Ebook Consults in obstetric anesthesiology: Part 2

Continued part 1, part 2 of ebook Consults in obstetric anesthesiology provide readers with content about: inherited thrombophilias; insulin pump; intracranial hypertension; intracranial mass lesions; Jehovah’s witnesses; Kawasaki disease; Klippel-Trenaunay syndrome; left ventricular assist devices (LVAD);... Please refer to the part 2 of ebook for details!

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_89

James P R Brown and Joanne Douglas

Background: Epidemiology

and Pathophysiology

Presentation

1 Parturients with inherited thrombophilias may present in

several ways Women may present after investigation for

a previous personal history of arterial or venous

thrombo-embolism (VTE)

2 Parturients may also be diagnosed after screening

follow-ing a confirmed family history of thrombophilia (Please

see Fig 89.1 for a depiction of the coagulation cascade.)

Incidence

1 VTE is a major cause of pregnancy mortality and

morbid-ity, affecting 0.5–3 per 1000 pregnancies [1] The rate of

VTE is increased six-fold in pregnancy compared with

the nonpregnant state, and inherited thrombophilias are

responsible for up to 50% of VTEs occurring in

preg-nancy [2] More VTEs occur in the left leg (the right iliac

artery causes mechanical compression of the left iliac

vein) [3]

2 In parturients with a previous personal history of VTE,

the risk for recurrence during pregnancy is as much as

12% [1]

3 Genes associated with an increased risk of VTE are ried by 15% of the Caucasian population [2] Factor V Leiden is the most common inherited thrombophilia

Interaction with Pregnancy Effects of Pregnancy on Disorders

1 By term gestation, the normal hematological response to pregnancy is to increase most coagulation factors and to decrease anticoagulant and fibrinolytic activity

2 This creates a prothrombotic environment, which tionarily protects against postpartum hemorrhage (PPH) (Please see Table 89.1.)

3 Because of the hypercoagulable state, inherited philias may present for the first time during pregnancy (Please see Table 89.2.)

4 In addition to the hypercoagulability of normal pregnancy and inherited thrombophilias, there are additional risk factors for VTE that may be present These include:

(a) Venous congestion of lower limbs (gravid uterus) (b) Bed rest for obstetric disorders

(c) Morbid obesity (d) Cesarean delivery (e) Chorioamnionitis (f) Advanced maternal age (g) Acquired thrombophilias, e.g., antiphospholipid syn-drome and nephrotic syndrome

Effects of Disorder on Pregnancy

1 Inherited thrombophilias are probably associated with an increased incidence of recurrent pregnancy loss, pre-eclampsia, and placental abruption

J P R Brown, MBChB, MRCP, FRCA, MD, FRCPC ( * )

UBC Department of Anesthesiology, Pharmacology and

Therapeutics, BC Women’s Hospital, Vancouver, BC, Canada

Department of Anesthesia, BC Women’s Hospital,

Vancouver, BC, Canada

e-mail: james.brown@cw.bc.ca

J Douglas, CM, MD, FRCPC

UBC Department of Anesthesiology, Pharmacology and

Therapeutics, BC Women’s Hospital, Vancouver, BC, Canada

e-mail: jdouglas@cw.bc.ca

89

Neonatal and Fetal Considerations

1 Inherited thrombophilias are associated with:

(a) Intrauterine growth restriction

(b) Intrauterine fetal death

(c) Ischemic strokes in neonates peripartum (neonatal

seizures, hemiparesis, cerebral palsy)

Management: Medical and Anesthetic

General Principles

1 An assessment for inherited thrombophilias should be

performed prior to pregnancy when possible

2 Where possible there should be an antenatal

multidisci-plinary (obstetric, anesthetic, hematology) meeting and

plan for management

3 Management should be tailored to the specific patient, according to individual risk stratification of likelihood of VTE during pregnancy

4 It is important that parturients are informed of signs and symptoms of VTE and encouraged to present urgently for medical assessment should they occur

5 There should be an agreed plan for management of coagulation peripartum, balancing individual risk of VTE with risk of PPH and allowing the opportunity for neur-axial analgesia or anesthesia

6 The risks and benefits of analgesic and anesthetic options should be discussed with the patient as part of the process

of informed consent

7 On arrival to the labor suite, a complete blood count (CBC), coagulation studies as indicated, and a type and screen should be ordered Adequate intravenous access should be obtained

8 In addition to any anticoagulation required, simple ods of reducing VTE should be employed, such as early mobilization, adequate hydration, and mechanical com-pression devices

9 The greatest risk of VTE is in the 6 weeks postpartum; prophylaxis needs to be extended for this period

Testing and Diagnosis [1]

1 Who should be screened?

(a) Patients with a previous personal or family history of VTE should be screened

2 When should screening happen?

Intrinsic pathway

Cryoprecipitate

Cryoprecipitate PCC

PCC rFVIIa Extrinsic

pathway

Final common pathway

FXII FXI

FIX

FX

Prothrombin (FII) Thrombin

Fiberinogen (FI) Fiberin Fiberin Clot

FXIIIa

FX FXa

FIXa VIIIa

Va

FVIIa

TF FVII FXIa

activated, TF tissue factor

Table 89.1 Changes in blood components at term with normal

preg-nancy, compared to baseline

Parameter Changes at term gestation

Abbreviations: F factor, P protein

a Although levels of protein C are unchanged, normal pregnancy is a

state of relative resistance to activation of protein C, a result of decreased

availability of protein S and increased factor VIII levels [4]

J P R Brown and J Douglas

(a) Ideally, screening and testing for thrombophilias

should take place when a patient is not pregnant and

not directly following a thrombotic event or while

being treated with anticoagulants

3 What screening tests should be ordered?

(a) Factor V Leiden

(b) Prothrombin gene mutation

(c) Antithrombin deficiency

(d) Protein C deficiency

(e) Protein S deficiency

4 It is important to know the exact diagnosis (including

confirmation of homozygous or heterozygous status),

because when considered with the personal history of

VTE, the diagnosis contributes to risk assessment for

recurrence of VTE in pregnancy The diagnosis ultimately

helps decision-making as to whether to anticoagulate the

patient during pregnancy and to what extent

Medical Management

1 For information on specific inherited prothrombotic

dis-orders, please see the appropriate chapters: 10

Antithrombin deficiency, 58 Factor V leiden, 58

Prothrombin gene mutation, 131 Protein C deficiency,

and 131 Protein S deficiency

2 Decisions on anticoagulation during pregnancy are

dependent on the risk of VTE occurring based on the

fol-lowing factors [1]:

(a) Personal history of VTE

(b) Number of previous VTEs

(c) Administration of long-term anticoagulation prior to

pregnancy

(d) Presence of specific inherited thrombophilias, classed

as either high or low risk of recurrent VTE as

4 There is also a choice of anticoagulation agents:

(a) Unfractionated heparin (UFH), either administered subcutaneously or intravenously Dose requirements increase as pregnancy progresses

to induction of labor or planned cesarean delivery, and to advise parturients to withhold the next dose of LMWH if she suspects she is going into labor [5]

Table 89.2 Risk of venous thromboembolism with specific thrombophilias in pregnancy

Prevalence in general population (%)

VTE risk per pregnancy (no history) (%)

VTE risk per pregnancy (previous VTE) (%)

Percentage of all VTE

Prothrombin gene mutation

Reproduced from the American Congress of Obstetricians and Gynecologists (ACOG) Practice Bulletin No 138(1)

Table 89.3 Thrombophilias with high and low risk of VTEs during

pregnancy

Factor V Leiden (heterozygous) Factor V Leiden (homozygous) Prothrombin gene mutation

Anesthetic Management

1 Labor analgesia may involve neuraxial block or

noninva-sive methods The latter should be discussed with

parturi-ents who have a contraindication to a neuraxial technique

(a) Non-pharmacological labor analgesia may involve:

• Relaxation, breathing exercises, and water bath

• Transcutaneous electrical nerve stimulation (TENS)

(b) Pharmacological labor analgesia may be achieved

with the following interventions:

• Intravenous opioids such as remifentanil

• Inhalation of Entonox (nitrous oxide and oxygen 50:50)

2 Labor epidural analgesia

(a) Placement of labor epidural analgesia needs to be

timed appropriately with the last dose of

anticoagula-tion, according to ASRA guidelines [5]

(b) No routine blood monitoring is recommended for

parturients receiving LMWH e.g., anti-Xa levels

(c) After prophylactic dosing of LMWH, 10–12 h should

elapse post-dose prior to attempting a neuraxial

procedure

(d) After therapeutic dosing of LMWH, wait 24 h post-

dose prior to attempting neuraxial procedure

(e) The most experienced clinician available should

per-form neuraxial procedures in these patients

(f) If the clinician is skilled in pre-puncture ultrasound,

this may decrease the risks of a bloody tap associated

with multiple attempts at insertion

(g) The lowest concentration of local anesthetic that

achieves analgesia should be used to allow

monitor-ing of motor function If the block density is greater

than expected, parturients should be actively

man-aged and investigated to exclude an epidural

hema-toma Urgent magnetic resonance imaging (MRI) is

the imaging of choice if there is clinical suspicion

(h) Please refer to the chapter on “anticoagulation

chapter 8.”

3 Anesthesia for cesarean delivery

(a) Similar considerations discussed above with labor

epi-dural analgesia apply to cesarean delivery anesthesia

(b) Spinals have relatively less risk of causing an

epi-dural hematoma when compared with epiepi-durals The

use of a smaller needle, a simpler technique, and no

indwelling catheter confers protections against spinal hematoma

(c) Caution is advised when combining aspirin or steroidal anti-inflammatories with anticoagulation given an increased hemorrhagic risk

neur-be investigated, i.e., urgent MRI and discussion with neurosurgery

4 VTE risk is greatest for 6 weeks postpartum In most cases, antepartum anticoagulation should be continued or increased for this 6 week postpartum period

References

1 American College of Obstetricians and Gynecologists Inherited thrombophilias in pregnancy Practice Bulletin no 138 Obstet Gynecol 2013;122:706–17.

2 Calderwood C, Greer I The role of factor V Leiden in nal health and the outcome of pregnancy Curr Drug Targets 2005;6:567–76.

3 Battinelli E, Bauer K Thrombophilias in pregnancy Hematol Oncol Clin N Am 2011;25:323.

4 Pierangeli S, Leader B, Barilaro G, Willis R, Ware Branch D Acquired and inherited thrombophilia disorders in pregnancy Obstet Gynecol Clin N Am 2011;38:271–95.

5 Horlocker T, Wedel D, Rowlingson J, Enneking F, Kopp S, Benzon H, et al Regional anesthesia in the patient receiving antithrombotic or thrombolytic therapy Reg Anesth Pain Med 2010;35:64–101.

6 Food and Drug Administration Updated recommendations to decrease risk of spinal column bleeding and paralysis in patients on low molecular weight heparins Drug Safety Communications 2013 http://www.fda.gov/downloads/Drugs/DrugSafety/UCM373735 pdf Accessed 4th May 2016.

J P R Brown and J Douglas

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_90

Catherine Traill and Stephen H Halpern

Presentation and Symptoms

Definition

An insulin pump is a device that provides a continuous

sub-cutaneous insulin infusion and may also have the ability to

provide continuous glucose monitoring

Incidence

During pregnancy insulin pumps are used primarily by type 1

diabetics Type 1 diabetes affects almost 1 in 300 people by

the age of 18 [1] In North America it is estimated that between

20 and 30% of type 1 diabetics use an insulin pump [2]

Interaction with Pregnancy

Effects of Pregnancy on Insulin Pump Use

1 Typically, insulin requirements decrease in the first

tri-mester of pregnancy, before increasing in the second and

third trimesters Increases of up to 181% from

prepreg-nancy doses have been reported [3] (see Fig 90.1)

Effects of Insulin Pump Use on Pregnancy

1 In infants born to type 1 diabetic mothers, four out of five

will suffer at least one complication related to diabetes [4]

2 Fetal complications include fetal macrosomia, neonatal

hypoglycemia, congenital malformations, premature

birth, and fetal death

3 Maternal risks of type 1 diabetes include severe cemia, diabetic ketoacidosis, preeclampsia, and increased risk of Cesarean or instrumental delivery

4 A large retrospective cohort study demonstrated that the use of an insulin pump, when compared with multiple daily insulin injections, resulted in a lower HbA1c, with

no increased risk of severe hypoglycemia or diabetic acidosis in pregnant patients with type 1 diabetes [5] The same study, however, did not demonstrate an improvement

keto-in pregnancy outcomes with respect to rate of Cesarean delivery or large for gestational age neonates

Medical Management Testing and Diagnosis

1 Type 1 diabetes is usually diagnosed prior to pregnancy, most commonly during childhood or adolescence

C Traill, M.D • S H Halpern, M.D ( * )

Department of Anesthesia, Sunnybrook Health Sciences Centre,

University of Toronto, Toronto, ON, Canada

e-mail: stephen.halpern@sunnybrook.ca

90

PC

1.4 1.2 1.0 0.8

Units / kg 0.6

0.4 0.2 0.0

Gestational age

Basal Bolus

Fig 90.1 Basal and bolus insulin dosing across gestation PC

precon-ception (Courtesy of Elsevier, Roeder et al (2012) Insulin pump dosing across gestation in women with well-controlled type 1 diabetes melli- tus American Journal of Obstetrics & Gynecology, 207(4), 324, e1–e5)

Medical Management

1 The goal of insulin pump therapy is to replace insulin in

a manner that resembles normal physiological insulin

delivery over a 24 h period This is achieved via basal

and bolus infusions of short-acting insulin [2]

2 The basal infusion rate is tailored specifically to an

indi-vidual, taking into account the patient’s sensitivity to

insulin and activity level It usually accounts for 50% of

the total daily insulin requirement in adults [2]

3 The bolus doses are required at meal times and can be

calculated by the insulin infusion pump, once the

patient’s current blood glucose level and amount of

car-bohydrate to be ingested are entered The pump is also

able to calculate how much insulin is still active in the

body, a feature which prevents the delivery of too much

insulin, and therefore protects against hypoglycemia [2]

4 Due to the fact that only short-acting insulin is infused

by an insulin pump, disconnection, occlusion, or pump

failure can induce insulin deficiency within an hour,

with complete insulin deficiency and subsequent risk of

hyperglycemia and ketosis within 4 h

5 Infusion catheters and insertion sites should be changed

every 2–3 days to prevent problems with absorption

6 The American Diabetes Association recommends an

HbA1c below 7.0% in type 1 diabetics prior to

becom-ing pregnant [6]

7 Tight glycemic control is recommended during

preg-nancy, with a target HbA1c of <6.0% and blood glucose

levels of 3.3–5.4 mmol/L before meals and at night and

5.4–7.1 mmol/L peak postprandial [6]

8 During labor, hourly testing and blood glucose levels of

between 3.9 and 6.1 mmol/L are recommended Patients

using an insulin pump should continue their basal

infu-sion rate during labor [7]

9 Strict glycemic control during labor has been shown to

reduce the risk of neonatal hypoglycemia [8]

10 A retrospective study involving 68 pregnant women

demonstrated that the use of insulin pump therapy is safe

to continue during labor and for Cesarean delivery

Appropriate blood glucose levels were achieved by all

patients, with no one requiring conversion to an

intrave-nous insulin infusion [9]

Anesthetic Management

Medication

1 Diabetes is not a contraindication to the antenatal

admin-istration of corticosteroids for fetal lung maturation

Additional insulin may be required in these cases [10]

2 Beta-agonists should be avoided for tocolysis in diabetic patients due to the potential for hyperglycemia [10]

Neuraxial Analgesia

1 Neuraxial analgesia is safe in diabetic patients and ates the increases in cortisol and adrenaline, which may lead to improved glucose control [11]

2 Any pre-existing neuropathy should be carefully mented prior to performance of neuraxial analgesia

Anesthesia for Cesarean Delivery

1 If operative delivery is required, either general or axial anesthesia can be safely used Neuraxial anesthesia allows continuous monitoring of maternal consciousness, which may facilitate detection of hypoglycemia, and allows for earlier return to normal diet

2 Diabetes is associated with gastroparesis which places these patients at an increased risk of aspiration in the event of general anesthesia While there are no consensus guidelines regarding fasting times in diabetic obstetric patients, greater restriction of oral intake than normal may be appropriate, and this should be determined on a case-by-case basis [12]

3 The timely administration of non-particulate antacids (e.g., sodium citrate), histamine antagonists, and/or meto-clopramide should be considered for aspiration prophy-laxis in these patients [12]

4 Blood glucose levels should be checked hourly in the operative period, until the patient is tolerating oral intake and able to manage their own blood glucose control [2]

5 Guidelines from the United Kingdom recommend tinuation of insulin pump therapy at 100% of basal rate during the perioperative period in nonpregnant patients [13] No guidelines currently exist for pregnant patients

6 During surgery, the use of an infusion catheter made entirely of plastic is preferred in order to minimize the potential risk of subcutaneous burn from a metal needle with the use of diathermy

7 The site of infusion to be used during surgery should be tant from the surgical site, away from the diathermy field, and readily accessible at all times by the anesthetist [2

Postoperative Management

1 Insulin requirements are reduced to lower than nancy levels in the postpartum period Close blood glu-cose monitoring should continue during this time [14]

prepreg-C Traill and S H Halpern

2 Post-delivery, insulin pump therapy should be ceased

temporarily to avoid hypoglycemia Once the serum

blood glucose level reaches >5.5 mmol/L, the insulin

pump may be restarted at prepregnancy levels [15]

3 Subcutaneous insulin bolus therapy may be recommenced

with the first ingestion of carbohydrates postoperatively

4 Breastfeeding mothers have a significantly decreased basal

insulin requirement of around 36%, thought to be due to

the use of glucose for milk production [16]

References

1 Maahs DM, West NA, Lawrence JM, Mayer-Davis

EJ Epidemiology of type 1 diabetes Endocrinol Metab Clin N Am

2010;39(3):481–97.

