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Operative Management of
VASCULAR
AN O M ALIES

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Operative Management of
VASCULAR
AN O M ALIES
Edited by
Arin K. Greene, MD, MMSc
Department o Plastic and Oral Surgery,
Boston Children’s Hospital;
Associate Pro essor o Surgery,
Harvard Medical School,
Boston, Massachusetts

Executive Editor: Sue Hodgson
Managing Editor: Elizabeth Palum bo
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Library of Congress Cataloging-in-Publication Data
Name: Greene, Arin K., author.
Title: Operative m anagement of vascular anom alies / Arin Greene,
MD, MMSc, Department of Plastic and Oral Surgery, Lymphedema
Program , Boston Children’s Hospital, Harvard Medical School,
Boston, Massachusetts.
Description: New York : Thieme, [2017]
Identi ers: LCCN 2016056385| ISBN 9781626236905 (print) |
ISBN 9781626237681 (e-book)
Subjects: LCSH: Blood-vessels--Surgery. | Blood-vessels--Diseases--
Treatment.
Classi cation: LCC RD598.5 .G74 2017 | DDC 617.4/13--dc23 LC
record available at
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v
DeDic at io n
T is book is dedicated to patients with vascular anomalies;
it is my hope that this shared knowledge
will improve their quality of life.

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vii
c o n t r ibu t o r s
Steven J. Fishman, MD
President, Physicians’ Organization; Senior Vice-
President, Access and Business Services; Stuart
and Jane Weitzman Family Chair; Vice-Chair o
Surgery, Clinical Operations; Co-Director, Vascular
Anomalies Center, Boston Children’s Hospital;
Pro essor o Surgery, Harvard Medical School,
Boston, Massachusetts
Arin K. Greene, MD, MMSc
Department o Plastic and Oral Surgery, Boston
Children’s Hospital; Associate Pro essor o Surgery,
Harvard Medical School, Boston, Massachusetts
Aladdin H. Hassanein, MD, MMSc
Fellow, Reconstructive Microsurgery, Department
o Plastic Surgery, Johns Hopkins University
School o Medicine, Baltimore, Maryland
Daniel Hedequist, MD
Orthopedic Surgeon, Boston Children’s Hospital;
Associate Pro essor o Orthopedic Surgery,
Harvard Medical School, Boston, Massachusetts
Bonnie L. Padwa, DMD, MD
Oral Surgeon-in-Chie , Section o Oral and
Maxillo acial Surgery, Boston Children’s Hospital;
Associate Pro essor in Oral and Maxillo acial
Surgery, Harvard School o Dental Medicine,
Boston, Massachusetts
Reza Rahbar, DMD, MD
Associate Otolaryngologist-in-Chie , Director o
Center or Airway Disorders, and Co-Director,
Head, Neck and Skull Base Surgery Program,
Boston Children’s Hospital; Associate Pro essor
o Otology and Laryngology, Harvard Medical
School, Boston, Massachusetts
Cory M. Resnick, MD, DMD
Attending Surgeon, Boston Children’s Hospital,
Boston, Massachusetts
Edward R. Smith, MD
Director, Pediatric Cerebrovascular Neurosurgery,
Co-Director, Cerebrovascular Surgery and
Interventions Center, Co-Director, Head, Neck
and Skull Base Surgery Program, and Associate
in Neurosurgery, Boston Children’s Hospital;
Associate Pro essor, Harvard Medical School,
Boston, Massachusetts
Samantha A. Spencer, MD
Orthopedic Surgeon, Boston Children’s Hospital;
Instructor in Orthopedic Surgery, Harvard Medical
School, Boston, Massachusetts
Amir H. Taghinia, MD
Attending Surgeon, Boston Children’s Hospital;
Assistant Pro essor o Surgery, Harvard Medical
School, Boston, Massachusetts
Cameron C. Trenor III, MD
Co-Director, Cerebrovascular Disorders and
Stroke Program, and Director o Clinical Research,
Vascular Anomalies Center, Boston Children’s
Hospital; Assistant Pro essor o Pediatrics, Harvard
Medical School, Boston Massachusetts
Joseph Upton, MD
Senior Associate in Surgery, Boston Children’s
Hospital; Associate Pro essor, Harvard Medical
School, Boston, Massachusetts
Ryan D. Walker, MD
Pediatric Otolaryngologist, Advanced EN ,
Voorhees, New Jersey

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ix
Pr efa c e
Vascular anomalies a ect approximately 5% o the population and can cause signi cant morbidity.
T e use o incorrect terminology and treatment has been decreasing because o continuing edu-
cation, as well as the creation o vascular anomalies centers in many academic institutions. T e
eld remains highly specialized, and patients with signi cant problems are usually concentrated
among large programs.
T e primary stimulus or this book resulted rom the types o questions I am asked when giving
lectures or advice to colleagues about vascular anomalies. Inquiries are less commonly about di-
agnosis and general treatment; they are more likely to be speci c questions regarding operative
management: “What are your surgical indications?” “When do you intervene?” “How do you
per orm the procedure?” “How do you reduce blood loss?” “Where do you place your incision?”
“What sutures do you use?” “How do you manage the patient postoperatively?” and “What are
the complications and how do you treat them?” Because current literature does not adequately
answer these questions, I realized the need or a “how-to” re erence or surgeons.
Another motivation or this text is that surgical approaches to vascular anomalies are unique and
of en cannot be translated rom the procedures described or other types o lesions. For example,
operative techniques or lip conditions usually describe vertical extirpations o malignant lesions.
By contrast, benign labial vascular anomalies do not require complete resection and can be re-
moved using di erent methods, such as transverse mucosal excision.
T is book is designed to have as much comprehensive, detailed in ormation as possible; it contains
abundant case examples with clear images to highlight important concepts, boxes and tables to
summarize key points, and videos to support the techniques described. T e book is also o ered
in ebook ormat so surgeons can access this content wherever they are.
T e contributors were chosen because o their expertise on the topic and have generously shared
their knowledge on a range o speci cs. Although the text is ocused on surgical treatment o vas-
cular anomalies, operative intervention cannot be considered unless the surgeon understands the
pathophysiology o the lesion and all available treatment options; this ramework is presented at
the beginning o the book. Chapters are organized based on the biologic classi cation o vascular
anomalies—tumors or mal ormations. T e last section o the text addresses speci c considerations
or vascular anomalies a ecting di erent organ systems.
In addition to serving as a roadmap or operative treatment o vascular anomalies, my hope is
that the book can be used as a source o in ormation or more in-depth study o the subject,
teaching, and/or research. Ideally, it will stimulate readers to innovate and improve our operative
approaches to these lesions. In the uture, I hope this text will no longer be needed as we replace
surgical treatment o vascular anomalies with pharmacotherapy.

x
Preface
ACKNOWLEDGMENTS
John Mulliken taught me the oundation or the operative management o vascular anomalies.
Steven Fishman has been a mentor in this eld. John Meara has given me pro essional support.
My gratitude to the contributors who shared their expertise in these pages. Executive Editor Sue
Hodgson and the T ieme team did an outstanding job o putting the book together. And nally, I
am grate ul to my wi e, Sarah, and three sons, Bert, Mac, and Henry, who encourage my passion
to improve the lives o patients with vascular anomalies.
Arin K. Greene

xi
Part I Introduction
1 Terminology and Classification 3
Arin K. Greene
2 Principles of Management 9
Arin K. Greene
3 Perioperative Hematologic Management 15
Cameron C. renor III
Part II Vascular Tumors
4 Infantile Hemangioma 25
Arin K. Greene
5 Pyogenic Granuloma 65
Arin K. Greene
6 Rare Vascular Tumors 77
Arin K. Greene
Part III Vascular Malformations
7 Capillary Malformations 95
Arin K. Greene
8 Lymphatic Malformations 109
Arin K. Greene
c o n t en t s

Contents
xii
9 Venous Malformations 135
Arin K. Greene
10 Arteriovenous Malformations 155
Arin K. Greene
Part IV Anatomic Considerations
11 Central Nervous System 183
Edward R. Smith
12 Craniofacial Bones 193
Arin K. Greene, Cory M. Resnick, Bonnie L. Padwa
13 Oral Cavity and Airway 205
Ryan D. Walker, Reza Rahbar
14 Chest, Abdomen, and Genitalia 215
Aladdin H. Hassanein, Steven J. Fishman
15 Hand and Upper Extremity 225
Amir H. aghinia, Joseph Upton
16 Vertebral Column and Lower Extremity 237
Samantha A. Spencer, Arin K. Greene, Daniel Hedequist
Credits 253

Index 255

xiii
ViDeo s
4-1 Infantile Hemangioma
Arin K. Greene
5-1 Pyogenic Granuloma
Arin K. Greene
6-1 Rare Tumor
Arin K. Greene
7-1 Capillary Malformation
Arin K. Greene
8-1 Lymphathic Malformation
Arin K. Greene
9-1 Venous Malformation
Arin K. Greene
10-1 Arteriovenous Malformation
Arin K. Greene
12-1 Craniofacial Bones
Cory M. Resnick
16-1 Intraarticular Venous Malformation
Samantha A. Spencer

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Operative Management of
VASCULAR
AN O M ALIES

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Par t I
In t r o d u c t io n

