemic risk.
Table 23.13). Cervera and coworkers133 found that the commonest
The airway should be assessed, as difficulty with endotracheal
clinical manifestations reported in a series of 1000 patients with APS
intubation can be caused by limited mouth opening due to sur-
were deep vein thrombosis (32%), thrombocytopenia (22%), livedo
rounding skin and connective tissue changes. Taut skin may also
reticularis (13%), cerebrovascular accident (13%), superficial throm-
diminish neck mobility and hardening of the tissue in the sub-
bophlebitis (9%), fetal loss (8%), transient ischemic attack (7%), and
mental triangle may limit the ability to align the oral, pharyngeal,
hemolytic anemia (7%). Both venous and arterial thromboses
and laryngeal axes during laryngoscopy.124,125 In addition, the
occur, although venous thrombosis is more frequent than arterial.
presence of oral and nasal telangiectasias should be noted as
Occasionally patients can present with ˜˜catastrophic antiphospholi-
dilated capillaries may bleed profusely if traumatized. Extreme
pid syndrome™™ (CAPS), which represents a severe form of the condi-
care should be taken during airway manipulation in these
tion associated with multiorgan failure secondary to thrombosis.
circumstances.
Antiphospholipid syndrome can either be primary (not asso-
Early epidural analgesia for labor124 is advised especially in
ciated with any underlying disease) or secondary (associated with
those patients with a potentially difficult intubation, as this allows
SLE or another autoimmune/rheumatic disease). The three most
the block to be extended if C/S is required. The skin of the lumbar
commonly elevated aPLs associated with this condition are anti-
region is frequently spared from cutaneous involvement making
cardiolipin (aCL), lupus anticoagulant (LA), and anti b2 glycopro-
regional needle insertion easy. Eisele and Reitan126 found sclero-
tein I antibodies.134 Low titers of aPLs are also found in healthy
derma patients have an increased sensitivity to local anesthetics
individuals, particularly the elderly, and they can be induced by
and suggested the use of reduced doses of these agents. The use of
infection or malignancy. Increased aPLs titers are also associated
slow incremental doses of local anesthesia via an epidural or
with SLE (23“47% of SLE patients have aCLs, 30% have LA, and
intrathecal catheter is the technique of choice as it enables the
20% have antibodies to b2 glycoprotein I)135 and other autoim-
anesthesiologist to titrate the dose of local anesthetic to the
mune diseases (e.g. RA, idiopathic thrombocytopenia).
desired level. Single-shot spinal anesthesia for C/S was reported
Although thrombosis, infarction, and vasculopathy are thought
in a parturient with scleroderma, severe PET, thrombocytopenia,
to account for the majority of clinical features, the exact etiology
and a potentially difficult airway.127 Intraoperative complications
included precipitous hypotension and a high sensory block
Table 23.13 Clinical features of antiphospholipid syndrome
(to T2) following intrathecal bupivacaine and diamorphine.
Prolonged duration of local anesthetics have been reported by
Thrombosis Deep vein thrombosis (calf is commonest
several authors124,126,128,129 and may result from diminished
(arterial/venous) venous site)
intravascular uptake due to disease-associated microvascular
Cerebral vascular accident (commonest
changes.
arterial site)
The successful use of general anesthesia125,130,131 for C/S in
Mesenteric infarction
scleroderma patients has been reported. Hseu and coworkers130
Hepatic/renal thrombosis
administered an opioid-based general anesthetic for C/S in a
Obstetric Recurrent fetal loss
scleroderma patient complicated by pulmonary hypertension
complications Intrauterine growth restriction
secondary to restrictive lung disease. General anesthesia was
Preeclampsia
chosen to enable airway control and hemodynamic stability
Skin Livedo reticularis (reticular pattern of
with the aid of a pulmonary artery catheter.
mottling over extremities or trunk)
The risk of aspiration in affected patients is greater than
Hematological Hemolytic anemia
in normal parturients as gastric hypomotility is compounded
Thrombocytopenia
by the normal physiological gastrointestinal changes that
Kidney Nephropathy
occur in pregnancy. Administer histamine-2 blockers during
Thrombosis
labor in case urgent C/S becomes necessary. The decision to
Neurological Seizures
use either regional or general anesthesia for C/S depends on
Cardiac Intracardiac/coronary thrombosis
the airway, urgency of delivery, and the presence of a function-
Mitral/aortic regurgitation (due to thickened
ing epidural. In patients with a potentially difficult airway,
valves)
the decision for a C/S should be made early to enable ade-
Pulmonary Pulmonary embolus
quate time for an awake fiberoptic intubation. As a result
Pulmonary arterial thrombosis
of decreased tear production in affected patients, hydrating
Adult respiratory distress syndrome
ointments and eye pads should be used to protect against
Gut Ischemic event
corneal damage.