2 Partridge H, Perkins B, Mathieu S, Nicholls A, Adeniji K Clinical

recommendations in the management of the patient with type 1

dia-betes on insulin pump therapy in the perioperative period: a primer

for the anaesthetist Br J Anaesth 2016;116(1):18–26.

3 Roeder HA, Moore TR, Ramos GA Insulin pump dosing across

gestation in women with well-controlled type 1 diabetes mellitus

Am J Obstet Gynecol 2012;207(4):324, e1 – e5.

4 Evers IM, de Valk HW, Visser GH Risk of complications of

preg-nancy in women with type 1 diabetes: nationwide prospective study

in the Netherlands Br Med J 2004;328(7445):915–21.

5 Kallas-Koeman MM, Kong JM, Klinke J, Butalia S, Lodha AK,

Lim KI, Duan QM, Donovan LE Insulin pump use in pregnancy is

associated with lower HbA1c without increasing the rate of severe

hypoglycemia or diabetic ketoacidosis in women with type 1

diabe-tes Diabetologica 2014;57(4):681–9.

6 American Diabetes Association Standards of medical care in

dia-betes Diabetes Care 2014;37(Suppl 1):S14–80.

7 American College of Obstetricians and Gynecologists’ Committee on

Practice Bulletins Clinical management guidelines for

Obstetrician-Gynecologists Number 60, Pregestational diabetes mellitus Obstet Gynecol 2005;105(3):675–85.

8 Stenninger E, Lindqvist A, Aman J, Ostlund I, Schvarcz

E Continuous subcutaneous glucose monitoring system in betic mothers during labor and postnatal glucose adaptation of their infants Diabet Med 2008;25(4):450–4.

9 Fresa R, Visalli N, Di Blasi V, Cavallaro V, Ansaldi E, Trifoglio O, Abbruzzese S, Bongiovanni M, Agrusta M, Napoli A Experiences

of continuous subcutaneous insulin infusion in pregnant women with Type 1 Diabetes during delivery from four Italian Centers: a retrospec- tive observational study Diabetes Technol Ther 2013;15(4):328–34.

10 Mugglestone MA Guidelines: Management of diabetes from conception to the postnatal period: summary of NICE guidance Br Med J 2008;336(7646):714–7.

11 McAnulty GR, Robertshaw HJ, Hall GM Anaesthetic management

of patients with diabetes mellitus Br J Anaesth 2000;85(1):80–90.

12 American Society of Anesthesiologists Practice guidelines for obstetric anesthesia: an updated report by the American Society

of Anesthesiologists Task Force on Obstetric Anesthesia and the Society for Obstetric Anesthesia and Perinatology Anesthesiology 2015;124(2):270–300.

13 Joint British Diabetes Societies for Inpatient Care Group (2011) Management of adults with diabetes undergoing surgery and elec- tive procedures: improving standards Report of a joint working party NHS Diabetes Available at http://www.diabetologists-abcd org.uk/jbds/JBDS_IP_Surgery_Adults_Full.pdf

14 Roeder HA, Moore TR, Ramos GA Changes in postpartum insulin requirements for patients with well-controlled type 1 diabetes Am

J Perinatol 2016;33(7):683–7.

15 Grunberger G, Abelseth J, Bailey T, Bode B, Handelsman

Y, Hellman R, Jovanovic L, Lane W, Raskin P, Tamborlane

W, Rothermel C Consensus statement by the American Association of Clinical Endocrinologists/American College of Endocrinology insulin pump management task force Endocr Pract 2014;20(5):463–89.

16 Riviello C, Mello G, Jovanovic L Breastfeeding and the basal lin requirement in type 1 diabetic women Endocr Pract 2009;15(3): 187–93.

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_91

Intracranial Hypertension

Rakesh B Vadhera and Rovnat Babazade

Introduction

Major causes of intracranial hypertension (ICH) in women

of childbearing age are similar to the general population

except idiopathic intracranial hypertension (IIH) [1]

1 The IIH previously termed as “benign intracranial

hyper-tension” or “pseudotumor cerebri” is defined as an

increase in intracranial pressure (ICP) without a

demon-strable etiology and is a diagnosis of exclusion [2]

2 The incidence of IIH in adult women is approximately

0.9/100,000, which increases to 19.3/100,000 in obese

nonpregnant women [3]

3 Data suggests that the prevalence of obesity in pregnancy

has increased from 10% to 16–19% in the last two decades

[4] Therefore, with the increasing obesity during

preg-nancy epidemic in the world, the incidence and

preva-lence of IIH in pregnancy are rising

Definition and Causes of Intracranial

Hypertension (ICH)

1 The skull protects the brain and all intracranial

compart-ments In healthy adults the fixed internal volume ranges

from 1400 to 1700 mL [5] Approximately 80% of this

volume is brain parenchyma, 10% is cerebrospinal fluid

(CSF), and around 10% is blood [6].The volume of brain

parenchyma is relatively constant in healthy adults,

although it can be altered by mass lesions or in the setting

of cerebral edema

2 However the rest of the 20% intracranial volumes (CSF

and blood) are frequently prone to changes and are

responsible for the most common causes of elevated intracranial pressure

3 In adults normal intracranial pressure is defined as sure ≤15 mmHg, and elevated intracranial hypertension (ICH) is present at pressures ≥20 mmHg [5 7]

4 Homeostatic mechanisms stabilize intracranial pressure (ICP), with occasional transient elevations associated with physiologic events (e.g., coughing, sneezing, and Valsalva maneuver)

5 The major causes of ICH in pregnancy are summarized in Table 91.1

6 Cerebral blood flow and perfusion pressure (a) Cerebral blood flow (CBF) is the blood supplied to the brain in a given period of time which is typically

750 mL/min or 15% of the total cardiac output [8] CBF is tightly regulated to meet the brain’s meta-bolic demands and increases with hypercapnia and hypoxia [9]

(b) CBF is a function of the pressure drop across the cerebral circulation divided by the cerebrovascular resistance [10]

(c) CBF = (CAP − JVP) ÷ CVR

CAP is carotid arterial pressure, JVP is jugular venous pressure, and CVR is cerebrovascular resistance

R B Vadhera, M.D., F.R.C.A ( * ) • R Babazade, M.D

Department of Anesthesiology, University of Texas Medical

Branch, Galveston, TX, USA

e-mail: rbvadher@UTMB.EDU ; rb@or.org ; robabaza@UTMB.EDU

91

Table 91.1 The major causes of intracranial hypertension

• Idiopathic intracranial hypertension (pseudotumor cerebri)

• Traumatic brain injury/intracranial hemorrhage

• Arteriovenous malformation or other vascular anomalies

• Subdural, epidural, or intraparenchymal hemorrhage

• Central nervous system infections

• Ischemic stroke

• Ruptured aneurysm

• Diffuse axonal injury

• Neoplasm and space-occupying lesions

• Vasculitis

• Hydrocephalus

(d) Cerebral perfusion pressure (CPP) is a clinical

sur-rogate for the adequacy of cerebral perfusion CPP is

defined as mean arterial pressure (MAP) minus

ICP Conditions associated with elevated ICP can be

associated with a reduction in CPP This can result in

devastating focal or global ischemia

7 Cerebrospinal fluid (CSF)

(a) CSF is produced from blood plasma filtration and is

basically similar, except that CSF is nearly

protein-free compared with plasma and has some modified

electrolyte levels

(b) CSF is continuously reabsorbed via the arachnoid

granulations into the venous system [11], and CSF

characteristics are presented in Table 91.2

(c) A new hypothesis suggests that there is no

unidirec-tional CSF circulation, but cardiac cycle-dependent

bidirectional systolic-diastolic to-and-fro

cranio-spi-nal CSF movements [12]

(d) Problems with CSF regulation generally result from

impaired outflow caused by ventricular obstruction

or venous congestion; the latter can occur in patients

with sagittal (or other) venous sinus thrombosis

Much less frequently, CSF production can become

pathologically increased; this may be seen in the

con-ditions such as choroid plexus papilloma

Pathogenesis of IIH (Pseudotumor Cerebri)

1 An “IHH” or “pseudotumor cerebri” or “benign intracranial

hypertension” PubMed Medical Subject Headings returns

4318 publications between 1949 and 2016 There are 1011

publications in the 1990s and 1073 publications just in the

last 6 years IIH appears to be on the rise Regardless of so

many articles, we know little more now than we did 50 years

ago about the cause of this mysterious disease

2 While the pathogenesis of IIH is unknown, risk factors

(Fig 91.1) and associated conditions (Table 91.3) have

been identified, and numerous theories postulating the

cause for IIH have been advanced The list of the

well-known proposed theories and their underlying nisms is presented in Fig 91.2

3 Much attention has been placed on the association of IIH with venous sinus stenosis However, most of the evi-dence at present suggests that apparent venous sinus nar-rowing is secondary to increased intracranial pressure and

is not the primary cause in most cases Proposed gies include cerebral venous outflow abnormalities (e.g., venous stenoses and venous hypertension), increased CSF outflow resistance at either the level of the arachnoid

etiolo-Table 91.2 Cerebrospinal fluid characteristics

Cerebrospinal fluid characteristics [ 13 ]

Clear, colorless

Circulating volume 100–160 mL

20 mL/h CSF produced by choroid plexus

White blood cell count 0–5 cells/ μL

Red blood cell count 0–10 cells/ μL

Riskfactors

Female

Reproductive age

Obesity

Recent weight gain

Family history

Fig 91.1 Known risk factors for idiopathic intracranial hypertension

Retinoids (isotretinoin and tretinoin) [ 19 ] Thyroid replacement [ 20 ]

Lithium [ 21 ] Corticosteroid withdrawal [ 22 ]

Systemic illnesses

Addison disease [ 23 ] Hypoparathyroidism [ 24 ] Anemia [ 25 ]

Sleep apnea [ 26 ] Polycystic ovary syndrome [ 27 ] Systemic lupus erythematosus (SLE) [ 28 ] Behcet’s syndrome [ 29 ]

Coagulation disorders [ 27 ]

granulations or CSF lymphatic drainage sites,

obesity-related increased abdominal and intracranial venous

pres-sure, altered sodium and water retention mechanisms, and

abnormalities of vitamin A metabolism [14]

Clinical Presentation

1 Recent studies showed that headache (84–92%), transient

visual obscurations (68–72%), tinnitus (52–60%),

photopsia (48–54%), back pain (53%), retrobulbar pain

(44%), diplopia (18–38%), and visual loss (26–32%)

were the most common symptoms of IIH [16, 30]

2 IIH is a disorder defined by clinical criteria alone that

include symptoms and signs isolated to those produced

by increased ICP (e.g., neck stiffness, headache,

papill-edema, vision loss) Many patients suffer from

intracta-ble, disabling headaches, and there is a risk of severe,

permanent vision loss

Diagnosis and Evaluation

1 IIH is defined as an increase in ICP without a

demon-strable etiology and is a diagnosis of exclusion [31] CSF

composition is normal, and no other cause of ICH is

evi-dent on neuroimaging or other evaluations

2 IIH is diagnosed according to the modified Dandy criteria summarized in Table 91.4 [32]

3 Neuroimaging (a) Increased ICP should be suspected in a patient with headache and papilledema

(b) Urgent neuroimaging is required to exclude ary causes of ICH Magnetic resonance imaging (MRI) with magnetic resonance venography (MRV)

second-is the preferred test, which does not involve radiation and can be safely performed in the pregnant patient (c) Another important tool for diagnosis of IIH is ele-vated opening pressure on lumbar puncture Furthermore, a complete ocular examination should document formal visual field examination, dilated fundus examination, and optic nerve photographs

Management Neurologic

1 Treatment consists of serial lumbar punctures to drain CSF Brain stem herniation usually does not occur, because the increase in ICP is uniformly distributed throughout the CNS

2 However, two cases of cerebellar tonsillar herniation have been reported after diagnostic lumbar puncture in patients with this syndrome [33] These patients presented with severe headache, neck pain, and focal neurologic signs

3 If the patient experiences progressive loss of vision, optic nerve decompression/fenestration [34, 35] or a shunting procedure, commonly a lumboperitoneal shunt, is usually required [36]

Effect of Pregnancy and Obstetric Management

1 Symptoms usually worsen during pregnancy [37, 38] gesting a hormonal etiology There is a report noting that visual loss occurred with the same frequency in pregnant

sug-Theories for IIH

Venous sinus stenosis

Intracranial venous pressure

Cerebral edema

↑ CSF production

or impaired absorption

Table 91.4 Modified Dandy criteria

Diagnostic criteria for idiopathic intracranial hypertension

• Symptoms and signs of increased intracranial pressure

• No other neurologic abnormalities or impaired level of consciousness except VI cranial nerve paresis

• Elevated intracranial pressure (>25 cm H 2 O) with normal CSF composition

• A neuroimaging study that shows no etiology for intracranial hypertension

• No other causes of intracranial hypertension apparent

91 Intracranial Hypertension

and nonpregnant patients and that obstetric complications

occurred more frequently in the controls [39]

2 The condition usually resolves postpartum

3 Obstetric management should not be significantly altered

by this disorder Treatment of IIH patients in pregnancy

should be the same as for nonpregnant IIH patients,

except that calorie restriction and acetazolamide use are

contraindicated Obstetric management is no different

from that of normal pregnancy [39]

Anesthetic Management

1 General anesthesia may be required for placement of a

lumboperitoneal shunt The usual precautions for

non-obstetric surgery during pregnancy are applied

2 Labor and delivery Central neuraxial analgesia for labor

and delivery has been described and deemed to be safe [34]

3 Cesarean delivery

(a) If a lumboperitoneal shunt is in place, general

anes-thesia may be preferable for cesarean section The

impact of the shunt on the extension of regional

anes-thesia is unknown; given unknown CSF volume or

pressure, it also might be difficult to adjust the dose

of local anesthetic

(b) Spinal anesthesia has been successfully used in

partu-rients with IIH and with a continuous CSF leak from

the base of the skull, with a presumably lower CSF

volume and pressure [40] It should be noted that

par-turients with IIH and associated headache often

undergo a diagnostic spinal tap Following this they

have the potential to develop a postdural puncture

headache, and an epidural blood patch may be

con-sidered Blood injected in the epidural space may

increase ICP as a result of hydraulic compression of

the dura mater

References

1 Thirumalaikumar L, Ramalingam K, Heafield T Idiopathic

intra-cranial hypertension in pregnancy Obstetrician Gynaecologist

2014;16(2):93–7.

2 Kapoor KG More than meets the eye? Redefining idiopathic

intra-cranial hypertension Int J Neurosci 2010;120:471–82.