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Vascular anomalies are common, a ecting approximately 5% o the population. Lesions a ect
all components o the vasculature ( or example, capillaries, arteries, veins, and lymphatics) and
can occur in any anatomic area. Because the integument is the most commonly involved organ,
patients most requently present to a plastic surgeon or dermatologist. Although some lesions
( or example, in antile hemangioma, pyogenic granuloma, and capillary mal ormation) can be
managed by a single physician who is ocused on this eld, many types o lesions ( or example,
venous mal ormation, lymphatic mal ormation, and arteriovenous mal ormation) require inter-
disciplinary care. Patients with vascular anomalies pervade most medical and surgical specialties.
Academic medical centers o en have vascular anomalies centers where patients with complex
lesions can be managed.
Historically, the eld has been handicapped because the diagnosis and treatment o vascular
anomalies are di cult. Physicians are o en intimidated by the subject matter, because lesions
have a similar appearance, and the terminology used to describe vascular anomalies has been
Arin K. Greene
Ter min o l o g y
an d Cl as sif ic at io n
Ch a Pt er 1
KEY POINTS
• Correct biologic terminology should be used to describe vascular anomalies.
• Vascular anomalies are divided into tumors and malformations.
• Four types of tumors and four types of malformations constitute approximately 95% of
lesions.
• Approximately 90% of vascular anomalies can be diagnosed by assessing the patient’s his-
tory and the physical examination.
3

4
Part I: Introduction
imprecise. T e topic is urther complicated because the type o intervention is commonly based
on the pre erence o the treating physician.
Be ore the surgeon determines the management o a patient with a vascular anomaly, the lesion
must be accurately diagnosed with the correct current terminology. Surgical management o an
individual with a vascular anomaly is not considered, unless the surgeon understands the patho-
physiology o the lesion and available treatment options. T is chapter serves as a ramework or
the role o surgical treatment o vascular anomalies.
TERMINOLOGY
Previously, vascular anomalies were labeled according to the type o ood they resembled ( or
example, cherry, strawberry, and port-wine). Capillary or strawberry hemangiomas became as-
sociated with a red in antile hemangioma that involved the skin. I an in antile hemangioma was
located below the integument and appeared bluish, it was o en called a cavernous hemangioma.
T e terms capillary and cavernous were also used to describe capillary mal ormation and venous
mal ormation, respectively. Another label or capillary mal ormation was port-wine stain. Cystic
hygroma and lymphangioma became terms or macrocystic lymphatic mal ormation and micro-
cystic lymphatic mal ormation, respectively. Hemangioma continues to be used to describe any
type o vascular anomaly.
In 1982 Mulliken and Glowacki clari ed the eld o vascular anomalies by creating a biologic
classi cation o these lesions. T ey reserved the su x -oma (increased cell division) or vascular
tumors. erms such as lymphangioma, cystic hygroma, and cavernous hemangioma, which describe
nonproli erating mal ormations, were no longer used. In 1996 the International Society or the
Study o Vascular Anomalies (ISSVA) adopted the biologic classi cation o vascular anomalies.
Although signi cant progress has been made, incorrect terminology continues to pervade the
medical community. Approximately 50% o patients evaluated at our center have an erroneous
re erral diagnosis; mal ormations are more likely to be misdiagnosed than tumors. Our review o
the 2009 literature revealed that 71% used the term hemangioma incorrectly to describe another
vascular anomaly. Patients whose lesions were mislabeled as a hemangioma had a 20% chance o
being managed incorrectly.
Table 1-1 Incorrect Terminology Used to Describe Vascular Anomalies
Tumors
Mal ormations
Biologic Name
Incorrect Term(s)
Biologic Name
Incorrect Term(s)
Infantile hemangioma
Strawberry hemangioma
Capillary hemangioma
Cavernous hemangioma
Capillary malformation
Port-wine stain
Capillary hemangioma
Congenital hemangioma
Infantile hemangioma
Lymphatic malformation
Cystic hygroma
Lymphangioma
Kaposiform hemangio-
endothelioma
Capillary hemangioma
Venous malformation
Cavernous hemangioma
Pyogenic granuloma
Hemangioma
Arteriovenous malformation
Arteriovenous hemangioma

5
Chapter 1: Terminology and Classification
CLASSIFICATION
T e biologic classi cation o vascular anomalies has been revised based on the evolving under-
standing o the eld (most recently in 2015). Vascular anomalies are classi ed based on their
clinical behavior and cellular characteristics. Applying this ramework, at least 90% o lesions
can be diagnosed by history and physical examination. T ere are two broad types o vascular
anomalies: tumors and mal ormations.
umors demonstrate endothelial proli eration and a ect approximately 5% o the population.
T ere are our major types:
. In antile hemangioma
. Congenital hemangioma
. Kaposi orm hemangioendothelioma
. Pyogenic granuloma
Table 1-2 Current Classif cation o Vascular Anomalies
Tumors
Mal ormations
Simple
Combined
O Major
Named Vessels
Associated With
Other Anomalies
Unclassif ed
Benign
Infantile hemangioma
Congenital hemangioma
Spindle-cell hemangioma
Epithelioid hemangioma
Pyogenic granuloma
Locally aggressive
KHE
Kaposi sarcoma
Malignant
Angiosarcoma
Epithelioid hemangio-
endothelioma
Capillary
HHT
CMTC
Fading stain
Lymphatic
Macrocystic
Microcystic
Mixed
GLA, Gorham
lymphedema
Venous
CMVM
Blue rubber bleb
GVM
CCM
Verrucous
Arteriovenous
HHT
CM-AVM
AV fistula
CVM
CLM
LVM
CLVM
CAVM
CLAVM
Aplasia
Hypoplasia
Stenosis
Ectasia
Aneurysm
Valves
Embryonal
Klippel-Trenaunay
Parkes Weber
Sturge-Weber
Maffucci
MCM
CLOVES
Proteus
BRR
Angiokeratoma
CAT
KLA
PTEN hamartoma
AV, Arteriovenous; BRR, Banayan Riley Rubalcava; CAT, cutaneovisceral angiomatosis with thrombocytopenia; CAVM, capillary arteriovenous
malformation; CCM, cerebral cavernous malformation; CLAVM, capillary-lymphatic-arteriovenous malformation; CLM, capillary lymphatic
malformation; CLOVES, congenital, lipomatous, overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies and/or scoliosis;
CLVM, capillary-lymphaticovenous malformation; CM-AVM, capillary malformation–arteriovenous malformation; CMTC, cutis marmorata telan-
giectatica congenital; CMVM, cutaneomucosal-venous malformation; CVM, capillary venous malformation; GLA, generalized lymphatic anomaly;
GVM, glomuvenous malformations; HHT, hereditary hemorrhagic telangiectasia; KHE, kaposiform hemangioendothelioma; KLA, kaposiform
lymphangiomatosis; LVM, lymphaticovenous malformation; MCM, macrocephaly-capillary malformation; PTEN, phosphatase and tensin homolog.

6
Part I: Introduction
Vascular mal ormations are errors in vascular development and have minimal endothelial turn-
over. T ey a ect approximately 0.5% o the population. T ere are our major categories based on
the primary vessel that is mal ormed:
. Capillary mal ormation
. Lymphatic mal ormation
. Venous mal ormation
. Arteriovenous mal ormation
Mal ormations can be urther divided into rheologically slow-f ow lesions ( or example, capillary,
lymphatic, or venous) and ast-f ow lesions ( or example, arteriovenous mal ormations).
T ere are many phenotypic subtypes o the major categories o vascular anomalies ( or example,
glomuvenous mal ormation), combined lesions ( or example, capillary-lymphatic-venous mal or-
mation), and eponymous syndromes ( or example, Parkes Weber). T e expanded classi cation o
vascular anomalies can be overwhelming or a clinician not ocused on these lesions. o simpli y
the eld, i a physician ocuses on the eight major types o lesions, he or she should be able to
manage approximately 95% o patients with a vascular anomaly. T e eight major types o vascular
anomalies are shown: A, In antile hemangioma; B, congenital hemangioma; C, kaposi orm he-
mangioendothelioma; D, pyogenic granuloma; E, capillary mal ormation; F, venous mal ormation;
G, lymphatic mal ormation; and H, arteriovenous mal ormation.
Fig. 1-1
A

C
B
F
G
D

E
H

7
Chapter 1: Terminology and Classification
T e classi cation o vascular anomalies continues to expand and become more precise as our
knowledge increases. Recently the causative somatic mutation or many types o lesions has been
identi ed. In the uture the delineation o these lesions will shi rom a clinical-histopathologic
perspective to a molecular ramework. Despite the widening classi cation o vascular anomalies,
about 1% o patients re erred to our center have a lesion that we are unable to diagnose.
Table 1-3 Simplif ed Classif cation o Vascular Anomalies
Tumors
Mal ormations
Slow-Flow
Fast-Flow
Overgrowth Syndromes
Infantile hemangioma
Capillary
Arteriovenous
CLOVES
Congenital hemangioma
Venous
Klippel-Trenaunay
Kaposiform hemangio-
endothelioma
Lymphatic
Parkes Weber
Pyogenic granuloma
Sturge-Weber
CLOVES, Congenital, lipomatous, overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies and/or scoliosis.
Table 1-4 Mutations Associated With Vascular Anomalies
Condition
Gene
Capillary malformation

GNAQ
Venous malformations
Sporadic venous malformation
Verrucous venous malformation
Glomuvenous malformation
Cutaneomucosal venous malformation
Cerebral cavernous malformation
TIE2
MAP3K3
Glomulin
TIE2
KRIT1
Lymphatic malformations
Sporadic lymphatic malformation
Familial congenital primary lymphedema
Lymphedema-distichiasis
Lymphedema-hypotrichosis-telangiectasia
Hennekam syndrome
PIK3CA
VEGFR3
FOXC2
SOX18
CCBE1
Arteriovenous malformations
Capillary malformation–arteriovenous malformation
Hereditary hemorrhagic telangiectasia type 1
Hereditary hemorrhagic telangiectasia type 2
PTEN-associated vascular anomaly
RASA1
ENG
ACVRL1
PTEN
ACVRL1, Activin A receptor type IL; CCBE1, collagen and calcium-binding endothelial growth factor domain-containing protein 1; ENG, endoglin;
FOXC2, forkhead box protein C2; GNAQ, guanine nucleotide binding protein alpha q; KRIT1, ankyrin repeat containing; MAP3K3, mitogen-activated
protein kinase kinase kinase 3; PIK3CA, phosphatidylinositol-4,5-bisphosphate 3-kinase, catalytic subunit alpha; PTEN, phosphatase and tensin
homolog; RASA1, RAS p21 protein activator 1; SOX18, SRY-related HMG-box 18; TIE2, endothelial TEK tyrosine kinase; VEGFR3, vascular endo-
thelial growth factor receptor 3.

