Download Pediatric Vascular Anomalies

Pediatric
Vascular Anomalies
Rameez Qudsi, Harvard Medical School
Gillian Lieberman, MD
Beth Israel Deaconess Medical Center
Children’s Hospital Boston
Agenda
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Index Patient Introduction
Disease Classification
Disease Descriptions
Imaging Workup
Treatment Options
Interventional Management
Index Patient: Baby B.G.
• History
• 2 mo M with left neck mass first seen prenatally at 37wk U/S. Pt born
via c/s at 39wk, otherwise unremarkable birth. Lesion has not changed
size since birth. No problems with growth, airway, feeding.
• Physical Exam
Companion Patient #1
• AVSS, boggy, 5x6cm left neck mass, no
overlying rash, warmth, or bruit.
• Brief Differential Diagnosis
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Vascular anomaly – most likely
Infection - smaller, transient, usually after birth
Solid tumor - (benign/malignant) – usually firm, midline
Branchial cleft cyst - later age, firm, smaller
Thyroglossal duct cyst - later age, central location
http://www.bago.com.bo/sbp/revista_
ped/vol41_2/imagenes/Image12.jpg
ISSVA Classification
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ISSVA - Int’l. Society for the Study of Vascular Anomalies
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Drs. Mulliken & Glowacki - Children’s Hospital Boston
Biologic Classification – differing course & treatments
Tumor vs Malformation
• “oma” = proliferation
• abnormal morphogenesis
• ↑ EC turnover /
hyperplasia, thick BM
• Normal EC, BM,
pathology
• ↑ Surface markers VEGF, bFGF, (PCNA)
• Minimal surface marker
expression
• Usually infancy/childhood
• Present at birth
• Naturally involuting
• Naturally persistent
• >3:1 female:male
• 1:1 female:male
Classes of Congenital Vascular
Anomalies
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Tumors
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Hemangiomas
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Infantile
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Congenital
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Tufted Hemangioma
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Hemangioendothelioma
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Acquired dermatologic
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Other syndromes
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Malformations
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Capillary
z Dermatologic
z Superficial laser tx
Lymphatic
z Microcystic
z Macrocystic
Venous
Arterial / Arteriovenous
Combined Forms / Syndromes
Hemangioma
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Benign endothelial cell tumor
z Tightly packed mass of vascular channels’
z 2 main types
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1. Infantile Hemangioma
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Usually has overlying patch of redness (superficial)
Most common tumor of infancy/childhood
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4-10% prevalence in Caucasian infants
3-5:1 females:males
Appears weeks/months after birth
Natural course - 3 stages
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1. Proliferating - first year
2. Involuting - few years
3. Involuted - most resolved by age 10
Children’s Hospital Boston
http://www.childrenshospital.or
g/clinicalservices/Site1964/main
pageS1964P8sublevel9.html
Hemangioma (cont.)
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2. Congenital Hemangioma
z Blue/gray hue w/ pale halo (skin)
z Rare (compared to infantile)
z Present at birth
z 2 types
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1. Non-Involuting (NICH) - persistent
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2. Rapidly Involuting (RICH) - resolved by 1-2 yrs
Children’s Hospital Boston
http://www.childrenshospital.or
g/clinicalservices/Site1964/main
pageS1964P8sublevel9.html
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Complications
z Ulceration, bleeding, infection, obstruction/displacement
of organs, high-output cardiac failure due to high
flow/shunting
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There are NO new-onset adult hemangiomas
Lymphatic Malformation (LM)
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Collection of lymph-filled
channels/cysts
Present at birth (5-6 wks G.A.)
↑ Swelling w/ infections
Soft w/ no overlying rash
Most common:
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1. head/neck
2. extremities/axilla
3. trunk
Children’s Hospital Boston
http://www.childrenshospital.org/az/Site1256/mainpageS1256P0.html
2 Types
1. Microcystic: multiple small vesicles
z 2. Macrocystic: few large septated cysts
z Complications: infection, bleeding, obstruction/displacement of organs,
overgrowth of involved tissue
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A.K.A. - “cystic hygroma”, “lymphangioma”
Venous Malformation (VM)
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Thin-walled, dilated veins
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Present at birth
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Companion Patients #2 and #3
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VM growth proportional to child’s
growth
Possible association with trauma,
hormones
Complications
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Children’s Hospital Boston
http://www.childrenshospital.org/az/Site1830/mainpageS183
0P0.html
Often unseen until symptomatic in
childhood
Soft w/ bluish skin hue
Waxing/waning size and symptoms
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Inadequate smooth muscle layer
Thrombosis, bleeding
A.K.A. - “cavernous hemangioma”
Arterio-Venous Malformation
(AVM)
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High-flow arterio-venous
communication - absence of
developed capillary bed
Present at birth
Reddish vascular hue (skin), often
warm
Complications
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Bleeding, compression /
displacement of organs, high-output
cardiac failure
Seen in hereditary hemorrhagic
telangiectasia (Osler-WeberRendu)
http://www.childrenshospital.org/az/Site593/main
pageS593P0.html
Baby B.G. - Focused DDx
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Review: soft left neck mass since birth, no change
in size, no warmth/redness
Narrowed Differential Diagnosis?
