• Users Online: 4807
  • Print this page
  • Email this page

Table of Contents
Year : 2018  |  Volume : 11  |  Issue : 3  |  Page : 91-93

Vascular anomalies

1 Division of Plastic and Reconstructive Surgery, AKH-Vienna, Medical University of Vienna, Austria
2 Division of Pediatric Surgery, AKH-Vienna, Medical University of Vienna, Austria

Date of Web Publication24-Sep-2018

Correspondence Address:
Kurosh Paya
Vienna Medical School, Vienna
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/HMJ.HMJ_51_18

Rights and Permissions

Vascular anomalies are rare congenital anomalies partly belonging to the Orphan diseaeses. Due to the intensive collaboration of the ISSVA a common categorization based upon histopathological attributes could be established. We present a short overview of the classification, manifestations and the diagnostic and therapeutical options.

Keywords: Magnetic resonance angiography, ultrasound imaging, vascular malformations, vascular anomalies

How to cite this article:
Roka J, Paya K. Vascular anomalies. Hamdan Med J 2018;11:91-3

How to cite this URL:
Roka J, Paya K. Vascular anomalies. Hamdan Med J [serial online] 2018 [cited 2023 Feb 8];11:91-3. Available from: http://www.hamdanjournal.org/text.asp?2018/11/3/91/236272

  Introduction Top

According to the International Society for the Study of Vascular Anomalies (ISSVA), vascular anomalies are classified into two subgroups: vasoproliferative tumours (e.g., haemangioma) and vascular malformations (e.g., lymphangiomatous, capillary, venous, arterial, arteriovenous and combined malformations and others).

Vascular tumours show pathohistologically abnormal cell proliferation-like neoplasms. They have a growth phase marked by endothelial proliferation and hypercellularity, often followed by an involutional phase. Vascular malformations instead are the result of abnormal development during vasculogenesis and angiogenesis.[1],[2] Diagnosis nearly always is challenging and treatment options are limited. All vascular anomalies should undergo ultrasound imaging with high-resolution Doppler as a basic diagnostic tool. In addition, magnetic resonance angiography (MRI), computed tomography (CT) scan and interventional angiography can be useful for imaging purpose.[3]

We will give a short overview of the characteristics, diagnostic pathways and current management, including the historical evolvement.

  Vascular Tumours Top

In 1982, J. Mulliken and J. Glowacki published a first classification based on the endothelial characteristics.[4],[5] They proposed to distinguish between proliferative and normal endothelial cell cycle as the main stake for further management.[4] Based on the work of J. Mulliken and A. Young, the ISSVA was founded in 1992. They developed a classifying table for vascular anomalies which until now is the only and most important valid classification (ISSVA Classification of Vascular Anomalies © 2014 ISSVA Available at “issva.org/classification” Accessed September 5, 2018).

The prevalence of vascular tumours is relatively high with 4%–10% and haemangiomas are the most common tumours in infancy. Eighty per cent of haemangiomas are single, while 20% are multiple. [Table 1] shows differentiation into benign, locally aggressive and malignant tumours.
Table 1: Abbreviated International Society for the Study of Vascular Anomalies classification for vascular anomalies by International Society for the Study of Vascular Anomalies is licensed under a Creative Commons Attribution 4.0 international license

Click here to view

The most common types are infantile (prevalence 2.6%–4.5%) and congenital haemangiomas. The infantile haemangioma presents most commonly as a small red mark or nodule appearing shortly after birth which increases rapidly during the 1st month and involutes during the first 5 years of life.[6] As a specific histopathological marker, GLUT-1 is positive in infantile haemangiomas.[7] Since 2008 infantile haemangiomas are preferably treated by topical or systemic Propranolol with high rates of positive response.[8],[9],[10],[11]

The congenital haemangioma is always present at birth and can be separated into two types: rapid involuting congenital haemangioma and noninvoluting congenital haemangioma (NICH). The first shows rapid involution (within 10–14 months of life) after a short period of enlargement ( first 3–6 months). The latter does not regress but continues to expand and needs further therapeutic management. Unfortunately, propranolol is not effective in NICH.

A rare entity is the Kaposiform hemangioendothelioma, which infiltrates deeper tissues and may become malignant. It is often associated with lymphatic malformations and can lead to Kasabach–Merritt Syndrome, a life-threatening disorder of coagulation. Therapy always should be radical resection or in special cases laser as a less invasive method.

  Vascular Malformations Top

As shown in [Table 1], they are mainly divided into lymphatic-, venous-, arteriovenous-, capillary and combined types. Based on their characteristics in the Doppler ultrasound, they can also be differentiated into the slow flow and high-flow malformations.[12]

The “stork bite” is a transient capillary malformation in newborns that mostly fades within the first 3 years of life. Usually, it does not necessarily need treatment.

Venous malformations result of the arrested development of the venous system during embryogenesis– they have been classified primarily in the Hamburg classification (precursor of the ISSVA class) as extratruncular and truncular lesions.[2],[13] In extratruncular lesions, the malformation arose from embryonic tissue ahead of the formation of the main vascular trunk and maintained its ability to proliferate. In truncular malformations, the vascular arrest occurred during the foetal phase; therefore, these lesions do not have embryonic characteristics such as the ability to proliferate.

Venous malformations may also be subclassified on their anatomical location. Aside from clinical examination, duplex ultrasound and Doppler mode are the most important diagnostic tools to particularly differentiate venous from arteriovenous malformations (AVMs).

