Skip to main content
SpringerLink
Log in

Cystic spinal dysraphism of the cervical and upper thoracic region

  • Original Paper
  • Published:
Child’s Nervous System Aims and scope Submit manuscript

Abstract

Background

Cystic dysraphic lesions of the cervical and upper thoracic region are rare and only a few series have been published about the topic. These malformations can be divided into categories that include both myelocystoceles and the so-called cervical meningoceles or myelomeningoceles.

Methods

A retrospective study of 18 patients was conducted.

Results

In 17 patients a squamous or a cicatricial epithelium of variable thickness covered the dome of the lesions, while the base was covered with full-thickness skin. In one case the skin was entirely normal. Four patients displayed associated CNS malformations and three more had systemic congenital anomalies. All patients underwent surgical exploration and the length of time between birth and surgery ranged from 6 h to 9 months. The most frequent surgical finding, seen in 14 patients, was a stalk connecting the dorsal surface of the spinal cord to the cyst. In three patients the findings were consistent with myelocystocele. Only in one case was a true meningocele found. Hydrocephalus and Chiari II malformation were not as consistently associated as in myelomeningoceles. Neurological signs and symptoms were not so marked as in myelomeningoceles and were found in the follow-up of four patients. In two of them there was a non-progressive deficit, probably expressing an imperceptible involvement of the nervous system in the first year of life. The histopathological findings were of three types: neuroglial stalks, fibrovascular stalks and myelocystoceles.

Conclusions

Cystic dysraphisms of the cervical and upper thoracic region differ clinically and structurally from meningomyelocele and have a more favorable outcome. We believe that these malformations have not been properly labeled and propose a classification based on the structures found inside the cyst.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Ando S, Moritake K, Takaia M (1993) MRI demonstration of cervical meningocele. Neuroradiology 35:551–552

    Article  CAS  PubMed  Google Scholar 

  2. Ankola PA, Fernandes Y, Tunnessen WW (1998) Picture of the month. Cervical mielomeningocele. Arch Pediatr Adolesc Med 152:299–300

    Article  CAS  PubMed  Google Scholar 

  3. Barson AJ (1970) Spina bifida: the significance of the level and the extent of the defect to the morphogenesis. Dev Med Child Neurol 12:129–144

    Article  CAS  PubMed  Google Scholar 

  4. Bhargawa R, Diamond DI, Benzie RJ, Ventureyra ECG, Higgins MJ, Martin DJ (1992) Prenatal demonstration of a cervical myelocistocele. Prenat Diagn 12:653–659

    Article  Google Scholar 

  5. Cameron AH (1956) The spinal cord lesion in spina bifida cystica. Lancet 2:171–174

    Article  Google Scholar 

  6. Choudhury AR, Gonog MA, Mahmood K (1996) Heterotopic spinal cord? A curiosity. Childs Nerv Syst 12:166–168

    Article  CAS  PubMed  Google Scholar 

  7. Delashaw JB, Park TS, Cail WM, Vollmer DG (1987) Cervical meningocele and associated spinal anomalies. Childs Nerv Syst 3:165–169

    Article  CAS  PubMed  Google Scholar 

  8. Doran PA, Guthkelch AN (1961) Studies in spina bifida cystica. I. General survey and reassessment of the problem. J Neurol Neurosurg Psychiatry 24:331–345

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  9. Duprez TP, Laterre EC (1995) Unusual form of closed dysraphysm of the cervical spine. Acta Neurol Belg 95:42–43

    CAS  PubMed  Google Scholar 

  10. El Shabrawi-Caelen L, White WL1, Soyer HP, Kim BS, Frieden IJ, McCalmont TH (2001) Rudimentary meningocele: remnant of a neural tube defect? Arch Dermatol 137:45–50

    Article  PubMed  Google Scholar 

  11. Erşahin Y, Barçin E, Mutluer S (2001) Is meningocele really an isolated lesion? Childs Nerv Syst 17:487–490

    Article  PubMed  Google Scholar 

  12. Feltes CH, Fountas KN, Dimopoulos VG, Escurra AI, Boev A, Kapsalaky EZ, Robinson JS, Troup EC (2004) Cervical meningocele in association with spinal abnormalities. Childs Nerv Syst 20:357–361