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Vascular anomaly
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Hemangioma?
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Infantile? - No - present since birth
Congenital? – Possible – too soon to distinguish NICH vs RICH
Lymphatic? – Possible
Venous? - Less likely but possible - no growth but only 2
months old, no bluish hue but not always present
Arterial / AV? - Less likely - no warmth/redness
With a focused differential diagnosis based on
history and physical, we proceed to radiologic
imaging to further characterize our patient’s
vascular malformation.
Imaging Options for Vascular
Malformations
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Ultrasound
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MRI
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Critical, often definitive
Radiographs
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Assess flow pattern
Limited benefit - bony structures, calcification
Quick and Cheap
Angiography
Imaging Workup Decision Tree
High flow
Mass-like
Hemangioma
MRI
No mass
AVM
Ultrasound
Low flow
MRI
Diffuse enhancement
with contrast
No/rim enhancement
with contrast
Venous
Lymphatic
Ultrasound Reference Images
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High Flow Lesions
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Hemangioma
Arterio-venous
Malformation
PACS, CHB, courtesy Dr. C. Johnson
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Low Flow Lesions
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Lymphatic Malformation
Venous Malformation
PACS, CHB, courtesy Dr. G. Chaudry
MRI Findings Table
Hemangioma
AVM
Lymphatic
Venous
T1
Isointense
Isointense
Hypointense
Hypo/isointense
T2
Hyperintense
Hyperintense
Hyperintense
Hyperintense
Post-contrast
Intense
enhancement
Intense
enhancement
No / Rim
enhancement
Diffuse
enhancement
MRI Images - High Flow Lesions
Axial MRI, T1
Axial MRI, T2 with fat saturation
*
Enjolras et al, Color Atlas of Vascular Tumors
and Vascular Malformations, Cambridge Univ.
Press 2007.
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Hemangioma
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PACS, CHB, courtesy Dr. C. Johnson
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Arterio-Venous Malformation
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Protruding mass (*)
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No mass
Flow voids – high-speed flow
Hemangioma
AVM
Lymphatic
Venous
T1
Isointense
Isointense
Hypointense
Hypo/isointense
T2
Hyperintense
Hyperintense
Hyperintense
Hyperintense
Post-contrast
Intense enhancement
Intense enhancement
No / Rim enhancement
Diffuse enhancement
MRI Images - Low Flow Lesions
Sagittal MRI, upper extremity
T1
T1 post-contrast
T2
Axial MRI
T1 post-contrast
w/ fat sat.
T1
*
www.imaging.consult.com
*
PACS, CHB, courtesy Dr. H. Padua
* Representative non-contrast image
PACS, CHB, courtesy Dr. C. Johnson
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Venous Malformation
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Diffuse enhancement w/ contrast
Lymphatic Malformation
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Septal (Left) / Rim (Rt) enhancement
Hemangioma
AVM
Lymphatic
Venous
T1
Isointense
Isointense
Hypointense
Hypo/isointense
T2
Hyperintense
Hyperintense
Hyperintense
Hyperintense
Post-contrast
Intense enhancement
Intense enhancement
No / Rim enhancement
Diffuse enhancement
Radiographic / (CT) Findings
Left upper
extremity
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Generally of limited use
Phleboliths seen w/ X-ray
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Calcifications
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Enjolras et al, Color Atlas of Vascular Tumors and Vascular Malformations,
Cambridge Univ. Press 2007.
Post venous thrombus
Suggest Venous
malformations
Angiography
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Performed to characterize AVM architecture
Encouraged prior to any injected therapy
No longer necessary for diagnosis of venous malformation
Arterio-venous
malformation,
Left thigh
PACS, CHB, courtesy Dr. C. Johnson
Venous
malformation,
Rt upper extremity
PACS, CHB, courtesy Dr. Watanabe
Baby B.G.’s Radiologic
Studies and Diagnosis
PACS, CHB, courtesy Dr. Padua
Radiograph
Ultrasound L Neck
Ultrasound w/ Doppler
• No phlebolith
• Low flow
• Large cysts
• T1 hypointense
Axial MRI L Neck, T1
(contrast study not performed at
outside referring hospital)
Axial MRI L Neck, T2
• Final Diagnosis?
