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Overview of vascular intervention and surgery for vascular anomalies

Overview of vascular intervention and surgery for vascular anomalies
Literature review current through: Jan 2024.
This topic last updated: Jan 11, 2024.

INTRODUCTION — Vascular anomalies are disorders of the endothelium that affect capillaries, arteries, veins, or lymphatics. They are relatively common, with a prevalence of about 5 percent. Vascular anomalies are broadly classified as either tumors or malformations. Certain lesions can have significant complications and require vascular intervention or surgical management. Recognition of these problematic lesions should prompt referral to an interdisciplinary vascular anomalies center. The major vascular anomalies and primary treatment modalities are summarized (table 1).

An overview of vascular intervention and surgical treatment of these lesions is provided. Additional detail regarding clinical features, diagnosis, and noninterventional management is provided in the linked reviews. (See "Vascular lesions in the newborn" and "Venous malformations" and "Arteriovenous malformations of the extremities".)

GENERAL PRINCIPLES — Successful management of vascular anomalies depends on accurate diagnosis and classification of the lesion. Ninety percent of lesions can be distinguished by history and physical examination. Although individual lesions have specific treatment indications, some generalizations can be made with respect to surgical interventions.

Goals of treatment

Prevent or reverse function-threatening complications.

Minimize disfigurement and psychosocial distress.

Prevent and treat complications.

Avoid aggressive procedures for lesions likely to have an excellent prognosis with no treatment.

Considerations for surgical resection — The following should be considered when surgery is discussed:

Surgical resection may be appropriate for small, well-localized lesions that can be completely removed.

Early surgical resection may be needed for lesions that threaten vital structures, such as the airway or the eye (ie, periorbital lesions).

Surgical resection is likely indicated for lesions that are refractory to nonoperative treatments, including endovascular therapies.

Surgical resection should not leave a visible deformity that is worse than the appearance of the anomaly.

Timing of surgery — Most vascular anomalies are diagnosed within the first few years of life, and many will not require intervention. Determining when to treat these lesions depends on many factors, including the type of lesion, location, size, associated complications, and symptoms. A few generalizations can be made:

Surgical resection for a lesion causing a visible deformity should be considered before four years of age to limit psychological morbidity. This is the age when long-term memory and self-esteem begin to form [1].

Surgical resection for other lesions that benefit from early resection should be delayed until after the age of 12 months, if possible. The risks associated with general anesthesia are lower after this age [2,3].

Some parents may elect to wait until the child is older and able to participate in decision-making and agree to proceeding with surgery, especially if the deformity is minor.

VASCULAR TUMORS — Vascular tumors have rapidly proliferating endothelium.

Infantile hemangioma — Infantile hemangiomas (IHs) are benign tumors of vascular endothelium and the most common tumors of childhood. Most children with an IH are observed. Approximately 90 percent will proliferate and involute without major sequelae, and no intervention is required. During the proliferative phase, 10 percent will ulcerate, bleed, or deform/obstruct important structures. (See "Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and complications" and "Infantile hemangiomas: Evaluation and diagnosis".)

In general, treatment is recommended for IH that: involves vital structures; causes functional impairment such as impairment of feeding or sight; is associated with structural abnormalities; or is at an increased risk of ulceration, scarring, and disfigurement [4]. The American Academy of Pediatrics published clinical practice guidelines to help providers identify high-risk patients who require referral to an IH specialist [4].

Superficial lesions that satisfy these conditions and are treated before 12 weeks of age respond well to topical timolol [4-6]. Thicker, well-localized hemangiomas <3 cm in diameter can be managed with intralesional triamcinolone [7]. Problematic thick hemangiomas >3 cm require systemic treatment; oral propranolol is first line and oral prednisolone is second line [4,8]. (See "Infantile hemangiomas: Management", section on 'Local therapies' and "Infantile hemangiomas: Management", section on 'First-line therapy'.)

