The growth and expansion of meningeal lymphatic networks are affected in craniosynostosis

Skull malformations are associated with vascular anomalies that can impair fluid balance in the central nervous system. We previously reported that humans with craniosynostosis and mutations in TWIST1 have dural venous sinus malformations. It is still unknown whether meningeal lymphatic networks, which are patterned alongside the venous sinuses, are also affected. We now show that the growth and expansion of meningeal lymphatics are perturbed in Twist1 craniosynostosis models. Changes to the local meningeal environment, including hypoplastic dura and venous malformations, affect the ability of lymphatic networks to sprout and remodel. Dorsal networks along the transverse sinus are hypoplastic with reduced branching. By contrast, basal networks closer to the skull base are more variably affected, showing exuberant growth in some animals suggesting they are compensating for vessel loss in dorsal networks. Injecting a molecular tracer into cerebrospinal fluid reveals significantly less drainage to the deep cervical lymph nodes, indicative of impaired lymphatic function. Collectively, our results show that meningeal lymphatic networks are affected in craniosynostosis, suggesting the clearance of beta-amyloid and waste from the central nervous system may be impeded.

Dural Venous Sinus Thrombosis: A Case Report

Dural Venous Sinus Thrombosis is the formation of blood clot within the cerebral sinus. It is very rare case with varying clinical presentation. It has non-specific signs and symptoms ranging from headache, papilledema, seizures, focal neurological deficits and mental state changes which is caused by genetic and acquired prothrombotic states, infections, inflammatory disease and trauma. Magnetic Resonance Imaging with Magnetic Resonance Venography is the specific imaging technique for the diagnosis. We have described a case of a patient who presented with headache over the temporal and occipital region and was disoriented. The Computed Tomography, Magnetic Resonance Imaging, Magnetic Resonance Venography report revealed presence of thrombus in the transverse and sigmoid sinus with hemorrhagic infarcts. He was then treated with anticoagulants Low Molecular Weight Heparin which was further substituted by Warfarin.

Diagnostic Accuracy of 3D Contrast-Enhanced T1 High Resolution Isotropic Volume Excitation MRI for Non-Cavernous Intracranial Dural Arteriovenous Fistula

Objective: To determine the accuracy of 3D contrast-enhanced THRIVE MRI in diagnosis of non-cavernous intracranial dural AVF compared with DSA. Materials and Methods: Thirty-three patients including fourteen dural AVF cases and nineteen control subjects, were included in the present study. They underwent 3D contrast-enhanced THRIVE MRI by 3T, contrast-enhanced MRA, and DSA. Two independent readers reviewed 3D contrast-enhanced THRIVE images for the presence of transosseous arterial feeders, low signal intensity curvilinear structures in the dural venous sinus, shaggy dural sinus, and cortical venous dilatation. Diagnostic performance values were calculated for 3D contrast-enhanced THRIVE MRI. Results: The 3D contrast-enhanced THRIVE MRI identified 23 from 29 locations of dural AVFs. The overall accuracy, sensitivity, specificity, positive predictive value, and negative predictive value were 91.4%, 79.3%, 93.5%, 67.6% and 96.3%, respectively. Shaggy dural sinus and dilated cortical veins were found in 78.6% and 85.7%, respectively. Conclusion: The 3D contrast-enhanced THRIVE MRI is a valuable tool for diagnosis of dural AVF as well as exclusion of dural AVF due to high sensitivity and specificity. Keywords: Dural arteriovenous fistula; Dural AVF; 3D contrast-enhanced THRIVE MRI

Dural venous sinus thrombosis: A rare complication of pulse corticosteroid therapy in pemphigus vulgaris

Systemic pulse corticosteroid therapy is used widely in the treatment of pemphigus vulgaris. Dural venous sinus thrombosis as a complication of pulse therapy has not been reported in the literature. A middle-aged female with pemphigus vulgaris was started on monthly pulse dexamethasone therapy with daily azathioprine as an adjuvant. After two pulse therapies, she developed throbbing headache, which on further evaluation was determined to be due to dural venous thrombosis. Other causes of dural venous thrombosis were excluded. Pulse therapy was stopped and other medications were started. The headache subsided within two weeks of stopping pulse therapy. Corticosteroids may play the role of a procoagulant in producing cerebral venous sinus thrombosis. Herein, we report a rare case of dural venous sinus thrombosis due to pulse steroid therapy in pemphigus vulgaris.

Traumatic injury of dural venous sinus as cause of epidural hematoma: importance of recognition and adequate surgical planning

Background: Although most traumatic epidural hematomas are secondary to arterial injuries, particularly rupture of the middle meningeal artery, around 9.7% of cases are associated with dural venous sinus injury, leading to poorer prognosis and greater complications. We report a case of a patient presenting cranial trauma with bone fracture and laceration of the transverse sinus producing epidural hematoma, and discuss the main aspects of this condition. Case description: A 47-year-old man struck by a motorcycle was admitted to the Emergency Room with a score of 15 on the Glasgow Coma Scale, evolving over 5 hours with reduced level of consciousness. A cranial CT scan was performed disclosing right parieto- occipital hyperdensity, consistent with acute epidural hematoma, and a parieto-occipital fracture line crossing the path of the ipsilateral transverse sinus. Parietal craniotomy was performed to drain the hematoma, revealing a venous sinus injury requiring immediate repair. Conclusions: This case and discussion highlight the many details and technical specificities to be taken into account by neurosurgeons when encountering intracranial hematoma caused by traumatic injury of the dural venous sinus, and which are pivotal in determining the efficacy of the treatment approach and prognosis of the patient.

Neonatal giant dural sinus ectasia: a multimodality imaging approach

Dural venous sinus ectasia is a rare anomaly characterised by the formation of a large vascular lake within the leaves of the dural sinuses, usually associated with thrombosis. These lesions can cause brain compression, cardiac insufficiency and disseminated intravascular coagulation, which may lead to poor prognosis. We present the case of a neonate who presented with an intracranial mass on prenatal ultrasound. Postnatal transcranial ultrasonography, cranial CT and cranial MRI demonstrated a large lesion predominantly occupying the dural sinus confluence, extending into the sagittal sinus, straight sinus and right transverse sinus. The left marginal sinus remains unfused and patent. Concomitant arteriovenous malformations were evident in the median interhemispheric fissure and the left Sylvian fissure. There are several published case reports and case series describing malformations of the dural sinuses in perinatal and neonatal patients in recent years, but this case is unique in that: (1) there is the presence of a vascular malformation concomitant to the dural sinus ectasia and (2) it highlights the importance of imaging in clinching the diagnosis of giant dural venous sinus ectasia, as it is often misdiagnosed as more common conditions such as extra-axial intracranial haemorrhage.