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Gregory J. Griepentrog, MD; Beverly Aagaard-Kienitz, MD; Burton J. Kushner, MD; Bermans J. Iskandar, MD; David M. Gamm, MD, PhD

Arch Ophthalmol. 2006;124:1359-1361.

Patients with potentially life-threatening intracranial vascular abnormalities may first come to an ophthalmologist with signs or symptoms that include visual disturbances, bruits, papilledema, or periorbital congestion commonly coexisting with other neurologic and cardiovascular problems. In this report, we discuss a girl seen first at 6 months of age with the rare finding of isolated, unilateral eyelid ptosis and fullness due to a dural arteriovenous fistula.

Report of a Case
A female child was noted at birth to have right upper eyelid swelling and ptosis. Although they were believed to be related to birth trauma, there was little change in her eyelid appearance over the next few months, prompting referral to a pediatric ophthalmologist (B.J.K.) when the girl was 6 months old. Fullness of the right upper eyelid was evident (Figure 1), but no palpable mass or orbital bruit was appreciated. The remainder of her ophthalmic examination, including eyelid function, motility, and sweep visual evoked potential testing, had normal findings for both eyes. The child had otherwise enjoyed good health and achieved appropriate developmental milestones.

View larger version (75K): [in this window] [in a new window] [as a PowerPoint slide]   Figure 1. Photograph on patient's initial visit. Note the right upper eyelid ptosis and fullness.

Magnetic resonance imaging revealed a dural arteriovenous fistula with massive engorgement and dilation of the dural venous sinuses, including the distal superior sagittal sinus, torcular herophili, and right transverse sinus (Figure 2A). Cerebral angiography identified multiple arterial feeding vessels, the most prominent of which were derived from the occipital arteries bilaterally (Figure 2B). Also noted were bilateral dilated superior ophthalmic veins due to shunting of cerebral venous blood to the external periorbital veins. There was no evidence of intracranial mass effect, infarct, hemorrhage, or other parenchymal abnormalities.

View larger version (47K): [in this window] [in a new window] [as a PowerPoint slide]   Figure 2. Preprocedural and postprocedural neuroimaging studies. A, T1-weighted, fat-suppressed, contrast-enhanced axial magnetic resonance image demonstrating abnormal enhancement and enlargement of the distal superior sagittal sinus (arrow) and the torcular herophili (arrowhead). B, Preprocedural mixed internal and external arterial-phase cerebral angiogram showing multiple feeder vessels, including branches of the middle meningeal artery (arrowheads) and transosseous feeding vessels originating from the occipital artery (arrow). The superior ophthalmic vein is not visible during this phase of the angiogram. C, Postprocedural venous phase cerebral angiogram with multiple embolization coils deposited in the distal superior sagittal sinus and torcular herophili (arrow). Note the persistent dilated superior ophthalmic vein (arrowhead).

With the poor natural history of dural arteriovenous fistula in children, we decided to attempt endovascular embolization of the lesion to prevent long-standing cerebral venous hypertension and potentially irreversible brain injury. A preoperative cardiovascular evaluation, including echocardiogram, revealed only a slightly enlarged left atrium due to mildly increased cardiac output.

At 7 months of age, the patient underwent initial endovascular treatment using a combined transvenous and transarterial approach with selective embolization of feeding branches from the right occipital and middle meningeal arteries, branches of the vertebral arteries, the superior sagittal sinus, and the torcular herophili (Figure 2C). Postoperatively, the child did well, and her parents noted an increase in alertness and playful activity and increased interaction with her siblings. Her right upper eyelid ptosis and fullness were unchanged, however, and an occipital bruit (first noted during a follow-up visit) remained. When the patient was 19 months of age, we performed a second embolization procedure because of persistence of part of the malformation and complaints of intermittent headaches. At age 4 years, the child's neurodevelopment continued to be normal, although her visual acuity as measured by the HOTV method was 20/70 OD and 20/60 OS despite an otherwise normal ocular examination. The cause of her decreased visual acuity was not clear, although a component of cortical damage from chronic cerebral venous hypertension was suspected. She continues to be monitored closely by her multidisciplinary medical team.

Comment
Dural arteriovenous fistula in the pediatric population is rare, often multifocal, and generally more complex than adult dural arteriovenous fistula.^1-2 In adults, an insult from trauma, intracranial surgery, thrombophlebitis, or dural venous thrombosis is believed to initiate an inflammatory cascade with ensuing neovascularization and angiogenesis, resulting in shunts at the arteriolar level.³ The exact causative triggers in children are unknown, although both embryologic and intrauterine-acquired etiologies have been proposed.^1-2 Signs and symptoms in infants typically include cardiac failure, hydrocephalus, hemorrhage, and seizures, although some children may only have bruits, proptosis, or papilledema.^1-2 The increased risk of heart failure necessitates a comprehensive pediatric cardiology evaluation. In our patient, normal internal jugular venous drainage was compromised by shunted arterial flow, leading to asymmetric anomalous drainage of venous blood through the cavernous and periorbital venous system, resulting in unilateral upper eyelid swelling and mechanical ptosis.

The major goal of therapy is to disconnect the fistula from its arterial supply and ensure that alternative venous outlets remain patent and functional.² Infants with multifocal lesions tend to have more serious neurological sequelae than adults, including neurocognitive delay, hemorrhage, or death, although the advent of newer endovascular treatments has improved overall outcomes.^{1, 3} In complex lesions with numerous arterial feeding arteries, multiple therapeutic embolizations may be required to definitively cure these life-threatening fistulae. Our case illustrates that children with potentially life-threatening intracranial vascular abnormalities may first visit the ophthalmologist with seemingly benign symptoms, making prompt diagnosis and treatment essential.

AUTHOR INFORMATION
Correspondence: Dr Gamm, T607 Waisman Center, 1500 Highland Ave, Madison, WI 53705 (dgamm{at}wisc.edu).

Financial Disclosure: None reported.

REFERENCES
1. Kincaid PK, Duckwiler GR, Gobin YP, Vinuela F. Dural arteriovenous fistula in children: endovascular treatment and outcomes in seven cases. AJNR Am J Neuroradiol. 2001;22:1217-1225. FREE FULL TEXT 2. Lasjaunias P, Magufis G, Goulao A, et al. Anatomoclinical aspects of dural arteriovenous shunts in children. Intervent Neuroradiol. 1996;2:179-191.3. Ng PP, Higashida RT, Cullen S, Malek R, Halbach VV, Dowd CF. Endovascular strategies for carotid cavernous and intracerebral dural arteriovenous fistulas. Neurosurg Focus. 2003;15:ECP1. PUBMED

SECTION EDITOR: W. RICHARD GREEN, MD

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