Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations.
OBJECTIVE
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).


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.
Bisdorff
A
A
Vascular Anomalies Center, Department of Radiology and Division of Plastic Surgery, Children's Hospital, Harvard Medical School, Boston, Mass, USA. pburrows@chpnet.org
Mulliken
J B
JB
Carrico
J
J
Robertson
R L
RL
Burrows
P E
PE
D000293:Adolescent; D000328:Adult; D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000473:pathology*; D002533:Cerebral Angiography; D002550:Cerebral Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D005260:Female; D020786:Hemangioma, Cavernous, Central Nervous System / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000473:pathology*; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008202:Lymphangioma / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000473:pathology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed