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Advances in Vestibular
Schwannoma
Microneurosurgery

Improving Results with New


Technologies
Luciano Mastronardi
Takanori Fukushima
Alberto Campione
Editors

123
Advances in Vestibular Schwannoma
Microneurosurgery
Luciano Mastronardi
Takanori Fukushima • Alberto Campione
Editors

Advances in Vestibular
Schwannoma
Microneurosurgery
Improving Results with New
Technologies
Editors
Luciano Mastronardi Takanori Fukushima
Unit of Neurosurgery Neurosurgery Department
ASLRoma1 Duke Medical Center
San Filippo Neri Hospital Raleigh, NC
Roma USA
Italy

Alberto Campione
Unit of Neurosurgery
ASLRoma1
San Filippo Neri Hospital
Rome
Italy

ISBN 978-3-030-03166-4    ISBN 978-3-030-03167-1 (eBook)


https://doi.org/10.1007/978-3-030-03167-1

Library of Congress Control Number: 2018966545

© Springer Nature Switzerland AG 2019


This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of
the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recita-
tion, broadcasting, reproduction on microfilms or in any other physical way, and transmission or infor-
mation storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar
methodology now known or hereafter developed.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this publica-
tion does not imply, even in the absence of a specific statement, that such names are exempt from the
relevant protective laws and regulations and therefore free for general use.
The publisher, the authors, and the editors are safe to assume that the advice and information in this book
are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the
editors give a warranty, express or implied, with respect to the material contained herein or for any errors
or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims
in published maps and institutional affiliations.

This Springer imprint is published by the registered company Springer Nature Switzerland AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
Foreword

Fortunate are the neurosurgeons who have the opportunity to visit the Department
of Neurosurgery at San Filippo Neri Hospital in Rome and Carolina Neuroscience
Institute in Raleigh, North Carolina. I am fortunate to have been one of them for so
many years.
Visitors to the heads of these departments, authors of this book, just can experi-
ence their natural warmth, honesty, and spontaneous integrity together with a gentle
sense of humor and love for music, together with an intense devotion for perfecting
their surgical experiences.
For me, it is an honor to be invited to write this foreword as their lifetime close
friend, having the same basic philosophy. This philosophy is simple and clean. I am
thankful for our continued long-term professional cooperation and friendship,
which feels like brothership.
For more than 20 years, I cooperated with the authors, my lifetime mentors,
heroes, and friends.
Both are recognized as world-class neurosurgeons.
The book is based upon the rich personal experiences of both neurosurgeons .
That other neurosurgeons may differ with some of these principles is to be expected,
and “to each his own.”
The stepwise illustrations and text bring an understanding of surgical anatomy
and important minute details of different surgical approaches and techniques in the
best way.
Through the years, they have made it a practice to sketch the steps and surgical
anatomy of these operative procedures, improving every time while teaching
cadaver hands-on courses.
As the title indicates, Drs. Mastronardi and Fukushima have written about neu-
rosurgery experiences on acoustic schwannomas, as performed in both centers;
however, knowing them so well, they have done much more. Both the authors are
famous teachers—and teaching is an art. The authors are truly master neurosur-
geons, and attention will be paid to every detail, including the operation room and
table, their personal habits, and the instruments: “the freedom of art.”
Surgery of acoustic neuroma is one of the most difficult in neurosurgery.
Surgeons must have extremely precise super-micro-operative technical skills,
proper usage of updated super-micro-instruments, sufficient knowledge of dis-
placed C-P angle microanatomy, and ample clinical case experience. These

v
vi Foreword

principles will result in surgeries with no mortality and negligible morbidity,


­including normal facial function and preservation of hearing or usable hearing
function.
Readers will find considerable practical advice in each chapter, and especially
important points will be summarized in all approaches: retrosigmoid, middle fossa,
and translabyrinthine. This book contains some of the most accurate and beautiful
illustrations of microsurgery of acoustic schwannomas I have ever seen. It deserves
to be presented in the language of every neurosurgeon in the world.
This volume is a symbol of the exhaustive labors of the authors and should also
be looked upon as eloquent evidence of the high professional caliber of the neuro-
surgical program at both neurosurgical centers, both in care of patients and in edu-
cation of neurosurgeons.

Ghent, Belgium Luc F. De Waele


Preface

 ew Technologies Available for the Surgical


N
Treatment of Acoustic Neuromas

The aim of this book is to report the results of microsurgical removal of acoustic
neuromas (AN) using new technologies: flexible handheld laser fibers, Sonopet
Ultrasound Aspirator, Facial Nerve “detector” monopolar stimulation (to localize
position and course of nerve), BAERs with LS CE-Chirp stimulus for hearing pres-
ervation, injectable bone substitute for closure, and so on. We report retrospective
clinical observations on patients operated on during the last 8 years.
From September 2010 to April 2018, 160 consecutive patients suffering from AN
have been operated on with a microsurgical technique by keyhole retrosigmoid (RS)
approach in the Division of Neurosurgery of San Filippo Neri Hospital, ASLRoma1.
In more than 100 cases 2μ-Thulium laser fiber was used for cutting, vaporizing, and
removal of tumor. In the same period, Sonopet Ultrasound aspirator was used for
tumor debulking and/or opening of the internal auditory canal in all cases. From
May 2015, hearing preservation by means of LS-CE-Chirp BAER was attempted in
patients with preoperative socially useful hearing (AAO-HNS class A and B). From
December 2017 we started to check the total removal of tumor inside the internal
auditory canal (IAC) with the flexible endoscope for completing tumor removal
near the fundus.
Overall time from incision to skin suture changed in relation to size of tumor and
was not affected by the use of new technologies. Facial nerve function was clini-
cally assessed with the House-Brackmann (HB) scale (I–IV) preoperatively, in the
early postoperative period (after 1 week), and at 6-month follow-up. In three cases
a preoperative facial nerve palsy was observed (2 HB III and 1 HB IV, respectively).
In the remaining cases, facial nerve preservation rate (HB I) was more than 90% at
6 months after surgery. Hearing preservation rate (AAO-HNS A and B, preopera-
tively and postoperatively) was about 50%. Total tumor and “nearly total” removal
was possible in about ¾ of cases and subtotal in about 20%. Dura closure with
underlying autologous pericranium and injectable bone substitute for closure after
bone flap repositioning minimized postoperative CSF leakage.
In conclusion, the use of new technologies in AN microsurgery appeared to be
safe and subjectively seems to facilitate tumor resection, especially in “difficult”

vii
viii Preface

conditions (e.g., highly vascularized or hard tumors). The good functional outcome
following conventional microsurgery could be further improved and the extent of
tumor removal could be increased by the new tools available in the neurosurgical
armamentarium.

Rome, Italy Luciano Mastronardi


Contents

Part I General Aspects


1 Introduction: Clinical and Radiological Diagnosis��������������������������������   3
Alberto Campione, Guglielmo Cacciotti, Raffaelino Roperto,
Carlo Giacobbo Scavo, Lori Radcliffe, and Luciano Mastronardi
2 Treatment Options and Surgical Indications������������������������������������������ 13
Luciano Mastronardi, Alberto Campione, Raffaelino Roperto,
Albert Sufianov, and Takanori Fukushima

Part II Illustrated Surgical Technique (Step-by-Step)


3 Patient Positioning ������������������������������������������������������������������������������������ 23
Luciano Mastronardi, Alberto Campione, Guglielmo Cacciotti,
Raffaelino Roperto, Fabio Crescenzi, Ali Zomorodi,
and Takanori Fukushima
4 Instrumentation for Acoustic Neuroma Microneurosurgery���������������� 31
Luciano Mastronardi, Alberto Campione, Guglielmo Cacciotti,
Raffaelino Roperto, Fabio Crescenzi, Ali Zomorodi,
and Takanori Fukushima
5 Retrosigmoid Approach���������������������������������������������������������������������������� 39
Luciano Mastronardi, Alberto Campione, Guglielmo Cacciotti,
Raffaelino Roperto, Carlo Giacobbo Scavo, Ali Zomorodi, and
Takanori Fukushima
6 Translabyrinthine Approach�������������������������������������������������������������������� 53
Luciano Mastronardi, Alberto Campione, Guglielmo Cacciotti,
Raffaelino Roperto, Carlo Giacobbo Scavo, Ali Zomorodi, and
Takanori Fukushima
7 Video Clips of the Surgical Steps�������������������������������������������������������������� 69
Luciano Mastronardi, Alberto Campione, Guglielmo Cacciotti,
Raffaelino Roperto, Carlo Giacobbo Scavo, Ali Zomorodi, and
Takanori Fukushima

ix
x Contents

8 Results in a Personal Series of 160 Cases������������������������������������������������ 73


Luciano Mastronardi, Alberto Campione, Guglielmo Cacciotti,
Raffaelino Roperto, and Carlo Giacobbo Scavo

