Split cord malformation in adults: Literature review and classification

Abstract

The objective of this study was to summarize the available literature describing the presentation, diagnostic evaluation, and management for adults with Type 1 and Type 2 split spinal cord malformations. A review of the literature was performed using the CINAHL, PubMed, Embase, and Web of Science database, alongside all associated bibliographies, to include studies describing Type 1 and Type 2 split cord malformations diagnosed in patients above the age of 18. All relevant studies of split cord malformations were included, regardless of the year published and terminology used to describe the dysraphism. Clinical case series (≥ 2 patients), cohort studies, and review articles comprising adult patients with radiographically diagnosed diastematomyelia, diplomyelia, or dimyelia were included (Class of Evidence I-IV). A total of 17 unique articles, describing 146 unique adult spinal cord malformation subjects, were included. The most common associated condition was tethered cord syndrome (59.8 %). Operative management for symptomatic split cord malformation was performed in 72.3 % of cases. For those with preoperative neurologic deficits, operative management resulted in symptomatic improvement in 96.6 %, compared to 0 % conservative management (p < 0.05). For those with pain alone, operative management resulted in improvement of 91.1 %, compared to 12.5 % conservative management (p < 0.05). To date, this is the only literature review to include all split cord malformations (SCM Types I and II) presenting in adulthood, with clinical characteristics, associated conditions, and long-term treatment outcomes.

Keywords: Adult SCM; Diastematomyelia; Dimyelia; Diplomyelia; Split cord malformation.