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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.

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Medical Therapy

Hemangiomas

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.

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Surgical Therapy

Hemangiomas

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.

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Complications

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.

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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.

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Contributor Information and Disclosures
Author

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.

Acknowledgements

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.

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