Tumors in certain locations (lip, neck, and anogenital) are at an increased risk of ulceration. Approximately 16 percent of all lesions will ulcerate at a median age of four months [9]. Those at increased risk of ulceration can be covered with hydrated petroleum to reduce desiccation. Once ulceration develops, the wound should be washed with soap and water at least twice daily and protected with a petroleum gauze barrier. Most lesions will heal with local wound care and prevention of incidental trauma.

Timing of intervention

Proliferative phase (infancy) — Surgical management during the proliferative phase in infancy is rare due to excess risks associated with excision of these highly vascular tumors (eg, excess blood loss, iatrogenic injury, inferior aesthetic outcome) compared with the minimal risk of excising residual fibrofatty tissue once the tumor has regressed. Morbidity associated with anesthesia is also greater in infants compared with children older than one year of age [2,3].

While operative intervention for IH generally is not advisable in infancy, appropriate indications include [4,10]:

Failure of local wound care and/or pharmacotherapy

Well-localized tumor in an anatomically safe area

Complicated reconstruction not required

Resection will be necessary in the future, and the eventual scar would be the same

As an example, a localized, ulcerated scalp IH may be managed by excision because the risk of iatrogenic injury is low and the laxity of the scalp in infancy facilitates closure.

Pulse-dye laser treatment for proliferating IH has been advocated by some authors; however, almost all tumors are beyond the reach of the laser. The laser penetrates only 0.75 to 1.2 mm into the dermis and thus only affects the superficial portion of the IH, causing some lightening. The pulse-dye laser has not been shown to decrease IH bulk or accelerate involution [11,12]. Rather, the thermal injury delivered by the laser to the ischemic dermis increases the risk of ulceration and subsequent hypopigmentation and scarring.

Involuted phase (after three years of age) — Resection of IH during early childhood is much safer because the lesion is less vascular and smaller. Pulse-dye laser has a definite role in treating residual telangiectasias in the involuted phase [13]. (See "Laser and light therapy for cutaneous vascular lesions", section on 'Infantile hemangiomas'.)

Approximately 50 percent of IH leave behind fibrofatty tissue or damaged skin after the tumor regresses, causing a deformity. Less often, children require reconstruction of damaged structures (ie, nose, ear, or lip). Most IHs have completed involution before four years of age [10,14]. Waiting until IH has fully involuted ensures that the least amount of fibrofatty tissue and damaged skin will need to be resected, leaving the smallest possible scar. Patients with telangiectasias, redundant skin, or scar tissue that is disfiguring or problematic should be referred to a hemangioma specialist [4].

If it is obvious that a patient will require an operation, it is best to intervene before four years of age, when long-term memory and personal identity begin to develop [1]. After three years of age, the hemangioma has completed most or all of involution, and the deformity can be corrected to prevent psychosocial morbidity. Some parents may elect to wait until the child is older and able to make the decision for operative intervention on their own, particularly if the deformity is minor.

Techniques — Before excising an IH, the area should be infiltrated with an epinephrine-containing local anesthetic to minimize blood loss. Because the tumor acts as a tissue expander, adequate skin is usually available for closure of the wound.

Traditional lenticular excision/linear closure is applicable in some locations, such as the scalp, eyelid, lip, and abdomen. Circular excision/purse-string closure should be considered for circular lesions located on the face (picture 1) [15]. This technique takes advantage of the skin laxity produced by the hemangioma and minimizes the length of the scar, as well as distortion of surrounding structures. Lenticular excision/linear closure results in a scar as long as three times the diameter of the lesion, whereas a two-stage circular resection followed by lenticular excision 6 to 12 months later will leave a scar approximately the same length as the diameter of the original hemangioma [15]. Not all parents elect for a second stage to convert the ovoid scar into a linear scar because the circular scar can be difficult to appreciate; it may appear similar to an acne or chicken pox scar. The disadvantage of circular excision and purse-string closure is that a second stage may be needed, and the risk of suture line dehiscence is higher.

