Orthopedic Surgery for Hemangioma Treatment & Management

Intramuscular hemangiomas

The natural history of many intramuscular hemangiomas is that of gradual fatty replacement, atrophy, and involution over time, as suggested by their greater frequency in individuals younger than 30 years and their relative rarity in older adults. Many intramuscular hemangiomas are asymptomatic or produce only mild symptoms with activity, even during the active adolescent years. Some, however, may increase in size and become more symptomatic in adulthood, leading to their diagnosis.

Asymptomatic hemangiomas can be observed. Symptomatic hemangiomas can be treated conservatively with activity modification and pain medications, but if the discomfort is refractory to conservative management, embolization or surgical excision can be considered. Hemangiomas can recur after surgical excision; often, more than one course of embolization will be required to treat the hemangioma.  

Synovial hemangiomas

The natural history of synovial hemangiomas may be similar to that of their intramuscular counterparts, but their rarity makes this difficult to document. The focal type is more frequently amenable to surgical excision than the diffuse type is.

Osseous hemangiomas

Symptomatic or locally aggressive vertebral hemangiomas are usually treated conservatively when symptoms are limited to mild-to-moderate pain, whereas neurologic deficit and intractable pain are widely accepted in the literature as common indications for surgical intervention. ^[34]  Large symptomatic osseous hemangiomas that affect long bones can cause considerable discomfort and give rise to a risk of pathologic fracture. Curettage, bone grafting or cementation, and prophylactic stabilization can be performed. Preoperative embolization should be considered to reduce intraoperative blood loss.  

Hemangiomatosis

Both osseous hemangiomatosis and skeletal-extraskeletal angiomatosis often become symptomatic during childhood, with pain and diffuse swelling. Perhaps even more significantly, extraskeletal manifestations of hemangiomatosis can lead to hepatic dysfunction and cardiac complications. Because of the extensive nature of the disease, chemotherapy has been used with some success.

Gorham disease

The natural history of Gorham disease is poorly defined. Extent and pace of bone loss are variable. No standard treatment is currently available for Gorham disease. Regression of lesions or stabilization of disease has been reported with steroids, radiation, surgery, bisphosphonates, zoledronic acid, and interferon alfa. ^[11]  Because of the unpredictable natural history and the potentially devastating effects of progressive disease, treatment generally should be instituted upon diagnosis.

Kasabach-Merritt syndrome

Kasabach-Merritt syndrome is a potentially life-threatening coagulopathy that is related to platelet trapping in a large cavernous hemangioma. Approximately 30% of patients who develop this complication die from hemorrhage or infection. Surgical resection of the hemangioma often is difficult. Consequently, steroids, radiation therapy, interferon alfa-2a, and pentoxifylline have been used in attempts at treatment.

Tumor-induced osteomalacia

Tumor-induced osteomalacia results in diffuse osteopenia with marked hypophosphatemia, low serum calcium, and increased serum alkaline phosphatase (ALP). Because osteomalacia generally resolves with excision of the tumor, surgical treatment usually is indicated.

Future developments

The true nature of hemangiomas remains controversial: There is no clear consensus on an inciting cause, nor is there agreement as to whether hemangiomas are neoplasms, hamartomas, or malformations. Future investigations likely will attempt to answer these questions, and may lead to innovations in therapy. Work is underway on the therapeutic use of angiogenic cytokines and angiogenesis inhibitors, including systemic administration of the antiangiogenic proteins AGM-1470 and angiostatin or of the matrix metalloproteinase (MMP) inhibitor batimastat, as well as gene gun therapy with interleukin (IL)-12.

Intramuscular hemangiomas

Observation is appropriate for asymptomatic or mildly symptomatic hemangiomas of skeletal muscle and bone. If symptoms cannot be managed adequately by means of activity modification and nonnarcotic analgesics, further treatment may be considered. Embolization may be used to provide symptomatic relief of intramuscular hemangiomas. When surgical excision is planned, embolization also may be used preoperatively to decrease intraoperative blood loss and postoperative recurrence. ^[39]

Synovial hemangiomas

Although local pedunculated synovial hemangiomas are removed surgically, more diffuse lesions may be treated with intra-articular low-dose radiation therapy, open excision, or both when sufficiently symptomatic.

Osseous hemangiomas

Hemangiomas of bone rarely require treatment. If symptoms are significant enough to warrant consideration of treatment, it is important to confirm the diagnosis; more aggressive neoplasms (eg, metastatic renal cell carcinoma) may masquerade as hemangioma.

Radiation may be considered for symptomatic hemangiomas in surgically inaccessible sites, such as vertebral hemangiomas. ^{[40, 41]} However, some authors have found that selective arterial embolization is safer and more effective in the treatment of symptomatic vertebral lesions.

Bleacher et al proposed a management algorithm for symptomatic and locally aggressive vertebral hemangiomas, ^[23] in which symptomatic patients were divided into those with pain only and those with neurologic deficits. Patients with pain were treated with transarterial embolization or vertebroplasty after failing conservative management, whereas patients who presented with neurologic deficits were treated with transarterial embolization, decompressive laminectomy, and vertebroplasty.

