Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome

Eur J Paediatr Neurol. 2012 Mar;16(2):103-17. doi: 10.1016/j.ejpn.2011.07.002. Epub 2011 Aug 11.

Abstract

Objective: Tight filum syndrome (TFS) is caused by a thick (abnormal T1 MRI), shortened (low-lying conus), or non-elastic filum (strictly normal MRI). We carefully analyzed children treated for suspect TFS with or without radiological abnormalities.

Methods: Twenty-five children, operated between 2002 and 2009, were retrospectively identified. All children had been evaluated by a multidisciplinary team preoperatively. Symptoms, signs and diagnostic test results were categorized (neurologic, urologic, orthopedic, dermatologic) and compared pre- and one year postoperatively. Normal MR was defined as conus medullaris (CM) at or above mid-body L2 and filum diameter less than 2 mm. Occult TFS (OTFS) was defined as TFS with normal MR.

Demographics: 17 girls, 8 boys, age 2-18 years, including 11 syndromal children.

Clinical presentation: all children had problems in the neurologic category and at least one other category: urologic (n = 17), orthopedic (n = 21), and dermatologic (n = 11). MR findings: low-lying CM (n = 14) including 2 with thick filum, normal CM but fatty filum (n = 2), strictly normal (n = 9). Clinical outcome one year postoperatively: neurologic 20 improved, 5 stabilized; urologic 13 improved, 3 stabilized, 1 worsened; orthopedic (8 children presenting with scoliosis) 3 improved, 4 stabilized, 1 worsened. All children with OTFS (n = 9) improved in at least one and 8 improved in all affected categories.

Conclusions: Children with strong clinical suspicion for TFS (≥ 2 affected categories) with or without abnormal MR findings will likely benefit from surgery. In such cases we suggest a detailed full spine MR, a multidisciplinary diagnostic work-up, and eventual untethering through an interlaminar microsurgical approach.

MeSH terms

  • Adolescent
  • Cauda Equina / pathology*
  • Cauda Equina / surgery*
  • Child
  • Child, Preschool
  • Congenital Abnormalities / etiology
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Nervous System Diseases / etiology
  • Neural Tube Defects / complications
  • Neural Tube Defects / pathology*
  • Neural Tube Defects / surgery*
  • Neurologic Examination
  • Neurosurgical Procedures / adverse effects
  • Neurosurgical Procedures / methods*
  • Patient Care Team
  • Postoperative Complications / epidemiology
  • Skin Diseases / etiology
  • Spinal Cord Compression / etiology
  • Spinal Cord Compression / pathology
  • Spine / pathology
  • Treatment Outcome
  • Urologic Diseases / etiology