Part III New Technologies


9 Intraoperative Identification and Location of Facial Nerve:
Type of Facial Nerve Displacement—How to Use
Monopolar Stimulator ������������������������������������������������������������������������������ 83
Luciano Mastronardi, Alberto Campione, Ali Zomorodi,
Ettore Di Scipio, Antonio Adornetti, and Takanori Fukushima
10 Hearing Preservation�������������������������������������������������������������������������������� 95
Luciano Mastronardi, Alberto Campione, Ali Zomorodi,
Ettore Di Scipio, Antonio Adornetti, and Takanori Fukushima
11 Usefulness of Laser and Ultrasound Aspirator �������������������������������������� 105
Luciano Mastronardi, Alberto Campione, Ali Zomorodi,
Raffaelino Roperto, Guglielmo Cacciotti, and Takanori Fukushima
12 Techniques of Dural Closure for Zero CSF Leak ���������������������������������� 113
Luciano Mastronardi, Guglielmo Cacciotti, Alberto Campione,
Ali Zomorodi, Raffaelino Roperto, and Takanori Fukushima
13 Face-to-Face Two-Surgeons Four-Hands Microsurgery������������������������ 117
Takanori Fukushima and Ali Zomorodi

Part IV Projects in Progress


14 Diluted Papaverine for Microvascular Protection
of Cranial Nerves �������������������������������������������������������������������������������������� 123
Alberto Campione, Carlo Giacobbo Scavo, Guglielmo Cacciotti,
Raffaelino Roperto, and Luciano Mastronardi
15 Flexible Endoscope for IAC Control of Tumor Removal ���������������������� 131
Alberto Campione, Carlo Giacobbo Scavo, Guglielmo Cacciotti,
Raffaelino Roperto, and Luciano Mastronardi
16 Fluid Cement for Bone Closure���������������������������������������������������������������� 137
Alberto Campione, Guglielmo Cacciotti, Raffaelino Roperto,
Carlo Giacobbo Scavo, and Luciano Mastronardi
17 Aspirin Administration for Control of Tumor Millimetric Residual���� 143
Alberto Campione, Guglielmo Cacciotti, Raffaelino Roperto,
Carlo Giacobbo Scavo, and Luciano Mastronardi
Contents xi

18 DTI for Facial Nerve Preoperative Prediction of


Position and Course ���������������������������������������������������������������������������������� 149
Alberto Campione, Guglielmo Cacciotti, Raffaelino Roperto,
Carlo Giacobbo Scavo, and Luciano Mastronardi
19 Vestibular Testing to Predict the Nerve of Origin of Vestibular
Schwannomas �������������������������������������������������������������������������������������������� 155
Alberto Campione, Guglielmo Cacciotti, Raffaelino Roperto,
Carlo Giacobbo Scavo, and Luciano Mastronardi
20 Microsurgery for Vestibular Schwannomas
After Failed Radiation Treatment������������������������������������������������������������ 161
Yoichi Nonaka and Takanori Fukushima
Conclusions�������������������������������������������������������������������������������������������������������� 173
Part I
General Aspects
Introduction: Clinical and Radiological
Diagnosis 1
Alberto Campione, Guglielmo Cacciotti,
Raffaelino Roperto, Carlo Giacobbo Scavo, Lori Radcliffe,
and Luciano Mastronardi

Vestibular schwannomas (VSs) (also known as acoustic neuromas) arise from


Schwann cells, which form the myelin sheath around the vestibulocochlear nerve.

1.1 Epidemiology

VSs predominantly affect adults in their fifth and sixth decades and are indeed much
rarer in children, in which are mainly due to neurofibromatosis type 2.
VSs account for 5–10% of intracranial tumors and are the most common neo-
plastic lesions in the cerebellopontine angle. The overall incidence is approximately
1 per 100,000 persons per year and appears to be increasing because of longer life
expectancy and improved diagnostic tools. For these reasons, the mean tumor size
at time of diagnosis has progressively declined over the years and is now 10–15 mm,
with larger tumors being only a minority [1–4].
No significant differences in incidence between sexes or prevalence of side have
been described in the literature.

1.2 Risk Factors

The main risk factor for VS is the exposure to radiation. Two main circumstances
have been associated with a higher incidence of the disease:

• Exposure to high-dose ionizing radiation [5]


• Childhood exposure to low-dose radiation for benign head and neck conditions [6, 7]

A. Campione (*) · G. Cacciotti · R. Roperto · C. Giacobbo Scavo · L. Mastronardi


Department of Neurosurgery, San Filippo Neri Hospital—ASLRoma1, Rome, Italy
e-mail: mastro@tin.it
L. Radcliffe
Carolina Neuroscience Institute, Raleigh, NC, USA
e-mail: lori@carolinaneuroscience.com
© Springer Nature Switzerland AG 2019 3
L. Mastronardi et al. (eds.), Advances in Vestibular Schwannoma
Microneurosurgery, https://doi.org/10.1007/978-3-030-03167-1_1
4 A. Campione et al.

It has recently been statistically ascertained that the exposure to leisure noise posi-
tively correlates with an elevated risk of VS. Indeed, severe acoustic trauma from
impulse noise can cause mechanical damage of N VIII and the surrounding tissues.
From a biochemical perspective, loud acoustic stimulation induces electrolytes disequi-
librium and release of free radicals in cochlear fluids, which could in turn be responsible
for DNA damage in cochlear hair cells. Thus, although the exact mechanism is still
unclear, it is plausible that VSs may arise due to chronic trauma due to impulse noise [8].
The role of cellular telephone in the pathogenesis of VS remains unclear and
controversial.

1.3 Pathogenesis and Pathology

From a genetic perspective, the origin of most sporadic VSs is the biallelic inactiva-
tion of gene NF2, which encodes a protein called merlin (also known as schwan-
nomin) that acts as a tumor suppressor. The gene NF2 was first discovered as the
locus on chromosome 22 harboring the mutation responsible for familiar and bilat-
eral VSs seen in neurofibromatosis type 2 (NF2) [1, 2].
VSs are classified as WHO grade I tumors and have a locally compressing effect
rather than an infiltrative tendency. The growth rate is <1 mm per year for more than
60% of patients and >3 mm per year for 12%. The average ki67 (MIB-1) index ranges
between 1.86 and 1.99%. However, some differences in terms of ki67 index have been
reported between unilateral and bilateral VSs, as well as between growing and stable
ones [9–11]. The prognostic value of such discrepancies has been deemed uncertain
and in need of future study [12]. Malignant degeneration is exceedingly rare.
Macroscopically, VSs appear as pale, capsulated globoid masses displacing or
splaying surrounding neural structures. Occasional foci of hemorrhage or cystic
degeneration may also be observed.
The site of origin is the inferior vestibular nerve (IVN) in 70% of cases and the
superior vestibular nerve (SVN) in 20% of cases. In both the circumstances, VSs
frequently arise at the Obersteiner-Redlich junction, i.e., the point of encounter of
central and peripheral myelin, near to porus acusticus. Less frequently, the tumor
can arise close to the meatus, and in this case it is referred to as the “medial variety,”
with a small amount of tumor in the lateral part of the internal auditory canal [13].
In rarer cases—10% of total—VSs may arise from the cochlear nerve [14].
As for the displacement of cranial nerves, intraoperative stimulation and neuro-­
monitoring have enabled to trace the course of both facial (N VII) and cochlear (N
VIII) nerves. In 70% of cases, the position of N VII is ventral or ventral-superior to
the tumor. Alternatively, N VII may be displaced superiorly (20% of cases), inferi-
orly or ventral-inferiorly (10%), and, rather exceptionally, dorsally. The position of
N VIII is usually inferior or ventral-inferior [14, 15].
Microscopically, neoplastic Schwann cells appear as arranged in two different
tissue patterns: Antoni A (dense cellularity) and Antoni B (sparse cellularity). As VSs
induce angiogenesis, this may result in telangiectatic formations and subsequent
intratumoral hemorrhages. A thick collagenous capsule is usually present [1, 4].
1 Introduction: Clinical and Radiological Diagnosis 5

1.4 Clinical Presentation

The clinical presentation of VSs correlates with the structures gradually and chroni-
cally compressed by the tumor. These are in the first place N VIII, trigeminal nerve
(N V), and N VII. Later, as the tumor enlarges, lower cranial nerves may be involved
and then the cerebellum and the brainstem.

• The most common symptoms are hearing loss (95% of patients) and tinnitus
(63%), usually with a chronic onset—although acute cases have been occasion-
ally reported. Preoperative tinnitus has been reported as a negative prognostic
sign for hearing preservation [16].
• Disequilibrium (61%) is typically mild to moderate in intensity and fluctuating
in timing.
• The involvement of N V (17%) is often reported after the hearing loss and mainly
presents as facial paresthesia, hypoesthesia, or pain.
• Facial paresis (6%) is usually of chronic onset and correlates (in most of cases)
with tumors large enough to compress the intralabyrinthine tract of N V or even
the geniculate ganglion. However, recent reports have described cases of acute
facial paresis as the exording sign of still intracanalicular VSs (Mastronardi
et al., in publication). This peculiar presentation is exceptional (1%) and may
pose a challenging differential diagnosis with N VII schwannoma, meningioma,
cavernoma, or malignancy.
• The symptoms of tumor progression may be of different intensity and timing.
Headache (32%) is rather common and early. Instead, nausea and vomiting (9%)
correlate with compression of cerebellum and brainstem, which may lead to
hydrocephalus.