3 Durcan FJ, Corbett JJ, Wall M The incidence of pseudotumor

cerebri Population studies in Iowa and Louisiana Arch Neurol

1988;45:875–7.

4 CMACE/RCOG Joint Guideline 2010 https://www.rcog.org.uk/

globalassets/documents/guidelines/cmacercogjointguidelineman-agementwomenobesitypregnancya.pdf

5 Rangel-Castilla L, Rangel-Castillo L, Gopinath S, Robertson

CS Management of intracranial hypertension Neurol Clin

2008;26:521–41.

6 Kaye AH, Laws ER Jr Brain tumors: an encyclopedic approach

New York: Churchill Livingstone; 2001.

7 Welch K The intracranial pressure in infants J Neurosurg 1980;52:693–9.

8 Querido JS, Sheel AW Regulation of cerebral blood flow during exercise Sports Med 2007;37:765–82.

9 Walters F Intracranial pressure and cerebral blood flow Physiology 1998;8:1–4.

10 Bruzzone P, Dionigi R, Bellinzona G, Imberti R, Stocchetti N Effects

of cerebral perfusion pressure on brain tissue PO2 in patients with severe head injury Acta Neurochir Suppl 1998;71:111–3.

11 Zakharov A, Papaiconomou C, Djenic J, Midha R, Johnston

M Lymphatic cerebrospinal fluid absorption pathways in neonatal sheep revealed by subarachnoid injection of Microfil Neuropathol Appl Neurobiol 2003;29:563–73.

12 Ore šković D, Klarica M A new look at cerebrospinal fluid ment Fluids Barriers CNS 2014;11:16.

13 Merril CR, Goldman D, Sedman SA, Ebert MH Ultrasensitive stain for proteins in polyacrylamide gels shows regional variation

in cerebrospinal fluid proteins Science 1981;211:1437–8.

14 Moskowitz Y, Leibowitz E, Ronen M, Aviel E Pseudotumor cerebri induced by vitamin A combined with minocycline Ann Ophthalmol 1993;25:306–8.

15 Salpietro V, Chimenz R, Arrigo T, Ruggieri M Pediatric idiopathic intracranial hypertension and extreme childhood obesity: a role for weight gain J Pediatr 2013;162:1084.

16 Wall M, George D Idiopathic intracranial hypertension A tive study of 50 patients Brain 1991;114:155–80.

17 Reeves GD, Doyle DA Growth hormone treatment and mor cerebri: coincidence or close relationship? J Pediatr Endocrinol Metab 2002;15(2):723–30.

18 Kesler A, Goldhammer Y, Hadayer A, Pianka P The outcome of pseudotumor cerebri induced by tetracycline therapy Acta Neurol Scand 2004;110:408–11.

19 Yeh YC, Tang HF, Fang IM Pseudotumor cerebri caused by trans-retinoic acid treatment for acute promyelocytic leukemia Jpn

all-J Ophthalmol 2006;50:295–6.

20 Raghavan S, DiMartino-Nardi J, Saenger P, Linder B Pseudotumor cerebri in an infant after L-thyroxine therapy for transient neonatal hypothyroidism J Pediatr 1997;130:478–80.

21 Hexom B, Barthel RP Lithium and pseudotumor cerebri J Am Acad Child Adolesc Psychiatry 2004;43:247–8.

22 Liu GT, Kay MD, Bienfang DC, Schatz NJ Pseudotumor cerebri associated with corticosteroid withdrawal in inflammatory bowel disease Am J Ophthalmol 1994;117:352–7.

23 Alexandrakis G, Filatov V, Walsh T Pseudotumor cerebri in

a 12-year-old boy with Addison’s disease Am J Ophthalmol 1993;116:650–1.

24 Sheldon RS, Becker WJ, Hanley DA, Culver RL Hypoparathyroidism and pseudotumor cerebri: an infrequent clinical association Can J Neurol Sci 1987;14:622–5.

25 Nazir SA, Siatkowski RM Pseudotumor cerebri in idiopathic aplastic anemia J AAPOS 2003;7:71–4.

26 Marcus DM, Lynn J, Miller JJ, Chaudhary O, Thomas D, Chaudhary B Sleep disorders: a risk factor for pseudotumor cere- bri? J Neuroophthalmol 2001;21:121–3.

27 Glueck CJ, Iyengar S, Goldenberg N, Smith LS, Wang P Idiopathic intracranial hypertension: associations with coagulation disorders and polycystic-ovary syndrome J Lab Clin Med 2003;142:35–45.

28 Hershko AY, Berkun Y, Mevorach D, Rubinow A, Naparstek

Y Increased intracranial pressure related to systemic lupus tosus: a 26-year experience Semin Arthritis Rheum 2008;38:110–5.

29 Celebisoy N, Seçil Y, Akyürekli O Pseudotumor cerebri: cal factors, presenting features and prognosis in the western part of Turkey Acta Neurol Scand 2002;106:367–70.

30 Wall M, Kupersmith MJ, Kieburtz KD, et al The idiopathic cranial hypertension treatment trial: clinical profile at baseline JAMA Neurol 2014;71:693–701.

33 Paruchuri SR, Lawlor M, Kleinhomer K, Mason L, Johnson C Risk

of cerebellar tonsillar herniation after diagnostic lumbar puncture

in pseudotumor cerebri Anesth Analg 1993;77:403–4.

34 Shekleton P, Fidler J, Grimwade J A case of benign intracranial

hypertension in pregnancy Br J Obstet Gynaecol 1980;87:345–7.

35 Rao DP, Rao VA Morbidly obese parturient: challenges for the

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36 Thurtell MJ, Wall M Idiopathic intracranial hypertension tumor cerebri): recognition, treatment, and ongoing management Curr Treat Options Neurol 2013;15:1–12.

37 Tang RA Management of idiopathic intracranial hypertension in pregnancy MedGenMed 2005;7:40.

38 Koontz WL, Herbert WN, Cefalo RC Pseudotumor cerebri in nancy Obstet Gynecol 1983;62:324–7.

39 Digre KB, Varner MW, Corbett JJ Pseudotumor cerebri and nancy Neurology 1984;34:721–9.

40 Schabel JE, Samora GJ, Steinberg ES Spinal anesthesia for a turient with an iatrogenic skull base defect and CSF leak J Clin Anesth 2002;14:592–4.

par-91 Intracranial Hypertension

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_92

Mohammed Faysal Malik, Ankit Patel, and Ami Attali

Introduction

Background

Primary brain tumors in women of reproductive age are rare,

yet the mortality is significant Primary intracranial mass

lesions are the fifth most common cause of cancer-related

deaths in women aged 20–39 years Metastatic intracranial

lesions are more common during pregnancy than primary

ones The incidence of intracranial mass lesions, whether

primary brain neoplasms or metastatic tumors, does not

appear to increase during pregnancy and seems to share

related characteristics with the nonpregnant patient, of

simi-lar demographics [1]

Types of Lesions [2, 3]

1 Gliomas and meningiomas represent the most common

group of primary intracranial neoplasms encountered,

accounting for 33–39% and 28–33% of cases,

respectively

2 This is followed by acoustic neuromas, 14%, pituitary

lesions, schwannomas, pilocytic astrocytomas each

accounting for 7%, and medulloblastomas, in 3% of

cases

3 There are reports of many other malignancies occurring

during pregnancy including lymphoma, meningeal

sar-coma, craniopharyngioma, ependymoma,

hemangioblas-toma, and others

4 The prevalence of metastatic brain tumors, most

com-monly from lung, breast, and colon cancers, is almost

twice that of primary brain tumors

2 Focal neurologic signs such as seizures, visual changes,

or hemiparesis may develop Thirty to fifty percent of patients with brain tumors present with seizure, and 10–30% develop seizures during the disease

3 Neurological compromise can occur due to mass effect from local brain parenchymal compression, vasogenic- and cyto-toxic-mediated edema, tumor enlargement, and raised intra-cranial pressure (ICP) with the potential for herniation

4 Presentation during pregnancy may be confused with eases such as hyperemesis gravidarum, eclampsia, or migraine headaches

(a) Careful history may help differentiate the cause

(b) For example, eclamptic seizures tend to be generalized, whereas tumor-induced seizures are characteristically focal ones that may develop into generalized ones [5] (c) Headaches associated with increased ICP are usually gradually progressive, daily, and worse with activities that increase ICP such as coughing, exertion, and bearing down [4]

Diagnosis

During pregnancy magnetic resonance imaging (MRI) is preferred to computed axial tomography due to greater reso-lution, increased sensitivity, and lack of radiation exposure

M F Malik, M.D • A Patel, M.D • A Attali, M.D., D.O ( * )

Department of Anesthesiology, Wayne State University (WSU)

School of Medicine, Henry Ford Hospital, Detroit, MI, USA

e-mail: MMALIK2@hfhs.org; aattali1@hfhs.org

92

Non-contrast MRI is better than contrast during pregnancy;

however, the use of gadolinium has not been shown to result

in birth defects [4] Ophthalmologic examination can be

helpful in the diagnosis and management of women with

brain tumors [6]

Interaction with Pregnancy

1 The frequency of primary brain tumors is not changed by

pregnancy

2 The incidence of symptomatic brain tumors during

preg-nancy may sometimes be less This could be due to

increased serum levels of circulating endogenous

steroids [4]

3 Some physiologic changes of pregnancy may be

detri-mental to women with brain tumors Cerebral blood flow

(CBF) increases during normal pregnancy The increased

blood volume and cardiac output that occurs during

preg-nancy combined with decreased plasma osmolality,

increased-free water, and sodium retention could lead to

or worsen cerebral edema There is also estrogen-

mediated vasodilation that increases CBF, which in the

third trimester is greater than 20% of values prior to

preg-nancy Thus increased CBF and edema could contribute

to increased ICP [3 5]

4 Some tumors may grow due to the hypervolemia and

hor-monal changes described above that occur during

preg-nancy [7]

(a) For example, benign meningiomas may enlarge due

to edema and hormones, given that 70–90% have

pro-gesterone receptors and 33–38% have estrogen

recep-tors Acoustic neuromas may also grow during

pregnancy due to these physiologic changes [4 8 9]

(b) There is physiologic enlargement of pituitary gland

during pregnancy due to estrogen and prolactin, and a

previously asymptomatic pituitary adenoma may

therefore become clinically apparent Five to fifteen

percent of pregnant women with pituitary tumors

may develop enlargement of the tumor along with

gland growth [4] In the fraction of patients that are

symptomatic, visual field defects are typically

wors-ened during pregnancy, and in tumors with

hypotha-lamic compression, diabetes insipidus may ensue

Such tumors may also secrete adenohypophyseal

hormones such as growth hormone and prolactin [2]

Clinical symptoms often improve postpartum, with

resolution of edema and normalization of blood

volume

(c) Pregnancy may also affect glioma growth through

activation of hormone receptors, particularly

proges-terone ones, and secretion of placental growth

hor-mone and other angiogenic factors that increase

cerebral blood volume This may be more common in high-grade gliomas and requires additional research [6, 7 10]

(d) Of the metastatic brain tumors, choriocarcinoma is the only one associated with a higher incidence dur-ing pregnancy, due to its insidious nature presenting late with clinically detectable metastases in up to 9–20% of patients at the time of diagnosis [11] Given that metastatic disease occurs after molar pregnancy, abortion, and ectopic or term pregnancy, treatment can be undertaken early

5 Neurologic deterioration during pregnancy is associated with preterm delivery, increased cesarean delivery (CD) rates, and admission to the neonatal intensive care unit [3]

Management Medical

Treatment for intracranial lesions in pregnancy takes into account the particular tumor cell type, symptomatology, and fetal gestational age Some tumors require immediate and aggressive treatment, and therefore termination is recom-mended if the fetus is not viable Treatment with chemother-apy, radiation therapy (RT), and surgical resection can sometimes be delayed if the tumor is less aggressive or if presentation occurs later in gestation, close to delivery

1 Steroids are commonly administered to treat atic tumors and may help to delay chemotherapy, RT, and surgery until after delivery Steroids decrease vasogenic edema surrounding the tumor and may enable earlier delivery by increasing fetal surfactant production

2 Antiepileptic medications are also used when indicated Some of these are associated with birth defects and are avoided However, several have been used with compli-cation, and the risk of uncontrolled seizures outweighs that of medications Lamotrigine and levetiracetam are often used [6]

3 Chemotherapy is usually delayed until after delivery However, some tumors have been treated with medica-tions such as bromocriptine, dopamine analogues, and octreotide, which have an acceptable safety profile

4 Carmustine-impregnated wafers have been used in nant patients with glioma Other chemotherapeutic agents

preg-to treat glioma such as temozolomide and bevacizumab are not typically administered during pregnancy [4]

5 RT has been associated with birth defects, malignancies

in offspring, and mental retardation RT is therefore not given during the first trimester and is usually avoided during pregnancy RT has been administered, albeit rarely,

in the second and third trimesters when indicated,

M F Malik et al.

minimizing fetal exposure by abdominal lead shielding

A better way to minimize radiation exposure involves

ste-reotactic radiosurgery, providing focal radiation treatment

through equipment such as the Gamma Knife [6 12]

6 In cases where surgical resection can be postponed,

neu-rosurgery may occur as soon as the fetus is viable, in the

immediate postpartum period [1]

Surgery may, however, have been indicated when

pregnant women exhibit symptoms from mass effect in

the presence of a nonviable fetus or for aggressive

malig-nant tumors where the risk to the mother’s life precludes

delay in delivery of the nonviable fetus Surgery is

con-sidered safe after the first trimester [13]

Anesthetic Management

1 Anesthesia for neurosurgery

(a) Goals include avoidance of aortocaval compression,

careful fluid management to avoid cerebral edema,

and invasive hemodynamic monitoring to maintain

adequate uteroplacental pressure and cerebral

perfu-sion pressure (CPP) [1]

(b) A rapid sequence induction can be done to

mini-mize aspiration and to quickly control respiratory

parameters The effect of succinylcholine on

increasing ICP is controversial Rocuronium may

be used

(c) The cautious administration of corticosteroids and

anticonvulsants may be necessary

(d) The administration of mannitol, a Food and Drug

Administration category C drug, can lead to increased

fetal plasma osmolality, hypernatremia, and renal

dysfunction in the form of oliguria and subsequent

oligohydramnios Despite this, it has been used in

low dose without any adverse effects [14] Loop

diuretics may also be used

(e) Other intraoperative measures used to control raised

ICP include hyperventilation in an effort to decrease

CBF This should be used with caution in the

preg-nant patient whose arterial PCO2 is already low

(typi-cally ranging 28–33 mmHg), as uterine artery

vasoconstriction has been described with hypocapnia

[15] Hypocapnia also induces a leftward shift in the

oxygen-hemoglobin, inducing fetal acidosis An end-

tidal arterial PCO2 of 30–35 mmHg has been

recom-mended [16]

(f) Hypercarbia, hypoxia, obstruction of venous return,

Valsalva maneuvers, seizures, hypertension, and

hypotension, by inducing cerebral vasodilation, can

all increase ICP Hyperthermia and hyperglycemia

should also be avoided Cerebrospinal fluid (CSF)

drainage may be indicated

2 There is currently no consensus on the best mode of delivery for pregnant women with mass lesions Plans for delivery should be individualized, depending on the size and location of the mass lesion A multidisciplinary col-laborative approach is highly recommended for safe delivery of the fetus and improved maternal morbidity and mortality There are reports of CD under both regional and general anesthesia as well as regional analgesia for labor [17, 18]

3 There is also no consensus on the optimal type of sia and anesthesia with practice based on case reports rather than randomized controlled studies

4 Labor analgesia

(a) In a normal pregnancy, CSF pressure is not changed, and ICP is 7–15 cmH2O However, during labor, CSF pressure has been reported to increase to

39 cmH2O with painful uterine contractions, ing to significantly raised ICP during normal labor [17, 19]

(b) For the pregnant patient with an intracranial mass lesion in labor, the second stage can lead to signifi-cantly higher levels of CSF pressure, as high as

71 cmH2O Instrumented delivery can also increase pressures to greater than 70 cmH2O [17]

This could result in cerebral herniation when the patient has increased ICP, particularly in the setting

of mass effect and obstructed CSF flow Therefore, avoidance of pushing in the second stage of labor with instrumental delivery in combination with a regional technique will facilitate a vaginal delivery in selected cases

