LINEBURG


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Anticonvulsants such as gabapentin (100 mg up to 1800 mg per sidered invasive as radiation may affect the fetus with the greatest
day) or pregabalin (75 mg up to 300 mg per day) can be added; fetal risk occurring between the eighth and fifteenth weeks of
however, many patients report side effects such as dizziness and gestation. Irradiation at this time can result in a reduction in
nausea. Therefore, it is important to begin with a low dose of intelligence quotient (IQ), an increased risk of cancer in later
gabapentin and slowly increase it (Lavand™ homme, personal life, an increased incidence of congenital abnormalities, and
a possible increased genetic risk to the next generation.94
communication, 2005). It seems likely that a multimodal
approach to postoperative pain control is the most rational and Generally, this risk will be very small compared with the normal
risks of pregnancy.94
effective option, with the added benefit of reducing individual
drug dosages and side effects. Although regional analgesic tech- Before a radioactive agent is administered to a mother who
niques form the basis of postoperative pain management, current is breast-feeding, consideration should be given as to whether:
evidence indicates that combining them with NSAIDs is more (1) the test could reasonably be delayed until the mother has
effective.89 Some studies have suggested that small doses of keta- stopped breast-feeding; and (2) the most appropriate radiophar-
maceutical has been chosen.94
mine enhance opioid analgesia, prevent tolerance to opioids, and
improve pain relief as well as reducing hyperalgesia.79

Summary
Uncommon chronic pain syndromes
Evidence suggests that inadequate relief of postoperative pain
Tarshis et al. reported a case of a pregnant patient with chronic may result in harmful physiological and psychological conse-
quences that lead to significant morbidity and mortality,95 a
pain secondary to hereditary chronic pancreatitis who was treated
with spinal morphine 3 mg/day through an implanted intrathecal delay in recovery, and a slower return to daily living. In addition,
pump. When labor began, the patient™s pain was very difficult to the presence of postoperative symptoms, including pain, signifi-
manage with intravenous drugs; however, after initiating epidural cantly contribute to patient dissatisfaction with the anesthetic
analgesia, pain management was optimal.90 With increasing use and surgical experience.96 Most importantly, it has been recog-
of intrathecal opioids for nonmalignant pain, it is likely that the nized that inadequately treated postoperative pain may lead to
chronic pain.5 These factors have not been well evaluated in the
anesthesiologist will encounter these patients more frequently.
Careful multidisciplinary planning can optimize pain manage- obstetrical field, but recent research has shown that women at
ment in the perioperative or peripartum period. Apparently, epi- risk from more severe acute postcesarean pain can be predicted
from various preoperative physical and psychological tests.97
dural analgesia can be used safely and effectively for labor
analgesia in this population. These tests, which include thermal pain thresholds and the
Pyke et al. reported a case of recurrence of pregnancy-induced State Trait and Anxiety Inventory, have yet to be used to deter-
intercostal neuralgia. The pain was related to stretching of the mine who is likely to develop chronic pain. However, it is



235
3 Nervous system disorders


reasonable to conclude that pain states both during and after 21. Holroyd, K. A. & Lipchik, G. L. Sex differences in recurrent headache dis-
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238
SECTION 4: METABOLIC DISORDERS

