In-Service Exam
Hemangiomas - 2002






PHOTO

A 72-year-old woman has had a long history of a compressible, nonpulsatile lesion on the lip and chin; photographs are shown above. The lesion swells with activity, sometimes produces pain, and is associated with episodes of bleeding and ulceration.

These findings are most consistent with which of the following types of malformation?

(A) Arteriovenous
(B) Capillary
(C) Lymphatic
(D) Venous

The correct response is Option D.

This 72-year-old woman has a classic venous malformation, a dark lesion that often becomes swollen with dependency and is exsanguinated easily. Venous malformations occur in 1% to 4% of patients and may vary from well-localized lesions to diffuse generalized ectasia. Intermittent pain can develop secondary to generalized thrombosis; analgesics are frequently prescribed for relief. Wedge resection is most appropriate for removal of this patient's small lesion on the chin. Other recommended procedures include sclerotherapy and surgical resection. Laser therapy can be used to remove small superficial lesions, and compression garments can be applied for lesions on the extremities. More invasive techniques are reserved for symptomatic malformations characterized by chronic pain, ulceration, and bleeding.

Arteriovenous malformations are generally pulsatile and not associated with pain. Surgery is often recommended because of the risk for hemorrhage. Capillary malformations (ie, port-wine stains), which involve the intradermal capillaries, are most frequently seen on the face and can be linked to other anomalies. Although lymphatic malformations appear as superficial lesions characterized by cutaneous vesicles, they are much deeper than their appearance, involving both the dermis and subcutaneous tissue.

References
1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412.
2. Upton J, Coombs CJ, Mulliken JB, et al. Vascular malformations of the upper limb: a review of 270 patients. J Hand Surg. 1999;24A:1019.
3. 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.

PHOTO

The photographs shown above are of a 40-year-old man who has congenital enlargement of the left side of his face, trunk, and leg. On physical examination, there is dermal hyperemia in the affected area; there is no bruit or thrill on the trunk. These findings are most consistent with which of the following conditions?

(A) Klippel-Trenaunay syndrome
(B) Parkes-Weber syndrome
(C) Rendu-Osler-Weber syndrome
(D) Sturge-Weber syndrome
(E) von Hippel-Lindau disease

The correct response is Option A.

This 40-year-old man has findings most consistent with Klippel-Trenaunay syndrome, characterized by a port-wine stain (typically involving one extremity) overlying venous and lymphatic malformations. Skeletal hypertrophy may also be seen; the lower extremity is most frequently involved. Patients with venous malformations may experience pain, ulceration, and ectasia; those with lymphatic malformations, such as this patient, typically report soft-tissue swelling and hypertrophy. This patient also has involvement of the trunk, which is rare.

Parkes-Weber syndrome is similar to Klippel-Trenaunay syndrome but is distinguished by the presence of arteriovenous fistulas.

Rendu-Osler-Weber syndrome, or hereditary hemorrhagic telangiectasia, is an autosomal dominant disorder characterized by multiple ectatic vessels involving the skin, mucous membranes, and visceral organs. Epistaxis, hematuria, hematemesis, and melena are frequently associated.

Sturge-Weber syndrome is associated with a large vascular malformation, usually a port-wine stain, in the distribution of the first and second branches of the trigeminal nerve (V1 and V2). Other findings include focal motor seizures, hemiparesis, and visual field defects, especially glaucoma. Mental retardation may result from leptomeningeal venous malformations.

Patients with von Hippel-Lindau disease have hemangiomas affecting the retina, as well as hemangioblastomas of the cerebellum and visceral organs. Seizures and mental retardation may also be associated.

References
1. Boyd JB, Mulliken JB, Kaban LB, et al. Skeletal changes associated with vascular malformations. Plast Reconstr Surg. 1984;74:789.
2. Mulliken JB. Cutaneous vascular anomalies. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;5:3191-3274.
3. Servelle M. Klippel and Trenaunay's syndrome: 768 operated cases. Ann Surg. 1985;201:365.
4. 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.

An otherwise healthy 3-year-old boy has enlargement and elongation of the right upper extremity. On examination, there is a port-wine stain on the forearm and tortuous vessels on the arm. A thrill can be palpated in the area of the vessels. These findings are most consistent with which of the following syndromes?

(A) Maffucci's syndrome
(B) Parkes-Weber syndrome
(C) Proteus syndrome
(D) Rendu-Osler-Weber syndrome
(E) Sturge-Weber syndrome

The correct response is Option B.

This 3-year-old boy has findings most consistent with Parkes-Weber syndrome, an anomaly characterized by skeletal hypertrophy of one upper extremity with associated port-wine stains. Deep soft-tissue involvement, including capillary, lymphatic, and venous malformations, is also seen. Klippel-Trenaunay syndrome is similar to Parkes-Weber syndrome but instead involves the lower extremities.

Patients with Maffucci's syndrome have multiple enchondromas, most frequently affecting the hand, and venous malformations. The limbs are foreshortened. Approximately 20% of patients with Maffucci's syndrome will develop chondrosarcoma.

Proteus syndrome manifests as partial gigantism of the extremities. Hemifacial hemihypertrophy, macrocephaly, macrodactyly, localized exostoses, subcutaneous lipomas, and vascular malformations are also associated.
Patients with Rendu-Osler-Weber syndrome have multiple bright red telangiectasias affecting the face, fingertips, and mucosal surfaces of the gastrointestinal tract, bladder, and bronchial lining. Brain involvement can lead to the onset of seizures.

Sturge-Weber syndrome is associated with a large vascular malformation, usually a port-wine stain, in the distribution of the first and second branches of the trigeminal nerve (V1 and V2). Other findings include vascular anomalies of the choroid plexus and leptomeninges, focal motor seizures, and visual field defects, especially glaucoma. Mental retardation may also be seen.

