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Neuroradiologie Scan 2022; 12(04): 269-291
DOI: 10.1055/a-1673-1345
CME-Fortbildung

Praktischer Ansatz für die Diagnose von Wirbelsäulendysplasien

A practical approach to diagnosis of spinal dysraphism
Bárbara Trapp
,
Tomás de Andrade Lourenção Freddi
,
Monique de Oliveira Morais Hans
,
Isadora F. T. Lemos Calixto
,
Emi Fujino
,
Laila C. Alves Rojas
,
Stênio Burlin
,
Danilo M. Cerqueira Costa
,
Henrique Carrete Junior
,
Nitamar Abdala
,
Luís A. Tobaru Tibana
,
Eduardo T. Takehara
,
Gustavo D. Gomez
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Spinale Dysrhaphien sind angeborene Fehlbildungen des Rückenmarks, die durch eine Störung in der komplexen Kaskade embryologischer Ereignisse bei der Entwicklung der Wirbelsäule bedingt sind. Die Kenntnis der normalen embryologischen Entwicklung des Rückenmarks ist entscheidend für das Verständnis der Pathogenese, der neuroradiologischen Szenarien und der klinisch-radiologischen Klassifizierung angeborener Fehlbildungen des Rückenmarks.

Abstract

Spinal dysraphisms (SDs) are congenital malformations of the spinal cord, determined by derangement in the complex cascade of embryologic events involved in spinal development. They represent a heterogeneous group ranging from mild clinical manifestations—going unnoticed or being discovered at clinical examination—to a causal factor of life quality impairment, especially when associated with musculoskeletal, gastrointestinal, genitourinary, or respiratory system malformations. Knowledge of the normal embryologic development of the spinal cord—which encompasses three main steps (gastrulation, primary neurulation, and secondary neurulation)—is crucial for understanding the pathogenesis, neuroradiologic scenarios, and clinical-radiologic classification of congenital malformations of the spinal cord. SDs can be divided with clinical examination or neuroradiologic study into two major groups: open SDs and closed SDs. Congenital malformations of the spinal cord include a wide range of abnormalities that vary considerably in imaging and clinical characteristics and complexity and therefore may represent a diagnostic challenge, even for the experienced radiologist.

Kernaussagen

  • Die Neurobildgebung spielt eine entscheidende Rolle bei der Diagnose, der Erkennung von Fehlbildungen, der Therapieplanung und der postoperativen Beurteilung von spinalen Dysraphien. Im Allgemeinen lassen sich diese am besten mit der MRT charakterisieren.

  • Nach der klinisch-radiologischen Klassifikation werden spinale Dysraphien in 2 Hauptgruppen eingeteilt: offene und geschlossene Formen, je nachdem, ob ein Hautdefekt über der Anomalie liegt. Bei offenen spinalen Dysraphien sind das Nervengewebe und die Rückenmarkshäute direkt der äußeren Umgebung ausgesetzt. Bei geschlossenen Formen sind das Nerven- und Meningealgewebe von Haut oder subkutanem Gewebe bedeckt, sodass keine Exposition der Plakode besteht.

  • Die Myelomeningozele ist ein neurochirurgischer Notfall wie alle offenen spinalen Dysraphien und stellt mit mehr als 98 % der Fälle die häufigste Form der offenen spinalen Dysraphien dar.

  • Lipome mit duralem Defekt bilden ein Kontinuum von Anomalien (Lipomyelomeningozele, Lipomyelozele und Lipomyeloschisis), die einem gemeinsamen pathophysiologischen Prozess entspringen. Sie unterscheiden sich voneinander durch die Lage der Schnittstelle zwischen Rückenmark und Lipom. Zusammen machen diese Anomalien 75,9 % aller spinalen Lipome und 16,4 % aller geschlossenen spinalen Dysraphien aus.

  • Die Unterscheidung zwischen den beiden Typen der Diastematomyelie hängt von der Entwicklung des Primitivstreifengewebes ab: Bei Typ I entwickelt sich der dazwischen liegende Primitivstreifen zu Knochen oder Knorpel und bildet eine Scheidewand, die den Duralsack in 2 Teile trennt. Bei Typ II wird der Primitivstreifen resorbiert oder bildet ein fibröses Septum und ein einziger Duralsack umschließt beide Duraschläuche.



Publication History

Article published online:
04 October 2022

© 2022. The Radiological Society of North America. All rights reserved. Originally published in English in RadioGraphics 2021; 41: 559–575. Online published in 10.1148/rg.2021200103. Translated and reprinted with permission of RSNA. RSNA is not responsible for any inaccuracy or error arising from the translation from English to German.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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