Congenital hemangioma — Congenital hemangioma is a rare vascular tumor that forms in utero. It is fully grown at birth and does not exhibit postnatal proliferation. The two main forms are a rapidly involuting congenital hemangioma (RICH), which undergoes accelerated regression over the first year of life [16,17], and a noninvoluting congenital hemangioma (NICH) that does not regress [18]. (See "Congenital hemangiomas: Rapidly involuting congenital hemangioma (RICH), noninvoluting congenital hemangioma (NICH), and partially involuting congenital hemangioma (PICH)".)

Timing of intervention — Reconstruction of damaged structures can be considered between three to four years of age, before long-term memory and self-esteem develop. Alternatively, some families may elect to wait until the child wishes to have the area improved later in childhood or during adolescence.

RICH does not require operative management in infancy because it undergoes rapid postnatal regression; 50 percent of lesions have completed involution by seven months of age, and the remaining tumors have resolved by 14 months [16,17]. After regression, RICH may cause a residual deformity such as atrophic skin, thin subcutaneous tissue, and prominent draining veins. RICH can leave behind fat atrophy, in contrast to IH. In some instances, a residual deformity can be improved by resection or fat transfer.

NICH is almost never problematic in infancy. If the lesion causes enough of a deformity that it bothers the patient, it can be excised.

Techniques

RICH — Operative intervention for RICH must not create a more obvious scar than the lesion. Because RICH may leave behind atrophic tissue, reconstruction with autologous grafts (fat, dermis) or acellular dermis may be indicated. Residual telangiectasias can be lightened with a pulse-dye laser. Prominent draining veins can be sclerosed.

NICH — Because the lesion is benign, the operative outcome of resecting the lesion must be less noticeable than the tumor itself. Pulse-dye laser therapy may improve the appearance of the NICH by eliminating telangiectasias.

Kaposiform hemangioendothelioma — Kaposiform hemangioendothelioma (KHE) is a low-grade malignancy that does not metastasize. KHE usually presents in infancy and is locally aggressive but does not metastasize [19-21]. The tumor causes deformity and pain, and 50 to 70 percent of patients exhibit Kasabach-Merritt phenomenon (KMP; thrombocytopenia <25,000/mm3, petechiae, and bleeding) [19-21]. Because lesions are typically greater than 5 cm, diffuse, and involve multiple tissue planes, they are rarely amenable to resection [19,20]. Symptomatic patients with KHE are treated with sirolimus or vincristine [22,23]. (See "Tufted angioma, kaposiform hemangioendothelioma (KHE), and Kasabach-Merritt phenomenon (KMP)".)

Patients diagnosed with KMP should not receive platelet transfusions [23]. The thrombocytopenia is not corrected, platelets become trapped in the lesion, and the tumor can enlarge [23]. Typically, platelet count will normalize by the age of two years once the tumor begins to undergo involution.

Operative management is possible for small lesions that present in older children and adults [20]. Extirpation of a larger tumor may be indicated for symptomatic patients with well-localized lesions or for those who have failed chemotherapy. Resection is not required for extensive tumors that are not causing functional problems because KHE is benign. The risks of the resection and the resulting deformity should be weighed against the appearance of the tumor.

Timing of intervention — Most KHEs are diffuse and difficult to excise; they are best managed with chemotherapy until they "burn out" in early childhood. KHE partially regresses after two years of age into mid-childhood. The tumor persists long term, and although most are asymptomatic, some patients have chronic pain and contractures [24]. Excision is considered for resectable lesions to prevent expansion, chemotherapeutic morbidity, or chronic pain.

Techniques — Small, localized KHE may be resected in a lenticular fashion and closed primarily. Wound closure should not be complicated by obtaining wide margins. Some defects require complex reconstruction with skin grafts or flaps. In addition, the tumor can cause secondary deformities, such as contractures, that may necessitate reconstruction.

Pyogenic granuloma — Pyogenic granulomas (PGs) are small (mean diameter 6 mm), benign vascular tumors often complicated by ulceration, bleeding, and deformity [25]. Most patients require operative intervention to prevent bleeding and ulceration and to improve a visible deformity. (See "Pyogenic granuloma (lobular capillary hemangioma)".)

