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good resource for the most recent information.43 The safety of using opioids and acetaminophen, but nonsteroidal anti-
inflamatory agents such as diclofenac should be avoided.33
drugs in porphyria is based on testing of in vitro chick embryos,
standardized in vivo rat models, and clinical case reports. The There are many drugs commonly used by obstetricians that
evidence for many drugs is conflicting due to interspecies varia- should be avoided: including the ergot derivatives, calcium chan-
tions in response or because clinical experience is insufficient to nel antagonists, hydralazine, clonidine, and a-methyl-dopa.
draw firm conclusions. It is important to recognize precipitants Midazolam, temazepam, lorazepam, droperidol, and the phe-
other than drugs, and to be aware that known triggers do not nothiazine antiemetics are likely safe. Oxytocin is not porphyro-
consistently induce attacks. Hyperemesis gravidarum has been genic and may be used. Severe post partum hemorrhage can be
reported as a possible trigger.39,44 treated with carboprost (Hemabate’).54

Regional anesthesia
While there is no evidence that regional anesthesia or analgesia
may cause an acute attack of porphyria, there is reluctance to use Acute porphyrias are rare but may be triggered by both anes-
it for fear of masking a potential peripheral neuropathy secondary thetics and drugs commonly used in obstetrics. Further, labor

4 Metabolic disorders

and delivery place the susceptible parturient at risk. While it is 15. Elstein, Y., Eisenberg, V., Granovsky-Grisaru, S. et al. Pregnancies in
Gaucher disease: a 5-year study. Am. J. Obstet. Gynecol. 2004; 190: 435“41.
impossible to catalog or remember which drugs are safe, updated
16. MacKenzie, J. J., Amato, D. & Clarke, J. T. Enzyme replacement therapy for
prescribing information is available on reliable websites. In spite
Gaucher™s disease: the early Canadian experience. C. M. A. J. 1998; 159:
of best efforts to reduce the risk in susceptible individuals, acute 1273“8.
attacks may occur. The diagnosis must be considered and sup- 17. Cleary, J. E., Burke, W. M. & Baxi, L. V. Pregnancy after avascular necrosis of
portive treatment initiated early in parturients with a history of the femur complicating Gaucher™s disease. Am. J. Obstet. Gynecol. 2001;
184: 233“4.
18. Ioscovich, A., Elstein, Y., Halpern, S. et al. Anesthesia for obstetric patients
Increasing numbers of women with inherited metabolic disor-
with Gaucher disease: survey and review. Int. J. Obstet. Anesth. 2004; 13:
ders are surviving into adulthood and, with new therapies, are 244“50.
able to get pregnant and have successful outcomes. However, 19. Elstein, D., Klutstein, M. W., Lahad, A., Hadas-Halpern, I. & Zimran, A.
much experience is still being acquired in the management of Echocardiographic assessment of pulmonary hypertension in Gaucher™s
disease. Lancet 1998; 351: 1544“6.
inherited metabolic disorders during pregnancy. Some of these
20. Longo, N. Inherited disorders of amino acid metabolism presenting in
