Vascular anomalies.pptx

Vascular anomalies, from
diagnosis to treatment
Mohamed M.A. Zaitoun, MD
Associate Professor of Interventional Radiology
Faculty of Medicine, Zagazig University, Egypt
FINR-Switzerland
Interventional Radiology Unit, Zagazig University Hospitals, Egypt
www.zaitounclinic.com
zaitoun2015@gmail.com

Disclosure
I have no actual or potential conflict of
interest in relation to this presentation.

Introduction
Classifications
Hemangiomas
Vascular Malformations
Post Treatment Appearance

Introduction
Vascular malformations and tumors comprise a
wide, heterogeneous spectrum of lesions that
involve all parts of the body and can cause
significant morbidity and even mortality in
both adults and children.
Vascular lesions represent the most common
cause of pediatric soft-tissue masses.
Navarro OM, Laffan EE, Ngan BY. Pediatric softtissue tumors and pseudotumors: MR imaging features with pathologic correlation. I.
Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. RadioGraphics 2009;29(3):88

The term hemangioma has been applied generically
to vascular lesions of differing cause and clinical
behavior.
Occasionally, confusion about terminology and
imaging guidelines continues to be responsible
for improper diagnosis and subsequent
treatment.
Since treatment strategy depends on the type of
malformation, correct diagnosis and classification
of a vascular anomaly are crucial.
Mulliken JB, Fishman SJ, Burrows PE. Vascular anomalies. Curr Probl Surg 2000;37(8):517–584.
. Hand JL, Frieden IJ. Vascular birthmarks of infancy: resolving nosologic confusion. Am J Med Genet 2002;108(4):257–264.

Our objective is to review the current
classification of vascular anomalies, to
describe the role of imaging in their diagnosis,
to summarize their distinctive
histopathogenic, clinical and imaging features,
and to discuss the treatment options.

Classification
Several classification systems have been
proposed for vascular anomalies:
1-Mulliken and Glowacki Classification
2-Jackson et al Classification
3-ISSVA Classification

1-Mulliken and Glowacki Classification
It is a biologic classification based on cellular
turnover, histologic features, natural history,
and physical findings.
They classified vascular anomalies as either
hemangiomas or vascular malformations.
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial
characteristics. Plast Reconstr Surg 1982;69(3):412–422.

Hemangiomas are auto-involutive, benign,
vascular tumors of infancy and childhood,
characterized by rapid growth of endothelial
cells and subsequent slow involution.
In comparison, vascular malformations arise
from dysplastic vessels without endothelial
proliferation.

They never regress, and, depending on flow
(high- or low-flow).
According to the preponderant vascular
channels, vascular malformations are
classified as venous, lymphatic, capillary,
arterial, or combined.
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial
characteristics. Plast Reconstr Surg 1982;69(3):412–422.
Moukaddam H, Pollak J, Haims AH. MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol
2009;38(6): 535–547.
Dubois J, Alison M. Vascular anomalies: what a radiologist needs to know. Pediatr Radiol 2010;40(6): 895–905.

https://en.wikipedia.org/wiki/Endothelium

2-Jackson et al Classification
in 1993 Jackson et al proposed a radiologic
classification formulated in combination with
the biologic classification of Mulliken and
Glowacki.
This calssification subcategorized vascular
malformations according to their flow
dynamics as low-flow or high-flow
malformations.

Jackson IT, Carreño R, Potparic Z, Hussain K. Hemangiomas, vascular malformations, and lymphovenous malformations: classification
and methods of treatment. Plast Reconstr Surg 1993;91(7): 1216–1230.

3-ISSVA Classification
In 1996, these systems were adopted and
expanded by the International Society for the
Study of Vascular Anomalies (ISSVA).
Two categories of vascular anomalies are
considered:
a) Vascular tumors (with infantile hemangioma
being the most common).
b) Vascular malformations.

Vascular malformations are subcategorized
according to their flow dynamics as:
(a) Low-flow malformations:
Venous
Lymphatic
Capillary
Capillary-venous
Capillary-lymphatic-venous.

(b) High-flow malformations:
Arteriovenous malformations (AVMs)
Arteriovenous fistulas (AVFs).
Thus, any malformation with an arterial
component is considered high flow, while
those without an arterial component are
considered low flow.
Enjolras O. Classification and management of the various superficial vascular anomalies: hemangiomas and vascular malformations. J
Dermatol 1997;24 (11):701–710.

Flors L, Park AW, Norton PT, Hagspiel KD, Leiva-Salinas C. Soft-tissue vascular malformations and tumors. Part 1: classification, role of
imaging and high-flow lesions. Malformaciones vasculares y tumores de partes blandas. Parte 1: clasificación, papel de las pruebas
imagen y lesiones de alto flujo. Radiologia (Engl Ed). 2019;61(1):4-15.

Flors L, Leiva-Salinas C, Maged IM, et al. MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy
follow-up. Radiographics. 2011;31(5):1321-1341.

Hemangiomas
The term hemangioma is used to designate a
group of benign endothelial neoplasms that
includes:
1-Infantile Hemangioma (common hemangioma
of infancy)
2-Congenital Hemangioma
3-Kaposiform Hemangioendothelioma
Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. RadioGraphics 2009;29(3):887–906

1-Infantile Hemangioma
Incidence
Clinical presentation
Imaging features
Treatment

Incidence
The most common vascular tumor of infancy,
with a prevalence of about 2-3% in all children
and a female predominance (female-to-male
ratio, 3-5:1).
The prevalence is even higher (10%) in
premature infants of very low birth weight.
Ernemann U, Kramer U, Miller S, et al. Current concepts in the classification, diagnosis and treatment of vascular anomalies. Eur J Radiol
2010;75 (1):2–11.

The most common location is the face and neck
(60% of cases), followed by the trunk (25%)
and extremities (15%).
Normally not yet visible at birth but manifest
during the 1st few weeks as rapidly growing
lesions, often becoming evident by 3 months
of age as subcutaneous bluish red masses that
resemble the surface of a strawberry.
Dubois J, Garel L. Imaging and therapeutic approach of hemangiomas and vascular malformations in the pediatric age group. Pediatr
Radiol 1999;29 (12):879–893.
Moukaddam H, Pollak J, Haims AH. MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal
Radiol 2009;38(6): 535–547.

Red, raspberry-like appearance of a superficially
located infantile hemangioma
Sadick M, Müller-Wille R, Wildgruber M, Wohlgemuth WA. Vascular Anomalies (Part I): Classification and Diagnostics of Vascular
Anomalies. Gefäßanomalien (Teil I): Klassifikation und Diagnostik von Gefäßanomalien. Rofo. 2018;190(9):825-835.

(a) Proliferating hemangioma at 3 months of age, (b) Same
hemangioma at involution at 4 years of age.
Richter GT, Friedman AB. Hemangiomas and vascular malformations: current theory and management. Int J Pediatr.
2012;2012:645678.

(a) Segmental hemangioma in trigeminal (V3) distribution. (b) Same
hemangioma after 2 months of therapy with propranolol
Richter GT, Friedman AB. Hemangiomas and vascular malformations: current theory and management. Int J Pediatr. 2012;2012:645678.

Reflecting the characteristic high-flow
component of this phase, they show bruit,
pulsatility, and warmth.
After a proliferating phase in the 1st few
months, a slow but constant regression
(involuting phase) can be seen, with the
process usually being completed by 7-10 years
of age.
Dermatol 1997;24 (11):701–710.
Ernemann U, Kramer U, Miller S, et al. Current concepts in the classification, diagnosis and treatment of vascular anomalies. Eur J
Radiol 2010;75 (1):2–11.

During that time, the hemangioma changes
color to grayish dark red, loses its toughness,
and alters in shape, developing into a
fibrofatty residuum.
Dermatol 1997;24 (11):701–710.
Radiol 2010;75 (1):2–11.

Histologically, in the proliferating phase,
hemangiomas consist of hyperplastic
proliferating endothelial cells that form
syncytial masses with increased turnover and
increased number of mast cells.
Later, the involuting phase shows progressive
perivascular deposition of fibrofatty tissue and
thinning of the endothelial lining.
Radiol 1999;29 (12):879–893.
Radiol 2009;38(6): 535–547.

Imaging features
a) Ultrasound:
The appearance on gray-scale US varies, but a
solid soft tissue mass is identified with
characteristic high vascularity on color
Doppler.
Arterial and venous waveforms can be seen on
spectral Doppler ultrasound.

The arterial flow is typically of low resistance
with relatively high velocities.
During the involuting phase, they show
decreased vascularity and increased vascular
resistance.
Paltiel HJ, Burrows PE, Kozakewich HP, Zurakowski D, Mulliken JB. Soft-tissue vascular anomalies: utility of US for diagnosis. Radiology.
2000;214:747---54.
Johnson CM, Navarro OM. Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 2: vascular malformations.
Pediatr Radiol. 2017;47:1196---208

Infantile hemangioma of the left parotid region showing
well-vascularized echogenic lesion on color Doppler
Sadick M, Müller-Wille R, Wildgruber M, Wohlgemuth WA. Vascular Anomalies (Part I): Classification and Diagnostics of Vascular
Anomalies. Gefäßanomalien (Teil I): Klassifikation und Diagnostik von Gefäßanomalien. Rofo. 2018;190(9):825-835.

b) MRI: Differ according to the biologic phase.
Proliferating phase:
they present as well-defined masses, hypointense
on T1 and hyperintense on T2, often with
presence of internal flow voids on spin-echo (SE)
imaging reflecting high-flow vessels.
High-flow vessels appear hyperintense on GRE
permitting the distinction with phlebolitis or
other calcifications, which are hypointense on all
imaging sequences.
. Flors L, Leiva-Salinas C, Maged IM, Norton PT, Matsumoto AH, Angle JF, et al. MR imaging of soft-tissue vascular malformations:
diagnosis, classification, and therapy follow-up. Radiographics. 2011;31:321---40 [discussion 1340-1].

perilesional edema should never be seen; if
present, other soft tissue tumors (e.g.
metastases from neuroblastoma or
rhabdomyosarcoma among many others)
should be suspected and biopsy is needed.
Abernethy LJ. Classification and imaging of vascular malformations in children. Eur Radiol. 2003;13:24
Dubois J, Alison M. Vascular anomalies: what a radiologist needs to know. Pediatr Radiol. 2010;40:895---905.

Early homogenous contrast enhancement is
characteristic during the proliferative phase,
and large feeding arteries are usually depicted
with time-resolved MRA.
Despite the high-flow nature of the
hemangioma during this phase, arteriovenous
shunting is not seen, whereas it is in AVMs.
Navarro OM, Laffan EE, Ngan BY. Pediatric soft-tissue tumors and pseudo-tumors: MR imaging features with pathologic correlation: part
1. Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. Radiographics. 2009;29: 887---906.
Moukaddam H, Pollak J, Haims AH. MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol
2009;38(6): 535–547.

1-Month-old infant with proliferating infantile hemangioma in the left supraclavicular
region. Coronal T1 (a) shows a well-defined lobulated hypointense mass, the mass is
hyperintense on STIR (b), signal voids within the lesion, reflecting fast flow vessels
(arrows), are also seen on these images (a, b). No perilesional edema is identified.
Arterial phase 3DMRA (c) image shows the characteristic early enhancement of the
lesion without arterio-venous shunting.

Proliferating infantile
hemangioma in a 1-year-old
infant.
(a) Photograph shows a lobulated
mass in the right breast with
superficial involvement, which
causes its strawberry-like
appearance.
(b) Axial T1 shows the well-
defined lobulated hypointense
mass in the right breast.
Signal voids in the lesion (arrow)
represent fast-flow vessels.
No perilesional edema is
identified.
(c) On a STIR, the mass is hyper-
intense. Arrow = signal voids in
the lesion.
(d) Image from arterial phase 3D
MR angiography shows
characteristic early enhancement
of the lesion without
arteriovenous shunting.
Flors L, Leiva-Salinas C, Maged IM, et al. MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-
up. Radiographics. 2011;31(5):1321-1341.

Involuting phase:
They appear as heterogeneous masses with
progressive deposition of internal fat
(hyperintense foci on T1), decreased flow
voids when compared to the proliferative
phase, and more heterogeneous contrast
enhancement.
Navarro OM, Laffan EE, Ngan BY. Pediatric soft-tissue tumors and pseudo-tumors: MR imaging features with pathologic correlation: part
1. Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. Radiographics. 2009;29: 887---906.
Restrepo R. Multimodality imaging of vascular anomalies. Pediatr Radiol. 2013;43 Suppl. 1:S141---54

Finally, after complete involution, a residual scar
is seen which appears hypointense on both T1
and T2.
Restrepo R. Multimodality imaging of vascular anomalies. Pediatr Radiol. 2013;43 Suppl. 1:S141—54.

Treatment
In most cases, no treatment is required because
of spontaneous involution.
Treatment may be needed when the
hemangioma is symptomatic or occurs in
regions where there is possible secondary loss
of function or lifetime aesthetic impairment.

Medical treatment is usually attempted first,
with propranolol used as a first-line therapy
with excellent results.
it reduces the expression of VEGF and other
proangiogenic factors while also inducing
apoptosis of vascular endothelial cells;
excellent results have been reported.
Léauté-Labrèze C, Taïeb A. Efficacy of betablockers in infantile capillary haemangiomas: the physiopathological significance and
therapeutic consequences [in French]. Ann Dermatol Venereol 2008;135(12):860–862.
Mulligan PR, Prajapati HJ, Martin LG, Patel TH. Vascular anomalies: classification, imaging characteristics and implications for
interventional radiology treatment approaches. Br J Radiol. 2014;87:20130392.

When propranolol is contraindicated, oral
prednisolone can be attempted, with complete
involution rate of 30% and stop progression in
40% of cases.
Embolization and surgery are reserved for
unresponsive cases.
Embolization can be performed for tumoral growth
control, preoperatively to reduce or minimize
bleeding, and for consumption coagulopathy.
Leaute-Labreze C, Taieb A. Efficacy of beta-blockers in infantile capillary haemangiomas: the physiopathological significance and
therapeutic consequences. Ann Dermatol Venereol. 2008;135:860-2.
Dubois J, Alison M. Vascular anomalies: what a radiologist needs to know. Pediatr Radiol. 2010;40:895-905.

2-Congenital Hemangioma
Incidence
Imaging features
Treatment

Incidence
A much less common vascular tumor, congenital
hemangioma is fully grown and clinically
evident at birth without gender
predominance.
Radiol 2009;38(6): 535–547.

They typically present as solitary lesions, most
commonly in the head and neck or the
extremities.
Two subtypes are identified:
*Rapidly involuting congenital hemangiomas
(RICHs):
Completely regress during the first 2 years of life.
*Non-involuting congenital hemangiomas (NICHs):
Demonstrate growth proportional to that of the
child without regression.
Restrepo R. Multimodality imaging of vascular anomalies. Pediatr Radiol. 2013;43 Suppl. 1:S141-54

Rapidly involuting congenital hemangiomas (RICH).
a RICH is fully formed at birth and then involutes, mostly during the first year of life, the patient’s
hematologic parameters were within the normal reference ranges.
b The same RICH involuted rapidly without any treatment, by 9 months of age, the lesion had involuted
completely, leaving dermal atrophy
Ji Y, Chen S, Yang K, Xia C, Li L. Kaposiform hemangioendothelioma: current knowledge and future perspectives. Orphanet J Rare Dis.
2020;15(1):39. Published 2020 Feb 3.

Imaging features
Due to important overlap, imaging features
alone do not allow differentiating congenital
from infantile hemangioma, and clinical
history is paramount.
Vascular aneurysms, intravascular thrombi, an
increased venous component, and
arteriovenous shunting are some distinctive
findings.
Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. RadioGraphics 2009;29(3):887–906.
Radiol 2009;38(6): 535–547.

1-Day-old infant with congenital hemangioma in the left scapular area.
Gray scale ultrasound image (a) shows a well-defined, solid soft-tissue mass.
High vascularity is demonstrated with color Doppler (b).
imaging and high-flow lesions. Malformaciones vasculares y tumores de partes blandas. Parte 1: clasificación, papel de las pruebas imagen y
lesiones de alto flujo. Radiologia (Engl Ed). 2019;61(1):4-15.

1-Day-old infant with congenital hemangioma in the left scapular area. A well
defined soft tissue mass is seen on MR images which is hypointense on
Coronal T1 (a) and hyperintense on STIR (b).
Signal voids within the lesion, reflecting fast flow vessels (arrows), are also
seen on these images (a, b).
No perilesional edema is identified.
Arterial phase contrast enhanced MRA (c) image shows the characteristic
early enhancement of the lesion.

Treatment
NICH require treatment and surgical resection is
the treatment of choice because embolization
is usually not effective.

3-Kaposiform Hemangioendothelioma
Incidence
Imaging features
Treatment

Incidence
Kaposiform Hemangioendothelioma (KHE) is a
rare locally aggressive tumor, usually present
at birth.
Lalaji TA, Haller JO, Burgess RJ. A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging. Pediatr
Radiol. 2001;31:876---8.

They typically occur in the first decade of life, and
most commonly involve the peritoneal or
retroperitoneal space, upper and lower
extremities, and head and neck region.
Patients may present Kasabach-Merritt syndrome
(KMP), a potentially life threatening
thrombocytopenia, anemia and coagulopathy.
The tumor has a very low malignant potential but,
regional nodal metastases can be seen rarely.
Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. RadioGraphics 2009;29(3):88
Radiol 2009;38(6): 535–547.

The manifestations of KHE are variable and
range from cutaneous lesions with wide
varieties of appearances to deep masses
without cutaneous signs.
In the majority of patients, KHE is a single soft
tissue mass with cutaneous findings that
range from an erythematous papule, plaque,
or nodule to an indurated, purple and firm
tumor.
Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB, et al. Kaposiform hemangioendothelioma: atypical features
and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr. 2013;162(1):142–7.
Ji Y, Yang K, Peng S, Chen S, Xiang B, Xu Z, et al. Kaposiform haemangioendothelioma: clinical features, complications and risk factors
for Kasabach-Merritt phenomenon. Br J Dermatol. 2018;179(2):457–63.

With KMP, these lesions are purpuric, hot to the
touch, swollen and very painful.
Most patients experience progressive lesion
enlargement and/or symptom progression,
however, a small but significant minority of
KHEs don’t grow.
Gruman A, Liang MG, Mulliken JB, Fishman SJ, Burrows PE, Kozakewich HPW, et al. Kaposiform hemangioendothelioma without Kasabach-
Merritt phenomenon. J Am Acad Dermatol. 2005;52(4):616–22.
Rodriguez V, Lee A, Witman PM, Anderson PA. Kasabach-Merritt Phenomenon. J Pediatr Hematol Oncol. 2009;31(7):522–6
Chan S, Cassarino DS. Rapidly enlarging “bruise” on the Back of an infant. JAMA Dermatol. 2013;149(11):1337
Zhang G, Gao Y, Liu X. Kaposiform haemangioendothelioma in a nine-yearold boy with Kasabach-Merritt phenomenon. Br J Haematol.
2017;179(1):9

A 3-month-old male infant presenting KHE associated with KMP.
The tumor lesion grew progressively after the first week of age and extended
through the whole thigh, scrotum and abdominal wall, with ‘extensive
thrombocytopenic purpura’.
2020;15(1):39. Published 2020 Feb 3.

