Diastematomyelia

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Classification according to ICD-10
Q06.2 Diastematomyelia
ICD-10 online (WHO version 2019)
Magnetic resonance imaging (T2 axial and sagittal) of diastematomyelia. The findings were not previously known in the 80-year-old woman.

The diastematomyelia is a rare congenital malformation of the spinal canal, are divided with parts of the spinal cord in the longitudinal direction. It belongs to the dysraphs .

The term is derived from ancient Greek μῦελόν = (back) marrow and διάστημα diástēma "space".

A bony, cartilaginous or connective tissue spur or a septum (septum) crosses the spinal canal over a length of one or more vertebral bodies and thus divides the spinal cord into two strands.

This malformation was first described by CP Oliver in 1837 .

Classification

Partial duplication of the spinal cord can also be referred to as diplomyelia .

By D. Pang and colleagues proposed in 1992, diastematomyelia and diplomyelia the term split cord malformation summarize.

A classification according to Pang in takes place

  • Split cord type I with two separate dura tubes
  • Split cord type II with a common dural tube

Clinical manifestations

Diagnostic criteria are:

A manifestation only in adulthood is considered rare. If the spinal cord is asymmetrical, neurological deficits are regularly found on the lean side. Malformation scoliosis often develops over time.

diagnosis

Already in an X-ray overview of the spine, changes and often bony spurs can usually be recognized in the lumbar region of the vertebral bodies, arch roots or the vertebral arch.

A detailed representation of cartilaginous changes can be reliably achieved with magnetic resonance imaging . A precise analysis of the dural tube is possible by means of myelography , usually in conjunction with computed tomography (myelo-CT).

The malformation can be detected in prenatal sonography even before birth .

In newborns and infants, a clinical suspicion can be confirmed sonographically.

pathology

As a result of the local fixation of the spinal cord, the ascension is hindered in the course of growth, so that ischemic compression results. This results in the gradually increasing orthopedic and neurological disease patterns.

therapy

Surgical treatment is required at the latest when clinical-neurological symptoms in the sense of a tethered cord appear.

literature

  • MS Dias, D. Pang: Split cord malformations. In: Neurosurgery clinics of North America. Volume 6, No. 2, April 1995, ISSN  1042-3680 , pp. 339-358, PMID 7620358 . (Review).
  • M. Roth, J. Krkoska, V. Hrazdirová, M. Lukesová, O. Gotfrýd: Diastematomyelia. In: The Radiologist. Volume 11, No. 1, January 1971, ISSN  0033-832X , pp. 33-36, PMID 5093503 .
  • AJ Barkovich: Pediatric Neuroimaging. 2nd Edition. Raven 1995, ISBN 0-7817-0179-1 , p. 513 ff.
  • U. Banniza von Bazan: The association between congenital elevation of the scapula and diastematomyelia: a preliminary report. In: The Journal of bone and joint surgery. British volume. Vol. 61, No. 1, February 1979, ISSN  0301-620X , pp. 59-63, PMID 422634 .

Individual evidence

  1. ↑ a b c Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  2. ↑ CP Oliver: Traité des Maladies de la Mòelle. In: Epinière. Vol 1, 3rd edition. Mequignon, Marvis, Paris, p. 189.
  3. ^ W. Pschyrembel: Clinical dictionary. 265th edition. Walter de Gruyter, 2014, ISBN 978-3-11-018534-8 , p. 362.
  4. ↑ D. Pang, MS Dias, M. Ahab-Barmada: Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations. In: Neurosurgery. Volume 31, No. 3, September 1992, ISSN  0148-396X , pp. 451-480, PMID 1407428 (review).
  5. ↑ D. Pang: Split cord malformation: Part II: Clinical syndrome. In: Neurosurgery. Volume 31, No. 3, September 1992, ISSN  0148-396X , pp. 481-500, PMID 1407429 (review).
  6. ↑ J. List, R. Stendel, KH Rudolph, M. Brock: A case of diastematomyelia (Split Cord Malformation Type I) with clinical manifestations in adulthood. In: Central Journal for Neurosurgery. Volume 55, No. 4, 1994, ISSN  0044-4251 , pp. 212-217, PMID 7887051 (English title: A case of diastematomyelia (split cord malformation type I) with clinical manifestation in adulthood ).
  7. ^ UK Banniza von Bazan, G. Rompe, A. Krastel, K. Martin: Dastematomyelie - its importance for the treatment of malformation scolioses. In: Journal for orthopedics and their border areas. Volume 114, No. 6, December 1976, ISSN  0044-3220 , pp. 881-889, PMID 795202 (English title: Diastematomyelia - its importance in the treatment of congenital scoliosis ).
  8. ↑ W. Schuster, D. Färber (Ed.): Children's radiology. Imaging diagnostics. Springer 1996, ISBN 3-540-60224-0 .
  9. ↑ H. Struben, E. Visca, W. Holzgreve, A. Kang, P. Hetzel, J. Schneider, S. Tercanli: Prenatal diagnosis of diastematomyelia and “tethered cord” - a case report with an overview of the literature. In: Ultrasound in Medicine. Volume 29, No. 1, February 2008, ISSN  0172-4614 , pp. 72-76, doi : 10.1055 / s-2007-963208 , PMID 17926255 (English title: Prenatal diagnosis of diastematomyelia and tethered cord - a case report and review of the literature ).
  10. ↑ C. Könner, I. Gassner, U. Mayr, A. Kreczy: For the diagnosis of diastematomyelia by means of ultrasound. In: Clinical Pediatrics. Volume 202, No. 2, Mar-Apr 1990, ISSN  0300-8630 , pp. 124-128, doi : 10.1055 / s-2007-1025504 , PMID 2182934 .

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