disorders can significantly affect the mother and the fetus. It is
adults. In Kasper, D. L., Braunwald, E., Fausi, A. C. et al. (eds.), Harrison™s
important to consider the possibility of an inherited metabolic Principles of Internal Medicine, 16th edn. New York, NY: McGraw-Hill
disorder being present in fetuses of pregnancies affected by non- Companies, Inc, 2005, pp. 2331“4.
immune hydrops, hemolysis, elevated liver enzymes, and low 21. Feillet, F., Abadie, V., Berthelot, J. et al. Maternal phenylketonuria: the
French survey. Europ. J. Pediat. 2004; 163: 540“6.
platelets (HELLP) syndrome or acute fatty liver of pregnancy.55
22. Lee, P. J., Ridout, D., Walter, J. H. & Cockburn, F. Maternal phenylketonuria:
There is a need for ongoing collection of data within registries to
report from the UK registry 1978“97. Arch. Dis. Child. 2005; 90: 143“6.
improve our understanding of these conditions.56 23. Ueland, P. M., Holm, P. I. & Hustad, S. Betaine: a key modulator of one-
carbon metabolism and homocysteine status. Clin. Chem. Lab. Med. 2005;
43: 1069“75.
24. Vilaseca, M. A., Cuartero, M. L., Martinez de Salinas, M. et al. Two successful
1. Chen, Y-T. Glycogen storage diseases and other inherited disorders of pregnancies in pyridoxine-nonresponsive homocystinuria. J. Inherit.
Metab. Dis. 2004; 27: 775“7.
carbohydrate metabolism. In Kasper, D. L., Braunwald, E., Fausi, A. C.
et al. (eds), Harrison™s Principles of Internal Medicine, 16th edn. New York, 25. Pierre, G., Gissen, P., Chakrapani, A. et al. Successful treatment of pyridox-
NY: McGraw-Hill Companies, Inc, 2005, pp. 2319“23. ine-unresponsive homocystinuria with betaine in pregnancy. J. Inherit.
Metab. Dis. 2006; 29: 688“9.
2. Hagemans, M. L., Winkel, L. P., Van Doorn, P. A. et al. Clinical manifestation
26. Mudd, S. H., Skovby, F., Levy, H. L. et al. The natural history of homocysti-
and natural course of late-onset Pompe™s disease in 54 Dutch patients.
Brain 2005; 128: 671“7. nuria due to cystathionine beta-synthase deficiency. Am. J. Human Genet.
3. Mellies, U., Ragette, R., Schwake, C. et al. Sleep-disordered breathing 1985; 37: 1“31.
27. Burke, G., Robinson, K., Refsum, H. et al. Intrauterine growth retardation,
and respiratory failure in acid maltase deficiency. Neurology 2001; 57:
1290“5. perinatal death, and maternal homocysteine levels. N. Eng. J. Med. 1992;
4. Winkel, L. P., Van den Hout, J. M., Kamphoven, J. H. et al. Enzyme replace- 326: 69“70.
ment therapy in late-onset Pompe™s disease: a three-year follow-up. Ann. 28. Calvert, S. M. & Rand, R. J. A successful pregnancy in a patient with homo-
cystinuria and a previous near-fatal postpartum cavernous sinus thrombo-
Neurol. 2004; 55: 495“502.
5. Ratinov, G., Baker, W. P. & Swaiman, K. F. McArdle™s syndrome with pre- sis. Br. J. Obstet. Gynaecol. 1995; 102: 751“2.
viously unreported electrocardiographic and serum enzyme abnormalities. 29. Kauppinen, R. Porphyrias. Lancet 2005; 365: 241“52.
Ann. Int. Med. 1965; 62: 328“34. 30. Hift, R. J. & Meissner, P. N. An analysis of 112 acute porphyric attacks in
Cape Town, South Africa: evidence that acute intermittent porphyria and
