C H A P T E R

20
The Geriatric Patient
STEVEN J. SCHWARTZ, MD  n
FREDERICK E. SIEBER, MD  n

n Perioperative considerations in myeloid metaplasia with

Huntington's Chorea (Huntington's Disease)
Amyloidosis
Idiopathic Pulmonary Fibrosis
Polycythemia Vera
Essential Thrombocythemia
Myeloid Metaplasia with Myelofibrosis
Polymyalgia Rheumatica
Goodpasture's Syndrome
Male Breast Cancer
Primary Hepatic Lymphoma
Creutzfeldt-Jakob Disease
KEY POINTS
n Patients with Huntington's disease are at higher risk of
pulmonary aspiration, altered anesthetic pharmacology,
and worsening generalized tonic spasms. Rapid-sequence
induction with cricoid pressure is recommended for
­general anesthesia.
n Autonomic dysfunction from amyloidosis has dramatic
perioperative ramifications. Administering ­ anesthetics
to patients with amyloidotic polyneuropathy risks sig-
nificant hypotension; depolarizing muscle relaxants are
controversial.
n In patients with idiopathic pulmonary fibrosis, anesthetic
evaluation focuses on degree of disease progression and
available pulmonary reserve.
n Uncontrolled polycythemia vera is associated with
a high risk of perioperative bleeding and postopera-
tive thrombosis, requiring aggressive disease control
preoperatively.
n Elderly patients with essential thrombocytosis are at high
risk for thrombotic and hemorrhagic complications.
Perioperative management of the patient with essential
thrombocythemia focuses on whether to normalize the
platelet count.
myelofibrosis include careful platelet count monitoring and
attention to hemorrhagic complications.
n In patients with polymyalgia rheumatica, preoperative
­history and physical examination focus on the airway and
symptoms related to systems affected by rheumatoid arthri-
tis (neurologic, pulmonary, cardiovascular). Advanced
planning is anticipated for identified or potentially difficult
intubation.
n In Goodpasture's syndrome, patients at risk for perioper-
ative renal failure have pre-existing renal insufficiency or
diabetes or require contrast procedures. ­Dialysis-dependent
patients require careful coordination of dialysis and ­elective
surgery.
n Preoperative evaluation of male patients with breast can-
cer is similar to that of female breast cancer patients and
should focus on the heart, lungs, and neurologic and hema-
tologic systems.
n In patients with primary hepatic lymphoma, degree of
hepatic dysfunction and infection risk must be determined
preoperatively.
n In patients with suspected Creutzfeldt-Jakob disease, any
tissue or body fluid is potentially infectious, and precau-
tions should be taken to prevent transmission.
Elderly surgical patients are subject to many of the same rare
diseases seen in younger populations. The focus in this c­ hapter
is on several uncommon diseases that are more unique to aged
individuals.
HUNTINGTON'S CHOREA (HUNTINGTON'S
DISEASE)
Huntington's chorea is a rare, autosomal dominant, inher-
ited degenerative disorder of the nervous system with an inci-
dence of 5 to 10 per 100,000 population. It is characterized
by the clinical hallmarks of progressive chorea and dementia.

573

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 574 ANESTHESIA AND UNCOMMON DISEASES
The onset is usually in the fourth or fifth decade of life, but
there is a wide range in age at onset, from childhood to late
life (>75 years). Symptoms appear to worsen progressively
with age.
Pathophysiology. Huntington's disease is an a­utosomal
dominant disorder with complete penetrance. The H ­ unting­ton's
disease gene, IT15, is located on chromosome 4p, contains
CAG-trinucleotide repeats, and codes for a protein called
huntingtin. The protein is found in neurons throughout the
brain; its normal function is unknown. Transgenic mice with
an expanded CAG repeat in the Huntington's disease gene
develop a progressive movement disorder. It is a basal ganglia
disease; caudate and putamen are the regions most severely
affected. The most significant neuropathologic change is a
preferential loss of medium-spiny neurons in the neostriatum.
Neurochemically, there is a marked decrease of γ-aminobutyric
acid (GABA) and its synthetic enzyme glutamic acid decar-
boxylase throughout the basal ganglia, as well as reductions
of other neurotransmitters (e.g., substance P, enkephalin). The
movement disorder is slowly progressive and may eventually
become disabling.
Diagnosis and Differential. The DNA-repeat expansion
forms the basis of a diagnostic blood test for the disease gene.
Patients having 38 or more CAG repeats in the Huntington's
disease gene have inherited the disease mutation and will
eventually develop symptoms if they live to an advanced age.
Each of their children has a 50% risk of also inheriting the
abnormal gene; a larger number of repeats is associated with
an earlier age at onset. Huntington's can also be diagnosed by
caudate atrophy on magnetic resonance imaging (MRI) in the
context of an appropriate clinical history.
Differential diagnosis of Huntington's disease includes
other choreas, hepatocerebral degeneration, schizophrenia
with tardive dyskinesia, Parkinson's disease, Alzheimer's dis-
ease, and other primary dementias and drug reactions.
Preoperative Preparation. Even though memory in
patients with Huntington's chorea is frequently not impaired
until late in the disease course, attention, judgment, aware-
ness, and executive functions may be seriously deficient
at an early stage. Depression, apathy, social withdrawal,
­irritability, ­fidgeting, and intermittent disinhibition are com-
mon. Delusions and obsessive-compulsive behavior may
occur. These signs, along with poor articulation of speech,
make preoperative evaluation and obtaining consent arduous
tasks. Characteristic choreoathetoid movements, along with
­frequent, irregular, sudden jerks and movements of any of the
limbs or trunk, make physical examination, as well as regional
anesthesia, difficult to perform.
Cachexia and frailty may be observed in the elderly
Huntington's patient. Pharyngeal muscle involvement leads
to dysphagia and makes these patients susceptible to pulmo-
nary aspiration.1 Before elective surgery, it is important to rule
out ongoing aspiration pneumonitis or pneumonia by careful
physical examination and chest radiography.
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There is no specific treatment to stop progression of
Huntington's disease, but the patient's movements and
behavioral changes may partially respond to phenothiazines,
haloperidol, benzodiazepines, or olanzapine. Selective sero-
tonin reuptake inhibitors (SSRIs) may help with associated
depression.
ANESTHETIC CONSIDERATIONS
Major concerns in anesthetic management of Huntington's
disease are potential difficult airway, sleep apnea, risk of aspi-
ration, and altered reactions to various drugs. A ­difficult
airway may result from a rigid, stiff, unstable posture with
­hyperextension of the neck. Sleep apnea may also be present.
It is controversial whether the pharmacology of a­nesthetic
agents is altered in Huntington's disease. Authors have
reported a decrease in plasma cholinesterase activity and a
prolonged effect of succinylcholine.2 In addition, patients may
have an exaggerated response to sodium thiopental3 or mid-
azolam.4 On the other hand, both thiopental5 and succinyl-
choline5,6 have been used safely in Huntington patients. Other
agents used safely include propofol7 and sevoflurane.5 The
safety profile and pharmacokinetics of the nondepolarizing
muscle relaxants mivacurium and rocuronium are similar to
those in patients without Huntington's disease.5,8,9
It is generally recommended that rapid-sequence or
modified rapid-sequence induction with cricoid pressure
be used for induction of general anesthesia in Huntington
patients. Others suggest a total intravenous anesthesia (TIVA)
­technique to reduce the risk of postoperative shivering related
to inhalational agents and thus avoid initiating generalized
tonic spasms.
AMYLOIDOSIS
Amyloidosis results from the deposition of insoluble, f­ ibrillar
proteins (amyloid), mainly in the extracellular spaces of organs
and tissues in amounts sufficient to impair normal func-
tion. Amyloid fibrils can be deposited locally or may involve
­virtually every organ system of the body. Symptoms and signs
depend on the organs and tissues involved.
Pathophysiology. The cause of amyloid production and its
deposition in tissues is unknown. All amyloid fibrils share an
identical secondary structure, the β-pleated sheet conforma-
tion. The polypeptide backbone of these protein precursors
assumes similar fibrillar morphology that renders them resis-
tant to proteolysis. The amyloidoses have been classified into
many subtypes,10 based on the amyloid protein involved. The
name of the amyloidosis subtype uses the capital letter A as
the first letter of designation and is followed by the protein
­designation. Three major types of amyloid and several less
common forms have been defined biochemically.
Whether an amyloidosis is systemic or localized (organ
limited) depends on the biochemical structure of the amy-
loid protein. Systemic amyloidoses include biochemically
distinct forms that are neoplastic, inflammatory, genetic, or
 Chapter 20  The Geriatric Patient 575

iatrogenic, whereas localized or organ-limited amyloidoses
are associated with aging and diabetes and occur in ­isolated
organs, often endocrine, without evidence of ­ systemic
involvement. Despite their biochemical differences, the var-
ious amyloidoses have similar pathophysiologic features
(Table 20-1).
The three major systemic clinical forms currently recog-
nized are primary or idiopathic amyloidosis (AL), secondary
amyloidosis (AA), and hereditary amyloidosis (Table  20-2).
The most common form of systemic amyloidosis seen in cur-
rent clinical practice is AL (light-chain amyloidosis, primary
idiopathic amyloidosis, or associated with multiple myeloma).
A fourth type of systemic amyloidosis is seen only in patients
with long-standing dialysis.
Diagnosis and Differential. Symptoms and signs of amy-
loidosis vary depending on the involved systems and organs.
The nephritic syndrome is the most striking early manifesta-
tion. The renal lesion is usually not reversible and p
­ rogressively
leads to azotemia and death.
Regardless of etiology, the clinical diagnosis of amyloidosis
is usually not made until the disease is far advanced, because
of its nonspecific symptoms and signs. The diagnosis is made
by identification of amyloid fibrils in biopsy or n
­ ecropsy ­tissue
sections using Congo red stain. A unique protein (member of
the pentraxin family of proteins) called AP (or serum AP)
is universally associated with all forms of amyloid and forms
the basis of a diagnostic test. Once amyloidosis is diagnosed,
it can be further classified by genomic DNA, protein, and
immunohistochemical studies; the r­elationship of immu-
­
noglobulin-related amyloid to multiple myeloma should be
confirmed by electrophoretic and i­mmunoelectrophoretic
studies.
Preoperative Preparation and Treatment. A comprehen-
sive survey of all systems should be performed, focusing on
the most frequently involved organs. Careful evaluation for
systemic involvement of amyloidosis or associated disease is
important, even in apparently isolated tumorous amyloidosis
(Table 20-3).

TABLE 20-1  n  Amyloidosis: Multisystem Involvement and Clinical Features

Involvement Manifestations

Nervous system
Polyneuropathy Sensory loss, carpal tunnel syndrome, myopathy, myelopathy, vitreous opacities
Autonomic neuropathy Postural hypotension, inability to sweat, sphincter incompetence
Respiratory system
Upper respiratory tract Localized tumor can be found in respiratory tracts and lungs
Nasal sinuses, larynx, and trachea Tracheobronchial lesions, or diffuse alveolar deposits
Tongue Macroglossia
Lower respiratory tract and lung parenchyma Accumulation of amyloid, which block the ducts; may resemble a neoplasm
Cardiovascular system
Conduction system Arrhythmia, heart block
Endocardium and valves Valvular diseases
Myocardium Cardiomyopathy: dilated, restrictive, and obstructive forms; congestive heart failure
Pericardium Pericarditis
Gastrointestinal system
Liver Hepatomegaly, abnormal liver function, portal hypertension
Gastrointestinal tract Unexplained GI disease, malabsorption; unexplained diarrhea or constipation; obstruction,
ulceration, and protein loss; esophageal motility disorders

Kidney Nephrotic syndrome, proteinuria, renal failure; renal tubular acidosis or renal vein
thrombosis
Spleen Spleen enlargement; not associated with leukopenia or anemia
Musculoskeletal system Pseudomyopathy; cystic bone lesions
Endocrine system
Thyroid gland Hypothyroidism; full-blown myxedema (almost invariably accompanies medullary
carcinoma of thyroid)
Adrenal gland Type II diabetes
Pituitary gland, pancreas Other endocrine abnormalities
Skin Lichen amyloidosis; papules; plaques; ecchymoses
Hematologic system Fibrinogenopenia, including fibrinolysis
Endothelial damage Selective deficiency of clotting factors (factor X)
Clotting abnormalities, abnormal bleeding time
Other Rheumatoid arthritis; chronic inflammation and infection

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 576 ANESTHESIA AND UNCOMMON DISEASES

TABLE 20-2  n  Major Systemic Amyloidosis: Clinical Features and Diagnosis

Primary (AL)* Secondary (AA)† Hereditary

Organs typically Localized amyloid tumors may be
involved found in respiratory tract.
Vascular system, especially heart
Other organs: tongue, thyroid gland,
heart, lung, liver, intestinal tract,
spleen, kidney, skin
Spleen, liver, kidney, adrenal glands, Peripheral sensory and motor
lymph nodes; vascular involvement neuropathy, often autonomic
No organ spared, but significant neuropathy
involvement of the heart is rare Carpal tunnel syndrome
Vitreous abnormalities
Cardiovascular and renal amyloid

Associated Multiple myeloma Infection (tuberculosis, bronchiectasis,

diseases osteomyelitis, leprosy)
Inflammation (rheumatoid arthritis,
granulomatous ileitis)
Familial Mediterranean fever
Tumors such as Hodgkin's disease

Diagnosis Monoclonal immunoglobulin in urine
or serum plus any of following:
macroglossia, cardiomyopathy,
hepatomegaly, malabsorption or
unexplained diarrhea or
constipation, unexplained nephrotic
syndrome, carpal tunnel syndrome,
or peripheral neuropathy
Chronic infection (osteomyelitis,
tuberculosis), chronic
inflammation (rheumatoid arthritis,
granulomatous ileitis) plus any
of following: hepatomegaly,
unexplained GI disease, or
proteinuria
Family history of neuropathy
plus any of following: early
sensorimotor dissociation,
vitreous opacities,
cardiovascular disease,
GI disease, autonomic
neuropathy, or renal disease

*or Idiopathic.
†or Secondary, acquired, reactive.

