A case of neonatal dural sinus malformation: Clinical symptoms, imaging and neuropathological investigations

• Published in: European journal of paediatric neurology Vol. 12; no. 1; pp. 41 - 45
• Main Authors: de Haan, T.R, Padberg, R.D, Hagebeuk, E.E.O, Aronica, E, van Rijn, R.R, Majoie, C.B.L.M, Kok, J.H
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01-01-2008
• Subjects: Central Nervous System Vascular Malformations - diagnostic imaging; Central Nervous System Vascular Malformations - pathology; Central Nervous System Vascular Malformations - physiopathology; Cranial Sinuses - pathology; Dural sinus malformation; Embryology; Humans; Imaging; Infant, Newborn; Magnetic Resonance Angiography; Magnetic Resonance Imaging; Neonatal; Neurology; Pathology; Pediatrics; Radiography; Therapy; Dural sinus malformation; Pathology; Therapy; Neonatal; Embryology; Imaging
• ISSN: 1090-3798; 1532-2130
• DOI: 10.1016/j.ejpn.2007.04.003

Abstract We report a case of neonatal dural sinus malformation already visible on antenatal ultrasound. This is a rare disease entity in infants and children. Clinical diagnosis was made by demonstrating a cranial murmur on auscultation; macrocrania and signs of progressive cardiac failure. Imaging studies as cerebral ultrasound, postnatal MRI scan and MR angiography demonstrated a large dural sinus malformation originating from the sagittal sinus with extensive arteriovenous fistulae. Due to the extent of the lesion, the existing ischemic brain damage and involvement of the torcular, no therapeutic options were available and the child died of irreversible cardiac failure. The diagnosis was confirmed with autopsy. We discuss the clinical presentation, imaging and neuropathological results and relate our findings to embryological data and the existing literature.