Imaging features
Ill-defined margins, smaller feeding and draining
vessels, involvement of multiple tissue planes,
hemosiderin deposits, and destructive
changes are some distinctive MR imaging
findings from those of infantile hemangioma.
Radiol 2009;38(6): 535–547.

Clinical and MRI
features of KHE with KMP.
a A 2-month-old boy was
found to have a chest wall
mass after birth.
The mass became
progressively indurated and
purpuric.
The boy developed
profound
thrombocytopenia and
consumptive coagulopathy.
b Axial T1 revealed that the
heterogeneous mass was
isointense relative to the
adjacent muscle on T1.
Axial (c) and sagittal (d) T2
revealed hyperintense
lesions infiltrating the right
lateral chest wall
2020;15(1):39. Published 2020 Feb 3.
A
B
C
D

Treatment
Multi-modality approach including surgical
resection, steroids, interferon, vincristine or
radiation therapy, has been attempted, but
resulted in variable effectiveness.
. Nozaki T, Nosaka S, Miyazaki O, Makidono A, Yamamoto A, Niwa T, et al. Syndromes associated with vascular tumors and malformations:
a pictorial review. Radiographics. 2013;33: 175---95.

Vascular Malformations
Vascular malformations are congenital
anomalies and are thus present at birth,
although not always evident.
They usually grow proportionally with the child
and show no regression.
Their growth can be exacerbated due to
hormonal changes during puberty or
pregnancy or as a result of thrombosis,
infection, trauma, or incomplete treatment.

Unlike hemangiomas, they may be infiltrative
and usually involve multiple tissue planes.
Classified into high flow and low flow, the latter
category accounts for more than 90% of
vascular lesions outside the central nervous
system.
This differentiation based on flow dynamics is
vital to planning surgical or image-guided
treatment procedures.
Radiol 2009;38(6): 535–547.

(I) Low-Flow Vascular Malformations
Venous Malformation
Lymphatic Malformation
Capillary Malformation
Mixed Low Flow Malformation
Fibro-Adipose Vascular Anomaly (FAVA)
Syndromes with low-flow vascular
malformations

Venous Malformation
Incidence
Imaging features
Treatment

Incidence
The most common peripheral vascular
malformation, account for almost two-thirds
of vascular malformations.
A venous malformation is defined as a simple
malformation with slow flow and an abnormal
venous network.
Dubois J, Soulez G, Oliva VL, Berthiaume MJ, Lapierre C, Therasse E. Soft-tissue venous malformations in adult patients: imaging and
therapeutic issues. RadioGraphics 2001;21(6):1519-1531.
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond --- part 2, slow-flow lesions. Am J Roentgenol. 2013;200:423-
36.

Usually located in the head and neck (40% of
cases), trunk (20%), and extremities (40%).
Already present at birth, but is usually not
clinically evident until late childhood or
adulthood, and may enlarge due to hormonal
changes during puberty.
Frequently asymptomatic.
Radiol 1999;29 (12):879–893.
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond --- part 2, slow-flow lesions. Am J Roentgenol. 2013;200:423---36.

On clinical exam they present as sponge like,
compressible and non-pulsatile masses which
vary in size and shape and may be localized or
diffuse.
When superficial, lesions typically have a bluish
discoloration.
Radiol. 1999;29:879-93.
Donnelly LF, Adams DM, Bisset GS 3rd. Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic. Am J
Roentgenol. 2000;174:597-608.
Fayad LM, Hazirolan T, Bluemke D, Mitchell S. Vascular malformations in the extremities: emphasis on MR imaging features that guide
treatment options. Skeletal Radiol. 2006;35: 127-37.

Davidson, Joseph & Ford, Kathryn. (2015). A Core Surgical Trainee's Guide to: Vascular Malformations in Children. Core Surgery
Journal. 5. 47.

Cervicofacial venous malformation involving the
right neck (a) and oropharyngeal mucosa (b).

They characteristically reduce with extremity
elevation and local compression and enlarge
with dependent position and Valsalva
maneuvers.
Stiffness and discomfort may occur secondary to
hemorrhage and thrombophlebitis.
Lack of increased local temperature or bruit is
characteristic in comparison with high-flow
lesions.
El-Merhi F, Garg D, Cura M, Ghaith O. Peripheral vascular tumors and vascular malformations: imaging (magnetic resonance imaging
and conventional angiography), pathologic correlation and treatment options. Int J Cardiovasc Imag. 2013;29:379-93.
Restrepo R. Multimodality imaging of vascular anomalies. Pediatr Radiol. 2013;43 Suppl. 1:S141-54.

Like all vascular malformations, VMs may
infiltrate across multiple tissue planes
including skin, subcutaneous fat, skeletal
muscle, bones, joints and, internal organs.
The involvement of deep structures is
underestimated on clinical examination, and
potential manifestations include pain,
impaired function, and skeletal deformity.
Radiol. 1999;29:879-93.
Donnelly LF, Adams DM, Bisset GS 3rd. Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic.
Am J Roentgenol. 2000;174:597-608.

Imaging features
a) Ultrasound:
Usually present as compressible, anechoic,
ectatic venous spaces separated by echogenic
septa and with scant monophasic low-velocity
flow.
The detection of flow can be enhanced by
applying compression or performing the
Valsalva maneuver.
Restrepo R. Multimodality imaging of vascular anomalies. Pediatr Radiol. 2013;43 Suppl. 1:S141-5
Pediatr Radiol. 2017;47:1196-2

When venous flow is not depicted, differentiation
from a lymphatic malformation can be
challenging.
Some US maneuvers may be helpful in highlighting
some changes in the venous channels.
Specifically, these will fill in during Valsalva
maneuvers, in dependent position, and drain
with compression, elevation of the body part
above the level of the heart or when Valsalva is
released.

Phleboliths are the hallmark of VMs and are
best depicted as small calcifications on
radiography and CT.
They can be seen on US as echogenic foci with
posterior acoustic shadowing.
Secondary signs of osseous involvement such as
bony expansion, osteolysisis, cortical thinning
and increased trabeculation can also be seen
on radiography and CT.
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond --- part 2, slow-flow lesions. Am J Roentgenol. 2013;200:423-
36.

41-Year-old female with venous malformations involving the left-sided chest wall.
US image (a) reveals a heterogenous subcutaneous lesion containing predominantly
anechoic vascular channels (arrows), image obtained with direct percutaneous
injection of contrast material (b) shows diffuse homogeneous enhancement of the
lesion, multiple phlebolites are noted along the left sided chest wall on a post
percutaneous contrast image (arrows).
Flors L, Hagspiel KD, Park AW, Norton PT, Leiva-Salinas C. Soft-tissue vascular malformations and tumors. Part 2: low-flow lesions.
Malformaciones vasculares y tumores de partes blandas. Parte 2: lesiones de bajo flujo. Radiologia (Engl Ed). 2019;61(2):124-133.

Phleboliths in forearm of a 5-year-old with VM.
(A) Radiograph demonstrates multiple calcified phleboliths within the soft tissues.
(B) Ultrasound shows shadowing echogenic foci representing the phleboliths.
Masand P. Radiographic findings associated with vascular anomalies. Semin Plast Surg. 2014;28(2):69-78.

b) MRI:
Present as lobulated, non-mass like lesions with
low to intermediate signal intensity on T1 and
hyperintensity on T2 and STIR.
Occasionally, hemorrhage or high protein
content may cause internal fluid-fluid levels.
In cases of thrombosis or hemorrhage,
heterogeneous signal intensity can be
observed on T1.
Radiol 2010;75 (1):2–11.
Flors L, Leiva-Salinas C, Maged IM, Norton PT, Matsumoto AH, Angle JF, et al. MR imaging of soft-tissue vascular malformations:
diagnosis, classification, and therapy follow-up. Radiographics. 2011;31:1321-40 [discussion 1340-1].

Right gluteal venous malformation, axial T1 (a) shows a hypointense
lobulated mass involving the right gluteal area (arrows), on axial
STIR (b), the venous malformation is hyperintense and has a
multilocular appearance due to abnormal venous lakes separated
by thin hypointense septa.

VMs may infiltrate multiple tissue planes and fat
suppressed T2 and STIR provide excellent
delineation of the extension of the lesions.
Contrast administration is helpful and often
shows slow, gradual, delayed heterogeneous
contrast filling with characteristic diffuse
enhancement of the slow flowing venous
channels on delayed post-contrast T1.
Flors L, Leiva-Salinas C, Maged IM, Norton PT, Matsumoto AH, Angle JF, et al. MR imaging of soft-tissue vascular malformations:
diagnosis, classification, and therapy follow-up. Radiographics. 2011;31:1321---40 [discussion 1340-1].

Delayed contrast-enhanced fat-suppressed T1 of a venous malformation in
the lower extremity shows diffuse homogeneous enhancement of the lesion
involving the right thigh.
Malformaciones vasculares y tumores de partes blandas. Parte 2: lesiones de bajo flujo. Radiologia (Engl Ed). 2019;61(2):124-133

Posterior cervical VM in a 41- year-
old woman with severe pain and
right upper extremity numbness.
(a) Sagittal T1 shows a hypointense
lobulated mass involving the
posterior cervical triangle.
(b) On a STIR, the VM is hyperintense
and has a multilocular appearance
due to abnormal venous lakes
separated by thin hypointense septa.
MRA showed no arterial or early
venous enhancement.
(c) Delayed contrast-enhanced fat-
suppressed T1 shows diffuse
homogeneous enhancement of the
lesion.
(d) Image obtained with direct
percutaneous injection of contrast
material also shows diffuse
homogeneous enhancement of the
lesion.