6. Lepoivre, T., Legendre, E. & Pinaud, M. Anesthesia for cesarean section in a
patient with McArdle disease and hereditary dilated cardiomyopathy. Ann. variegate porphyria differ in susceptibility and severity. Medicine 2005; 84:
Fr. Anesth. Reanimat. 2002; 21: 517“27. 48“60.
31. Aziz Ibrahim, A. & Esen, U. I. Porphyria cutanea tarda in pregnancy: a case
7. Coleman, P. McArdle™s disease. Problems of anaesthetic management for
Caesarean section. Anaesthesia 1984; 39: 784“7. report. J. Obstet. Gynaecol. 2004; 24: 574“5.
8. Bollig, G., Mohr, S. & Raeder, J. McArdle™s disease and anaesthesia: case 32. Thadani, H., Deacon, A. & Peters, T. Regular review: diagnosis and manage-
reports. Review of potential problems and association with malignant ment of porphyria. Br. Med. J. 2000; 320: 1647“51.
33. James, M. F. M. & Hift, R. J. Porphyrias. Br. J. Anaesth. 2000; 85: 143“53.
hyperthermia. Acta Anaesthesiol. Scand. 2005; 49: 1077“83.
9. Lee, P. Successful pregnancy in a patient with type III glycogen storage 34. Soonawalla, Z. F., Orug, T., Badminton, M. N. et al. Liver transplantation as
disease managed with cornstarch supplements. Br. J. Obstet. Gynaecol. a cure for acute intermittent porphyria. Lancet 2004; 363: 705“6.
1999; 106: 181“2. 35. Engelhardt, K., Trinka, E., Franz, G. et al. Refractory status epilepticus due to
acute hepatic porphyria in a pregnant woman: induced abortion as the sole
10. Mairovitz, V., Labrune, P., Fernandez, H., Audibert, F. & Frydman, R.
Contraception and pregnancy in women affected by glycogen storage dis- therapeutic option? Eur. J. Neurol. 2004; 11: 693“7.
eases. Eur. J. Pediatr. 2002; 161: S97“101. 36. Keung, Y. K., Chuahirun, T. & Cobos, E. Acute intermittent porphyria with
seizure and paralysis in the puerperium. J. Am. Board Fam. Pract. 2000; 13:
11. Exantus, J., Ranchin, B., Dubourg, L. et al. Acute renal failure in a patient
with phosphofructokinase deficiency. Pediatr. Nephrol. 2004; 19: 111“13.
12. Hopkin, R. J. & Grabowski, G. A. Lysosomal storage diseases. In Kasper, 37. Shenhav, S., Gemer, O., Sassoon, E. & Segal, S. Acute intermittent porphyria
D. L., Braunwald, E., Fausi, A. C. et al. (eds.), Harrison™s Principles of precipitated by hyperemesis and metoclopramide treatment in pregnancy.
Acta Obstet. Gynecol. Scand. 1997; 76: 484“5.
Internal Medicine, 16th edn. New York, NY: McGraw-Hill Companies, Inc,
2005, pp. 2315“19. 38. Soriano, D., Seidman, D. S., Mashiach, S., Sela, B. A. & Blonder, J. Acute
13. Beutler, E. & Gelbart, T. Gaucher disease mutations in non-Jewish patients. intermittent porphyria first diagnosed in the third trimester of pregnancy.
Br. J. Haematol. 1993; 85: 401“5. Case report. J. Perinatal Med. 1996; 24: 185“9.
39. Andersson, C., Innala, E. & Backstrom, T. Acute intermittent porphyria in
14. Gillis, S., Hyam, E., Abrahamov, A., Elstein, D. & Zimran, A. Platelet
function abnormalities in Gaucher disease patients. Am. J. Hematol. women: clinical expression, use and experience of exogenous sex hormones.
1999; 61: 103“6. A population-based study in northern Sweden. J. Int. Med. 2003; 254: 176“83.