(c) Prior to placement of a neuraxial block, it is tant to obtain both history and imaging in pregnant women with brain tumors to rule out the presence of increased ICP and to determine the location of the lesion Signs of increased ICP might include nausea, vomiting, headache, seizures, hemiparesis, altered consciousness, pupillary abnormalities, and papill-edema Findings on imaging include tense dura, flat-tened gyri, narrowed sulci, effaced cisterns, ventricular compression, dilated ventricles, and mid-line shift [18]

(d) Epidural placement for labor has generally been sidered to be contraindicated in patients with increased ICP Epidural labor analgesia always car-ries a risk of dural puncture on placement Inadvertent dural puncture in pregnant women with increased ICP has traditionally been thought to lead to a risk of cerebral herniation [20] CSF loss could increase the gradient between supratentorial and infratentorial components leading to herniation This complication may be mitigated but not eradicated with the use of small-gauge spinal needles [21]

(e) In addition, boluses into the epidural space could also

increase ICP as has been demonstrated in a porcine

model This is likely due to thecal sac compression,

decreased subarachnoid space compliance, and

movement of CSF caudally Epidural infusions may

also increase ICP by decreasing the compliance of

the subarachnoid space

(f) However, without evidence of increased ICP or

obstruction to CSF flow, the patient carries minimal

to no risk of herniation [18] In the absence of CSF

obstruction, epidural placement can be offered It

may be prudent, however, to inject small volumes

into the epidural space

5 Cesarean delivery [17, 18, 22]

Elective CD is preferred by many providers to avoid the

high ICP levels seen with labor Anesthesia for CD is

controversial

(a) There have been reports of the successful use of spinal

anesthesia for CD [17, 18, 23] If the patient has no

obstructive hydrocephalus, spinal should be

consid-ered An arterial line may be indicated to maintain

CPP

(b) If general endotracheal anesthesia is required for a

patient with increased ICP, attempts should be made

to decrease the response to laryngoscopy and

extuba-tion that could aggravate increased ICP

• Opioids and lidocaine may attenuate this response

Succinylcholine causes a transient raised ICP but

provides excellent intubating conditions;

rocuronium may be a better alternative Propofol is

good induction agent that decreases ICP and at

2 mg/kg likely has little effect on CPP

• Maintenance of anesthesia commonly involves a

combined technique with volatile anesthetics and

opioids Nitrous oxide should be avoided given the

propensity to increase ICP, as a cerebral

vasodila-tor, and also contributes to postoperative nausea

and vomiting, which increases ICP Total

intrave-nous anesthesia with propofol and remifentanil

may decrease ICP and maintain CPP also

provid-ing antiemetic and antiseizure effects on

parturients

• The goals for patients with increased ICP are, as

stated above, to maintain CPP, avoid additional

edema, and prevent increased ICP This can be

accomplished by avoiding hypotension,

hyperten-sion, hypercarbia, hypocarbia, increased

intratho-racic pressure, and positive end- expiratory pressure

(PEEP) and maintaining normovolemia

– PaCO2 should be maintained above 25 mmHg,

avoiding hypocapnia, which can decrease CBF

and cause uterine artery vasoconstriction as

well as a left shift in the maternal bin dissociation curve

oxyhemoglo-– Tidal volumes should be minimized as thoracic pressure and PEEP lead to increased ICP

intra-– Normovolemia should be maintained with tonic, isonatremic solutions in an effort not to aggravate cerebral edema Mannitol in low doses has been used but is generally delayed until after delivery if possible Similarly hyper-tonic saline, albumin, and glucose-containing fluids are usually avoided

iso-– Antiemetics and analgesia are important in postoperative management Of note, metoclo-pramide may increase ICP though this needs to

be substantiated Good pain relief through a transverse abdominis plane block and ketorolac

as adjuncts to opioids is advised

References

1 Stevenson CB, Thompson RC The clinical management of intracranial neoplasms in pregnancy Clin Obstet Gynecol 2005;48(1):24–37.

2 Swensen R, Kirsch W Brain neoplasms in women: a review Clin Obstet Gynecol 2002;45(3):904–27.

3 Ravindra VM, Braca JA, Jensen RL, Duckworth EAM, Duckworth EAM Management of intracranial pathology during pregnancy: case example and review of management strategies Surg Neurol Int 2015;6:43.

4 Bonfield CM, Engh JA Pregnancy and brain tumors Neurol Clin 2012;30(3):937–46.

5 Pallud J, Mandonnet E, Deroulers C, Fontaine D, Badoual M, Capelle

L, et al Pregnancy increases the growth rates of World Health Organization grade II gliomas Ann Neurol 2010;67(3):398–404.

6 Zwinkels H, Dörr J, Kloet F, Taphoorn MJB, Vecht CJ Pregnancy

in women with gliomas: a case-series and review of the literature J Neuro-Oncol 2013;155(2):293–01.

7 Han Z, Du Y, Qi H, Yin W Post-traumatic malignant glioma in a pregnant woman: case report and review of the literature Neurol Med Chir 2013;53:630–4.

8 Simon R Brain tumors in pregnancy Semin Neurol 1988;8(3):214–21.

9 Carroll RS, Zhang J, Black PM Expression of estrogen tors alpha and beta in human meningiomas J Neuro-Oncol 1999;42(2):109–16.

recep-10 Yust-Katz S, De Groot JF, Liu D, Wu J, Yuan Y, Anderson

MD, et al Pregnancy and glial brain tumors Neuro-Oncology 2014;16(9):1289–94.

11 Soper JT, Spillman M, Sampson JH, Kirkpatrick JP, Wolf JK, Clarke-Pearson DL High-risk gestational trophoblastic neo- plasia with brain metastases: individualized multidisciplinary therapy in the management of four patients Gynecol Oncol 2007;104(3):691–4.

12 Lynch JC, Gouvêa F, Emmerich JC, Kokinovrachos G, Pereira C, Welling L, et al Management strategy for brain tumour diagnosed during pregnancy Br J Neurosurg 2011;25(2):225–30.

13 Verheecke M, Halaska MJ, Lok CA, Ottevanger PB, Fruscio R, Dahl-Steffensen K, et al Primary brain tumours, meningiomas and

M F Malik et al.

brain metastases in pregnancy: report on 27 cases and review of

literature Eur J Cancer 2014;50(8):1462–71.

14 Wang LP, Paech MJ Neuroanesthesia for the pregnant woman

Anesth Analg 2008;107(1):193–200.

15 Carlin A, Alfirevic Z Physiological changes of pregnancy and

mon-itoring Best Pract Res Clin Obstet Gynaecol 2008;22(5):801–23.

16 Chung J-H, Rho J-H, Jung T-H, Cha S-C, Jung H-K, Lee C, et al

Anesthetic management of a parturient for combined cesarean

section and surgical removal of pituitary tumor - a case report

Korean J Anesthesiol 2012;62(6):579–83.

17 Anson JA, Vaida S, Giampetro DM, McQuillan PM Anesthetic

management of labor and delivery in patients with elevated

intra-cranial pressure Int J Obstet Anesth 2015;24(2):147–60.

18 Leffert LR, Schwamm LH Neuraxial anesthesia in parturients with

intracranial pathology Anesthesiology 2013;119(3):703–18.

19 Marx GF, Zemaitis MT, Orkin LR Cerebrospinal fluid sures during labor and obstetrical anesthesia Anesthesiology 1961;22:348–54.

pres-20 Su T-M, Lan C-M, Yang L-C, Lee T-C, Wang K-W, Hung K-S Brain tumor presenting with fatal herniation following deliv- ery under epidural anesthesia Anesthesiology 2002;96(2):508–9.

21 van Crevel H, Hijdra A, de Gans J Lumbar puncture and the risk of herniation: when should we first perform CT? J Neurol 2002;249(2):129–37.

22 El-Refai NA-R Anesthetic management for parturients with logical disorders Anesth Essays Res 2013;7(2):147.

23 Atanassoff PG, Alon E, Weiss BM, Lauper U Spinal anaesthesia for Caesarean section in a patient with brain neoplasma Can J Anaesth 1994;41(2):163–4.

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_93

Jehovah’s Witnesses

Shobana Chandrasekhar

Background

Definition

Jehovah’s witnesses are known among health-care providers

for their refusal of blood or blood components based on their

faith This may include not only blood but also other

compo-nents such as plasma, red blood cells, and platelets This is

based on the belief that blood is holy and this sacred product

cannot be taken in any form

Mortality, Informed Consent, and Advanced

Directives

1 Obstetric hemorrhage is a leading cause of maternal

mor-tality, and pregnant Jehovah’s witnesses have a 44-fold

increased risk of mortality if they bleed in the peripartum

period [1] The physicians involved in the care of these

patients need to be aware of medical and ethical issues

involved

2 Informed consent ensures that maternal wishes are

dis-cussed and executed during the birthing event and ensures

active involvement of the patient in her medical

treat-ment In a pregnant patient, maternal-fetal conflict of

interest, time limitations especially in urgent situations,

and unexpected changes of labor course can complicate

management for the team [2] This process becomes even

more challenging in a Jehovah’s witness patient who is

pregnant A pregnant patient usually aligns her interest

with that of the unborn child However, a patient who is a

Jehovah’s witness may make choices about refusing

blood transfusion even in a life-threatening hemorrhage

Recent court rulings have allowed competent pregnant

women to refuse blood transfusion based on the patient’s self-determination and the right to die [3]

3 An advance directive is a formal document that dictates a patient’s explicit wishes regarding her health-care deci-sions A durable power of attorney is established for health care, and this is a person who is appointed as the patient’s surrogate decision-maker or health-care proxy This person will execute the patient’s wishes when the patient is incapacitated The wishes declared in the advance directive should be respected and carried out by the health-care providers However, if the health-care pro-vider perceives a moral or ethical conflict, then they should hand over care to another competent person if possible

Management Considerations

Medical Management

1 Jehovah’s witnesses usually carry advance health-care directives such as durable power of attorney or health- care proxy These advance directives help guide decision- making processes when a pregnant Jehovah’s witness is admitted

2 In addition, some hospitals have a separate consent form and discussions are held and patient’s wishes are clearly documented Acceptable alternative treatments must be discussed and documented Risks regarding withholding administration of lifesaving therapy should be discussed, and patients must be counseled about massive obstetric hemorrhage and its consequences Multidisciplinary management approach includes obstetrician, anesthesiol-ogist, blood bank team, neonatologists, and nurses

3 Preoperative pharmacologic therapies such as iron and folate supplementation are used High-dose recombinant erythropoietin to increase hemoglobin synthesis has been shown to increase hemoglobin levels and avoid blood transfusions [4 5]

S Chandrasekhar, M.D.

Department of Anesthesiology,

Baylor Medical Center, Houston, TX, USA

93

4 Studies indicate that erythropoietin has been used during

pregnancy with no reported fetal or maternal

complica-tions due to its administration in pregnancy The

hyper-coagulable state of pregnancy may predispose to

thrombosis when erythropoietin-stimulating agents are

administered [6]

Surgical Management

1 These are primarily techniques that minimize blood loss

during surgery Skilled and experienced surgeons should

perform the operation and use electrocautery, argon beam,

topical hemostatic agents, and ligasure vessel sealing as

needed

2 Uterine tamponade and special techniques for suturing

should be employed if needed at the discretion of the

most experienced surgeon

3 Prophylactic uterine artery embolization should be

dis-cussed with interventional radiology

4 Finally, quick decisions regarding cesarean hysterectomy

have to be made early to prevent massive blood loss

Anesthetic Management

1 Anesthetic management includes discussing and clearly

understanding the patient’s wishes regarding refusal of

blood and blood products even under life-threatening

circumstances Advance directives with documentation

of the patient’s wishes should be upheld, and no one

including family members can change the documented

wishes of the patient if she becomes incapacitated

2 Some patients may accept blood derivatives such as

platelets, plasma, or albumin, and this should be clearly

discussed preoperatively

3 Acceptable alternative therapy such as antifibrinolytic

agents and topical hemostatic agents is available and

discussed with the operative team

4 Anesthetic technique should be geared to maximizing

oxygen delivery and decreasing oxygen consumption

Deliberate hypotension to minimize blood loss may not

be used in a pregnant patient due to compromise of

uteroplacental circulation

5 In those patients requiring an epidural blood patch for

post-dural-puncture headache, a closed circuit has to be

maintained A continuous line from the epidural needle

to the intravenous line and a stopcock for blood

aspira-tion and injecaspira-tion have been described to meet the

wishes of Jehovah’s witnesses that the drawn blood is

still part of their circulatory system

6 Blood conservation strategies such as cell salvage or

preoperative autologous normovolemic hemodilution

should be offered to patients as some may accept this intervention, as the withdrawn blood is in a continuous cycle and is in contact with their circulatory system

7 In normovolemic hemodilution whole blood is drawn from the patient before blood shedding preopera-tively, and concurrently, the removed volume is replaced with crystalloids or colloids to maintain euvolemia [7] This blood is maintained in a continuous system in the collected bag and is administered back to the patient after surgical blood loss Candidates for this technique should have preoperative hemoglobin in the range of

with-12 g/dl with hemorrhage greater than 50% of the lating blood volume anticipated intraoperatively One of the advantages is that the removed whole blood has more red blood cells per ml, and reinfusing it will pro-vide whole blood consisting of red blood cells, platelets, and clotting factors In addition, the decreased blood viscosity due to hemodilution improves microcircula-tory flow and cardiac output This technique however has limited benefits in pregnant patients as the dilutional anemia of pregnancy itself limits the volume of whole blood that can be removed preoperatively

8 Cell saver devices collect the blood intraoperatively, wash, and filter the autologous red cells, which are then transfused back to the patient It has been shown to improve postoperative hemoglobin and reduce the need for blood transfusions [8, 9] This is useful during mas-sive obstetric hemorrhage and has been described in man-agement of Jehovah’s witnesses However, the disadvantages of cell salvage during a cesarean delivery include the coordination of a transfusionist to set up the cell saver device, the risk of amniotic fluid embolism, and risk of maternal alloimmunization if there is incompati-bility between maternal and fetal red blood cell antigens

9 Antifibrinolytic agents such as tranexamic acid and e-aminocaproic acid are other options in reducing blood loss [10] Recombinant factor VIIa and prothrombin complex concentrate are commercially available but expensive

10 Desmopressin, a synthetic analog of vasopressin that releases von Willebrand factor from endothelial cells, has been used in Jehovah’s witnesses with coagulopathy

In addition many Jehovah’s witnesses accept albumin or volume expansion

References

1 Singla AK, Lapinski RH, Berkowitz RL, Saphier CJ Are women who are Jehovah’s witness at risk of maternal death? Am J Obstet Gynecol 2001;185(4):893–5.

2 Broaddus B, Chandrasekhar S Informed consent in obstetric thesia Anesth Analg 2011;112(4):912–5.

3 Oddi AS The tort of interference with the right to die: the wrongful

living cause of action Georgetown Law J 1986;75:625.

4 Beholz S, Liu J, Thoelke R, Spiess C, Konertz W Use of

desmo-pressin and erythropoietin in an anemic Jehovah’s witness patient

with severely impaired coagulation capacity undergoing stentless

aortic valve replacement Perfusion 2001;16:485–9.

5 Belfort M, Kofford S, Varner M Massive obstetric hemorrhage in a

Jehovah’s witness: intraoperative strategies and high dose

erythro-poietin use Am J Perinatol 2011;28:207–10.

6 Sienas L, Wong T, Collins R, Smith J Contemporary uses of

eryth-ropoietin in pregnancy: a literature review Obstet Gynecol Surv

2013;68:594–602.

7 Nagy CJ, Wheler AS, Archer TL Acute normovolemic

hemodi-lution, intraoperative cell salvage, and Pulse CO hemodynamic

monitoring in a Jehovah’s witness with placenta accrete Int J Obstet Anesth 2008;17:159–63.

8 Liumbruno GM, Meschini A, Liumbruno C, Rafanelli D The duction of intra-operative cell salvage in obstetric clinical practice:

intro-a review of the intro-avintro-ailintro-able evidence Eur J Obstet Gynecol Reprod Biol 2011;159:19–25.

9 Ralph CJ, Sullivan I, Faulds J Intraoperative cell salvaged blood as part of a blood conservation strategy in cesarean section: is fetal red cell contamination important? Br J Anaesth 2011;107:404–8.