Low-flow malformations are characterized by a lack
of arterial and early venous enhancement, and
absence of enlarged feeding vessels or
arteriovenous shunting.
Pleboliths, septations, or thrombosed vessels may
simulate flow voids on MRI, pleboliths and
calcifications typically appear as low signal
nodular foci on all sequences whereas signal
voids related to high flow characteristically
appear as high signal foci on GRE sequences and
demonstrate contrast enhancement.
Moukaddam H, Pollak J, Haims AH. MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol.
2009;38:535-47.
Flors L, Leiva-Salinas C, Norton PT, Park AW, Ogur T, Hagspiel KD. Ten frequently asked questions about MRI evaluation of soft-tissue vascular
anomalies. Am J Roentgenol. 2013;201:W554-62.

The best clue to identify a VM is the presence of phleboliths, these are
seen as small calcifications on radiography (a) and CT (b, c) and as
low signal small foci on all MRI sequences (d), the images presented
here correspond to three different patients.

5-year-old with forearm VVM. (A) Coronal STIR shows predominant
hyperintense signal, lobulated contours, and dark signal intensity flow
voids due to phleboliths. (B) Coronal T1 shows iso- to mild hyperintense
signal within the malformation with some fatty atrophy of the underlying
muscle. (C,D) Axial pre-contrast and fat-suppressed post-contrast T1 show
gradual moderate enhancement within the VVM.
Masand P. Radiographic findings associated with vascular anomalies. Semin Plast Surg. 2014;28(2):69-78.

Demonstration of a connection between a
malformation and the deep venous system is
useful for planning treatment, since such a
finding increases the risk of deep venous
thrombosis.
Konez O, Burrows PE, Mulliken JB, Fishman SJ, Kozakewich HP. Angiographic features of rapidly involuting congenital hemangioma
(RICH). Pediatr Radiol 2003;33(1):15–19.

Treatment
Conservative management
Sclerotherapy
Endovenous ablation techniques
Post-procedural care
Müller-Wille R, Wildgruber M, Sadick M, Wohlgemuth WA. Vascular Anomalies (Part II): Interventional Therapy of Peripheral Vascular
Malformations [published online ahead of print, 2018 Feb 7]. Gefäßanomalien (Teil II): Interventionelle Therapie von peripheren
Gefäßmalformationen [published online ahead of print, 2018 Feb 7]. Rofo. 2018;10.1055/s-0044-101266.

Conservative management of VMs includes the
prescription of individually adjusted
compression garments.
Appropriately fitted compression garments can
reduce pain, slow progression, and deformity.
Paroxysmal pain secondary to intralesional
localized thrombosis can be managed with
generally available pain-relieving agents.
Legiehn GM, Heran MK. A Step-by-Step Practical Approach to Imaging Diagnosis and Interventional Radiologic Therapy in Vascular
Malformations. Semin Intervent Radiol 2010; 27: 209–231

Low-molecular-weight heparin (LMWH) can be
used to prevent ongoing clotting.
Occasionally, cessation of oral contraceptives
could be considered.
Unfortunately, direct medical treatment of VMs
has been quite limited until now.
Promising small case series showed that mTOR
inhibitors (sirolimus) seem to have a positive
clinical effect on VMs.
Triana P, Dore M, Cerezo VN et al. Sirolimus in the Treatment of Vascular Anomalies. Eur J Pediatr Surg 2017; 27: 86–90
Goldenberg DC, Carvas M, Adams D et al. Successful Treatment of a Complex Vascular Malformation With Sirolimus and Surgical
Resection. J Pediatr Hematol Oncol 2017; 39: e191–e195

Sclerotherapy
Invasive therapy is indicated in conjunction with
conservative management in symptomatic VMs
to reduce pain, disfigurement, hemorrhage, and
impairment of neighboring structures or to
reduce the thromboembolic risk.
Percutaneous sclerotherapy is the first-choice
invasive treatment method and can be combined
with additional laser therapy or surgical
procedures.
van der Vleuten CJ, Kater A, Wijnen MH et al. Effectiveness of sclerotherapy, surgery, and laser therapy in patients with venous
malformations: a systematic review. Cardiovasc Intervent Radiol 2014; 37: 977– 989
Ranieri M, Wohlgemuth W, Muller-Wille R et al. Vascular malformations of upper and lower extremity – from radiological
interventional therapy to surgical soft tissue reconstruction – an interdisciplinary treatment. Clin Hemorheol Microcirc 2017; 67: 355
–372.

The aim of sclerotherapy is to damage the
endothelial lining of the VM, resulting in
thrombosis, inflammation, and subsequent
fibrosis of the abnormal vein channels with a
reduction of the size.
Frequently used sclerosants for VMs are ethanol,
ethanol gel, polidocanol, sodium tetradecyl
sulfate, and bleomycin, however, systematic
reviews could not identify a significantly superior
sclerosing agent in terms of effectiveness.
Green D. Mechanism of action of sclerotherapy. Semin Dermatol 1993; 12: 88–97
Qiu Y, Chen H, Lin X et al. Outcomes and complications of sclerotherapy for venous malformations. Vasc Endovascular Surg 2013; 47: 454–461
van der Vleuten CJ, Kater A, Wijnen MH et al. Effectiveness of sclerotherapy, surgery, and laser therapy in patients with venous malformations: a
systematic review. Cardiovasc Intervent Radiol 2014; 37: 977– 989
ch SE, Lokhorst MM, Saeed P et al. Sclerotherapy for low-flow vascular malformations of the head and neck: A systematic review of sclerosing
agents. J Plast Reconstr Aesthet Surg 2016; 69: 295–304

1-Ethanol:
Highly concentrated ethanol is a very effective
sclerosant for the treatment of VMs.
It causes precipitation of endothelial cells and
thrombosis.
Nonetheless, absolute ethanol can result in serious
local and systemic side effects like compartment
compression, necrosis, ulcer, hyperpigmentation,
nerve injury, hypoglycemia, deep vein
thrombosis, pulmonary thrombosis, pulmonary
vasospasm, cardiac collapse, and death.
Zhang J, Li HB, Zhou SY et al. Comparison between absolute ethanol and bleomycin for the treatment of venous malformation in
children. Exp Ther Med 2013; 6: 305–309
Steiner F, FitzJohn T, Tan ST. Ethanol sclerotherapy for venous malformation. ANZ J Surg 2016; 86: 790
Ali S, Weiss CR, Sinha A et al. The treatment of venous malformations with percutaneous sclerotherapy at a single academic medical
center. Phlebology 2016; 31: 603–609

It has been shown that ethanol has a
significantly higher complication rate
compared to other sclerosants, therefore, it
should be used only by experienced
interventional radiologist.
Total dose of 0.2ml per kg appears to be the
threshold to reduce side effects.
Ethanol can be mixed with lipiodol for
radiopacity.
Spence J, Krings T, TerBrugge KG et al. Percutaneous treatment of facial venous malformations: a matched comparison of alcohol and
bleomycin sclerotherapy. Head Neck 2011; 33: 125–130
Bisdorff A, Mazighi M, Saint-Maurice JP et al. Ethanol threshold doses for systemic complications during sclerotherapy of superficial
venous malformations: a retrospective study. Neuroradiology 2011; 53: 891 –894

2-Ethanol gel:
To limit diffusion and to keep ethanol in the
malformation, it can be administrated in
highly viscous gel form.
Ethanol gel has a favorable safety profile in the
treatment of VMs compared to pure ethanol.
Dompmartin A, Blaizot X, Theron J et al. Radio-opaque ethylcellulose-ethanol is a safe and efficient sclerosing agent for venous
malformations. Eur Radiol 2011; 21: 2647–26
Schumacher M, Dupuy P, Bartoli JM et al. Treatment of venous malformations: first experience with a new sclerosing agent–a
multicenter study. Eur J Radiol 2011; 80: e366–e372
Teusch VI, Wohlgemuth WA, Hammer S et al. Ethanol-Gel Sclerotherapy of Venous Malformations: Effectiveness and Safety. Am J
Roentgenol 2017; 209: 1390–1395. doi:10.2214/AJR.16.17603

3-Polidocanol:
A local anesthetic also used as a sclerosant for
VMs with fewer side effects than absolute
ethanol.
There is some evidence that polidocanol foam,
made by mixing polidocanol with sterile air
(Tessari technique), has a higher rate of
obliteration compared to the application of
liquid polidocanol.
Tessari L, Cavezzi A, Frullini A. Preliminary experience with a new sclerosing foam in the treatment of varicose veins. Dermatol Surg 2001; 27: 58–60
Yamaki T, Nozaki M, Sakurai H et al. Prospective randomized efficacy of ultrasound-guided foam sclerotherapy compared with ultrasound-guided liquid sclerotherapy in the
treatment of symptomatic venous malformations. J Vasc Surg 2008; 47: 57
Horbach SE, Lokhorst MM, Saeed P et al. Sclerotherapy for low-flow vascular malformations of the head and neck: A systematic review of sclerosing agents. J Plast Reconstr Aesthet
Surg 2016; 69
Weitz-Tuoretmaa A, Keski-Nisula L, Rautio R et al. Quality of life after endovascular sclerotherapy of low-flow venous malformations: the efficacy of polidocanol compared with
ethanol. Acta Radiol 2017. doi:10.1177/0284185117741774

Polidocanol foam
Preparation of
Aethoxysklerol foam.
Gefäßmalformationen [published online ahead of print, 2018 Feb 7]. Rofo. 2018;10.1055/s-0044-101266

4-Sodium tetradecyl sulfate (STS):
STS is the active component of the sclerosant
drug Sotradecol.
It has been demonstrated that STS foam is an
effective sclerosing agent for VMs with a low
complication risk.
Alakailly X, Kummoona R, Quereshy FA et al. The use of sodium tetradecyl sulphate for the treatment of venous malformations of the
head and neck. J Maxillofac Oral Surg 2015; 14: 332–338
Park HS, Do YS, Park KB et al. Clinical outcome and predictors of treatment response in foam sodium tetradecyl sulfate sclerotherapy
of venous malformations. Eur Radiol 2016; 26: 1301–1310

