Acta Neurochirurgica

, Volume 137, Issue 3–4, pp 199–206 | Cite as

Surgical treatment of late neurological deterioration in children with myelodysplasia

  • M. Caldarelli
  • C. Di Rocco
  • C. ColosimoJr.
  • G. Fariello
  • M. Di Gennaro
Clinical Articles


Late deterioration of the neurological condition in patients operated on for myelomeningocele repair has been repeatedly reported in the literature. At the present time magnetic resonance imaging (MRI) allows one to recognize various pathological conditions which can be amenable to surgical correction in these patients, such as Chiari type II malformation, hydro/syringomyelia, decompensated hydrocephalus, and tethered spinal cord.

The authors report their experience with 26 myelodysplastic children operated on for myelomeningocele repair in the early neonatal period, who exhibited late deterioration at variable time intervals from the first operation. The children were examined pre-operatively by means of MRI; the results were compared with those provided by MRI in 46 myelodysplastic children who underwent the investigation as a routine follow-up control. The MRI findings were subdivided into 4 main groups of increasing severity from 1 to 4; in some subjects, associated pathological conditions (Chiari II malformation, hydromyelia, etc.) were detected as well.

Twenty-two out of the 26 patients with late neurological deterioration were operated on. Eleven of them (grades 2 to 4) underwent detethering of the fixed conus, with an improvement of their clinical picture from mild to good. On the other hand the remaining 11 subjects (grades 1 to 2) improved their condition following the correction of the associated abnormalities (malfunctioning CSF shunt: 5 cases; hydromyelia: 4 cases; symptomatic Chiari II malformation: 2 cases).


Tethered spinal cord spinal dysraphism magnetic resonance imaging post-repair myelomeningocele 


