title: Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome.
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.
  
  
  RESULTS
  
  
  
  METHODS
  17 girls, 8 boys, age 2-18 years, including 11 syndromal children.
  
  
  METHODS
  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.
authors.affiliation: Department of Neurosurgery, Maastricht University Medical Center, PO Box 5800, 6202 AZ, Maastricht, The Netherlands. e.cornips@mumc.nl
authors.forename: Erwin M J
authors.initials: EM
authors.lastname: Cornips
authors.forename: Ilse M P
authors.initials: IM
authors.lastname: Vereijken
authors.forename: Emile A M
authors.initials: EA
authors.lastname: Beuls
authors.forename: Jacobiene W
authors.initials: JW
authors.lastname: Weber
authors.forename: Dan L H M
authors.initials: DL
authors.lastname: Soudant
authors.forename: Lodewijk W
authors.initials: LW
authors.lastname: van Rhijn
authors.forename: Piet R H
authors.initials: PR
authors.lastname: Callewaert
authors.forename: Johan S H
authors.initials: JS
authors.lastname: Vles
mesh_terms: D000293:Adolescent; D002420:Cauda Equina / Q000473:pathology* / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D000013:Congenital Abnormalities / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology; D009436:Neural Tube Defects / Q000150:complications / Q000473:pathology* / Q000601:surgery*; D009460:Neurologic Examination; D019635:Neurosurgical Procedures / Q000009:adverse effects / Q000379:methods*; D010348:Patient Care Team; D011183:Postoperative Complications / Q000453:epidemiology; D012871:Skin Diseases / Q000209:etiology; D013117:Spinal Cord Compression / Q000209:etiology / Q000473:pathology; D013131:Spine / Q000473:pathology; D016896:Treatment Outcome; D014570:Urologic Diseases / Q000209:etiology