8
Part I: Introduction
CONCLUSION
T e eld o vascular anomalies can be con using because lesions look similar, and imprecise
terminology continues to be employed. Management o vascular anomalies is simpli ed using
biologic nomenclature and by understanding the major types o tumors and mal ormations.
Be ore considering operative management o a vascular anomaly, the surgeon must comprehend
the context o the lesion within the classi cation ramework.
SUGGESTED READINGS
Enjolras O, Mulliken JB. Vascular tumors and vascular mal ormations (new issues). Adv Dermatol : , .
Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application o a new classi cation. J Pediatr
Surg : , .
Greene AK, Liu AS, Mulliken JB, et al. Vascular anomalies in patients: guidelines or re erral. J Pediatr Surg
: , .
Hassanein A, Mulliken JB, Fishman SJ, et al. Evaluation o terminology or vascular anomalies in current literature.
Plast Reconstr Surg : , .
Meyer JS, Ho er FA, Barnes PD, et al. Biological classi cation o so -tissue vascular anomalies: MR correlation. AJR
Am J Roentgenol : , .
Mulliken JB, Glowacki J. Hemangiomas and vascular mal ormations in in ants and children: a classi cation based
on endothelial characteristics. Plast Reconstr Surg : , .
Wasse M, Blei F, Adams D, Alomari A, Baselga E, Bernstein A, Burrows P, Frieden IJ, Garzon MC, Lopez- Gutierrez
JC, Lord DJ, Mitchel S, Powell J, Prendiville J, Vikkula M. ISSVA Board and Scienti c Committee. Vascular
anomalies classi cation: recommendations rom the International Society or the Study o Vascular Anomalies.
Pediatrics :e , .

T e eld o vascular anomalies is con using, because lesions look similar and terminology is
di cult. Although some vascular anomalies can be treated by a single physician, many require
interdisciplinary care (particularly vascular mal ormations). Patients generally are best managed
in a vascular anomalies center by providers who are ocused on these lesions. Several types o
treatments are used: embolization, laser, pharmacotherapy (topical, intralesional, and systemic),
resection, and sclerotherapy. O en a consensus does not exist about the best intervention, be-
cause there is a lack o randomized, prospective evidence. Evaluation o treatments is urther
handicapped by the heterogeneity o the lesions. For example, one arteriovenous mal ormation
can be small and located on the ace, whereas another may involve an entire extremity. T e in-
tervention chosen to treat a vascular anomaly is requently based on retrospective evidence and
the pre erence o the physician.
Arin K. Greene
Pr in c ipl es o f Man ag emen t
Ch a Pt er 2
KEY POINTS
• Accurate diagnosis of a vascular anomaly must be confirmed before determining the appro-
priate management.
• Treatment of a vascular anomaly may include observation, pharmacotherapy (topical, intrale-
sional, systemic), embolization, sclerotherapy, laser, and/or surgery.
• Intervention is based on the type of vascular anomaly, symptoms, and the physician’s pref-
erence.
• Patients may require interdisciplinary care and are often best managed by physicians who
are focused on vascular anomalies.
9

10
Part I: Introduction
Principles o management include the ollowing:
• Patients should be treated by physicians who ocus on this eld.
• A correct diagnosis must be con rmed be ore choosing management.
• Many lesions can be observed and intervention is not mandatory.
• T e goal o treatment usually is to control rather than to cure the vascular anomaly.
• Complete resection rarely is required.
• Surgical intervention should not create a worse de ormity than the appearance o the lesion.
VARIABLES THAT DETERMINE MANAGEMENT
T ree variables determine how a vascular anomaly is managed:
. Diagnosis
. Morbidity
. Physician pre erence
Table 2-1 Major Vascular Anomalies and Treatments
Tumors
Mal ormations
Biologic Name
Morbidity
Treatment
Biologic Name
Morbidity
Treatment
Infantile hemangioma
Deformity
Obstruction
Destruction
Ulceration
Observation
Topical timolol
Triamcinolone (Kenalog)
injection
Oral prednisolone
Oral propranolol
Pulsed-dye laser
Resection
Capillary malformation
Deformity
Tissue overgrowth
Observation
Pulsed-dye laser
Resection
Congenital hemangi-
oma
Deformity
Observation
Resection
Lymphatic malforma-
tion
Deformity
Bleeding
Infection
Pain
Observation
Sclerotherapy
Radiofrequency
Ablation
Carbon dioxide
laser
Sirolimus
Resection
Kaposiform hemangio-
endothelioma
Deformity
Thrombocytopenia
Fibrosis
Pain
Vincristine
Sirolimus
Venous malformation
Deformity
Phlebothrombosis
Pain
Observation
Sclerotherapy
Resection
Pyogenic granuloma
Deformity
Bleeding
Cautery
Resection
Arteriovenous
malformation
Deformity
Bleeding
Ulceration
Pain
Observation
Embolization
Resection

11
Chapter 2: Principles of Management
Dia g n o s is
T e most important determinant o how a vascular anomaly is managed is the patient’s diagnosis.
Be ore a physician treats a vascular anomaly, the lesion must be accurately identi ed with the
correct terminology. At least 90% o vascular anomalies can be diagnosed by history and physical
examination; the remainder requires imaging (about 9%) or histopathology (about 1%). Some
lesions o en do not require treatment ( or example, in antile hemangioma, congenital heman-
gioma, and capillary mal ormation), whereas others almost always necessitate intervention ( or
example, kaposi orm hemangioendothelioma and pyogenic granuloma). Because most vascular
anomalies are asymptomatic at the time o initial diagnosis, treatment is requently based on
the natural history o the lesion. For example, a macrocystic lymphatic mal ormation is usually
treated prophylactically with sclerotherapy, because it is at risk or enlargement, bleeding, and
in ection. In contrast, an arteriovenous mal ormation most commonly is managed by observation;
embolization and/or resection typically are per ormed only i the lesion becomes symptomatic.
Mo r b iDit y
A er the patient’s vascular anomaly has been diagnosed, treatment is o en based on symptoms.
Because vascular anomalies usually cause a de ormity, the most common problem is lowered
sel -esteem. Many patients do not require intervention until they are 3 to 4 years o age to improve
their appearance (long-term memory and sel -esteem begin to orm at approximately 4 years o
age). Asymptomatic individuals with lesions that are not at risk or signi cant unctional prob-
lems can be observed until they are old enough to decide i they would like to proceed with
intervention.
I a patient is su ering unctional problems, intervention is mandatory, regardless o his or her
age or diagnosis. Local complications consist o bleeding, destruction/obstruction o anatomic
structures, in ection, pain, thrombosis, and ulceration. Systemic morbidity can include congestive
heart ailure, disseminated intravascular coagulation, pulmonary embolism, thrombocytopenia,
and sepsis. Many types o vascular anomalies are progressive, recur a er treatment, and are un-
able to be cured.
T e decision to treat a problematic lesion is easier than prophylactic intervention or an asymp-
tomatic vascular anomaly. Some lesions can remain nonproblematic or a prolonged period but
are at risk or complications. For example, pulsed-dye laser treatment o a capillary mal ormation
in in ancy may slow cutaneous discoloration and skin thickening. Sclerotherapy o a lymphatic
mal ormation can prevent intralesional bleeding or in ection.
Ph ys ic ia n Pr e f e r e n c e
T e decision to treat a patient with a vascular anomaly and the type o intervention are o en based
on the experience o the physician. A medical specialist may eel more com ortable managing
a patient with oral pharmacotherapy, whereas a surgeon may pre er resection or intralesional
treatment. T e eld o vascular anomalies lacks prospective, randomized data comparing di erent
types o interventions. Consequently, a physician’s treatment choice is requently based on case
series and his or her training.

12
Part I: Introduction
MANAGEMENT OPTIONS
o b s e r va t io n
T e most common “intervention” or a vascular anomaly is observation. More than 90% o in-
antile hemangiomas proli erate and involute without consequence. Congenital hemangiomas
rarely require treatment. Capillary, lymphatic, or venous mal ormations that are small or located
in inconspicuous areas are usually observed. Large, di use arteriovenous mal ormations that are
too di cult to resect are monitored until they become symptomatic.
t o Pic a l Ph a r Ma c o t h e r a Py
opical drugs are e cacious only or the management o a problematic proli erating hemangioma.
imolol can inhibit the growth o a super cial hemangioma i it is administered early (less than
8 weeks o age). High-potency corticosteroids have been used or in antile hemangioma, but are
generally not recommended, because they can cause atrophy and hypopigmentation.
in t r a l e s io n a l Ph a r Ma c o t h e r a Py
Injecting a drug into a vascular anomaly is commonly used or a problematic, proli erating in an-
tile hemangioma. riamcinolone e ectively stops the growth o this tumor and usually causes it
to decrease in size. Intralesional administration o pharmacotherapy or other types o vascular
anomalies is not available.
s ys t e Mic Ph a r Ma c o t h e r a Py
Systemic drugs or vascular anomalies are available to treat some tumors with proli erating endo-
thelium. A problematic in antile hemangioma that is too large to inject with triamcinolone will
respond to oral prednisone or propranolol. Kaposi orm hemangioendothelioma is managed with
either intravenous vincristine or oral sirolimus. Recently, systemic pharmacotherapy has been
expanded to include vascular mal ormations; microcystic lymphatic mal ormations have shown
a response to oral sirolimus.
s c l e r o t h e r a Py
Sclerotherapy is the direct injection o an irritant into a lesion that causes endothelial damage,
brosis, shrinkage, and improved symptoms. Sclerotherapy is used to treat macrocystic lym-
phatic mal ormations and venous mal ormations. T e method can also be used or the venous
component o an arteriovenous mal ormation. Recently bleomycin has been used success ully or
microcystic lymphatic mal ormations, which previously were unable to be treated with sclero-
therapy. Direct injection o cutaneous lymphatic vesicles with a sclerosant can minimize bleeding
and lymphorrhea.
e Mb o l iz a t io n
Embolization is used to treat arteriovenous mal ormations. T e technique involves the cannula-
tion o an artery remote rom the lesion, guiding a catheter into the nidus, or draining veins and
then injecting an embolic substance. T e embolus reduces ischemia and improves symptoms.

