Retinal arteriovenous malformation (AVM) is a rare unilateral developmental disease that is stable over time in most cases. The authors present a case of a man who was diagnosed with an asymptomatic juxtapapillary AVM of the left eye at the age of 25 years. Visual acuity was 20/20. Fundus pictures realized during an 11-year follow-up disclosed a slowly progressive enlargement of the lesion with vascular loops increasing in number and size. Vision remained stable. No complication was observed. This case demonstrates that retinal AVMs may progressively grow and remain asymptomatic and uncomplicated for a long period of time.
[Ophthalmic Surg Lasers Imaging Retina. 2018;49:e284–e286.]
Congenital retinal arteriovenous malformation (AVM) is a rare unilateral developmental anomaly that consists of one or more arteriovenous anastomoses.1
Visual impairment is inconstant and depends on the severity of the vascular anomaly.
In most cases, AVM remains stationary for considerable periods of time.2 It may be complicated by arterial or venous occlusion with retinal hemorrhage and sheathing of the wall of the vessels.3 Rarely, spontaneous involution has been described. Only one case of progression of an AVM has been published in a 6-year-old boy.4 Here, we report the case of an adult with a documented asymptomatic enlargement of a retinal AVM during an 11-year follow-up.
A 25-year-old man was diagnosed with a juxtapapillary retinal AVM of the left eye during a routine examination. Visual acuity (VA) was 20/20 in each eye. Fundus examination also disclosed mild epiretinal membrane of the left eye. (Figure 1) Examination of the right eye was unremarkable. The patient had no skin lesions or history of any neurologic disorder. Magnetic resonance imaging of the brain and the orbit was normal. He was monitored during an 11-year period. VA remained stable.
Fundus picture of the left eye of a 25-year-old man with a juxtapapillary retinal arteriovenous malformation. A mild epiretinal membrane with retinal folds can be noted.
Fluorescein angiogram confirmed the diagnosis of juxtapapillary retinal AVM and revealed a clinically overlooked arterial loop superonasal to the disc. (Figure 2) The fellow eye was unremarkable.
The arteriovenous malformation is well delineated on fluorescein angiography of the posterior retina performed at the age of 36. On arterial phase of the angiogram, the malformation is completely filled with dye, taking the appearance of a “bag of worms.” An arterial loop is also visible (arrow) (a). No fluorescein leakage is observed on late frame (b).
Fundus pictures realized at each visit disclosed a progressive growth of the AVM with vascular loops increasing in size and number (Figure 3).
Progressive growth of a juxtapapillary retinal arteriovenous malformation of the left eye of a 25-year-old man followed for 11 years. Initial fundus picture (a), the patient was then examined at the age of 27 (b), 29 (c), 30 (d), 33 (e), 34 (f) and 36 (g) years.
Retinal AVM is a rare congenital finding due to a direct connection of major vessels without interposition of capillaries.1 A wide spectrum of retinal AVM has been described ranging from a small arteriovenous communication to a complex anastomotic system involving the entire retina. Retinal AVM can be associated with cerebral AVM. Generally, the risk of developing a cerebral AVM is related to the extent and the complexity of the retinal AVM. Our patient had a moderate AVM (stage 2 according to Archer's classification) and MRI of the brain was unremarkable.
AVM can infrequently be complicated by thrombosis and vessel occlusion.1,2 However, most cases reported were stable during follow-up. So far, the only published case of progressive growth of an AVM occurred in a 6-year-old child.4 At this age, it may be hypothesized that retinal vessels involved in the malformation did not reach total maturation. In the case we report, the progressive enlargement of the components of AVM in an adult is in favor of a hemodynamic origin. The growth of the malformation could be due to the permanent high blood flow within the lesion due to the direct communication between the arterial and venous component in the absence of capillary interposition.
Patients with retinal AVM must be monitored as ocular complications such as tractional retinal detachment or neovascular glaucoma may occur.2
Clinicians should know that during follow-up, they might notice a progressive growth of the lesion. The enlargement of the AVM can remain asymptomatic and uncomplicated for a long period of time. Hence, they should not worry the patient needlessly when they observe a slight evolution of the lesion.
This case demonstrates that retinal AVM may progressively grow without complication during adulthood.
- Archer DB, Deutman A, Ernest T, Krill AE. Arteriovenous communication of the retina. Am J Ophthalmol. 1973;75:224–241. doi:10.1016/0002-9394(73)91018-0 [CrossRef]
- Schmidt D, Pache M, Schumacher M. The Congenital Unilateral Retinocephalic Vascular Malformation Syndrome (Bonnet-Dechaume-Blanc Syndrome or Wyburn-Mason Syndrome): Review of the Literature. Surv Ophthalmol. 2008;53:227–249. doi:10.1016/j.survophthal.2007.10.001 [CrossRef]
- Augsburger JJ, Goldberg RE, Shields JA, Mulberger RD, Magargal LE. Changing appearance of retinal arteriovenous malformation. Graefes Arch Clin Exp Ophthalmol. 1980;215:65–70. doi:10.1007/BF00413398 [CrossRef]
- Ehrt O. Slowly progressive retinal arteriovenous malformation and relative amblyopia. Arch Ophthalmol. 2004;122:408–409. doi:10.1001/archopht.122.3.408 [CrossRef]