Topical Review: Pediatric Syringomyelia

Abstract

Syringomyelia was first recognized as a disease process some 400 years ago. The process of cystic dilation of the spinal cord is unpredictable and may result in a delay of many decades before the symptoms and signs of neurologic and orthopedic changes become apparent and commensurate with the observed cystic changes within the spinal cord. The syringomyelic process is usually associated with trauma, tumor, or congenital abnormalities at the craniocervical junction or along the spinal neuraxis. Several theories have been proposed as to processes involved in the development of spinal cyst formation, although none are completely compatible with the observed clinical pathology of syrinx development. Magnetic resonance imaging has markedly improved our ability to study the anatomy and natural history of syrinx formation, but to date, our understanding of the process remains imprecise. In view of the limited understanding of the pathophysiology of this disease process, it is not surprising that a variety of treatment regimens have been proposed and their efficacy remains difficult to fully evaluate.

Syringomyelia and chronic respiratory failure in a term infant delivered by Caesarean section

“…The infant had a history of difficult head extraction and all the non-specific symptoms of spinal cord injury: severe perinatal asphyxia, generalized hypotonia, absent tendon reflexes, and paradoxical breathing. 1,[4][5][6][7][8][9] Syringomyelia, without associated abnormalities or trauma, is usually asymptomatic in early life. In adolescence or early adulthood, symptoms such as sensory disturbances, muscular weakness, and spastic paraparesis can be present.…”

“…Discussion Syringomyelia is a rare disease in neonates, usually associated with other conditions. 1 In this case, investigations such as head CT, brain MRI, cranial ultrasound, and lumbar puncture did not reveal any malformations (myelomeningocele, Chiari II malformation, or fourth ventricle communication), intramedullar tumours, or infection. Syringomyelia can also be idiopathic or secondary to a spinal cord injury.…”

“…In adults, fewer than 10% of cases are associated with spinal cord injury. Data on neonates [1][2][3] are not available.…”

“…1 There is usually a history of difficult breech delivery or application of forceps. However, it can develop in cephalic vaginal delivery if an excessive rotational force is applied to the head.…”

Atypical presentation of Chiari I malformation with monoplegia and acquired torticollis

“…Syringomyelia is the development of a fluid-filled cavity or syrinx within the spinal cord. Syringomyelia is recognized as disease process for over 400 years now [2] and is frequently associated with the external compressions at the foramen magnum, particularly a CMI [3]. Other common causes for development of syrinx include trauma, tumor, or congenital abnormalities at the craniocervical junction or along the spinal neuraxis [2].…”