Giant dural venous sinus ectasia in neonates

Abstract

<jats:p>In this report, the authors describe 4 recent cases of posterior giant dural venous sinus ectasia in neonates diagnosed during pregnancy and encountered at 3 different institutions. Posterior giant venous sinus ectasia was diagnosed in 4 patients using antenatal ultrasonography and confirmed in 2 patients using prenatal MR imaging and in 3 patients using postnatal MR angiography. In 2 children angiography was performed at the age of 6 months. The pregnancy was terminated in 1 case, and the fetus underwent an autopsy. The 3 children who were born presented with various degree of cardiac insufficiency and were admitted to the intensive care unit after birth. Signs of increased intracranial pressure were present immediately after birth, including a bulging fontanel. No endovascular treatment was used in these cases. Surgery was performed in 2 cases as an attempt to alleviate increased intracranial pressure symptoms, without any real benefit. A slow venous flow in the ectasia was shown by ultrasonography in the case in which the pregnancy was terminated. Angiography or MR angiography did not show an obvious arteriovenous malformation in any of the cases, but an arteriovenous fistula secondary or contributing to the formation of the venous ectasia is one of the physiopathological hypotheses of the cause of this condition. Interestingly, spontaneous progressive thrombosis and regression of the intravascular component of the venous sinus ectasia was observed in all cases. The clinical outcome was acceptable in 1 child (who had a moderate handicap after the surgery) and good for the other 2 children (who had normal neurological development). Stratified thrombi of different ages are found in these giant venous ectasias and develop within the leaves of the dura close to the confluence of the major posterior venous sinuses. Therefore, it appears that the formation of a progressive thrombosis represents the normal evolution of these giant dural venous sinus ectasias, which explains the favorable outcome in some cases without specific surgical treatment, except for resuscitation techniques.</jats:p>

Thrombosed Dural Sinus Malformation in a Fetus: A Case Report

“…We believe that the increased cranial circumference in the present patient may be a result of thrombosis, since other reports have described a persistent cranial enlargement even after the regression of thrombosis . Five patients who exhibited macrocephaly have been previously reported ,. Two patients with AVF died due to cardiac decompensation and respiratory failure, and 2 other cases with a bulging fontanelle experienced mild hemiparesis .…”

“…Of the 31 reported patients, 6 underwent surgical intervention including embolization for AVF, surgical derivation for major hydrocephalus, craniotomy for evacuation of the hematoma, and decompression . Patients treated by embolization or surgical derivation had a favorable prognosis, while those who underwent craniotomy or decompression had an unfavorable prognosis . Infants with hydrocephalus or circulatory insufficiency due to AVF died in the absence of surgical treatment ,…”

“…Clinical data on these patients are summarized in Table 1. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] The clinical course of live birth cases with thrombosed DSM is variable, ranging from death and neurological complications to normal neural development. However, many patients have a favorable outcome, as indicated in Table 1.…”

“…15 Five patients who exhibited macrocephaly have been previously reported. 1,7,8,15 Two patients with AVF died due to cardiac decompensation and respiratory failure, and 2 other cases with a bulging fontanelle experienced mild hemiparesis. 7 These findings, together with those of the patient case who had favorable prognosis, suggest that macrocephaly alone may not be a negative prognostic factor.…”

Fetal MR imaging of posterior intracranial dural sinus thrombosis: a report of three cases with variable outcomes

“…The consensus in the literature as to the cause of this type of fetal dural sinus thrombosis at the torcular Herophili is a type of congenital dural sinus malformation (DSM) with low-flow arteriovenous shunts [3][4][5][6]16]. DSMs are classified into midline with giant a b The midline dural sinus malformation with giant pouches is typified by abnormal development and growth of the posterior sinuses (transverse sinuses, torcular Herophili and posterior SSS) associated with multiple slow-flow arteriovenous shunting within the wall of the malformed sinus [4].…”