Chief Complaint: 3 year old male with refusal to bear weight on his leg Amanda Conti, MD Melody Brewer, MD Brendan Kelly, MD Baystate Medical Center, Springfield, MA

Case Presentation 3 year old Caucasian male with autism Presented to the Emergency Department with a reluctance to bear weight on his right leg While at school earlier on the day, he suddenly jumped up, started crying, and would not bear weight on his right leg This was not associated with changes in mental status, seizure like activity, vomiting, or headache The patient had no known trauma

Case Presentation On initial evaluation, patient seemed to be in pain and preferred to keep his eyes closed, stating that he was dizzy Physical exam and x-rays of his right leg were unrevealing It was thought that perhaps the patient had iliopsoas irritation accounting for the reason that he would not stand, therefore an abdominal x-ray and ultrasound were completed, and both of these were normal Given the unusual story and the fact that the patient refused to walk, he was admitted to the hospital for further work-up and management

Hospital Course Patient’s pain and dizziness improved over twenty-four hours and he slowly started to bear weight on his leg He was asked to walk down the hallway and was noted to veer to the right and walk into the wall Repeat neurological exams revealed persistent ataxia

Hospital Course Patient had an MRI … Images on next 4 slides

Hospital Course MRI that demonstrated acute and subacute infarcts in the left PCA territory Chronic infarcts were also present within the right inferior cerebellum in the distribution of the right PICA MRA of the neck revealed dissection of the right vertebral artery at the level of C2 Pt was started on anticoagulation

Learning Objectives Review incidence of ischemic stroke in children Review key aspects of vertebral artery dissection Review the differences and similarities of cerebral artery dissection in adult and pediatric patients Discuss the management of craniocervical dissection

Case Discussion The risk of ischemic stroke in childhood is 7.8 per 100,000. Craniocervical artery dissection [CAD] is the cause of ischemic stroke in 7.5% of childhood cases. The risk is higher for males and African American children.

Case Discussion Vertebral artery dissection usually occurs in the upper part of the vertebral artery at the C1–C2 level of the vertebrae. This most likely occurs because of the rotation of the head at the atlanto-axial joint. Dissection may be precipitated by fibromuscular dysplasia, connective tissue disorder, trauma, or may be classified as spontaneous. Spontaneous dissection of the cervical arteries is a rare cause of stroke in the general population, but in the young, it is a major cause, accounting for about 10%– 25% of ischemic events.

Case Discussion Both adults and children with CAD most commonly have intracranial dissections and these are more common in males. CAD differs in adults and children as the most common symptom(s) for adults is unilateral occipital headache and/or posterior neck pain. In children, pain is not a principal symptom, with only half of children complaining of head or neck pain. Adults commonly have vertigo, diplopia, or nausea and vomiting, whereas children commonly experience hemiparesis.

Case Discussion Treatment in children with CAD is similar to that in adults. Both adults and children should be treated with LMWH or coumadin for at least 6 weeks, with ongoing treatment dependent on radiologic assessment. Head injury is common in children and we recommended restricting activity and wearing a helmet for our patient while being treated with Coumadin.

Case Discussion It should be noted that our patient had a full thrombophilic and cardiac work-up that was negative for abnormalities. His bilateral infarcts were thought to be secondary to his unilateral vertebral dissection.

Question #1 Spontaneous dissection in the young accounts for about ………of ischemic events A) 1-3% B) 5% C) 10-25% D) 30% E) 40-50%

Answer - C Spontaneous dissection in the young accounts for about ………of ischemic events A) 1-3% B) 5% C) 10-25% D) 30% E) 40-50%

Question #2 Craniocervical artery dissection in children often presents with: A)Amaurosis Fugax B)Vertigo C)Diplopia D)Nausea and Vomiting E)Hemiparesis

Answer - E Craniocervical artery dissection in children often presents with: A)Amaurosis Fugax B)Vertigo C)Diplopia D)Nausea and Vomiting E)Hemiparesis

References Roach, ES. Et al. Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke Sep;39(9): Epub 2008 Jul 17. Rayfay, MF. et al. Craniocervical arterial dissection in children: clinical and radiographic presentation and outcome. J Child Neurol Jan;21(1):8-16. Fullerton, H. Arterial Dissection and Stroke in Children. Neurology : Kochan, J. and Kanamalla, Uday. Carotid and Vertebral Artery Dissection. Emedicine. 6/17/ Halevy, A. et al. Vertebral artery dissection and posterior stroke in a child. J Child Neurol May;23(5): Epub 2008 Feb 15. Feudale F. Liebelt E. Recognizing vertebral artery dissection in children: a case report. Pediatr Emerg Care Jun;16(3): Bacigaluppi S. et al. Vertebral artery dissection in a child. Is "spontaneous" still an appropriate definition? 1: Neurol Sci Nov;27(5): Monagle P. et al. Antithrombotic therapy in neonates and children: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest 2008 Jun;133(6 Suppl):887S-968S.).