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Eur J Paediatr Neurol ; 16(2): 103-17, 2012 Mar.
Article in English | MEDLINE | ID: mdl-21835656

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.


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