Vascular Anomalies in Children

  • Oumama El EzziEmail author
  • Anthony de Buys Roessingh


Vascular anomalies are rare but frequently encountered in the pediatric population. It is important to distinguish between vascular tumors and vascular malformations.

Vascular tumors are proliferative lesions due to an accelerated turnover of endothelial cells. Vascular malformations are structural anomalies due to innate errors in embryonic development of blood vessels. They are generally classified by their dominant abnormal vessel: venous, lymphatic, capillary, arteriovenous, or combined malformations. We also commonly divide them to low- or high-flow malformation.

The ISSVA classification has greatly facilitated the understanding of this pathology.

The diagnosis is often determined by the medical history and the clinical findings. Ultrasonography, Doppler flow imaging, and magnetic resonance imaging help in identifying the lesion nature and its extent.

The majority of these vascular anomalies do not require treatment. Their management is complicated and requires a multidisciplinary approach. The proper diagnosis is the starting point for an optimal treatment.


Vascular anomalies Hemangioma Angioma Venous malformation Capillary malformation Lymphatic malformation 



The authors are grateful to Annette Wagnière for reviewing the English text.


  1. 1.
    Mulliken JB, Glowacki PD. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69(3):412–20.CrossRefGoogle Scholar
  2. 2.
    Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, et al. Vascular anomalies classification: recommendations from the International society for the study of vascular anomalies. Pediatrics. 2015;136(1):203–14.CrossRefGoogle Scholar
  3. 3.
  4. 4.
    Amir J, Metzker A, Krickler R, Reisner SH. Strawberry haemangioma in preterm infants. Pediatr Dermatol. 1986;3:331–2.CrossRefGoogle Scholar
  5. 5.
    Drolet BA, Esterly NB, Frieden IJ. Hemangioma in children. N Engl J Med. 1999;341:173–81.CrossRefGoogle Scholar
  6. 6.
    Dompmartin A, Boon LM, Labbe D. Infantile hemangiomas: differential diagnosis and associated anomalies. Ann Chir Plast Esthet. 2006;51(4–5):300–9.CrossRefGoogle Scholar
  7. 7.
    Frieden IJ, Reese V, Cohen D. PHACE syndrome: the association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol. 1996;132(3):307–11.CrossRefGoogle Scholar
  8. 8.
    Metry D, Heyer G, Hess C, Garzon M, Haggstrom A, Frommelt P, et al. Consensus statement on diagnostic criteria for PHACE syndrome. Pediatrics. 2009;124(5):1447–56.CrossRefGoogle Scholar
  9. 9.
    Iacobas I, Burrows PE, Frieden IJ, Liang MG, Mulliken JB, Mancini AJ, et al. LUMBAR: association between cutaneous infantile hemangiomas of the lower body and regional congenital anomalies. J Pediatr. 2010;157(5):795–801.CrossRefGoogle Scholar
  10. 10.
    Millischer-Bellaïche AE, Enjolras O, André C, Bursztyn J, Kalifa G, Adamsbaum C. Eyelid hemangiomas in infants: contribution of MRI. J Radiol. 2004;85:2019–28.CrossRefGoogle Scholar
  11. 11.
    Eivazi B, Cremer HJ, Mangold C, Teymoortash A, Wiegand S, Werner JA. Hemangiomas of the nasal tip: an approach to a therapeutic challenge. Int J Pediatr Otorhinolaryngol. 2011;75:368–75.CrossRefGoogle Scholar
  12. 12.
    El Ezzi O, Hohlfeld J, de Buys Roessingh A. Propranolol in infantile haemangioma: simplifying pretreatment monitoring. Swiss Med Wkly. 2014;144:w13943.PubMedGoogle Scholar
  13. 13.
    Léauté-Labrèze C, Hoeger P, Mazereeuw-Hautier J, Guibaud L, Baselga E, Posiunas G, et al. A randomized, controlled trial of oral propranolol in infantile hemangioma. N Engl J Med. 2015;372:735–46.CrossRefGoogle Scholar
  14. 14.
