Historical Perspective of Split Cord Malformations: A Tale of Two Cords

Abstract

Introduction: Our appreciation and understanding of what is now known as the split cord malformation (SCM) have a long history. The oldest known example of SCM is from roughly AD 100. Other isolated examples can be found in the large body of work of the pathologists of the 1800s, where the SCMs were found incidentally during autopsies. Conclusions: SCM has a rich history and has intrigued physicians for over 200 years. Many well-known figures from the past such as Chiari and von Recklinghausen, both pathologists, made early postmortem descriptions of SCM. With the advent of MRI, these pathological embryological derailments can now often be detected and appreciated early and during life. Our understanding and ability to treat these congenital malformations as well as the terminology used to describe them have changed over the last several decades.

Chinese expert consensus on diagnosis and management of split cord malformation

“…Postulated by Pang et al [1], SCMs can be categorized into two types. Type I SCM accounts for 40% of all SCMs and is a condition in which two hemicords, each with individual central canal and pia mater, inhabit their own dural sacs and are transfixed by a dural-sheathed rigid, bony median septum.…”

“…Split cord malformation (SCM) is a neural tube defect that the spinal cord is longitudinally separated into two hemicords with individual functions, which causes severe spinal cord impairment and sensorimotor deficit. French pathologist Ollivier d' Angers initiated "diastematomyelia" from the Greek "diastema" and "myelos", meaning "cleft" and "spinal cord" to denote the malformation in 1837 [1,2]. Some authors named the malformation as "split notochord syndrome" [3] for its ontogenetic mechanism.…”