Abstract
Vascular malformations are a heterogeneous group of entities, many of which present in the pediatric age group. Sonography plays a major role in the management of children with these vascular anomalies by providing information that helps in diagnosing them, in assessing lesion extent and complications, and in monitoring response to therapy. The interpretation of sonographic findings requires correlation with clinical findings, some of which can be easily obtained at the time of scanning. This has to be combined with the use of appropriate nomenclature and the most updated classification in order to categorize these patients into the appropriate management pathway. Some vascular malformations are part of combined vascular anomalies or are associated with syndromes that include other disorders, frequently limb overgrowth, and these are now being reclassified based on their underlying genetic mutation. Sonography has limitations in the evaluation of some vascular malformations and in these cases MR imaging might be considered the imaging modality of choice, particularly for lesions that are large, that involve multiple compartments or are associated with other soft-tissue and bone abnormalities. In this article, which is part 2 of a two-part series, the authors review the most relevant clinical and sonographic features of arteriovenous, capillary, venous and lymphatic malformations as well as vascular malformations that are part of more complex conditions or associated with syndromes, including Parkes–Weber syndrome, phosphatase and tensin homologue (PTEN) hamartoma tumor syndromes, Klippel–Trénaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi and skeletal anomalies) syndrome, fibro-adipose vascular anomaly and Proteus syndrome.
Similar content being viewed by others
References
International Society for the Study of Vascular Anomalies (2014) ISSVA classification of vascular anomalies. http://www.issva.org/UserFiles/file/Classifications-2014-Final.pdf. Accessed 12 April 2017
Frieden I, Enjolras O, Esterly N (2003) Vascular birthmarks and other abnormalities of blood vessels and lymphatics. In: Schachner LA, Hansen RC (eds) Pediatric dermatology, 3rd edn. Mosby, New York, pp 833–862
Merrow AC, Gupta A, Patel MN et al (2016) 2014 revised classification of vascular lesions from the International Society for the Study of Vascular Anomalies: radiologic–pathologic update. Radiographics 36:1494–1516
Uller W, Alomari AI, Richter GT (2014) Arteriovenous malformations. Semin Pediatr Surg 23:203–207
Enjolras O, Wassef M, Chapot R (2007) Color atlas of vascular tumors and vascular malformations, 1st edn. Cambridge University Press, New York
Paltiel HJ, Burrows PE, Kozakewich HP et al (2000) Soft-tissue vascular anomalies: utility of US for diagnosis. Radiology 214:747–754
Dubois J, Garel L (1999) Imaging and therapeutic approach of hemangiomas and vascular malformations in the pediatric age group. Pediatr Radiol 29:879–893
Legiehn GM, Heran MK (2006) Classification, diagnosis and interventional radiologic management of vascular malformations. Orthop Clin N Am 37:435–474
Dunham GM, Ingraham CR, Maki JH et al (2016) Finding the nidus: detection and workup of non-central nervous system arteriovenous malformations. Radiographics 36:891–903
Lowe L, Marchant TC, Rivard DC et al (2012) Vascular malformations: classification and terminology the radiologist needs to know. Semin Roentgenol 47:106–117
Dubois J, Patriquin HB, Garel L et al (1998) Soft-tissue hemangiomas in infants and children: diagnosis using Doppler sonography. AJR Am J Roentgenol 171:247–252
Dubois J, Alison M (2010) Vascular anomalies: what a radiologist needs to know. Pediatr Radiol 40:895–905
Ballah D, Cahill AM, Fontalvo L et al (2011) Vascular anomalies: what they are, how to diagnose them, and how to treat them. Curr Probl Diagn Radiol 40:233–247
Patel AS, Schulman JM, Ruben BS et al (2015) Atypical MRI features in soft-tissue arteriovenous malformation: a novel imaging appearance with radiologic–pathologic correlation. Pediatr Radiol 45:1515–1521
Sheybani EF, Eutsler EP, Navarro OM (2016) Fat-containing soft-tissue masses in children. Pediatr Radiol 46:1760–1773
Lee MS, Liang MG, Mulliken JB (2013) Diffuse capillary malformation with overgrowth: a clinical subtype of vascular anomalies with hypertrophy. J Am Acad Dermatol 69:589–594
Troilius A, Svendsen G, Ljunggren B (2000) Ultrasound investigation of port wine stains. Acta Derm Venereol 80:196–199