10 Shahid A, Khan A Tranexamic acid in decreasing blood loss during and after cesarean section J Coll Physicians Surg Pak 2013;23:459–62.

93 Jehovah’s Witnesses

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_94

Paul D Weyker and Christopher Allen-John Webb

Background

Definition

Kawasaki disease (KD) is a rare, self-limiting generalized

vasculitis that primarily affects the coronary arteries While

the exact etiology is unknown, it is considered to be the most

common cause of coronary artery disease in young

children

Etiology

1 While the etiology of KD has yet to be fully elucidated,

the leading theories suggest two main categories as

poten-tial triggers: environment toxins and infections [1 20]

Interestingly, the common pathway for these KD triggers

appears to be the respiratory tract Given the seasonal

pat-terns of KD in Japan, Hawaii, and parts of the western

United States, many experts believe that tropospheric

winds from northeastern China are responsible for

spread-ing the KD trigger

(a) Once in the upper respiratory tract, the antigen

trig-gers an immune-mediated response (innate and

adap-tive) within the arterial walls

(b) There appears to be a predilection for coronary

arter-ies and other medium-sized muscular arterarter-ies with

histopathological studies demonstrating necrotizing

arteritis, vasculitis, and luminal myofibroblastic liferation as the three most common vasculopathic processes

(c) Depending on the vasculopathic process, the end result is arterial wall weakening leading to aneurysm formation or coronary artery stenosis and thus myo-cardial ischemia

2 In addition, it is commonly believed that there is a genetic susceptibility for developing KD

(a) This is supported by the fact that Japanese children, regardless of their geographical location, continue to have an increased risk for developing KD

(b) Based on the available literature, many experts believe that susceptibility to KD is polygenic and var-ies among different ethnic groups

(c) Epidemiological studies have shown that siblings

of children with KD are ten times more likely to develop KD when compared to the general population Additionally, children of parents with

a history of KD are twice as likely to also develop KD

Signs

1 Signs of acute infection include fever of 5 days duration, bilateral conjunctivitis, lip and oral mucosal alterations (strawberry tongue, oropharyngeal edema, red fissured lips), polymorphous rash, cervical lymphadenopathy, and

P D Weyker, M.D ( * )

Department of Anesthesiology, Kaiser Permanente South

San Francisco, South San Francisco, CA, USA

e-mail: paul.d.weyker@kp.org

C A.-J Webb, M.D

Regional Anesthesia and Acute Pain Medicine, Department of

Anesthesiology, Kaiser Permanente South San Francisco,

South San Francisco, CA, USA

e-mail: Christopher.A.Webb@KP.ORG

94

changes in the extremities such as erythema and edema

The acute phase lasts for 7–14 days followed by a

sub-acute one for 2–4 weeks, when there is desquamation of

the hands and feet and thrombocytosis with a risk for

coronary artery thrombosis When all criteria are not met

but there is a high clinical suspicion for KD, imaging can

be helpful in the diagnosis Echocardiography and

coro-nary angiography may demonstrate corocoro-nary aneurysms,

stenosis, or myocardial ischemia

(a) The incidence of coronary artery aneurysm formation

varies with rates ranging from 15% to 25% being

reported in the literature

(b) Long-term cohort studies show that 55% of patients

who develop coronary artery aneurysms will have

regression, with 90% showing regression within

2 years of KD resolution

2 Sudden death, coronary artery aneurysms, cardiac

isch-emia, myocardial fibrosis, valvular abnormalities, and

heart failure may develop from coronary arteritis

Patients with large aneurysms may develop thrombosis

and ischemia Patients may require stenting, CABG, or

transplant Patients with a history of KD, and these

potential complications, are most commonly seen during

pregnancy

Interaction with Pregnancy

1 Studies analyzing the interaction of KD with pregnancy

are limited and mainly involve case reports and small case

studies However, the complications of over 80 deliveries

in women with a history of KD have been reported

2 Based on the limited data, there may be an increased rate

of premature rupture of membrane and preterm labor

though most women deliver at term There were rare

reports of postpartum hemorrhage

3 Although most mothers do well, there are many reports of

cardiac decompensation during pregnancy

4 The hemodynamic changes during pregnancy may place

these women at increased risk for myocardial ischemia

during pregnancy as increased heart rate, cardiac output,

and contractility all increase myocardial oxygen

demand Additionally, these hemodynamic changes also

increase arterial wall stress, which can lead to

progres-sion of known aneurysms and potentially aneurysm

rupture

Testing

Case reports have documented the management of

parturi-ents presenting with acute KD and parturiparturi-ents with a known

history of KD

1 Parturients with acute KD should have echocardiography

to determine wall motion abnormalities, valvulopathies, and aortic root measurements

(a) Parturients with acute KD and normal cardiac tion can undergo expectant management with sys-temic therapy for KD involving the use of intravenous immunoglobulin (IVIG)

(b) Patients with abnormal stress echocardiogram or onary artery dilation should have further workup with magnetic resonance angiography (MRA)

2 Women with a history of KD with cardiovascular ment will need stress echocardiography and computed tomography angiography (CTA) prior to pregnancy or MRA after conception to assess the coronary arteries

3 Women with a known history of KD and an unknown diac status should have baseline echocardiography (a) Prior to conception, computed tomography (CT) cal-cium scores should be obtained If the latter is abnor-mal, then either a CTA or MRA is indicated

(b) If the patient presents after conception, then a stress echocardiogram and MRA should be performed (c) The need for repeat echocardiograms is dictated based on symptoms or signs of worsening heart fail-ure and/or ischemia

Management Medical

1 Management of patients with active KD involves ing inflammation and mitigating the development of thrombus and aneurysm formation

2 Coordinated care between maternal fetal medicine, ology, and obstetric anesthesia is imperative

3 Once the diagnosis of KD is confirmed, treatment with IVIG and aspirin is indicated

4 If patients are treated with IVIG within the first 10 days of fever onset, the risk of aneurysm formation can be decreased from 25% to 5% For patients with known aneu-rysms in whom long-term antiplatelet therapy is indicated, care must be coordinated with maternal fetal medicine

5 Pregnancy termination should be discussed with patients with severe heart failure or significant ischemia who might benefit from percutaneous coronary interventions

or cardiac surgery

Anesthetic Management

1 Anesthetic management of these patients will vary depending on their preoperative antiplatelet regimen (enoxaparin) and their cardiovascular function/status

P D Weyker and C A.-J Webb

2 The mode of delivery should be dictated by obstetrical

indications

3 When not contraindicated by anticoagulation, neuraxial

analgesia and anesthesia may be performed In fact, labor

epidural analgesia will be beneficial to the pregnant

woman with myocardial ischemia, aneurysm, and heart

failure Epidural analgesia will decrease maternal

tachy-cardia, hypertension, and oxygen consumption Assisted

second stage is often recommended, and for these patients,

epidural analgesia is beneficial

4 Similarly, a low-dose combined spinal-epidural or

epi-dural could be used for surgical anesthesia Depending on

the severity of cardiac involvement, a spinal may not be

the best choice if hypotension and tachycardia could be

detrimental

5 In addition to standard ASA monitors, the need for

inva-sive monitoring (arterial line, central venous pressure,

TEE) is indicated on a case-by-case basis Arrhythmias

may occur

6 The use of intravenous beta-blockers, calcium channel

blockers, and nitrates is indicated to closely control blood

pressure in patients with known coronary artery

aneurysms

7 Similar to nonpregnant patients at risk for myocardial

ischemia, the goals are to maximize myocardial oxygen

supply and minimize tachycardia and other determinants

of myocardial oxygen demand

References

1 Council on Cardiovascular Disease in the Young, Committee on

Rheumatic Fever, Endocarditis, and Kawasaki Disease, American

Heart Association Diagnostic guidelines for Kawasaki disease

Circulation 2001;103(2):335–6.

2 Daniels LB, Gordon JB, Burns JC Kawasaki disease: late

cardio-vascular sequelae Curr Opin Cardiol 2012;27(6):572–7.

3 Dean AG, Melish ME, Hicks R, Palumbo NE An epidemic of

Kawasaki syndrome in Hawaii J Pediatr 1982;100(4):552–7.

4 Fujita Y, Nakamura Y, Sakata K, Hara N, Kobayashi M, Nagai M,

et al Kawasaki disease in families Pediatrics 1989;84(4):666–9.

5 Gordon CT, Jimenez-Fernandez S, Daniels LB, Kahn AM, Tarsa M, Matsubara T, et al Pregnancy in women with a history of Kawasaki disease: management and outcomes BJOG 2014;121(11):1431–8.

6 Kanno K, Sakai H, Nakajima M, Satake A, Konishi T, Iizuka H An adult case of Kawasaki disease in a pregnant Japanese woman: a case report Case Rep Dermatol 2011;3(1):98–102.

7 Kato H, Sugimura T, Akagi T, Sato N, Hashino K, Maeno

Y, et al Long-term consequences of Kawasaki disease A 10- to 21-year follow-up study of 594 patients Circulation 1996;94(6):1379–85.

8 Lefkou E, Mahadeva U, Jones A, Hancock J, Hunt BJ Kawasaki syndrome during pregnancy: a case report and literature review Obstet Med 2008;1(1):24–8.

9 Newburger JW, Takahashi M, Burns JC Kawasaki disease J Am Coll Cardiol 2016;67:1738–49.

10 Orenstein JM, Shulman ST, Fox LM, Baker SC, Takahashi M, Bhatti TR, et al Three linked vasculopathic processes character- ize kawasaki disease: a light and transmission electron microscopic study PLoS One 2012;7(6):e38998.

11 Rodo X, Curcoll R, Robinson M, Ballester J, Burns JC, Cayan DR,

et al Tropospheric winds from northeastern China carry the logic agent of Kawasaki disease from its source to Japan Proc Natl Acad Sci U S A 2014;111(22):7952–7.

12 Rowley AH Kawasaki disease: novel insights into etiology and genetic susceptibility Annu Rev Med 2011;62(1):69–77.

13 Samada K, Shiraishi H, Sato A, Momoi MY Grown-up Kawasaki disease patients who have giant coronary aneurysms World J Pediatr 2010;6(1):38–42.

14 Senzaki H Long-term outcome of Kawasaki disease Circulation 2008;118(25):2763–72.

15 Uehara R, Yashiro M, Nakamura Y, Yanagawa H Kawasaki disease

in parents and children Acta Paediatr 2003;92(6):694–7.

16 Taniguchi K, Ono H, Sato A, Kinomoto S, Tagawa N, Umehara

N, et al Strict management of a pregnant patient with giant onary artery aneurysm due to Kawasaki disease Pediatr Int 2015;57(5):990–2.

cor-17 To L, Krazit ST, Kaye AD Perioperative considerations of Kawasaki disease Ochsner J 2013;13(2):208–13.

18 Tsuda E, Kawamata K, Neki R, Echigo S, Chiba Y Nationwide survey of pregnancy and delivery in patients with coronary arte- rial lesions caused by Kawasaki disease in Japan Cardiol Young 2006;16(2):173.

19 Tsuda E Pregnancy and delivery in patients with coronary artery lesions caused by Kawasaki disease Heart 2005;91(11):1481–2.

20 Youssef A, Ben Amara F, M’barki M, Reziga H, Magherbi H, Neji

K Successful pregnancy and delivery in patient with artery bypass grafting for coronary lesion caused by Kawasaki disease Tunis Med 2013;91(3):217–8.

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_95

Klippel-Trenaunay Syndrome

Adam M Gerber and Suzanne K W Mankowitz

Presentation and Symptoms

Definition

Klippel-Trénaunay syndrome (KTS, formerly known as

Klippel-Trénaunay-Weber Syndrome) is a rare and complex

congenital vascular disorder affecting multiple organ

sys-tems Although the etiology remains unclear, the syndrome

is characterized by hypertrophy and/or atrophy of the

extremities and vascular malformations throughout the body

The disease is extremely rare and as such has a low incidence

in pregnant patients

Symptoms

1 Patients with KTS, almost without exception, present

with nevus flammeus (port wine stain) on one or more

extremities Most commonly these are located on the legs

followed by the arms and trunk and, rarely, on the head

and neck Several atypical KTS variants may present

without nevus flammeus; however, these are far less

com-mon [1 2] Other features of capillary involvement

include hemangiomas throughout the body [3]

2 Soft and bony tissue hypertrophy must be present for the

diagnosis of KTS Most often seen in the legs, it typically

results in local limb gigantism and is associated with

sen-sations of heaviness, pain, and poor mobility of the

affected limb Extremities displaying gigantism are often

prone to skin breakdown, ulceration, and cellulitis [4]

3 Venous and lymph malformations are another major feature of KTS Issues with lymph drainage and vessel malformation may result in lymphedema further increas-ing the risk of infection Venous malformations are seen

as varicosities near the skin on the affected limb(s) Importantly, these are also seen in the deep veins of the limbs, spine, and internal organs including the uterus and the vagina

4 Less commonly KTS may be associated with anatomic abnormalities Duplication or deletion of venous structures, especially those of the affected limb, may be seen In some cases, syndactyly and polydactyly have been observed [4]

5 Due to the presence of venous abnormalities, especially those of the deep veins of the limbs, KTS patients are at higher risk for thrombotic events and subsequent pulmo-nary embolism Following major trauma, surgery, and/or hemorrhage, these patients are also at high risk for coagulopathy

6 These patients may have Kasabach-Merritt coagulopathy,

a consumptive coagulopathy caused by cellular tion and platelet trapping Approximately 45% may suffer from this coagulopathy [5]

Incidence

Globally 1 in 100,000 people is thought to suffer from KTS [4]

Interaction with Pregnancy

1 Given the low prevalence of KTS, it is not commonly observed in parturients, and only case reports guide man-agement [6]

2 Although KTS patients have successfully delivered healthy infants, the unique anatomic and physiologic changes associated with the disease require special consideration

A M Gerber, M.D., Ph.D.

Department of Anesthesiology, Columbia University Medical

Center, New York, NY, USA

e-mail: amg2291@cumc.columbia.edu

S K W Mankowitz, M.D ( * )

Associate Professor, Department of Anesthesiology, Columbia

University Medical Center, New York, NY, USA

e-mail: sw397@cumc.columbia.edu

95

(a) Thromboembolic risk The hypercoagulable state

during pregnancy is further exacerbated by the

pres-ence of multiple deep venous abnormalities in KTS

patients Pulmonary and right ventricular thrombi

have been reported during pregnancy [7 8]

(b) Diffuse intravascular coagulation (DIC) Pregnant

patients with KTS also appear to be at a higher risk of

coagulation abnormalities, specifically DIC The

eti-ology underlying the increased risk is unclear;

how-ever it appears to be related to the vascular anomalies

themselves [7 9]

(c) Vascular anomalies KTS patients often have multiple

vascular anomalies throughout their body, not just in

the affected limb Venous and arterial-venous

malfor-mations (AVMs) and angiomas have been reported on

the abdominal viscera, including the uterus,

abdomi-nal wall, as well as within the vagiabdomi-nal caabdomi-nal This

dra-matically raises the risk of major hemorrhage during

vaginal or surgical delivery of the fetus [10, 11]

(d) The immunocompromised state of pregnancy may

aggravate the predisposition to infection from ulcers

and lymphedema

(e) The fetus typically develops normally in patients with

KTS, and full-term healthy infants have been delivered

However, pregnancies should be carefully monitored

Testing

1 Given the rarity of the disease, there is no consensus on any

particular testing regime during pregnancy However,

based on the pathophysiology of KTS some logical

recom-mendations can be made; coagulation parameters should

be monitored in conjunction with diagnostic imaging to

determine the location of any critical vascular anomalies

2 Serial ultrasonography and prenatal testing are also

advised to ensure fetal health [12]

Management

Medical Management

1 Paturients with KTS may require compression garments and

anticoagulation therapy to prevent thromboembolic events

2 However, there is no general consensus on medical

manage-ment, and decisions are made on a case-by-case basis [710]

Anesthetic Management

1 The presence of vascular anomalies in the vaginal canal,

uterus, or abdominal wall has the potential to result in

severe maternal hemorrhage either during vaginal or cesarean delivery As such, diagnostic imaging is advised

to determine the locations of these malformations and hence the bleeding risk prior to delivery [9 11, 13]

2 Given the high risk of hemorrhage, close monitoring of blood counts, particularly platelets, prothrombin time, par-tial thromboplastin time, and fibrinogen levels, is advised Also ensuring the immediate availability of appropriate blood products should be of high priority If the patient has significant coagulation abnormalities at baseline, a hema-tology consult may be warranted Good intravenous access

is essential, and an arterial line may be useful [3, 9, 10, 13]

3 Neuraxial anesthesia for these patients may also present significant risk [3 5 9 14]

(a) Malformations have been observed in the spinal cord and in the epidural and subdural spaces Imaging of the spine is strongly recommended to prevent inadvertently compromising one of these structures After normal imaging epidurals, spinal and combined spinal- epidurals have been used in pregnant women with KTS (b) Also given the risk of coagulopathy, close monitoring

of platelets and coagulation status is advised both prior to placement and before removal of epidural catheters [4 9 10, 13]

4 General anesthesia must be undertaken with extreme tion as soft tissue hypertrophy and hemangiomas can make airway management challenging [5]

cau-References

1 Jacob AG, Driscoll DJ, Shaughnessy WJ, Stanson AW, Clay RP, Gloviczki P Klippel-Trénaunay syndrome: spectrum and manage- ment Mayo Clin Proc 1998;73(1):28–36.