13
Chapter 2: Principles of Management
l a s e r t h e r a Py
Pulsed-dye is the most common type o laser or the treatment o vascular anomalies. It removes
residual telangiectasia a er in antile hemangioma has regressed (i used during the proli erative
phase, it may cause ulceration, hypopigmentation, and scarring). A pulsed-dye laser e ectively
lightens the color o capillary mal ormations. A carbon dioxide laser improves cutaneous lym-
phatic vesicles and hyperkeratotic, bleeding, verrucous venous mal ormations. Radio requency
ablation can treat intraoral lymphatic vesicles.
s u r g ic a l in t e r v e n t io n
Most vascular anomalies do not require radical resection and reconstruction. T e only lesion that
is primarily managed operatively is pyogenic granuloma. Because pyogenic granuloma involves
the reticular dermis, treatments other than ull-thickness skin resection or cautery have a high
recurrence rate. Problematic proli erating in antile hemangiomas are managed with pharmaco-
therapy; resection during in ancy is rare. In antile hemangiomas that have le a residual de ormity
a er involution may be removed. Rapidly involuting congenital hemangioma can cause subcu-
taneous atrophy, which is improved with at gra ing. I a noninvoluting congenital hemangioma
causes a de ormity, it can be excised. Kaposi orm hemangioendothelioma usually is too large and
di use to remove; surgical intervention is reserved or small lesions or reconstruction o brosis
and contractures. A capillary mal ormation can cause hypertrophy o tissues beneath the stain,
which can be improved with surgery. Resection is considered or lymphatic mal ormations i they
are small, microcystic, or remain symptomatic a er sclerotherapy. Venous mal ormations are
typically excised i they are small or remain symptomatic a er sclerotherapy. Surgical interven-
tion or an arteriovenous mal ormation is reserved or asymptomatic, localized lesions that can
potentially be removed or “cure,” or subtotal extirpation o a large lesion to improve symptoms.
CONCLUSION
Many treatment options exist or vascular anomalies. Management is based on the type o lesion,
symptoms, and physician pre erence. Patients o en bene t rom interdisciplinary care o the
lesion. Surgeons should understand the di erent treatment options available or a speci c type
o vascular anomaly be ore choosing operative intervention.
SUGGESTED READINGS
Couto JA, Maclellan RA, Greene AK. In antile hemangioma: treatment rate during the proli erating phase. J Cra-
nio ac Surg : , .
Greene AK, ed. Vascular Anomalies: Classi cation, Diagnosis, and Management. St Louis: Quality Medical Pub-
lishing, .
Greene AK, Liu AS, Mulliken JB, et al. Vascular anomalies in patients: guidelines or re erral. J Pediatr Surg
: , .
Hassanein A, Mulliken JB, Fishman SJ, Greene AK. Evaluation o terminology or vascular anomalies in current
literature. Plast Reconstr Surg : - , .

(Trang không có chữ)

Although there has been a recent emergence o medical therapies or a variety o vascular anoma-
lies, procedural management remains central to the care o many o these disorders. T is chapter
will cover how to assess or bleeding and thrombotic complications and minimize these risks be-
ore, during, and a er procedures. T e combination o bleeding and thrombotic risk in the same
patient requires expertise and vigilant, expectant management. Interdisciplinary discussion and
coordination o perioperative care are critical to this process. In addition to surgical disciplines,
anesthesia, hematology, blood bank, critical care, and interventional radiology can play critical
roles.
Cameron C. Trenor III
Per io per at ive H emat o l o g ic
Man ag emen t
Ch a Pt er 3
KEY POINTS
• Operative planning for high-risk vascular anomalies requires laboratory and/or imaging at
least 2 weeks before surgery.
• The best laboratory predictor of bleeding risk in patients with consumptive coagulopathy is
a low fibrinogen level.
• The best predictor of thrombotic risk is patent ectatic veins connecting to the deep venous
system.
• Consumptive coagulopathy can be normalized with anticoagulation and cryoprecipitate in
most patients.
• Interdisciplinary communication throughout the planning, surgery, and postoperative care
is critical to achieve safe and effective surgical management of vascular anomalies.
15

16
Part I: Introduction
FUNDAMENTALS OF HEMOSTASIS AND THROMBOSIS
Hemostasis and thrombosis involve three separate but intertwined biologic processes:
. Slowed blood f ow: Platelets are responsible or the initial adhesion to damaged tissues,
and ormation o the platelet plug initiates clot ormation. T is is particularly true or mu-
cosal and cutaneous sur aces. Activated platelets can occur in vascular anomalies and can
be predisposed to the initiation o thrombosis, especially around procedures and during
inf ammation.
. Endothelial damage: Subendothelial tissue actor or oreign materials ( or example, cathe-
ters) can initiate the coagulation cascade, an ampli ying proteolytic series o events leading
to activated circulating coagulation actors. T is process culminates in brinogen cleavage
by thrombin into brin, which is crosslinked and orms the brin clot. T e conversion o
soluble brinogen to a solid brin clot stops urther bleeding and allows healing o the
underlying injury.
. Hypercoagulability: T e balance o brin clot breakdown is controlled by plasmin, which
proteolytically cleaves crosslinked brin, releasing D-dimers into the circulation and recan-
alizing the vessel. Fibrinolysis is understudied in vascular anomalies, but hypo brinolytic
states promote thrombosis, whereas hyper brinolytic states promote recurrent bleeding
and poor wound healing. Hyper brinolysis is targeted by anti brinolytic drugs, such as
epsilon-aminocaproic acid or tranexamic acid; these agents are most e ective or mucosal
bleeding.
Each o these has relevance in vascular anomalies. Slowed blood f ow is generally considered or
periods o immobility, cardiac insu ciency, and vascular compression. In addition, or patients
with vascular mal ormations, dilation (ectasia) o venous channels and saccular vascular struc-
tures creates areas o slowed, swirling, stagnant, or even reversed blood f ow. Adequate blood
f ow is protective rom thrombosis by diluting activated clotting actors. By allowing activated
platelets and clotting actors to concentrate in areas o slowed blood f ow, localized intravascular
coagulopathy occurs in slow-f ow vascular mal ormations. Studies comparing intralesional with
systemic blood have demonstrated the concentration o coagulopathy within intralesional blood.
Endothelial damage generally re ers to surgery, catheter placement, and trauma. Vascular anoma-
lies are lined with abnormal endothelium, allowing exposure o subendothelial collagen and tissue
actor and resultant activation o platelets and clotting actors. In healthy circulation, vascular
endothelium is coated with antithrombotic proteins, including thrombomodulin and endothelial
protein C receptor. T e loss o these proteins and their endogenous anticoagulant properties also
contributes to thrombotic risk. Furthermore, the main therapies or slow-f ow vascular mal orma-
tions are surgical resection and endovascular sclerotherapy, which trigger additional endothelial
injury.
Hypercoagulability re ers to the inherent properties o the blood avoring thrombosis. Common
inherited causes are actor V Leiden and prothrombin gene mutation G20210A, which occur in
5% and 2% o whites, respectively. De ciencies in anticoagulant proteins such as antithrombin,
protein C, or protein S can be inherited or acquired in the setting o thrombosis. Inf ammatory
and rheumatologic disorders, especially those associated with antiphospholipid antibodies, are
also prothrombotic. Speci c laboratory assessment or hypercoagulability is not indicated or all
patients with vascular anomalies but should be considered or patients with a amily history o
thrombosis or a personal history o thrombosis outside o the vascular mal ormation or severe
extensive or recurrent thrombosis.

17
Chapter 3: Perioperative Hematologic Management
HEMATOLOGIC ABNORMALITIES IN VASCULAR
ANOMALIES
T ere are two general types o coagulopathy in vascular anomalies:
. Platelet trapping within vascular tumors
. Intralesional thrombosis, leading to consumptive coagulopathy in vascular mal ormations
T ese are distinct processes occurring in di erent types o vascular anomalies.
Platelet trapping within vascular tumors is generally re erred to as Kasabach-Merritt phenomenon.
T e classic vascular tumor associated with platelet trapping is kaposi orm hemangioendothelioma
(KHE), an in ltrative vascular tumor occurring mainly during in ancy. T is can also occur in
tu ed angioma, cutaneovisceral angiomatosis with thrombocytopenia, and kaposi orm lymph-
angiomatosis. Alterations in coagulation testing can occur concurrently in severe cases. Although
not proved in the literature, the leading hypothesis is that normal circulating platelets adhere to
abnormal lymphatic endothelium. Rapid consumption o trans used platelets in the vascular
tumor supports this hypothesis. On adherence, platelets are activated and begin to aggregate and
orm platelet-rich thrombi. T ese are visible on histologic ndings in KHE. Activated platelets
can also initiate brin clot ormation, although this is secondary, in contrast to consumptive co-
agulopathy within slow-f ow vascular mal ormations.
T rombocytopenia can also occur in association with vascular anomalies or other reasons. Cen-
tral hemorrhage into a large, hepatic, rapidly involuting congenital hemangioma can cause throm-
bocytopenia. Di use gastrointestinal bleeding or secondary hypersplenism rom portal hyperten-
sion can also lead to mild-to-moderate thrombocytopenia. Massive venous ectasia with slow or
stagnant blood f ow has also been associated with mild thrombocytopenia and thrombotic risk.
Table 3-1 Hematologic E ects o Vascular Anomalies
Feature
Slow-Flow Vascular Mal ormation*
Kaposi orm Hemangioendothelioma With
Kasabach-Merritt Phenomenon
Platelets
Normal or mildly low
Moderately to severely low
Fibrinogen
Significantly low
Significantly decreased in severe cases
PT/aPTT
Often prolonged, correlating with fibrinogen
Can be prolonged in severe cases
D-dimer
Moderately to significantly elevated
Normal to mildly elevated
Pathogenesis
Intralesional thrombin generation, causing
consumptive coagulopathy
Platelet trapping within the tumor; when severe can lead to
consumptive coagulopathy
Management
Anticoagulation, support with cryoprecipitate,
and fresh-frozen plasma
Steroid and vincristine or sirolimus; withhold platelets
unless bleeding or for procedure (will worsen tumor
engorgement and pain)
*Venous malformation, venolymphatic malformation (blue rubber bleb nevus syndrome), and capillary lymphaticovenous malformation (Klippel-
Trenaunay).
PT/aPTT, Prothrombin time/activated partial thromboplastin time.