13 DISORDERS OF INTERMEDIARY METABOLISM
Stephen Halpern and Bhadresh Shah



Introduction debilitated and require walking aids or a wheelchair in early
adult life. Some require respiratory support for at least part of
There are a large number of inherited conditions that result from
the day.2 Obstructive sleep apnea may also be present.3 Thus,
disorders of intermediary metabolism. These cause symptoms
careful preoperative assessment of respiratory function, includ-
because of the build up of precursors, the absence of the final
ing arterial blood gas analysis and pulmonary function testing, is
product, an excess of toxic intermediaries, or a combination of
necessary before deciding on anesthetic management for surgery
these mechanisms. Many are fatal in childhood, but some are
during pregnancy. Specific enzyme replacement therapy may be
compatible with adult life and pregnancy. These disorders may be
beneficial4 when it becomes widely available. Currently, there are
encountered by an obstetric anesthesiologist (either because of
no case reports of parturients with Pompe disease.
their prevalence or, for some rare conditions, because modern
McArdle disease or Cori type V is a rare autosomal recessive
management confers an improved chance of fertility). Some
hereditary myopathy due to a deficiency of glycogen myopho-
examples of these disorders can be found in Table 13.1.
sphorylase, which is required for the conversion of glycogen to
Malignant hyperthermia, plasma pseudocholinesterase defi-
lactate during anerobic exercise.
ciency, and inherited hematological, endocrine, connective tis-
Although more prevalent in males, affected females are other-
sue, or bone disorders are discussed elsewhere.
wise healthy and, as fertility is unaffected, pregnancy is likely. The
In all these rare disorders, a basic tenet of management is to
accumulation of glycogen in skeletal muscle produces symptoms
refer to a specialist in the relevant field of medicine, to coordinate a
of muscle fatigue and cramping during exercise. The diagnosis is
multidisciplinary team approach and to make a thorough and
usually made in early adulthood and affected individuals, who
early antenatal assessment, with documentation of a plan of
may find exercise levels at which they remain asymptomatic,
management.
lead a normal existence. Symptoms can be reduced by glucose
or fructose ingestion before exercise. A history of myoglobinuria is
usually present after heavy exercise, but rarely precipitates acute
Glycogen storage diseases
renal failure. The myocardium and myometrium are usually unaf-
Glucose metabolism plays a fundamental role in supplying fected, although one case with an abnormal cardiac conduction
pathway5 and another with a familial cardiomyopathy have been
energy for most cellular metabolic processes. Glycogen, the stor-
reported.6 The liver is normal and muscle wasting is uncommon,
age form of glucose, is composed of a branched polymer of
glucose residues and can be found in muscle and liver. Defects except in older patients who may exhibit upper limb wasting.
in glycogen metabolism typically cause accumulation of glycogen Diagnosis is based on assay of serum lactate (with failure to rise),
in the tissues.1 Historically, the glycogen storage diseases have pyruvate, muscle enzymes, and myoglobin during ischemic exer-
been classified numerically according to the specific enzyme cise testing. Confirmation is by muscle biopsy and genetic studies.
defect. Since the main manifestations are either primarily related
to liver or muscle, it may be more useful to categorize them
Obstetric and anesthetic implications
according to the primary organ involved. Unlike some disorders
of lipid metabolism, many of these disorders are responsive to Pregnancy in women with McArdle disease has been described
and appears relatively uneventful.7 Regional anesthesia likely
dietary therapy.
Pompe disease or Cori type II glycogen storage disease is one of confers its usual advantages. Successful, uneventful epidural
a group of genetic disorders involving pathways for the synthesis, anesthesia for cesarean section (C/S) and postoperative analgesia
storage, and utilization of glycogen. Those enzyme deficiencies has been reported in the first pregnancy of a woman with
McArdle disease.6 General anesthesia for C/S has also been
related to liver conversion of glycogen to glucose are classified
described in the same woman.6 On theoretical grounds, succinyl-
hepatic-hypoglycemic forms, and those related to muscle conver-
sion of glycogen to lactate form the muscle-energy group. Pompe choline should be avoided due to the risk of myoglobinemia,
disease, one of the latter, is a deficiency of a-glucosidase and has myoglobinuria, and possible renal failure. Response to nondepo-
an incidence of about 1 in 100 000.1 It is often misdiagnosed as larizing muscle relaxants appears to be normal.6,7 A modified
muscular dystrophy although a specific biochemical test is avail- rapid sequence induction using a nondepolarizing drug, with
able to help differentiate the two. Cardiomyopathy is not a feature monitoring of neuromuscular block, would seem appropriate.
of the disease. Weakness, fatigue, and muscle cramps and back Rocuronium is probably the preferred agent, because of its
pain are common initial complaints. Patients may become rapid onset and intermediate duration, although atracurium


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



Table 13.1 Examples of metabolic diseases caused by intermediary metabolic defects

Obstetric and anesthetic
Category Examples Key features implications

Lysosomal Gaucher Liver and spleen enlargement; Anticipate postpartum hemorrhage;
storage thrombocytopenia; pulmonary thrombocytopenia; platelet
diseases abnormalities; skeletal deformities. function defect; pulmonary
hypertension.
Glycogen Liver Von Gierke; Forbes; Growth retardation; enlarged liver and Successful pregnancies have been
storage Cori I and III kidneys; hypoglycemia; elevated reported.
diseases blood lactate, cholesterol, Avoid hypoglycemia. Coagulation
triglycerides and uric acid; bleeding screen.
diathesis.
Muscle McArdle; Pompe; Tarui Exercise intolerance; muscle cramps; Theoretical contraindication to
myoglobinuria. succinylcholine. Minimize
shivering.
Perioperative blood glucose control.
Frequent use of noninvasive blood
pressure cuff measurements may
lead to muscle cramping (see text).
Disorders of Phenylalanine Phenylketonuria Mental retardation (if not treated No anesthetic implications. Diet
amino acid in the neonatal period). control is important for neonatal
metabolism outcome.
Homocystine Homocystinuria Marfanoid features; lens dislocation; Thrombosis. Pregnancies
mental retardation; skeletal complicated by preeclampsia.
abnormalities.


