Orthopedic Surgery for Hemangioma Clinical Presentation

Updated: Sep 12, 2016
  • Author: Brian J Kistler, MD; Chief Editor: Harris Gellman, MD  more...
  • Print
Presentation

History and Physical Examination

Intramuscular hemangiomas

Intramuscular hemangiomas occur most often in young adults, with 80-90% presenting in individuals younger than 30 years. They occur most often in the lower extremities, especially the thigh, and typically present with a palpable mass, but the overlying skin typically is not discolored.

Intramuscular hemangiomas can be asymptomatic or can present with symptoms including increased girth of the extremity, increased temperature in the area, discoloration of the overlying skin, and pain. They typically are compressible and decrease in size with elevation of the extremity. Exercise often exacerbates the symptoms of pain and swelling due to vascular dilation from increased blood flow through the hemangioma. Larger hemangiomas may be associated with a bruit or thrill.

Often, intramuscular hemangiomas cannot be distinguished definitively from soft-tissue sarcomas on the basis of clinical examination alone.

Large intramuscular hemangiomas occasionally may be associated with significant shunting of blood flow. This is uncommon, but in rare cases, it may lead to heart murmurs, congestive heart failure, or both. If significant shunting exists within the hemangioma, the presentation may be similar to that of an arteriovenous fistula. In this case, it may be possible to elicit the Branham sign, a reflex bradycardia following compression of the arteriovenous fistula, due to reduction in the shunt.

Synovial hemangiomas

Synovial hemangiomas are rare. In tendinous synovium, they typically present as a painless mass. In the synovium of a joint, they may present with recurrent effusions, progressive onset of pain, limited range of motion, limping, and even mechanical symptoms suggesting intra-articular derangement. [25, 19]  A palpable, spongy, compressible mass may be present, and it may decrease in size with elevation of the extremity. The synovium is particularly hypertrophic and causes recurrent hemarthrosis.

The knee is by far the most commonly involved joint, where the presentation may be confused with meniscal or ligamentous pathology. [26]  Both localized and diffuse forms exist.

Osseous hemangiomas

Hemangiomas of bone are often incidental findings and the vast majority are asymptomatic but may cause pain and swelling. Those in the skull may be associated with swelling, erythema, tenderness, or facial deformity.

A locally aggressive subtype of vertebral hemangiomas, though rare (only ~1-2% of lesions), is now well reported. This subtype may cause problems ranging from back pain to nerve root or cord compression and fractures. These locally aggressive vertebral hemangiomas can mimic other pathologies (eg, primary bony malignancy or metastatic disease) and therefore must be accurately diagnosed. [27, 28]

Osseous hemangiomas may be solitary (affecting a single bone) or focal (affecting one bone or, according to some authors, contiguous bones in a focal site). Various authors define hemangiomatosis differently. Some authors define it as multiple hemangiomas located in noncontiguous bones. The condition of multiple bony hemangiomas also has been referred to as cystic angiomatosis of bone when no soft-tissue component is present. Skeletal-extraskeletal angiomatosis has been defined as hemangiomas affecting the medullary canal of a bone, as well as one nonosseous site. The nonosseous site most often is adjacent soft tissue, but alternatively, it may be noncontiguous viscera.

Other authors define hemangiomatosis as lesions involving skin, muscle, and bone, which usually become symptomatic during childhood, with diffuse, persistent swelling and discoloration, with or without pain. Hemangiomatosis can also present with pathologic fracture.

Rarely, hemangioma may be associated with induction of osteomalacia. It is one of many tumor types that may cause osteomalacia.

Gorham disease

Gorham disease can present with dull localized aching pain, swelling, progressive deformity, weakness, and/or muscle atrophy. Patients are often asymptomatic during the osteolytic process until a pathologic fracture occurs or a mass develops. Hemangiomas and vascular malformations can also be present in as many as 60% of patients. [11]  The disease is rarely suspected prior to radiographic evaluation. Patients are usually younger than 40 years.

Kasabach-Merritt syndrome

Kasabach-Merritt syndrome can present with diffuse petechiae and ecchymosis in association with a large soft-tissue mass. If platelet counts drop low enough, spontaneous hemorrhage may result.