Management of midline dural sinus malformations and review of the literature

Purpose: Dural sinus malformations (DSMs) are rare pediatric vascular lesions that have variable presentations and outcomes. We present three cases of midline DSMs and discuss the treatment strategy employed for each lesion. A review of the literature was completed to summarize current literature and treatment practices. Methods: A retrospective review of the electronic medical record and all available imaging studies was performed for each of our patients. Results: Patient 1 had a prenatally diagnosed DSM which decreased in size despite no intervention. She was born without complication and continues to do well at 15 months of age. Patient 2 presented 2 weeks after birth with cardiac failure, intracranial hemorrhage, and seizures and imaging showed a large midline DSM with multiple high-flow shunts. She required multiple endovascular embolizations with complete occlusion of the lesion. At her 3-year follow-up, she was neurologically normal. The third patient was diagnosed prenatally with an enlarging DSM. Multiple endovascular embolizations, surgical decompression, cranial expansion, and CSF diversion were required for treatment. At her 2.5-year follow-up, she was meeting developmental milestones, with some motor delay. Conclusion: Early diagnosis and treatment, if necessary, of DSMs are critical to prevent cardiac failure or parenchymal injury from chronic venous hypertension. Management should be decided on individual case basis depending on the angioarchitecture and progression of the lesion and can involve observation, endovascular embolization, surgical interventions, or a combination of treatments. A personalized approach to treating these variable lesions can be associated with good outcomes.