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Spontaneous arteriovenous malformation of the orbit
  1. RICHARD J HESSE,
  2. VINOD K JINDAL,
  3. NILS MUNGAN
  1. Department of Ophthalmology, Ochsner Clinic and Alton Ochsner Medical Foundation, New Orleans, LA 70121, USA
  2. Department of Pathology
  3. Department of Ophthalmology
  1. JOHN C SCHARFENBERG
  1. Department of Ophthalmology, Ochsner Clinic and Alton Ochsner Medical Foundation, New Orleans, LA 70121, USA
  2. Department of Pathology
  3. Department of Ophthalmology
  1. LUIS BERNHARD
  1. Department of Ophthalmology, Ochsner Clinic and Alton Ochsner Medical Foundation, New Orleans, LA 70121, USA
  2. Department of Pathology
  3. Department of Ophthalmology
  1. Dr Richard J Hesse, Department of Ophthalmology, Ochsner Clinic, 1514 Jefferson Highway, New Orleans, LA 70121, USA rhesse.csspo.css{at}ochsner.org

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Editor,—Arteriovenous malformations (AVMs) and fistulas are rare in the orbit and generally have been associated with previous trauma. Of the 627 orbital tumours reported by Wright,1 only three were arteriovenous malformations. We report the clinical and pathological findings in a patient with an apparent spontaneous malformation.

CASE REPORT

A 74 year old white woman presented with a 2 month history of painless, progressive swelling of the right upper eyelid with no antecedent history of inflammation, trauma, or surgery. Ocular examination revealed visual acuity 20/30 in both eyes and an elastic, partially compressible, movable, pulsating tumour of 2.5 × 1 cm in size, located superiorly in the right upper eyelid, with a thrill over the lesion but no bruit and no accentuation on Valsalva. The remainder of the ophthalmic examination was unremarkable. Computed tomography (Fig 1A) showed a localised, homogeneous retroseptal mass. Because of the extremely anterior location of the lesion, further investigation with MRI/MRA or with selective angiography was not undertaken. During surgery (Fig 1B), a pulsating lesion was found with several tributaries. Each tributary was ligated and excised individually. The pulsations after excision of the temporal aspect of the tumour suggested a nasal origin, apparently arising from the medial palpebral branches of the ophthalmic artery. Pathology (Fig 2) showed a typical AVM.

Figure 1

(A) Computed tomographic scan shows a soft tissue homogeneous mass in the right upper eyelid, 2.5 × 1 cm in size, retroseptal, anteriorly placed, separate from lacrimal gland and eyeball, and without any extension in the orbit or cranium. (B) Surgical exposure of arteriovenous malformation via anterior approach shows large sausage-shaped, thick walled, pulsating lesion with several tributaries.

Figure 2

(A) Congeneries of arteries and veins in arteriovenous malformation (elastic tissue stain, original magnification ×25). (B) Arteriovenous malformation (haematoxylin and eosin stain, original magnification ×50).

COMMENT

AVM is the least common of the various orbital and periorbital vascular tumours.1 AVM consists of a communication between artery and veins with no interposed capillary bed. Pathologically, this communication is lined with endothelium and has characteristics of both arteries and veins.2 3 Many of these will have a palpable thrill even if a bruit is not present.2 3 Currently, a complete surgical excision of the AVM remains the most successful therapy.2-4 In this case, the surgical excision of the tumour through an anterior approach produced excellent cosmetic and functional results. Deeper orbital AVMs can haemorrhage severely without careful control of the feeder vessels.5 To our knowledge, this is the first reported case of a spontaneous orbital AVM presenting as a pulsatile tumour of the eyelid.

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