Treatment of localized amyloid tumors is surgical exci-

TABLE 20-3  n Amyloidosis: Preoperative Assessment sion. However, no effective treatment exists for systemic amy-
and Workup loidosis. Currently, care is generally supportive, and therapy
is directed at reducing production of and promoting lysis of
System Assessment
amyloid fibrils. Hemodialysis and immunosuppressive ther-
Nervous Peripheral neuropathy: document apy may be useful. Current treatment of primary amyloidosis
pre-existing peripheral neurologic includes a program of prednisone/melphalan or prednisone/
symptoms melphalan/colchicine. Liver transplantation, kidney trans-
Autonomic neuropathy: orthostatic blood plantation, and stem cell transplants have yielded promising
pressure, etc.
results. In certain familial amyloidoses, genetic counseling is
Airway Macroglossia an important aspect of treatment.
Pulmonary Diffuse dysfunction: pulmonary function
Ultimately, some people with amyloidosis continue to
studies deteriorate. The major causes of death are heart disease and
renal failure. Sudden death is common, presumably caused by
Cardiac Arrhythmia, cardiomyopathy, and
arrhythmias.
valvular involvement: cardiac function
echocardiogram and electrocardiogram
ANESTHETIC CONSIDERATIONS
Gastrointestinal Esophageal motility abnormality, Localized amyloid deposition has been reported at ­various
intestinal obstruction
sites. Amyloid in the tongue can cause macroglossia to a degree
Liver Liver function studies requiring glossectomy.11 In addition, amyloid macroglossia
Kidney Abnormal renal function: electrolytes,
may be associated with coexisting hypothyroidism.12 Laryngeal
renal function studies amyloidosis is fragile and carries the risk of ­spontaneous
massive hemorrhage, even without m
­ ­ anipulation.13 The
Hematology Enlarged spleen; check CBC: RBCs,
­airway tumor should be assessed by noninvasive ­imaging, such
platelets, coagulation coagulopathy,
and factor deficiency as computed tomography (CT) or MRI. Before i­ntubation,
­preparations should be made for both difficult airway and
Endocrine Pancreatic or adrenal gland involvement; massive hemorrhage.
thyroid function test to rule out
hypothyroidism
A smaller endotracheal tube (ETT) may be ­considered. In
addition, direct laryngoscopy monitored by a fiberscope-video

