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Surgery for Congenital Arterial, Venous, and Lymphatic Anomalies Treatment & Management

  • Author: Jaime Shalkow, MD, FACS; Chief Editor: Mary C Mancini, MD, PhD, MMM  more...
Updated: Jul 08, 2016

Approach Considerations

The nature of vascular anomalies is quite diverse and encompasses many types of lesions; accordingly, indications for treatment are nuanced and variable. In general, the decision to intervene should take into consideration the type of lesion, the associated symptomology, and the desires of the patient and family.

Most hemangiomas are small tumors that eventually undergo involution. However, treatment may be warranted if the tumor is large, grows rapidly, is complicated by severe ulceration, is in a threatening location, or might otherwise cause complications.

Indications for treatment of venous malformations include appearance, impairment of function, and protracted pain.

Capillary malformations are largely treated for cosmetic reasons; however, excision may be warranted in patients who develop fibronodular hypertrophy or who have extensive facial involvement.

Treatment of lymphatic malformations should be geared toward preventing infection and bleeding, correcting cosmetic deformity, and improving function of affected areas.

Quiescent arteriovenous malformations (AVMs) may be managed expectantly; however, pain, bleeding, ulceration, and extensive enlargement are all indications for treatment. Symptomatic lesions that are not amenable to surgical treatment may be considered for palliative embolization therapy.

Treatment is indicated to mitigate the sequelae of combined malformations, including hypertrophy, lymphatic weeping, pain, and loss of functionality.


Medical Therapy


For symptomatic hemangiomas that require intervention (~10% of all cases), topical, intralesional, or systemic corticosteroids are the first line of therapy. If corticosteroid therapy fails, second-line pharmacotherapy with vincristine and interferon alfa may be attempted. Second-line therapy may also be used first if corticosteroids are contraindicated for other reasons (eg, patient preference). At present, pulsed dye laser therapy is indicated for ulcerating lesions and persistent telangiectasias.[6]

Venous malformations

Initial treatment of venous malformations consists of elastic compression aids to reduce swelling and pain. Daily aspirin may also be administered to prevent thrombotic complications. Sclerotherapy (most often with ethanol) is the mainstay of treatment and may be performed for lesions refractory to the above measures. Sclerotherapy should be performed with general anesthesia, under ultrasonographic or fluoroscopic guidance. With repeated therapy, the success rate of improvement in function and reduction in symptoms can reach 76%.[42]

In 2014, the International Union of Phlebology issued an updated guideline expressing a current consensus on the diagnosis and management of venous malformations.[43]

Capillary malformations

Cosmetic camouflage and laser photocoagulation are the current first-line treatments for capillary malformations.[6] Flashlamp pulsed dye lasers are most often used, though the results of such therapy are mixed. Multiple treatments are usually required, and nearly half of all lesions will darken within 5 years of treatment.[44]

Lymphatic malformations

Macrocytic lesions may be effectively treated with sclerotherapy. Both bleomycin and OK-432 (attenuated group A Streptococcus pyogenes) have been shown to be effective as intralesional sclerotic agents for lymphatic malformations.[45, 46] A 2008 series by Burrows et al evaluated the use of doxycycline as an effective sclerosing agent for lymphatic malformations.[47]

Carbon dioxide, argon, and yttrium-aluminum-garnet (YAG) laser therapy can also be used to treat mucosal lesions, though malformations treated with this therapy often recur, requiring repeat treatment.

Arteriovenous malformations

Medical therapy plays no significant role in the management of AVMs.


Surgical Therapy


Early tracheostomy may be warranted for lesions that threaten the airway.

Surgical resection may be appropriate for lesions that are refractory to medical management and that continue to present problems such as ulceration. Resection may be performed at any of the three stages of the life cycle. In general, resection should be deferred until the involuted phase (late childhood), when the lesion has matured and the anesthetic risk to the child is decreased. However, specific indications have been suggested for resection at all phases, as summarized by Marler and Mulliken[6] :

  • Infancy (proliferative phase) - Indications for resection include obstruction (visual or subglottic), deformity (eg, periorbital distortion), bleeding, ulceration, involvement of the scalp (to prevent alopecia of the effected region), and anticipation of a scar caused naturally by the lesion that would be more marked than that caused by surgical intervention
  • Early childhood (involuting phase) - Large protuberant lesions are resected in this phase, as children of this age generally become aware of physical appearance; excision may be performed at this point if resection is considered inevitable, the resultant scar is similar to that achieved by waiting to excise in the involuted phase, and the scar may be easily hidden
  • Late childhood (involuted phase) - Indications for resection in this phase include damaged skin, abnormal contour, and distortion of skin or surrounding structures

Because no two lesions or patients are alike, optimal timing and technique of surgical excision should be determined on a case-by-case basis.