Alfageme Roldán F, Salgüero Fernández I, Muñoz Garza Z (2016) Update on the use of ultrasound in vascular anomalies. Actas Dermosifiliogr 107:284–293
Legiehn GM, Heran MK (2008) Venous malformations: classification, development, diagnosis, and interventional radiologic management. Radiol Clin N Am 46:545–597
Behr GG, Johnson CM (2013) Vascular anomalies: hemangiomas and beyond — part 2, slow-flow lesions. AJR Am J Roentgenol 200:423–436
Trop I, Dubois J, Guibaud L et al (1999) Soft-tissue venous malformations in pediatric and young adult patients: diagnosis with Doppler US. Radiology 212:841–845
Restrepo R (2013) Multimodality imaging of vascular anomalies. Pediatr Radiol 43:S141–S154
Olivieri B, White CL, Restrepo R et al (2016) Low-flow vascular malformation pitfalls: from clinical examination to practical imaging evaluation — part 2, venous malformation mimickers. AJR Am J Roentgenol 206:952–962
Dalmonte P, Granata C, Fulcheri E et al (2012) Intra-articular venous malformations of the knee. J Pediatr Orthop 32:394–398
Wassef M, Blei F, Adams D et al (2015) Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics 136:e203–e214
Hammill AM, Wentzel M, Gupta A et al (2011) Sirolimus for the treatment of complicated vascular anomalies in children. Pediatr Blood Cancer 57:1018–1024
Acevedo JL, Shah RK, Brietzke SE (2008) Nonsurgical therapies for lymphangiomas: a systematic review. Otolaryngol Head Neck Surg 138:418–424
Cahill AM, Nijs EL (2011) Pediatric vascular malformations: pathophysiology, diagnosis, and the role of interventional radiology. Cardiovasc Intervent Radiol 34:691–704
Morrow MS, Oliveira AM (2014) Imaging of lumps and bumps in pediatric patients: an algorithm for appropriate imaging and pictorial review. Semin Ultrasound CT MR 35:415–429
White CL, Olivieri B, Restrepo R et al (2016) Low-flow vascular malformation pitfalls: from clinical examination to practical imaging evaluation — part 1, lymphatic malformation mimickers. AJR Am J Roentgenol 206:940–951
Yikilmaz A, Ngan BY, Navarro OM (2015) Imaging of childhood angiomatoid fibrous histiocytoma with pathological correlation. Pediatr Radiol 45:1796–1802
Lobo-Mueller E, Amaral JG, Babyn PS et al (2009) Complex combined vascular malformations and vascular malformation syndromes affecting the extremities in children. Semin Musculoskelet Radiol 13:255–276
Uller W, Fishman SJ, Alomari AI (2014) Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg 23:208–215
Navarro OM (2016) Magnetic resonance imaging of pediatric soft-tissue vascular anomalies. Pediatr Radiol 46:891–901
Clemens RK, Pfammatter T, Meier TO et al (2015) Combined and complex vascular malformations. Vasa 44:92–105
Kim C, Ko CJ, Baker KE et al (2015) Histopathologic and ultrasound characteristics of cutaneous capillary malformations in a patient with capillary malformation-arteriovenous malformation syndrome. Pediatr Dermatol 32:128–131
Tan WH, Baris HN, Burrows PE et al (2007) The spectrum of vascular anomalies in patients with PTEN mutations: implications for diagnosis and management. J Med Genet 44:594–602
Kurek KC, Howard E, Tenant L (2012) PTEN hamartoma of soft tissue: a distinctive lesion in PTEN syndromes. Am J Surg Pathol 36:671–687
Iacobas I, Burrows PE, Adams DM et al (2011) Oral rapamycin in the treatment of patients with hamartoma syndromes and PTEN mutation. Pediatr Blood Cancer 57:321–323
Martinez-Lopez A, Blasco-Morente G, Perez-Lopez I et al (2017) CLOVES syndrome: review of a PIK3CA-related overgrowth spectrum (PROS). Clin Genet 91:14–21
Hagen SL, Hook KP (2016) Overgrowth syndromes with vascular malformations. Semin Cutan Med Surg 35:161–169
Alomari AI, Spencer SA, Arnold RW et al (2014) Fibro-adipose vascular anomaly: clinical–radiologic–pathologic features of a newly delineated disorder of the extremity. J Pediatr Orthop 34:109–117
Kaduthodil MJ, Prasad DS, Lowe AS et al (2012) Imaging manifestations in Proteus syndrome — an unusual multisystem developmental disorder. Br J Radiol 85:e793–e799
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflicts of interest
The authors have no financial interests, investigational or off-label uses to disclose.
Rights and permissions
About this article
Cite this article
Johnson, C.M., Navarro, O.M. Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 2: vascular malformations. Pediatr Radiol 47, 1196–1208 (2017). https://doi.org/10.1007/s00247-017-3906-x
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-017-3906-x