Childs Nerv Syst (2006) 22:1631–1633 DOI 10.1007/s00381-006-0159-7 CASE REPORT Spontaneous isolated non-haemorrhagic thrombosis in a child with development venous anomaly: case report and review of the literature A. Vieira Santos & P. Saraiva Received: 20 October 2005 / Revised: 3 November 2005 / Published online: 28 October 2006 # Springer-Verlag 2006 Abstract Introduction The natural history of developmental venous anomalies (DVAs) is said to be relatively benign. Case report We herein report the clinical case of a 9-yearold female child with sudden right hemiparesis. An ischaemic infarct secondary to an isolated spontaneous thrombosis of a DVA was diagnosed by brain magnetic resonance imaging, further confirmed by digital subtraction angiography. Conclusion Considering the therapeutic options, early diagnosis is mandatory. Keywords DVAs . Ischemic venous infarct . Rare . MRI . Cerebral angiography . Plasminogen activator inhibitor-1 Introduction Developmental venous anomalies (DVAs), a term first proposed by Lasjaunias et al. [1], are considered to be an anatomical variation to the usual pattern of venous drainage, presumably due to maldevelopment of superficial or deep local veins. They consist of a group of small, dilated medullary veins converging into a bigger venous collector that drains either superficially or into the deep venous system. On angiography they are synchronous with the venous phase. Developmental venous anomalies constitute a relatively common finding, with a reported incidence of 2.5% at autopsies [2]. They do not seem to be associated for the A. Vieira Santos (*) : P. Saraiva Neuroradiology Department, Garcia de Orta Hospital, Almada, Portugal e-mail: gisantos@iol.pt most part with a specific clinical presentation [3]. Nevertheless, DVAs have been related with seizures, focal deficits and headaches [4]. Bleeding has been attributed to its common association with cavernous haemangiomas, the latter being responsible for the haemorrhagic presentation [5]. Ischemic events have seldom been reported [6]. Case report A 9-year-old female child was transferred to our hospital because of weakness involving her right upper and lower limbs, starting acutely 2 days before admission. On entrance, her neurological examination was positive for a right side hemiparesis. She had been diagnosed with diabetes mellitus type I when she was 4 years old. She had had two episodes of poorly characterized seizures during childhood, easily controlled with phenobarbital. Laboratory tests showed her to be homozygous for the 4G allele of the plasminogen activator inhibitor-1 (PAI-1). Brain magnetic resonance imaging (MRI) performed 1 day after admission disclosed a bright peri-central area on T2 and diffusion weighted images (DWI) surrounding a DVA draining into the superior sagittal sinus. Apparent diffusion coefficient (ADC) maps were normal. On sagittal T1WI and axial T2WI, the DVA collector was partially hyperintense (Figs. 1 and 2), suggesting thrombus in subacute phase. An area of cortical dysplasia was seen on the cortex overlying the DVA. A digital subtraction angiography (DSA) was performed showing the typical angiographic pattern of the caput medusae, appearing during the venous phase of the angiogram, with an irregular filling defect in the distal part of the DVA collector (Fig. 3). Stenosis at the dural aperture of the collecting vein was not seen. The arterial phase of the DSA was normal. 1632 Childs Nerv Syst (2006) 22:1631–1633 Fig. 1 Sagittal T1WI (initial MRI): spontaneous bright signal in the DVA collector suggesting thrombus in subacute phase The child was treated with low-molecular-weight heparin and dismissed from hospital 13 days later with a slight improvement of her right hemiparesis. On a follow-up brain MRI performed 14 months latter, an area of focal atrophy was seen surrounding the DVA (Fig. 4). Fig. 3 DSA: typical morphology of a DVA with filling defects on the distal part of the DVA collector The introduction of non-invasive central nervous system imaging dramatically changed the frequency with which DVAs were diagnosed [7]. DVAs constitute probably the most common anomaly of the intracranial vasculature [8] and are considered to be clinically silent for the most part [9]. According to Lasjaunias et al. [10], DVAs become symptomatic either due to the normal aging process of the venous system or by their association with cavernous malformations. Dillon et al. [11] proposed that stenosis at the dural opening could in part be responsible for symptomatic venous angiomas, giving rise to a haemorrhagic presenta- tion. Other authors further believe that under certain circumstances, cavernous malformation may be the consequence of this venous restrictive disease [12]. In our case, where no stenotic dural opening was noticed at angiography, thrombus formation in the draining vein might have been related to homozygosis for the 4G allele of the PAI-1, a prothrombotic that has been implicated in cerebral vein and sinus thrombosis [13, 14]. Early partial recanalization of the venous collector, as has been reported before [15], may have prevented parenchymal haemorrhage to develop, giving rise to ischaemic infarct. Normal ADC maps in these circumstances may merely reflect the coexistence of cytotoxic and vasogenic oedema [16], considering the time elapsed between the ictus and the initial brain MRI [17]. Fig. 2 Coronal T2WI (initial MRI): spontaneous bright signal in the DVA collector suggesting thrombus in subacute phase Fig. 4 Axial T2WI (follow-up MRI 14 months latter): focal atrophy surrounding the DVA Discussion Childs Nerv Syst (2006) 22:1631–1633 Conclusion Although it is likely that DVAs are for the most part asymptomatic, one should not dismiss the possibility of spontaneous isolated thrombosis, a potential source of ischaemic infarction. Moreover, the image demonstration of the intraluminal clot may have therapeutic implications, prompting the institution of anticoagulation in symptomatic DVAs. References 1. Lasjaunias P, Burrows P, Planet C (1986) Developmental venous anomalies (DVA): the so-called venous angioma. Neurosurg Rev 9(3):233–242 2. Sarwar M, McCormick WF (1978) Intracerebral venous angioma. Case report and review. Arch Neurol 35(5):323–325 3. Topper R, Jurgens E, Reul J, Thron A (1999) Clinical significance of intracranial developmental venous anomalies. 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