Chapter 13

40. Brodie, M. J., Moore, M. R., Thompson, G. G., Goldberg, A. & Low, R. A. 48. Brennan, L., Halfacre, J. A. & Woods, S. D. Regional anaesthesia in por-
Pregnancy and the acute porphyrias. Br. J. Obstet. Gynaecol. 1977; 84: 726“31. phyria. Br. J. Anaesth. 1990; 65: 594.
41. Kanaan, C., Veille, J. C. & Lakin, M. Pregnancy and acute intermittent 49. Shenhav, S., Gemer, O., Sassoon, E. & Segal, S. Acute intermittent porphyria
porphyria. Obstet. Gynecol. Surv. 1989; 44: 244“9. precipitated by hyperemesis and metoclopramide treatment in pregnancy.
42. Anderson, K. E., Bloomer, J. R., Bonkovsky, H. L. et al. Recommendations for Acta Obstet. Gynecol. Scand. 1997; 76: 484“5.
the diagnosis and treatment of the acute porphyrias. Ann. Int. Med. 2005; 50. Kanbak, M. Ketamine in porphyria. Anesth. Analg. 1997; 84: 1395.
142: 439“50. 51. Durmus, M., Turkoz, A., Togal, T. et al. Remifentanil and acute intermittent
43. Porphyria Educational Services. European Porphyria Initiative. www.por- porphyria. Eur. J. Anaesthesiol. 2002; 19: 839“40. 52. Evans, P. R., Graham, S. & Kumar, C. M. The use of sevoflurane in acute
44. Milo, R., Neuman, M., Klein, C., Caspi, E. & Arlazoroff, A. Acute intermittent intermittent porphyria. Anaesthesia 2001; 56: 388“9.
porphyria in pregnancy. Obstet. Gynecol. 1989; 73: 450“2. 53. Messmer, M., Gerheuser, F. & Forst, H. Desflurane in acute intermittent
45. Consolo, D., Ouardirhi, Y., Wessels, C. & Girard, C. Obstetrical anaesthesia porphyria. Anaesthesist 2004; 53: 244“8.
and porphyrias. Ann. Fr. Anesth. Reanimat. 2005; 24: 428“31. 54. The drug database for acute porphyria.
46. McNeill, M. J. & Bennet, A. Use of regional anaesthesia in a patient with 55. Preece, M. A. & Green, A. Pregnancy and inherited metabolic disorders:
acute porphyria. Br. J. Anaesth. 1990; 64: 371“3. maternal and fetal complications. Ann. Clin. Biochem. 2002; 39: 444“55.
47. Kantor, G. & Rolbin, S. H. Acute intermittent porphyria and caesarean 56. Lee, P. J. Pregnancy issues in inherited metabolic disorders. J. Inherit.
delivery. Can. J. Anaesth. 1992; 39: 282“5. Metab. Dis. 2006; 29: 311“16.

