Abstract
The word diastematomyelia was first introduced in 1837 by Ollivier1 to delineate a congenital malformation of the spinal cord and spinal column. It is characterized by a splitting of the spinal cord associated with a bony or a cartilaginous septum transfixing the neural tissue, and with other vertebral abnormalities. Such anatomic criteria are widely adopted to define diastematomyelia which represents an extreme degree of the occult spinal dysraphism according to the hypothesis postulated by Lichtenstein2 in 1940. Diastematomyelia basically differs from diplomyelia by the fact that the former is a sagittal division of a single cord into two “half-cords” by a septum, while the later is a true duplication of the cord into two “twin cords” with no bony transfixion. Since intermediate varieties do exist, these two conditions will be described together in this section, along with those involving the cauda equina.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Similar content being viewed by others
References
Ollivier CP: Traité des Maladies de la Moélle Épiniére, Third edition, Vol. I. Mequignon-Marvis, Paris, 1837.
Lichtenstein BW: “Spinal dysraphysm”. Spina bifida and myelodysplasia. Arch Neurol Psychiatry 44: 792–810, 1940.
Herren RX, Edwards JE: Diplomyelia (duplication of the spinal cord). Arch Pathol 30: 1203–1214, 1940.
Matson DD, Woods RP, Campbell JB, Ingraham FD: Diastematomyelia (congenital clefts of the spinal cord). Diagnosis and surgical treatment. Pediatrics 6: 98–112, 1950.
Bremer JL: Dorsal intestinal fistula, accessory neurenteric canal, diastematomyelia. Arch Pathol 54: 132–138, 1952.
James CCM, Lassman LP: Spinal dysraphism: an orthopaedic syndrome in children accompanying occult forms. Arch Dis Child 35: 315–327, 1960.
Winter RB, Haven JJ, Moe JH, Lagaard SM: Diastematomyelia and congenital spine deformities. J Bone Joint Surg 56: 27–39, 1974.
Hilal SK, Marton D, Pollack E: Diastematomyelia in children. Radiology 112: 609–621, 1974.
Guthkelch AN: Diastematomyelia with median septum. Brain 4: 729–742, 1974.
Neuhauser EB, Wittenborg MH, Delhinger K: Diastematomyelia transfixation of the cord or cauda equina with congenital anomalies of the spine. Radiology 54: 659–664, 1950.
Liliequist B: Diastematomyelia: report of a case examined by gas myelography. Acta Radiol (Diagn) 3: 497–502, 1965.
Banniza von Bazan UK, Rompe G, Krastel A, Martin K: Diastematomyelie ihre Bedeutung für die behandlung von Mibildungsskoliosen. Z Orthop 114: 881–889, 1976.
Frerebeau P, Dimeglio A, Gras A, Harbi H: Diastematomyelia. Report of 21 cases surgically treated by a neurological and orthopedic team. Childs Brain 10: 328–339, 1983.
James CCM, Lassman LP: Diastematomyelia with median septum in spina bifida occulta. Indications for surgery. Z Kinderchir Grenzgeb 22: 460–464, 1977.
McMaster MJ: Occult intraspinal anomalies and congenital scoliosis. J Bone Joint Surg 66: 588–601, 1984.
Anand AK, Kuchner EF, James R: Cervical diastematomyelia: uncommon presentation of a rare congenital disorder. Comput Radiol 9: 45–49, 1985.
Arredondo F, Haughton VM, Hemmy DC, Zelaya B, Williams AL: The computed tomographic appearance of the spinal cord in diastematomyelia Radiology 136: 685–688, 1980.
Beyerl BD, Ojemann RG, Davis KR, Hedley Whyte ET, Mayberg MR: Cervical diastematomyelia presenting in adulthood. Case report. J Neurosurg 62: 449–453, 1985.
Claussen CD, Lohkamp FW, von Bazan UB: The diagnosis of congenital spinal disorders in computed tomography (C.T.) Neuropaediatrie 8: 405–417, 1977.
Claussen C, Banniza von Bazan U, Jaschke W, Schilling V: Die Bedeutung der Computertomographie in der Diagnostik Kongenitales Spinaler Missbildungen, Insbesondere der Diastematomyelie. Roefo Fortschr Geb Roentgenstr Nuklearmed 133:520-527, 1980,
Fernbach SK, Naidich TP, McLone DG, Leestma JE: Computed tomography of primary intrathecal Wilms tumor with diastematomyelia. J Comput Assist Tomogr 8: 523–528, 1984.
