Spinal cord arteriovenous malformation: a pediatric presentation

Abstract

Spinal vascular malformations in child are rare and still under-diagnosed entities that typically lead to progressive spinal cord symptoms and myelopathy if not properly treated 1 . This malformation can be differentiated into congenital (arteriovenous malformations and cavernomas) or acquired (dural arteriovenous fistulae) forms 1 . Spinal cord arteriovenous malformations (SCAVM) comprise a complex and heterogeneous group of lesions with a number of subtypes that have distinct clinical presentations, anatomic features and natural histories 2,3 . While there are important differences between subtypes, the majority of patients with SCAVM present first clinical manifestations only in adolescence or adulthood 2 . We describe the characteristics on child with SCAVM and show the possible treatment of this disease on childhood because only few cases have been published. CASEA 4-year-old girl presented sudden onset of lumbar and lower limbs pain, weakness in right lower limb, difficulty walking, constipation and urinary retention. Her parents denied previous similar episodes. She was born at term and was the first child of non-consanguineous parents and the only affected case in the family. The psychomotor development was normal.Physical examination did not reveal any abnormalities. Neurological examination showed hypotonia in lower limbs (right more than left); muscle strength grade 3 (MRC scale) in the right lower limb and grade 5 in the bilateral upper and left lower limbs; absent right patellar reflex; bilateral Babinski sign; decreased pain, pinprick and light touch sensibility in the right lower limb; and abnormal joint position sense in the right lower limb.The symptoms and signs were thought to be consistent with spinal cord abnormality, and the investigation showed a cerebrospinal fluid with 5 leukocytes/mm 3 , 140 erythrocytes/mm 3 , glucose 55 mg/dl and protein 25.7 mg/dl; needle electromyography and sensory nerve conduction study were normal, but motor nerve conduction study demonstrated absent F-wave in right peroneal nerve; magnetic resonance imaging (MRI) revealed high signal on T1-weighted images and hypointense signal on T2-weighted images in area of the T11-T12 spinal cord with concomitant swelling of the T11-L1 cord (Fig 1); and selective spinal angiography demonstrated a small arteriovenous fistula with posterior aneurysmatic formation on T11-T12 spine cord area which was fed by the anterior spinal artery arising from the right 12 th and left 9 th intercostal arteries (Fig 2A and 2B).After this initial investigation the patient was submitted to management of the SCAVM. Surgery was performed with clipping of the supplies arteries resulting complete obliteration of the fistula on postoperative spinal angiography (Fig 2C). Followup shows that her symptoms and neurological status improved after surgery.All studies were done following informed consent of parents.

Cervical Spinal Arteriovenous Malformation with Intramedullar Hemathoma

“…Incidence of AVM range is between 20 to 30% of all spinal vascular shunts [1]. According to our knowledge and available literature, AVM in our region are rare.…”

“…After endovascular treatment patient has resumed rehabilitation. Currently she has a light weakness of right limb, plegic standard for analysing anatomical, morphological and architectural features necessary for therapeutic decisions in both pediatrics and adult population [1]. The experience of one Polish study suggested that endovascular treatment is effective and safe method for the treatment of AVM located in the cervical region of spinal cord [4].…”