    Article  PubMed  Google Scholar 

  13. Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry 28:247–259

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  14. Golden JA, Chernoff GF (1993) Intermittent pattern of neural tube closure in two strains of mice. Teratology 47:73–80

    Article  CAS  PubMed  Google Scholar 

  15. Heuser CH, Streeter GL (1941) Development of the macaque embryo. Contrib Embryo 29:15–55

    Google Scholar 

  16. Lorber J (1973) Early results of selective treatment of spina bifida cystica. Br Med J 4:201–204

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  17. Matson DD (1969) Neurosurgery of infancy and childhood, 2nd edn. Thomas, Springfield, IL

    Google Scholar 

  18. McLone DG (1998) The biological resolution of malformations of the central nervous system. Neurosurgery 43:1375–1381

    CAS  PubMed  Google Scholar 

  19. McLone DG, Knepper PA (1989) The cause of Chiari II malformation: a unified theory. Pediatr Neurosci 15:1–12

    Article  CAS  PubMed  Google Scholar 

  20. May D, Rilliet B, Berney J (1992) Méningocèles et méningomyélocystocèles cervicales. A propos de 4 cas. Neurochirurgie 38:347–352

    CAS  PubMed  Google Scholar 

  21. Meyer-Heim AD, Klein A, Boltshauser E (2003) Cervical myelomeningocele. Follow-up of five patients. Eur J Paediatr Neurol 7:407–412

    Article  PubMed  Google Scholar 

  22. Muñoz BM, Diaz Llopis I, Martinez Angullo E, Lopez A, Garcia Aymerich V, Forner Valero JV (1994) A multicentre study of the hospital care of 1500 patients with mielomeningocele. Paraplegia 32:561–564

    Article  Google Scholar 

  23. Nishino A, Shirane R, So K, Arai H, Suzuki H, Sakurai Y (1998) Cervical myelocystocele with Chiari II Malformation: magnetic ressonance imaging and surgical treatment. Surg Neurol 49:269–273

    Article  CAS  PubMed  Google Scholar 

  24. Nishio S, Morioka T, Hikino S, Fukui M (2001) Cervical (myelo)meningoncoele: report of 2 cases. J Clin Neurosci 8:586–587

    Article  CAS  PubMed  Google Scholar 

  25. Odabasi Z, Gökçil Z, Kütürkçü Y, Vural O, Yardim M (1998) Mirror movements associated with cervical meningocele: case report. Minim Invasive Neurosurg 41:99–100

    Article  CAS  PubMed  Google Scholar 

  26. Pang D, Dias MS (1993) Cervical myelomeningoceles. Neurosurgery 33:363–373

    Article  CAS  PubMed  Google Scholar 

  27. Pang D, Dias MS, Ahab -Barmada M (1992) Split cord malformation. I. A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 31:451–480

    Article  CAS  PubMed  Google Scholar 

  28. Penfield W, Coburn DF (1938) Arnold–Chiari malformation and its operative treatment. Arch Neurol Psychiatry 40:328–336

    Article  Google Scholar 

  29. Perez LM, Wilbanks JT, Joseph DB, Oakes WJ (2000) Urological outcome of patients with cervical and upper thoracic mielomeningocele. J Urol 164:962–964

    Article  CAS  PubMed  Google Scholar 

  30. Raimondi AJ (1987) Pediatric neurosurgery. Theoretic principles. Art of surgical techniques. Springer, Berlin Heidelberg New York

    Google Scholar 

  31. Rosenberg D, Canterino I (1992) Myéloméningocèle cervico-dorsale; syringomyélie. Pédiatrie 47:155

    CAS  PubMed  Google Scholar 

  32. Smith GK, Smith ED (1973) Selection for treatment in spina bifida cystica. Br Med J 4:189–197

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  33. Stedman’s Medical Dictionary (1995) 26 edn. Williams and Wilkins, Baltimore

  34. Stein SC, Schut L, Ames MD (1974) Selection for early treatment in myelomeningocele: a retrospective analysis of various selection procedures. Pediatrics 54:553–557