Venous malformations

Sclerotherapy is the primary interventional treatment for venous malformations; however, surgical excision may be offered for selected lesions. Small localized lesions are the best candidates for surgical intervention. In general, sclerotherapy should be used to shrink lesions prior to surgical excision.

Capillary malformations

Small fibrovascular lesions can easily be excised in most locations. More extensive excision and grafting of select capillary malformations may also be performed. Facial lesions with concomitant disfigurement may require excision with full- or split-thickness grafts accompanied by contour resection and correction of maxillofacial distortion.[48, 49]

Lymphatic malformations

Surgical resection is the mainstay treatment for lymphatic malformations. In general, resection should be deferred until late infancy or early childhood to minimize anesthetic risk and to allow easier dissection of neurovascular structures often associated with complex lesions.

Often, lymphatic malformations encompass vital structures, precluding complete excision. Stages approached are often used. The approach to resection varies with lesion location. Generally, total excision of the lesion is attempted, with careful identification and preservation of involved major nerves (eg, preservation of the brachial plexus when excising an axillary lesion).

Resections tend to be involved, and wound complications such as infection, drainage, swelling, and seroma formation are common.

Arteriovenous malformations

Treatment of AVMs consists of a combination of embolization, sclerotherapy, and surgical resection. Angiography is required for intervention, in that it delineates the extent of the lesion and clearly outlines the feeding and draining vessels. Surgical ligation of feeding vessels should not be performed, in that it prevents further access for angiography and only induces recruitment of new vessels into the lesion.[6]

Embolization may be performed with coils, particles, or glue via a venous or arterial approach. Sclerotherapy may be delivered in the nidus of the lesion with concomitant occlusion of feeding and draining vessels. Various sclerosing agents (eg, absolute ethanol and N-butyl cyanoacrylate [NBCA]) have been described.[50] Combinations of embolization and sclerotherapy may be used to treat lesions that may not be amenable to surgical resection, though these measures provide only temporary improvement, as new vessels are readily recruited into the lesion.

If a lesion is deemed appropriate for surgical excision, preoperative embolization is usually recommended to aid in resection. Select lesions (eg, small malformations on the extremities) may be excised without preoperative embolization. Operative intervention is indicated to facilitate complete excision and to minimize recurrence (staged procedures are usually not performed).

Excision should include the nidus of the lesion, as well as any involved skin or deeper tissues. Wide excision is often necessary, with the extent of the resection based on delineation of the lesion by preoperative imaging, bleeding patterns at the resection margin (ie, consistent with normal dermal vasculature or extensive bleeding indicative of the malformation), and frozen sections of the resection margin. Large excisions may necessitate grafting or tissue transfer for adequate wound closure.

Deep intracranial and complex craniofacial AVMs present a unique therapeutic challenge, in that surgical excision is often impossible. Embolization is the usual treatment for such lesions. Radiotherapy has been also successfully employed to treat such intracranial lesions[51] ; the International Radiosurgery Association has published guidelines for its use in this setting.[52]

In 2013, the International Union of Angiology published a consensus document on the management of AVMs.[53]

Combined malformations

Surgical treatment should be specifically tailored for these lesions. Usually, surgical intervention is reserved for correcting orthopedic disfigurement of the extremities or improving functionality of limbs and digits.



Complications of therapy depend on the nature of the lesion and the type of intervention carried out.

Systemic corticosteroid therapy used to treat hemangiomas may produce the expected complications of such therapy (eg, gastric irritation, temporary growth retardation, and cushingoid appearance). Local intralesional administration may be complicated by damage to surrounding structures such as the eye.[54]

In general, treatment of malformations with sclerotherapy may result in local complications, including nerve injury, cutaneous necrosis, blistering, and injury to surrounding structures.[42]

Laser or phototherapy often fails to permanently resolve lesions, with high rates of recurrence.

Short-term complications of surgical excision may include bleeding and infection. Protracted wound problems may also occur, depending on the nature of the lesion. Edema, pain, seromas, and prolonged drainage may occur at resection sites. Functional loss (of limbs and hands) and need for repeat excisions (as with lymphatic malformations) may also be potential complications of surgical management.