5-Bleomycin:
Bleomycin is a cytotoxic, antineoplastic
antibiotic derived from Streptomyces
verticillus.
The sclerosing effect of bleomycin on the vessel
endothelium can be used for the treatment of
VMs.
Post-procedural swelling is less intensive after
bleomycin application compared to ethanol.
Spence J, Krings T, TerBrugge KG et al. Percutaneous treatment of facial venous malformations: a matched comparison of alcohol and bleomycin sclerotherapy.
Head Neck 2011; 33: 125–130
Zhang J, Li HB, Zhou SY et al. Comparison between absolute ethanol and bleomycin for the treatment of venous malformation in children. Exp Ther Med 2013; 6:
305–309
Songsaeng D, Churojana A, Khumthong R et al. Comparative outcomes for sclerotherapy of head and neck venous vascular malformation between alcohol and
bleomycin. J Med Assoc Thai 2015; 98:408-413

These properties made bleomycin the sclerosant of
choice in patients with airway compression.
However, there is a potential risk of pulmonary
fibrosis after bleomycin admission.
Therefore, bleomycin must be used in a very small
dose with no more than 1mg/kg body weight per
session.
Bleomycin may induce neoplasms, thus its use in
children is to be considered with special caution.
Malformations. Semin Intervent Radiol 2010; 27: 209–231

Applications techniques
Direct needle puncture of the VM is performed with
a 20- or 21-gauge needle under real-time
ultrasound guidance.
The needle is connected to a 10 ml syringe of saline
and is gradually withdrawn while applying low
suction.
As soon as blood returns, a radiopaque contrast
agent is injected to obtain a phlebogram of the
VM to confirm the position, estimate the lesion
volume and compartmentalization and to identify
draining veins.

Venous malformation
(a) STIR sequence showed
a hyperintense venous
malformation with central
phleboliths (arrow)
(b) Percutaneous
puncture of the venous
malformation.
(c) Injection of contrast
media into the venous
malformation
(d) Injection of Polidocanol
foam.

Four different phlebographic patterns of VMs
can be observed:
Type I lesions: are VMs without considerable
venous drainage under fluoroscopy.
Type II VMs: have normal-sized venous drainage
Type III VMs: have enlarged venous drainage.
Type IV lesions: are composed of basically
ectatic dysplastic vein.
Puig S, Aref H, Chigot V et al. Classification of venous malformations in children and implications for sclerotherapy. Pediatr Radiol
2003; 33: 99–103

Classification of venous
malformations
Type I lesions are
isolated
malformations
without
phlebographically
visible venous
drainage.
Type II lesions
demonstrate normal-
sized venous drainage.
Type III lesions
demonstrate enlarged
venous drainage.
Type IV lesions are
composed of
essentially ectatic
dysplastic veins.

VMs with large draining veins suggest a higher risk
of complications during sclerotherapy.
After identification of the phlebographic patterns,
the sclerosant can be injected slowly under
fluoroscopy to displace the previously injected
contrast agent residing in the malformation.
A tourniquet or a pneumatic cuff at the venous
outflow minimizes the risk of accidental
migration into the deep venous system.
Puig S, Aref H, Chigot V et al. Classification of venous malformations in children and implications for sclerotherapy. Pediatr Radiol 2003;
33: 99–103

Additionally, local compression of visible
draining veins may be considered.
In some cases it is necessary to puncture the VM
more than once to treat the lesion completely.
However, injection has to be stopped if there is
increased resistance, extravasation of the
sclerosant, or skin blanching.

N.B. Additional venous outflow occlusion:
In cases with large draining veins, additional
occlusion may be indicated to avoid overflow
of the sclerosing agent into the deep vein
system.
Fibered microcoils or plugs of various types can
be placed through an access needle or a
catheter into the outflow vessel of the VM.
Burrows PE, Mason KP. Percutaneous treatment of low flow vascular malformations. J Vasc Interv Radiol 2004; 15: 431–445

Endovenous ablation techniques
Endovenous ablation techniques like endovenous
laser ablation (ELVA) or endovenous
radiofrequency ablation (ERFA) were successfully
used to close large embryonic venous channels
such as the lateral marginal vein in patients with
Klippel-Trenaunay syndrome (KTS).
It is recommended that patients presenting with
such anomalous veins be considered for
endovenous ablation therapy as early as possible
to reduce the risk of thromboembolism.
Berber O, Holt P, Hinchliffe R et al. Endovenous therapy for the treatment of congenital venous malformations. Ann Vasc Surg 2010;
24: 415 e13–417
Patel PA, Barnacle AM, Stuart S et al. Endovenous laser ablation therapy in children: applications and outcomes. Pediatr Radiol 2017;
47: 1353– 1363

Patients should wear their compression garments
to help involution of the lesion.
Limb elevation, ice packs, and pain medication (an
NSAID is normally sufficient) may be indicated.
To prevent deep vein thrombosis (DVT),
prophylactic anticoagulation with LMWH is
recommended.
Ultrasound should be performed to exclude DVT
one day after therapy of limb VMs.

Lymphatic Malformation
Incidence
Imaging features
Treatment

Incidence
Congenital lesions of the lymphatic system and
consist of cystic spaces filled with lymphatic
fluid and dilated lymphatic channels.
The second most common type of vascular
malformation after venous malformations.
Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. 2005;32:99-116.
Radiol. 2009;38:535---47.
Restrepo R. Multimodality imaging of vascular anomalies. Pediatr Radiol. 2013;43 Suppl. 1:S

Traditionally misnamed as ‘‘lymphangioma or
cystic hygroma’’, LMs can be divided into
macrocystic, micro-cystic and mixed types.
Microcystic LMs are composed of multiple cysts
smaller than 2 mm in a background of solid
matrix, whereas macrocystic lesions, have
larger cysts of variable sizes.

Sometimes, this distinction is based on the
sonographic characteristic of the lesion since it
will define treatment options; lesions are
categorized as macro-cystic when the size of the
cysts permits a needle to be inserted in.
Lymphatic malformations are commonly mixed,
containing both micro and macrocystic
components as well as other types of vascular
malformations, most commonly venous
malformations.
Sanlialp I, Karnak I, Tanyel FC, Senocak ME, Buyukpamukcu N. Sclerotherapy for lymphangioma in children. Int J Pediatr Otorhinolaryngol.
2003;67:795-800.
Radiol. 2009;38:535-47.
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond --- part 2, slow-flow lesions. Am J Roentgenol. 2013;200:423-36.

Unlike VMs, most LMs are identified in the first
two years of life.
LMs are usually found in the neck (70-80%),
especially in the posterior cervical triangle,
and axillary region (20%).
Less commonly, the mediastinum,
retroperitoneum and the extremities may be
involved.
Radiol. 1999;29:879-93.
Radiol. 2009;38:535-47.
El-Merhi F, Garg D, Cura M, Ghaith O. Peripheral vascular tumors and vascular malformations: imaging (magnetic resonance imaging
and conventional angiography), pathologic correlation and treatment options. Int J Cardiovasc Imag. 2013;29:379-93.

Sievers W, Rathner JA, Green RA, et al. Innervation of supraclavicular adipose tissue: A human cadaveric study. PLoS One.
2020;15(7):e0236286. Published 2020 Jul 23.

Upon physical exam, they present as smooth,
non-pulsatile, soft tissue masses with a
rubbery consistency and without bruit or
increased temperature.
Dermal extension is common, especially with
microcystic LMs, and it is seen as numerous
small vesicles with associated skin thickening
and surrounding lymphedema.
Restrepo R. Multimodality imaging of vascular anomalies. Pediatr Radiol. 2013;43 Suppl. 1:S14

The macrocystic counter parts are seen as
smooth, translucent lobulated masses under
the normal cutis.
Unlike VMs, LMs are non-compressible.
They can get complicate by infection or bleeding
thus presenting with tenderness or sudden
enlargement of the lesion.
Radiol. 2009;38:535-47.
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond-part 2, slow-flow lesions. Am J Roentgenol. 2013;200:423-36.
Ernemann U, Kramer U, Miller S, Bisdas S, Rebmann H, Breuninger H, et al. Current concepts in the classification, diagnosis and
treatment of vascular anomalies. Eur J Radiol. 2010;75:2-11.

Hassan, M.A., Gatea, H.K. & Ja’afar, T.K. Surgical excision: an effective initial therapeutic option in the management of giant macrocystic
lymphatic malformations in children. Ann Pediatr Surg 16, 1 (2020).

(a) Macrocystic lymphatic malformation (LM) of right neck in
toddler. (b) Microcystic lip LM displaying mucosal vesicles.

Imaging features
(a) Ultrasound:
Macrocystic LMs appear as thin-walled cystic
lesions with posterior acoustic enhancement
on US.
Thin septa are often present.
Characteristically, arterial or venous waveforms
are absent within the cysts on Doppler US, but
may be detected within the septa.
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond- part 2, slow-flow lesions. Am J Roentgenol. 2013;200:423-36.
Johnson CM, Navarro OM. Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 2: vascular
malformations. Pediatr Radiol. 2017;47:1196-208.

Unlike VMs, no change in appearance will occur
with Valsalva maneuvers, compression or
change in position.
The cysts in microcystic LMs are often too small
to be discernible by ultrasound, and they
often present as ill-defined hyperechoic
lesions; the posterior acoustic enhancement
suggests the cystic nature of the lesion.
Absent flow is demonstrated by Doppler.
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond- part 2, slow-flow lesions. Am J Roentgenol. 2013;200:423-36.
Pediatr Radiol. 2017;47:1196-208.

Lymphatic malformation has macrocysts (asterisks) and
microcysts (arrow) containing anechoic fluid separated by
fibrous septa.
White CL, Olivieri B, Restrepo R, McKeon B, Karakas SP, Lee EY. Low-Flow Vascular Malformation Pitfalls: From Clinical Examination to
Practical Imaging Evaluation--Part 1, Lymphatic Malformation Mimickers. AJR Am J Roentgenol. 2016;206(5):940-951.