    Ji Y, Chen S, Xu C, Li L, Xiang B. The use of propranolol in the treatment of infantile haemangiomas: an update on potential mechanisms of action. Br J Dermatol. 2015;172(1):24–32.CrossRefGoogle Scholar
  15. 15.
    de Graaf M, Breur J, Raphaël MF, Vos M, Breugem CC, Pasmans S. Adverse effects of propranolol when used in the treatment of hemangiomas: a case series of 28 infantsJ Am Acad Dermatol. 2011;65:320–7.CrossRefGoogle Scholar
  16. 16.
    Rodríguez-Ruiza M, Telladoa MG, del Pozo Losadab J. Combination of pulsed dye laser and propranolol in the treatment of ulcerated infantile haemangioma. An Pediatr (Engl Ed). 2016;84(2):92–6.CrossRefGoogle Scholar
  17. 17.
    Boon LM, Enjolras O, Mulliken JB. Congenital hemangioma: evidence of accelerated involution. J Pediatr. 1996;128:329–35.CrossRefGoogle Scholar
  18. 18.
    Enjolras O, Mulliken JB, Boon LM, et al. Non involuting congenital hemangioma: a rare cutaneous vascular anomaly. Plast Reconstr Surg. 2001;107:1647–54.CrossRefGoogle Scholar
  19. 19.
    Marilyn G, Liang MD, Ilona J, Frieden MD. Infantile and congenital hemangiomas. Semin Pediatr Surg. 2014;23:162–7.CrossRefGoogle Scholar
  20. 20.
    Mahady K, Thust S, Berkeley R, Stuart S, Barnacle A, Robertson F, Mankad K. Vascular anomalies of the head and neck in children. Quant Imaging Med Surg. 2015;5(6):886–97.PubMedPubMedCentralGoogle Scholar
  21. 21.
    North PE, Waner M, Mizeracki A, Mihm MC Jr. GLUT 1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol. 2000;31(1):11–22.CrossRefGoogle Scholar
  22. 22.
    Drolet BA, Trenor CC, Brandão LR, Chiu YE, Chun RH, Dasgupta R, et al. Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr. 2013;163(1):285–91.CrossRefGoogle Scholar
  23. 23.
    Wong BL, Lee VN, Tikka T, Kim D, Dwivedi RC. Kaposiform haemangioendothelioma of the head and neck. Crit Rev Oncol Hematol. 2016 Aug;104:156–68.CrossRefGoogle Scholar
  24. 24.
    Alfageme Roldán F, Salgüero Fernández I, Zamanta Munoz Garza I, Roustán Gullóna G. Update on the use of ultrasound in vascular anomalies. Actas Dermosifiliogr. 2016;107(4):284–93.CrossRefGoogle Scholar
  25. 25.
    Shaikh SM, Goswami M, Singh S, Singh D. Sturge Weber syndrome. A case report. J Oral Biol Craniofac Res. 2015;5:53–6.CrossRefGoogle Scholar
  26. 26.
    Higueros E, Roe E, Granell E, Baselga E. Sturge-Weber Syndrome: a review. Actas Dermo-Sifiliogr (Engl Ed). 2017;108(5):407–17.Google Scholar
  27. 27.
    Mermod T, El Ezzi O, Raffoul W, Erba P, de Buys Roessingh A. Assessment of the role of LASER-Doppler in the treatment of port-wine stains in infants. J Pediatr Surg. 2015;50:1388–92.CrossRefGoogle Scholar
  28. 28.
    Elluru RG, Azizkhan RG. Cervicofacial vascular anomalies. II. Vascular malformations. Semin Pediatr Surg. 2006;15:133–9.CrossRefGoogle Scholar
  29. 29.
    Defnet AM, Bagrodia N, Hernandez SL, et al. Pediatric lymphatic malformations: evolving understanding and therapeutic options. Pediatr Surg Int. 2016;32:425–33.CrossRefGoogle Scholar
  30. 30.
    Lerata J, Mounayerb C, Scomparina A, Orsela S, Bessedea JP, Aubrya K. Head and neck lymphatic malformation and treatment: clinical study of 23 cases. Eur Ann Otorhinolaryngol Head Neck Dis. 2016;133:393–6.CrossRefGoogle Scholar
  31. 31.
    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;17:1639–48.CrossRefGoogle Scholar
  32. 32.
    Greene AK, Liu AS, Mulliken JB, et al. Vascular anomalies in 5,621 patients: guidelines for referral. J Pediatr Surg. 2011;46:1784–9.CrossRefGoogle Scholar
  33. 33.
    Xia HF, Ren JG, Zhu JY, Yu ZL, Zhang W, Sun YF, Zhao YF, Chen G. Downregulation of miR-145 in venous malformations: its association with disorganized vessels and sclerotherapy. Eur J Pharm Sci. 2017;100:126–31.CrossRefGoogle Scholar
