E1822. An Approach to Congenital Neurovascular Disorders
  1. Shehbaz Ansari; Seth GS Medical College and KEM Hospital
  2. Rashmi Saraf; Seth GS Medical College and KEM Hospital
  3. Krushnadas Radadiya; Seth GS Medical College and KEM Hospital
  4. Nida Narvel; JIIU's Indian Institute of Medical Science and Research
  5. Sumeet Dhulshette; Seth GS Medical College and KEM Hospital
Multiple congenital neurovascular syndromes are known. However, in order to understand, diagnose and treat them, knowing the embryology of the craniofacial vessels becomes important. The cephalic neural crest cells forms tunica media and pericytes of blood vessels in corresponding regions of the face and the brain. Hence, a defect in them will result in vascular anomalies in metameric distribution. The process of angiogenesis and vasculogenesis in fetal life are driven by multiple molecular pathways. These molecular pathways function irrespective of, and across metameres. Hence, a defect in them would result in nonsegmental multisystemic vascular anomalies. Lastly, certain syndromes are known to cause vascular lesions restricted to the central nervous system. These are independent from the other categories and display individualistic features by which they are diagnosed.

Educational Goals / Teaching Points
1. To understand the embryological development of cranial vessels so as to better comprehend the pathophysiology of common vascular malformations. 2. To put forward a simplified approach for differentiating amongst the various congenital neurovascular disorders. 3. To show sample cases of these disorders to familiarize the audience with their different manifestations.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
Imaging plays a key role in the diagnosis and management of neurovascular syndromes. The choice of modality depends upon the type of disorder and the presentation of the patient. Digital Subtraction Angiography helps in delineating the angioarchitecture of lesions, apart from being therapeutic, while Magnetic Resonance Imaging displays their relationship with and their effect on the neuroparenchyma. Computed Tomography can be useful in emergency setting when the lesion bleeds acutely. Knowing the type and location of the vascular anomalies helps one to narrow down the differential. Arterial supply and the drainage pattern helps in therapeutic planning. Not all lesions require treatment. Some can be observed, especially the intracranial slow flow malformations. Patients with multiple lesions have to undergo numerous interventions. Life threatening lesions get precedence over the one causing mainly cosmetic defects. Prognosis is variable and depends upon the type of disorder and the number of lesions.

Although the diseases that will be discussed in the presentation are rare, it will help us understand the embryology of neural vasculature and pathophysiology of the more common, isolated vascular anomalies. Better understanding of pathophysiology may open up new therapeutic targets. Also, a flow chart approach (slide 2 of the presentation) will help in reaching a diagnosis from a myriad of complex congenital disorders such as this.