1.5 Diagnosis

The diagnosis of VS requires both clinical suspicion and laboratory/imaging tests.


VS must be suspected in case of unilateral hearing loss of chronic onset, with a
positive Rinne test and a Weber test lateralized on the unaffected side. Both the tests
are needed to confirm the sensorineural origin of the hearing impairment.
Functional laboratory tests constitute the next step of diagnostic workup.
Audiometry is the best initial screening test as only 5% of affected patients have
normal results. Pure tone and speech discrimination audiometry should be per-
formed for a correct classification of the patient’s hearing damage. Test results typi-
cally show hearing loss for high frequencies and a disproportionately negative
speech discrimination score [2, 4]. The American Academy of Otolaryngology-­
Head and Neck Surgery (AAO-HNS) hearing classification is the current grading
system that, according to pure-tone average and speech discrimination percentage,
determines whether the hearing is still functional and to what extent (Table 1.1).
Auditory brainstem evoked response (ABR) is a further functional screening
measure that may be used in case of abnormal results of audiometric tests. The main
6 A. Campione et al.

Table 1.1 American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) hearing
classification
Class Pure-tone average (dB) Speech discrimination (%)
A: Useful ≤30 ≥70
B: Socially useful >30 and ≤50 ≥50
C: Capable of aid (serviceable) >50 ≥50
D: Nonfunctional Any level <50

+100 nV

III IV V
I II

11
80

ms
0 1 2 3 4 5 6 7 8 9 10
dB nHL

Fig. 1.1 Normal ABR. Reprinted from Clinical Neurology and Neurosurgery, 165, Luciano
Mastronardi, Ettore Di Scipio, Guglielmo Cacciotti, Raffaelino Roperto, Vestibular schwannoma
and hearing preservation: Usefulness of level specific CE-Chirp ABR monitoring. A retrospective
study on 25 cases with preoperative socially useful hearing, Pages No. 108–115, 2018, with per-
mission from Elsevier

advantage of ABR is that it is a patient-independent test and shows objective results


about the damage to the different levels of the auditory pathway. ABR results typi-
cally show a major delay in cochlear nerve conduction, with increased latency in
wave III and, later, in waves V and VI [4] (Figs. 1.1 and 1.2).
The technique of ABR is not only useful as a diagnostic tool but also as an intra-
operative neuro-monitoring instrument that enables the surgeon to correctly identify
and preserve N VIII. At the end of surgical operation, ABR reflects the functional
state of N VIII, which is shown in Fig. 1.3. ABR may appear as normal, destruc-
tured, or delayed.
Imaging tests constitute the final step of diagnostic workup. MRI with gadolinium
contrast is the gold standard for radiological diagnosis. VS appears as lesions of the
internal auditory canal (IAC) with variable extension into the cerebellopontine angle
(CPA). Different grading systems have been proposed to stage tumor progression.
Samii’s classification is one of the most commonly used and is mainly based on the
anatomical relationship around the tumor [17] (Table 1.2) (Figs. 1.4 and 1.5).
1 Introduction: Clinical and Radiological Diagnosis 7

III
I

90 L

ms
0 1 2 3 4 5 6 7 8 9 10

Fig. 1.2 Pathologic ABR. Reprinted from Clinical Neurology and Neurosurgery, 165, Luciano
Mastronardi, Ettore Di Scipio, Guglielmo Cacciotti, Raffaelino Roperto, Vestibular schwannoma and
hearing preservation: Usefulness of level specific CE-Chirp ABR monitoring. A retrospective study on 25
cases with preoperative socially useful hearing, Pages No. 108–115, 2018, with permission from Elsevier

a
V
I III

90 L

ms
0 1 2 3 4 5 6 7 8 9 10
b

90 R

dB nHL ms
0 1 2 3 4 5 6 7 8 9 10

Fig. 1.3 Postoperative ABRs. (a) Normal postoperative ABR (registered from the same patient as
in Fig. 1.2). (b) Destructured ABR. (c) Delayed ABR. Reprinted from Clinical Neurology and
Neurosurgery, 165, Luciano Mastronardi, Ettore Di Scipio, Guglielmo Cacciotti, Raffaelino
Roperto, Vestibular schwannoma and hearing preservation: Usefulness of level specific CE-Chirp
ABR monitoring. A retrospective study on 25 cases with preoperative socially useful hearing,
Pages No. 108–115, 2018, with permission from Elsevier
8 A. Campione et al.

c
V
I
III

90 R

dB nHL ms
0 1 2 3 4 5 6 7 8 9 10

Fig. 1.3 (continued)

Table 1.2 Samii’s classification of VSs


Tumor description
T1 Confining to IAC
T2 Surpassing IAC
T3a Tumor occupying CPA
T3b Tumor occupying CPA and contacting brainstem without compression
T4a Tumor compressing the brainstem
T4b Severe brainstem displacement and deformation of the fourth ventricle under tumor
compression

a b

Fig. 1.4 Samii’s classification of VSs. (a) Stage T1, tumor indicated by arrow. (b) Stage T2,
tumor indicated by arrow
1 Introduction: Clinical and Radiological Diagnosis 9

a b

c d

Fig. 1.5 Samii’s classification of VSs. (a) Stage T3a, tumor indicated by arrow. (b) Stage T3b,
tumor indicated by arrow. (c) Stage T4a, tumor indicated by arrow. Note that despite the brainstem
compression the fourth ventricle still retains its normal morphology. (d) Stage T4b, tumors indi-
cated by arrows. Note that this is a rare case of bilateral giant VSs. Figures (b and c) reprinted from
Clinical Neurology and Neurosurgery, 165, Luciano Mastronardi, Ettore Di Scipio, Guglielmo
Cacciotti, Raffaelino Roperto, Vestibular schwannoma and hearing preservation: Usefulness of
level specific CE-Chirp ABR monitoring. A retrospective study on 25 cases with preoperative
socially useful hearing, Pages No. 108–115, 2018, with permission from Elsevier

VSs characteristically have an intracanalicular component which widens the


porus acusticus, leading to the “trumpeted internal acoustic meatus sign” in
MRI. The extracanalicular component typically has a globoid shape and is obvi-
ously connected to the cone-like intracanalicular component, thus resembling an
“ice-cream cone appearance.” VSs are described as isointense/hypointense lesions
on T1-weighted images and are strongly contrast enhanced. On T2-weighted
images, they are hyperintense. Cystic lesions may be observed in 10–15% of cases,
especially when the neoplastic mass reaches a big size.
10 A. Campione et al.

CT in VS diagnosis is reserved to patients who do not tolerate MRI but may be


useful—especially in the case of giant tumors—to observe the degree of bone ero-
sion around the tumor for a better operative planning.
Radiological differential diagnosis of VSs includes meningiomas and epider-
moid cysts. Meningiomas have a similar appearance on T1- and T2-weighted
images. However, calcifications are usually present inside the neoplastic mass, and
a broad dural base may also be observed. In addition, meningiomas may induce
hyperostosis of adjacent bone, while VSs may be associated with bone erosion.
Exceptionally, meningiomas can grow inside the IAC [18, 19], and in these cases,
the differential diagnosis can be obtained only during surgical removal [20–22].
Epidermoid cysts are isointense to cerebrospinal fluid in both T1- and T2-weighted
images, are not contrast enhancing, and do not extend in the IAC.