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system, rather than blind insertion of the ETT through vocal
cords over a fiberoptic bronchoscope,14 has been advocated.
It is controversial whether depolarizing muscle relaxants
should be administered to patients with amyloidosis, especially
those with cardiac involvement. Patients with familial amyloid
polyneuropathy have a high incidence of cardiac arrhythmias
during anesthesia. Exaggerated elevations in potassium con-
centration may occur after succinylcholine administration and
may be a contributing factor.15 However, Viana et al.16 reported
that the average increase in plasma potassium concentrations
after succinylcholine administration in patients with familial
amyloid polyneuropathy was similar to the increase observed
in a normal population. However, they could not exclude that
a dangerous rise in serum potassium concentration might not
occur in a certain percentage of patients with familial amy-
loid after administration of succinylcholine. This may also be
true in patients with amyloidosis who also have long-standing
polyneuropathy.17 Thus, it may be prudent to avoid admin-
istration of depolarizing muscle relaxants in patients with
amyloidosis, especially in the presence of coexisting polyneu-
ropathy or cardiac disease.
Autonomic dysfunction secondary to ­ amyloidosis has
dramatic perioperative ramifications.17 In particular, the
­ patients.
administration of anesthetic drugs to patients with ­amyloidotic
polyneuropathy presents a risk of significant hypotension (even
use of ketamine does not prevent hypotension). Patients with
decreased preload are especially sensitive. In addition, hypo-
tension is frequent even in patients with adequate p ­ reload
as a result of low systemic vascular resistance. Given these
­observations, the anesthesiologist should consider using inva-
sive blood pressure monitoring and preparation of a vasocon-
strictor infusion for effective anesthetic management of these
patients.
IDIOPATHIC PULMONARY FIBROSIS
The pathophysiology of idiopathic pulmonary fibrosis (IPF)
is not currently understood. IPF may represent a model of
chronic dysregulated repair and lung remodeling, resulting
from an epithelial/endothelial insult and persistent inflam-
matory cell activation.18 The initial injury event remains
undefined. However, evidence suggests that viral infections
or environmental factors may provide mediating events.
Interestingly, a majority of patients with IPF have a smoking
history.
Symptoms associated with IPF include breathlessness,
fatigue, weight loss, and a chronic dry cough. On physical
examination, dry “Velcro” crackles may be heard throughout
the lung fields. Cyanosis and clubbing may also be observed.
As the disease progresses, signs of pulmonary hypertension
and right-sided heart failure (loud S2 heart sound, right ven-
tricular heave, or pedal edema) may be present.
A chest radiograph may show interstitial infiltrates in
the lung bases. CT is more sensitive than a chest radiograph
for detecting disease early. Typically, CT shows a pattern of
patchy white lines in the lower lungs. In areas of more severe
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Chapter 20  The Geriatric Patient 577
involvement, the thick scarring often creates a honeycomb
appearance. Pulmonary function studies show a restrictive
pattern. Arterial blood gas (ABG) analysis may show hypox-
emia with minimal exercise and, as the disease progresses,
even at rest. However, the definitive test to confirm diagnosis
of IPF is lung biopsy.
The diagnosis of idiopathic pulmonary fibrosis should be
reserved for patients with a specific type of fibrosing inter-
stitial pneumonia known as usual interstitial pneumonia.
Foremost in the differential diagnosis is to distinguish usual
interstitial pneumonia from other idiopathic interstitial pneu-
monias. This distinction is made on a pathologic basis.19
Numerous other disease processes may lead to IPF and
should be ruled out as diagnoses (Box  20-1). Fibroses may
occur as a result of occupational or environmental exposure
to toxic substances, lung infection, drug exposure, connective
tissue disease, and sarcoidosis.
Idiopathic pulmonary fibrosis may be associated with
respiratory failure and chronic hypoxemia in the later stages.
Polycythemia also occurs in this context. Cor pulmonale
should be specifically sought in evaluation of these patients.
Incidence of bronchogenic carcinoma is increased in IPF
BOX 20-1   n  IDIOPATHIC PULMONARY FIBROSIS
Symptoms: Breathlessness; dry cough; weight loss; fatigue
Physical findings: Change in shape of fingers and toenails (clubbing);
cyanosis (late stages of disease); dry “Velcro” crackles throughout
lung fields on auscultation
Differential Diagnosis
Pathologic distinction from other types of fibrosing interstitial
pneumonia:
n Desquamative interstitial pneumonia (respiratory bronchitis,
interstitial lung disease)
n Acute interstitial pneumonia
n Nonspecific interstitial pneumonia
n Cryptogenic organizing pneumonia (bronchiolitis obliterans,
organizing pneumonia)
Pulmonary fibrosis resulting from occupational or environmental
exposure: asbestosis, silicosis, farmer's lung, bird breeder's lung;
exposure to metal dust, bacteria, fumes, animals, other dusts, or
gases
Fibrosis resulting from infection: tuberculosis; pneumococci;
Pneumocystis jiroveci (P. carinii); bacterial, fungal, viral pneumonia
Drug exposure: bleomycin
Connective tissue disease: rheumatoid arthritis, systemic sclerosis
Sarcoidosis
Comorbidities: Respiratory failure, chronic hypoxemia, cor pulmonale,
polycythemia, increased incidence of lung cancer
Critical Questions
n Is the patient approaching end-stage disease?
n Is there a history of respiratory failure?
n Is there a need for home oxygen?
n Are there any signs and symptoms of chronic hypoxemia?
n Is there any evidence of cor pulmonale?
Chronic medications: Corticosteroids, cyclophosphamide (Cytoxan),
oxygen, colchicine
 578 ANESTHESIA AND UNCOMMON DISEASES
The critical questions to ask the patient and family
­ hysician should focus on determining how advanced the
p BOX 20-2   n  POLYCYTHEMIA VERA
­disease has become and how much pulmonary reserve is
present (see Box 20-1). In appropriate patients, the clinician
should assess for the long-term complications of corticoste-
roid administration.
At present, the therapeutic options available to treat patients
with IPF are limited (see Box 20-1). Many patients receive cor-
ticosteroids or immunosuppressants despite no studies clearly
documenting their efficacy. Many patients require home
oxygen.
ANESTHETIC CONSIDERATIONS
Typical surgical procedures where the anesthesiolo-
gist may encounter idiopathic pulmonary fibrosis include
open or ­ thoracoscopic lung biopsy and lung transplan-
tation. In these procedures, one-lung ventilation is often
required. Therefore, the major anesthetic consideration is
the inability to tolerate one-lung ventilation secondary to
hypoxemia or the ­generation of high airway pressures.20 In
addition, hypercapnia may occur in these patients during
one-lung ventilation.21 An arterial catheter is indicated when
­anesthetizing these patients, because frequent ABG studies
may be required. In addition, central venous access should
be strongly considered.
These patients may generate large negative intrathoracic
pressures during spontaneous ventilation. Therefore, special
care must be taken to prevent air emboli during placement
of the central line. Access to inhaled nitric oxide should be
available for patients with cor pulmonale.22 In patients with
­limited pulmonary reserve, regional or local anesthesia should
be ­considered if the surgical procedure permits.
POLYCYTHEMIA VERA
Polycythemia vera is a clonal stem cell disorder in which all
three myeloid components are involved. Erythrocytosis is
the foremost expression of the disease. Studies suggest that
impaired signaling of hematopoietic growth factors may be
an underlying pathophysiologic mechanism of polycythemia
vera.23,24
Patients with polycythemia vera are prone to both throm-
botic and hemorrhagic events. The mechanisms underlying
thrombotic complications may be related to the increased
red blood cell (RBC) mass.24 An elevated hematocrit (Hct)
increases both blood viscosity and RBC aggregation, inducing
a hypercoagulable state.23 Hemorrhagic events are associated
with elevations in absolute platelet count. Defects in platelet
function have been reported in polycythemia vera. In addi-
tion, an acquired von Willebrand's disease occurs with elevated
platelet counts in myeloproliferative syndromes. This acquired
von Willebrand's disease is characterized by decreased large
von Willebrand multimers and increased cleavage products.24
Polycythemia can evoke both general symptoms and
those secondary to underlying thrombotic or hemorrhagic
pathologic processes. General symptoms include bone pain
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Symptoms
General: Headaches, tinnitus, fatigue, shortness of breath, pruritus
(aquagenic), tingling or burning of hands and feet, visual changes,
bone pain, weight loss, night sweats, vertigo
Thrombotic: Cerebrovascular accident (stroke), myocardial infarction,
angina, intermittent claudication
Hemorrhagic: Bleeding diathesis, GI bleeding, unusual bleeding from
minor cuts, epistaxis
Physical findings: Splenomegaly (later stages), hepatomegaly, retinal
vein engorgement, ruddy complexion, hypertension
Differential Diagnosis
Is there an underlying decrease in tissue oxygenation secondary
to lung disease, high altitude, intracardiac shunt, hypoventilation
syndromes, abnormal hemoglobin, smoking, or carbon monoxide
poisoning?
Is there aberrant erythropoietin production secondary to tumors
(brain, liver, uterus) or cysts (especially renal)?
Has hemoconcentration occurred secondary to diuretics, burns,
diarrhea, or stress?
Comorbid Conditions
Hemorrhagic: gastric ulcer, epistaxis
Thrombotic: Budd-Chiari syndrome; cerebral, coronary, mesenteric, or
pulmonary thrombosis
Other: gout
Critical Questions
n Does the patient undergo phlebotomy?
n Is
the patient on myelosuppressive therapy?
n What are the most recent hematocrit and platelet counts?
n What are the results of the most recent coagulation studies?
Chronic medications: Cytoreductive drugs, including 32P; alkylating
agents (chlorambucil, busulfan), hydroxyurea, interferon-α,
paroxetine, aspirin
Anesthetic Management
Ensure adequate access and availability of blood products, including
platelets.
Use of regional techniques is controversial.
and tingling or burning of the hands and feet (Box 20-2). In
addition, exposure to warm water may provoke an intense
pruritus. Patients may initially present with ischemic or
­
thrombotic ­vascular symptoms, including stroke, intermittent
claudication, or angina. Signs of a bleeding diathesis can also
be ­present, such as epistaxis or gastrointestinal (GI) bleeding.
On physical examination a ruddy complexion or pleth-
ora may be noted. Polycythemia vera is also associated with
hypertension. Patients may complain of visual changes, and
retinal vein engorgement may be noted. Hepatomegaly and
splenomegaly occur late in the course of the disease.
Alternative conditions that should be considered when
presented with a polycythemic patient include any underlying
mechanism that decreases blood oxygenation. Aberrant eryth-
ropoietin production may cause polycythemia. Polycythemia
may also result from hemoconcentration. The comorbidities
observed with polycythemia vera may be of a hemorrhagic or
thrombotic nature (Box 20-2). In addition, gout often occurs
in these patients.