418
Chapter 23


Anesthetic management
and pathogenesis remain unclear. Treatment of APS has been
directed towards the prevention of thrombosis by the use of
Two case series describe the anesthetic management of 20146 and
anticoagulants (heparin or warfarin) or antiaggregants (aspirin
27147 parturients with APS. Both authors emphasized the like-
or clopidrogrel), or at the reduction of antibodies (immuno-
lihood of patients requiring anesthetic involvement. Of the 47
suppressives: corticosteroids, cytotoxic agents). However, the
cases, 32 received regional anesthesia (22 epidurals, 5 spinals,
mainstay of treatment is anticoagulants, with immuno-
1 combined spinal“epidural) and 19 patients required C/S.
suppressive therapy reserved for those refractory to conven-
Despite antithrombotic treatment, five patients experienced
tional treatment.
thrombotic episodes (including transient ischemic attack, pulmon-
There is an increased risk of premature death in APS patients,
ary embolus, bilateral renal vein thrombosis, deep vein thrombosis)
although prognosis is variable. Mortality is related to the in-
during pregnancy and the early postpartum period.
creased propensity to thromboembolism and the increased rates
Parturients should be assessed early in pregnancy. Coexisting
of malignancy, ischemic heart disease, adverse effects of anti-
autoimmune disease (particularly SLE) and organ involvement
coagulant medication, and associated diseases (e.g. SLE). Patients
should be evaluated. Patients with lupus anticoagulant (LA) have
with differing subclasses of aPLs may have varying degrees of
an artifactual in vitro prolongation of the aPTT, despite normal or
disease risk. Sammaitano and coworkers136 found that patients
increased in vivo coagulation. Clearly, regional anesthesia is not
with elevated titers of IgG aCLs (subclass 2) had an increased
contraindicated in these cases. However, patients with APS may
thrombotic risk compared with those having different aPLs.
have significant thrombocytopenia, be receiving anticoagulant
More than 50% of patients with APS who have experienced a
therapy, or rarely have antibodies to a number of clotting factors
thrombotic event will have a subsequent clinical thrombosis,
(including VIII, IX, XII, and XIII). This may result in significant
and in approximately 70% the thrombosis will occur on the
coagulopathy and preclude the use of regional anesthesia. The
same side of the vascular tree.137
development of thrombocytopenia in a parturient with APS may
Development of CAPS results in a grave prognosis. There is a 50%
be due to PET, APS, or heparin-induced thrombocytopenia. In
mortality rate, and among survivors, 20% have further recurrent
those patients receiving anticoagulation, the use of regional
thromboembolic events despite anticoagulation.138 Precipitating
anesthesia is permitted once the coagulation profile has normal-
factors for CAPS include surgery (even minor operative proce-
ized after UFH (remember that the aPTT can be artificially raised
dures), infection, and the withdrawal of anticoagulants.139
in the presence of LA). For those receiving LMWH, 12 hours
should have elapsed after the last thromboprophylactic dose (or
Effect of pregnancy 24 hours for those patients fully anticoagulated with LMWH). The
advantages of regional anesthesia in a particular patient must be
Prevalence of aPLs in the normal obstetric population is approxi-
weighed against the small risk of a spinal hematoma.148
mately 5%140 and 5“50% (mean 15%) in parturients with recurrent
Atypical antibodies can make cross-matching and typing of
miscarriages.134,141 Pregnancy compounds the already elevated
blood difficult and time consuming. Therefore blood should be
risk of thrombosis in patients with APS. Additional pregnancy-
typed or cross-matched early in APS patients at risk of significant
associated complications of APS include infertility,142 recurrent
hemorrhage, or those with residual anticoagulant effect.
miscarriage (>10 weeks™ gestation),142,143 early severe PET, eclamp-
Patients with APS are at risk of thromboembolism, PET, and
sia, HELLP syndrome (hemolysis, elevated liver enzymes, and low
postpartum bleeding (due to anticoagulants or thrombo-
platelets), thrombocytopenia, prematurity, stillbirth, and IUGR.144
cytopenia). Prophylactic antithrombotic measures, such as adequate
The precise mechanism for fetal loss (>10 weeks™ gestation) is
hydration, early mobilization, and the use of antiembolic stockings,
unknown. However, it seems likely that the thrombotic risk of APS
should be instituted as soon as feasible. Thromboprophylaxis or
contributes to placental insufficiency. Paradoxically, APS may
therapeutic anticoagulation (depending on the individual risk
protect against recurrent early fetal loss (<10 weeks™ gestation).
of thromboembolism) should be restarted (12 hours post C/S,
One possible explanation is that in early normal pregnancy (<10
4“6 hours following vaginal delivery) and continued for at least
weeks™ gestation) the trophoblast is exposed to reduced utero-
six weeks postpartum. Long-term anticoagulation should be con-
placental blood flow and low oxygen partial pressures, i.e. condi-
sidered if thrombotic events are unrelated to the pregnant state.
tions that are normally found in patients with APS.
Several case reports describe the problems associated with
Although low-dose aspirin and heparin are recommended for
anesthetizing nonobstetric patients with CAPS.149,150,151 The
parturients with APS, scientific evidence is weak, largely due to
risks of opposing complications present an intraoperative chal-
poor trial design and inter-trial clinical heterogeneity.145 Benefits
lenge; catastrophic exacerbation of the thrombotic tendency may
of low molecular weight heparin (LMWH) compared to unfrac-
be triggered by the surgical stimulus and major bleeding may
tionated heparin (UFH) include better bioavailability, improved
result from the necessary anticoagulation.
dosage regime, reduced risk of heparin-induced thrombo-
cytopenia, and diminished risk of osteoporosis. There is no evi-
dence to support the use of corticosteroids or intravenous
Summary
immunoglobulin in affected parturients. Women on long-term
warfarin anticoagulation should consider converting to heparin Autoimmune diseases are more frequent in the female popula-
to avoid any potential teratogenic risks. tion and hence their occurrence in parturients is not uncommon.