Long-Term Monitoring

In general, vascular anomalies constitute a chronic and complex group of lesions that require long-term follow-up and care. Although no treatments can be said to be curative for all problematic lesions, most malformations can be treated to achieve mitigation of symptoms if the correct diagnosis is made early and the proper interventions implemented. Patients should be observed in multidisciplinary clinics, if appropriate, and consultation with a surgeon should be sought early.

Contributor Information and Disclosures

Jaime Shalkow, MD, FACS Director, National Pediatric Cancer Program, National Center for Pediatric and Adolescent Health (CeNSIA); Attending Pediatric Surgical Oncologist, Cancer Center at the American British Cowdray Medical Center

Jaime Shalkow, MD, FACS is a member of the following medical societies: American College of Surgeons, International Society of Paediatric Surgical Oncology, Pacific Association of Pediatric Surgery, Mexican Association of Pediatric Surgery, Mexican Society of Oncology, Mexican Association of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Jonah Odim, MD, PhD, MBA Section Chief of Clinical Transplantation, Transplantation Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)

Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American Association for Physician Leadership, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, Association for Academic Surgery, Association for Surgical Education, International Society for Heart and Lung Transplantation, National Medical Association, New York Academy of Sciences, Royal College of Physicians and Surgeons of Canada, Society of Critical Care Medicine, Society of Thoracic Surgeons, Canadian Cardiovascular Society

Disclosure: Nothing to disclose.

Chief Editor

Mary C Mancini, MD, PhD, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.


Jeff L Myers, MD, PhD Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts General Hospital; Associate Professor of Surgery, Harvard Medical School

Jeff L Myers, MD, PhD is a member of the following medical societies: American College of Surgeons, American Heart Association, and International Society for Heart and Lung Transplantation

Disclosure: Nothing to disclose.

Ahmad Y Sheikh, MD Resident Physician, Department of General Surgery, Massachusetts General Hospital

Ahmad Y Sheikh, MD is a member of the following medical societies: American College of Surgeons and American Heart Association

Disclosure: Nothing to disclose.

  1. Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg. 1983 Dec. 18(6):894-900. [Medline].

  2. Konez O, Burrows PE. Magnetic resonance of vascular anomalies. Magn Reson Imaging Clin N Am. 2002 May. 10(2):363-88, vii. [Medline].

  3. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982 Mar. 69(3):412-22. [Medline].

  4. Redondo P. [Vascular malformations (I). Concept, classification, pathogenesis and clinical features]. Actas Dermosifiliogr. 2007 Apr. 98(3):141-58. [Medline].

  5. Chang MW. Updated classification of hemangiomas and other vascular anomalies. Lymphat Res Biol. 2003. 1(4):259-65. [Medline].

  6. Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. 2005 Jan. 32(1):99-116, ix. [Medline].

  7. 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 Jul. 136 (1):e203-14. [Medline]. [Full Text].

  8. Enjolras O, Mulliken JB, Boon LM, Wassef M, Kozakewich HP, Burrows PE. Noninvoluting congenital hemangioma: a rare cutaneous vascular anomaly. Plast Reconstr Surg. 2001 Jun. 107(7):1647-54. [Medline].

  9. Takahashi K, Mulliken JB, Kozakewich HP, Rogers RA, Folkman J, Ezekowitz RA. Cellular markers that distinguish the phases of hemangioma during infancy and childhood. J Clin Invest. 1994 Jun. 93(6):2357-64. [Medline]. [Full Text].

  10. Mulliken JB, Fishman SJ, Burrows PE. Wells SA, Creswell LL, eds. Vascular Anomalies. St Louis: Mosby; 2000.

  11. Atherton DJ. Infantile haemangiomas. Early Hum Dev. 2006 Dec. 82(12):789-95. [Medline].

  12. Boye E, Jinnin M, Olsen BR. Infantile hemangioma: challenges, new insights, and therapeutic promise. J Craniofac Surg. 2009 Mar. 20 Suppl 1:678-84. [Medline].

  13. Cheung DS, Warman ML, Mulliken JB. Hemangioma in twins. Ann Plast Surg. 1997 Mar. 38(3):269-74. [Medline].

  14. Folkman J. Fundamental concepts of the angiogenic process. Curr Mol Med. 2003 Nov. 3(7):643-51. [Medline].

  15. Vikkula M, Boon LM, Carraway KL 3rd, et al. Vascular dysmorphogenesis caused by an activating mutation in the receptor tyrosine kinase TIE2. Cell. 1996 Dec 27. 87(7):1181-90. [Medline].