Preoperative Preparation. Before surgery, it is important to
ascertain how the elevated RBC mass has been treated, if at all.
Phlebotomy is an effective remedy for the hypercoagulability
observed with polycythemia vera. A recommended therapeu-
tic end point is Hct less than 42% in women and 45% in men.25
Optimally, Hct should be normalized 2 to 4 months before
elective surgery. Patients older than 60 years or with a previous
thrombotic episode are defined as “high risk.” These individu-
als may also receive cytoreductive treatment in an attempt to
aggressively treat the disease. Perioperative risk of thrombotic
or hemorrhagic events is influenced by how aggressively the
polycythemia has been treated. Thus, it is important to obtain
a history of the platelet counts and Hct values to determine
past treatment of the disease. Coagulation studies, including
bleeding time, should be obtained before surgery.
Cytoreductive agents are administered in high-risk patients.
Aspirin is often used as adjunctive therapy, even in low-risk
individuals. Patients with severe pruritus may be treated with
interferon-α or paroxetine (see Box 20-2).
ANESTHETIC CONSIDERATIONS
Uncontrolled polycythemia vera is associated with a high
risk of perioperative bleeding and postoperative thrombosis.
Control of the disease before surgery will reduce the incidence
of these complications.
It is important to ensure adequate vascular access in case
bleeding occurs. In addition, the ready availability of platelet
transfusion should be ensured in larger blood loss cases. At
present there is insufficient evidence to determine whether
antiplatelet drugs are contraindicated during the periopera-
tive management of the polycythemic patient.
The use of regional versus general anesthesia is controver-
sial. Both techniques have been used successfully in patients
with polycythemia vera. Studies suggest a lower incidence
of deep vein thrombosis with regional techniques. However,
this moderate effect must be weighed against the risk of
­epidural or subarachnoid hemorrhage in a patient who may
be ­predisposed toward bleeding events.
ESSENTIAL THROMBOCYTHEMIA
The World Health Organization (WHO) has defined essential
thrombocythemia as a sustained platelet count of greater than
600,000 cells/mm3 with a bone marrow biopsy showing mainly
proliferation of the megakaryocytic lineage. In addition,
patients must show no evidence of polycythemia vera, chronic
myeloid leukemia, idiopathic myelofibrosis, ­myelodysplastic
syndrome, or reactive thrombocytosis.26
The principal feature of essential thrombocythemia is an
increase in megakaryocytes and platelets. Disease patho-
genesis more than likely involves alterations in the signaling
­pathways that regulate thrombopoiesis.
The principal pathophysiologic features of essential thrombo-
cytosis are thrombosis and hemorrhage. Thrombosis may involve
the microcirculation or large-vessel ­occlusions, ­predominantly
of the arteries. The incidence rate is approximately 8% per
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Chapter 20  The Geriatric Patient 579
patient-year in untreated patients.27 Hemorrhagic complications
only occur with very high platelet counts and are ­secondary to
abnormal platelet function.
Symptoms of essential thrombocythemia are associated
with vasomotor changes in the cerebral and peripheral circu-
lation, including headache, transient ischemic attacks (TIAs),
or migraines (Box  20-3). The principal clinical features of
essential thrombocythemia are thrombosis affecting the arte-
rial more frequently than venous circulation and hemor-
rhage. Major arterial thrombosis may include both stroke and
peripheral arterial occlusion. The most common presentation
of bleeding involves the GI tract, although bleeding may occur
from the skin, gums, and nose.
Patients may present with splenomegaly and hepatomegaly
secondary to extramedullary hematopoiesis. In the peripheral
circulation, acrocyanosis and erythromelalgia are common
complaints. Erythromelalgia is characterized by burning pain
and erythema of the digits, especially of the lower extremity.
Of note, the pain associated with essential thrombocytopenia
increases with heat and improves with cold. Likewise, ­pruritus
may occur in the extremities when exposed to warmth but
improves with colder temperatures.
BOX 20-3   n  ESSENTIAL THROMBOCYTHEMIA
Signs: Splenomegaly; digital pain that increases with heat, improves
with cold; sweating, pruritus, low-grade fever, hepatomegaly;
bleeding from skin, gums, or nose
Symptoms
Vasomotor symptoms of cerebral circulation: Headache, dizziness,
visual disturbances, TIAs, migraines
Vasomotor symptoms of peripheral circulation: Paresthesias,
acrocyanosis, erythromelalgia
Thrombotic symptoms: Venous thrombotic events, superficial
thrombophlebitis, deep vein thrombosis, portal or splenic venous
thrombosis, major arterial thrombosis, including stroke
Hemorrhagic symptoms: Bleeding diathesis, especially GI bleeding
Differential Diagnosis
Clonal thrombocytosis: Associated with other chronic
myeloproliferative disorders
Reactive thrombocytosis: Acute bleeding, hemolysis, iron deficiency
anemia, acute and chronic inflammatory conditions (e.g., arthritis,
stress, surgery), osteoporosis, metastatic cancer, severe trauma,
splenectomy, medication
Critical Questions
n Does patient have a history of previous thrombosis? Platelet
count? Obesity? Smoking? Age?
n Any evidence of ongoing bleeding?
n How has the platelet count been managed?
Chronic medications: Cytoreductive drugs, including hydroxyurea,
anagrelide, interferon-α, or 32P; low-dose aspirin
Anesthetic Management
Platelet counts should be normalized before surgery.
Consider plateletpheresis in emergency situations to achieve a rapid
decrease in platelet count.
Administer cytoreductive therapy to decrease platelet count before
