Isolated Internal Cerebral Venous Thrombosis in a Neonate with Increased Lipoprotein (a) Level: Diagnostic and Therapeutic Considerations

Abstract

Background

Internal cerebral venous thrombosis is a life-threatening condition, which requires immediate therapy. Being infrequent in childhood, internal venous thrombosis is very rare in the neonate and has never been observed without concomitant occlusion of further dural sinuses.

Case Description

We report a neonate born at term, who developed seizures on the third day of life after normal pregnancy and uneventful delivery. Ultrasound and CT disclosed bilateral intraventricular and intracerebral hemorrhage with an unusual distribution for germinal matrix hemorrhage. MRI disclosed thrombotic occlusion of the straight sinus and the internal cerebral veins with concomitant bleeding into the ventricles, the basal ganglia, thalamus and the periventricular hemispheres. The other sinuses were not affected. The clinical condition of the child improved after initiation of a low-dose heparin treatment with subsequent partial recanalization of the occluded vessels. The screening for risk factors disclosed an elevated lipoprotein (a) level, also present in both parents.

Conclusion

Internal cerebral venous thrombosis may be encountered in neonates and must be included in the list of differential diagnosis of perinatal intraventricular and intracerebral bleeding. MRI allows the diagnosis even in the absence of widespread dural sinus occlusion. Low dose heparin may be a therapeutic option in these cases. This is the first report of neonatal internal venous thrombosis due to hereditary lipoprotein (a) level elevation, which must be included in the list of possible predisposing conditions.

Key words

MRI - neonatal - CVT - lipoprotein (a) - treatment

References

• 1 Andrew M. Developmental hemostasis: relevance to hemostatic problems during childhood.  Semin Thromb Hemost. 1995;  21 341-356
• 2 Bousser M G. Cerebral venous thrombosis: diagnosis and management.  J Neurol. 2000;  247 252-258
• 3 Dean L M, Taylor G A. The intracranial venous system in infants: normal and abnormal findings on duplex and color Doppler sonography.  AJR Am J Roentgenol. 1995;  164 151-156
• 4 Einhäupl K M, Villringer A, Meister W. et al . Heparin treatment in sinus venous thrombosis.  Lancet. 1991;  338 597-600
• 5 Go C, Bernstein J M, de Jong A L, Sulek M, Friedman E M. Intracranial complications of acute mastoiditis.  Int J Pediatr Otorhinolaryngol. 2000;  52 143-148
• 6 Gomori J M, Grossman R I, Goldberg H I, Zimmerman R A, Bilaniuk L T. Intracranial hematomas: imaging by high-field MR.  Radiology. 1985;  157 87-93
• 7 Govaert P, Swarte R, Oostra A, Zecic A, Vanzieleghem B, Van Langenhove P. Neonatal infarction within basal cerebral vein territory.  Dev Med Child Neurol. 2001;  43 559-562
• 8 Grow J L, Fliman P J, Pipe S W. Neonatal sinovenous thrombosis associated with homozygous thermolabile methylenetetrahydrofolate reductase in both mother and infant.  J Perinatol. 2002;  22 175-178
• 9 Keller E, Flacke S, Urbach H, Schild H H. Diffusion- and perfusion-weighted magnetic resonance imaging in deep cerebral venous thrombosis.  Stroke. 1999;  30 1144-1146
• 10 Kuker W, Schmidt F, Friese S, Block F, Weller M. Unilateral thalamic edema in internal cerebral venous thrombosis: is it mostly left?.  Cerebrovasc Dis. 2001;  12 341-345
• 11 Lasjaunias P. Vascular Diseases in Neonates, Infants and Children. Heidelberg; Springer Verlag 1997
• 12 Mitchell L G, Sutor A H, Andrew M. Hemostasis in childhood acute lymphoblastic leukemia: coagulopathy induced by disease and treatment.  Semin Thromb Hemost. 1995;  21 390-401
• 13 Nowak-Göttl U, Junker R, Hartmeier M. et al . Increased lipoprotein(a) is an important risk factor for venous thromboembolism in childhood.  Circulation. 1999;  100 743-748
• 14 Nowak-Göttl U, Junker R, Kreuz W. et al . Risk of recurrent venous thrombosis in children with combined prothrombotic risk factors.  Blood. 2001;  97 858-862
• 15 Nowak-Göttl U, Strater R, Heinecke A. et al . Lipoprotein (a) and genetic polymorphisms of clotting factor V, prothrombin, and methylenetetrahydrofolate reductase are risk factors of spontaneous ischemic stroke in childhood.  Blood. 1999;  94 3678-3682
• 16 Ramenghi L A, Gill B J, Tanner S F, Martinez D, Arthur R, Levene M I. Cerebral venous thrombosis, intraventricular haemorrhage and white matter lesions in a preterm newborn with factor V (Leiden) mutation.  Neuropediatrics. 2002;  33 97-99
• 17 Rivkin M J, Anderson M L, Kaye E M. Neonatal idiopathic cerebral venous thrombosis: an unrecognized cause of transient seizures or lethargy.  Ann Neurol. 1992;  32 51-56
• 18 Schmidt B, Andrew M. Neonatal thrombosis: report of a prospective Canadian and international registry.  Pediatrics. 1995;  96 939-943
• 19 Shevell M I, Silver K, O'Gorman A M, Watters G V, Montes J L. Neonatal dural sinus thrombosis.  Pediatr Neurol. 1989;  5 161-165
• 20 Spandow O, Gothefors L, Fagerlund M, Kristensen B, Holm S. Lateral sinus thrombosis after untreated otitis media. A clinical problem - again?.  Eur Arch Otorhinolaryngol. 2000;  257 1-5
• 21 Stiefel D, Eich G, Sacher P. Posttraumatic dural sinus thrombosis in children.  Eur J Pediatr Surg. 2000;  10 41-44
• 22 Uziel Y, Laxer R M, Blaser S, Andrew M, Schneider R, Silverman E D. Cerebral vein thrombosis in childhood systemic lupus erythematosus.  J Pediatr. 1995;  126 722-727
• 23 Valeriano J, Bhagavatula K, Ku A, Snyder P J. Isolated straight sinus thrombosis: clinical and neuroradiologic correlates.  J Neuroimaging. 1998;  8 106-108