In-Service Exam
Hemangiomas - 2001





A 7-year-old boy has a mass covering the entire right cheek that was present at birth and has progressively enlarged with the child. On physical examination, a thrill can be palpated over the mass, and the skin temperature is increased in the area of the lesion. A bruit can be heard on auscultation. MRI shows a high-flow lesion with multiple feeding vessels.

Which of the following is the most appropriate management?

(A) External beam radiation therapy
(B) Ligation of feeding vessels
(C) Laser ablation
(D) Angiographic embolization
(E) Embolization followed by excision

The correct response is Option E.

This patient has an arteriovenous malformation, or AVM, which is characterized by rapid, turbulent blood flow through multiple feeding vessels from the high-pressure arterial system to the low-pressure venous system. These lesions are often pulsatile; a thrill or bruit will be heard on auscultation. MRI is most helpful in delineating the anatomy of the AVM and its flow characteristics.

Because treatment can be associated with the same life-threatening complications as the existence of the lesion itself, appropriate management typically requires multi-modality therapy, including preoperative correction of any underlying consumptive coagulopathy, angiographic embolization of feeding vessels, complete surgical excision of the malformation, and soft-tissue reconstruction.

Radiation therapy and laser ablation are not appropriate management options for a patient with a congenital AVM. Ligation of feeding vessels or angiographic embolization alone will only worsen this patient's condition.

References
1. Pribaz JJ, Weiss DD, Mulliken JB, et al. Prelaminated free flap reconstruction of complex central facial defects. Plast Reconstr Surg. 1999;104:357-365.
2. Young AE. Venous and arterial malformations. In: Mulliken JB, Young AE, eds. Vascular Birthmarks: Hemangiomas and Malformations. Philadelphia, Pa: WB Saunders Co; 1988:196-214.

A 5-month-old infant has a nodular, nonulcerated 2-cm capillary hemangioma of the upper eyelid that is obstructing the visual axis; a photograph is shown above. His parents report that the lesion grew rapidly and then decreased in size. Which of the following is the most appropriate next step in management?

(A) Systemic administration of corticosteroids
(B) Intralesional injection of corticosteroids
(C) Interferon alfaÐ2a therapy
(D) Radiation therapy
(E) Surgical resection of the lesion

The correct response is Option E.

In this infant who has a hemangioma that is obstructing the visual axis, the most appropriate management is immediate surgical resection to preserve vision. Studies have shown that children younger than age 1 year are susceptible to the development of amblyopia if visual obstruction is present for a period of time as short as one week. Once the hemangioma has been carefully excised, primary closure can be attempted; larger wounds should be covered with a skin graft or flap because of the risk for lagophthalmos and corneal exposure.

Because systemic administration of corticosteroids has been shown to result in significant regression of lesions, it is the recommended course of therapy in patients with hemangiomas that do not obstruct the visual axis or airway. In these patients, doses of prednisone 2 mg/kg/daily to 4 mg/kg/daily are prescribed for two to three weeks and then tapered to 1 mg/kg/daily before the prednisone is discontinued. Although success rates of as high as 60% have been reported, associated complications in infants can include adrenal suppression, immunosuppression, growth arrest, and the development of cushingoid facies.

Intralesional corticosteroids such as betamethasone, dexamethasone, and triamcinolone can be extremely effective when injected at multiple sites under sedation or general anesthesia. As many as three separate injections can be administered every four to eight weeks, and marked involution has been reported in a majority of patients. Nevertheless, this course of treatment is rarely recommended because patients are at risk for significant complications, including perforation of the globe, infection, renal artery embolism, blindness, eyelid necrosis, and atrophy of soft tissues.

Interferon alfaÐ2a is a newly recognized treatment of hemangiomas. This agent is thought to inhibit the migration and proliferation of endothelial cells through a blockade of basic fibroblast growth factor. Complications, which are infrequent, can include low-grade fever, a transient increase in liver enzymes, and rebound tumor growth, which can be prevented by prolonging the course of therapy. However, investigational studies of the use of interferon alfa-2a for the treatment of hemangiomas have been limited to those patients in whom corticosteroid therapy has been unsuccessful.

Radiation therapy is effective for treatment of proliferative hemangiomas but is often associated with significant adverse effects, especially in infants. These include the potential for injury to the globe and optic nerve, as well as the risk for subsequent induction of malignancy, such as thyroid carcinoma. Therefore, this treatment is limited to patients who have high risk lesions that have not responded to corticosteroid therapy.