  16. Boon LM, Brouillard P, Irrthum A, et al. A gene for inherited cutaneous venous anomalies ("glomangiomas") localizes to chromosome 1p21-22. Am J Hum Genet. 1999 Jul. 65(1):125-33. [Medline]. [Full Text].

  17. Tille JC, Pepper MS. Hereditary vascular anomalies: new insights into their pathogenesis. Arterioscler Thromb Vasc Biol. 2004 Sep. 24(9):1578-90. [Medline].

  18. Wang QK. Update on the molecular genetics of vascular anomalies. Lymphat Res Biol. 2005. 3(4):226-33. [Medline].

  19. Mulliken JB. Mulliken JB, Young AE. Vascular Birthmarks: Hemangiomas and Vascular Malformations. Philadelphia: WB Saunders; 1988.

  20. Bukowinski AT, Ryan MA, Slymen DJ, Sevick CJ, Alcaraz JE, Smith TC. Haemangiomas and associated congenital malformations in a large population-based sample of infants. Paediatr Perinat Epidemiol. 2008 Nov. 22(6):520-9. [Medline].

  21. Amir J, Metzker A, Krikler R, Reisner SH. Strawberry hemangioma in preterm infants. Pediatr Dermatol. 1986 Sep. 3(4):331-2. [Medline].

  22. Eifert S, Villavicencio JL, Kao TC, et al. Prevalence of deep venous anomalies in congenital vascular malformations of venous predominance. J Vasc Surg. 2000 Mar. 31(3):462-71. [Medline].

  23. Ambrosio MR, Rocca BJ, Di Mari N, Ambrosio A, Lazzi S. Multifocal capillary hemangioma (hemangiomatosis) of the spleen. Tumori. 2012 Jan-Feb. 98(1):e22-6. [Medline].

  24. 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 Mar. 132(3):307-11. [Medline].

  25. Christison-Lagay ER, Fishman SJ. Vascular anomalies. Surg Clin North Am. 2006 Apr. 86(2):393-425, x. [Medline].

  26. Jacobs AH, Walton RG. The incidence of birthmarks in the neonate. Pediatrics. 1976 Aug. 58(2):218-22. [Medline].

  27. Enjolras O, Riche MC, Merland JJ. Facial port-wine stains and Sturge-Weber syndrome. Pediatrics. 1985 Jul. 76(1):48-51. [Medline].

  28. Enjolras O, Mulliken JB. The current management of vascular birthmarks. Pediatr Dermatol. 1993 Dec. 10(4):311-3. [Medline].

  29. Bauer BS, Kernahan DA, Hugo NE. Lymphangioma circumscriptum--a clinicopathological review. Ann Plast Surg. 1981 Oct. 7(4):318-26. [Medline].

  30. Heldner MR, Arnold M, Nedeltchev K, Gralla J, Beck J, Fischer U. Vascular Diseases of the Spinal Cord: A Review. Curr Treat Options Neurol. 2012 Aug 9. [Medline].

  31. Marler JJ, Fishman SJ, Upton J, Burrows PE, Paltiel HJ, Jennings RW, et al. Prenatal diagnosis of vascular anomalies. J Pediatr Surg. 2002 Mar. 37(3):318-26. [Medline].

  32. Trán-Ngoc-Ninh, Trán-Xuân-Ninh. Cystic hygroma in children: a report of 126 cases. J Pediatr Surg. 1974 Apr. 9(2):191-5. [Medline].

  33. Tunç M, Sadri E, Char DH. Orbital lymphangioma: an analysis of 26 patients. Br J Ophthalmol. 1999 Jan. 83(1):76-80. [Medline].

  34. Mulliken JB. Aston SJ, Beasly RW, eds. Grabb and Smith's Plastic Surgery. Lippincott-Raven; 1997. 191-204.

  35. Richter GT, Suen JY. Pediatric extracranial arteriovenous malformations. Curr Opin Otolaryngol Head Neck Surg. 2011 Dec. 19(6):455-61. [Medline].

  36. Harikrishnan S, Bimal F, Ajithkumar V, Bhat A, Krishnamoorthy KM, Sivasubramonian S, et al. Percutaneous treatment of congenital coronary arteriovenous fistulas. J Interv Cardiol. 2011 Jun. 24(3):208-15. [Medline].