(b) MRI:
LMs are usually seen as lobulated, septated
masses with intermediate to decreased T1
signal intensity and, like other vascular
anomalies, increased signal intensity on T2
and STIR.
Internal fluid-fluid levels are common.
LMs tend to infiltrate across fat planes and
involving multiple adjacent tissues.
Donnelly LF, Adams DM, Bisset GS 3rd. Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic. Am
J Roentgenol. 2000;174:597-608.
Radiol. 2009;38:535-47.

3-month-old boy with
mixed (macro- and
microcystic) lymphatic
malformation containing
blood at different stages
involving left chest wall
and arm.
Axial T2 shows large
extension of lymphatic
malformation, lesion
contains locules of
different sizes and various
signal intensities, some of
which contain fluid-fluid
levels (arrows) indicative of
blood at different stages.
In arm, lymphatic
malformation is causing
mass effect on
musculature (asterisks). L =
lung, H = humerus.

The pattern of contrast enhancement on MRI
will depend on the type of LM.
Microcystic LMs don’t usually enhance, whereas
macrocystic LMs exhibit rim and septal
enhancement with characteristic lack of
internal enhancement of the cystic structures.
Abernethy LJ. Classification and imaging of vascular malformations in children. Eur Radiol. 2003;13:2483-97
Radiol. 2009;38:535-47.

9-year-old girl with macrocystic lymphatic malformation of neck.
Coronal contrast-enhanced T1 with fat saturation shows faint septal
and capsular contrast enhancement (arrows), no enhancing solid
component is seen.

Microcystic lymphatic
malformation of the
forearm in a 5-year-old girl.
(a) Coronal STIR image
shows a hyperintense,
lobulated, septated mass
(arrowheads) involving the
subcutaneous tissue of the
distal left forearm and
hand.
(b) Delayed contrast-
enhanced 3D VIBE image
shows no significant
enhancement of the mass,
a finding characteristic of a
microcystic lymphatic
malformation.

Macrocystic lymphatic
malformation in a 6- month-old
infant with a swollen mass in the
submandibular triangle.
(a) T1 shows a well-defined,
multilobulated, septated mass
that is mildly hyperintense
relative to the muscles, the
increased signal intensity is most
likely related to a high
proteinaceous component, note
the fluid-fluid level (arrow) in the
posterior component of the mass.
(b) On STIR, the mass is highly
hyperintense.
Arrow = fluid-fluid level.
(c) Axial gadolinium-enhanced fat-
suppressed T1- weighted image
shows rim and septal
enhancement (arrowheads) with
no enhancement of the lymph-
filled spaces.
Arrow = fluid-fluid level.

32-Year-old women with
macrocystic lymphatic
malformation involving the
right orbit and right
maxillary sinus.
Coronal (a) and sagittal T2(b)
show a multicystic lesion (*)
with several internal fluid-
fluid levels (arrows) due to
hemorrhage.
Lack of enhancement was
demonstrated on post-
contrast imaging (not
shown).

Occasionally, microcystic lymphatic malformations
or combined lymphatic-venous malformations
may show diffuse enhancement, which is due to
septal enhancement of the small, nonperceptible
cysts in microcystic lymphatic malformations or
enhancement of the venous component in mixed
malformations.
This appearance may render them indistinguishable
from venous malformations.
Laor T, Burrows PE. Congenital anomalies and vascular birthmarks of the lower extremities. Magn Reson Imaging Clin N Am
1998;6(3):497–519.
treatment options. Skeletal Radiol 2006;35(3):127–137

malformation in a 5-year-
old boy.
(a) Axial STIR shows a
hyperintense, septated
subcutaneous mass in the
medial aspect of the left
knee (arrows).
Contrast-enhanced MR
angiography showed no
arterial or venous
enhancement.
(b) Delayed contrast-
enhanced 3D VIBE image
shows mildly increased
signal intensity due to
enhancement of the septa
(arrowheads) between the
microcysts.

malformation of the left arm
and chest wall in a 5-year-old
girl.
Coronal STIR image (a) shows
a hyperintense, lobulated,
septated lesion (arrowheads)
involving the subcutaneous
(arrows) soft tissue.
The lesion is hypointense
onT1 (b).
Delayed contrast-enhanced
3D VIBE (c) image shows
mildly increased signal
intensity in parts of the lesion
due to enhancement of the
septa (arrowheads) between
the microcysts.

Treatment
Sclerotherapy

Small asymptomatic LMs can be monitored
without immediate treatment.
Antibiotic medications are indicated to prevent
and treat infections of LMs.
Recent studies demonstrated that mTOR
inhibitors (sirolimus) had a positive clinical
effect on extensive LMs especially in infants
with cervicofacial lesions.
Lackner H, Karastaneva A, Schwinger W et al. Sirolimus for the treatment of children with various complicated vascular anomalies. Eur J
Pediatr 2015; 174: 1579–1584
Triana P, Dore M, Cerezo VN et al. Sirolimus in the Treatment of Vascular Anomalies. Eur J Pediatr Surg 2017; 27: 86–90
Strychowsky JE, Rahbar R, O’Hare MJ et al. Sirolimus as treatment for 19 patients with refractory cervicofacial lymphatic malformation.
Laryngoscope 2017. doi:10.1002/lary.2678

Sclerotherapy
Indications for invasive therapy of LMs are
usually:
Recurrent infection
Recurrent hemorrhage
Impairment of neighboring structures (e. g.
upper airways)
Disfigurement.
Alomari AI, Karian VE, Lord DJ et al. Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-
evaluated improvement. J Vasc Interv Radiol 2006; 17: 1639–16

It has been demonstrated that percutaneous
sclerotherapy is very effective in reducing the
size and symptoms of macrocytic lesions with
a low risk for adverse events.
However, microcystic LM lesions respond less to
percutaneous sclerotherapy.
STS seems to be less effective and injection of
ethanol carries the risk of increased
complications.

The most commonly used sclerosants are:
Picibanil (OK-432)
Bleomycin
Doxycycline.
Alomari AI, Karian VE, Lord DJ et al. Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-
evaluated improvement. J Vasc Interv Radiol 2006; 17: 1639–16

Picibanil (OK-432):
Is a lyophilized mixture of group A Streptococcus
pyogenes with a high capacity to produce
fibrosis.
Intracystic injection of Picibanil has been shown
to be an effective and safe treatment for
macrocystic LMs in children.
Repeated injections are often required to
achieve clinical success.
Rebuffini E, Zuccarino L, Grecchi E et al. Picibanil (OK-432) in the treatment of head and neck lymphangiomas in children. Dent Res J (Isfahan) 2012;
9 (Suppl. 2): S192–S196
Gurgacz S, Zamora L, Scott NA. Percutaneous sclerotherapy for vascular malformations: a systematic review. Ann Vasc Surg 2014; 28: 1335– 1349
Motz KM, Nickley KB, Bedwell JR et al. OK432 versus doxycycline for treatment of macrocystic lymphatic malformations. Ann Otol Rhinol Laryngol
2014; 123: 81–88

Induces severe swelling for more than one week
after treatment.
In cases with potential airway compression,
elective intubation and ventilation following
sclerotherapy may be necessary.
Another typical side effect of Picibanil is post-
procedural fever.
It can be successfully treated with paracetamol
and resolves after 1-3 days.
Ravindranathan H, Gillis J, Lord DJ. Intensive care experience with sclerotherapy for cervicofacial lymphatic malformations. Pediatr Crit
Care Med 2008; 9: 304–309
Rebuffini E, Zuccarino L, Grecchi E et al. Picibanil (OK-432) in the treatment of head and neck lymphangiomas in children. Dent Res J
(Isfahan) 2012; 9 (Suppl. 2): S192–S196

Bleomycin:
The sclerosing effect of bleomycin has been well
known for over forty years.
Due to its low risk of swelling, it is a preferred
agent in patients with macrocystic LMs of the
head and neck area.
Must be used in very small doses to avoid side
effects like pulmonary fibrosis.
Yura J, Hashimoto T, Tsuruga N et al. Bleomycin treatment for cystic hygroma in children. Nihon Geka Hokan 1977; 46: 607–614
Malformations. Semin Intervent Radiol 2010; 2
Yang Y, Sun M, Ma Q et al. Bleomycin A5 sclerotherapy for cervicofacial lymphatic malformations. J Vasc Surg 2011; 53: 150–155

Doxycycline:
Very effective for the treatment of macrocystic
and mixed head and neck lymphatic
malformations in children.
Positive effects are often seen after a single
session.
It has minimal side effects and is widely
available.
Motz KM, Nickley KB, Bedwell JR et al. OK432 versus doxycycline for treatment of macrocystic lymphatic malformations. Ann Otol Rhinol
Laryngol 2014; 123: 81–88
Cheng J. Doxycycline sclerotherapy in children with head and neck lymphatic malformations. J Pediatr Surg 2015; 50: 214
Thomas DM, Wieck MM, Grant CN et al. Doxycycline Sclerotherapy Is Superior in the Treatment of Pediatric Lymphatic Malformations. J Vasc
Interv Radiol 2016; 27: 1846–1856

Applications techniques
Cysts are cannulated with a needle under real-time
ultrasound guidance.
Alternatively, a pigtail catheter (3 to 5 French) can
be inserted in larger cysts, as the multiple side
holes facilitate aspiration of the lymphatic fluid
before injection of the sclerosing agent.
Contrast media can be injected to visualize the
whole lesion under fluoroscopy.
After aspiration of the entire cyst content, the LM
can then be treated with the sclerosant.
Gefäßmalformationen [published online ahead of print, 2018 Feb 7]. Rofo. 2018;10.1055/s-0044-101266.

