Pediatric Vascular Anomalies

Rameez Qudsi, Harvard Medical SchoolGillian Lieberman, MD

Beth Israel Deaconess Medical CenterChildren’s Hospital Boston

Agenda

• 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• AVSS, boggy, 5x6cm left neck mass, no

overlying rash, warmth, or bruit.• Brief Differential Diagnosis

• 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

Companion Patient #1

ISSVA ClassificationISSVA - Int’l. Society for the Study of Vascular Anomalies

Drs. Mulliken & Glowacki - Children’s Hospital BostonBiologic Classification – differing course & treatments

Tumor vs Malformation

• “oma” = proliferation• ↑ EC turnover / hyperplasia, thick BM• ↑ Surface markers - VEGF, bFGF, (PCNA)• Usually infancy/childhood• Naturally involuting• >3:1 female:male

• abnormal morphogenesis • Normal EC, BM, pathology• Minimal surface marker expression• Present at birth• Naturally persistent• 1:1 female:male

Classes of Congenital Vascular Anomalies

TumorsHemangiomas

InfantileCongenital

Tufted HemangiomaHemangioendotheliomaAcquired dermatologicOther syndromes

MalformationsCapillary

DermatologicSuperficial laser tx

LymphaticMicrocysticMacrocystic

VenousArterial / ArteriovenousCombined Forms / Syndromes

HemangiomaBenign endothelial cell tumor

Tightly packed mass of vascular channels’2 main types

1. Infantile HemangiomaUsually has overlying patch of redness (superficial)Most common tumor of infancy/childhood

4-10% prevalence in Caucasian infants3-5:1 females:males

Appears weeks/months after birthNatural course - 3 stages

1. Proliferating - first year2. Involuting - few years3. Involuted - most resolved by age 10

Children’s Hospital Boston http://www.childrenshospital.or

g/clinicalservices/Site1964/main pageS1964P8sublevel9.html

Hemangioma (cont.)2. Congenital Hemangioma

Blue/gray hue w/ pale halo (skin)Rare (compared to infantile)Present at birth2 types

1. Non-Involuting (NICH) - persistent2. Rapidly Involuting (RICH) - resolved by 1-2 yrs

Complications Ulceration, bleeding, infection, obstruction/displacement of organs, high-output cardiac failure due to high flow/shunting

There are NO new-onset adult hemangiomas

Children’s Hospital Boston http://www.childrenshospital.or

g/clinicalservices/Site1964/main pageS1964P8sublevel9.html

Lymphatic Malformation (LM)Collection of lymph-filled channels/cystsPresent at birth (5-6 wks G.A.)↑ Swelling w/ infectionsSoft w/ no overlying rashMost common:

1. head/neck 2. extremities/axilla3. trunk

2 Types1. Microcystic: multiple small vesicles2. Macrocystic: few large septated cysts

Complications: infection, bleeding, obstruction/displacement of organs, overgrowth of involved tissueA.K.A. - “cystic hygroma”, “lymphangioma”

Children’s Hospital Boston http://www.childrenshospital.org/az/Site1256/mainpageS1256P0.html

Venous Malformation (VM)Thin-walled, dilated veins

Inadequate smooth muscle layerPresent at birth

Often unseen until symptomatic in childhood

Soft w/ bluish skin hueWaxing/waning size and symptoms

VM growth proportional to child’s growthPossible association with trauma, hormones

ComplicationsThrombosis, bleeding

A.K.A. - “cavernous hemangioma”Children’s Hospital Boston http://www.childrenshospital.org/az/Site1830/mainpageS183

0P0.html

Companion Patients #2 and #3

Arterio-Venous Malformation (AVM)

High-flow arterio-venous communication - absence of developed capillary bedPresent at birthReddish vascular hue (skin), often warmComplications

Bleeding, compression / displacement of organs, high-output cardiac failure

Seen in hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu) http://www.childrenshospital.org/az/Site593/main

pageS593P0.html

Baby B.G. - Focused DDx

Review: soft left neck mass since birth, no change in size, no warmth/rednessNarrowed Differential Diagnosis?

Vascular anomalyHemangioma?

Infantile? - No - present since birthCongenital? – Possible – too soon to distinguish NICH vs RICH

Lymphatic? – PossibleVenous? - Less likely but possible - no growth but only 2 months old, no bluish hue but not always presentArterial / 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

Ultrasound Assess flow pattern

MRICritical, often definitive

RadiographsLimited benefit - bony structures, calcificationQuick and Cheap

Angiography

Imaging Workup Decision Tree

Venous

Hemangioma

AVMUltrasound

Lymphatic

Low flow

Mass-like

No mass

Diffuse enhancement with contrast

No/rim enhancement with contrast

Ultrasound Reference Images

High Flow LesionsHemangiomaArterio-venous Malformation

Low Flow LesionsLymphatic MalformationVenous Malformation

PACS, CHB, courtesy Dr. C. Johnson

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

HemangiomaProtruding mass (*)

Axial MRI, T2 with fat saturation

Arterio-Venous MalformationNo massFlow voids – high-speed flow

Post-contrast Intense enhancement Intense enhancement No / Rim enhancement Diffuse enhancement

Enjolras et al, Color Atlas of Vascular Tumors

and Vascular Malformations, Cambridge Univ. Press 2007.

