Occult Tethered Cord Syndrome: A Review

Background: Tethered cord syndrome is a well-defined condition, the management of which is fairly uniform. In contrast, occult tethered cord syndrome is a recently defined entity, where the management is still controversial. The pathophysiology is unclear and may be conceptually incongruent with current understanding of typical tethered cord syndrome. Presentation, investigation, and management of this condition are reviewed, and current understanding is presented.

Purpose: The aim of this study is to review the presentation, pathophysiology, investigation, and management of occult tethered cord syndrome.

Methods: Literature review.

Results: Patients with occult tethered cord syndrome presents predominantly with urologic symptoms. Adult and pediatric patients vary slightly in their presentation with a higher incidence of pain in the former and incontinence in the latter. Operative management for these patients is associated with consistent improvement in urologic function in particular, although surgery is also associated with risk of worsening symptoms. The natural history of untreated patients is unknown.

Conclusions: As occult tethered cord syndrome becomes increasingly recognized, it is important to be aware of the potential benefits of operative intervention for appropriately selected patients. Given that the natural history of this entity remains unknown, a clinical trial is currently underway that may assist in defining the role for operative management in treating this condition.