2 Ziyeh S, Spreer J, Rössler J, Strecker R, Hochmuth A, Schumacher

M, et al Parkes Weber or Klippel-Trenaunay syndrome? Non- invasive diagnosis with MR projection angiography Eur Radiol 2004;14(11):2025–9.

3 Dwivedi D, Sheshadri K, Tandon U, Chakraborty S Klippel- Trenaunay syndrome: a rare entity with anesthesia concerns J Clin Anesth 2016;35:233–4.

4 Cohen MM Klippel-Trenaunay syndrome Am J Med Genet 2000;93(3):171–5.

5 Holak EJ, Pagel PS Successful use of spinal anesthesia in a patient with severe Klippel-Trenaunay syndrome associated with upper airway abnormalities and chronic Kasabach-Merritt coagulopathy

J Anesth 2010;24(1):134–8.

6 Rebarber A, Roman AS, Roshan D, Blei F Obstetric management

of Klippel-Trenaunay syndrome Obstet Gynecol 2004;104(5 Pt 2):1205–8.

7 Gianlupi A, Harper RW, Dwyre DM, Marelich GP Recurrent monary embolism associated with Klippel-Trenaunay-Weber syn- drome Chest 1999;115(4):1199–201.

8 Yamada T, Ohba T, Yamamoto T, Kimata N, Inami T, Munakata R,

et al A 17-year-old girl with Klippel-Weber syndrome complicated with a pulmonary thromboembolism and RV thrombus Intern Med 2013;52(12):1337–40.

9 Neubert AG, Golden MA, Rose NC Kasabach-Merritt

coagulopa-thy complicating Klippel-Trenaunay-Weber syndrome in

preg-nancy Obstet Gynecol 1995;85(5 Pt 2):831–3.

10 Sivaprakasam MJ, Dolak JA Anesthetic and obstetric

consider-ations in a parturient with Klippel-Trenaunay syndrome Can J

Anaesth 2006;53(5):487–91.

11 Yara N, Masamoto H, Iraha Y, Wakayama A, Chinen Y, Nitta H,

et al Diffuse venous malformation of the uterus in a pregnant

woman with Klippel-Trénaunay syndrome diagnosed by DCE-

MRI Case Rep Obstet Gynecol 2016;2016:1–5.

12 Chen C-P, Lin S-P, Chang T-Y, Lee H-C, Hung H-Y, Lin H-Y, et al Prenatal sonographic findings of Klippel-Trénaunay-Weber syn- drome J Clin Ultrasound 2007;35(7):409–12.

13 Hergesell K, Kröger K, Petruschkat S, Santosa F, Herborn C, Rudofsky G Klippel-Trenaunay syndrome and pregnancy Int Angiol 2003;22(2):194–8.

14 Barbara DW, Wilson JL Anesthesia for surgery related to klippel- trenaunay syndrome: a review of 136 anesthetics Anesth Analg 2011;113:98–102.

95 Klippel-Trenaunay Syndrome

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_96

Ami Attali, Ashley Taylor, and Michael Isley

Background

End-Stage Heart Failure

Pregnancy in patients with pre-existing cardiac disease has

presented challenges to practitioners for many years Medical

management of cardiac disease has advanced to such a

degree that many patients with congenital cardiac disease

have survived to adulthood In the Western world, an

increas-ing incidence of acquired cardiac disease in young patients

of childbearing age also contributes to this challenging

patient load [1] In the past, cardiomyopathy has conferred a

relative risk of 17 with up to 1% mortality during pregnancy

In the United States today, cardiac disease has increased

sig-nificantly and is now the third leading cause of maternal

mortality, contributing to 15% of maternal deaths [2]

Currently in the United States, 0.41 per 100 deliveries are

complicated by cardiac disease [3] The use of mechanical

circulatory support for fulminant heart failure is an evolving

field, and its use in pregnancy is an even more rare

occur-rence with unknown side effects and yet to be established

guidelines Recent advances in the field have demonstrated

that multiple forms of mechanical support have been able to

sustain pregnancy [4]

Mechanical Support for Cardiac Function

Mechanical circulatory support has become a more frequent management modality for both acute and chronic heart fail-ure in the parturient Multiple cases of postpartum placement

of left ventricular assist devices (LVAD) and other forms of mechanical support have been described [5 6] This has most commonly been described in patients with peripartum cardiomyopathy where LVAD works well as a bridge to either transplant or recovery [5 7] However, pregnancy in a patient with a device already in place is relatively rare Many

of these women are informed about the increased risk nancy brings to their cardiac status [8] As more of these devices are implanted, more cases are likely

LVADs and Pregnancy

Cases and Counseling

1 There are only a few cases in the literature describing the management of parturients with LVADs

(a) One of the earliest cases describes a myocardial infarction in a parturient who had an unsuccessful coronary artery bypass surgery followed by extracor-poreal membrane oxygenation and LVAD placement though the pregnancy resulted in spontaneous abor-tion during the first trimester [9]

(b) Another case reported good maternal and fetal comes after a woman with an LVAD, which was placed after her first pregnancy for peripartum car-diomyopathy, presented at 33 weeks and 5 days of gestation She had been noncompliant with antico-agulation, beta-blocker, and angiotensin-converting enzyme inhibitor therapy The decision was made to proceed with cesarean delivery under general anes-thesia prior to the additional increased cardiac output that occurs during pregnancy This case was compli-cated only by postpartum hemorrhage requiring

out-A Attali, M.D., D.O ( * )

Department of Anesthesiology, Wayne State University (WSU)

School of Medicine, Henry Ford Hospital,

Detroit, MI, USA

e-mail: aattali1@hfhs.org

A Taylor, M.D.

Wayne State University (WSU) School of Medicine, Henry Ford

Hospital, Detroit, MI, USA

e-mail: ATAYLO11@hfhs.org

M Isley, M.D

Division of Obstetrical Anesthesiology, Wayne State University

(WSU) School of Medicine, Henry Ford Hospital,

Detroit, MI, USA

e-mail: misley1@hfhs.org

96

interruption of anticoagulation and transfusion as

well as neonatal intensive care admission for

continu-ous positive airway pressure [10]

(c) Another case describes a woman who became pregnant

6 months after placement of a continuous-flow LVAD

who also had good fetal and maternal outcome after a

vaginal delivery under epidural labor analgesia at 34

weeks and 4 days of gestation when she went into labor

During active labor, she did have worsening right

ven-tricular function, mitral regurgitation, and increased left

ventricular end-diastolic dysfunction that responded to

milrinone, nitroglycerin, and furosemide [11]

2 When women of childbearing years have an LVAD, they

are counseled not to become pregnant Management

out-side of pregnancy typically involves anticoagulation with

warfarin and antiplatelet agents, angiotensin-converting

enzymes, and beta-blockers With the exception of the

latter, these medications are contraindicated in early

preg-nancy and should be discontinued Anticoagulation is

essential to LVAD therapy and may be continued with

therapeutic low molecular weight heparin injection at

home or a carefully titrated heparin infusion in the

hospi-tal Patients must be informed of the unknown maternal

and fetal risks in parturients with LVADs There may be

inadequate hemodynamic support during the

develop-ment of the fetus Bleeding is a serious risk in parturients

who are anticoagulated and who also develop an acquired

von Willebrand syndrome from the LVAD The gravid

uterus could cause injury to the inflow or outflow

con-duits or the driveline of the LVAD Right ventricular

fail-ure is a major concern in these women with the increased

blood volume during pregnancy and particularly after

delivery [11, 12]

LVAD Management During Pregnancy

Cardiac output increases by 30–40% during pregnancy with

the maximum increase during pregnancy at approximately

24 weeks Cardiac output increases further during labor by

50% above pre-labor values and is greatest immediately

postpartum, to more than 100% of nonpregnant values This

is usually accomplished by an increase in stroke volume

with a modest contribution from heart rate In the patient

with an LVAD in place, the cardiac output increase that is

required as pregnancy progresses can be managed by

increasing the flows of the device Careful fluid

administra-tion minimizes the chance of right heart failure though this

is more of an issue during labor and the postpartum period

Continuous evaluation of cardiac function through

echocar-diography and physical exam may be helpful in adjusting

the LVAD speed Serial growth scans as well as additional

antenatal testing should be done to assess fetal growth and well-being

Delivery

Delivery is a time of immense fluid shifts and cardiovascular stress Patients with heart failure and LVADs require careful assessment during labor and delivery by a multidisciplinary team

1 These patients will unlikely deliver at full-term gestation LVAD flow may be at maximum limits at the end of the second trimester Preterm delivery is likely higher in this patient population as higher cardiac outputs are required for both maternal and fetal well-being

2 As described above, the mode of delivery may be by cesarean or vaginal A vaginal delivery may be preferable

in these patients to avoid the fluid shifts that occur during

a surgical one, which could necessitate inotropic support and lead to cardiac decompensation There appears to be less blood loss and hemodynamic change in pregnant women with cardiac disease who have a vaginal delivery compared to a surgical one Sometimes an assisted sec-ond stage is indicated [13] In addition, the driveline exit site of the LVAD in the abdomen could be damaged dur-ing a surgical delivery

3 Regardless of the route of delivery, an arterial line and pulmonary artery catheter should be considered to monitor the blood pressure and cardiac status through-out delivery Transesophageal echocardiography may also be useful Nonpulsatile flow may interfere with both pulse oximetry and noninvasive blood pressure monitoring There should also be a practitioner well versed in LVADs to manipulate parameters as needed [12, 14, 15]

4 Neuraxial analgesia and anesthesia could be performed However, placement must be arranged to occur after coagulation has been discontinued for the appropriate amount of time In addition patients with LVADs develop

an acquired von Willebrand syndrome This has been treated with cryoprecipitate prior to both epidural place-ment for labor and general anesthesia for cerclage [11,

12, 14]

5 Autotransfusion in the setting of right heart failure should

be anticipated and has been managed by decreasing load with furosemide, nitroglycerin, and removal of blood from the circulation Inotropic support and altering the flow on the LVAD device may be indicated [11]

6 Bleeding appears to be a common problem, and blood products should be available

A Attali et al.

Conclusion

Patients with severe heart failure and LVAD therapy are at

risk of fetal loss and maternal decompensation The safety

of LVADs during pregnancy has not yet been established

A reliable method of birth control is highly recommended

If these patients become pregnant, management at an

experienced institution with all the resources available to

optimize the outcome of these difficult cases is

recommended

References

1 Chen H-P, Sung W-C, Hui Y-L, Hui C-K Anesthetic management

of a repeat cesarean section in a parturient with severe peripartum

cardiomyopathy requiring ECMO in a previous pregnancy: a case

report Chang Gung Med J 2011;34(6 Suppl):28–33.

2 Huisman CM, Zwart JJ, Roos-Hesselink JW, Duvekot JJ, van

Roosmalen J Incidence and predictors of maternal cardiovascular

mortality and severe morbidity in the netherlands: a prospective

cohort study PLoS One 2013;8(2):e56494.

3 Autore C, Conte MR, Piccininno M, Bernabò P, Bonfiglio G, Bruzzi

P, et al Risk associated with pregnancy in hypertrophic

cardiomy-opathy J Am Coll Cardiol 2002;40(10):1864–9.

4 Agerstrand C, Abrams D, Biscotti M, Moroz L, Rosenzweig EB,

D’Alton M, et al Extracorporeal membrane oxygenation for

car-diopulmonary failure during pregnancy and postpartum Ann

Thorac Surg 2016;102(3):774–9.

5 Lueck S, Sindermann J, Martens S, Scherer M Mechanical

circula-tory support for patients with peripartum cardiomyopathy J Artif

7 Lund LH, Grinnemo K-H, Svenarud P, van der Linden J, Eriksson

MJ Myocardial recovery in peri-partum cardiomyopathy after continuous flow left ventricular assist device J Cardiothorac Surg 2011;6:150.

8 Kovacs AH, Harrison JL, Colman JM, Sermer M, Siu SC, Silversides CK Pregnancy and contraception in congeni- tal heart disease: what women are not told J Am Coll Cardiol 2008;52(7):577–8.

9 Etz C, Welp H, Scheld HH, Schmid C Near fatal infection of a patient with a left ventricular assist device due to unrecognized fetal death Eur J Cardiothorac Surg 2005;27(4):722–3.

10 LaRue S, Shanks A, Wang I, Ewald G, Anderson D, Joseph S Left ventricular assist device in pregnancy Obstet Gynecol 2011;118(2

Pt 2):426–8.

11 Sims DB, Vink J, Uriel N, Cleary KL, Smiley RM, Jorde UP, et al

A successful pregnancy during mechanical circulatory device port J Heart Lung Transplant 2011;30:1065–7.

12 Degnan M, Brodt J, Rodriguez-Blanco Y Perioperative ment of patients with left ventricular assist devices undergoing non- cardiac surgery Ann Card Anaesth 2016;19(4):676–86.

13 Thorne SA Pregnancy in heart disease Heart 2004;90(4):450–6.

14 Nelson EW, Heinke T, Finley A, Guldan GJ, Gaddy P, Matthew Toole J, et al Management of LVAD patients for noncardiac sur- gery: a single-institution study J Cardiothorac Vasc Anesth 2015;29(4):898–900.

15 Stone M, Hinchey J, Sattler C, Evans A Trends in the ment of patients with left ventricular assist devices presenting for noncardiac surgery: a 10-year institutional experience Semin Cardiothorac Vasc Anesth 2016;20(3):197–204.

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_97

Long QT Syndrome

Scott Mankowitz

Presentation

Definition

1 The Long QT Syndrome (LQTS) is a group of disorders

characterized by abnormal cardiac ion channels resulting

in a prolonged QT interval on ECG and leading to

arrhyth-mias, particularly torsades de pointes (TdP), syncope, and

sudden death See figure 97.1

2 There are several mechanisms to measure QT prolongation

The simplest is to measure the time from the beginning of

the QRS complex to the end of the T wave The lateral leads

are best for this purpose The problem with this method is

that it can yield misleading results when the heart rate is

particularly fast or particularly slow The Bazett formula is

the most commonly used version of the “corrected” QT

interval, also known as the QTc In an adult female, QTc

over 470 is considered abnormal [1] A more complicated

risk stratification formula assigning point values for various

factors such as QTc width, syncope, and family history was

proposed by Schwartz et al [2]

3 LQTS has a high mortality Untreated, 20% of LQTS

patients will die within the first year after a syncopal

event, and 50% will die within 10 years With treatment,

mortality drops to 3–4% [3]

4 The most feared complication of LQTS is a particular

form of polymorphic ventricular tachycardia called

tors-ades de pointes (Fr “twisting of the points”) In this

arrhythmia, the QRS axis changes from beat to beat

2 QT prolongation is associated with many conditions

(a) Inherited conditions such as Jervell and

Lange-Nielsen syndrome (JLN)—an autosomal recessive condition characterized by bilateral deafness—and

Romano-Ward (RW) syndrome, an autosomal nant condition without deafness

(b) Drugs—the list of medications which can prolong

QT interval is very large (See Box 97.1 below for common examples.)