Giordano GB, Davidovits P, Cerisoli M, Giulioni M: Cervical diplomyelia revealed by computed tomography (C.T.). Neuropediatrics 13: 93–94, 1982.
Gras M, Dimeglio A, Frerebeau P, Rolin B, Bourbotte G, Castan P: Diastematomye- lie: exploration neuroradiologique moderne et corrélations anatomo-cliniques. A propos de vingt observations. Rev Int Pediatr 144: 35–36, 1984.
Han JS, Benson JE, Kaufman B, Rekate HL, Alfidi RJ, Bohlman HH: Demonstration of diastematomyelia and associated abnormalities with MR imaging. Am J Neuro- radiol 6: 215–219, 1985.
Haughton VM: Present status of C.T. in the lumbar spine examination. Eur Neurol 21: 198–203, 1982.
Kuchner EF, Anand AK, Kaufman BM: Cervical diastematomyelia: a case report with operative management. Neurosurgery 16: 538–542, 1985.
Levine RS, Geremia GK, McNeill TW: C.T. demonstration of cervical diastematomyelia. J Comput Assist Tomogr 9: 592–594, 1985.
Lohkapip F, Claussen C, Schuhmacher G: C.T. demonstration of pathologic changes of the spinal cord accompanying spina bifida and diastematomyelia. Prog Pediatr Radiol 6: 200–227, 1978.
Scatliff JH, Bidgood WD, Killebrew K, Staab EV: Computed tomography and spinal dysraphism: clinical and phantom studies. Neuroradiology 17: 71–75, 1979.
Scotti G, Musgrave MA, Harwood-Nash DC, Fitz CR, Chuang SH: Diastematomyelia in children. Metrizamide and CT metrizamide myelograph. Am J Roengenol 135: 1225–1232, 1980.
Tadmor R, Davis KR, Roberson GM, Chapman PH: The diagnosis of diastematomyelia by computed tomography. Surg Neurol 8: 434–436, 1977.
Whittle IR, Besser M: Congenital neural abnormalities presenting with mirror movements in a patient with Klippel-Feil syndrome. J Neurosurg 59: 891–894, 1983.
Roosen N, De Moor J: Cervicobrachialgia with congenital vertebral anomalies and diastematomyelia. Surg Neurol 21: 493–496, 1984.
Schiffer J, Till K: Spinal dysraphism in the cervical and dorsal regions in childhood. Childs Brain 9: 73–84, 1982.
Rokos J: Pathogenesis of diastematomyelia and spina bifida. J Pathol 117: 155–161, 1975.
Lourie H, Biemy JP: Diastematomyelia with two spurs and intradural neural crest elements. J Neurosurg 32: 248, 1970.
McClelland RR, Marsh DG: Double diastematomyelia. Radiology 123: 378, 1977.
Abraham E: Sacral agenesis with associated anomalies (caudal regression syndrome): autopsy case report. Clin Orthop Relat Res 145: 168–171, 1979.
Cameron AH: The Arnold-Chiari and other neuro-anatomical malformations associated with spina bifida. J Pathol Bacteriol 73: 195–211, 1957.
Davis ED: Diastematomyelia with early Arnold-Chiari syndrome and congenital dys- plasic hip. Clin Orthop 52: 179–184, 1968.
Kapsenberg JG, Vanlookeren-Campagne JA: A case of spina bifida combined with diastematomyelia, the anomaly of Chiari and hydrocephalus. Acta Anat 7: 366–388, 1949.
Messimy R, Metzger J, Bar D: Diastematomyelie et malformation dArnold-Chiari associées. A propos de 3 cas. Livre Jubilaire du Pr P.F. Girard, Lyon, 1976.
Moes CA, Hendrick EB: Diastematomyelia. J Pediatr 63: 238–248, 1963.
Chiari H: Veber Veränderungen des kleinhirns infolge von hydrocephalic des Grosshirns. Dtsch Med Wochenschr 42: 1172–1175, 1891.