18
Part I: Introduction
Slow-f ow venous mal ormations are at risk o consumptive coagulopathy. T is is most common
in extensive cases, especially those o pure venous mal ormation or venolymphatic mal orma-
tion (blue rubber bleb nevus syndrome). Other mixed-type vascular anomalies with slow blood
circulation are also at risk. Activation o the clotting cascade by exposure to abnormal vascular
endothelium and subendothelial proteins triggers this process, which is concentrated in a slow-
f ow environment. In mild cases, aspirin may modulate symptomatic thrombophlebitis. Peripro-
cedurally and in more severe cases, aspirin is insu cient, and anticoagulation is indicated to
suppress intralesional thrombin generation and thrombosis. Consumptive coagulopathy may
re er to D-dimer elevation, prolonged prothrombin time (P ) or activated partial thromboplas-
tin time (aP ), or a de ciency o brinogen. Low brinogen is predictive o bleeding risk and
is corrected with anticoagulation and/or cryoprecipitate. I P /aP prolongation remains a er
brinogen correction, urther hematologic workup is recommended.
LABORATORY ASSESSMENT OF COAGULOPATHY
Assessment and monitoring o bleeding can be done with widely available laboratory studies. A
complete blood count assesses or anemia and thrombocytopenia. Coagulation times, P , and
aP measure the time until the brin clot orms. Prolongation o these in vitro clotting times
may indicate a predilection or bleeding. In vascular anomalies, consumptive coagulopathy is the
most common reason or prolonged P and aP . D-dimer measures crosslinked brin cleavage
products, and elevation indicates activation o the coagulation cascade and brinolysis. Many
patients have D-dimer elevation at baseline that worsens during thrombosis or inf ammation or
a er sclerotherapy or surgical procedures. Fibrinogen should be speci cally measured rather than
estimated rom P /aP assessments. Although P and aP measure the e ects o multiple
proteins involved in clot ormation, brinogen measurement speci cally tests or a de ciency o
the nal protein needed to make a brin clot. Low brinogen indicates signi cant consumption
and correlates with bleeding risk. T ese laboratory tests must be assessed preoperatively and ar
enough in advance to allow time or correction with anticoagulation, which is generally 2 weeks.
Additional laboratory testing can be considered, ideally in a research setting. Whole-blood assays,
such as thromboelastography or rotational thromboelastography, have been considered, as have
markers o brinolysis. Historically a comparison o intralesional to systemic blood showed a
concentration o the coagulopathy within the vascular anomaly.
MANAGEMENT OF PERIOPERATIVE BLEEDING RISK
Although bleeding risk is mainly intraoperative, this risk continues postoperatively and should
be vigilantly anticipated. Contributions to bleeding risk include a personal history o bleeding
and the degree o coagulopathy and vascularity o the mal ormation to be incised. Anticipatory
control o intraoperative bleeding begins weeks be ore the procedures. A ailure to recognize
high-f ow vascular diagnoses or consumptive coagulopathy can lead to catastrophic bleeding
during procedures.
Preoperative embolization should be considered or a reduction in intralesional circulation in
ast-f ow vascular anomalies. T is is common or arteriovenous mal ormations but should be
discussed or any hypervascular-enhancing lesions. Although not generally needed or involuting
in antile hemangioma, other vascular tumors such as Enzinger hemangioma and juvenile nasal
angio broma are resected with less blood loss a er embolization.

19
Chapter 3: Perioperative Hematologic Management
Assessment or coagulopathy includes measurement o platelet count, P /aP , brinogen, and
D-dimer at least 2 weeks be ore the procedure. Patients with extensive venous mal ormation, blue
rubber bleb nevus syndrome (venous and lymphatic), or Klippel- renaunay syndrome (capillary
and lymphatic and venous) should be evaluated or coagulopathy. Aggressive lymphatic disorders
and tumors such as kaposi orm lymphangiomatosis or KHE with Kasabach-Merritt phenomenon
may also mani est consumptive coagulopathy in severe cases.
Although P /aP prolongation and D-dimer elevation are clues to coagulopathy, bleeding risk
correlates with def ciency o f brinogen. Anticoagulation, which typically occurs with low-molecular-
weight heparin ( or example, enoxaparin, dalteparin, and tinzaparin), will improve coagulopathy
over several days. Dosage is titrated until normalization o brinogen levels and ranges rom 0.5
to 1 mg/kg/dose every 12 hours in most patients. A standard approach is to initiate anticoagulation
2 weeks be ore the procedure and recheck laboratory testing or coagulopathy 1 week later. I the
initial anticoagulation is insu cient to ully correct coagulation tests, higher doses should be
considered, and expert consultation is advised to de ne and reverse the bleeding disorder be ore
the procedure. In most patients anticoagulation is held be ore the procedure and resumed a er
surgical hemostasis is established. In high-risk cases anticoagulation can be continued through
the procedure. T is may seem counterintuitive, but used in this manner or consumptive coag-
ulopathy, anticoagulation is restoring normal hemostasis rather than promoting bleeding. T is
approach extends to surgical cases beyond the vascular mal ormation itsel . For example, dental
extraction can lead to dramatic bleeding i coagulopathy is not corrected be ore this procedure.
Intraoperatively, close communication with anesthesia and ongoing laboratory monitoring are
essential. Fibrinogen levels should be maintained greater than 100 to 150 mg/dl throughout the
case with anticoagulation or cryoprecipitate. Volume maintenance is particularly important to
vascular surgical cases, but large-volume trans usion can lead to dilutional coagulopathy. An
experienced anesthesia, blood bank, and/or hematology team can manage this with resh- rozen
plasma, along with red cell and platelet trans usions. Monitoring and correction o hypocalcemia
rom citrate in used with trans usions are also important to hemostasis. Other intraoperative
techniques to assist locally with hemostasis are covered elsewhere in other chapters, including
tourniquet use, topical thrombin, topical anti brinolytics, and Aquamantys.
Platelet trans usion or thrombocytopenia is also important to hemostasis. An exception to this is
Kasabach-Merritt phenomenon associated with KHE. Advances in medical therapy or KHE have
replaced surgical treatments in most patients. Platelet trans usion in KHE with Kasabach-Merritt
phenomenon will not correct the thrombocytopenia but will uel platelet trapping within the le-
sion, leading to rapid engorgement o the tumor and worsening coagulopathy. Platelet trans usion
should be reserved or signi cant bleeding in this disorder. Postoperatively consumptive coagu-
lopathy o en worsens. Close monitoring and continued correction are critical to both bleeding
prevention and wound healing. Reinstitution o anticoagulation and use o cryoprecipitate are
o en su cient.
MANAGEMENT OF PERIOPERATIVE THROMBOTIC RISK
Preoperative identi cation o prothrombotic risk actors allows a tailored approach to inter-
ventions. Patients with extensive venous mal ormation, CLOVES (congenital, lipomatous, over-
growth, vascular mal ormations, epidermal nevi, spinal/skeletal anomalies and/or scoliosis), or
Klippel- renaunay syndrome should be assessed or venous ectasia. Although venous ectasia
o en involves the overgrown extremity, evaluation o pelvic, marginal trunk, and upper extremity
veins may reveal additional veins at risk o intraoperative or postoperative thromboembolism.