was used in the case cited above. While there is no known link Pregnancy in patients with von Gierke disease (Cori type Ia) has
between McArdle disease and malignant hyperthermia, some been described but is rare. The main challenge is to maintain
patients may have a positive in vitro contracture test.8 While euglycemia. This is often accomplished with supplementary
cornstarch feeding.10 Pregnancy has also been described follow-
these authors suggest that potent inhalational agents, neuroleptics,
and sympathomimetic agents (such as ketamine) be avoided, there ing combined renal-hepatic transplantation in a woman with von
is currently insufficient information to endorse these recommen- Gierke disease.
dations. The risk of compromised postoperative respiratory func- Tarui disease (Cori type VII) is caused by phosphofructokinase
tion secondary to myopathy is low in the reproductive age group. deficiency. The disease is phenotypically similar to McArdle dis-
The use of tourniquets or frequent repeated noninvasive blood ease and Forbes disease since patients suffer from clinically sig-
pressure (BP) recordings is inadvisable since repeated use of an nificant muscle weakness. To date there are no clinical reports of
automated BP device has precipitated muscle cramps.7 The use of this disease associated with pregnancy.
a manual BP cuff may reduce tourniquet time. Alternatively, an Tarui disease can present as acute renal failure and hemolytic
arterial line may be used if there is a specific need for tight BP anemia in affected women of childbearing age. This may be the
control. Pyrexia, hypothermia, and shivering should all be result of rhabdomyolysis following strenuous exercise. Diagnosis
can be confirmed by 31P-magnetic resonance spectroscopy.11
avoided because of poor temperature compensatory mechan-
isms and the risk of myoglobinemia with severe shivering. The
perioperative administration of intravenous (i.v.) dextrose as a
Lysosomal storage disorders
substrate has also been recommended, but requires titration to
maintain normoglycemia, since the fetal and neonatal conse- Lysosomes are cytoplasmic organelles, which enclose an acid
quences of maternal hyperglycemia must be considered. environment and contain enzymes that hydrolyze macromole-
Cori disease (type III), also known as Forbes disease or cules. The diseases discussed involve mainly single gene defects
Debrancher enzyme deficiency is a rare hepatic-hypoglycemic affecting one or more lysosomal enzymes, leading to accumulation
of substrate. Most are inherited as autosomal recessive disorders.12
form of glycogen storage disease, which in mild cases may
first present in adulthood with a myopathy similar to McArdle
disease.1 Important additional anesthetic considerations are the
Gaucher disease
high risk of hypoglycemia with fasting, and the presence of car-
diac myopathy. In severe cases, hypoglycemia must be prevented Gaucher disease, the most common sphingolipidosis, is caused by
in order to preserve pregnancy.9 deficiency of the lysosomal enzyme glucocerebrosidase. Adult



240
Chapter 13


Gaucher disease is one of three forms of Gaucher disease. It is is safe, it may be considered in the absence of other laboratory
marked by absence of neurological involvement by virtue of the evidence of coagulopathy or history of abnormal bleeding.
presence of the common 1226G (N370S) mutation. It is especially Pulmonary involvement in Gaucher disease may be life threat-
prevalent among the Ashkenazi Jewish population, with a disease ening. Although it usually correlates with disease severity,
frequency of about 1:850 live births. It is present in other ethnic enzyme replacement therapy does not seem to relieve symptoms.
groups with a disease frequency between 1:40 000 to 1:60 000 in the Some authors have recommended that patients on enzyme repla-
general population.13 There is tremendous heterogeneity in the cement therapy should have an echocardiogram to assess the
severity of pulmonary hypertension.19
severity of clinical manifestations of type I disease, ranging from
asymptomatic to patients who experience lifelong debilitating
disease.
Mucolipidoses II
Most patients present with enlargement of the spleen and
liver, thrombocytopenia, and anemia. In some, splenectomy is
Mucolipidoses II is a rare disorder presenting with progressive
required because of the size of the spleen and to reduce the
skeletal deformities from the first decade and mild mental retar-
severity of thrombocytopenia. Liver function tests are rarely
dation. Survival to adult life is common in females.12 Its specific
affected. Platelet function defects, unrelated to absolute num-
importance to the anesthesiologist lies in the likelihood of aortic
bers of platelets, may be more common than previously appre-
and mitral valvular disease. To date, there have been no reported
ciated with 22% of patients having defects in platelet
cases during pregnancy.
aggregation, response to ADP, collagen, and/or epinephrine.14
Skeletal involvement may include osteopenia, avascular necrosis
of the large joints, pathological fractures, and lytic lesions.
Disorders of amino acid metabolism and storage
Visceral involvement may include lung parenchymal disease,
Phenylketonuria

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