Treatment of Periorbital and Palpebral Arteriovenous Malformations

Abstract

Objectives: To clarify clinically challenging palpebral arteriovenous malformations (AVMs) and to propose a novel therapeutic modality, we developed a multi-disciplinary approach for the management of AVMs with ulcer. Approach: First, the central retinal artery was secured with embolization by the transophthalmic arterial, a terminal branch of the internal carotid artery (ICA), and then, the branches of the external carotid artery (ECA) were embolized to cause a response in the AVM vasculature followed by sclerotherapy and surgery. Results: Over a 3-year follow-up of palpebral and periorbital AVMs in four females and one male 20 to 50 years of age with a mean age of 38 years, complete remission of the lesions were seen with no major complication, such as blindness, ptosis, or cerebral infarction, with functionally sound and esthetically acceptable results, with no recurrence or worsening even with one case of ulceration postembolization. Innovation: Planned treatment of palpebral and periorbital AVMs, which have been often left untreated because of their complex vasculature and a risk of total blindness due to occlusion of the central retinal artery. A "wait-and-watch" approach is frequently taken. It is important to secure the periphery to the bifurcation of the central retinal artery of the ICA, and then, embolization through the ECA results in complete remission of the lesion, followed by sclerotherapy and surgery, which are successful both in terms of function and esthetics. Conclusion: First, securing the central retinal artery leads to safer and complete resolution of palpebral and periorbital AVMs; wounding or therapeutic complications such as skin necrosis may be seen, but this approach results in complete remission in 3 years with no major complications.

Keywords: Schobinger staging; angiographic diagram; central retinal artery; external carotid artery; following sclerotherapy and surgery; transophthalmic arterial embolization.

Sadanori Akita, MD, PhD

Figure 1.

A diagram of the four types of AVMs based on nidus morphology. Type I (arteriovenous fistulae) AVMs: no more than three separate arteries shunt to the initial part of a single venous component. Type II (arteriolovenous fistulae): multiple arterioles shunt to the initial part of a single venous component, in which the arterial components show a plexiform appearance on angiography. Type IIIa (arteriolovenulous fistulae with nondilated fistula): fine multiple shunts are present between arterioles and venules and appear as a blush or fine striation on angiography. Type IIIb (arteriolovenulous fistulae with dilated fistula): multiple shunts are present between arterioles and venules and appear as a complex vascular network on angiography. In types IIIa and IIIb, multiple venulous components of the fistula unit collect to a draining vein. A, arterial compartment of the fistula unit; AVM, arteriovenous malformations; S, shunt; V, venous compartment of the fistula unit.

Figure 2.

A 20-year-old female with Schobinger stage II and angiographic IIIa in the left upper eyelid with mass and upper gaze visual field impairment. (A) Frontal view. Bulging in the lateral left upper eyelid with pulsation, thrill, and bruit. (B) Lateral view. Left eyelid bulging is prominent. (C) Angiographic and coil embolization. Both ICA and ECA demonstrate vasculature, and after securing the periphery to the bifurcation of the ophthalmic artery with coils, as demonstrated by a red arrow, ECA branches were embolized with superabsorbent polymer microsphere beads. There is no vascular tree in the upper eyelids postembolization. (D) Frontal view. At 48 h postembolization, the mass was nearly completely removed and was not observed in 3.2 years. (E) Lateral view. The mass subsided in 3.2 years. ECA, external carotid artery; ICA, internal carotid artery. Color images are available online.

Figure 3.

A 40-year-old female with Schobinger stage III and angiographic IIIb in the left upper eyelid with mass and upper gaze visual field impairment. (A) Frontal view. Bulging in the left upper eyelid with pulsation, thrill, and bruit, and skin discoloration. (B) Lateral view. Left eyelid bulging is prominent. (C) Angiographic and coil embolization. Both ophthalmic artery and ECA demonstrate vasculature and after securing the periphery to the bifurcation of the ophthalmic artery with coils, ECA branches were embolized with 25% NBCA, as demonstrated by a blue arrow. There is no vascular tree in the upper eyelids postembolization or most of the branches of ECA near the eyelid subsided. (D) Frontal view. At 48 h postembolization, the lateral upper eyelid and temporal skin were necrotized. (E) Surgical removal of the necrotic tissue; the levator muscle and conjunctiva were preserved. (F) Frontal view. After debridement of necrotic tissue, preauricular skin grafting was performed and was stabilized in 3 years. (G) Lateral view. After debridement and preauricular skin grafting, in 3 years, the bulging mass and symptoms disappeared. NCBA, N-butyl cyanoacrylate. Color images are available online.