  37. Kohout MP, Hansen M, Pribaz JJ, Mulliken JB. Arteriovenous malformations of the head and neck: natural history and management. Plast Reconstr Surg. 1998 Sep. 102(3):643-54. [Medline].

  38. Yamamoto Y, Ushijima T, Takata M, Watanabe G. Surgical treatment of bilateral coronary-to-pulmonary artery fistulas. Interact Cardiovasc Thorac Surg. 2012 Jan. 14(1):102-4. [Medline]. [Full Text].

  39. Pejhan S, Rahmanijoo N, Farzanegan R, Rahimi M. Surgically treatable pulmonary arteriovenous fistula. Ann Thorac Cardiovasc Surg. 2012. 18(1):36-8. [Medline].

  40. Upton J, Coombs CJ, Mulliken JB, Burrows PE, Pap S. Vascular malformations of the upper limb: a review of 270 patients. J Hand Surg [Am]. 1999 Sep. 24(5):1019-35. [Medline].

  41. Hardwicke JT, Titley OG. Thermographic Assessment of a Vascular Malformation of the Hand: A New Imaging Modality. J Clin Imaging Sci. 2016. 6:9. [Medline]. [Full Text].

  42. Berenguer B, Burrows PE, Zurakowski D, Mulliken JB. Sclerotherapy of craniofacial venous malformations: complications and results. Plast Reconstr Surg. 1999 Jul. 104(1):1-11; discussion 12-5. [Medline].

  43. [Guideline] Lee BB, Baumgartner I, Berlien P, Bianchini G, Burrows P, Gloviczki P, et al. Diagnosis and Treatment of Venous Malformations. Consensus Document of the International Union of Phlebology (IUP): updated 2013. Int Angiol. 2015 Apr. 34 (2):97-149. [Medline].

  44. Arneja JS, Gosain AK. Vascular malformations. Plast Reconstr Surg. 2008 Apr. 121(4):195e-206e. [Medline].

  45. Okada A, Kubota A, Fukuzawa M, Imura K, Kamata S. Injection of bleomycin as a primary therapy of cystic lymphangioma. J Pediatr Surg. 1992 Apr. 27(4):440-3. [Medline].

  46. Greinwald JH Jr, Burke DK, Sato Y, Poust RI, Kimura K, Bauman NM, et al. Treatment of lymphangiomas in children: an update of Picibanil (OK-432) sclerotherapy. Otolaryngol Head Neck Surg. 1999 Oct. 121(4):381-7. [Medline].

  47. Burrows PE, Mitri RK, Alomari A, Padua HM, Lord DJ, Sylvia MB, et al. Percutaneous sclerotherapy of lymphatic malformations with doxycycline. Lymphat Res Biol. 2008. 6(3-4):209-16. [Medline].

  48. Zide BM, Glat PM, Stile FL, Longaker MT. Vascular lip enlargement: Part I. Hemangiomas--tenets of therapy. Plast Reconstr Surg. 1997 Dec. 100(7):1664-73. [Medline].

  49. Zide BM, Glat PM, Stile FL, Longaker MT. Vascular lip enlargement: Part II. Port-wine macrocheilia--tenets of therapy based on normative values. Plast Reconstr Surg. 1997 Dec. 100(7):1674-81. [Medline].

  50. Lee BB, Do YS, Yakes W, Kim DI, Mattassi R, Hyon WS. Management of arteriovenous malformations: a multidisciplinary approach. J Vasc Surg. 2004 Mar. 39(3):590-600. [Medline].

  51. Starke RM, Komotar RJ, Hwang BY, Fischer LE, Otten ML, Merkow MB, et al. A comprehensive review of radiosurgery for cerebral arteriovenous malformations: outcomes, predictive factors, and grading scales. Stereotact Funct Neurosurg. 2008. 86(3):191-9. [Medline].

  52. [Guideline] Stereotactic radiosurgery for patients with intracranial arteriovenous malformations (AVM). International RadioSurgery Association (IRSA). Available at March 2009; Accessed: June 24, 2016.

  53. [Guideline] Lee BB, Baumgartner I, Berlien HP, et al; International Union of Angiology. Consensus Document of the International Union of Angiology (IUA)-2013. Current concept on the management of arterio-venous management. Int Angiol. 2013 Feb. 32 (1):9-36. [Medline].

  54. Elsas FJ, Lewis AR. Topical treatment of periocular capillary hemangioma. J Pediatr Ophthalmol Strabismus. 1994 May-Jun. 31(3):153-6. [Medline].

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