20
Part I: Introduction
Patients with CLOVES are more likely to have marginal veins on the torso and massively ectatic
upper extremity veins than other diagnoses. Expert diagnosis and closure o these veins are rec-
ommended be ore any surgical procedure. Closure must occur in a proximal to distal direction
to prevent proximal extension o the clot into the deep venous system and embolization risk. In
some patients, in erior (or even superior) vena caval lters have been placed perioperatively to
prevent massive pulmonary embolism, although this practice is not evidence based. Patients with
signi cant ectasia or large slow-f ow mal ormations with coagulopathy have improved blood f ow
and thrombotic risk with preoperative anticoagulation.
T ere are no speci c laboratory values predictive o thrombosis in vascular anomalies procedures.
Imaging or ectasia, personal history o thrombosis, and speci c vascular anomaly diagnosis are
the best predictors. Patients with massive venous ectasia may develop mild thrombocytopenia
rom slow blood f ow, but thrombocytopenia alone is not predictive o thrombosis. Isolated el-
evation o D-dimer is common in vascular anomalies (and many clinical disciplines) and is not
speci c or thrombosis or thrombotic risk. Negative D-dimer is reassuring but is rarely ound in
high-risk patients planning or procedures.
Intraoperatively, maintenance o intravascular volume and blood pressure lowers thrombotic risk.
Mechanical measures, such as pneumatic compression boots or similar devices, should be used i
easible. Postoperatively, early ambulation, physical therapy, pneumatic compression boots, and
anticoagulation are important in high-risk patients. Generally, anticoagulation is used or 2 weeks
or until return to baseline ambulatory status, whichever is longer.
Table 3-2 Hematologic Abnormalities in Vascular Anomalies
Vascular Anomaly
Bleeding Risk Considerations
Thrombotic Risk Considerations
Tumors
Infantile hemangioma
KHE with KMP
CAT/MLT
Other large vascular tumors
Procedural bleeding, highest in proliferative phase
Thrombocytopenia; severe cases with
coagulopathy
Thrombocytopenia
Consider preoperative embolization
No increased risk
No increased risk
No increased risk
No increased risk
Malformations
Capillary malformations
Venous malformations
BRBNS
CLOVES
Klippel-Trenaunay
AVM
Lymphatic malformation
Kaposiform lymphangioma-
tosis
Minor procedural bleeding risk
Correct consumptive coagulopathy
Correct consumptive coagulopathy
Correct consumptive coagulopathy; preoperative
embolization if fast-flow lesion
Correct consumptive coagulopathy
Preoperative embolization
Minor procedural bleeding risk
Correct coagulopathy and thrombocytopenia
No increased risk
Close ectatic veins, anticoagulation
No increased risk
Close ectatic veins, anticoagulation
Close ectatic veins, anticoagulation
No increased risk
No increased risk
No increased risk
AVM, Arteriovenous malformation; BRBNS, blue rubber bleb nevus syndrome; CAT, cutaneovisceral angiomatosis with thrombocytopenia;
CLOVES, congenital, lipomatous, overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies and/or scoliosis; KHE, kaposiform
hemangioendothelioma; KMP, Kasabach-Merritt phenomenon; MLT, multifocal lymphangioendotheliomatosis with thrombocytopenia.

21
Chapter 3: Perioperative Hematologic Management
OTHER CONSIDERATIONS FOR THROMBOTIC RISK
Hormonal contraception can increase the risk o blood clots two old to seven old, depending on
the product. Be ore a major surgery, it is reasonable to stop hormonal contraception a month
prior to surgery to allow normalization o hypercoagulability. In patients with vascular anomalies
with a high risk o thrombosis, these medications are o en avoided to prevent this complication.
Currently the term hormonal contraception is pre erred, because not all products are taken orally.
Nonhormonal means o birth control, intrauterine devices, and progestin-only pills do not add
to thrombotic risk. Injectable progestin products con er a two old risk o thrombosis. Combined
hormonal contraceptives have increased thrombotic risk correlating with estrogen dose and gen-
eration o progestin. Higher estrogen doses and drospirenone-containing products ( or example,
Yaz or Yasmin) con er the highest risk o thrombosis. Contrary to expectations, transdermal
patches and intravaginal delivery systems ( or example, vaginal rings) present some o the highest
risks o thrombosis, attributed to continuous exposure and lack o rst-pass hepatic metabolism.
Pregnancy in patients with vascular anomalies can o en be managed success ully but should be
approached proactively with interdisciplinary conversation between experts in vascular anoma-
lies and high-risk maternal- etal medicine specialists. Exacerbation o some vascular anomalies
occurs with increased exposure to hormones and expanded circulatory demands o pregnancy.
Furthermore, pregnancy is a prothrombotic state, which increases the risk o thrombosis 6- old to
10- old. T rombosis risk is highest in the third trimester and rst 6 weeks a er delivery. T ere are
case reports describing the management o thrombotic risk with low-molecular-weight heparin
through pregnancy and a er delivery or mothers with vascular anomalies.
Although most patients with vascular anomalies should not be routinely assessed or thrombo-
philia, certain patients warrant consideration, o en in consultation with a hematologist. I there
is a rst-degree or second-degree amily history o unprovoked deep vein thrombosis or pulmo-
nary embolism, heart attack or stroke be ore 50 years o age, or recurrent miscarriage a er the
rst trimester, evaluation or inherited thrombophilia is reasonable. T is evaluation should also
be considered or patients with extensive or recurrent thrombosis, especially i occurring outside
o the actual vascular mal ormation. T rombophilia evaluation rarely changes management; pa-
tients with strong personal or amily histories should be managed more aggressively, regardless o
ndings. False reassurance occurs i testing is negative, and this should be proactively managed.
Patients with certain vascular mal ormations have signi cantly increased risks o thrombosis
related to slow f ow and damaged endothelium, independent o hypercoagulability.
CONCLUSION
Surgical interventions are o en needed in patients with vascular anomalies and signi cant bleed-
ing and clotting risk. An assessment o these risks must begin preoperatively and includes clinical,
radiographic, and laboratory assessment in at-risk patients. Preoperative planning to identi y
consumptive coagulopathy and venous ectasia is recommended in patients with high-risk vascular
diagnoses or signi cant personal history o bleeding or thrombosis. Consumptive coagulopathy
and bleeding risk can be normalized with anticoagulation in most patients. Ectatic veins should be
ligated—surgically or endovascularly—and closed or removed be ore major surgery to minimize
intraoperative and postoperative thrombosis. Intraoperative blood product support in collabora-

22
Part I: Introduction
tion with anesthesia and local surgical techniques is essential to hemostasis during consumptive
coagulopathy or bleeding diathesis. Postoperative monitoring and correction o coagulopathy
with anticoagulation and/or blood product support will support wound healing and minimize
bleeding during recovery. Intraoperative and postoperative attention to prevention o thrombosis
is important in patients with slow-f ow vascular mal ormations and particularly in patients with
overgrowth syndromes ( or example, CLOVES or Klippel- renaunay syndrome) with associated
venous ectasia. Excellent communication across the interdisciplinary care team is required, and
high-risk procedures should only be undertaken by experienced teams.
SUGGESTED READINGS
Alomari AI, Burrows PE, Lee EY, et al. CLOVES syndrome with thoracic and central phlebectasia: increased risk o
pulmonary embolism. J T orac Cardiovasc Surg : , .
Croteau SE, Liang MG, Kozakewich HP, et al. Kaposi orm hemangioendothelioma: atypical eatures and risks o
Kasabach-Merritt phenomenon in re errrals. J Pediatr : , .
Dompmartin A, Acher A, T ibon P, et al. Association o localized intravascular coagulopathy with venous mal or-
mations. Arch Dermatol : , .
Mazoyer E, Enjorlas O, Bisdor A, et al. Coagulation disorders in patients with venous mal ormation o the limbs
and trunk: a case series o patients. Arch Dermatol : , .
Mulliken JB, Anupindi S, Ezekowitz RA. Case - : a newborn girl with a large cutaneous lesion, thrombocyto-
penia and anemia. N Engl J Med : , .

Par t II
Vas c u l ar Tu mo r s

(Trang không có chữ)

In antile hemangioma is the most common tumor o in ancy, a ecting 5% o in ants. Eighty
percent o lesions involve the integument and are red; 20% are located subcutaneously and may
appear bluish or have normal overlying skin. An in antile hemangioma has a unique growth
cycle; it is usually noted 2 weeks a er birth and enlarges rapidly during the rst 9 months o li e
(proli erating phase). Eighty percent o its size is achieved by 3.2 (± 1.7) months o age. Between
9 and 12 months o age, the lesion begins to slowly shrink (involuting phase); the appearance
improves until 31⁄2 years o age (involuted phase). A er involution, 50% o in antile hemangiomas
will leave behind a permanent de ormity: residual bro atty tissue, anetoderma, scar, redundant
skin, telangiectasias, and/or destroyed anatomic structures.
Arin K. Greene
In f an t il e H eman g io ma
Ch a Pt er 4
KEY POINTS
• Resection of an infantile hemangioma during the proliferative phase is uncommon.
• Surgical intervention for an infantile hemangioma is usually performed between 3 and
4 years of age after the lesion has completed regression.
• Complex reconstruction after extirpation of an infantile hemangioma is rarely required;
wounds typically can be closed with local tissues.
• Round lesions located in visible areas can be removed with a circular excision and purse-
string closure to limit the size of the scar.
• Patients with an infantile hemangioma are best managed by physicians focused on this tumor.
25

26
Part II: Vascular Tumors
SURGICAL INDICATIONS
T e primary morbidity o an in antile hemangioma is psychosocial, because the lesion can cause a
de ormity. Surgical procedures are generally carried out to improve a “cosmetic” problem. Because
long-term memory and sel -esteem do not begin to develop until 4 years o age, there is rarely
an indication to remove a lesion be ore it has nished improving at 3 years o age. An involuted
hemangioma can lower sel -esteem i there is residual bro atty tissue, scarring rom ulceration
in in ancy, anetoderma, redundant skin, altered anatomic structures, or telangiectasias. I only
telangiectasias remain, the patient should be treated with pulsed-dye laser. T is laser will erase the
cutaneous blood vessels without scarring. T e only way to improve redundant skin, anetoderma,
or subcutaneous adipose tissue is resection. Altered anatomic structures (usually o the central
ace) are improved with the same principles used or other reconstructive problems.
During in ancy an in antile hemangioma can cause unctional problems by obstructing vision,
the nose (in ants younger than 3 months o age are primarily nasal breathers), or the oral airway.
Resection o lesions in these areas should be the last option; removal o a proli erating in antile
hemangioma is avoided when possible. Instead, problematic lesions are managed with topical or
intralesional medications (i localized) or oral pharmacotherapy (i di use). T ese drugs will stop
the growth o the tumor (and of en decrease its size) until natural involution begins at 9 months
o age. Sixteen percent o in antile hemangiomas ulcerate during the proli erating phase. T e
wounds will heal within 2 to 4 weeks af er applying hydrated petroleum and a barrier dressing
and eliminating the trauma that caused the wound. Ulcerated lesions do not require resection
and are not at risk or in ection, because the wound is open (even i urine and stool cover the
area). In antile hemangiomas involving the genitalia will not a ect sexual or urinary unction.
SURGICAL MANAGEMENT
t iMin g o f in t e r v e n t io n
Intervention will depend on the phase o the hemangioma: during the proli erating phase or the
involuted phase (see Video 4-1, In antile Hemangioma).
Pro life ra tin g P h a s e
Most in antile hemangiomas do not require treatment; they are small and nonproblematic. Lesions
are allowed to proli erate and involute without intervention; af er regression there is minimal ev-
idence that the tumor was ever present. In my practice, which is biased toward re errals or prob-
lematic lesions, only 22% o in ants are treated: 5% undergo corticosteroid injection, 8% receive
oral pharmacotherapy, 2% undergo resection, and 7% receive topical timolol. T e indications or
treatment o a proli erating in antile hemangioma are:
• T e lesion is at risk or obstruction/destruction o an important anatomic structure.
• It may leave behind a permanent de ormity, requiring treatment later in childhood.
Almost all lesions that necessitate early intervention are located on the ace or neck. My rst-line
treatment or localized lesions that are less than 3 cm in diameter is corticosteroid injection not
to exceed 3 mg/kg (triamcinolone 40 mg/ml) per injection. I the in antile hemangioma is too
large to inject, a patient is given systemic pharmacotherapy (prednisone or propranolol). Both
drugs are e ective and sa e. A super cial hemangioma will respond to topical timolol i treatment
is initiated early, usually be ore 10 weeks o age.

