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DOI: 10.1055/s-2007-963209
© Georg Thieme Verlag KG Stuttgart · New York
Sonografische Diagnose eines kaudalen Regressionssyndroms
Sonographic Diagnosis of Caudal Regression SyndromePublication History
eingereicht: 27.4.2006
angenommen: 5.4.2007
Publication Date:
05 October 2007 (online)

Zusammenfassung
Das kaudale Regressionssyndrom (KRS) ist eine seltene angeborene Fehlbildung der unteren Wirbelsäulensegmente und des Neuralrohres. Die Symptome sind sowohl motorische als auch sensorische neurologische Defizite der unteren Extremität und eine verminderte Kontrolle der Blasen- und Darmfunktion. Die Ätiologie und Pathogenese des KRS sind weitgehend unbekannt. Wir stellen ein männliches Neugeborenes vor, das postnatal durch Fehlstellungen der unteren Extremität aufgefallen war. Sonografisch zeigte sich ein lumbal endender Spinalkanal. Der sonografische Verdacht auf ein kaudales Regressionssyndrom wurde in einer Kernspintomografie bestätigt. Dopplersonografisch zeigte das abdominelle Gefäßsystem eine Fehlbildung der unpaaren Äste der Aorta. Im Folgenden werden Klinik, Diagnostik und mögliche Ätiologien des kaudalen Regressionssyndroms diskutiert.
Abstract
Caudal regression sequence (CRS) is a rare developmental defect of the lower spinal segments and the neural tube. Motor and sensory neurological deficits of the lower extremities as well as a reduced control of bowel and bladder functions are the main symptoms. Etiology and pathogenesis are widely unknown. This article discusses a newborn male with postnatal anomalies of the lower extremities. Sonographically, the spinal cord ended in the lumbar region. NMR confirmed the suspected sonographic finding of CRS. Sonographic examination of the abdominal vessel system depicted a common origin and junction of the hepatic artery, splenic artery and superior mesenteric artery from one common truncus. This points to a possible relict of a persisting vitelline artery. As previously described in sirenomelia, the findings in the present case indicate a possible vascular etiology of CRS.
Key words
Caudal regression syndrome - dysplasias - ultrasound - mesentery artery
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Dr. Eva Stierkorb
Klinik für Allgem. Pädiatrie und Neonatologie, Universitätsklinikum des Saarlandes
Homburg/Saar
Kirrberger Strasse 1
66421 Homburg/Saar
Phone: ++49/68 41/1 62 80 00
Fax: ++49/68 41/1 62 84 51
Email: kiesti@uniklinikum-saarland.de