Timing of intervention — PG should be treated soon after diagnosis to prevent or stop ulceration and bleeding. Although PG may become temporarily smaller after bleeding and crusting, the lesion typically enlarges to become symptomatic again.

Techniques — Numerous treatment methods have been described for PG, including curettage, shave excision, laser therapy, imiquimod cream, cryotherapy, and silver nitrate cauterization [25,26]. Because the lesion involves the reticular dermis, a component is not reached by some of these modalities, and recurrence rates as high as 50 percent have been reported [25]. The definitive treatment is full-thickness cutaneous excision. With full-thickness excision, the recurrence rate is close to 0 percent [25,26].

VASCULAR MALFORMATIONS — Vascular malformations are anomalies of the vasculature resulting from developmental defects involving the arterial and venous origins, but without significant endothelial cell hyperplasia that occurs in vascular tumors. Vascular malformations can include arterial, capillary, lymphatic, or venous elements, or a mixture of these. Vascular malformations are classified as slow-flow (low-flow) or fast-flow (high-flow) lesions, which determines the nature of treatment.

Capillary malformation — Capillary malformations (CM; port wine stains or nevus flammeus, MIM #163000) are slow-flow vascular malformations composed of dermal capillaries and postcapillary venules. Capillary malformation causes cosmetic and psychologic concerns as the color darkens and the skin thickens, sometimes with raised fibrovascular cobblestoning. Pulse-dye laser therapy can improve the appearance by lightening the color: the head and neck region has a better response compared with the extremities. Outcomes are also superior for smaller lesions and for those treated at a younger age [27]. After pulse-dye laser treatment, CM often re-darkens over time [28]. Pyogenic granulomas may develop in CM, causing ulceration and bleeding. CM also can lead to soft tissue and skeletal overgrowth below the stain, worsening the patient's deformity [29]. (See 'Pyogenic granuloma' above and "Capillary malformations (port wine birthmarks) and associated syndromes".)

Timing of intervention — Facial CM is best treated with pulse-dye laser early in childhood, before memory or self-awareness develops. Intervention in infancy may achieve superior lightening as well as reduce the risk of subsequent darkening and hypertrophy, compared with photocoagulation in later childhood [27]. Some families may elect to wait to treat CM of the trunk or extremities until the child is old enough to make the decision. Because overgrowth is not present at birth and is progressive, most patients do not require contouring until adolescence or adulthood. Cutaneous fibrovascular hypertrophy occurs over many years, requiring intervention in adulthood. Malocclusion may be corrected in adolescence with orthodontic manipulation. If orthodontics are insufficient, an orthognathic procedure is considered when the jaws are completely grown, usually at age 16 years in females and age 18 years in males.

Techniques — Infants can be treated with pulse-dye laser while awake (using topical anesthesia) depending on the size and location of the CM. After infancy, general anesthesia is preferred unless the lesion is small, given the difficulties restraining an awake child. Adolescents generally tolerate laser treatment while awake, depending on the location and extent of the CM. Multiple treatments, spaced six weeks apart, are often required until the CM fails to improve with additional treatments.

Soft tissue overgrowth may be associated with increased subcutaneous adipose tissue. Suction-assisted lipectomy can improve contour while avoiding a large incision [30]. Lesions associated with ulcerated PG (picture 2), severe cutaneous thickening, and cobblestoning can be resected and reconstructed using linear closure or local flaps.

If a significant occlusal cant or malocclusion remains after orthodontic treatment, Le Fort 1 osteotomy or bimaxillary procedure may be necessary. Facial asymmetry caused by overgrowth of the zygoma, maxilla, or mandible can be improved by contour burring.

Lymphatic malformation — Lymphatic malformation (LM) is an anomaly involving the development of the lymphatic vasculature. Lymphatic malformations can be classified as macrocystic or microcystic. LM does not require intervention, unless it is large or symptomatic [31]. Complications of LM can include obstruction or distortion of vital structures, infection, bleeding, and pain. A newborn with a cervicofacial LM may have respiratory compromise and require tracheostomy.

