Child's Nervous System

, Volume 26, Issue 10, pp 1417–1433

Update on pediatric extracranial vascular anomalies of the head and neck

  • Katherine B. Puttgen
  • Monica Pearl
  • Aylin Tekes
  • Sally E. Mitchell
Special Annual Issue



Vascular anomalies most frequently present at birth or in early childhood, and the craniofacial region is the most common site of involvement. A long history of misleading nomenclature born of confusion about the presentation and natural history of various vascular anomalies has made appropriate diagnosis difficult. The present article emphasizes the importance of clarity of nomenclature for proper diagnosis, both clinically and radiographically, to guide appropriate therapy. In addition, updates on clinical concepts, imaging, and treatment strategies will be discussed. Pediatric vascular anomalies can be divided into two broad categories: vascular tumors and vascular malformations. This biologic classification is based on differences in natural history, cellular turnover, and histology. An updated classification was introduced in 1996 by the International Society for the Study of Vascular Anomalies (ISSVA) to include infantile hemangioma variants, other benign vascular tumors, and combined lesions. Widespread confusion propagated throughout the literature and in clinical practice stems from the continued improper use of many of the terms used to describe vascular tumors and malformations ignoring their pathophysiology. This leads to errors in diagnosis and the dissemination of misinformation to patients and clinicians. Certain terms should be abandoned for more appropriate terms. The clinical presentation usually identifies what general type of vascular anomaly is present, either vascular tumor or vascular malformation. Imaging provides crucial information about the initial diagnosis and aids in follow-up.


Adoption and use of uniform nomenclature in the ISSVA classification system is the first vital step in correct diagnosis and treatment of often complicated vascular tumors and vascular malformations. A multidisciplinary team approach is necessary to provide optimal care for patients, and the necessity for specialists in all areas to communicate using standardized terminology cannot be overemphasized.


Vascular anomaly Hemangioma Venous malformation Arteriovenous malformation Capillary malformation 


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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Katherine B. Puttgen
    • 1
  • Monica Pearl
    • 2
  • Aylin Tekes
    • 3
  • Sally E. Mitchell
    • 4
  1. 1.Department of Dermatology, Division of Pediatric DermatologyThe Johns Hopkins HospitalBaltimoreUSA
  2. 2.Department of Radiology, Division of Interventional NeuroradiologyThe Johns Hopkins HospitalBaltimoreUSA
  3. 3.Department of Radiology, Division of Pediatric RadiologyThe Johns Hopkins HospitalBaltimoreUSA
  4. 4.Department of Radiology, Division of Interventional RadiologyThe Johns Hopkins HospitalBaltimoreUSA

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