Intracranial Vascular Anomalies in Patients with Periorbital Lymphatic and Lymphaticovenous Malformations

• Published in: American Journal of Neuroradiology Vol. 28; no. 2; pp. 335 - 341
• Main Authors: Bisdorff, A, Mulliken, J.B, Carrico, J, Robertson, R.L, Burrows, P.E
• Format: Journal Article
• Language: English
• Published: Oak Brook, IL Am Soc Neuroradiology 01.02.2007; American Society of Neuroradiology
• Subjects: Adolescent; Adult; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Arteriovenous Malformations - diagnostic imaging; Arteriovenous Malformations - epidemiology; Arteriovenous Malformations - pathology; Biological and medical sciences; Bone marrow, stem cells transplantation. Graft versus host reaction; Cerebral Angiography; Cerebral Veins - abnormalities; Cerebral Veins - diagnostic imaging; Child; Child, Preschool; Female; Head & Neck; Hemangioma, Cavernous, Central Nervous System - diagnostic imaging; Hemangioma, Cavernous, Central Nervous System - epidemiology; Hemangioma, Cavernous, Central Nervous System - pathology; Human viral diseases; Humans; Incidence; Infant; Infant, Newborn; Infectious diseases; Investigative techniques, diagnostic techniques (general aspects); Lymphangioma - diagnostic imaging; Lymphangioma - epidemiology; Lymphangioma - pathology; Magnetic Resonance Imaging; Male; Medical sciences; Nervous system; Orbital Neoplasms - diagnostic imaging; Orbital Neoplasms - epidemiology; Orbital Neoplasms - pathology; Radiodiagnosis. Nmr imagery. Nmr spectrometry; Retrospective Studies; Tomography, X-Ray Computed; Transfusions. Complications. Transfusion reactions. Cell and gene therapy; Viral diseases; Viral diseases of the lymphoid tissue and the blood. Aids; Human; Intracranial; Nervous system diseases; Radiodiagnosis; Malformation
• ISSN: 0195-6108; 1936-959X; 1432-1920

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). 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). 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. 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.