Sclerotherapy shrinks the lesion and alleviates symptoms and is first-line treatment for problematic macrocystic LM [32,33]. Sclerotherapy has superior efficacy and a lower complication rate compared with resection and should be considered for large, asymptomatic macrocystic lesions to reduce the risk of future intralesional bleeding or infection. Surgical removal of macrocystic LM is indicated if the lesion is symptomatic and sclerotherapy is no longer possible, or resection is possibly curative because the lesion is small and well localized.

A microcystic LM is a lesion that is comprised of cysts too small to be sclerosed (typically <5 mm). Nonproblematic microcystic LMs can be observed. Nonoperative management options include carbon dioxide laser, which is used for cutaneous bleeding and weeping of lymph fluid, and radiofrequency ablation, which is useful for intraoral and airway lesions [34]. Oral sirolimus can be effective for diffuse problematic lesions [35]. Intralesional bleomycin may be used to shrink microcystic LMs [33]. Resection should be considered for lesions causing disfigurement, but the risks and deformity caused by the procedure should be weighed against the appearance of the lesion.

Timing of intervention — Nonproblematic microcystic LM that cannot be easily sclerosed may be observed; resection is reserved until the lesion becomes symptomatic. Sclerotherapy for asymptomatic macrocystic LM should be considered early to lower the risk of future bleeding or infection. If possible, intervention should be postponed until after the patient is 12 months of age when the risks associated with anesthesia are lower.

Because LM progresses over time, asymptomatic patients often seek intervention because of a worsening deformity during late childhood or adolescence.

Techniques — Sclerotherapy is used to treat macrocystic lymphatic malformation. The cysts are aspirated and then injected with an inflammatory substance to cause scarring and to shrink the lesion. Commonly used sclerosing agents include doxycycline, sodium tetradecyl sulfate, ethanol, and bleomycin. The procedure is typically performed with general anesthesia, and imaging guidance with ultrasound or fluoroscopy is used. The results of treatment take up to eight weeks to be seen, and some lesions require serial injections every six to eight weeks. Ulceration is the most common complication of sclerotherapy and is managed with local wound care. For mucosal vesicles in the oral cavity, radiofrequency ablation is the preferred treatment modality [34].

Rarely, a localized LM may be totally excised and the wound edges reapproximated without complex reconstruction (picture 3). Because LM often is diffuse and involves multiple tissue planes, complete resection is difficult, and recurrence is common. Local anesthetic with epinephrine should be used because significant blood loss can occur during extirpation. Subtotal resections of problematic areas, such as bleeding vesicles or an overgrown lip, should be carried out rather than attempting "complete" excision, which would result in a worse deformity than the malformation. Diffuse lymphatic malformations often require a staged resection of defined anatomic regions. LM involving the head and neck may be resected using a coronal (forehead, orbit), tarsal (eyelid), preauricular-melolabial-transoral (cheek), or transverse mucosal (lip) incision. Macroglossia may require a tongue reduction procedure. Bony overgrowth is corrected by osseous contouring, and malocclusion may require orthognathic correction.

Venous malformation — Venous malformations (VMs) are slow-flow vascular malformations composed of an abnormal network of veins. VMs can cause pain and psychological distress. VMs also can obstruct or distort important anatomic structures. First-line management is sclerotherapy, which is safer and more effective than resection [36]. The sclerosant destroys the endothelium of the small channels, causing thrombosis, inflammation, scarring, and shrinkage of the lesion. Multiple treatments are often required. Ulceration is the most common complication but occurs in less than 5 percent of patients. Although sclerotherapy effectively reduces the size of the lesion and improves symptoms, it does not remove the malformation. The lesions often re-expand, and patients may require serial treatments. (See "Venous malformations".)

