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Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome.
Eur J Paediatr Neurol. 2012 Mar; 16(2):103-17.EJ

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.

Authors+Show Affiliations

Department of Neurosurgery, Maastricht University Medical Center, PO Box 5800, 6202 AZ, Maastricht, The Netherlands. e.cornips@mumc.nlNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

21835656

Citation

Cornips, Erwin M J., et al. "Clinical Characteristics and Surgical Outcome in 25 Cases of Childhood Tight Filum Syndrome." European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society, vol. 16, no. 2, 2012, pp. 103-17.
Cornips EM, Vereijken IM, Beuls EA, et al. Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome. Eur J Paediatr Neurol. 2012;16(2):103-17.
Cornips, E. M., Vereijken, I. M., Beuls, E. A., Weber, J. W., Soudant, D. L., van Rhijn, L. W., Callewaert, P. R., & Vles, J. S. (2012). Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome. European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society, 16(2), 103-17. https://doi.org/10.1016/j.ejpn.2011.07.002
Cornips EM, et al. Clinical Characteristics and Surgical Outcome in 25 Cases of Childhood Tight Filum Syndrome. Eur J Paediatr Neurol. 2012;16(2):103-17. PubMed PMID: 21835656.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome. AU - Cornips,Erwin M J, AU - Vereijken,Ilse M P, AU - Beuls,Emile A M, AU - Weber,Jacobiene W, AU - Soudant,Dan L H M, AU - van Rhijn,Lodewijk W, AU - Callewaert,Piet R H, AU - Vles,Johan S H, Y1 - 2011/08/11/ PY - 2011/04/06/received PY - 2011/07/18/accepted PY - 2011/8/13/entrez PY - 2011/8/13/pubmed PY - 2012/6/13/medline SP - 103 EP - 17 JF - European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society JO - Eur. J. Paediatr. Neurol. VL - 16 IS - 2 N2 - 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. SN - 1532-2130 UR - https://www.unboundmedicine.com/medline/citation/21835656/Clinical_characteristics_and_surgical_outcome_in_25_cases_of_childhood_tight_filum_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1090-3798(11)00134-6 DB - PRIME DP - Unbound Medicine ER -