  34. 34.
    Wouters V, et al. Hereditary cutaneomucosal venous malformations are caused by TIE2 mutations with widely variable hyper-phosphorylating effects. Eur J Hum Genet. 2010;18(4):414–20.CrossRefGoogle Scholar
  35. 35.
    Fowella C, Verea Linares C, Jones R, Nishikawad H, Monaghane A. Venous malformations of the head and neck: current concepts in management. Br J Oral Maxillofac Surg. 2017;55:3–9.CrossRefGoogle Scholar
  36. 36.
    Sepúlveda P, Zavala A, Zúñiga P. Factors associated with thrombotic complications in pediatric patients with vascular malformations. J Pediatr Surg. 2017;52:400–4.CrossRefGoogle Scholar
  37. 37.
    Tucci FM, De Vincentiis GC, Sitzia E, Giuzio L, Trozzi M, Bottero S. Head and neck vascular anomalies in children. Int J Pediatr Otorhinolaryngol. 2009;73:71–6.CrossRefGoogle Scholar
  38. 38.
    Simon F, Le Clerc N, Salvan D, Sauvaget E, Faucon B, Borsik M, Herman P, Bisdorff A. Diode endovascular laser treatment in venous malformations of the upper aerodigestive tract. J Craniomaxillofac Surg. 2016;44:533–7.CrossRefGoogle Scholar
  39. 39.
    Ballieux F, Boon LM, Vikkula M. Blue bleb rubber nevus syndrome. Handb Clin Neurol. 2015;132:223–30.CrossRefGoogle Scholar
  40. 40.
    Ramírez M, López Gutiérrez JC, Díaz M, Soto C, Miguel M, de la Torre Ramos CA, et al. Bean or blue rubber bleb nevus syndrome. Presentation of 6 patients. Cir Pediatr. 2010;23:241–4.3.Google Scholar
  41. 41.
    Sheu M, Fauteux G, Chang H, Taylor W, Stopa E, Robinson-Bostom L. Sinus pericranii: dermatologic considerations and literature review. J Am Acad Dermatol. 2002;46:934–41.CrossRefGoogle Scholar
  42. 42.
    McMillan K, Dunphy L, Nishikawa H, Monaghan A. Experiences in managing arteriovenous malformations of the head and neck. Br J Oral Maxillofac Surg. 2016;54:643–7.CrossRefGoogle Scholar
  43. 43.
    Richter GT, Suen JY. Clinical course of arteriovenous malformations of the head and neck: a case series. Otolaryngol Head Neck Surg. 2010;142:184–90.CrossRefGoogle Scholar
  44. 44.
    Schöbinger R. In: Proceedings of International Society for the Study of vascular anomalies congress. 1994.Google Scholar
  45. 45.
    Wassef M, Vanwijck R, Clapuyt P, Boon L, Magalon G. Vascular tumours and malformations, classification, pathology and imaging. Ann Chir Plast Esthet. 2006;51(4–5):263–81.CrossRefGoogle Scholar
  46. 46.
    Goldenberg DC, Hiraki PY, Caldas JG, Puglia P, Marques TM, Gemperli R. Surgical treatment of extracranial arteriovenous malformations after multiple embolizations: outcomes in a series of 31 patients. Plast Reconstr Surg. 2015;135:543–52.CrossRefGoogle Scholar
  47. 47.
    Vidal V, Jacquier A, Le Corroller T, Moulin G, Bartoli JM. Endovascular management of arteriovenous malformations. Ann Chir Plast Esthet. 2006;51(4–5):447–55.CrossRefGoogle Scholar
  48. 48.
    Boon LM, Mulliken JB, Vikkula M. RASA1: variable phenotype with capillary and arteriovenous malformations. Curr Opin Genet Dev. 2005;15(3):265–9.CrossRefGoogle Scholar
  49. 49.
    Eerola I, et al. Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations. Am J Hum Genet. 2003;73(6):1240–9.CrossRefGoogle Scholar
  50. 50.
    Tubridy Clark M, Brooks EL, Chong W, Pappas C. Fahey M. Cobb Syndrome: a case report and systematic review of the literature pediatric neurology. 2008;39:423–5.Google Scholar
  51. 51.
    Dienstmann R, Rodon J, Serra V, Tabernero J. Picking the point of inhibition: a comparative review of PI3K/AKT/mTOR pathway inhibitors. Mol Cancer Ther. 2014;13:1021e31.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Pediatric SurgeryCentre Hospitalier Universitaire Vaudois (CHUV)LausanneSwitzerland

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