Symptomatic and locally aggressive vertebral hemangiomas have been reported in the pediatric population; however, because of the paucity of clinical data in this group of patients, no clinical guidelines have been established in the literature. Accordingly, treatment should be individualized. ^[21]

Hemangiomatosis

Chemotherapy has been used in the treatment of extensive hemangiomatosis, particularly when the vascular proliferation is life- or limb-threatening.

Gorham disease

No standard treatment is currently recognized for Gorham disease. Regression of lesions or stabilization of disease has been reported with steroids, radiation, surgery, bisphosphonates, zoledronic acid, and interferon alfa. ^[11] Local resection with arthroplasty has been reported, with no evidence of recurrence in periarticular disease.

Kasabach-Merritt syndrome

Kasabach-Merritt syndrome is treated with supportive measures, particularly transfusion of platelets. ^[42] Steroids also have been used in the treatment of Kasabach-Merritt syndrome, with some success. Sirolimus has shown some success when steroids are ineffective. ^[43]

Good results of treatment with pentoxifylline were reported by de Prost et al. ^[44] Pentoxifylline acts to restore blood flow and seems to possess antithrombotic activity as well. Pharmacologic management with interferon alfa-2a has also been attempted.

Radiation therapy has had variable success. Surgical resection of the hemangioma often is difficult.

In a study evaluating the clinical characteristics, treatments, and outcomes of neonates with Kasabach-Merritt syndrome, Wang et al concluded that steroid therapy had a low degree of efficacy and was associated with a high rate of relapse, whereas arterial embolization was effective. ^[45] Accordingly, they recommended that a combination of steroid therapy and embolization therapy be considered as first-line treatment of neonatal Kasabach-Merritt syndrome and that vincristine be considered if that approach is ineffective.

Intramuscular hemangiomas

Angiography is an important aspect of preoperative planning when the vascular supply of the lesion is in question and when preoperative embolization is considered. Embolization of high-flow lesions may be performed by interventional radiology to decrease intraoperative blood loss and to decrease the risk of postoperative recurrence. Low-flow lesions are not treatable by embolization. Instead, sclerosing agents may be used to decrease blood flow through low-flow hemangiomas.

Surgical excision attempts to achieve a marginal border unless the hemangioma is contained within a single muscle belly that can be excised completely to achieve a wide margin. The rate of local recurrence following wide excision has been reported as less than 10%, whereas local recurrence rates after marginal excision range from 25% to much higher. Meticulous hemostasis is essential in the prevention of postoperative hematoma.

Excision of symptomatic intramuscular hemangiomas can provide permanent relief. However, because complete excision is required for long-lasting satisfactory results, this treatment option generally is restricted to hemangiomas contained within a single muscle belly. ^[46] Even so, complete resection is not always possible; when incompletely resected, hemangiomas nearly always recur. In addition, surgery can be associated with large-volume blood loss, even when preoperative embolization is employed.

Laser-knife excision of hemangiomas is a technique developed to allow better control of intraoperative bleeding. Preoperative ultrasound-guided hook-wire localization may aid in defining the extent of a hemangioma during excision. Wang et al found it to be safe and effective for nonpalpable intramuscular hemangiomas and concluded that it may provide a better cosmetic result and improved functional recovery. ^[47]  Radiation has been used to treat soft-tissue hemangiomas in surgically inaccessible or potentially dangerous sites.

Postoperatively, the surgical site is wrapped in a compressive dressing. The patient is required to maintain a minimal level of activity. Both of these measures are instituted to prevent the occurrence of postoperative hematoma.

Osseous hemangiomas

Transarterial embolization can be a definitive treatment option or a preoperative intervention ^[48] followed by surgical decompression for the treatment of symptomatic vertebral hemangiomas. Significant blood loss has been described as a complication of surgical treatment of locally aggressive vertebral hemangioma in patients with neurologic deficits. ^{[11, 23]}

In a retrospective study of 15 patients (six men, nine women) with aggressive vertebral hemangiomas, Handa et al reported that a combination of preoperative transarterial embolization and total tumor excision (including the tumor margins) yielded satisfactory results. ^[49]  

A multicenter study using data compiled by the AOSpine Knowledge Forum Tumor Investigators found that formal en-bloc resection was not required for symptomatic primary spinal hemangiomas and that aggressive intralesional resection during index surgery was capable of yielding excellent rates of local control and long-term survival. ^[50]

Synovial hemangiomas

Local pedunculated synovial hemangiomas are removed surgically, often through an arthroscope.

In both intramuscular and osseous hemangiomas, hemorrhage can occur spontaneously during biopsy or during surgical resection; such hemorrhages can be massive. ^{[26, 23]} Furthermore, hemangiomas typically recur following incomplete surgical excision. Some large hemangiomas may result in shunting of the blood to a degree significant enough to cause congestive heart failure.

Rarely, cord compression and neurologic deficits can result from vertebral hemangiomas. Approximately 30% of patients with Kasabach-Merritt syndrome die of hemorrhage or infection. Osteomalacia is an uncommon complication that has been associated with hemangioma.

Follow-up is required to assess symptomatic relief and to monitor for possible recurrence.