References
1. Mulliken JB. Cutaneous vascular anomalies. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;5:3191-3274.
2. Mulliken JB. Cutaneous vascular lesions in children. In: Serafin D, Georgiade NG, eds. Pediatric Plastic Surgery. Saint Louis, Mo: CV Mosby Co; 1984:137-154.

Which of the following modalities best delineates the extent of involvement of an arteriovenous malformation with its surrounding tissues?

(A) Angiography
(B) CT scan
(C) MRI
(D) PET scan
(E) Ultrasonography
The correct response is Option C.

Arteriovenous malformations are structural anomalies that develop during the fourth through sixth weeks of gestation secondary to inhibited development of the capillary network. These malformations are typically present at birth but may be seen initially in persons of all ages. Hormonal changes, such as those seen in puberty and adolescence, can result in progression of the lesion; its growth is thought to be caused by increased blood flow and expansion and not by cellular proliferation.

Although angiography has long been the standard for diagnosis of vascular lesions, defining their vascular anatomy and flow characteristics, this technique does not adequately delineate the involvement of the surrounding soft tissues. Thus, MRI and magnetic resonance angiography (MRA) are currently recommended as noninvasive means of determining both the extent of the lesion and its flow characteristics. Because contrast enhancement and ionizing radiation are unnecessary, both MRI and MRA can be obtained in the same setting. Angiography is still reserved for delineation of large, highly vascular lesions that require preoperative embolization.

CT scan is used for bony evaluation but is now secondary to MRI as the diagnostic procedure of choice. PET scan is not effective in evaluating vascular malformations. Doppler ultrasonography may be performed in conjunction with MRI to determine the lesion's flow characteristics but is not used alone.

References
1. Baum RA, Rutter CM, Sunshine JH, et al. Multicenter trial to evaluate vascular magnetic resonance angiography of the lower extremity. JAMA. 1995;274:875.
2. Disa JJ, Chung KC, Gellad FE, et al. Efficacy of magnetic resonance angiography in the evaluation of vascular malformations of the hand. Plast Reconstr Surg. 1997;99:136.
3. 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.

What percentage of hemangiomas are noted in the first month of life?

(A) 20%
(B) 40%
(C) 60%
(D) 80%
(E) 100%

The correct response is Option D.

Approximately 80% of hemangiomas are noted in the first month of life. Hemangiomas are the most common tumors of infancy; their incidence at birth ranges from 1% to 3%, and nearly one-third are noted before the neonate is discharged from the nursery. Boys are reportedly three times more likely than girls to be affected. Approximately 60% of hemangiomas occur in the head and neck region.

References
1. Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg. 1983;18:894.
2. Mulliken JB. Cutaneous vascular anomalies. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;5:3191-3274.
3. Osburn K, Schosser RH, Everett MA. Congenital pigmented and vascular lesions in newborn infants. J Am Acad Dermatol. 1987;16:788.
4. Watson WL, McCarthy WD. Blood vessel and lymph vessel tumors: a report of 1,056 cases. Surg Gynecol Obstet. 1940;71:569.

Which of the following best describes a hemangioma?

(A) Aplasia
(B) Dysplasia
(C) Fibroplasia
(D) Hyperplasia
(E) Metaplasia

The correct response is Option D.

Hemangiomas are vascular lesions that enlarge through a process of rapid cellular proliferation known as hyperplasia. These lesions have an increased rate of cellular turnover uncommon to all other vascular malformations. Instead, vascular malformations, which can be classified as arterial, capillary, lymphatic, or venous, have structural anomalies not present in hemangiomas.

Aplasia describes the lack of development of an organ or tissue; dysplasia refers to an abnormality in the developmental process. Fibroplasia is the formation of fibrous tissue. Metaplasia describes a change in the type of adult cells within a tissue to another form of cells not normal for that tissue.

References
1. Mulliken JB. Cutaneous vascular anomalies. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;5:3191-3274.
2. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412.

Which of the following syndromes is NOT associated with capillary malformations?

(A) Kasabach-Merritt syndrome
(B) Klippel-Trenaunay syndrome
(C) Parkes-Weber syndrome
(D) Sturge-Weber syndrome
The correct response is Option A.

Kasabach-Merritt syndrome is a disorder characterized by profound thrombocytopenia associated with a solitary hemangioma or diffuse hemangiomatosis. Capillary malformations, or port-wine stains, are not seen. Systemic heparin should not be administered to patients with Kasabach-Merritt syndrome because of the associated risk for exacerbation of bleeding diatheses. Instead, appropriate management includes embolization or compression of lesions, interferon therapy, radiation therapy, and systemic administration of corticosteroids.

All of the other syndromes are associated with port-wine stains. Klippel-Trenaunay syndrome is characterized by a port-wine stain, typically involving one extremity, overlying venous and lymphatic malformations. Skeletal hypertrophy may also be seen. Although patients with Parkes-Weber syndrome have findings similar to Klippel-Trenaunay syndrome, arteriovenous fistulas are a distinguishing feature. Port-wine stains are most frequently seen in conjunction with Sturge-Weber syndrome. The malformation usually occurs in the distribution of the first and second branches of the trigeminal nerve (V1 and V2), and is associated with focal motor seizures, hemiparesis, and visual field defects, especially glaucoma. Mental retardation may result from leptomeningeal venous malformations.

References
1. Enjolras O, Riche MC, Merland JJ. Facial port-wine stains and Sturge-Weber syndrome. Pediatrics. 1985;76:48.
2. Sakar M, Mulliken JB, Kozakewich HP, et al. Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstr Surg. 1997;100:1377.
3. Servelle M. Klippel and Trenaunay's syndrome: 768 operated cases. Ann Surg. 1985;201:365.
4. 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.