Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations

Abstract

Background and purpose: The purpose of this study was to determine the nature, incidence, and radiologic appearance of intracranial vascular anomalies that occur in association with periorbital lymphatic malformation (LM) and lymphaticovenous malformation (LVM).

Materials and methods: We retrospectively reviewed clinical records and imaging studies of 33 patients ranging in age from the neonatal period to 39 years (mean age, 5.1 years; median age, 1.0 year) who were evaluated for orbital LM or LVM at our institution between 1953 and 2002. Imaging studies, including CT, MR imaging, and cerebral angiograms, were evaluated by 2 radiologists to determine morphologic features of orbital LM and to identify associated noncontiguous intracranial vascular and parenchymal anomalies, including arteriovenous malformations (AVM), cerebral cavernous malformations (CCM), developmental venous anomalies (DVA), dural arteriovenous malformations (DAVM), and sinus pericranii (SP).

Results: The malformation was left-sided in 70% of patients. Twenty-two patients (70%) had intracranial vascular anomalies: DVA (n = 20; 61%), CCM (n = 2; 6%), DAVM (n = 4; 12%), pial AVM (n = 1; 3%), and SP (n = 1; 3%). Arterial shunts were present in the soft tissues in 2 patients (6%). Three patients had jugular venous anomalies. Three patients (9%) had cerebral hemiatrophy, 2 (6%) had focal cerebral atrophy, and 2 had Chiari I malformation.

Conclusions: Intracranial vascular anomalies, some of which are potentially symptomatic and require treatment, are present in more than two thirds of patients with periorbital LM. Initial imaging of patients with orbital LM should include the brain as well as the orbit.

Fig 1.

Postcontrast axial CT scan in a patient with a left orbitofrontal lymphatic malformation demonstrates a posterior fossa DVA draining into a transpontine collector (arrow).

Fig 2.

A and B, Postgadolinium axial T1-weighted MR images of a patient with a left preseptal orbitofrontal lymphatic malformation shows ipsilateral posterior fossa DVAs.

Fig 3.

A and B, Posterior fossa DVA in another patient is confirmed angiographically. Frontal (A) and lateral (B) venous phase of left vertebral angiogram shows the left cerebellar and left occipital DVAs draining into the left transverse sinus.

Fig 4.

A and B, Cavernoma-like lesion in a patient with a left orbital lymphatic malformation. A, Coronal T2-weighted MR imaging shows a focal T2-hyperintense area in the left basal ganglia, associated with ventricular dilation. B, Axial image showing the orbital and basal ganglia lesions.

Fig 5.

A and B, Right orbital/periorbital lymphatic malformation with right posterior dural AVM. A, Axial T2-weighted image shows the orbital and periorbital low-flow vascular malformation as dilation of the right transverse sinus which has flow voids in its wall. Associated right hemispheric atrophy is indicated by dilation of the temporal horn of the right lateral ventricle. B, Gradient recalled-echo sequence confirms the high-flow nature of this dural sinus lesion.

Fig 6.

Serial imaging of a girl with an extensive left orbitofrontal lymphatic malformation associated with a left posterior dural AV fistula, dural sinus enlargement, left cerebral hemiatrophy, sinus pericranii, and progressive formation of cavernoma-like lesions. The patient presented at birth in high output cardiac failure and underwent ligation of numerous extracranial arteries with clinical improvement. She subsequently underwent numerous surgical procedures to treat the orbital LM, which was complicated by recurrent hemorrhage. At age 17, she presented with severe orbital chemosmosis and underwent angiography. It is possible that some of the anterior vascular abnormalities are secondary to the craniotomies performed to debulk the left orbit. A, Axial CT image after intravenous contrast administration in the first week of life shows the large straight and left transverse sinuses and left hemiatrophy. Note the absence of a focal mass lesion in the left middle cranial fossa. B, Axial T2-weighted MR imaging of the brain at 10 years of age shows persistent enlargement of the left transverse sinus, and a cavenomalike lesion in the left middle fossa. C, Axial T1-weighted postgadolinium MR image at 17 years of age, after numerous orbital debulking procedures. There are enhancing channels within the orbital LM and adjacent sphenoid bone. These may represent small arterial lymphatic communications or a pure venous component. D, Coronal T2-weighted image from the same study as (C) shows LM involvement of the left infratemporal space, left orbit, left sphenoid and frontal bones, and the scalp. E, Coronal T2-weighted and MR image at the level of the frontal horns demonstrates enlargement of the cavenomalike lesion in the Sylvian fissure. F, Postgadolinium T1-weighed image shows attenuated enhancement of the extra-axial mass. A periventricular DVA is also present. G, Left middle meningeal angiogram, lateral projection, (via cervical collateral) shows supply of the dural AVM by the parietal branch of the middle meningeal artery. H, Left internal carotid angiogram, lateral projection, shows additional supply to the posterior dural AVM by the tentorial branches of the internal carotid artery. Note a second, more anterior lesion (arrow). I, Venous phase of the left internal carotid angiogram shows opacification of anomalous veins probably constituting part of the vascular mass in the Sylvian fissure.

Fig 7.

Selective ophthalmic arteriogram in a patient with recurrent bleeding into a postseptal orbital lymphatic malformation demonstrates tiny arterial communications with the soft issue malformation (arrows).