Complications are bleeding, thrombosis, ulceration, chronic venous insufficiency, pain, obstruction or thromboembolism. Treatment options depend on localisation, size, morphology and involved structures, among them sclerotherapy with variable endovascular agents seems to be one of the most indicated modalities.[13]

AVMs are extremely rare (prevalence 0.2%) and often located within the central nervous system or the face. In AVM, veins and arteries do communicate directly without capillaries in between, causing shunts, pain and symptoms of expansion and steal phenomenon with the risk of bleeding episodes, haemorrhage of the brain, epileptic seizures, cephalea and paraesthesia, necrosis and cardiac insufficiency as a result of increasing shunting volume. Doppler ultrasound identifies the high-flow pattern and MRI can define the extent of the lesion, followed CT angiography or by angiography for diagnosing the feeding and draining vessels eventually combined with therapeutic embolisation, if possible.

Some AVM occurs as part of a syndrome, for example, the Parkes Weber syndrome. It consists of arteriovenous fistulas of the limbs, consecutive hypertrophy of the affected limb, naevus flammeus or port wine stain, and may also cause congestive heart failure in the later course.

Lymphatic malformations are rare low-flow vascular anomalies. They can occur everywhere but predominantly appear at the neck, face and axilla. They consist of single endothelial cell-lined channels, vesicles or pouches filled with lymphatic fluid [14] and may be categorised into macrocystic (>1 cm), microcystic or mixed lymphatic malformations. All lymphatic malformations are congenital but usually appear in early childhood or adolescence.

Diagnosis always includes ultrasound and MRI. Therapeutic approaches are surgical excision for small, circumscribed lesions without causing damage to involved nerves (e.g., facial nerve), vessels and other vital structures or mutilation of the face. Since 1994 streptococcal lyophilisate (Picibanil) instillation is used in macrocystic cysts with high success rates.[15] Some authors recommend always to try the first line with Picibanil, and only second-line surgical excision. Furthermore, other additional sclerosing agents such as bleomycin and others have been introduced as highly effective, but with significant possible side effects.[16] Beyond that, new systemic approaches have been promising for extensive microcystic lymphatic malformations.[17],[18]

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg 2005;32:99-116, ix.  Back to cited text no. 1
Belov S. Anatomopathological classification of congenital vascular defects. Semin Vasc Surg 1993;6:219-24.  Back to cited text no. 2
Dubois J, Alison M. Vascular anomalies: What a radiologist needs to know. Pediatr Radiol 2010;40:895-905.  Back to cited text no. 3
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.  Back to cited text no. 4
Mulliken JB, Zetter BR, Folkman J.In vitro characteristics of endothelium from hemangiomas and vascular malformations. Surgery 1982;92:348-53.  Back to cited text no. 5
Hoeger PH, Harper JI, Baselga E, Bonnet D, Boon LM, Ciofi Degli Atti M, et al. Treatment of infantile haemangiomas: Recommendations of a European expert group. Eur J Pediatr 2015;174:855-65.  Back to cited text no. 6
North PE, Waner M, Mizeracki A, Mihm MC Jr. GLUT1: A newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol 2000;31:11-22.  Back to cited text no. 7
Léauté-Labrèze C, Taïeb A. Efficacy of beta-blockers in infantile capillary haemangiomas: The physiopathological significance and therapeutic consequences. Ann Dermatol Venereol 2008;135:860-2.  Back to cited text no. 8
Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taïeb A, et al. Propranolol for severe hemangiomas of infancy. N Engl J Med 2008;358:2649-51.  Back to cited text no. 9
Léauté-Labrèze C, Hoeger P, Mazereeuw-Hautier J, Guibaud L, Baselga E, Posiunas G, et al. Arandomized, controlled trial of oral propranolol in infantile hemangioma. N Engl J Med 2015;372:735-46.  Back to cited text no. 10
Léauté-Labrèze C, Voisard JJ, Moore N. Oral propranolol for infantile hemangioma. N Engl J Med 2015;373:284-5.  Back to cited text no. 11
Nosher JL, Murillo PG, Liszewski M, Gendel V, Gribbin CE. Vascular anomalies: A pictorial review of nomenclature, diagnosis and treatment. World J Radiol 2014;6:677-92.  Back to cited text no. 12
Lee BB, Bergan J, Gloviczki P, Laredo J, Loose DA, Mattassi R, et al. Diagnosis and treatment of venous malformations. Consensus document of the international union of phlebology (IUP)-2009. Int Angiol 2009;28:434-51.  Back to cited text no. 13
Ramani P, Shah A. Lymphangiomatosis. Histologic and immunohistochemical analysis of four cases. Am J Surg Pathol 1993;17:329-35.  Back to cited text no. 14
Ogita S, Tsuto T, Nakamura K, Deguchi E, Iwai N. OK-432 therapy in 64 patients with lymphangioma. J Pediatr Surg 1994;29:784-5.  Back to cited text no. 15
Horbach SE, Rigter IM, Smitt JH, Reekers JA, Spuls PI, van der Horst CM, et al. Intralesional bleomycin injections for vascular malformations: A systematic review and meta-analysis. Plast Reconstr Surg 2016;137:244-56.  Back to cited text no. 16
Danial C, Tichy AL, Tariq U, Swetman GL, Khuu P, Leung TH, et al. An open-label study to evaluate sildenafil for the treatment of lymphatic malformations. J Am Acad Dermatol 2014;70:1050-7.  Back to cited text no. 17
Lackner H, Karastaneva A, Schwinger W, Benesch M, Sovinz P, Seidel M, et al. Sirolimus for the treatment of children with various complicated vascular anomalies. Eur J Pediatr 2015;174:1579-84.  Back to cited text no. 18


  [Table 1]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  Vascular Malform...
  In this article
Vascular Tumours
Article Tables

 Article Access Statistics
    PDF Downloaded250    
    Comments [Add]    

Recommend this journal