• Antiarrhythmics such as quinidine, lidocaine, and procainamide

• Obstetrical drugs such as oxytocin and terbutaline

• Anesthetic drugs such as thiopental, droperidol, volatile anesthetics, succinylcholine, ketamine, glycopyrrolate, neostigmine, and metoclopramide

• Psychiatric drugs, such as haloperidol and clic antidepressants

(c) Medical conditions, such as intracranial hemorrhage (d) Cardiac diseases, such as ischemic heart disease, mitral valve prolapse, and cardiomyopathies

(e) Metabolic conditions such as hypothyroidism or hypothermia

S Mankowitz, M.D

Emergency Medical Associates, Parsippany, NJ, USA

Emergency Medicine Specialist, East Orange General Hospital,

East Orange, NJ, USA

e-mail: scott@mankowitz.org

97

Fig 97.1 LQTS Congenital long QT syndrome (Credit: lifeinthefastlane.com )

Fig 97.2 Torsades de pointes A particular type of polymorphic

ventricular tachycardia where the direction of the QRS complex

changes from beat to beat (Image public domain by Wikimedia

commons https://upload.wikimedia.org/wikipedia/commons/e/ed/ Torsades_converted_by_AICD_ECG_strip_Lead_II.JPG )

Table 97.1 LQT1–3 represent the majority of genotypes in the long

QT syndrome Each is linked to a specific gene which encodes for a

particular cardiac channelopathy Each is associated with particular

Sodium channel (INa)

Trigger Stress, exertion Loud noises Sleep

Notes Responsible for

consider pacing

RW Romano-Ward, JLN Jervell-Lange-Nielsen

Box 97.1 List of drugs contraindicated in LQTS (Adapted

from crediblemeds.org ; medications unavailable in the

USA have been removed) [ 5 ]

Generic name Brand names (Partial list)

Albuterol Proventil ® , Ventolin ® , Ventolin-HFA ® ,

Accuneb ® , Combivent ® , Vospire-ER ® , ProAir HFA ® , Duoneb ®

Alfuzosin Uroxatral ®

Amantadine Symmetrel ® , Symadine ®

Amiodarone Cordarone ® , Pacerone ® , Nexterone ®

Amitriptyline Elavil ® (Discontinued 6/13),

Tryptomer ® , Tryptizol ® , Laroxyl ® , Saroten ® , Sarotex ® , Lentizol ® , Endep ®

Generic name Brand names (Partial list) Amphetamine Adderall-XR ® , Dexedrine ® ,

Dextroamp ®

Anagrelide Agrylin ® , Xagrid ®

Apomorphine Apokyn ® , Ixense ® , Spontane ® ,

Uprima ®

Arformoterol Brovana ®

Aripiprazole Abilify ® , Aripiprex ®

Arsenic trioxide Trisenox ®

Artenimol + piperaquine

Buprenorphine Butrans ® , Belbuca ® , Bunavail ® ,

Buprenex ® , Suboxone ® , Zubsolv ®

Capecitabine Xeloda ®

Ceritinib Zykadia ®

Chloral hydrate Aquachloral ® , Novo-Chlorhydrate ® ,

Somnos ® , Noctec ® , Somnote ®

Chloroquine Aralen ®

Chlorpromazine Thorazine ® , Largactil ® , Megaphen ®

Cilostazol Pletal ®

Ciprofloxacin Cipro ® , Cipro-XR ® , Neofloxin ®

Citalopram Celexa ® , Cipramil ®

Clarithromycin Biaxin ® , Prevpac ®

Clomipramine Anafranil ®

Clozapine Clozaril ® , Fazaclo ® , Versacloz ®

Desipramine Pertofrane ® , Norpramine ®

Dexmedetomidine Precedex ® , Dexdor ® , Dexdomitor ®

Dexmethylphenidate Focalin ® , Focalin-XR ®

Dextroamphetamine Dexedrine ® , dexamphetamine,

dexamfetamine, (S)-(+)-amphetamine, Dextrostat ®

Diphenhydramine Benadryl ® , Nytol ® , Unisom ® ,

Sominex ® , Dimedrol ® , Daedalon ®

Doxepin Sinequan ® , Silenor ® , Aponal ® ,

Adapine ® , Doxal ® , Deptran ® , Sinquan ®

Dronedarone Multaq ®

Droperidol Inapsine ® , Droleptan ® , Dridol ® ,

Xomolix ®

Ephedrine Rynatuss ® , Broncholate ®

Epinephrine Primatene ® , Bronkaid ®

Eribulin mesylate Halaven ®

Erythromycin E.E.S ® , Robimycin ® , EMycin ® ,

Erymax ® , Ery-Tab ® , Eryc Ranbaxy ® , Erypar ® , Eryped ® , Erythrocin Stearate Filmtab ® , Erythrocot ® , E-Base ® , Erythroped ® , Ilosone ® , MY-E ® , Pediamycin ® , Zineryt ® , Abboticin ® , Abboticin-ES ® , Erycin ® , PCE Dispertab ® , Stiemycine ® , Acnasol ® , Tiloryth ®

Escitalopram Cipralex ® , Lexapro ® , Nexito ® ,

Anxiset-E ® (India), Exodus ® (Brazil), Esto ® (Israel), Seroplex ® , Elicea ® , Lexamil ® , Lexam ® , Entact ® (Greece), Losita ® (Bangladesh), Reposil ® (Chile), Animaxen ® (Colombia), Esitalo ®

(Australia), Lexamil ® (South Africa) Ezogabine Potiga ® , Trobalt ®

Famotidine Pepcid ® , Fluxid ® , Quamatel ®

Felbamate Felbatol ®

Fingolimod Gilenya ®

Flecainide Tambocor®, Almarytm®, Apocard®,

Ecrinal®, Flécaine®

Fluconazole Diflucan ® , Trican ®

Fluoxetine Prozac ® , Sarafem ® , Fontex ®

Formoterol Foradil ® , Foradile ® , Oxeze ® , Oxis ® ,

Atock ® , Atimos ® , Atimos Modulite ® , Perforomist ® , Dulera ® , Symbiocort ® , Vannair ® , Quikhale FB ®

Generic name Brand names (Partial list) Foscarnet Foscavir ®

Furosemide Lasix ® , Fusid ® , Frumex ®

Galantamine Reminyl ® , Nivalin ® , Razadyne-ER ®

Gemifloxacin Factive ®

Granisetron Kytril ® , Sancuso ® , Granisol ®

Grepafloxacin Raxar ®

Halofantrine Halfan ®

Haloperidol Haldol ® (US & UK), Aloperidin ® ,

Bioperidolo ® , Brotopon ® , Dozic ® , Duraperidol ® (Germany), Einalon S ® , Eukystol ® , Halosten ® , Keselan ® , Linton ® , Peluces ® , Serenace ® , Serenase ® , Sigaperidol ®

Hydrochlorothiazide Apo-Hydro ® , Aquazide H ® , BP Zide ® ,

Dichlotride ® , Hydrodiuril ® , HydroSaluric ® , Hydrochlorot ® , Microzide ® , Esidrex ® , Oretic ®

Hydrocodone - ER Hysingla™ ER, Zohydro ER Hydroxychloroquine Plaquenil ® , Quineprox ®

Hydroxyzine Atarax ® , Vistaril ® , Aterax ® , Alamon ® ,

Durrax ® , Equipose ® , Masmoran ® , Orgatrax ® , Paxistil ® , Quiess ® , Tran-Q ® , Tranquizine Ibutilide Corvert ®

Iloperidone Fanapt ® , Fanapta ® , Zomaril ®

Imipramine Tofranil ®

Indapamide Lozol ® , Natrilix ® , Insig ®

Isoproterenol Medihaler-Iso ® , Isuprel ®

Isradipine Dynacirc ®

Itraconazole Sporanox ® , Onmel ®

Ivabradine Procoralan ® , Coralan ® , Corlentor ® ,

Coraxan ® , Ivabid ® , Bradia ®

Ketoconazole Nizoral ® , Sebizole ® , Ketomed ® , Keton ®

Lapatinib Tykerb ® , Tyverb ®

Lenvatinib Lenvima ®

Leuprolide Lupron ® , Eligard ® , Viadur ® ,

Carcinil ® , Enanton ® , Leuplin ® , Lucrin ® , Procren ® , Prostap ® , and others

Levalbuterol Xopenex ® , Levolin ® , Axazest ®

Levofloxacin Levaquin ® , Tavanic ®

Lisdexamfetamine Vyvanse ®

Lithium Eskalith ® , Lithobid ®

Loperamide Imodium ® and many other OTC and

Rx brands Metaproterenol Metaprel ® , Alupent ®

Methadone Dolophine ® , Symoron ® , Amidone ® ,

Methadose ® , Physeptone ® , Heptadon ®

Methamphetamine Desoxyn ® , Pervitin ® , Anadrex ® ,

Methedrine ® , Syndrox ®

Methylphenidate Ritalin ® , Concerta ® , Focalin ® ,

Daytrana ® , Methylin ® , Metadate CD ®

Metoclopramide Reglan ® , Afipran ® , Maxolon ® , Cerucal ® ,

Clopamon ® , Clopra ® , Maxeran ® , Maxolon ® , Metozolv ® , Plasil ® , Pramin ® , Primperan ® , Perinorm ®

97 Long QT Syndrome

Generic name Brand names (Partial list)

Metronidazole Flagyl ® and many others

Midodrine ProAmatine ® , Amatine ® , Gutron ®

Mifepristone Korlym ® , Mifeprex ®

Mirabegron Myrbetriq ®

Mirtazapine Remeron

Moexipril/HCTZ Uniretic ® , Univasc ®

Moxifloxacin Avelox ® , Avalox ® , Avelon ®

Nelfinavir Viracept ®

Nicardipine Cardene ®

Nilotinib Tasigna ®

Norepinephrine Levophed ®

Norfloxacin Noroxin ® , Ambigram ®

Nortriptyline Pamelor ® , Sensoval ® , Aventyl ® ,

Norpress ® , Allegron ® , Noritren ® , Nortrilen ®

Ofloxacin Floxin ®

Olanzapine Zyprexa ® , Zydis ® , Relprevv ®

Ondansetron Zofran ® , Anset ® , Ondemet ® , Zuplenz ® ,

Emetron ® , Ondavell ® , Emeset ® , Ondisolv ® , Setronax ®

Osimertinib Tagrisso ®

Oxaliplatin Eloxatin ®

Oxytocin Pitocin ® , Syntocinon ®

Paliperidone Invega ® , Xeplion ®

Phenylephrine Neosynephrine ®

Phenylpropanolamine Acutrim ® , Dexatrim ®

Posaconazole Noxafil ® , Posamol ®

Procainamide Pronestyl ® , Procan ®

Promethazine Phenergan ®

Propofol Diprivan ® , Propoven ®

Pseudoephedrine PediaCare ® , Sudafed ®

Quetiapine Seroquel ®

Generic name Brand names (Partial list) Quinidine Quinaglute ® , Duraquin ® , Quinact ® ,

Quinidex ® , Cin-Quin ® , Quinora ®

Quinine sulfate Qualaquin ®

Ranolazine Ranexa ® , Ranozex ®

Rilpivirine Edurant ® , Complera ® , Eviplera ®

Risperidone Risperdal ®

Ritonavir Norvir ®

Salmeterol Serevent ® , Advair ®

Saquinavir Invirase ® (combo) Sertraline Zoloft ® , Lustral ® , Daxid ® , Altruline ® ,

Besitran ® , Deprax ® , Elrval ® , Emergen ® , Gladem ® , Implicane ® , Sedoran ® , Sealdin ® , SerivoLowfin ® , Stimuloton ® , Tresleen ® , Sertralin Bluefish ®

Sevoflurane Ulane ® , Sojourn ®

Tetrabenazine Nitoman ® , Xenazine ®

Thioridazine Mellaril ® , Novoridazine ® , Thioril ®

Tizanidine Zanaflex ® , Sirdalud ®

Tolterodine Detrol ® , Detrusitol ®

Toremifene Fareston ®

Torsemide Demadex ® , Diuver ® , Examide ®

Trazodone Desyrel ® (discontinued 6/13),

Oleptro ® , Beneficat ® , Deprax ® , Desirel ® , Molipaxin ® , Thombran ® , Trazorel ® , Trialodine ® , Trittico ® , Mesyrel ®

Sulfamethoxazole

Trimethoprim-Septra ® , Bactrim ® , Sulfatrim ® , Biseptol ® , Co-trimoxazole ® , Cotrim ® , Septrin ® , Trisul ®

Trimipramine Surmontil ® , Rhotrimine ® , Stangyl ®

Effect on Pregnancy

How Does Pregnancy Affect the Disease?

1 Physiological changes in pregnancy include increased

sympathetic tone which increases the risk of arrhythmia

(see entry “Physiological Changes of Pregnancy”)

2 In patients with LQTS, the rate of cardiac events during

pregnancy is similar to prepregnancy levels Syncope and

arrhythmias are [5 6] significantly increased in the

post-partum period [6 7]

How Does the Disease Affect Pregnancy?

1 Many women with congenital LQTS are treated with

pro-phylactic beta-blocker therapy, which can cross the

pla-centa Neonatal bradycardia and hypoglycemia may also

be seen

Management

In Consultation

1 If a previously undiagnosed prolonged QTc is discovered

on routine preanesthetic testing, a thorough search should

be undertaken to determine the etiology In many cases,

cardiology (electrophysiology) consultation will be

required

2 The mainstay of treatment for LQTS is beta-blocker

therapy, which should be continued throughout

preg-nancy and the postpartum period Patients who are

refractory to beta-blockers may require placement of

automated implanted cardioverter-defibrillators (ICDs)

In rare cases, a cervicothoracic denervation (also called

left cardiac sympathetic denervation, LCSD) may be

indicated

3 Serum electrolytes should be checked and corrected if

necessary

4 Many drugs can prolong the QT interval If the patient is

taking any, consider alternatives Before any new drug is

given to the patient, an assiduous search should be made

for possible interactions Many electronic medical record

systems will perform this check automatically See Box

97.1 for a partial list of medications, which prolong the

QT interval

5 Genetic counseling should be provided, as patients with

LQTS often have offspring with LQTS

Perioperative Management

1 During labor, the patient should have continuous ECG monitoring, with close attention to the QT interval Electrical conducting pads should be placed on the patient in case defibrillation or pacing is necessary

2 Consider placing an arterial line for frequent blood gas monitoring and a central venous catheter to manage fluid status

3 Prevent adrenergic stressors such as pain, anxiety, hypoxia, hypercarbia, hypothermia, shivering, hypoglycemia, and hyperglycemia Epidural labor analgesia is advised

4 Prophylactic magnesium infusion may be beneficial as it prevents the inward flow of potassium and sodium avoiding early after depolarizations which predispose to torsades

5 See Box 97.1 for a list of medications to avoid in LQTS patients

6 High peak airway pressures and the Valsalva maneuver may precipitate runs of VT through vagal stimulation Therefore, it has been suggested that the second stage of labor be shortened and patient advised to push as little as possible

7 Regional anesthesia is preferable to general anesthesia

as it decreases catecholamine levels; however care must

be taken to titrate slowly to avoid hypotension and bradycardia

(a) Spinal anesthesia is associated with a transient longation of the QTc Theoretically, a combined spinal-epidural (CSE) approach may be better as it allows more finely titrated doses of local anesthetic and prevention of hypotension

(b) If regional anesthesia is not possible, general thesia should be considered Since all volatile anes-thetics have the potential to prolong QTc, some have advocated using total intravenous anesthesia with propofol Rocuronium is preferred over succinyl-choline for intubation

8 In patients who have had LCSD, it is prudent to ment a complete pre-procedure neurological exam, as pain/temperature sensation may be altered at baseline and it may become difficult to assess the effectiveness of regional anesthesia In addition, the sympathectomy normally seen with regional blockade is attenuated

9 If the patient becomes hypotensive, use phenylephrine

Avoid ephedrine, dopamine, and dobutamine

10 If torsades occur, treat with magnesium sulfate, 1–2 g

over 5 min In an unstable patient, electrical sion is an option

cardiover-97 Long QT Syndrome

3 Drake E, et al Anesthetic implications of long QT syndrome in

pregnancy Can J Anesth 2007;54(7):561–72.

4 Meregalli P, et al Pregnancy and the risk of torsades de pointes in

congenital long-QT syndrome Neth Heart J 2008;16(12):422–5.