References
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TW, editor. UpToDate. Waltham: UpToDate. Accessed 14 Jan 2018.
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and skull base surgery. 1st ed. New York: Elsevier; 2017. xxx, 285 p.
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ial and sporadic vestibular schwannomas: implications for novel therapeutics. J Neurosurg.
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8. Chen M, Fan Z, Zheng X, Cao F, Wang L. Risk factors of acoustic neuroma: systematic review
and meta-analysis. Yonsei Med J. 2016;57(3):776–83.
9. Niemczyk K, Vaneecloo FM, Lecomte MH, Lejeune JP, Lemaitre L, Skarzyński H, et al.
Correlation between Ki-67 index and some clinical aspects of acoustic neuromas (vestibular
schwannomas). Otolaryngol Head Neck Surg. 2000;123(6):779–83.
10. Saito K, Kato M, Susaki N, Nagatani T, Nagasaka T, Yoshida J. Expression of Ki-67 antigen
and vascular endothelial growth factor in sporadic and neurofibromatosis type 2-associated
schwannomas. Clin Neuropathol. 2003;22(1):30–4.
11. Steinhart H, Triebswetter F, Wolf S, Gress H, Bohlender J, Iro H. Growth of sporadic ves-
tibular schwannomas correlates with Ki-67 proliferation index. Laryngorhinootologie.
2003;82(5):318–21.
12. Sughrue ME, Fung KM, Van Gompel JJ, Peterson JEG, Olson JJ. Congress of neurological
surgeons systematic review and evidence-based guidelines on pathological methods and prog-
nostic factors in vestibular schwannomas. Neurosurgery. 2018;82(2):E47–8.
13. Dunn IF, Bi WL, Erkmen K, Kadri PA, Hasan D, Tang CT, et al. Medial acoustic neuromas:
clinical and surgical implications. J Neurosurg. 2014;120(5):1095–104.
14. Mastronardi L, Cacciotti G, Roperto R, Di Scipio E, Tonelli MP, Carpineta E. Position and
course of facial nerve and postoperative facial nerve results in vestibular schwannoma micro-
surgery. World Neurosurg. 2016;94:174–80.
1 Introduction: Clinical and Radiological Diagnosis 11

15. Sameshima T, Morita A, Tanikawa R, Fukushima T, Friedman AH, Zenga F, et al. Evaluation
of variation in the course of the facial nerve, nerve adhesion to tumors, and postoperative facial
palsy in acoustic neuroma. J Neurol Surg B Skull Base. 2013;74(1):39–43.
16. Mastronardi L, Cacciotti G, Roperto R, DI Scipio E. Negative influence of preoperative tinni-
tus on hearing preservation in vestibular schwannoma surgery. J Neurosurg Sci. 2017. https://
doi.org/10.23736/S0390-5616.17.04187-X.
17. Wu H, Zhang L, Han D, Mao Y, Yang J, Wang Z, et al. Summary and consensus in 7th
International Conference on acoustic neuroma: an update for the management of sporadic
acoustic neuromas. World J Otorhinolaryngol Head Neck Surg. 2016;2(4):234–9.
18. Amato MC, Colli BO, Carlotti Junior CG, dos Santos AC, Féres MC, Neder L. Meningioma of
the internal auditory canal: case report. Arq Neuropsiquiatr. 2003;61(3A):659–62.
19. Watanabe K, Cobb MIH, Zomorodi AR, Cunningham CD, Nonaka Y, Satoh S, et al. Rare
lesions of the internal auditory canal. World Neurosurg. 2017;99:200–9.
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meningioma mimicking vestibular schwannoma. AJNR Am J Neuroradiol. 2002;23(9):1493–6.
21. Chae SW, Park MK. Meningioma mimicking vestibular schwannoma. Ear Nose Throat J.
2011;90(7):299–300.
22. Roos DE, Patel SG, Potter AE, Zacest AC. When is an acoustic neuroma not an acoustic neu-
roma? Pitfalls for radiosurgeons. J Med Imaging Radiat Oncol. 2015;59(4):474–9.
Treatment Options and Surgical
Indications 2
Luciano Mastronardi, Alberto Campione,
Raffaelino Roperto, Albert Sufianov,
and Takanori Fukushima

The management of vestibular schwannomas (VSs) is diverse and depends on char-


acteristics of both the patient and the tumor. Tumor size and growth pattern as well
as patient’s age, symptoms, and comorbidities determine the treatment of choice
among three main options: conservative therapy with watchful waiting, radiation
therapy (RT), and surgery.
The goal of modern management of VSs is to improve the quality of life and to
preserve the neurological functions while maintaining mortality and morbidity rates
as low as possible. The treatment strategy must also be weighed against the patient’s
desires and motivation so that the final approach is carefully individualized [1–3].

2.1 “Wait and Watch” (Fig. 2.1)

Watchful waiting consists of periodical MRI scans to monitor the size of the tumor.
Although different growth patterns can be observed in time [4, 5], the natural his-
tory of VSs may follow three different paths: no growth, slow growth (max 2 mm1/
year), and fast growth (≥3 mm/year). The scheme of scans to follow is not

1
As the thickness of the slices in MRI scans is 1 mm, anything smaller is attributable to interob-
server variation; this is the reason why the cutoffs to discriminate between slow and fast growth are
integers.
L. Mastronardi (*) · A. Campione · R. Roperto
Department of Neurosurgery, San Filippo Neri Hospital—ASLRoma1, Rome, Italy
e-mail: mastro@tin.it
A. Sufianov
Federal Centre of Neurosurgery, Tyumen, Russia
T. Fukushima
Division of Neurosurgery, Duke University Medical Center, Carolina Neuroscience Institute,
Raleigh, NC, USA
e-mail: Fukushima@carolinaneuroscience.com

© Springer Nature Switzerland AG 2019 13


L. Mastronardi et al. (eds.), Advances in Vestibular Schwannoma
Microneurosurgery, https://doi.org/10.1007/978-3-030-03167-1_2
14 L. Mastronardi et al.

Diagnosis: VS

Hearing Hearing not


preserved preserved

Large (>2 cm) or


Surgery Small (<2 cm)
Cystic

Stable Growing
Total Non-total and or Surgery
No Symptoms Symptomatic

Patient in good Debilitated


Follow-up WWa WW patient (ASA ≥4)
conditions

Surgery RTb Surgery RT

Fig. 2.1 Treatment strategies in vestibular schwannomas (VSs). The paths leading to watchful
waiting are emphasized so as to clarify its main indications. aWatchful waiting. bRadiation
therapy

standardized, and diverse ones have been proposed in different studies [4–7]. As a
general rule, annual imaging studies should be performed for the rest of the patient’s
life. On one hand, it has been argued that as many as 64% of VSs showed a growth
pattern that had remained uniform for the first 5 years of follow-up, thus confirming
a large predictability of the natural history of those tumors [5, 6]. Nevertheless,
cases of sudden tumor growth after over 10 years of follow-up have been reported
[5] and would have been missed in case of a time-limited watchful waiting.
This type of strategy mainly applies to incidentally diagnosed VSs with the fol-
lowing characteristics:

• Patient with nonfunctional hearing (AAO-HNS class C/D)


• Tumor size ≤2 cm at diagnosis [1, 4]
• Tumor growth rate max 2 mm/year
• No neurological symptoms due to trigeminal (NV), facial (NVII), or vestibulo-
cochlear (NVIII) nerve compression or hydrocephalus

As far as hearing function in concerned, some authors have proposed to extend


the watchful waiting strategy to intrameatal VSs in younger patient who still have
2 Treatment Options and Surgical Indications 15

serviceable hearing as these tumors show a higher tendency to remain stable over
time when compared to larger ones [5]. However, as the goal in the treatment of VSs
is to preserve neurological functions and, in addition, hearing deterioration can
occur even in non-growing tumors, small VSs in younger patients who still retain
hearing function should be treated with hearing preservation surgery [8].
As for the patient’s characteristics that have been proposed to encourage a watch-
ful waiting approach—being tumor size and growth rate constant and within the
aforementioned values, these are [1–3]:

• Age > 65 years


• Poor clinical conditions contraindicating any interventional strategy

Only a minority of patients eligible for watchful waiting still retain hearing func-
tions at the time of diagnosis. This may be due to the concurrent setting of presby-
cusis as they are mostly elderly. In terms of hearing preservation, 30–50% [6, 9] of
patients maintain hearing function during a 5-year watchful waiting follow-up.
The greatest concern about watchful waiting is the risk of injury to
NVII. According to recent studies [4, 5], a conservative strategy does not imply per
se a poor facial outcome. However, the possible increase of tumor size may lead to
poorer results in NVII preservation in case of surgical resection. This risk should be
weighed against the fact that, when the cohort is chosen accurately, watchful wait-
ing fails in a minority of cases, as reported in the series by the same authors [4, 5].

2.2 Radiation Therapy (Fig. 2.2)

RT protocols for the management of VSs differ in dose and radiation/particles


administered. There are three types of RT: stereotactic radiosurgery (SRS or Gamma
Knife), fractionated stereotactic radiation therapy (FSRT), and proton therapy (PT).
The common feature of these therapies is local control rather than tumor eradication
[1, 2, 10]. No studies have compared two or all the three modalities [11].
RT is indicated in case of [1, 2]:

• Patient with nonfunctional hearing (AAO-HNS class C/D)


• Tumor size <2 cm
• Tumor growth is ≥3 mm/year
• Poor clinical conditions contraindicating surgical intervention (ASA ≥ 4)

Older or comorbid patients may benefit from RT instead of surgery so as to avoid


the complications related to anesthesia and surgery itself (Fig. 2.2). However, RT
itself may lead to complications such as hearing loss, NV and NVII deficits, adhe-
sions, hydrocephalus, and potential (although very rare) malignant transformation
[1, 11]. Rare sequelae yet to be considered are delayed severe headache, severe
facial pain, and new motor deficits.
16 L. Mastronardi et al.