surgery.
 580 ANESTHESIA AND UNCOMMON DISEASES
In particular, two differential diagnoses should be consid-
ered when encountering a patient with essential thrombocyto-
sis (see Box 20-3). First, essential thrombocytosis may represent
part of a continuum of the myeloproliferative disorders. Over
the course of time patients with essential thrombocytosis may
develop myelofibrosis, myelodysplastic syndrome, or acute
myelocytic leukemia. In addition, both polycythemia vera and
myelofibrosis may present as thrombocytosis. Second, reactive
thrombocytosis must be ruled out. Numerous conditions, both
acute and chronic, may produce thrombocytosis.
The comorbidities of interest to the anesthesiologist that
occur with essential thrombocytosis are associated with the
complications of hemorrhage and thrombosis.
The clinician must first assess a patient's risk of throm-
bosis (Box 20-3). Studies show that the primary risk factors
for a thrombotic event are history of previous thrombosis
and age. Other, less significant risk factors include a history
of smoking and obesity. Retrospective studies show that the
­incidence of thrombotic events per year is 1.7%, 6.3%, and
15.1%, at younger than 40 years, 40 to 60 years, and older
than 60 years, respectively.28 In addition, the incidence rate
of thrombosis has been reported at 31.4% and 3.4% per year
in patients with and without a history of previous throm-
bosis. The absolute platelet count cannot provide a defini-
tive assessment of t­ hrombotic risk. Thrombotic events have
been reported with platelet counts in the range of 400,000 to
600,000/mm3.29 Although the platelet count does not neces-
sarily predict the risk of thrombosis, evidence suggests that
controlling the platelet count does decrease the incidence
of thrombosis. Prospective studies comparing long-term
risk of thrombosis in patients treated with myelosuppres-
sive ­therapy versus those without found incidence rates of
8% versus 1.5% per patient-year in untreated and treated
patients, respectively.27 Thus, the degree of controlling the
platelet count assumes importance in assessing thrombotic
risk. The main risk factor for ­hemorrhage is a platelet count
greater than 1.5 million/mm3.25
Treatment of essential thrombocytosis consists of chronic
myelosuppressive therapy to manage the platelet count in
high-risk individuals. Antiplatelet drugs may also be included
in the regimen. Low-dose aspirin has been shown to be effica-
cious in managing both erythromelalgia and TIAs associated
with essential thrombocytosis.30
ANESTHETIC CONSIDERATIONS
The most important issue in perioperative management of
the patient with essential thrombocythemia is whether to
normalize the platelet count before surgery. No clear guide-
lines exist as to which patients should be aggressively nor-
malized preoperatively, and consultation with a hematologist
should be pursued. However, elderly patients with consistently
­elevated platelet counts and a history of prior thrombosis
­represent a high-risk group requiring aggressive management.
Elective surgical patients may have adequate time to undergo
­cytoreductive therapy preoperatively. In the case of urgent or
­emergency surgery, plateletpheresis may be considered.
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MYELOID METAPLASIA WITH
MYELOFIBROSIS
The intrinsic characteristics of this metaplasia include both
myeloproliferation and myelofibrosis. It is currently hypoth-
esized that dysregulated Janus kinase (JAK) signaling may be
an underlying etiology for myelofibrosis.
Myeloid metaplasia with myelofibrosis may present in a
variety of ways. Constitutional symptoms may relate to the
catabolic aspects of this disease and include cachexia, fatigue,
weight loss, low-grade fever, and night sweats (Box  20-4).
Extramedullary hematopoiesis may evoke a constellation of
symptoms and signs. The most common presenting ­feature
is hypersplenism. Extramedullary hematopoiesis may also
occur in other organ systems such that lymphadenopathy,
acute cardiac tamponade, hematuria, papular skin nodes,
­pleural ­effusion, pulmonary hypertension, and spinal cord
­compression may be observed. Of note, patients with pulmo-
nary hypertension have a poor prognosis.
From 50% to 75% of patients are anemic at diagnosis,
whereas the white blood cell (WBC) count and platelet count
initially may be either increased or decreased. An early find-
ing on peripheral blood smear, myelophthisis, is characterized
by teardrop-shaped RBCs, immature granulocytes, and nucle-
ated RBCs. Several disorders are also associated with myelo-
phthisis and possible myelofibrosis (Box 20-4). Therefore, the
­differential diagnosis must include other malignancies, such
as chronic myeloid leukemia, myelodysplastic syndrome,
­metastatic cancer, lymphoma, Hodgkin's disease, and plasma
BOX 20-4   n MYELOID METAPLASIA WITH
MYELOFIBROSIS
History: Constitutional symptoms, including weight loss, night
sweats, and low-grade fever
Physical findings: Splenomegaly, anemia, pallor, petechiae and
ecchymosis, gout
Findings related to extramedullary hematopoiesis: Acute cardiac
tamponade, hematuria, lymphadenopathy, papular skin nodes,
pleural effusion, spinal cord compression
Laboratory findings: Anemia, WBC count increased or decreased,
platelet count increased or decreased, myelophthisis
Differential Diagnosis
Malignancies that may display bone marrow fibrosis
Essential thrombocythemia
Granulomatous involvement of bone marrow (e.g., histoplasmosis,
tuberculosis)
Associated Conditions
Portal hypertension
Splenic infarction
Complications related to extramedullary hematopoiesis
Anesthetic Management
Obtain CBC and platelet count.
Consider cytoreductive therapy in patients without significant
thrombocytopenia.
Bleeding may require platelet transfusion or cryoprecipitate.
Obtain DIC panel.