5 CredibleMeds Risk categories for drugs that prolong QT & induce Torsades de Pointes (TdP) https://crediblemeds.org/new-drug-list/ Accessed 16 May 2016.

6 Priori S, et al HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes Heart Rhythm 2013;10(12):1932–63.

7 Rashba E, et al Influence of pregnancy on the risk for cardiac events in patients with hereditary long QT syndrome Circulation 1998;97:451–6.

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_98

Lysosomal Storage Diseases

Janis M Ferns and Stephen H Halpern

Presentation and Symptoms

Definition

Lysosomal storage diseases are a group of rare inherited

dis-eases caused by enzyme deficiencies involved in glycolipid

storage Most are inherited as autosomal recessive disorders,

and the majority of patients with lysosomal storage diseases

do not survive until adulthood The diseases that affect adults

with implications for anesthesia are considered here:

1 Gaucher disease (GD) is an autosomal recessive disorder,

caused by a mutation in lysosomal enzyme acid

β-glucocerebrosidase resulting in deficiency or decreased

activity This leads to accumulation of glucocerebroside

in monocyte-macrophage cells, infiltrating tissue [1]

2 Fabry disease is an X-linked disorder due to alpha-

galactosidase deficiency Cells are unable to break down

some membrane glycosphingolipids resulting in

accumu-lation in tissues [2]

Symptoms

Clinical manifestations vary but can affect multiple

systems

1 There are three types of Gaucher disease; type I accounts

for the majority of disease in adults and is characterized

by lack of neurologic involvement Types II and III have

neurologic involvement Type II is fatal in early

child-hood Patients with type III disease may live into

adult-hood, but there are no cases of pregnancy described in

this population Therefore, only type I will be considered further

(a) Anemia, thrombocytopenia, and organomegaly are common findings The main presenting feature is hemorrhagic phenomena [1 3]

(b) Skeletal involvement may include osteoporosis, necrosis, and pathological fractures

(c) On rare occasions, lung involvement with pulmonary hypertension may occur

(d) Lymphatic, skin, eye, heart, and renal manifestations may be present

2 Fabry disease can cause a wide variety of cardiac, logical, and gastrointestinal symptoms [2]

(a) Peripheral neuropathic pain of the hands and feet and dysregulation of vascular and autonomic tone are common neurologic findings

(b) Stroke, myocardial ischemia and infarction, mias, hypertrophic cardiomyopathy, and valvular insufficiency may be present

(c) Abdominal pain and diarrhea may be present

(d) Other manifestations include temperature and cise intolerance, obstructive lung disease, and chronic renal impairment leading to renal failure

(e) Up to 70% of heterogenous female “carriers” will develop symptoms [4], and though they are diag-nosed later in life, they are still at risk of major organ involvement

Incidence

1 Gaucher disease type I has an incidence ranging from 1:40,000 to 1:60,000 in the general population However, the incidence in Ashkenazi Jews is higher with an inci-dence as high as 1:850 [5]

2 Fabry disease occurs in 1:40,000 males [2] The female carrier rate is unknown

J M Ferns, M.B.B.Chir., F.R.C.A • S H Halpern, M.D ( * )

Department of Anesthesia, Sunnybrook Health Sciences Centre,

University of Toronto, Toronto, ON, Canada

e-mail: janismei.ferns@sunnybrook.ca;

stephen.halpern@sunnybrook.ca

98

Interaction with Pregnancy

Effect of Pregnancy on Condition

1 Gaucher disease: Pregnancy can exacerbate existing

manifestations or trigger new symptoms However the

majority of reports in the literature, including a

retro-spective study of 43 women [6], note that pregnant

women with Gaucher disease show no change to their

clinical condition and have uncomplicated pregnancies

[1, 3 6]

(a) Anemia and thrombocytopenia can worsen more

than expected compared to normal pregnancy

Combining this with the expanding blood volume

and dilutional anemia in pregnancy, it will

poten-tially lower the threshold for transfusion in these

patients that are already at risk of hemorrhage

Abnormalities in blood count will require

follow-up with hematology and Gaucher disease

specialists

(b) Third trimester monitoring of platelet function as

well as platelet number and coagulation profile is

advised

(c) If patients have rare severe complications prior to

pregnancy such as pulmonary hypertension, then

appropriate counseling is needed regarding the

risk of conception or continuing with the

pregnancy

(d) Bone crises, such as bone pain, infarction, or

frac-tures, can occur during pregnancy [6]

(e) Hepatosplenomegaly can worsen If concerns exist,

this should be monitored with ultrasound Care

should be taken at delivery to prevent injury through

pressure on enlarged organs

2 Fabry disease: There are cases of uncomplicated

preg-nancy when there is no vital organ involvement [4] In

one case report, minor symptoms such as paresthesia

improved during pregnancy and then returned 6 months

postpartum [4]

(a) Women with cardiovascular complications (left

ven-tricular hypertrophy, hypertrophic cardiomyopathy,

ischemia) are at risk of worsening clinical status

due to the cardiovascular changes in pregnancy [2

4]

(b) Cardiac problems are the most common cause of

death in female carriers; therefore prepregnancy/

postconception counseling is advised in these high-

risk patients

(c) Renal manifestations could worsen, and renal

func-tion should be evaluated throughout pregnancy

(d) Gastrointestinal symptoms from the disease may be

hard to differentiate from those related to

pregnancy

Effect of Condition on Pregnancy

1 Gaucher disease: The majority of women will delivery normal healthy infants [1 3]

(a) In moderate to severe disease, hepatomegaly can interfere with fetal growth due to mass effect

(b) Women may be at increased risk of spontaneous abortion

(c) Excessive bleeding is a risk throughout pregnancy, delivery, and postpartum irrespective of treatment and disease severity There is a higher incidence of postpartum hemorrhage and need for blood transfu-sion [6] Bleeding at any point prior to delivery is a risk for the fetus

2 Fabry disease: The presence of renal disease can increase the risk of hypertensive disorders of pregnancy, intrauterine growth restriction, and prematurity [4]

(a) There is potential for placental dysfunction, so close fetal growth monitoring is required

Medical Management Testing and Diagnosis

1 In Gaucher disease β-glucocerebrosidase activity is sured and will be decreased in the presence of disease DNA testing is an adjunct [5]

2 In Fabry disease, DNA analysis is the gold standard Enzymatic activity is used, but activity is variable in females and can be normal [2]

Medical Management

Enzyme replacement therapy (ERT) is the main treatment for both Gaucher and Fabry disease Multidisciplinary team involvement is essential in these patients, ensuring expert advice for complications and the best management approach

(b) FDA guidance recommends use in pregnancy if indication and need are clear European guidance advises considering imiglucerase in women prior to conception to optimize health n the symptomatic patient

(c) ERT can prevent disease-related pregnancy

compli-cations, including reduction in risk of spontaneous

abortion and of bleeding complications at delivery

and postpartum [7] Women already established on

ERT should consider continuing during pregnancy

(d) There was no evidence of adverse effects on the fetus

or newborn from velaglucerase alpha in a case series

of 25 patients Velaglucerase alpha is classified by the

FDA as “category B.” Animal studies at higher than

normal doses do not adversely affect fetal rats or

rab-bits Human studies suggest the drug is safe, but

experience is limited [7] Imiglucerase has an FDA

rating of “category C,” but there is one case report of

its successful use in pregnancy [8]

2 Complications of Gaucher disease need to be managed,

and some medications such as the bisphosphonates for

osteoporosis are contraindicated in pregnancy [1]

(a) Vitamin B12 deficiency can coexist with Gaucher

disease, and supplementation is advised [1]

(b) Women may have had a splenectomy as part of their

management These women are at risk of infection

They should receive appropriate vaccinations prior to

conception and continue prophylactic antibiotics

ensuring compatibility with pregnancy [1]

3 Fabry disease: ERT with agalsidase beta is the main

treat-ment in patients with significant symptoms [2]

(a) There are only two case reports of use in pregnancy

with no adverse effects to the mother or fetus [9 10]

(b) ERT delays disease progression and reduces cardiac,

renal, and neurological manifestations

4 Treatment and prevention of complications of Fabry

dis-ease include prophylactic antiplatelet medication to

pre-vent ischemic stroke Monitoring of cardiac function

includes serial echocardiograms and electrocardiograms

Cardiac manifestations are serious but can be

asymptom-atic for a long time Renal impairment may lead to end-

stage renal failure requiring dialysis

(a) Prepregnancy baseline functional tests are

recom-mended for cardiovascular, neurological,

ophthalmo-logical, and renal complications

Anesthetic Management

Medication

There is little evidence in case reports of adverse interactions

with medications used during labor and operative delivery

As there is a bleeding tendency in Gaucher disease [1],

avoiding medications that can worsen this, such as

nonsteroi-dal anti-inflammatory drugs, is recommended Being

pre-pared for postpartum hemorrhage with good intravenous

access and availability of blood products is advisable

Neuraxial Analgesia

1 Discussion about the appropriateness of epidural sia should be part of birth planning in Gaucher disease [1] Limitations will depend on hematological parame-ters, particularly platelet number and function

(a) Platelet counts are mandatory before proceeding with neuraxial analgesia Patients may have abnormal platelet function resulting in clinically evident bleed-ing (abnormal bruising, or petechiae)

(b) Skeletal deformities may make neuraxial placement challenging

2 In patients with Fabry disease, antiplatelet medication may be continued in pregnancy to prevent stroke [2] Depending on the type and timing of the last dose, this may preclude insertion of neuraxial analgesia The most recent guidelines on regional anesthesia and antiplatelet and anticoagulant medication should be followed [11]

Anesthesia for Cesarean Delivery

1 For women with Gaucher disease, the mode of delivery will depend on factors such as limitations to movement from bone disease, skeletal deformities, or prostheses Vaginal delivery is preferred [1]

(a) If cesarean section is indicated, similar concerns of regional anesthesia exist as for insertion of epidural analgesia If platelet count and function allow, regional anesthesia can be performed

(b) Bleeding at delivery is a concern Gaucher disease is an independent risk factor [1] Other risk factors for bleed-ing should be minimized and preparations for anticipated hemorrhage made including blood product replacement (c) Attention to positioning and transfer of patient is needed, regardless of mode of delivery, to prevent skeletal damage [5]

2 Fabry disease: Similar considerations as neuraxial analgesia regarding antiplatelet and anticoagulant medications exist with regional anesthesia There are no documented cases of women with Fabry disease and their anesthetic manage-ment for cesarean section The presence of any cardiac and renal complications will guide anesthetic management

Postoperative Management

Women with Gaucher disease are still at risk of bleeding in the postpartum period [1] Especially after cesarean section, they should be monitored in the hospital for 24–48 h There may be

a higher incidence of postoperative infection in symptomatic patient taking ERT, so vigilance is required Breastfeeding with ERT is likely to be of minimal risk to infants

98 Lysosomal Storage Diseases

1 Granovsky-Grisaru A, Belmatoug N, Vom Dahl S, Mengel E,

Morriw E, Zimran A The management of pregnancy in Gaucher

disease Eur J Obstet Gynecol Reprod Biol 2011;156(1):3–8.

2 Wilcox WR, Oliveira JP, Hopkins RJ, Ortiz A, Banikazemi M,

Feldt-Rasmussen U, et al Females with Fabry disease frequently

have major organ involvement: lessons from the Fabry Registry

Mol Genet Metab 2008;93(2):112–28.

3 Rosenbaum H Management of women with Gaucher disease in the

reproductive age Thromb Res 2015;135(Suppl 1):S49–51.

4 Parent E, Wax JR, Smith W, Blaszyk H, Pinette MG, Carin A, et al

Fabry disease complicating pregnancy J Matern Fetal Neonatal

Med 2010;23(10):1253–6.

5 Isoscovich A, Elstein Y, Halpern S, Grisaru-Granovsky S, Elstein

D Anesthesia for obstetric patients with Gaucher disease: survey

and review Int J Obstet Anesth 2004;13(4):244–50.

6 Elstein Y, Eisenberg V, Granovsky-Grisaru S, Rabinowitz R, Samueloff A, Zimran A, et al Pregnancies in Gaucher disease: a 5-year study Am J Obstet Gynecol 2004;190:435–41.

7 Elstein D, Hughes D, Goker-Alpan O, Stivel M, Baris HN, Cohen IJ,

et al Outcome of pregnancies in women receiving velaglucerase alfa for Gaucher disease J Obstet Gynaecol Res 2014;40(4):968–75.

8 Giannubilo SR, Pasculli A, Ciavattini A Replacment therapy for gaucher disease during pregnancy: a case report J Reprod Infertil 2015;16(1):53–7.

9 Kalkum G, Maccheilla D, Reinke J, Kolbl H, Beck M Enzyme replacement therapy with agalsidase alfa in pregnant women with Fabry disease Eur J Obstet Gynecol Reprod Biol 2009;144(1):92.

10 Politei J Treatment with agalsidase beta during pregnancy in Fabry disease J Obstet Gynaecol Res 2010;36(2):428–9.

11 Horlocker TT, Wedel DJ, Rowlingson JC, et al Regional thesia in the patient receiving antithrombotic therapy or thrombo- lytic therapy: American Society of Regional Anesthesia and Pain Medicine Evidence-Based Guidelines (Third Edition) Reg Anesth Pain Med 2010;35(1):64–101.

© Springer International Publishing AG, part of Springer Nature 2018

S K W Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_99

Malignancy

Roulhac D Toledano

Abbreviations

ALL Acute lymphoblastic leukemia

AML Acute myeloid leukemia

ASRA American Society of Regional Anesthesia and Pain

Medicine

CNS Central nervous system

CSF Cerebrospinal fluid

GTN Gestational trophoblastic neoplasia

HPV Human papilloma virus

ICP Intracranial pressure

ITP Idiopathic thrombocytopenic purpura

MRI Magnetic resonance imaging

PCA Patient-controlled analgesia

TSH Thyroid-stimulating hormone

General Information

Incidence

Malignancies diagnosed either prenatally or within the first

12 months postpartum affect an estimated 1 in 1000

pregnan-cies, and this figure is likely to increase as women delay

childbearing into their later reproductive years Cancers of

the reproductive tract (primarily cervical and ovarian),

mela-noma, breast cancer, lymphoma and leukemia, and thyroid

cancer comprise the majority of these malignancies

The management of malignant cancer encountered during

pregnancy requires consideration of several maternal and

fetal factors, including the type and stage of the cancer, the

maternal risks associated with delaying treatment, the desire

for pregnancy continuation, gestational age, and the potential

harmful effects of surgery, radiation (diagnostic and

therapeutic), and chemotherapy on the fetus Anesthetic agement may be complicated by therapeutic interventions, concern for dissemination of cancer cells when performing neuraxial techniques, and by aggressive thromboprophylaxis regimens, among other things This chapter reviews the most common malignancies diagnosed during pregnancy and sum-marizes key anesthetic implications

Specific Malignancies Gynecological

Cancers of the female reproductive tract comprise the ity of malignancies encountered during pregnancy Of those, malignant cervical and ovarian cancers are far more common than malignancies of the uterus, vulva, and vagina

major-Squamous cell carcinomas comprise the vast majority of invasive cervical cancers, followed by adenocarcinomas Lesions suspicious for invasive cervical cancer on routine screening require modified staging during pregnancy; if pos-sible, cervical conization is avoided In most cases, pregnant patients are diagnosed with early-stage disease, and defini-tive treatment can be delayed until several weeks postpar-tum The decision regarding whether and when to undertake surgery and chemoradiotherapy in cases of truly invasive cervical cancer must be individualized The mode of delivery for pregnancies complicated by invasive cervical cancer remains controversial except in cases in which a bulky or fri-able lesion renders vaginal delivery indisputably prohibitive

While benign ovarian masses are relatively common among women of reproductive age, ovarian malignancy is uncommon in pregnant women Most ovarian masses are asymptomatic and are detected incidentally on routine ultra-sound during pregnancy; however, some may result in acute abdominal pain related to ovarian torsion or hemorrhage In general, management depends on the size and appearance of the mass and is similar among pregnant and nonpregnant individuals, with the caveat that gestational age may impact

R D Toledano, M.D., Ph.D.

Department of Anesthesiology, Preoperative Care, and Pain

Medicine, NYU Langone Hospital – Brooklyn, Brooklyn, NY, USA

99