Diagnosis: VS

Hearing Hearing not


preserved preserved

Large (>2 cm) or


Surgery Small (<2 cm)
Cystic

Stable Growing
Total Non-total and or Surgery
No Symptoms Symptomatic

Patient in good Debilitated


Follow-up WWa WW patient (ASA ≥4)
conditions

Surgery RTb Surgery RT

Fig. 2.2 Treatment strategies in vestibular schwannomas (VSs). The paths leading to radiation
therapy are emphasized so as to clarify its main indications. aWatchful waiting. bRadiation therapy.
After “non-total” removal of tumor, retreatment could be necessary if the residue grows during
WW follow-up

Moreover, the treatment of debilitated (ASA ≥ 4) patients still remains contro-


versial. While for larger tumors a debulking surgery, i.e., intended not to be total,
may be the sole possible treatment strategy, small (<2 cm) tumors and—even
more—intrameatal ones may be eligible for watchful waiting. Nevertheless, many
studies have proposed RT for this kind of VSs as well, independently of the patient’s
hearing function. Recent systematic reviews [4, 5] have examined the role of RT in
the treatment of VSs and found that it is neither standardized nor immediately clear.
In fact, as a considerable portion of these tumors would remain stable or grow
slowly, the successes claimed of RT may have been at least partially facilitated by
the nature of the tumor itself. As a consequence, recent guidelines recommend that
intrameatal and small VSs without tinnitus be observed as observation does not
have a negative impact on tumor growth or hearing preservation compared to RT
treatment [11].
RT has an additional role in retreatment in case of failure of previous surgery or
RT protocol [1, 9–11]. However, in the perspective of multiple recurrences that
require surgical intervention, RT may pose problems in terms of NVII preservation
because of the numerous radiation-induced adhesions.
Another random document with
no related content on Scribd:
I had heard of people being made away with when out shooting,
but in my case it would hardly be possible. In the first place, I did not
shoot, having always had a disinclination to the brutal killing of
animals for the sake of pleasure. There remained as far as I could
see only the ordinary means of poisoning, with all their attendant
dangers.
An instantaneous poison would be most convenient, as it was
highly unlikely that I should have sufficient access to Lord Gascoyne
to deal with him slowly.
I rose in the morning with one scheme after another chasing itself
through my brain. Dressing rapidly, I went for a walk before breakfast
round the ancient battlements. These were quite a mile in
circumference, and the climbing of worn steps, hazardous scalings
of the walls from whence to get a better view, and a careful
examination of the various architectural designs of which the building
was constructed, occupied me very pleasantly for a full hour, and it
was half-past nine, the time for breakfast, when I turned to re-enter
the castle. As I descended some steps which led down to the
quadrangle, I was astonished to come face to face with a girl of
about nineteen or twenty and a child. She was obviously a lady, but
she had not been at the dinner-table the evening before, or in the
picture-gallery afterwards. I concluded that she was the little boy’s
governess, but who the child might be I could not imagine. I detected
at once that the girl was beautiful, and when I say I detected I use
the word advisedly, because it was not a beauty which would be
immediately appreciated. The gray eyes, oval face, threaded gold
hair, straight nose, and delicately-cut mouth were almost too frail to
impress the casual observer. I saw at once, however, that she was
rarely perfect in such a type of beauty as I always imagined Burns’
Mary must have possessed. She wore a quaint hood, almost like a
child’s, edged with some inexpensive gray fur. The little boy looked
at me shyly, and I held out my arms. A smile broke over his face and
he offered me his ball, inviting me to play with him. In less than a
minute we were all three laughing like old friends.
After having made myself sufficiently agreeable, I escaped from
the child, who was clamouring to me to continue the game, and ran
down the steps. I found Lady Gascoyne in sole possession of the
breakfast-table. “I am afraid we are the only early risers, Mr. Rank,”
she said.
As a matter of fact, I rather fancied I had caught a glimpse of
Lady Enid going along the road towards Hammerton woods, and I
think I was fairly correct in guessing that she was not walking at such
a rapid pace for the pleasure of her own company. Indeed, I would
not have minded taking long odds that Sir Cheveley was not very far
away.
I, of course, said nothing of this to Lady Gascoyne, but
mentioned my meeting with the girl in the gray hood.
Lady Gascoyne smiled.
“Now, isn’t she pretty, Mr. Rank?”
“Almost beautiful,” I hazarded. It is dangerous to be enthusiastic
about another woman, even to the nicest of her sex.
“That shows your good taste. Some people cannot see it.”
“It is the sort of beauty which is very rare, and not exactly showy.”
“She is to my mind wonderful. It does one good to look at her. I
expect you are wondering who the little boy is. It is a sad story. I had
a great school friend whose father was enormously rich. About a
year after her marriage he failed, and her husband’s fortune went in
the same crash. Her husband shot himself, and she died six months
later, leaving her little boy in my care. Oh!” she added quickly,
evidently afraid that my inward comment might be a disparagement
on her dead friend, “she did so at my express desire. We loved one
another so, that it was the most natural thing she could do.”
“He seems a dear little chap.”
“He is a darling, and devoted to Hammerton already. Lord
Gascoyne is so good about it, and lets me have him here always.
Miss Lane is his governess.”
Sir Cheveley Drummond, looking a little conscious, came in at
this moment.
“How energetic everybody is this morning,” said Lady Gascoyne.
Mr. Puttock, who appeared on the scene almost as she spoke,
looked anything but energetic. He looked as effete as only a
decadent young American can look. Lady Enid, who followed,
managed to convey with great art the impression that she had just
left her room. Lady Briardale did not appear, but Lady Branksome,
unable to trust Lady Enid, arrived in good time, although she
confided to me later that she thought breakfasting in public a
barbarous practice.
After breakfast, the whole party, with the exception of Sir
Cheveley Drummond, went to church. Poor Sir Cheveley hardly saw
the point of going when he knew perfectly well that he would not be
permitted to share Lady Enid’s hymn-book.
I distinctly heard Lady Briardale say, as we were all waiting in the
hall:
“I should have thought, my dear, that he would have preferred a
synagogue.”
At church a thrill passed through me when I found that I was
seated next to Esther Lane. It was an infinite pleasure to me to be
sitting beside her through the long and stupid sermon. The yellow
winter sunlight fell across the recumbent effigies of dead and gone
Gascoynes, and made the painted window to the east a blaze of
colour.
Her presence and the surroundings filled me with a sense of
purity and peace, and I surrendered myself to the primitive emotions.
I suppose a less subtle soul would have been oppressed with a
sense of past sins, and would in such a building have been filled with
despair at the consciousness of irrevocable guilt. I fortunately had
schooled myself to control. The sensation of goodness can, like
other things, be acquired. When I had obtained the prize for which I
was striving I had not the least doubt that I should find it easy to put
away from me any unworthy feeling of regret. Why should I not? The
harm was after all very questionable. It was not as if, so far, I had
made widows and orphans. The amount of suffering I had inflicted
was limited, and at any rate I should not leave poverty, the greatest
of all ills, in my track. Indeed, under the influence of that Sunday
morning service I felt quite regenerated. When we left the church
Esther Lane and her pupil went through the great gates of the castle
into the woods beyond, and I would have given worlds to follow her,
but Lady Branksome told me that she agreed with her son, and that I
was decidedly amusing. She insisted on my going for a walk with
her. At the same time she took good care to see that Mr. Puttock and
Lady Enid were close behind.
In the afternoon I manœuvred a meeting with Esther Lane. I
surmised that she and her pupil would walk away from the castle,
and so I kept watch on the drawbridge. Everybody was more or less
occupied. I was aware that Lady Branksome had, before retiring for
her afternoon nap, left Mr. Puttock in possession of Lady Enid, who
had got rid of him with all the ease imaginable, and was now walking
with Sir Cheveley on the battlements.
Lord Gascoyne had pleaded letters, and I had arranged to fetch
him in an hour or so and go for a tramp.
As Esther Lane and her pupil crossed the drawbridge I was
leaning over the extreme end of the parapet in the most natural
manner in the world. I pretended not to notice them, and only
permitted myself to be aroused from a contemplation of the beautiful
scenery which lay below by the child flinging his arms with a scream
of delight round my legs.
She blushed as she apologised for her charge. She was evidently
a little nervous as to what her employers might think if they saw her
walking with one of their guests, and, after a short interchange of
commonplaces, tried to get rid of me. I refused to be shaken off, and
a couple of hundred yards took us out of sight of the castle.
We dropped at once into a style of conversation which was
almost intimate, and although I was with her barely three quarters of
an hour she confided a great deal in me.
Not that there was much to confide. She was the daughter of a
solicitor who had left nothing but debts. Her mother was dead, and
she was absolutely alone in the world. Her only relations were some
very distant cousins who were so poor that it had been impossible
for them to help her in any way.
“I was very lucky,” she said, gratefully, “to get such a good
situation, and it came about in the quaintest way. Lady Gascoyne
had seen all sorts of people with diplomas and recommendations
which I had not got, and she saw me sitting in the waiting-room as
she passed out. I don’t think the agent quite liked her engaging me,
but Lady Gascoyne insisted that I was just the person she wanted,
and here I am.”
She smiled contentedly. She evidently considered herself an
extremely fortunate young woman.
She attracted me enormously without in any way usurping the