Strict postoperative observation of the upper
airway is recommended after treatment of
patients with large cervical LMs.
Fever after injection of Picibanil can be treated
with paracetamol.
The positive effect of sclerotherapy is not visible
immediately, but after 4-6 weeks.
Ravindranathan H, Gillis J, Lord DJ. Intensive care experience with sclerotherapy for cervicofacial lymphatic malformations. Pediatr Crit
Care Med 2008; 9: 304–309

Capillary Malformation
Incidence
Imaging features
Treatment

Incidence
Present at birth in around 0.3% of children.
Traditionally named port-wine stains, they are
the least common and the most superficial of
all low flow vascular malformations.
Histologically, they are composed of ectatic thin-
walled capillary channels surrounded by
disorganized collagen.
Radiol. 2009;38:535---47.
Restrepo R. Multimodality imaging of vascular anomalies. Pediatr Radiol. 2013;43 Suppl. 1

They are usually limited to the superficial dermis
or mucous membranes; but, on occasions,
they may be the hallmark of complex
syndromes such as Sturge-Weber, Klippel-
Trenaunay, Parkes-Weber or Proteous
syndrome.
. Moukaddam H, Pollak J, Haims AH. MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal
Radiol. 2009;38:535-47.
Ernemann U, Kramer U, Miller S, Bisdas S, Rebmann H, Breuninger H, et al. Current concepts in the classification, diagnosis and
treatment of vascular anomalies. Eur J Radiol. 2010;75:2---11.

Unlike VMs, CMs are usually present as a
macular pink to dark red patch with irregular
borders without bruit or local warmth.
Like LMs, they are usually localized in the head
and neck region.
Symptoms may be the result of deeper
associated malformations.
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast
Reconstr Surg. 1982;69:412-22.
Berenguer B, Burrows PE, Zurakowski D, Mulliken JB. Sclerotherapy of craniofacial venous malformations: complications and results. Plast Reconstr Surg.
1999;104:1-11 [discussion 12-5].
Fayad LM, Hazirolan T, Bluemke D, Mitchell S. Vascular malformations in the extremities: emphasis on MR imaging features that guide treatment options.
Skeletal Radiol. 2006;35: 127-37.
Ernemann U, Kramer U, Miller S, Bisdas S, Rebmann H, Breuninger H, et al. Current concepts in the classification, diagnosis and treatment of vascular
anomalies. Eur J Radiol. 2010;75:2-11.
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond -part 2, slow-flow lesions. Am J Roentgenol. 2013;200:423-36.

Capillary malformation (port wine stain) of the left
face in infant.
Richter GT, Friedman AB. Hemangiomas and vascular malformations: current theory and management. Int J Pediatr.
2012;2012:645678.

Imaging features
Due to their superficial nature, diagnosis is usually
made by clinical exam and history.
Imaging is, therefore, not required for their
diagnosis but can be sometimes indicated to
exclude underlying disorders.
Skin thickening is usually the only finding on US.
MRI findings are also subtle, with skin thickening
and occasional increased subcutaneous thickness
and faint focal T2 hyperintensity and contrast
enhancement.
Moukaddam H, Pollak J, Haims AH. MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol.
2009;38:535-47.
Restrepo R. Multimodality imaging of vascular anomalies. Pediatr Radiol. 2013;43 Suppl. 1:S141-54.

Treatment
Laser therapy is the standard treatment.
Surgical procedure may be considered when
there is overgrowth of soft tissue or bone
enlargement.

Mixed Low Flow Malformation
Incidence
Imaging features
Treatment

Incidence
This group includes capillary-venous
malformations, which are combined low-flow
malformations formed from dysplastic
capillary vessels and enlarged post-capillary
vascular spaces, and mixed venous and
lymphatic malformations.
.

Clinical presentation depends on location and
size of the lesion.
Malformaciones vasculares y tumores de partes blandas. Parte 2: lesiones de bajo flujo. Radiologia (Engl Ed). 2019;61(2):124-133

Imaging features
Imaging findings in capillary-venous may be
indistinguishable from those of VMs and
dynamic contrast-enhanced MRI can be useful
for this purpose, as capillary-venous
malformations will typically show early
enhancement, whereas only delayed
enhancement is seen in VMs.
Mixed lymphatic venous malformations present
as partially enhancing multi-cystic lesions.
van Rijswijk CS, van der Linden E, van der Woude HJ, van Baalen JM, Bloem JL. Value of dynamic contrast-enhanced MR imaging in
diagnosing and classifying peripheral vascular malformations. AJR Am J Roentgenol 2002;178(5):1181–1187.
Radiol. 2009;38:535-47.

Capillary-venous
malformation of the calf in
a 32-year-old woman.
(a) Axial T2 shows
hyperintense ill-defined
subcutaneous involvement
(arrows) of the lateral
aspect of the distal left
lower extremity.
(b) Image from MR
angiography shows
characteristic early diffuse
enhancement of the lesion
(*) and early venous
shunting (arrows).
Note the absence of
dilated arteries and
draining veins.

4-Year-old male with mixed
venous-lymphatic malformation.
Axial T1 (a) shows a hypointense
lobulated mass malformations
involving the perineum and
extending into the scrotum and
right thigh.
Axial STIR (b) shows a well-
defined septate hyperintense
lesion with few fluid-fluid levels
(arrowhead).
Delayed contrast-enhanced fat-
suppressed axial T1 (c)
demonstrates partial
enhancement of the lesion
(arrows).
A phlebolit is also noted as a
hypointense foci on this image
( arrowhead).
There was no arterial
enhancement (not shown).

Treatment
These lesions are treated with a combination of
methods for venous and lymphatic
malformations, as described before.

Fibro-Adipose Vascular Anomaly (FAVA)
Incidence
Imaging features
Treatment

Incidence
Recently described by Alomari et al, FAVA
constitutes a new rare vascular disorder with
distinct clinical, radiologic, and histopathologic
features.
Histologically, FAVA is characterized by fatty and
dense fibrous tissue with a venous malformation
involving the affected muscle.
Although this new entity shares some similarities
with the more common intra-muscular VMs, it is
important to recognize FAVA because of a
different management approach.
Alomari AI, Spencer SA, Arnold RW, Chaudry G, Kasser JR, Burrows PE, et al. Fibro-adipose vascular anomaly: clinical-radiologic-
pathologic features of a newly delineated disorder of the extremity. J Pediatr Orthop. 2014;34: 109-17.
Amarneh M, Shaikh R. Clinical and imaging features in fibro-adipose vascular anomaly (FAVA). Pediatr Radiol. 2020;50(3):380-387.

Patients present with complex symptoms
including persistent pain, discomfort,
functional impairment and contracture.
The clinical and imaging features can be
confusing and often overlap with those of
other vascular malformations and tumors.
The gastrocnemius muscle is the most
commonly affected muscle in FAVA.

De Crée C (2015) Rupture of the Medial Head of the Gastrocnemius Muscle in Late-Career and Former Elite Jūdōka: A Case Report.
Ann Sports Med Res 2(5): 1032.

Imaging features
(a) Ultrasound:
Unlike VMs, a non-compressible, echogenic mass
characterizes FAVA on US.
(b) MRI:
The dominant fibrofatty solid component is seen
with associated phlebectasia characterized by
heterogeneous moderately hyperintense signal
on T2 which is less hyperintense than that seen in
common VMs.
Moderate to strong and homogeneous post-
contrast enhancement is also seen.

Focal mass-like fibro-adipose
vascular anomaly (FAVA) in a
14-year-old girl with
progressive thigh pain over
the last 3 years.
a Axial T1 demonstrates the
muscle replaced by focal
heterogeneous FAVA lesion
(arrow), which is
hyperintense, representing
intralesional fat.
b, c Axial (b) and coronal (c)
fat-saturated T2 demonstrate
heterogeneous hyperintense
lesion along the direction of
the muscle.
d Contrast-enhanced axial fat-
saturated T1 demonstrates
heterogeneous enhancement.

Focal infiltrative fibro-adipose
vascular anomaly (FAVA) in a 12-
year-old girl with posterior thigh pain.
a Axial T1 demonstrates an ill-defined
focal infiltrative FAVA lesion (arrow).
B Axial fat-saturated T2 demonstrates
heterogeneous hyperintense lesion
with interspersed dilated intralesional
veins.
C Sonographic image demonstrates
ill-defined hyperechoic infiltrative
lesion (thin arrow) involving the
muscle, also seen is an ectatic
anomalous vein (thick arrow).
d Intralesional venography
demonstrates a superficial anomalous
ectatic vein (thin white arrow),
embolization coils (wide white arrow)
placed in a deeper anomalous vein,
and beaded appearance of
intralesional veins (black arrow).

Diffuse infiltrative fibro-adipose
vascular anomaly (FAVA) in a 13- year-
old boy with history of left hip
birthmark.
a Clinical photograph shows cutaneous
lymphatic vesicles (thick arrow), left
lower-extremity asymmetrical
overgrowth, and painful and dilated
superficial veins (thin arrows).
b, c Coronal fat-saturated T2 (b) and
axial T1 (c) demonstrate diffuse
infiltrative lesions in the gluteal and
hamstring muscles and the overlying
subcutis., asymmetrical overgrowth of
the subcutaneous fat is infiltrated by
isointense tissue, representing fibrous
tissue, lymphatic infiltrates, small
venous malformations and
phlebectasia (arrow).
d Axial fat-saturated T2 demonstrates
heterogeneous hyperintense lesion
replacing gastrocnemius muscle (thin
arrow)with an anomalous dilated
transfascial vein (thick arrow).