Banniza von Bazan U: Kaudales Regressionssyndrom und Diastematomyelie. Was Enthaelt Hohls Erstbeschreibung Einer Kreuzbe indysgenesie. Z Orthop Grenzgeb 1: 65–72, 1977.
Noeldge G, Billmann P, Boehm N: Kaudale Regression mit Sirenomelie und Dysplasia Renofaeialis (Potter-syndrom). Roefoe Fortschr Geb Roentgenstr Nuklearmed 136: 587–591, 1982.
Schwartz W, Poulsen HK, Andersen PE: Sirenomelia: das Kaudale Regressionsyn- drom. Monatsschr Kinderheilkd 130: 565–566, 1982.
Talamo TS, Macpherson TA, Dominguez R: Sirenomelia. Angiographie demonstration of vascular anomalies. Arch Pathol Lab Med 106: 347–348, 1982.
Blocklehurst G: The pathogenesis of spina bifida: a study of the relationship between observations, hypothesis and surgical incentive. Dev Med Child Neurol 13: 148, 1971.
Gardner WJ: Diastematomyelia and Kliffel-Feil syndrome. Relationship to hydrocephalus, syringomyelia, meningocela, myelomeningocele and anencephalus. Cleve Clin Quart 31: 19–44, 1964.
Marr GE, Uihlein A: Diplomyelia and compression of spinal cord and not of cauda equina, by congenital anomaly of third lumbar vertebra. Surg Clin North Am 24: 963–968, 1944.
Matson DD: Neurosurgery of Infancy and Childhood, Second edition. Charles C Thomas, Springfield, IL, 1969.
Barson AJ: The vertebral level of termination of the spinal cord during normal and abnormal development. J Anat 106: 487–497, 1970.
Vandresse JH, Cornelis G: Diastematomyelia: report of eight observations. Neuroradiology 10: 87–93, 1975.
James CCM, Lassman LP: Diastematomyelia. Arch Dis Child 33: 356, 1958.
Anderson FM: Diastematomyelia. Report of 2 case submitted to laminectomy. Acta Orthop Scand 36: 257–264, 1966.
Arcomano JP, Sengstacken RL, Wunderlich HO: Diastematomyelia. J Dis Child 104: 393, 1962.
Basauri L, Palma A, Zuleta A, Holzer F, Poblete R: Diastematomyelia. Report of 18 cases. Acta Neurochir 51: 91–96, 1979.
Benstead JC: A case of diastematomyelia. J Pathol Bacteriol 6: 553–557, 1954.
Chambers WR: Diastematomyelia case diagnosed pre-operatively. J Paediatr 45: 668–671, 1954.
Freenan LW: Late symptoms from diastematomyelia. J Neurosurg 18: 538–541, 1961.
Holman CB, Svien HJ, Bickel WH: Diastematomyelia. Pediatrics 15: 191–194, 1955.
Hood RW, Riseborough EJ, Nehme AM, Micheli U, Strand RD, Neuhauser EBD: Diastematomyelia and structural spinal deformities. J Bone Joint Surg 64: 520–528, 1980.
James CCM, Lassman LP: Diastematomyelia. A critical survey of 24 cases submitted to laminectomy. Arch Dis Child 39: 125–130, 1964.
Jones JB: Diastematomyelia. Clin Orthop 21: 164–168, 1962.
Kapsalakis Z: Diastematomyelia in 2 sisters. J Neurosurg 21: 66–67, 1964.
Kennedy PR: New data on diastematomyelia. J Neurosurg 51: 355–361, 1979.
Lapras C, Bret P, Capdeville J: La diastématomyélie. Réflexion à propos d’une série de 6 observations. Neurochirurgie 24: 381–389, 1978.
Lassale B, Rigault P, Pouliquen JC, Padovani JP, Guyonvarch G: La diatematomyelie (ou syndrome de la double chorde). Etude de 21 cas. Rev Chir Orthoped 66: 123–139, 1980.
Lefevre J, Klein MR, Faure C: Diastematomyélie. Rev Neurol 94: 357–362, 1956.
McCann P: Diastematomyelia. Irish J Med Sei 6: 155–160, 1966.
Maroun FB, Jacob JC, Heneghan WD: La diastematomyélie, ses manifestations cliniques et son traitement chirurgical. Neurochirurgie 18: 285–316, 1972.