M. J. Paech and K. Scott

Liver disease cirrhosis, portal hypertension, acute liver failure, or hepatic rup-
ture pose major anesthetic challenges, while many uncommon
Effects of pregnancy on the liver
conditions involving the liver confer minimal or no risk to mother
Pregnancy induces anatomic, physiologic, and functional or fetus. These relatively benign disorders include the hyper-
changes in the liver because of an increase in serum estrogen bilirubinemias, which are characterized by elevations of un-
and progesterone. These changes reverse postpartum, but can conjugated bilirubin (Gilbert disease) or conjugated bilirubin
cause diagnostic difficulties during pregnancy if liver disease is (Dubin-Johnson and Rotor syndromes). Bilirubin concentrations
present. For example, spider nevi and palmar erythema are stig- rise during pregnancy in about 50% of women affected by these
mata of liver disease, but may be seen in some pregnant women disorders, but fetal outcomes are good. Several multisystem dis-
in response to increased estrogen levels. eases involve the liver, including preeclampsia, systemic lupus
In normal pregnancy, liver size does not change significantly so erythematosus, and hemachromatosis.
hepatomegaly suggests liver disease. Hepatic blood flow remains
unchanged, despite the physiologic increase in blood volume and
Viral hepatitis
cardiac output. In fact, the portion of cardiac output delivered to
the liver falls by 35%. There is increased splanchnic, portal, and Viral hepatitis (see Table 14.4) is the most common cause of
hepatic dysfunction and jaundice in pregnancy.1,2,3,4,5,6,7 In addi-
esophageal venous pressure in late pregnancy, and 60% of
healthy women will develop esophageal varices that resolve post- tion to hepatitis A, B, C, D, E, and G viruses, a number of other
partum. Clearance of drugs dependent on hepatic blood flow is viruses cause hepatitis during the acute systemic infection phase,
reduced because of a larger volume of distribution. especially in immunosuppressed patients. These other viruses
Serum albumin concentration falls by up to 60% secondary to include herpes simplex virus (HSV), which is more likely to
an increase in plasma volume, leading to a fall of total serum cause fulminant hepatitis in the pregnant patient than in the
protein by 20% in mid-pregnancy. Serum globulins alter slightly, nonpregnant patient, cytomegalovirus, and Epstein Barr virus.
with an increase in a and b fractions, and a-fetoprotein concen- Herpes simplex virus hepatitis causes a prodromal illness with
tration, but a reduction in g-globulin. There is increased produc- fever, oropharyngeal or genital lesions, coagulopathy, and very
tion of fibrinogen and factors VII, VIII, IX, X and von Willebrand high serum aminotransferase, but near normal bilirubin levels.
factor. Ceruloplasmin and transferrin levels increase, as do sev- Treatment with acyclovir is appropriate and results are encoura-
ging.6 Health care workers are at risk of contracting hepatitis,
eral specific binding proteins (e.g. thyroxine, vitamin D, and
corticosteroids). There are minor changes in porphyrin metabo- especially hepatitis B virus (HBV), hepatitis C virus (HCV), and
lism. Serum bilirubin tends to fall because of hemodilution and a hepatitis D virus (HDV), through blood contact, so the application
lower albumin concentration, so an increase in serum bilirubin of universal precautions is essential.
suggests liver disease.
Serum triglycerides rise progressively to term, as do very-low Hepatitis A
density lipoproteins and serum cholesterol. Serum alkaline phos- Hepatitis A virus (HAV) infection varies in prevalence geo-
phatase (ALP) increases four-fold by the third trimester and graphically and is endemic in Africa, Asia, and Central America.
returns to normal by three weeks postpartum. Serum lactate Transmission is fecal“oral and most infections are asymptomatic
dehydrogenase (LDH) is normal or marginally increased, whereas or subclinical, but serum AST and ALT levels are elevated.
serum g-glutamyl-transpeptidase (GGT) declines slightly and Immunoglobin M anti-HAV is present in acute infection and
50 nucleotidase increases slightly. Serum aspartate aminotrans- since HAV clears after a few weeks, acquired immunity (anti
ferase (AST) and alanine aminotransferase (ALT) remain within HAV IgG-positive serostatus) is achieved.
the normal range (see Table 14.1).1,2 Hepatitis A virus affects 1 in 1000 pregnant women in the USA.
The clinical presentation in pregnancy differs in that pruritus is
more common, because of high estrogen levels. Treatment is
Causes of liver disease in pregnancy
supportive. Both inactivated vaccine and postexposure immuno-
Various congenital and acquired liver diseases may present globulin prophylaxis are safe during pregnancy. Although vertical
during pregnancy (see Tables 14.2 and 14.3). The most common transmission has not been described, immunoglobulin may be
is viral hepatitis, but a number of uncommon diseases unique given to the neonate and close household contacts, and breast-
feeding should be encouraged.3,4,5,6,7
to pregnancy are important causes of mortality. Women with