MRI of a diffuse fibro-adipose vascular anomaly
(FAVA) of the right calf.
A, Axial T1 Both heads of the gastrocnemius
muscle were diffusely replaced by heterogeneous
soft tissue (arrows) with signal intensity higher
than adjacent normal muscles, note the
transfascial fatty component of the mass.
B, Axial T2 the heterogeneous high signal
intensity is higher than that of the normal
muscles but less intense than the fluid signal
typically seen in venous malformations, note the
dilated intramuscular vein (black arrow),
phlebolith (bent arrow), and thickened
subcutaneous fat (white arrows).
C, Axial fat-saturated T1 following contrast
administration demonstrating moderate to strong
enhancement.
D, Sagittal T1 depicts the longitudinal distribution
of the disease along the entire course of the
gastrocnemius (white and gray arrows), note
subcutaneous phlebectasia (black arrow).
E, Ultrasonography of a focal calf FAVA, the
affected part of the gastrocnemius muscle
demonstrated extensive, solid, and echogenic
changes entirely replacing the
normal fibrillary pattern, the dilated
intramuscular veins (straight arrows) contained a
clot (bent arrow), which was very tender.
F, Venous phase of angiography in a diffuse calf
FAVA. Note the marked phlebectasia (long
arrows) of both intrafascial and extrafascial
compartments, the deep veins were normal (short
arrows).

Treatment
Although sclerotherapy can be performed on the
generally smaller venous component of FAVA, the
dominant solid fibro-fatty component is not
amenable to this intervention and depending on
the severity of symptoms, physical therapy
and/or surgical resection may be needed.
There is a report of image-guided percutaneous
cryoablation for control of symptoms in FAVA
lesions with significant improvement in pain.
Shaikh R, Alomari AI, Kerr CL, Miller P, Spencer SA. Cryoablation in fibro-adipose vascular anomaly (AVFA): a minimally invasive
treatment option. Pediatr Radiol. 2016;46: 1179-86.

Syndromes with low-flow vascular
malformations
Soft-tissue vascular anomalies associated with
syndromes are usually low-flow.
VMs or combined LM-VM are found in Blue
rubber bleb nevus, Proteus and Maffuci
syndromes.
Capillary malformation may be the hallmark of
Sturge-Weber and Klippel-Trenaunay
syndromes.

Blue rubber bleb nevus syndrome
Proteus syndrome
Maffucci syndrome
Sturge-Weber syndrome
Klippel-Trenaunay syndrome

Blue Rubber Bleb Nevus Syndrome
Blue rubber bleb nevus syndrome (BRBNS), or
Bean syndrome is a rare disorder, first
described by Bean in 1958 and characterized
by multiple cutaneous and gastrointestinal
VMs.
Patients can present with gastrointestinal
hemorrhage and bloody stools.
Intermittent small bowel obstruction caused by
intussusception or volvulus can also be seen.
Nozaki T, Nosaka S, Miyazaki O, Makidono A, Yamamoto A, Niwa T, et al. Syndromes associated with vascular tumors and malformations:
a pictorial review. Radiographics. 2013;33: 175-95.

Blue rubber bleb nevus syndrome
in a 32-year-old woman with an
extensive subcutaneous and
intramuscular venous
malformation.
(a) STIR shows the extent of the
venous malformation, which
appears as a hyperintense,
multilobulated, septated mass
involving the subcutaneous tissue
and muscles of the right upper
extremity, right chest wall, and
right pleural space, multiple
phleboliths (arrows) are seen as
signal voids in the lesion, the
presence of any pathologic
arterial inflow was excluded with
contrast-enhanced MRA.
(b) Delayed contrast-enhanced
fat-suppressed 3D VIBE T1 shows
diffuse nodular enhancement of
the venous malformation.

Blue rubber bleb nevus
syndrome in a 32-year-old
woman.
MR images demonstrate
an extensive subcutaneous
and intramuscular venous
malformation involving the
left forearm and left hand;
the lesion is hypointense
on axial T1 fat sat image
(a), hyperintense on axial
STIR (b) and demonstrates
diffuse delayed
enhancement on coronal
post contrast image (c).

2-year-old boy with subcutaneous
and intramuscular venous
malformations.
(a) Axial T2 of pelvis shows multiple
well-circumscribed strongly
hyperintense masses (arrows) in
subcutaneous tissues, muscles, and
retroperitoneum, this type of T2
signal is characteristic of vascular
anomalies such as venous
malformations.
(b) AP DSA of abdomen after
percutaneous injection of contrast
material into venous malformation of
abdominal wall shows contrast
material pooling in lesion.
(c) Axial T2 of pelvis obtained with fat
saturation shows bilateral parailiac
lesions (arrows), additional lesions
can be seen adjacent to right iliac
vessels and along right anterior
abdominal wall (arrowheads)
,symmetric iliac location is
characteristic for blue rubber bleb
nevus syndrome.
Kassarjian A, Fishman SJ, Fox VL, Burrows PE. Imaging characteristics of blue rubber bleb nevus syndrome. AJR Am J Roentgenol.
2003;181(4):1041-1048.

Proteus Syndrome
A rare sporadic condition with complex
multisystemic involvement and wide clinical
variability.
It is characterized by asymmetric overgrowth of
the bones, skin, and other tissues, cutaneous
and visceral combined lymphatic-venous
malformations, bilateral ovarian
cystadenomas or a parotid monomorphic
adenoma, lung cysts and facial abnormalities.
Nozaki T, Nosaka S, Miyazaki O, Makidono A, Yamamoto A, Niwa T, et al. Syndromes associated with vascular tumors and
malformations: a pictorial review. Radiographics. 2013;33: 175-95.

Limb length discrepancy.
AP radiographs of the
pelvis and thighs (a) and
the legs and feet (b) of a
12-year-old male patient
show asymmetric
overgrowth of bones and
soft tissues in the right
side of the pelvis and the
right lower extremity, limb
length discrepancy, and
bowing in the left femur
and right fibula.
Jamis-Dow CA, Turner J, Biesecker LG, Choyke PL. Radiologic manifestations of Proteus syndrome. Radiographics. 2004;24(4):1051-
1068.

Venous malformations.
Axial MR image obtained
with a short inversion time
inversion recovery
sequence at the level of
the thighs in a patient aged
27 years shows multiple
enlarged veins in the
subcutaneous tissues and
posterior muscles of the
right thigh (arrows), as well
as a slight enlargement of
the right thigh with
increased subcutaneous
fat, which causes a mild
asymmetry in the cross-
sectional area of the
thighs.
Jamis-Dow CA, Turner J, Biesecker LG, Choyke PL. Radiologic manifestations of Proteus syndrome. Radiographics. 2004;24(4):1051-
1068.

Maffucci Syndrome
A rare sporadic disorder characterized by diffuse
enchondromatosis involving the phalanges of
the hands and feet associated with multiple
venous or lymphatic malformations.
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond - part 2, slow-flow lesions. Am J Roentgenol. 2013;200:423-36.

Frontal (a) and oblique (b) radiographs of the hands show the typical
ring-and-arc appearance of chondroid lesions in the matrix of the
osseous lesions, the well-defined round calcifications in the soft-tissue
masses are typical of phleboliths.
Zwenneke Flach H, Ginai AZ, Wolter Oosterhuis J. Best cases from the AFIP. Maffucci syndrome: radiologic and pathologic findings.
Armed Forces Institutes of Pathology. Radiographics. 2001;21(5):1311-1316.

(a) Photograph of the hand obtained during surgery shows the grayish
enchondromas, clearly visible after incisions were made and the skin
was moved aside. (b) Photograph of the hand obtained during surgery
shows the appearance after removal of the enchondromas.
Zwenneke Flach H, Ginai AZ, Wolter Oosterhuis J. Best cases from the AFIP. Maffucci syndrome: radiologic and pathologic findings.
Armed Forces Institutes of Pathology. Radiographics. 2001;21(5):1311-1316.

Sturge-Weber Syndrome
Also called encephalotrigeminal angiomatosis.
It is a neurocutaneous disorder that combines a
unilateral capillary malformation in the
trigeminal nerve distribution (port wine stain)
with a capillary-venous malformation in the
pia and arachnoid mater and choroid of the
eye, and atrophy and calcification in the
subjacent cerebral cortex.
Donnelly LF, Adams DM, Bisset GS 3rd. Vascular malformationsand hemangiomas: a practical approach in a multidisciplinaryclinic. Am J
Roentgenol. 2000;174:597-608
Nozaki T, Nosaka S, Miyazaki O, Makidono A, Yamamoto A,Niwa T, et al. Syndromes associated with vascular tumors and malformations: a
pictorial review. Radiographics. 2013;33:175-95
Flors L, Leiva-Salinas C, Norton PT, Park AW, Ogur T,Hagspiel KD. Ten frequently asked questions about MRI evaluation of soft-tissue
vascular anomalies. Am J Roentgenol.2013;201:W554-62

17-Year-old female with
Surge-Weber syndrome.
Susceptibility weighted image
(a) shows atrophy and cortical
mineralization involving the
sulcus of the right parietal-
temporal occipital convexity
(arrows), reflecting low
vascular malformations in the
pia mater.
Marked right calvarial
thickening is seen on axial T2
(b) and coronal T1 (c) images.
Facial capillary malformation
was present on clinical exam.

Klippel-Trenaunay Syndrome
Is a condition of unknown etiology characterized
by combined capillary, venous and lymphatic
malformations of the extremities, usually the
lower limbs, in association with bone and soft-
tissue hypertrophy resulting from overgrowth.
Donnelly LF, Adams DM, Bisset GS 3rd. Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic. Am J
Roentgenol. 2000;174:597-608
Dubois J, Alison M. Vascular anomalies: what a radiologist needs to know. Pediatr Radiol. 2010;40:895-905
Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond - part 2, slow-flow lesions. Am J Roentgenol.2013;200:423-36
Nozaki T, Nosaka S, Miyazaki O, Makidono A, Yamamoto A,Niwa T, et al. Syndromes associated with vascular tumorsand malformations:
a pictorial review. Radiographics. 2013;33:175-95

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