cell dyscrasia. Granulomatous involvement of the bone ­marrow
may cause myelofibrosis. Thus, tuberculosis and histoplasmo-
sis must also be entertained as possible diagnoses. In patients
presenting with elevated platelet counts and minimal myelofi-
brosis, it may be difficult to exclude the diagnosis of essential
thrombocythemia.
The conditions commonly associated with myelofibrosis
and myeloid metaplasia result from the underlying pathophys-
iology. Portal hypertension may result from either increased
portal flow secondary to splenomegaly or thrombotic obstruc-
tion of small hepatic veins.
ANESTHETIC CONSIDERATIONS
The greatest experience in perioperative management of
myelofibrosis and myeloid metaplasia has occurred with sple-
nectomy. Indications for splenectomy include splenomegaly
refractory to chemotherapy, portal hypertension, or progres-
sive anemia. Morbidity and mortality after this procedure
have been reported at 30.5% and 9%, respectively.31 Significant
perioperative complications after splenectomy include hemor-
rhage (14.8%), infection (8.5%), and thrombosis (7.5%). The
primary cause of death includes hemorrhage (4.5%), infection
(2.7%), and thrombosis (1.3%). The only preoperative variable
that correlates with increased hemorrhage or thrombosis is a
platelet count of less than 100,000/mm3.
With this experience in mind, the critical information
and interventions before surgery would include a complete
blood cell count (CBC) and platelet count. In patients without
thrombocytopenia, prophylactic cytoreductive therapy should
be considered to reduce the risk of perioperative thrombosis.
Adequate blood products should be available before ­surgery,
including access to platelets and cryoprecipitate. Occult
­disseminated intravascular coagulation (DIC) has been asso-
ciated with perioperative bleeding, so a preoperative DIC
panel should be performed. Splenectomy should be postponed
in patients with d-dimer levels greater than 0.5 μg/mL.31
POLYMYALGIA RHEUMATICA
Patients with polymyalgia rheumatica are classically older
than 50 years (90% older than 60), and most are Caucasian.32
It is a syndrome characterized by pain and morning stiffness
in the neck, shoulder girdle, and pelvic girdle, with consti-
tutional symptoms such as malaise and fatigue (Fig.  20-1).
Typically the erythrocyte sedimentation rate (ESR) is higher
than 50 and frequently higher than 80 mm/hr. Polymyalgia
rheumatica can occur as a separate entity or in association
with temporal arthritis. Patients who have polymyalgia rheu-
matica dramatically improve with low doses of prednisone
(10-15 mg/day). Once symptoms have resolved and ESR has
normalized, the dose of prednisone is tapered slowly while
the patient is closely monitored for recurrence of symptoms
or increased ESR.
A wide variety of conditions can mimic polymyalgia rheu-
matica.33 When present, distal-limb symptoms may initially
make it difficult to differentiate polymyalgia rheumatica from
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Chapter 20  The Geriatric Patient 581
FIGURE 20-1  Untreated hands of patient with polymyalgia
rheumatica.
rheumatoid arthritis (RA) or similar syndromes. Pronounced
symmetric involvement of peripheral joints, seropositivity
for rheumatoid factor, and joint erosions and extra-­articular
manifestations clearly differentiate RA from polymyalgia
­rheumatica. In elderly patients, systemic lupus erythemato-
sus may present as polymyalgia rheumatica.34 The ­presence
of pleuritis or pericarditis (common in late-onset SLE),
­leukopenia or thrombocytopenia, and antinuclear antibodies
should raise the clinical suspicion of SLE. The predominant
­proximal muscular weakness demonstrated with movement,
rather than pain and an increase in muscular enzyme levels,
differentiate polymyositis from polymyalgia rheumatica.35 The
­presence of peripheral enthesitis, dactylitis, anterior uveitis,
and ­radiologic evidence of sacroiliitis differentiate late-onset
spondyloarthropathy from polymyalgia rheumatica.36
ANESTHETIC CONSIDERATIONS
The preoperative history and physical examination should
focus on symptoms related to the many organ systems
affected by RA, in particular the airway and neurologic, pul-
monary, and cardiovascular systems. A careful history may
elicit ­neurologic deficits, neck and upper extremity pain,
and a crunching sound with neck movement. Patients with
neurologic deficits or symptoms of long-standing, severely
deforming disease, or those scheduled to undergo proce-
dures requiring manipulation of the cervical spine or special
positioning (e.g., turning prone), require anteroposterior and
lateral cervical radiographs with special flexion, extension,
and open-mouth odontoid views.37 Significant ­abnormalities
(anterior atlas-dens interval >9 mm or p ­ osterior interval
<14 mm) may benefit from consultation with a ­neurologist
or neurosurgeon. However, the duration, severity, or symp-
toms of the disease do not correlate with cervical spine
subluxation.
Preoperative documentation of deformities and neurologic
deficits is important to establish baseline level of function. For
patients with significant hoarseness, referral to an otolaryn-
gologist to assess mobility of the vocal cords and the degree of
cricoarytenoid arthritis may be of benefit.38 Acute or worsen-
ing pulmonary symptoms may trigger a need for additional
 582 ANESTHESIA AND UNCOMMON DISEASES
pulmonary workup. Muffled heart sounds, pericardial rubs,
and an enlarged heart detected by examination or on a chest
radiograph together with low voltage on an electrocardiogram
(ECG) suggest a pericardial effusion, which can be further
evaluated.
Advanced planning for the management of identified
or potentially difficult intubation should be anticipated and
­discussion of regional anesthetic options undertaken in appro-
priate clinical circumstances. Continuation of steroids and
chronic pain medications is optimal, but drugs with antiplate-
let effects are often discontinued, and immunosuppressants
may need to be temporarily stopped to allow normalization
of blood counts. Patients with complex regimens and severe
disease are best managed in concert with a rheumatologist or
primary physician.
GOODPASTURE'S SYNDROME
Goodpasture's syndrome, or anti–glomerular basement
­membrane (anti-GBM) antibody nephritis, is a rare autoim-
mune disease characterized by formation of anti-GBM anti-
bodies and, in 60% of patients, antibodies to pulmonary
alveolar basement membranes.39 Confirmatory ­ diagnosis
involves the finding of circulating anti-GBM antibodies and,
on immunofluorescent imaging of renal biopsy samples,
severe crescentic glomerulonephritis with linear deposition of
immunoglobulin G on GBMs (Fig. 20-2).
In elderly patients, Goodpasture's syndrome is chiefly
found in women and may not present with overt pulmo-
nary hemorrhage. With extensive crescents, the prognosis
for recovery is poor without aggressive therapy. Treatment
consists of oral cyclophosphamide (in reduced dosage), oral
and intravenous (IV) glucocorticoids, and aggressive plasma
exchange. Circulating antibody levels decrease quickly but
FIGURE 20-2  Nephron in patient with Goodpasture's syndrome
(anti-GBM antibody nephritis).
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renal recovery depends on the extent of damage at the time
treatment is begun.39 Dialysis-dependent patients with serum
creatinine of more than 7 mg/dL have only a 10% chance of
recovery. If life-threatening pulmonary hemorrhage is also
present, plasma exchange may be lifesaving.
ANESTHETIC CONSIDERATIONS
Patients with Goodpasture's syndrome at risk for periopera-
tive renal failure include those with pre-existing renal insuf-
ficiency (the single strongest predictor) or diabetes, especially
in combination, and those undergoing procedures with the
administration of contrast medium. If all three conditions
are present, the risk of renal failure may be as high as 12%
to 50%. Preoperative identification of at-risk patients alters
management, such as hydration, administration of sodium
bicarbonate, change in type of contrast medium, and avoid-
ance of hypovolemia. Many clinical trials have failed to
include ­sufficient numbers of elderly persons, making it dif-
ficult to translate therapeutic recommendations. In addition,
the risks of aggressive treatment of glomerular disease may
be enhanced in older patients, which is crucial in therapeutic
decision making.
Drugs with particular implications for anesthesia and
surgery are the low-molecular-weight heparins (LMWHs)
because there is no easy method of monitoring their anti-
coagulation effects. All the LMWHs available in the United
States are cleared by the kidneys and are not removed during
dialysis. Therefore, LMWH may have a prolonged duration
of action in patients with chronic kidney disease, possibly
increasing the risk of bleeding with neuraxial anesthesia.
Nonsteroidal anti-inflammatory drugs (NSAIDs) and cyclo-
oxygenase (COX-2) inhibitors interfere with autoregulation
of renal perfusion and should be avoided or discontinued in
patients with or at risk for renal insufficiency. Cyclosporine
and aminoglycoside antibiotics can cause renal insufficiency.
Angiotensin-converting enzyme (ACE) inhibitors and alpha-
receptor blockers (ARBs) prevent deterioration in patients
with diabetes or renal insufficiency but may worsen function
during hypoperfusion states.
In patients on dialysis, coordinating the scheduling of dial-
ysis and elective surgery is an important aspect of preopera-
tive care. Preoperative renal replacement therapy (dialysis)
schedules should be determined, with scheduling of surgery
ideally within 24 hours after dialysis. In elective cases, dialysis
is best performed within 24 hours of surgery but not immedi-
ately before, because of acute volume depletion and electrolyte
alterations. Dialysis should be performed to correct volume
overload, hyperkalemia, and acidosis.
MALE BREAST CANCER
The incidence of male breast carcinoma in the United States
is 1 per 100,000, almost 150 to 200 times less common than
female breast cancer. The median age of male breast cancer
patients varies between 63 years39 and 70 years. Because of its
infrequent nature and presentation in elderly patients, who