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.
4 Metabolic disorders

include cirrhosis, liver failure, and hepatocellular carcinoma. The
Table 14.1 Liver function tests in normal pregnancy prevalence of HCV varies geographically from 1 to 6%, and areas
with high endemic rates include Asia, Middle East, Africa, and
Pregnancy Trimester of
southern and eastern Europe. Hepatitis C virus infection is
Test effect maximum change
increasing in other countries, often from intravenous drug use
# 20“60%
albumin 2
(IVDU), blood transfusion, incarceration, tattooing, body pier-
slight " 3
cing, and organ transplantation. Chronic hepatitis develops in
slight " 3
70% of infected individuals, most of whom remain asymptomatic.
nil to slight # 3
Of those, 20% will develop cirrhosis within 40 years of viral acqui-
# 50%
fibrinogen 2
sition and a small number will develop hepatocarcinoma.4,5,6,7
ceruloplasmin 3
The risk of sexual transmission is low, but vertical transmis-
transferrin 3
sion from mother to fetus occurs in 6% of women who are HCV
bilirubin nil “
polymerase chain reaction (PCR) positive. Quantitative HCV-
2“4 ‚ "
alkaline phosphatase (ALP) 3
RNA testing is a marker of the risk of vertical transmission,
nil or slight # “
and more often positive in the presence of coinfection with
nil or slight "
lactate dehydrogenase 3
human immunodeficiency virus (HIV) and certain HCV geno-
aspartate aminotransferase
types.5,7 As with HIV, transmission occurs mainly at delivery,
(AST) nil “
through contact with contaminated vaginal secretions. There is
alanine aminotransferase (ALT) nil “
no association between vertical transmission of HCV and gesta-
nil or slight "
5 nucleotidase “
tional age at delivery or the presence of chorioamnionitis.7
bile acids nil “
Elective cesarean section (C/S) is not necessary to reduce viral
2“3 ‚ "
triglyceride 3
transmission in HCV infected women. However, HCV/HIV-coin-
cholesterol 3
fected women may require C/S based on HIV status (see
Chapter 18). Both HBV and HCV are detectable in human milk
but breast-feeding is considered safe as long as there is no coin-
fection with HIV.7 Surveillance of the neonate is by HCV antibody
Hepatitis B
screen at 12 months (maternal antibody detectable for 18 months)
Hepatitis B virus is a highly infectious double-stranded enveloped
or HCV-RNA by PCR within the first few months of life. There is
virus transmitted by cutaneous (especially needle sharing) or
no vaccine or immunoglobulin to prevent spread of the disease.
mucosal exposure, sexually, and vertically from mother to fetus.
During pregnancy, liver function tests often return to normal.
Most acute infections are subclinical, but nausea, vomiting,
The treatment of HCV includes drugs such as interferon and
abdominal pain, and jaundice may occur. During the acute
ribavirin, although in most cases these fail to clear the infection.
phase, the diagnosis is made by detection of HBV surface antigen
These drugs are only appropriate after pregnancy because they
in the serum or other secretions and is confirmed by detection
are teratogenic and have serious adverse effects.
of IgM antibodies to HBV core antigen. Hepatitis e (envelope)
antigen is also present and as anti-HBVe antibody develops,
Hepatitis D, E, and G
patient infectivity decreases, but infectivity remains when
Hepatitis D virus is a single-stranded RNA virus that depends on
HbsAg is present. About 5% of patients develop chronic infection.
the presence of HBV for replication. Infection is more common
Acute HBV complicates 1 in 500“1000 pregnancies in the USA,
in IVDU and is endemic in some areas, e.g. southern Italy.6
and chronic HBV infection is present in up to 1.5% of pregnant
Coinfection with HDV and HBV is associated with more severe
women. The presence of HbsAg does not pose additional risk for
disease and a higher chronicity rate than HBV infection alone. Up
the pregnancy. Interferon, used for those with active disease and
to 80% of coinfected patients develop cirrhosis more rapidly than
liver damage, has many side effects and patients taking interferon
seen with HBV infection alone. Infection is rare in pregnant women
should not become pregnant. Transplacental transmission of
and children, which suggests vertical transmission is uncommon.
HBV occurs in early pregnancy occasionally, but most perinatal
Hepatitis E virus (HEV) is a single-stranded, nonenveloped RNA
transmission takes place intrapartum. The risk of mother-to-child
virus, endemic in some parts of Africa, India, and Mexico. It
transmission increases as pregnancy advances, increasing from
shares similarities with HAV and is spread via the fecal“oral
10% in the second trimester to 90% in the third trimester in
route. Acute infection in pregnancy, especially the third trimester,
HBVeAg positive women. Passive and active immunization is
can cause fulminant hepatitis with 20% mortality. Vertical intra-
highly effective in preventing neonatal transmission, and chil-
partum transmission occurs in 50“100%, conferring a high risk of
dren born to mothers at high risk can be vaccinated.
neonatal hepatic failure.5,6,7
Hepatitis C Hepatitis G virus (HGV) can only be detected by reverse-
transcriptase PCR and the epidemiology parallels that of HIV and
Hepatitis C is the most common chronic bloodborne infection in
the USA accounting for 20% of acute viral hepatitis.7 Hepatitis C other hepatitis viruses. Transmission is similar to HCV, but signifi-
cant liver disease is unlikely. Vertical transmission occurs in 60% of
virus produces a six- to nine-week illness that is often subclinical
cases, without detriment to the infant.6
or mild. Chronic infection is common and long-term sequelae