Surgical extirpation is not favored in general because the entire lesion can rarely be removed, resection may cause a worse deformity than the lesion, the risk of recurrence is high, and the risk of blood loss and iatrogenic injury is significant. However, surgery may be preferred to sclerotherapy as the initial treatment for the following lesions [37]:

Small, well-localized lesions that can be easily excised for cure

Glomuvenous malformations that respond less favorably to sclerotherapy

VMs involving the palmar aspect of the hand or adjacent to an important nerve, such as the facial nerve

Resection should also be considered for persistent deformity after completion of sclerotherapy (patent channels are not accessible for further injection). After sclerotherapy, the VM is replaced by scar and thus the risk of blood loss, iatrogenic injury, and recurrence is low. In addition, fibrosis facilitates resection and reconstruction.

Timing of intervention — Nonproblematic VMs may be observed. Intervention is reserved for symptomatic lesions that cause pain, significant deformity, or threaten vital structures, or for asymptomatic phlebectatic areas at risk for thromboembolism. Most children do not require treatment at the time of diagnosis. Because VM slowly expands, patients may develop pain or worsening deformity, which then stimulates intervention. Less commonly, VM involving an anatomically sensitive area or causing gross deformity necessitates management as early as infancy.

Techniques — Almost all VMs should have sclerotherapy prior to operative intervention to facilitate the resection and improve the outcome. Some small, well-localized VMs may be removed without preoperative sclerotherapy.

Larger lesions can require a staged approach with serial excision and reconstruction. Occasionally, large VMs can be managed with a single-stage excision and linear closure (picture 4). Subtotal excision may be appropriate for problematic lesions if a larger resection may result in a worse deformity.

Head and neck VMs can be resected through the following approaches: coronal (forehead, orbit), tarsal (eyelid), preauricular-melolabial-transoral (cheek), or transverse mucosal (lip) incision. Preoperative counseling should include discussion that it is common for VMs to expand following excision and that additional operative intervention may be required.

Arteriovenous malformation — Arteriovenous malformations (AVMs) are fast-flow lesions that involve the connection of arterial and venous vessels through either a nidus or fistula. AVMs expand over time and can cause bleeding, pain, ulceration, and destruction of vital structures. Intervention depends on symptoms and the location of the lesion. AVMs are benign, and management is aimed at alleviating symptoms, preserving vital functions, and improving deformity [38]. Options for treatment include sclerotherapy, embolization, and resection. The lesions are staged according to the Schobinger staging system (table 2). (See "Arteriovenous malformations of the extremities", section on 'Clinical staging'.)

AVMs are treated by embolization, usually followed by resection. AVM is often diffuse and involves important anatomical structures. Consequently, cure is rare, and the usual goal of treatment is to control the lesion. Embolization effectively treats symptoms and complications by reducing bleeding, pain, and the size of the malformation. Embolization does not remove the AVM, and the lesions are at high risk of re-expansion. Embolization is indicated for large, diffuse lesions that are not easily amenable to resection or to manage acute bleeding.

Resection has a lower recurrence rate than embolization and should be considered for well-localized lesions or to correct focal deformities caused by the AVM (eg, labial hypertrophy). Wide resection and reconstruction of large, diffuse AVM should be exercised with caution because cure is rare, recurrence rate is high, the resulting deformity is often worse than the appearance of the malformation, and the procedure is associated with major blood loss, iatrogenic injury, and morbidity.

Timing of intervention — Symptomatic AVMs should be treated. Approximately two-thirds of patients will require intervention in childhood or adolescence; the remaining patients do not require management until adulthood.

Asymptomatic lesions in a difficult area (face or hand) are best observed because resection and reconstruction may result in a more noticeable deformity or worse functional problem. In addition, embolization or incomplete excision can cause the lesion to enlarge and become symptomatic. Asymptomatic AVM in a nonanatomically important location (trunk, proximal limb) may be removed with minimal morbidity before the lesion progresses to a higher stage, which will be more difficult to resect and is associated with a higher recurrence rate. Similarly, a small, well-localized AVM should be excised for possible cure before it expands and complete removal is no longer possible. Although the recurrence rate is lower when asymptomatic AVMs are resected, re-expansion is still common, though some AVMs remain nonproblematic into adulthood [39].

