Dural Sinus Malformations (DSM) with Giant Lakes, in Neonates and Infants

Abstract

<jats:sec><jats:title>Background and Purpose</jats:title><jats:p> Dural Arteriovenous Shunt (DAVS) in children include Dural sinus malformation (DSM), infantile and adult types. They are rare and seldom reported. Our purpose was to highlight the angiographic features of the DSM sub group for prognosis of clinical evolution and outcome and to lay guidelines for management. </jats:p></jats:sec><jats:sec><jats:title>Methods:</jats:title><jats:p> From a dedicated neurovascular data bank, there were 52 cases of arteriovenous dural shunts in children from 1985 to 2003. Of these, there were 30 patients with DSM, which we analysed the various angioarchitecture, presentation and neurological outcome. Children clinical status was evaluated and scored at admission and follow up. </jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p> There was an overall male dominance of 2:1. Antenatal diagnosis was obtained in 8/30 (26.7%) cases. Mean age of diagnosis was 5 months. Mean age at first consultation was 8.7 months. No patient was diagnosed during childhood. The most common clinical presentations were macrocrania 76.7%, seizures 23.3% and mental retardation 23.3%. In 14/30 (35.7%) of the patients, the therapeutic decision was to manage conservatively; in 5/14 (30.7%) with predictable favourable evolution and in 9/14 (64.3%) with irreversible poor neurological outcome. In the remaining 16/30 (53.3%) patients, endovascular treatment was performed. In 12/16 (75.0%) patients the neurological outcome was good, 3/16 (18.8%) patients had unfavourable evolution despite embolization. There was no morbidity mortality related to the procedures themselves. 1/16 (6.3%) patient was lost to follow-up. Overall 12/29 (45.8%) patients had an unfavourable neurological outcome with 11 patients dead and 1 with severe neurological deficit. In the surviving group of children, 17/18 (94.4%) have a good neurological outcome; in 10/18 (55.5%) the lesion is morphologically excluded. </jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p> DSM is rare disease with high mortality. They usually proceed to either total or partial spontaneous thrombosis before the age of 2 thus compromising normal cerebral venous drainage. DSM away from the torcular, good cavernous sinus, cavernous capture of sylvian veins, absence of pial veins, straight sinus or superior sagital sinus (SSS) reflux and absence of jugular bulb dys-maturation represent factors of good prognosis. Such patients will highly benefit for endovascular treatment. In partial endovascular approach the aim being is to separate the brain drainage from DSM drainage. This will be achieved by the transarterial approach to the associated mural arteriovenous shunts (AVS) and by disconnecting the pial reflux by transvenous route. </jats:p></jats:sec>

Antenatal thrombosis of torcular herophili presenting with anemia, consumption coagulopathy and high-output cardiac failure in a preterm infant

“…3 Our patient had an acute decline in hematocrit along with an extension of the thrombus as documented on the CT scan. At the same time, her coagulation profile was indicative of consumption coagulopathy.…”

“…Prognosis varies from normal neurodevelopment outcomes 1 to mental retardation and death. 3 To the best of our knowledge, our patient is the first reported case of torcular thrombosis in a preterm infant who survived severe anemia, consumption coagulopathy and high-output cardiac failure during the neonatal period and had a normal neurological outcome.…”

“…The arteriovenous shunts in this malformation are generally of low velocity and rarely cause systemic hemodynamic complications. 3 We speculate that the arteriovenous shunt do become clinically significant in the setting of a premature subject with other complications such as anemia.…”

“…The clinical progression and prognosis is variable per descriptions. [3][4][5] We report the first case of torcular herophili thrombosis in a preterm infant causing anemia, consumption coagulopathy and high-output cardiac failure. Despite the acuity, aggressive clinical management led to the survival of the infant with a normal neuro-developmental outcome.…”

The role of percutaneous embolization techniques in the management of dural sinus malformations with atypical angioarchitecture in neonates: report of 2 cases

“…3,5,6 When they are midline in location, they have been reported to have a worse prognosis. 1 Midline DSMs with giant venous pouches are thought to result from uncontrolled dilation of the posterior sinuses, including the torcula and posterior SSS, with associated multiple low-flow AV shunts. 9 In a young infant with a midline DSM, there is venous hypertension within the sinuses, which is exacerbated when there is also associated immaturity of the jugular veins.…”