Introduction: Dural sinus malformations (DSMs) are rare pediatric vascular lesions. Two types are recognized, with midline DSMs reported to have a poor prognosis and have been traditionally more difficult to cure.
Methods: We describe the clinical presentation of three midline DSMs and discuss the management for each lesion. The first two differed from previously reported DSMs in that neither involved low flow AV shunts. Patient 1 presented with cardiac failure, intracranial hemorrhage, and seizures. She had a large midline DSM with multiple high flow dural and pial AV shunts. In the second patient, a large midline DSM with no AV connections was identified prenatally. Both patients were successfully treated with techniques including percutaneous transfontanelle and endovascular embolization, posterior fossa decompression, cranial expansion, and CSF diversion. The third patient was found prenatally to have a large dural sinus malformation involving the torcula with no evidence of ventriculomegaly or brainstem compression. On serial imaging, the DSM continues to decrease in size despite no intervention.
Results: Patient 1 was meeting developmental milestones at 24 month follow up with imaging showing marked decrease in the thrombosed midline venous pouch and no new AV shunts. At 12 month follow-up, Patient 2 was meeting most developmental milestones, with some motor delay. Serial MRI imaging revealed significant reduction in the size of the DSM and decreased ventriculomegaly. Patient 3 was born without complication and continues to do well with no intervention.
Conclusions: Early diagnosis and treatment, if necessary, of DSMs is 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. Options including observation, percutaneous/endovascular embolization and adjuvant neurosurgical decompression with CSF diversion is shown in our cases to treat these lesions with good outcomes.
Patient Care: Dural sinus malformations can be life threatening and their early identification and treatment allows for prevention of associated complications or sequelae.
Learning Objectives: By the conclusion of this session, participants should be able to 1) Describe importance of early identification of dural sinus malformations, 2) Discuss in small groups the classifications of DSMs and their presentations, 3)Identify potential treatment options for DSMs