27
Chapter 4: Infantile Hemangioma
Resection o a proli erating in antile hemangioma is generally not recommended. T e tumor is
highly vascular during this period, and there is a risk o blood loss, iatrogenic injury, and an in-
erior outcome compared with excising residual tissue a er the tumor has regressed. Indications
or surgical intervention during this phase include:
• T ere is a ailure or contraindication to pharmacotherapy.
• T e patient has a well-localized tumor in an anatomically avorable area.
• Resection will be necessary in the uture and the scar would be the same.
Many parents want an in antile hemangioma removed as soon as possible, because it attracts
the attention o others. I counsel amilies that it is better to wait until the lesion has completed
involution (between 3 and 4 years o age) or the ollowing reasons:
• T e tumor will be smaller, which acilitates the procedure and gives the child the shortest
possible scar.
• T ere is no urgency to remove the lesion, because the child’s long-term memory and sel -
esteem do not begin to orm until 4 years o age.
In my practice only 2% o in antile hemangiomas are resected during the proli erating phase; the
rest are excised a er they have involuted. During the proli erating phase I have removed ulcerated
lesions that the parents were unable to manage or were less likely to heal quickly ( or example,
back o the neck where the child sleeps on the area). Because the scalp has signi cant laxity during
in ancy, this is a avorable time to remove large ulcerated lesions in this location, because the area
is more easily closed linearly. Retroauricular in antile hemangiomas can cause a prominent ear
de ormity; although the hemangioma will ultimately involute, the child may require an otoplasty.
Consequently, lesions a ecting the ear may bene t rom removal during in ancy.
Invo lu te d P h a s e
T ere is rarely an indication to remove a hemangioma during the involuting phase (9 months
through 3 years), because the tumor is either problematic while it is rapidly growing, or it leaves
behind a permanent de ormity. A er involution, approximately 50% o in antile hemangiomas will
have bro atty tissue or damaged skin, causing a de ormity. It is best to wait until the tumor has
completed involution between 3 and 4 years o age be ore deciding whether surgical intervention
is indicated. O en a tumor in in ancy that is predicted to leave behind a de ormity regresses better
than expected, and the child does not need surgery. I the child requires a procedure, it is sa er
a er involution, because the tumor is much less vascular. In addition, the in antile hemangioma
is signi cantly smaller, and thus the scar is much shorter.
I I believe a patient may require surgical intervention, they return or an evaluation when they
turn 3 years o age. I the parents state that there has been minimal or no improvement over
the previous ew months, the surgery is scheduled. I the in antile hemangioma is continuing
to improve or is in a very problematic location (philtrum, nasal tip, or eyelid), they will return
in 6 months or another examination, and the procedure will be scheduled be ore their ourth
birthday to allow the lesion to spontaneously improve or as long as possible. Occasionally an
in antile hemangioma will stop improving between 18 and 36 months o age, and i so, I will inter-
vene be ore 3 years o age. Some children will have a minor de ormity, and it is unclear whether:
• T e area will be bothersome to the patient.
• T e scar rom an operation would be more noticeable than the residual in antile heman-
gioma.
In this situation it is best to observe the child and intervene later i the child requests a procedure
when he or she is older.

28
Part II: Vascular Tumors
Pr in c iPl e s
An in antile hemangioma is benign and does not require resection, unless it causes lowered
sel -esteem. Patients will requently have minor de ormities, and the surgeon must determine
i the resulting scar will be an improvement over the residual in antile hemangioma. reatment
o a benign vascular anomaly should never cause a worse de ormity than the lesion. T e goal o
the surgery is to improve the appearance o the area and not complete extirpation o the in antile
hemangioma. Consequently, most resections are subtotal, and it is pre erable to leave some resid-
ual broadipose tissue rather than attempting to per orm complete resection, which increases the
risk o nerve injury and gives the patient a longer scar. An in antile hemangioma almost always
involves the skin and subcutaneous tissue only; however, i deeper structures contain residual
bro atty tissue, they do not need to be removed. Only a super cial lesion should be removed
to improve the patient’s appearance; nerve dissections and deep resections are not per ormed.
In antile hemangiomas in anatomically sensitive areas ( or example, the ace) should have minimal
or no margins taken. I the overlying skin is normal and there is a greater subcutaneous compo-
nent, a smaller incision can be made, skin f aps are elevated, and the underlying hemangioma
is removed without per orming a large skin resection. Residual adipose tissue at the base o the
wound can be cauterized, which will cause atrophy and urther improvement without resection.
O en an in antile hemangioma has le residual bro atty tissue, with or without damaged skin,
surrounded by an area o telangiectasias. Instead o removing the entire abnormal area, the lesion
should be managed by two methods to reduce the length o scar:
• elangiectasias are treated with a pulsed-dye laser.
• Damaged skin/ bro atty tissue is resected.
ypically I will rst treat the telangiectasias with a pulsed-dye laser and then per orm the resection
secondarily to give the smallest possible scar. Less commonly I will per orm the resection initially
and then treat peripheral telangiectasias later.
l e n t ic u l a r e xc is io n
Because an in antile hemangioma expands the skin, almost all lesions can be removed with len-
ticular excision and linear closure. I the entire area cannot be extirpated, serial resection can
be per ormed. I have never had to use a skin gra or f ap to reconstruct an area a er I removed
an in antile hemangioma. Although in antile hemangiomas bleed more than other types o skin
lesions, blood loss is minimized by in using a local anesthetic with epinephrine throughout the
surgical site. I the volume o local anesthetic with epinephrine is limited by the weight o the
child, I will in ltrate an epinephrine-only solution to ensure maximum vasoconstriction o the
operative site (1 cc o 1:000 epinephrine in 200 cc normal saline solution = 1:200,000 solution).
On the rare occasion that a proli erating lesion is removed, bleeding is more problematic, be-
cause the tumor is highly vascular, and an in ant is less tolerant o blood loss compared with an
older child. A er the area is in ltrated with epinephrine, part o the area is incised, bleeding is
controlled with cautery, and staged resection is continued rather than making the entire incision
initially. With the use o epinephrine and cautery, I have never had signi cant blood loss or had
a child undergo trans usion a er I per ormed a resection o an in antile hemangioma.
Lenticular excisions are used or most in antile hemangiomas, including circular lesions in loca-
tions avorable or a one-stage excision and linear closure. Because in antile hemangiomas expand
the skin, wide undermining is not usually necessary to obtain a tension- ree closure. Incisions

29
Chapter 4: Infantile Hemangioma
are closed with interrupted Vicryl sutures or the deep dermis and either interrupted chromic or
subcuticular Monocryl suture to approximate the epidermis and super cial dermis, because most
children cannot tolerate suture removal. T e incisions are dressed with cyanoacrylate glue, Steri-
Strips, and a gauze pressure dressing. I use 7-0 chromic sutures or acial lesions; suture marks
are not visible because the diameter o the suture is similar to pore sizes. In older children and
adolescents, I typically use interrupted nylon suture that is removed in the o ce. T e incisions
are then rein orced with Steri-Strips or 6 weeks to minimize widening o the scar. Patients are
instructed to avoid sun exposure or 12 months.
I a hemangioma is too large to resect in one stage, serial excision is per ormed. I usually per orm
the next stage 6 to 12 weeks later. I a large hemangioma is resected rom the upper extremity, a
so gauze and elastic dressing is applied to protect the area and limit the use o the arm. I signi -
icant wound tension exists, occasionally a splint is placed or 2 to 3 weeks. Extirpation o large le-
sions on the lower extremity is ollowed by placement o a knee immobilizer or 2 to 3 weeks. T e
child can remove the device when sleeping and showering. Previously I would place patients in a
cast but ound that the knee immobilizer is equally e ective and easier or patients and parents.
c ir c u l a r e xc is io n
Many in antile hemangiomas have a circular morphologic structure and are amenable to circular
excision and purse-string closure. In antile hemangiomas are particularly avorable or purse-
string closure, because they usually expand the surrounding skin. Lenticular excision o a circular
lesion will result in a scar two to three times its diameter, because the area is removed in an ellipse
to prevent dog-ears. Circular excision recruits the surrounding skin and gives a circular scar
that is one third to one hal the area o the in antile hemangioma. In my practice approximately
50% o patients and amilies are satis ed with the circular scar; it appears as a “pockmark” scar
( or example, rom acne or chicken pox). Other patients undergo a second stage to convert the
circular scar into a line. Because the circle is smaller than the original lesion, the nal scar is
shorter. Ultimately, the two-stage approach leaves a linear scar approximately the same length
as the diameter o the original in antile hemangioma. I the lesion is large, multiple purse-string
excisions and circular closures can be per ormed to continually reduce the area o the scar until
it is acceptable to convert into a line.
Another advantage o circular excision and purse-string closure is that it causes less de ormation
o surrounding structures compared with lenticular resection. Almost all in antile hemangiomas
that undergo circular excision are located on the ace, where it is most important to limit the
length o the scar and accept the morbidity o more than one procedure. Lenticular excisions
and linear scars on the trunk and extremities placed in relaxed skin-tension lines have avorable
appearances. I will per orm circular excisions outside o the ace i the lesion is very large in a
noticeable area ( or example, a orearm), or i I would like to minimize distortion o adjacent
structures. I will also use a circular skin excision over a large subcutaneous in antile hemangi-
oma to allow the best access to the lesion with the smallest scar. A er the subcutaneous tissue
is removed, the skin undergoes purse-string closure, and the circular scar is later converted to
a line. T e only location where a circular excision is contraindicated is the scalp. T e scalp is an
un avorable location or purse-string closure because:
• It has poor elasticity.
• Limiting the length o a linear scar is not important, because it is camouf aged by hair.
• A circular scar in the scalp is much more likely than linear cicatrix to cause visible alopecia
(especially in males).