Macrocystic Lymphatic Malformation
Further Workup Options
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Biopsy
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Aspirate of lesion
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Pathology / Microbiology
Blood vs Lymph
Pathology / Microbiology
Molecular Markers
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Of lesion sample
Of patient’s serum/urine
Treatment Options
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Observation
Dermatologic
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Pharmacologic
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Laser therapy – capillary malformation
Hemangioma – steroids, IFN-α, vincristine
Surgical (excision)
Interventional Radiology (IR): minimally invasive
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Sclerotherapy - LM & VM (low-flow lesions)
Embolization - AVM
Sclerotherapy Overview
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Primary IR treatment for VM/LM
Intralesional injection of irritant/sclerosant
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U/S & fluoroscopically guided
Induces fibrosis, contraction over 4-8 weeks
Sclerosants
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Doxycycline: sufficient for LM
Bleomycin: experimental for microcystic LM
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Theoretical concern for systemic effects – pulmonary fibrosis
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Sodium Tetradecyl Sulfate (STS): detergent for VM/LM
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OK-432: experimental, lyophilized S. pyogenes cells
EtOH: avoided in children
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Sclerotherapy Setup
Dr. Konez, http://www.birthmarks.us/sclerotherapy.htm
U/S Pre & Post VM Sclerotherapy
PACS, CHB, courtesy Dr. C. Johnson
U/S Normal Muscle
Fascicular horizontal
lines noted
U/S Pre STS
Venous channels
circled, fibrotic
(grainy echogenicity)
muscle surrounding
4-6wks Post Sclero
Reduced channel size,
sclerotic/fibrotic
muscle surrounding
Baby B.G. Sclerotherapy:
Left Neck Ultrasound
Lymphatic
Macrocyst
(Fluid = black)
*
Injection
Doxycycline
after fluid
aspiration
U/S guided
needle
insertion
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PACS, CHB, courtesy Dr. Padua
Post
injection
Baby B.G. Sclerotherapy:
Fluoroscopy
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PACS, CHB, courtesy Dr. H. Padua
Fluoroscopy – Doxycycline
injection of cyst
Post injection with
contrast/sclerosant filled cyst
Pre & Post Doxycycline for LM
Companion Patient #4
P. Burrows et al. Percutaneous sclerotherapy of lymphatic malformations with doxycycline. Lymphatic Research and Biology. December
2008, 6(3-4): 209-216.
Representative images of neck lymphatic malformation
pre and 2 years post sclerotherapy with doxycycline
Pre & Post Sclerotherapy for VM
Companion Patient #5
Children’s Hospital Boston
http://www.childrenshospital.org/az/Site1830/mainpageS1830P0.html
Summary
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Classification: Tumor vs Malformation
Imaging Workup
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1. Ultrasound - High vs Low Flow
2. MRI - T2/T1, contrast enhancing (blood), flow voids (high flow)
Hemangioma
AVM
Lymphatic
Venous
T1
Isointense
Isointense
Hypointense
Hypo/isointense
T2
Hyperintense
Hyperintense
Hyperintense
Hyperintense
Post-contrast
Intense enhancement
Intense enhancement
No / Rim enhancement
Diffuse enhancement
Treatment: Pharmacologic, Surgical, Interventional
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Hemangioma - Steroids
Lymphatic Malformation - Surgery / Sclerotherapy (Doxycycline)
Venous Malformation - Surgery / Sclerotherapy (STS)
Arterial - Embolization
Acknowledgements
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Dr. Craig Johnson – CHB IR
Dr. Meguru Watanabe – CHB IR
Dr. Horacio Padua – CHB IR
Dr. Gulraiz Chaudry – CHB IR
Dr. Ahmad Alomari – CHB IR
Dr. Steven Fishman – CHB Surgery
Dr. Diana Rodriguez – CHB Radiology
Dr. Gillian Lieberman – BIDMC Radiology
Maria Levantakis – BIDMC Radiology
References
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O. Enjolras, M. Wassef, R. Chapot. Color Atlas of Vascular Tumors and Vascular
Malformations. Cambridge University Press, 2007.
G. Fleisher, S Ludwig, F. Henretig, R. Ruddy, B. Silverman. Pediatric Emergency
Medicine. Lippincott Williams & Wilkins, 2005.
L. Donnelly. Pediatric Imaging, The Fundamentals. Saunders Elsevier, 2009.
P. Burrows, R. Mitri, A. Alomari, H. Padua, D. Lord, M. Sylvia, S. Fishman, J.
Mulliken. Percutaneous sclerotherapy of lymphatic malformations with
doxycycline. Lymphatic Research and Biology. December 2008, 6(3-4): 209-216.
Children’s Hospital Boston. Vascular Anomalies Center.
http://www.childrenshospital.org/clinicalservices/Site1964/mainpageS1964P0.html
M. Cohen, S. Maimon, D. Ben-Amitai, E. Bensimon. Vascular, Lymphatic
Malformations. Emedicine from WebMD.
http://emedicine.medscape.com/article/1296163-overview
Konez, Orhan. Hemangiomas and Vascular Anomalies. http://www.birthmarks.us/
Imaging Consult. Elsevier Health, 2009. http://imaging.consult.com/
Thank You
Questions?
Contact [email protected]