Arteriovenous Hemangioma Malformation

INTRODUCTION The nomenclature of vascular lesions of the skin remains very unclear and there are no clear-cut guidelines for clinicians. Despite attempts at better classifications hemangiomas and malformations are still often confused. Most authorities classify vascular lesions as hemangiomas (hamartomas) or malformations (developmental anomalies). This is further refined based on endothelial characteristics and flow type. When arteriovenous lesions occur in the skin they are often referred to as hemangiomas, but are synonymous with what are referred to as arteriovenous malformations (AVM) elsewhere. These fast-flow lesions can occur either as congenital defects or less commonly they can develop following trauma, surgery or even as a sequel to inflammation. They are uncommon on the face and particularly rare on the eyelids. Unlike capillary hemangiomas, AVM's do not involute, and often become progressively worse.

CLINICAL PRESENTATION The lesion appears as a pulsating single vessel or mass, or a tangle of blood vessels, red to purple in coloration. It is compressible and spongy, but refills quickly after compression. There is often a palpable thrill and sometimes an auditory bruit in this pulsatile lesion. Bleeding often occurs either into the surrounding tissue or onto the surface. The Valsalva maneuver or bending forward often increases the size of the lesion and gives the subjective perception of throbbing. Orbital involvement may result in proptosis and can affect vision. Diagnosis is often aided with Doppler ultrasonography, CT scan and carotid arteriography, or digital subtraction angiography.

Arteriovenous Malformation Eyelids

Arteriovenous Hemangioma/Malformation (Contd.)

Arteriovenous Hemangioma

HISTOPATHOLOGY This form of hemangioma is composed of numerous mostly thick walled vessels resembling both arteries and veins. The muscular walls have variable elastic lamina, and arteriovenous anastomoses and/or thrombi may be present. Eyelid arteriovenous hemangiomas are usually well-circumscribed intradermal masses.

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes lymphangioma, hemangioma, venous lake, plexiform neurofibroma, varicose veins, and carotid-cavernous fistula.

TREATMENT Treatment is not indicated during infancy or childhood unless visual symptoms or amblyopia threatens. When treatment is required surgical resection with prior occlusion of feeder vessels is best, but is usually very difficult and can be mutilating. Identification and ligation of feeder vessels will give only temporary relief since rapid recruitment of flow from nearby arteries will reestablish blood supply in most cases. Embolization can be considered, but it carries a risk of undesirable intracranial or retinal vessel obstruction when employed in periorbital lesions. Sclerotherapy can be useful if the feeder vessels are first ligated. While laser therapy is useful for slow flow venous malformations they are less useful for the AVM.

REFERENCES

Buckmiller LM. Update on hemangiomas and vascular malformations. Curr Opin Otolaryngol Head Neck Surg 2004; 12:476-487.

Fishman SJ, Mulliken JB. Hemangiomas and vascular malformations of infancy and childhood. Pediatr Clin North Am 1993; 40:1177-1200.

Forman AR, Lussenhop AJ, Limaye SR. Ocular findings in patients with AVM of the head and neck. Am J Ophthalmol 1975; 79:626-633.

Holt JE, Holt GR, Thornton WR. Traumatic arteriovenous malformation of the eyelid. Ophthalmic Surg 1980; 11:771-777.

Hundeiler M. What is a hemangioma, what is a malformation? On the differential diagnosis of vascular tumors. Kongressbd

Dtsch Ges Chir Kongr 2001; 118:439-442. Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg 2005; 32:99-116.

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. Rootman J. Vascular malformations of the orbit: hemodynamic concepts. Orbit 2003; 22:103-120.

Arteriovenous Hemangioma/Malformation (Contd.)

Rusin LJ, Harrell ER. Arteriovenous fistula: Cutaneous manifestations. Arch Dermatol 1976; 112:1135-1138. Spring MA, Bentz ML. Cutaneous vascular lesions. Clin Plast Surg 2005; 32:171-186.

Very M, Nagy M, Carr M, Collins S, Brodsky L. Hemangiomas and vascular malformations: Analysis of diagnostic accuracy.

Laryngoscope 2002; 112:612-615. Zweep HP, Rieu PN, van Die CE, Boll AP, Steijlen PM, Spauwen PH. Haemangiomas and congenital vascular malformations: their classification and diagnosis. Ned Tijdschr Geneeskd 2002; 146:1072-1077.

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