place of the two women who already counted for so much in my life.
All too soon I was obliged to leave her and hurry back to Lord
Gascoyne, whom I found waiting for me.
It was the first opportunity I had had of really impressing him, and
I did not waste my time. I took such an absorbing interest in
everything about me that I fancy he was surprised to find himself
talking so much and so intimately.
I was perfectly ready to enter into the subjects which interested
him most. He was evidently deeply imbued with a belief in the divine
right of aristocracy, and with no superficial sense of its
responsibilities. He was above all things a serious man, with little
sense of humour. I imagined that Lady Gascoyne must find him dull,
but his qualities were essentially those which command women’s
respect and hold them with a certain kind of fear.
As is the case with his class—a class which the popular organs
are fond of describing as irresponsible and brainless—his knowledge
and grasp of life were very extensive. His individual sympathies may
not have been very great, but he had a general sense of justice
which marked him out as an administrator, from many who were
perhaps much his intellectual superiors. What he knew was of use to
him.
I was surprised at my own capacity for conciliating him. I
accompanied him with ease into the region of politics. He was
evidently impressed, and it was satisfactory to feel him gradually
treating me with less and less formality.
“Your race gave us one of our greatest statesmen,” he said, “for I
do not believe that anyone has understood real statesmanship better
than Disraeli.”
“Some people seem to think he was insincere,” I replied, “but I
don’t believe it. His cynicism was simply the complement of a mind
with a singularly large outlook.”
So subtle an appreciation impressed the man whose own nature
was all in a straight line. The curves of a less direct character
appealed to him as insight.
In listening to him, however, I could not help reflecting that his
kind, whatever the moralist may pretend, is far more vulgar than the
abnormal. The orchid is the most fascinating of flowers in all its
varieties, but it is rare; or do we only call that type normal which
prevails for the moment? Which is the more moral man—he who by
reason of a lack of imagination ranges himself as the mercenary of
tradition and convention, or he who rebels and finds himself
wounded and struck at wherever he turns? Not that I can claim to be
a martyr to moral restlessness, although at one time I sincerely
believe I had in me the makings of a reformer. Nevertheless, there is
always something a little vulgar about the man who ranges himself
definitely on one side.
We returned to the castle on very good terms indeed. The rest of
the party were at tea in the long picture-gallery. I like, even at this
unpleasant crisis, to linger over the memory of the picture-gallery at
Hammerton on that Sunday afternoon in midwinter. The long,
straight windows through which the frosty sunset flushed the gilded
frames and old tapestries, the firelight playing on the silver of the
tea-table drawn up before Lady Gascoyne—for there were no
servants to desecrate the most convivial of all meals—made up a
delightful picture, whilst the child Walter Chard, in his sailor clothes,
ran from one group to the other as happy and unconscious as if he
had a prescriptive right to the enchantment of the castle.
I was delighted on our assembling for dinner to find that Esther
Lane was of the party. She was dressed simply in gray, with a couple
of blush roses at her bosom. Lady Enid was talking to her when I
came in.
It fell to my lot to take her in to dinner, and we thoroughly enjoyed
ourselves. She was quite unconscious amongst these great folk, and
unaffectedly joyous. I almost fancied that Lady Gascoyne looked at
me once or twice with the faintest sign of surprise. I sincerely hoped
she would not inform Miss Lane of the fact that I was engaged,
although it was more than probable. Esther Lane was one of those
women who are, to a certain extent, lacking in the natural defences
of their sex as a result of their own honesty and simplicity. That she
was prepared to be interested in me was obvious, and I made the
most of my time. After dinner she played to us. It was not a brilliant
performance, but she was accurate and had feeling, and she
touched the keys wooingly and caressingly, making the piano sing, a
gift rare even among some so-called finished performers. If people
cannot make instruments sing they had better leave them alone.
Afterwards I played and sang, and she declared herself ashamed of
her own performance. Music had the advantage of giving us an
excuse for remaining at the piano together, and later, when
everybody else settled down to cards some way off, we were left
trying over one song after the other. When I murmured that it
seemed as if we had known each other all our lives she blushed.
Later, in the smoking-room, Sir Cheveley said he was quite
astonished to find how pretty she was. It was a fact which he
declared had grown on him gradually.
The next morning I returned to town, but I had made such good
use of my time that I carried with me an invitation to return in a
fortnight, and it was Lord Gascoyne who brought it me from his wife.
He had made quite a friend of me. It was not probable that he
had ever had a friend with anything of the bizarre about him before.
Chapter XXII
The time passed slowly, but in a fortnight I found myself again at
Hammerton sleeping beneath the same roof as Esther Lane. I met
her again on the terrace on Sunday morning, as I had expected. I
could quite follow the workings of her mind. Because she was
possessed of great self-respect, she had determined not to be on the
terrace that morning, but because she was very much in love she
was there after all. Before many moments had passed I saw that she
was aware of my engagement. There was a look of suffering in her
eyes as she turned them on me. She had the most wonderful way of
suddenly subjecting the person to whom she was speaking to their
full glance. If she could suffer because I was engaged to another
woman, she had enough sentimental interest in me to excuse my
going far. She was the character to appreciate the simulation of
agony born of a struggle between duty and affection, and I was
ready to ring up the curtain on the comedy. She was fully conscious
that I loved her. Yes, I loved her quite passionately, and yet it was not
altogether passion. It is one of the presumptuous platitudes of
conventional moralists to describe a man’s love when it ceases to be
concentrated on one individual as lust and base passion. Side by
side with this contention they will declare that the highest morality is
to love your neighbour as yourself, so little are they given to their
own boasted virtue of consistency.
I talked to her for some time about the most ordinary matters, but
I could see that she was trembling.
“I have thought of you a good deal,” I ventured.
She ignored the question, but without a show of indifference.
“I think Walter and I ought to go in.” She moved away a little
awkwardly.
“I suppose I ought to go in too.” I went to breakfast, leaving the
impression I had intended.
In the afternoon I saw her again, and before I was quite aware of
it, I had told her I loved her beyond all women in the world, and
having done so was compelled to anticipate the scene which I had
been mentally preparing.
I assured her passionately that I was not the fickle person I might
seem to be, that directly I saw her I knew that I had made a mistake,
and that I could never be happy with anyone but her.
She was too much in love to do more than make a pretence of
forbidding me to speak on the subject.
She walked straight into the carefully-hidden trap, and found
herself being made love to without the question of my engagement
being discussed, and once she had thrown down the barriers of
reserve, with the enemy actually in her camp, it was impossible to
replace them. She accused herself vehemently, however, of being
unworthy of her trust. She would resign her position. She could
never, she asserted, remain, and be guilty of duplicity. I beat down
her poor little attempts at self-defence at once. If she threw up such
a position I should never forgive myself. I would go away and never
see her again. The threat appeared to terrify her. I persisted that the
view of such affairs taken by most people was entirely wrong. I
showed that, on the contrary, it was wrong to lie and say you do not
love a person when you do, that it was quite possible to talk of our
love, to cherish it, and to welcome its influences for good, without
allowing it to get the upper hand. All of which the poor fluttering little
morsel of sentiment drank in with greedy ears, because it was just
what she wanted to believe.
The child found us dull, and cried out that he wanted to play, so I
left her and returned to keep an appointment with Lord Gascoyne.
Esther Lane was included in the dinner-party that evening, and I
again took her in. The arrangement was not so pleasant as on the
previous occasion, for there was a distinct feeling of strain between
us. I surmounted it with ease; but she was without experience, and
she suffered. I could see that she had been crying.
“You must not let yourself be wretched,” I said, as we stood a little
apart from the others waiting for the move towards the dining-room.
“It hurts me.”
“I had intended to remain in my room,” she murmured.
“Why?”
“I feel as if everyone must see that I am an impostor.”
I was about to reply lightly, but checked myself, remembering that
the heroine of the comedy was an ingénue.
“If you were not so good you would not have a sense of guilt
where there is no guilt.”
We went in to dinner.
I talked to her incessantly, and inasmuch as her happiness lay in
being with me, she was prepared to be charmed out of her misery for
however brief a period.
I was myself somewhat astonished at the hold I had secured over
her so soon. I suppose in her inmost heart she was dreaming
dreams in which all would come right. But even if I were safely
ensconced as Earl Gascoyne I could not have made such a sacrifice
as to marry her. Besides, in the sense of a mate, to take my name
and reign with me, I would not have changed Edith Gascoyne for
anyone in the world, not even for Sibella.
There is among modern English ballads one which has always
struck me as having a claim to live because of its simplicity and the
heart-throb in it. It is called ‘For ever and for ever.’ It possesses a
perfect blending of music and idea, unpretentious, but full of feeling.
As before, we spent the evening at the piano, and I sang this
song to her almost under my breath:

“I would, alas! it were not so


For ever and for ever.”