References
1. Goldberg NS, Rosanova MA. Periorbital hemangiomas. Dermatol Clin. 1992;10:653-661.
2. Greinwald JH Jr, Burke DK, Bonthius DJ, et al. An update on the treatment of hemangiomas in children with interferon alfa-2a. Arch Otolaryngol Head Neck Surg. 1999;125:21-27.
3. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412-410.
4. Ohlms LA, Jones DT, McGill TJ, et al. Interferon alfa-2a therapy for airway hemangiomas. Ann Otol Rhinol Laryngol. 1994;103:1-8.
5. Thompson H. Cutaneous hemangiomas and lymphangiomas. Clin Plast Surg. 1987;14:341-356.
6. White CW, Wolf SJ, Korones DN, et al. Treatment of childhood angiomatous diseases with recombinant interferon alfa-2a. J Pediatr. 1991;118:59-66.
7. Wisnicki JL. Hemangiomas and vascular malformations. Ann Plast Surg. 1984;12:41.

A 40-year-old man has a dark purple "cobblestone" lesion covering the entire right cheek. This finding is most consistent with

(A) an arteriovenous malformation
(B) a capillary malformation
(C) a hemangioma
(D) a lymphatic malformation
(E) a venous malformation

The correct response is Option B.

This 40-year-old man has a capillary malformation, or port-wine stain. These lesions are often seen in the distribution of the abducens (VI) and facial (VII) nerves. If left untreated, cobblestoning and progressive darkening may occur due to ectasia of the vessels within the capillary malformation.

An arteriovenous malformation is a high-flow lesion often characterized by a palpable thrill or bruit. Compression of the lesion results in occlusion of the associated arteriovenous fistula, causing a baroreceptor response and an increase in blood pressure. This leads to a decrease in heart rate and is known as the Branham sign.

Hemangiomas are vascular tumors that appear just after birth and rapidly enlarge during the first year of life. Because spontaneous regression is common, conservative management is suggested for non-life-threatening lesions that do not obstruct the airway or visual axis.

Lymphatic malformations, also known as cystic hygromas, occur most frequently in the head and neck region and often enlarge in response to an adjacent infection. Recurrent swelling can lead to scarring, which will significantly decrease the size of the lesion.

Venous malformations are low-flow lesions composed of dilated venous channels. These lesions are compressible and have a propensity to fill with blood when the patient changes body positions. When the malformation is associated with thrombosis, pain may result.

References
1. Jackson IT, Carreno R, Potparic Z, et al. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg. 1993;91:1216.
2. Kohout MP, Hansen M, Pribaz JJ, et al. Arteriovenous malformations of the head and neck: natural history and management. Plast Reconstr Surg. 1998;102:643.
3. Mueller BU, Mulliken JB. The infant with a vascular tumor. Semin Perinatol. 1999;23:332.

An 8-year-old boy has a bulky soft-tissue anomaly of the right upper extremity. Radiographs show bony destruction of the underlying humerus. These findings are most consistent with

(A) arteriovenous malformation
(B) hemangioma
(C) hereditary hemorrhagic telangiectasia
(D) lymphatic malformation
(E) venous malformation
The correct response is Option A.

This 8-year-old boy has findings consistent with an arteriovenous malformation, a congenital vascular soft-tissue anomaly that has extended to cause destruction of the underlying humerus. Arteriovenous malformations are a subtype of vascular malformation involving arterial channels; they are present at birth and thus grow commensurately with the child's growth. Associated physical findings can include increased warmth in the area of the malformation, mass effect, the presence of a thrill or bruit on auscultation, ulceration, and distal ischemia, which is related to the "steal" phenomenon (ie, diversion of normal blood flow). Skeletal changes, especially intraosseous extension and lytic changes, occur in 34% of affected patients.

Hemangiomas are also classified as vascular anomalies but instead appear shortly after birth and are characterized by a period of rapid growth (typically six to nine months) followed by slow involution. Rapid endothelial proliferation is characteristic, but skeletal changes are not, occurring in only 1% of patients. Lytic lesions are also not seen. Although hemangiomas are often unpleasant looking, they are typically only removed surgically if they begin to obstruct the airway or visual fields.

Hereditary hemorrhagic telangiectasia, or Osler-Weber-Rendu syndrome, is an autosomal dominant disorder that first manifests following puberty and is characterized by lesions with ectatic vessels involving the skin, mucous membranes, and viscera that worsen over time. Epistaxis, hematuria, and hematemesis are frequently associated. Neurologic symptoms may occur in patients who have involvement of the central nervous system. Skeletal malformations are not associated.

In patients with lymphatic malformations, skeletal hypertrophy and distortion are frequent. Venous malformations, especially those affecting the extremities, can cause hypoplasia and bone demineralization.

References
1. Boyd JB, Mulliken JB, Kaban LB, et al. Skeletal changes associated with vascular malformations. Plast Reconstr Surg. 1984;74:789-797.
2. Haimovici H, Sprayregen S. Congenital microarteriovenous shunts: angiographic and Doppler ultrasonographic identification. Arch Surg. 1986;121:1065-1070.
3. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412-422.
4. Williams HB. Vascular neoplasms. Clin Plast Surg. 1980;7:397-411.