Techniques — Embolic material should be delivered to the nidus of the lesion, not into the feeding arterial vessels. Occlusion of inflow causes collateralization and expansion of the AVM; furthermore, access to the nidus may be blocked, preventing future embolization. When resection is planned, preoperative embolization facilitates the procedure by reducing blood flow to the lesion and facilitating the dissection (picture 5). Multiple embolizations are often required prior to resection. Ideally, resection should be carried out within one week after the final embolization. Patients and families are counseled that many AVMs treated by resection will recur within five years following resection [38].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Infantile hemangiomas and PHACE syndrome" and "Society guideline links: Vascular anomalies".)

SUMMARY AND RECOMMENDATIONS

Vascular anomalies – Vascular anomalies encompass a heterogeneous group of vascular tumors and malformations. The terminology surrounding these lesions is complex, which can lead to frequent errors in diagnosis and management. Therefore, problematic lesions are often best managed at a multidisciplinary vascular anomalies center. (See 'Introduction' above.)

General principles – Indications and timing of surgery vary depending on the type of anomaly. The major vascular anomalies and primary treatment modalities are summarized (table 1). In general, surgery is reserved for symptomatic lesions refractory to nonoperative management and small, localized lesions that are easily resected. Early intervention may be needed for lesions that threaten anatomically vital structures. The surgical scar should not result in a deformity greater than that caused by the vascular anomaly. (See 'General principles' above.)

Vascular tumors – Vascular tumors are the result of pathologic angiogenesis. These include infantile hemangioma, congenital hemangioma, kaposiform hemangioendothelioma, and pyogenic granuloma.

Infantile hemangioma – Most children with infantile hemangioma are observed. When indicated, surgical intervention should be postponed until the completion of the involuting phase after three years of age to minimize the risk of bleeding and complications. (See 'Infantile hemangioma' above.)

Congenital hemangioma – Rapidly involuting congenital hemangioma (RICH) does not require operative management in infancy because it undergoes rapid postnatal regression. Non-involuting congenital hemangioma (NICH) does not regress, and therefore the threshold to resect problematic NICH is lower. (See 'Congenital hemangioma' above.)

Kaposiform hemangioendotheliomas – Most kaposiform hemangioendotheliomas are not amenable to resection and are managed with chemotherapy. They can be associated with thrombocytopenia, petechiae, and bleeding known as the Kasabach-Merritt phenomenon. (See 'Kaposiform hemangioendothelioma' above.)

Pyogenic granuloma – Pyogenic granuloma commonly presents as a small, ulcerated lesion. Surgical resection is almost always required soon after diagnosis to prevent or stop ulceration and bleeding. (See 'Pyogenic granuloma' above.)

Vascular malformations – Vascular malformations are anomalies of the vasculature resulting from developmental defects involving the arterial and venous origins. Vascular malformations can include arterial, capillary, lymphatic, or venous elements, or a mixture of these.

Capillary malformations – Capillary malformations are commonly treated with pulse-dye laser. Surgery may be required for lesions associated with overgrowth. (See 'Capillary malformation' above.)

Lymphatic malformations – Sclerotherapy is the first-line treatment for problematic macrocystic lymphatic malformations. Treatment options for problematic microcystic lymphatic malformations include resection, carbon dioxide laser, radiofrequency ablation, intralesional bleomycin injection, and sirolimus. (See 'Lymphatic malformation' above.)

Venous malformations – Sclerotherapy is also the first-line treatment for problematic venous malformations. It should almost always be performed prior to surgery to help reduce the size of the venous malformation, minimize blood loss, and reduce recurrence. (See 'Venous malformation' above.)

Arteriovenous malformations – Arteriovenous malformations are rarely cured, and interventions are aimed at controlling symptoms. Embolization helps to palliate symptoms and minimize bleeding due to resection. Most lesions require total or subtotal resection. (See 'Arteriovenous malformation' above.)

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Topic 121010 Version 6.0

References

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