30
Part II: Vascular Tumors
When per orming a circular excision, no margins should be included to maintain as much skin
as possible to close the wound under minimal tension. Wide skin undermining is carried out in
a 360-degree direction to recruit adjacent tissue. I typically use 4-0 or 5-0 Vicryl on the ace and
2-0 or 3-0 Vicryl on the trunk and extremity or the purse-string suture. T e less tension on the
closure, the more likely the patient will have a smaller circular scar. T e Vicryl suture is placed in
the deep dermis, and the epidermis/super cial dermis is approximated with chromic suture (5-0
or 6-0 in the ace; 3-0 or 4-0 in the trunk and extremity). Suture marks are di cult to appreciate
along the circular scar. T e area is then dressed with cyanoacrylate glue ollowed by Steri-Strips
and a pressure dressing (gauze with egaderm or tape). Steri-Strips are applied to the scar or
6 weeks a er surgery to prevent the circular scar rom widening. T e child is evaluated 6 to 12
months later to determine i he or she would bene t rom another circular excision or conversion
to a linear scar. In some locations the circular scar will mature in an orientation along a relaxed
skin-tension line.
s u c t io n -a s s is t e D l iPe c t o My
Liposuction can be used to remove subcutaneous bro atty residuum a er the hemangioma has
involuted. T e technique avoids a scar over the area, because the cannula can access the lesion
rom a remote 1 cm incision placed in an inconspicuous location. Suction-assisted lipectomy is
rarely used, because almost all in antile hemangiomas have damaged skin that needs to be re-
moved. However, i the integument is normal, liposuction should be considered. Suction-assisted
lipectomy o an involuted hemangioma is more di cult compared with removal o normal adi-
pose tissue, because the tissue is more brous and vascular. T ere is increased bleeding, and tissue
removal is less predictable than open excision. Consequently, I generally do not use liposuction
and instead pre er to make an incision and remove excess subcutaneous tissue sharply under
direct vision. I believe in the principle o trading scar or contour. Patients are most satis ed with
achieving the best symmetry, which is noticed beyond conversational distance at the expense o
a scar that can only be appreciated on close examination (similar to having a longer scar instead
o a shorter scar and dog-ears at each end).
Another option or removal o an in antile hemangioma o the subcutaneous cheek that has nor-
mal overlying skin is to per orm an intraoral resection. Similar to liposuction, this technique is
more di cult and less predictable than a direct cutaneous extirpation. In addition, this approach
has increased risk o in ection and injury to the acial nerve. As a result, I do not pre er intraoral
removal o subcutaneous tissues.
a n a t o Mic c o n s iDe r a t io n s
Sc a lp
Proli erating in antile hemangiomas o the scalp have an increased risk o ulceration because o
incidental trauma, particularly when the patient is sleeping. Indications to removal o an involuted
in antile hemangioma o the scalp are alopecia and/or residual bro atty tissue that is causing a
visible de ormity. Anetoderma and telangiectasias are camouf aged by hair and usually do not
require treatment. In antile hemangiomas damage hair ollicles, and thus lesions in the scalp will
cause an area o decreased hair density. I an ulceration occurred in in ancy, the resulting scar
will not have any hair growing through it. T e larger the size o the in antile hemangioma and

31
Chapter 4: Infantile Hemangioma
area o ulceration, the more likely the patient will have visible alopecia (especially in males who
have shorter hair).
An involuted in antile hemangioma causing a de ormity should be removed by lenticular excision
and linear closure. Limiting the length o the scar is not critical in the scalp, because it is hidden
by hair; circular excision and purse-string closure should not be per ormed. When the surgeon
per orms a linear closure, the scar should be as narrow as possible so that the adjacent hair is able
to cover it. Because Steri-Strips cannot be applied to an incision in the scalp to limit scar widening,
I place a galeal/intradermal PDS suture ollowed by interrupted cutaneous sutures.
Fo re h e a d
In antile hemangiomas located near the hairline can be removed with a lenticular excision and
linear closure, because the scar is camouf aged by hair. I possible, lenticular excisions should
be designed horizontally along the relaxed skin-tension lines. Lesions near the eyebrows and
glabella are also usually excised with a lenticular excision and linear closure. Scars can be placed
horizontally along the eyebrow or vertically extended toward the medial canthal area. In antile
hemangiomas located in the middle o the orehead are usually managed by circular excision and
purse-string closure to limit the length o the scar and to minimize distortion o the eyebrow.
No s e
Cutaneous in antile hemangiomas are best removed by circular excision and purse-string closure
to limit the scar burden on the nose. Subcutaneous nasal tip lesions require open rhinoplasty.
A er the skin is elevated, the underlying hemangioma is excised over the lower lateral cartilages.
Because the tumor usually separates the cartilage, the dome/middle crus o each lower lateral
cartilage is sutured together in the midline with 5-0 PDS suture. T e hemangioma has usually
expanded the overlying skin, and thus the skin is trimmed along the columella and rim incisions
be ore the surgeon closes the wound. I overlying skin discoloration remains, it is treated with a
pulsed-dye laser.
Lip
In antile hemangiomas o the cutaneous lip may be removed with circular excision to limit distor-
tion o structures in the area and length o the scar. However, some lesions are best removed along
one o the philtral columns or mucocutaneous junction in a linear ashion. I the hemangioma
has e aced the mucocutaneous junction and residual telangiectasias are present, I will rst treat
the area with a pulsed-dye laser to better de ne the cutaneous lip rom the red vermilion.
In antile hemangiomas a ecting only the vermilion and mucosa are removed with a transverse
mucosal incision. Usually the ellipse is centered along the junction o the keratinized and non-
keratinized vermilion. I the hemangioma has pre erentially expanded the keratinized vermilion
or mucosa, the elliptical resection is adjusted toward the most overgrown area. A er the surgeon
marks the outline o the planned resection, the incision lines are tattooed, because marker is
usually erased a er application o povidone-iodine (Betadine). Next, local anesthetic with epi-
nephrine is in used throughout the operative eld; a minimal amount is used, because too much
volume will distort the lip and increase the di culty o the procedure.

32
Part II: Vascular Tumors
A er the expanded skin and residual broadipose tissue are removed, the area should be slightly
overcorrected, because the postoperative swelling will create brosis that will contribute to lip
ullness. o minimize swelling, patients are given dexamethasone (Decadron) intraoperatively.
Be ore wound closure, the vermilion and mucosa are undermined and approximated with 4-0 or
5-0 Vicryl suture. Next, interrupted 6-0 chromic suture is used or the super cial closure and does
not leave suture marks in the vermilion. In the upper lip the wound should be closed starting at
the commissure and ending at the median tubercle, so that a dog-ear is in the midline where the
lip is ullest. In the lower lip the incision should be closed starting medially and ending laterally,
so that a dog-ear is hidden in the commissure. o protect the area rom dehiscence, the patient
is given a ull-liquid diet or 2 weeks.
Ch e e k
Most in antile hemangiomas o the cheek should be removed by circular excision and purse-string
closure to limit the length o the scar. Lenticular excision may be used or lesions located in the
preauricular area, where a aceli -like incision can be used. Large in antile hemangiomas may
bene t rom more than one circular excision to give the child the best possible outcome. I the
overlying skin is normal and the de ormity is caused by excessive subcutaneous tissue, suction-
assisted lipectomy can then be considered with a cannula placed through a preauricular incision.
However, direct resection over the area typically gives a more predictable and avorable outcome.
I do not advocate or intraoral resection o subcutaneous lesions.
Ea r
Involuted hemangiomas o the ear can be removed with a lenticular excision. Lesions that have
expanded the lobule may bene t rom circular excision, so that the ear lobe is reduced in two
dimensions. Retroauricular hemangiomas can result in a prominent ear de ormity because:
• Increased blood f ow causes overgrowth o cartilage.
• Posterior pressure o the cartilage away rom the mastoid will permanently de orm the
cartilage, which is moldable during the rst ew months o li e.
A prominent ear can be set back with a conchal bowl resection and/or scapha-conchal sutures.
Ne c k
In antile hemangiomas o the neck are usually resected with lenticular excisions, because linear
incisions are well concealed. T e neck has avorable relaxed skin-tension lines. T e surgeon should
not remove too much skin in this area. I a large residual lesion is present, it should be removed
by serial excision.
Tru n k
Most residual hemangiomas o the trunk are managed by lenticular excision. Occasionally a large
lesion o the chest can be removed as a circle to limit the length o the scar. I resection o a lesion
near the nipple/areola might injure the breast bud in a emale, the procedure is not per ormed
until the patient’s breast has completed growth (usually 16 years o age). In antile hemangiomas
involving the breast bud do not a ect breast development, but iatrogenic injury during resection
in childhood may inhibit breast growth, requiring later breast augmentation.