Mathieu JP, Decarie M, Dube J, Marton D: La diastematomyélie. Etude de 69 cas. Chir Pediatr 23: 29–35, 1982.
Perret G: Symptoms and diagnosis of diastematomyelia. Neurology 10: 51–60, 1960.
Rigault P, Pouliquen JC, Guyonvarch G, Durand Y: Quatre cas de diastematomyélie. Rev Chir Orthop 58: 33–50, 1972.
Ritchie GW, Flanagan MN: Diastematomyélia. CMAJ 100:, 1969.
Seaman WB, Schwartz HG: Diastematomyelia in adults. Radiology 70: 692, 1958.
Shorey WD: Diastematomyelia associated with dorsal kyphosis producing paraplegia J Neurosurg 12: 300–305, 1955.
Ugarte N, Gonzalez-Crussi F, Sotelo-Avila C: Diastematomyelia associated with teratomas. J Neurosurg 53: 720–725, 1980.
English WJ, Maltby GL: Diastematomyelia in adults. J Neurosurg 27: 260–264, 1967.
Albert-Jay L: La diastematomyélie. Rapport d’un cas chez l’adulte. Revue de la littérature. Thèse Méd Paris, 1979, 144 pp.
Chehrazi B, Haldeman S: Adult onset of tethered spinal cord syndrome due to fibrous diastematomyelia: case report. Neurosurgery 16: 681–685, 1985.
Garcia FA, Kranzler LI, Siqueira EB, Weinberg PE, Kranzler KJ: Diastematomyelia in an adult. Surg Neurol 14: 93–94, 1980.
Garza-Mercado R: Diastematomyelia and intramedullary epidermoid spinal cord tumor combined with extradural teratoma in an adult: case report. J Neurosurg 58: 954–958, 1983.
Kühner A, Hame J, Krastell A, Banniza UK, Martin R: Role de la diastematomyélie dans le diagnostic différentiel et le traitement des paraplégies cypho-scoliotiques. Neurochirurgie 22: 755–759, 1976.
McEwen GD, Bunnel WP, Sriram K: Acute neurological complications in the treatment of scoliosis. A report of the scoliosis research society. J Bone Joint Surg 57A: 404, 1975.
Faithfull DK: Diastematomyelia complicating congenital scoliosis. J Neurosurg 18: 538–541, 1961.
Tax HR, Person V, Tuccio M: A podiatric presentation of diastematomyelia. J Am Podiatry Assoc 72: 337–341, 1982.
Claudy AL, Thivolet J, Meyer F: La diastematomyelie: une cause rare de mal perforant plantaire. Lyon Méd 6: 503–509, 1976.
Mann KS, Khosla VK, Gulati DR, Malik AK: Spinal neurenteric cyst. Association with vertebral anomalies, diastematomyelia, dorsal fistula and lipoma. Surg Neurol 21: 358–362, 1984.
Scatliff JH, Till K, Hoare RD: Incomplete, false and true diastematomyelia. Radiological evaluation by air myelography and tomography. Neuroradiology 116: 349–354, 1975.
Weinstein MA, Rothner AD, Duehesnau P, Dohn DF: Computed tomography in diastematomyelia. Radiology 118: 609–611, 1975.
Meacham WF: Surgical treatment of diastematomyelia. J Neurosurg 27: 78–85, 1967.
Pang D, Parrish RG: Regrowth of diastematomyelic bone spur after extradural resection. J Neurosurg 59: 887–890, 1983.
Gunasekera WSL, Richardson AE, Seneviratne KN, Eversden ID: Clinical correlation of urodynamie findings in patients with localized partial lesions of the spinal cord and cauda equina. Surg Neurol 21: 148–154, 1984.
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1989 Springer-Verlag New York Inc.
About this chapter
Cite this chapter
Bret, P., Patet, J.D., Lapras, C. (1989). Diastematomyelia and Diplomyelia. In: Raimondi, A.J., Choux, M., Di Rocco, C. (eds) The Pediatric Spine II. Principles of Pediatric Neurosurgery. Springer, New York, NY. https://doi.org/10.1007/978-1-4613-8829-6_4
Download citation
DOI: https://doi.org/10.1007/978-1-4613-8829-6_4
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4613-8831-9
Online ISBN: 978-1-4613-8829-6
eBook Packages: Springer Book Archive