Axial MRI, T1

MRI Images - Low Flow Lesions

Venous MalformationDiffuse enhancement w/ contrast

Sagittal MRI, upper extremityT1 T1 post-contrast T2

Lymphatic MalformationSeptal (Left) / Rim (Rt) enhancement

Axial MRI

T1 post-contrastw/ fat sat. T1

PACS, CHB, courtesy Dr. H. Padua* Representative non-contrast image

www.imaging.consult.com

Radiographic / (CT) Findings

Generally of limited usePhleboliths seen w/ X-ray

Calcifications Post venous thrombus

Suggest Venous malformations

Enjolras et al, Color Atlas of Vascular Tumors and Vascular Malformations,

Cambridge Univ. Press 2007.

Left upper extremity

AngiographyPerformed to characterize AVM architectureEncouraged prior to any injected therapyNo longer necessary for diagnosis of venous malformation

PACS, CHB, courtesy Dr. Watanabe

Arterio-venous malformation, Left thigh

Venous malformation,

Rt upper extremity

Baby B.G.’s Radiologic Studies and Diagnosis

PACS, CHB, courtesy Dr. Padua

Axial MRI L Neck, T2Axial MRI L Neck, T1(contrast study not performed at

outside referring hospital) Macrocystic Lymphatic Malformation

• No phlebolith

• Low flow

• Large cysts

• T1 hypointense

• Final Diagnosis?

Radiograph Ultrasound L Neck Ultrasound w/ Doppler

Further Workup Options

BiopsyPathology / Microbiology

Aspirate of lesionBlood vs LymphPathology / Microbiology

Molecular MarkersOf lesion sampleOf patient’s serum/urine

Treatment Options

ObservationDermatologic

Laser therapy – capillary malformationPharmacologic

Hemangioma – steroids, IFN-α, vincristineSurgical (excision)Interventional Radiology (IR): minimally invasive

Sclerotherapy - LM & VM (low-flow lesions)Embolization - AVM

Sclerotherapy OverviewPrimary IR treatment for VM/LMIntralesional injection of irritant/sclerosant

U/S & fluoroscopically guidedInduces fibrosis, contraction over 4-8 weeks

SclerosantsDoxycycline: sufficient for LMBleomycin: experimental for microcystic LM

Theoretical concern for systemic effects – pulmonary fibrosisSodium Tetradecyl Sulfate (STS): detergent for VM/LMOK-432: experimental, lyophilized S. pyogenes cellsEtOH: avoided in children

Sclerotherapy Setup

Dr. Konez, http://www.birthmarks.us/sclerotherapy.htm

U/S Pre & Post VM Sclerotherapy

U/S Normal MuscleFascicular horizontal

lines noted

U/S Pre STSVenous channels circled, fibrotic

(grainy echogenicity) muscle surrounding

4-6wks Post ScleroReduced channel size,

sclerotic/fibrotic muscle surrounding

Baby B.G. Sclerotherapy: Left Neck Ultrasound

U/S guidedneedle

insertion

InjectionDoxycycline

after fluid aspiration

Postinjection

LymphaticMacrocyst

(Fluid = black)

PACS, CHB, courtesy Dr. Padua

Baby B.G. Sclerotherapy: Fluoroscopy

Fluoroscopy – Doxycycline injection of cyst

Post injection with contrast/sclerosant filled cyst

PACS, CHB, courtesy Dr. H. Padua

Pre & Post Doxycycline for LM

Representative images of neck lymphatic malformationpre and 2 years post sclerotherapy with doxycycline

P. Burrows et al. Percutaneous sclerotherapy of lymphatic malformations with doxycycline. Lymphatic Research and Biology. December 2008, 6(3-4): 209-216.

Pre & Post Sclerotherapy for VM

Children’s Hospital Boston http://www.childrenshospital.org/az/Site1830/mainpageS1830P0.html

Summary

Classification: Tumor vs MalformationImaging Workup

1. Ultrasound - High vs Low Flow2. MRI - T2/T1, contrast enhancing (blood), flow voids (high flow)

Treatment: Pharmacologic, Surgical, InterventionalHemangioma - SteroidsLymphatic Malformation - Surgery / Sclerotherapy (Doxycycline)Venous Malformation - Surgery / Sclerotherapy (STS)Arterial - Embolization

Acknowledgements

Dr. Craig Johnson – CHB IRDr. Meguru Watanabe – CHB IRDr. Horacio Padua – CHB IRDr. Gulraiz Chaudry – CHB IRDr. Ahmad Alomari – CHB IRDr. Steven Fishman – CHB Surgery

Dr. Diana Rodriguez – CHB RadiologyDr. Gillian Lieberman – BIDMC RadiologyMaria Levantakis – BIDMC Radiology

References

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.htmlM. Cohen, S. Maimon, D. Ben-Amitai, E. Bensimon. Vascular, Lymphatic Malformations. Emedicine from WebMD. http://emedicine.medscape.com/article/1296163-overviewKonez, Orhan. Hemangiomas and Vascular Anomalies. http://www.birthmarks.us/Imaging Consult. Elsevier Health, 2009. http://imaging.consult.com/

Thank You

Questions?Contact qudsi@post.harvard.edu

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