I could see that the tears were raining down her cheeks as she
listened. She held a fan so as to conceal her face from the others in
the room.
“We shall see each other again soon,” I murmured, as I said
good-night.
Lord Gascoyne and I were the last to leave the smoking-room,
and he parted from me at the foot of the stairs that led to the
bachelors’ quarters. My bedroom was half-way down a long corridor,
at the end of which there was a solid door, which gave on to the
battlements. It was bolted inside, but not locked, and I had more than
once used it to take an evening stroll when the inhabitants of
Hammerton Castle were asleep. This evening I opened the door
noiselessly and walked out on to the walls. It was a clear starlight
night and bitterly cold. I did not mind this, for I had on a thick fur coat.
I strolled along, thinking deeply, when suddenly I was brought to a
standstill by a ray of light that fell right across my path.
I looked in the direction from whence it came, and was
astonished to see Esther Lane leaning out of a window a few feet
from me. The terrace at this point took an abrupt turn, and a
comparatively new part of the castle had been built out at a tangent.
She had not noticed my approach, for I wore house-shoes, which
made no noise.
She was looking at the stars as if their ceaseless splendour might
be symbolic of an inevitable dawn of happiness somewhere. They
could not have been completely reassuring, for she was weeping,
and as I stood and watched, a convulsive sob broke from her. The
picture of the forlorn little dependent, a frail white figure in the patch
of light, with the gloomy towers and battlements of Hammerton
looming round her, affected me strangely. I leant forward over the
low wall and murmured her name.
“Esther!”
She started and looked round, drawing back quickly as she saw
me clearly defined in the moonlight.
“You must not cry. It breaks my heart.”
At the moment I fully believed what I said.
Her eyes, full of tears, were turned upon me, and, with a strange
look of fear, which haunts me to this hour, she said:
“I thought I should never see you again.”
“You were going away?”
She saw that she had betrayed her intention, and tried to excuse
herself.
“It will be better.”
“Why? If you go away I shall never come here again.”
I was upon the low wall, the ground full sixty feet below me.
“Oh, go back! you will fall.”
But I had my hand on her window-sill and one foot on a ledge a
short distance below it, whilst the other remained on the wall. She
was helpless; to have attempted to stay me would have been to
send me in all probability to certain death. She clasped her hands
and held her breath. The next moment I was in the room and by her
side.
“Don’t be afraid; only I must speak to you. We must understand
each other.”
“Oh, go away, please.”
She hid her face in her hands, utterly shamed by the presence of
a man in her room.
Poor Esther! I think she was happier. I verily believe that every
woman is happier for love fulfilled. I knew that once having chosen
her path she would follow it unflinchingly, and that she would be true
as steel. I had discerned from the first that she was capable of
martyrdom. From that day she never mentioned the word marriage.
She declared ever afterwards that it was her fault, that she should
have closed her window on me, that she had accepted the position
of mistress, and that she could not complain.
At the same time, however, it was no easy task to persuade her
to remain at Hammerton. She implored me to let her come away to
London. She vowed that she would not be an encumbrance, not
even an expense. She was sure that she could get work to do,
sufficient to keep herself; but I was firm. I had at one time some idea
of letting her live at Clapham in my deserted house, but I had always
had a superstition about allowing anyone else to live in it, otherwise I
should have sold it long before. Besides, I did not see what excuse
she was to give Lady Gascoyne for wishing to leave her, and the
latter had grown so fond of her that it was not likely she would accept
her resignation without a great deal of inquiry. Esther declared that
living a lie made her feel miserable, that she was unworthy of her
charge, and ought to resign it.
Chapter XXIII
I had still made no plans as regards Lord Gascoyne and his heir.
I must confess that I had qualms about the child, which shows how
illogical and unreasoning sentiment is. It is surely a greater crime to
kill a grown individual who has a place in the practical work of the
world, and who would be missed by hundreds, than to remove an
infant whose loss could only affect his parents. It was not easy for
me to inflict pain on a child, as my fondness for children is
exceptional. The means would have to be sudden and violent, and
they were difficult to think of. It might have been made to appear that
his death had been caused through some apparent negligence of the
nurse, had not the latter been a careful old lady through whose
hands two former generations of the Hammertons had passed. I had,
unperceived, followed her in her walks with the Gascoyne heir, and I
was afraid it would be impossible to find her careless.
I was prepared to make away with father or child first; whichever
event came most readily to hand would have to take place. A novice
might have been impatient, but experience had taught me that one
had only to watch and wait, and the opportunity would come at the
most unexpected moment.
It was nearly time for Miss Gascoyne and her uncle and aunt to
return to town. I was wondering whether they would be asked down
with me to Hammerton. It would be somewhat awkward, but I had
infinite belief in my own powers of dissimulation and tact.
In the meantime I concentrated my thoughts on little Lord
Hammerton. I had read of a child being smothered by a cat going to
sleep over its mouth. For a time the somewhat impracticable idea
possessed me of obtaining a gutta-percha baby, which I would fill
with hot water and arrange so that it could breathe mechanically, and
then train a cat to lie upon it. The weirdness and humour of the idea
commended itself to me. The far-fetched nature of the scheme,
however, became more apparent the more I considered it.
I thought I might become a proficient with the catapult, and aim at
the child unseen. In such a case the blame would probably fall on a
village boy. This, too, seemed rather far-fetched.
I had seen Hammerton’s nursery, or rather nurseries. Even a
child of his rank seldom has such a suite of apartments:—a night
and day nursery of lofty proportions, with rooms for the head-nurse
and her assistant opening off. It was the size and loftiness of the
rooms that were exceptional. Lady Gascoyne was a great enthusiast
on hygiene, and declared that fresh air was the best food a child
could have. In fact, I was not likely to be spared my unpleasant task
through any neglect of the little Viscount’s health.
My objection to inflicting pain upon a child gradually grew weaker.
Something had to be done.
I was seriously considering the matter when Providence put a
weapon into my hand.
I arrived one Saturday evening to find Lady Gascoyne somewhat
uneasy. Walter Chard was not well. Something quite trifling, no
doubt, but he was feverish.
I asked if I might go and see him, but Lady Gascoyne said that
she would rather I did not. It was always difficult to say how these
childish ailments would develop. It might be something infectious,
and she had Hammerton to think of. If anything happened to him
Lord Gascoyne would be broken-hearted.
“Miss Lane is with him,” went on Lady Gascoyne. “She absolutely
declines to allow anyone else to nurse him.”
I wondered whether Esther Lane had known that her undertaking
to nurse the child would prevent her from seeing me. For the
moment I was a little annoyed, as even the most supercilious man
will be when he imagines the woman he is thinking of very much at
the moment has found a duty which she places above her love.