often exhibit typical senile gynecomastia, delays in diagno-
sis by physicians (and delayed recognition of symptoms by
patients) are common.40,41 This is one factor accounting for the
percentage of patients with positive axillary nodes on diagno-
sis (50%) and metastatic disease (80%).
Klinefelter's syndrome is the only known risk factor for
male breast cancer. Presentation is usually with a lump or
retraction of the skin or nipple. Male breast cancers are usu-
ally eccentric masses, whereas gynecomastia is almost always
central. Infiltration of the skin or nipple occurs much earlier
in male breast cancer because of the smaller breast volume.
Mammography is valuable in determining whether breast
enlargement is caused by gynecomastia or breast cancer. When
in doubt, fine-needle aspiration should be performed to estab-
lish a definitive diagnosis. The histology and prognosis for each
tumor stage are similar to those for female breast cancer.
ANESTHETIC CONSIDERATIONS
Preoperative evaluation of male breast cancer patients is simi-
lar to that of female breast cancer patients and should focus
on the heart, lungs, and neurologic and hematologic systems.
Previous head and neck irradiation may cause carotid artery
disease, hypothyroidism, or difficulty with airway manage-
ment.42 Mediastinal, chest wall, or left breast irradiation can
cause pericarditis, conduction abnormalities, cardiomyopa-
thy, valvular abnormalities, and premature coronary artery
disease, even without traditional risk factors.43 Preoperative
neuroimaging, if available, should be reviewed for evidence of
metastasis.
Breast tumors often metastasize to bone and liver. Bone
lesions can result in hypercalcemia or pancytopenia. Lung
metastases can compromise pulmonary function. Paraneoplastic
syndromes can complicate almost any malignancy and may
be associated with hypercalcemia, inappropriate secretion of
antidiuretic hormone, Lambert-Eaton or Cushing's syndromes,
and neuropathy.
PRIMARY HEPATIC LYMPHOMA
The primary hepatic form is a rare presentation of lymphoma
that mainly affects middle-age or older men. The major-
ity of primary hepatic lymphomas are diffuse, large-B-cell
FIGURE 20-3  Computed tomography scan (left) and pathologic specimen (right) of patient with primary hepatic lymphoma.
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Chapter 20  The Geriatric Patient 583
lymphomas, and as many as 40% of patients with primary
hepatic lymphoma have an underlying immunologic abnor-
mality, including immunodeficiency caused by human immu-
nodeficiency virus (HIV). Primary hepatic lymphoma has
also been associated with infection with hepatitis B or hepati-
tis C virus and long-standing chronic inflammation caused by
tuberculosis.44–46 Primary hepatic lymphoma is treated similar
to lymphoma at other sites, if the diagnosis can be made before
a liver resection (Fig. 20-3).
ANESTHETIC CONSIDERATIONS
Important issues to explore include the cause and degree of
hepatic dysfunction. The presence of encephalopathy, coag-
ulopathy, ascites, volume overload, and infection risk needs
to be determined preoperatively. New-onset or worsening
encephalopathy is frequently caused by an acute insult such as
infection, GI bleeding, hypovolemia, or sedatives. It is impor-
tant to determine reversible factors and treat accordingly; lact-
ulose is first-line therapy.
Coagulopathy can be a result of vitamin K deficiency caused
by an inability to secrete bile (cholestatic disorders), ­deficiency
of coagulation factors because of loss of s­ynthetic function
as a result of cirrhosis, or thrombocytopenia ­secondary to
splenomegaly and portal hypertension. Therapy to correct
­
coagulopathy is directed at the cause. Vitamin K, fresh-­frozen
plasma (FFP), or platelets are used to correct deficiencies.
Vitamin K, 1 to 5 mg orally or subcutaneously daily for 1 to
3 days, may correct a prolonged prothrombin time (PT) and
carries minimal risk. However, the coagulopathy in patients
with synthetic failure will probably not correct with such mea-
sures, and performing a type and screen will prepare the patient
for platelet and FFP transfusions, with the goal of achieving
a platelet count higher than 50,000/mm3 and ­international
­normalized ratio (INR) less than 1.5, respectively.
Reduction of ascites preoperatively may decrease the
risk of wound dehiscence and improve pulmonary func-
tion. Sodium restriction (in diet and IV solutions), diuretics
(especially spironolactone, which inhibits aldosterone), and
even paracentesis are useful. If paracentesis is ­performed, it
is important to analyze the fluid for infection. Correction
of anemia is controversial but may limit renal dysfunc-
tion. Lactulose, 30 mL orally every 6 hours for 3 days before
 584 ANESTHESIA AND UNCOMMON DISEASES
surgery, with the last dose given within 12 hours of surgery,
or oral bile salts with IV hydration beginning the night before
surgery, may reduce perioperative progression of renal dis-
ease in patients at risk.47
CREUTZFELDT-JAKOB DISEASE
Creutzfeldt-Jakob disease (CJD) is a rare illness that may be
acquired by infection and secondarily transmitted. The most
common form (sporadic CJD) affects only approximately 1 to
2 per 1 million population per year, most of whom are mid-
dle aged or elderly. CJD is a spongiform encephalopathy, clas-
sified as a prion disease.48 A limited number of other prion
diseases are known; kuru is linked to cannibalism in Papua,
New Guinea, and Gerstmann-Sträussler-Scheinker syndrome
is usually inherited. Other diseases of this type have been doc-
umented in animals.49
All these prion diseases are widespread degenerative
­diseases of the central nervous system with long incubation
periods. Once symptoms occur, there is a rapid progression to
death, typically in 6 months. Patients with sporadic CJD have
dementia of rapid onset, cerebellar dysfunction with ataxia,
increased tone, and sometimes myoclonus. Cortical blind-
ness may occur, as well as rapid deterioration with epileptic
seizures. Diagnosis of CJD is usually made clinically and con-
firmed by brain biopsy. Treatment is symptomatic because no
satisfactory therapy is yet available.
The infective agent can only be isolated from the brain,
spinal cord, and other tissues. Iatrogenic transmission of
­ 19. Gross TJ, Hunninghake GW: Idiopathic pulmonary fibrosis, N Engl J Med
­prions can occur in neurosurgical procedures, corneal grafts,
and with growth hormone injections obtained from cadaveric
pituitaries.50 Currently the risk of transmitting prions causing
CJD is unknown. It was found in the lymphoreticular system
in 1999 during a tonsillar biopsy.
ANESTHETIC CONSIDERATIONS
Any tissue or body fluid should be considered potentially
infectious. Accidental transmission has occurred by con-
taminated instruments as well as a contaminated dural graft.
Particular care should be taken during any brain biopsy in
patients with undiagnosed dementia. If CJD is suspected,
biopsy may not be advisable. The anesthesiologist should be
gowned and gloved and masked appropriately with water-
proof garments.51 All equipment should be disposable and
incinerated if possible.
Anesthetic management may be complicated by autonomic
dysfunction in patients with Creutzfeldt-Jakob disease.
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