History of Management of Syringomyelia in Children

Understanding of Disease

• 1827 – first report: The term syringomyelia was first coined by d’Angers in 1827, although a spinal cord cavitation was first described in 1546 by Esteine in a treatise entitled “La Dissection Du Corps Humain” (57, 58, 64, 65).
• 1804 – first description of clinical presentation: Portal first described the clinical signs and symptoms of syringomyelia in 1804; and until the introduction of imaging, in particular MRI, the diagnosis of syringomyelia was clinically suspected and later confirmed by autopsy (57, 66).

Technological Development

• Improved imaging: Similar to advances applicable to treatment of Chiari I malformation, the main technological developments applicable to syringomyelia are dynamic MRI and flow study techniques. These new technologies have improved diagnostic accuracy and understanding of the pathophysiology, while they have practically eliminated the need for more invasive studies, such as myelograms.

Surgical Technique

• 1700 – Brunner describes first surgery: The first treatment attempts were described by Brunner in 1700 when he punctured an intramedullary cyst in a newborn with lumbosacral dysraphism; unfortunately, the infant developed hydrocephalus and died (68, 69).

• 1892 – Abbe and Coley describe using hemilaminectomy: A similar procedure performed through a hemilaminectomy was devised by Abbe and Coley in 1892 (67). There was no reported clinical benefit. Since then, several systems of drainage of intramedullary cysts have been used, but with further understanding of the pathophysiology of syringomyelia these “syringostomies” have been mostly considered a second choice after the primary etiology causing the syrinx has been addressed.

• Modern era – introduction of stenting/shunting: Advances in microsurgical technique and shunt materials have allowed for some satisfactory results in cases of syrinx shunting/stenting in the modern era (57, 58).