‘I could not love thee, dear, so much,


Loved I not honour more,’
is very trash in the case of both sexes. People who say they cannot
love where they cannot respect are talking nonsense. Affection is
independent of and survives prejudice, which I should surmise is an
added terror in the lives of good people.
“You are not afraid of infection, are you?” asked Lady Gascoyne.
“Oh dear no, but what makes you think it is something
infectious?”
“I don’t know. I had a sort of instinct that it was so directly I looked
at Walter.”
I thought nothing more of Walter Chard during my visit except to
ask how he was.
“The doctor says he cannot decide for a few hours what is the
matter with him,” replied Lady Gascoyne.
On Monday morning, however, the Castle was thrown into a state
of the greatest consternation. The doctor pronounced him to be
suffering from scarlet fever, and Lord and Lady Gascoyne were filled
with alarm for the safety of their own child.
Walter Chard had been constantly with him, and any moment
little Lord Hammerton might develop the complaint.
The sick child was immediately secluded at the extreme end of
the castle, and a trained nurse was sent for. From the moment I
heard that it was scarlet fever my mind was at work. Supposing
Hammerton did not develop the complaint, would it be possible to
convey the infection to him?
If I could get near Walter Chard in the convalescent stage it
would be easy enough.
Of course, it did not follow that even if Lord Hammerton took the
infection he would die, but there was the chance that he might.
Lady Gascoyne talked of removing him from the castle at once,
but the doctor decided that it was not necessary.
“Our patient is too far away to do any mischief. He is for practical
purposes quite isolated, and if you disinfect the rooms he has been
living in you need not be in the least alarmed.”
On my return to town I waited a week, and then wrote to Lady
Gascoyne, saying that I hoped Lord Hammerton showed no signs of
having caught the infection. I displayed so exactly the right amount
of solicitude that on meeting Lord Gascoyne in town he greeted me,
for him, almost effusively.
“My wife was quite touched, Rank. Naturally, we were alarmed,
but I fancy it’s all right. The other little chap will soon be on the road
to recovery. Come down with me to-morrow if you are not afraid.”
It was exactly what I wanted. The chance might not again offer
itself to carry out my design. I met Lord Gascoyne at Waterloo, and
we travelled down together. It was the sort of travelling that suited
me to perfection; a saloon carriage had been reserved for us, and all
along the line we were treated like royalty.
I had been a little surprised that when he and Lady Gascoyne
attended anything in the shape of a function in the neighbourhood of
Hammerton they rode in a chariot with outriders. The effect was
singularly picturesque, and appealed to my Jewish love of colour. I
determined that if ever I succeeded I would retain the custom.
When we reached Hammerton we found Lady Gascoyne at the
station, quite radiant. Notwithstanding that the patient was at the
most infectious stage, there was very little danger, owing to the
complete way in which he had been isolated.
I was the only guest, and we retired early. I had, of course, seen
nothing of Esther Lane. As I walked on the terrace and smoked my
cigar I wondered if she were thinking of me. I paused at a spot which
was at the extreme end of one of the horns of a semicircle. At the
end opposite to me were the rooms in which she was nursing her
invalid. I stood looking at them thoughtfully. I was thinking of what
Lady Gascoyne had said as to the disease now being at its most
infectious stage. Contact, or the use of the same article, such as a
towel or a handkerchief, might convey the infection. I might be doing
something to advance my cause instead of idly puffing at my cigar.
The invalid’s rooms were several hundred feet away, and to reach
them I should have to scale a parapet some ten feet in height, but it
was no more than I could manage. I walked slowly round the
semicircle. The night was strangely mild, and it was not improbable
that the windows would be open.
I knew the apartments. They had once been occupied by a mad
Earl Gascoyne, and had in his day been securely barred.
These bars had, however, long since been removed, and I
remembered thinking the rooms almost the pleasantest in the castle.
They were some distance from the rest of the living part. When I
reached the terrace I saw that there was a narrow stone staircase
which I had not before noticed. At the top of this was an iron gate
which would have to be climbed. Just then I heard steps on the
terrace above me, and I had hardly time to draw into the shadow of a
buttress when Esther Lane appeared, leaning over the low wall
above my head. I threw down my cigar and stamped on it, for it
might have been its scent borne on the night air which had attracted
her.
She looked about her for a few seconds and went back. I waited
for an hour or so until the lights were turned down for the night. I
wondered which of the two women was watching by the bedside of
the invalid.
After a time I ascended the staircase and surveyed the iron gate
at the top. It looked rickety, and it would be difficult to climb over it
without making a noise. If the trained nurse found me it would not be
easy to explain my presence.
I climbed over the gate and dropped on to the other side. In the
still night air it rattled horribly, but no one seemed to have heard. I
had on house-shoes, and stole cautiously forward. I knew that the
temporary hospital was a large room with three windows opening to
the ground. Danger being almost out of the question here, the
windows had been left partly open. I stole cautiously forward and
looked in. By the dim light I saw the sick child lying on a bed well out
in the middle of the room. On a long, low chair by the fire lay Esther
Lane, in a white dressing-gown. I thought she looked exceedingly
beautiful, if a little worn with watching. Crossing to the window
furthest from where she lay, and having quite assured myself that
she was asleep, I entered cautiously and looked round. I went first to
the door that I knew gave on to the corridor. Luckily, the key was on
the inside, and I locked it. There was another door leading to an
inner room, which made me a little anxious, as I concluded that it
was occupied by the nurse. It had no key, and I quietly placed a chair
in front of it. Noiselessly I stole towards the bed. The child’s flushed
face was resting on his hand, in which was clasped a handkerchief.
This was the very thing I wanted, and with infinite care I managed to
unclasp the little fingers and draw it forth without waking him.
Wrapping it in my own handkerchief, I thrust them both into my
pocket.
I had just finished and was turning away when I became aware
that Esther Lane’s eyes were slowly opening upon me.
Luckily she did not cry out, but rose quickly with a half-smothered
exclamation. As I went swiftly but silently to her I heard the boy stir in
the bed behind me, and we both stood waiting in suspense to see if
he would wake. Luckily he only murmured once or twice, and sank to
sleep again. I was near the light, however, and ready to extinguish it
at once should he show the least sign of waking. When his regular
breathing had reassured me I drew her on to the terrace.
“I was obliged to come,” I murmured passionately. “I had to see
you.”
“You should not have done it,” she said helplessly.—“you should
not have done it.”
“I am very sorry.”
I took my cue and passed into the mood of the reckless, love-sick
youth. I knew that to feel a passion is the only way to be convincing.
“It is dangerous, and most dangerous now.” She began to weep;
she was evidently unstrung from watching. I drew her away into a
sheltered corner of the battlements, where the moon made it almost
as light as day.
I told her that I was only happy when I was with her, and that all
my days in London were given up to thinking of her. The poor child
believed me. It appeared that she had been attempting to make
some expiation for what she considered the betrayal of Lady
Gascoyne’s confidence by her devotion to Walter Chard during his
illness.
I held her in my arms, and we remained perfectly happy and in
silence for a long time. She certainly stirred my blood to an
extraordinary degree.
She was terrified lest my visit should convey infection to the rest
of the household. I reassured her. At the same time, her having seen
me was very awkward. Hers was a character which, impelled by
conscience, might be capable of all sorts of extraordinary things,
great renunciations and burning sacrifices. I held out a vague
promise of taking her to London and allowing her to a certain extent
to share in my life. This filled her with a guilty delight, and she clung
to me with a sigh. Love in its fierce, personal aspect meant more to
her than to any woman I have ever met.
The poor little soul went back and procured some strong stuff
with which she instructed me to disinfect my clothing.
I returned to my room almost laughing with glee at having the
handkerchief from the sick boy’s bed with me.
When I reached my bedroom I packed the two handkerchiefs
carefully up in several layers of paper and then went to bed. Of
course, I had to reckon with the fact that I might take the infection,
but I will do myself the justice to say that at no time during my career
have I been much affected by a consideration for my own safety. It
has always appeared to me that an absolute disregard for my life
was the only method on which to proceed.
The next morning I excused myself from accompanying Lord and
Lady Gascoyne to church. Lady Gascoyne quite sympathised with
me.
“A gallop will do you much more good.”
I thanked her, but said I preferred a tramp.
As soon as they had walked over to the church I made my way to
Lord Hammerton’s nursery. I was no stranger to it, and was a
favourite with the nurses. The little viscount knew me quite well, and
crowed directly I appeared.
Old Mrs. Howick looked at me indulgently as I took the child in
my arms. After a time she went into the inner room, leaving me in
possession for a few minutes. I hastily took out of my pocket the
handkerchief, which was still wrapped in my own, and let it fall
across the child’s face. He clutched it with two little fat hands, and I
allowed him to play with it, and then thrust it in between his frock and
his chest and left it there. I had previously taken good care to
ascertain that it was unmarked, and was not likely to be recognised.
When the nurse came back I was still playing with the child, and
she took him from me and dressed him for his morning’s outing
without discovering the presence of the handkerchief.
I then went for a long tramp through the woods, laughing at
myself for what I could not help feeling to be a piece of absurdity. On
my return to the house I went again to the nursery. The little viscount
was asleep, and I managed to extract the handkerchief and lay it
where the air he was breathing must pass over it. Then I removed it,
and congratulated myself that I had left no foolish clue whereby my
action might be identified. Mrs. Howick was delighted at my
attentions, and told Lady Gascoyne that she never had seen his
lordship take to anyone as he had taken to me. Inwardly I hoped that
he might take to the infection with even greater ardour.
There was still the question of Esther Lane to be dealt with, if it
were possible to deal with it at all. If the child should fall ill she would
be sure to think of me and find out whether I had been to visit him.
As is always the case, my romantic susceptibilities had landed me in
a position of danger which all my other intrigues had not done. If
Esther Lane should tell the truth, my entire house of cards would fall
to the ground. The telling of the truth would involve a general
exposure. It would mean farewell to Miss Gascoyne, and also the
shutting of the gates of Hammerton Castle in my face, which would
be a far more fatal obstacle to my success.
I did not bless the day on which I had met Esther Lane, and
further cursed my own weakness. This is a habit of men when they
have obtained their desire.
I should have remembered the dictum of a celebrated
countryman of my own, which prophesies material doom for those
who are unable to control their affections. He should perhaps have
substituted another word for affections.
There were no measures for self-protection to be taken as far as I
could see. My strongest card was Esther’s love for me, and if that
were not strong enough to prevent her betraying me, nothing would.
I had wit enough to see that it was likely to prove a still stronger
weapon if I kept away from her.
I waited some days, and read one morning in a halfpenny paper
which deals in such tittle-tattle that Lord and Lady Gascoyne were in
the greatest anxiety about their only child, the heir to the title, who
was suffering from a severe attack of scarlet fever.

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