Chapter 14

Table 14.2 Liver diseases unique to pregnancy (excluding preeclampsia)

Disorder Key clinical features Obstetric implications Anesthetic implications

Acute fatty liver of 1. Third trimester malaise, nausea, 1. Intensive fetal 1. Optimal medical management;
pregnancy (AFLP) abdominal pain, and, later, jaundice. monitoring. invasive monitoring.
2. Hypoglycemia, metabolic acidosis, 2. Early delivery mandatory. 2. Plan for C/S.
coagulopathy, liver and hepatorenal 3. Correct coagulopathy and use
failure. regional if not contraindicated.
3. Preeclampsia in 40%. 4. Prepare for peripartum or
4. Intensive supportive care including postpartum hemorrhage.
dextrose, antibiotics, vitamin K. 5. Use anesthetic and analgesic drugs
Expedite delivery. appropriate to severe hepatic
5. Complete postpartum resolution. dysfunction.

Intrahepatic cholestasis 1. Late pregnancy pruritus, then malaise 1. Monitor fetal status. 1. Monitor liver function and
of pregnancy (IHCP) and mild jaundice. 2. Preterm delivery, C/S and coagulation.
2. Treat pruritus with ursodeoxycholic poor fetal outcome 2. Prepare for C/S, postpartum
acid. common. hemorrhage.
3. Rapid postpartum resolution.

Hyperemesis 1. Severe vomiting and dehydration in 1. Increased risk of early fetal 1. Optimal antiemetic therapy.
gravidarum early pregnancy. loss but otherwise good 2. Correct fluid and electrolyte
2. Minor liver function abnormalities. outcomes. imbalance.
3. Occasionally severe complications
as a result of vomiting.

Table 14.3 Rare liver diseases

Disorder Key clinical features Obstetric implications Anesthetic implications

Wilson disease 1. Excessive copper deposition in liver and 1. Subfertile unless well controlled. 1. Monitor liver function; coagulation;
brain, causing hepatic dysfunction and esophageal varices; bulbar
motor or psychiatric disturbance. involvement; and drug effects.
2. Chelators or binding agents to reduce 2. Continued treatment to 2. Use anesthetic drugs appropriate
copper levels. Zinc recommended. avoid relapse. for patients with severe liver
3. Prepare for postpartum hemorrhage.

Budd-Chiari 1. Hepatic vein obstruction causing 1. Consult a hepatic physician. 1. Monitor liver function, coagulation.
syndrome ascites, hepatomegaly, and liver failure.
2. Coagulopathy common. 2. Anticoagulation and portocaval 2. Use anesthetic drugs appropriate
shunt may be required. for patients with severe liver
3. May be associated with prothrombotic 3. Poor maternal and fetal prognosis. dysfunction.
and other disorders.

Primary biliary 1. Variable presentation from asymptomatic 1. Pregnancy and the fetus 1. Monitor liver function and
cirrhosis to cirrhosis. unaffected if well-compensated coagulation.
2. Diagnosis based on mitochondrial disease. 2. Use regional block if possible and
antibodies and liver biopsy. anesthetic drugs appropriate to severe
liver dysfunction.

Anesthetic implications of viral hepatitis dark urine in most cases. In addition, the patient may have nau-
sea, fever, and abdominal pain. There may be mild anemia, vita-
The onset of inflammatory hepatocytic disease can be gradual or
min K deficiency, lymphocytosis, and coagulation abnormalities.
sudden with incubation periods from 2“24 weeks. Clinically the
Fulminant hepatic failure is uncommon (the incidence is the
patient may be anorexic, jaundiced, fatigued, and will demonstrate

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