419
5 Other disorders


Specific conditions are variable in terms of disease activity and 23. Ostensen, M. Safety on nonsteroidal anti-inflammatory drugs during preg-
nancy and lactation. Immunopharmacology 1996; 4: 31“41.
organs affected. The anesthesiologist should carefully analyze the
24. Soscia, P. N. & Zurier, R. B. Antirheumatic drug therapy during pregnancy.
disease impact in order to minimize deleterious outcomes during
Bull. Rheum. Dis. 1997; 46: 2“4.
any medical intervention. In addition, the consequences of drug 25. Committee on Drugs, American Academy of Pediatrics. The transfer of
treatment on both the mother and fetus should be assessed. drugs and other chemicals into human breast milk. Pediatrics 1994; 93:
137“50.
26. Park-Wyllie, L., Mazzotta, P. I., Pastuszak, A. et al. Birth defects after
REFERENCES
maternal exposure to corticosteroids: prospective cohort study and meta-
1. Ware Branch, D. & Flint Porter, T. Autoimmune disease. In James, D. K., analysis of epidemiological studies. Teratology 2000; 62: 385“92.
Steer, P. J., Weiner, C. P. & Gonik, B. (eds.), High Risk Pregnancy: 27. Petri, M. Immunosuppressive drug use in pregnancy. Autoimmunity 2003;
Management Options. London: W. B. Saunders, 1999. 36: 51“6.
28. Alstead, E. M., Ritchie, J. K., Lennard-Jones, J. E., Farthing, M. J. & Clark,
2. Lawrence, R. C., Helmick, C. G., Arnett, F. C. et al. Estimates of the preva-
lence of arthritis and selected musculoskeletal disorders in the United M. L. Safety of azathioprine in pregnancy in inflammatory bowel disease.
States. Arthritis Rheum. 1998; 41: 778“99. Gastroenterology 1990; 99: 443“6.
3. Masi, A. T. Incidence of rheumatoid arthritis: do the observed age“sex 29. Miehle, W. Current aspects of D-penicillamine and pregnancy. Z. Rheumatol.
1988; 47: 20“3.
interaction patterns support a role of androgenic-anabolic steroid defi-
ciency in its pathogenesis? Br. J. Rheumatol. 1994; 33: 697“9. 30. Zeldis, J. B. Pregnancy and inflammatory bowel disease. West. J. Med. 1989;
4. Ansar-Ahmed, S., Dauphinee, M. J. & Talal, N. Effects of short term admin- 151: 168“71.
31. Buchanan, N. M., Toubi, E., Khamashta, M. A. et al. Hydroxychloroquine
istration of sex hormones on normal and autoimmune mice. J. Immunol.
and lupus pregnancy: review of a series of 36 cases. Am. Rheum. Dis. 1996;
1985; 134: 204“10.
5. Takagi, H., Ishiguro, N., Iwata, H. & Kanamono, T. Genetic association 55: 486“8.
between rheumatoid arthritis and estrogen microsatellite polymorphisms. 32. Fiddler, M. A. Rheumatoid arthritis and pregnancy: issues for consideration
in clinical management. Arthritis Care Res. 1997; 10: 264“72.
J. Rheumatol. 2000; 27: 1638“42.

<< . .

 82
( 87)



. . >>

Copyright Design by: Sunlight webdesign