    CAS  PubMed  Google Scholar 

  35. Steinbok P (1995) Dysraphic lesions of the cervical spinal cord. Neurosurg Clin N Am 6:367–376

    CAS  PubMed  Google Scholar 

  36. Steinbok P, Cochrane DD (1991) The nature of congenital posterior cervical or cervicothoracic midline cutaneous mass lesions. J Neurosurg 75:206–212

    Article  CAS  PubMed  Google Scholar 

  37. Steinbok P, Cochrane DD (1995) Cervical meningoceles and myelocystoceles: a unifying hypothesis. Pediatr Neurosurg 23:317–322

    Article  CAS  PubMed  Google Scholar 

  38. Sun JCL, Steinbok P, Cochrane DD (2000) Cervical myelocystoceles and meningoceles: long-term follow-up. Pediatr Neurosurg 33:118–122

    Article  CAS  PubMed  Google Scholar 

  39. Suneson A, Kalimo H (1979) Myelocystocele with cerebellar heterotopia. Case report. J Neurosurg 51:392–396

    Article  CAS  PubMed  Google Scholar 

  40. Talwalker VC, Dastur DK (1970) “Meningoceles” and “meningomyeloceles” (ectopic spinal cord). Clinicopathological basis of a new classification. J Neurol Neurosurg Psychiatry 33:251–262

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  41. Tomlinson BE (1965) Heterotopic, non-functioning masses of nervous tissue in spina bifida cystica. J Clin Pathol 18:732–736

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  42. Van Allen MI, Kalousek DK, Chernoff GF, Juriloff D, Harris M, McGillivray BC, Yong SL, Langlois S, MacLeod PM, Chitayat D, Friedman JM, Wilson RD, McFadden D, Pantzar J, Richtie S, Hall JD (1993) Evidence of multi-site closure of the neural tube in humans. Am J Med Genet 47:723–743

    Article  PubMed  Google Scholar 

  43. Vogter DM, Culberson JL, Schochet SS, Gabriele OF, Kaufman HH (1987) “High spinal” dysrhaphism. Case report of a complex cervical meningocele. Acta Neurochir (Wien) 84:136–139

    Article  CAS  Google Scholar 

  44. Wu JK, Scott RM (1990) Myelopathy presenting decades after surgery for congenital cervical cutaneous lesions. Neurosurgery 27:635–637

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Section of Pediatric Neurosurgery, Department of Pediatric Surgery, Fernandes Figueira Institute, Oswaldo Cruz Foundation (M.S.–Fiocruz), Av. Rui Barbosa, 716, 22250-020, Rio de Janeiro, Brazil

    J. Francisco Salomão, René D. Leibinger & Antonio R. Bellas

  2. Section of Pediatric Neurosurgery, Federal University of São Paulo, Rua Botucatu 591/42, 04023-062, São Paulo, Brazil

    Sérgio Cavalheiro

  3. Section of Pediatric Neurosurgery, São Paulo University Medical School, Av. Dr. Enéas de Carvalho Aguiar, 255, 05403-000, São Paulo, Brazil

    Hamilton Matushita

  4. Department of Pathology, Fernandes Figueira Institute, Oswaldo Cruz Foundation (M.S.–Fiocruz), Av. Rui Barbosa, 716, 22250-020, Rio de Janeiro, Brazil

    Elide Vanazzi

  5. CT Scan Centro de Diagnóstico, Rua Santo Amaro, 80, 22211-230, Rio de Janeiro, Brazil

    Luiz A. M. de Souza & Andréa G. Nardi

Authors
  1. J. Francisco Salomão
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sérgio Cavalheiro
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Hamilton Matushita
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. René D. Leibinger
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Antonio R. Bellas
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Elide Vanazzi
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Luiz A. M. de Souza
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Andréa G. Nardi
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J. Francisco Salomão.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Salomão, J.F., Cavalheiro, S., Matushita, H. et al. Cystic spinal dysraphism of the cervical and upper thoracic region. Childs Nerv Syst 22, 234–242 (2006). https://doi.org/10.1007/s00381-005-1161-1

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-005-1161-1

Keywords

Search

Navigation