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  1. 1.
    Altman NR, Altman DH (1987) MR imaging of spinal dysraphism. AJNR 8: 533–538PubMedGoogle Scholar
  2. 2.
    Balasubramaniam C, Laurent JP, McCluggage C, Oshman D, Cheek WR (1990) Tethered cord syndrome after repair of meningomyelocele. Childs Nerv Syst 6: 208–211CrossRefPubMedGoogle Scholar
  3. 3.
    Barnes PD, Lester PD, Yamanashi WS, Prince JR (1986) Magnetic resonance imaging in infants and children with spinal dysraphism. AJNR 7: 465–472Google Scholar
  4. 4.
    Begeer JH, Meinhuizen de Regt MJ, Hogen Esch I, Ter Weeme CA, Moojt JJ, Vencken LM (1986) Progressive neurological deficit in children with spina bifida aperta. Z Kinderchir 41 [Suppl 1]: 13–15PubMedGoogle Scholar
  5. 5.
    Barolat GC, Schaefer D, Zeme S (1991) Recurrent spinal cord tethering by sacral nerve root following lipomyelomeningocele surgery. J Neurosurg 75: 143–145PubMedGoogle Scholar
  6. 6.
    Caldarelli M, Ceddia A, Di Rocco C, Iannelli A, Velardi F (1989) La sindrome del Tethered Cord nei bambini operati per mielomeningocele. Atti XXXVIII Congresso della Società Italiana di Neurochirurgia, Minerva Medica, Torino, pp 719–723Google Scholar
  7. 7.
    Chaseling RW, Johnston IH, Besser M (1985) Meningoceles and the tethered cord syndrome. Childs Nerv Syst 1: 105–108CrossRefPubMedGoogle Scholar
  8. 8.
    Dolan EJ, Tator CH, Transfeldt EE, Tafor CH, Simmons EH, Hughes KF (1980) The effect of spinal cord distraction on regional spinal cord blood flow in cats. J Neurosurg 53: 756–764PubMedGoogle Scholar
  9. 9.
    Fitz CR, Harwood-Nash DC (1975) The tethered conus. AJR 125: 515–523Google Scholar
  10. 10.
    Gryspeerdt GL (1963) Myelographic assessment of occult forms of spinal dysraphism. Acta Radiol 1: 702–717Google Scholar
  11. 11.
    Gupta RK, Sharma A, Jena A, Tyagi G, Prakash B, Khushu S (1990) Magnetic resonance evaluation of spinal dysraphism in children. Childs Nerv Syst 6: 161–165CrossRefPubMedGoogle Scholar
  12. 12.
    Hall WA, Albright AL, Brunberg JA (1988) Diagnosis of tethered cords by magnetic resonance imaging. Surg Neurol 30: 40–60Google Scholar
  13. 13.
    Heinz ER, Rosenbaum AE, Scarff TB, Reigel DH, Drayer BP (1979) Tethered spinal cord following myelomeningocele repair. Radiology 131: 153–160PubMedGoogle Scholar
  14. 14.
    Hoffman HJ, Hendrick EB, Humphreys RP (1976) The tethered spinal cord. Its protean manifestations, and surgical correction. Childs Brain 2: 145–155PubMedGoogle Scholar
  15. 15.
    Just M, Schwarz M, Ermert JA, Higer HP, Voth D, Pfannenstiel P (1988) Magnetic resonance imaging of dysraphic myelodysplasia. Findings in 56 children and adolescents with post-repair meningomyelocele. Childs Nerv Syst 4: 149–153CrossRefPubMedGoogle Scholar
  16. 16.
    Kang JK, Kim MC, Kim DS, Song JU (1987) Effects of tethering on regional spinal cord blood flow and sensory-evoked potentials in growing cats. Childs Nerv Syst 3: 35–39CrossRefPubMedGoogle Scholar
  17. 17.
    Linder M, Rosenstein J, Sklar FH (1982) Functional improvement after spinal surgery for the dysraphic malformations. Neurosurgery 11: 622–624PubMedGoogle Scholar
  18. 18.
    McLone DG, Naidich TP (1989) Myelomeningocele: outcome and late complication. In: McLaurin RL, Schut L, Venes JL, Epstein F (eds) Pediatrie neurosurgery. Saunders, Philadelphia, pp 53–70Google Scholar
  19. 19.
    McLone DG, Naidich TP (1989) The tethered spinal cord. In: McLaurin RL, Schut L, Venes JL, Epstein F (eds) Pediatric neurosurgery. Saunders, Philadelphia, pp 71–96Google Scholar
  20. 20.
    Modic MT, Weinstein MA, Pavlicek W, Starnes DL, Duchesneau PM, Boumphrey F, Hardy RJ (1983) Nuclear magnetic resonance imaging of the spine. Radiology 148: 757–762PubMedGoogle Scholar
  21. 21.
    Naidich TP, Fernback SK, McLone DG, Shkoluik A (1984) Sonography of the caudal spine and back. Congenital anomalies in children. AJR 142: 1229–1242PubMedGoogle Scholar
  22. 22.
    Oi S, Yamada H, Matsumoto S (1990) Tethered cord syndrome versus low-placed conus medullaris in an over-distended spinal cord following initial repair for myelodysplasia. Childs Nerv Syst 6: 264–269CrossRefPubMedGoogle Scholar
  23. 23.
    Pang D, Wilberger JE Jr (1982) Tethered cord syndrome in adults. J Neurosurg 57: 32–47PubMedGoogle Scholar
  24. 24.
    Reigel DH (1983) Tethered spinal cord. In: Humphreys RP (ed) Concepts in pediatric neurosurgery, Vol 4. Karger, Basel, pp 142–164Google Scholar
  25. 25.
    Sakamoto H, Hakuba A, Fujitani K, Nishimura S (1991) Surgical treatment of retethered spinal cord after repair of lipomyelomeningocele. J Neurosurg 74: 709–714PubMedGoogle Scholar
  26. 26.
    Sarwar M, Virapongse C, Bhimani S (1984) Primary tethered cord syndrome: a new hypothesis of its origin. AJNR 5: 235–242PubMedGoogle Scholar
  27. 27.
    Tamaki N, Shirataki K, Kojima N, Shouse Y, Matsumoto S (1988) Tethered cord syndrome of delayed onset following repair of myelomeningocele. J Neurosurg 69: 116–123Google Scholar
  28. 28.
    Tani S, Yamada S, Knighton RS (1987) Extensibility of the lumbar and sacral cord. Pathophysiology of the tethered spinal cord in cats. J Neurosurg 66: 116–123PubMedGoogle Scholar
  29. 29.
    Venes JL, Stevens EA (1983) Surgical pathology in tethered cord secondary to myelomeningocele repair. Implications for initial closure technique. In: Humphreys RP (ed) Concepts in pediatric neurosurgery, Vol 4. Karger, Basel, pp 165–185Google Scholar
  30. 30.
    Venes JL (1985) Surgical considerations in the initial repair of myelomeningocele and the introduction of a technical modification. Neurosurgery 17: 111–113PubMedGoogle Scholar
  31. 31.
    Wilden JN, Hadley D (1989) Delayed tethered cord syndrome after myelomeningocele repair (letter). J Neurosurg 70: 815PubMedGoogle Scholar
  32. 32.
    Yamada S, Zinke DE, Sanders D (1981) Pathophysiology of “tethered cord syndrome”. J Neurosurg 54: 494–503PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1995

Authors and Affiliations

  • M. Caldarelli
    • 1
  • C. Di Rocco
    • 1
  • C. ColosimoJr.
    • 1
    • 2
  • G. Fariello
    • 1
    • 3
  • M. Di Gennaro
    • 1
    • 4
  1. 1.Department of Neurosurgery, Section of Paediatric NeurosurgeryCatholic University Medical SchoolRome
  2. 2.Department of RadiologyCatholic University Medical SchoolRome
  3. 3.Department of Radiology“Bambino Gesū'” HospitalRomeItaly
  4. 4.Department of Urology“Bambino Gesū'” HospitalRomeItaly

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