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Isolierte, nichtsyndromale Kraniosynostosen

Aktuelle Diagnostik- und Therapiekonzepte

Isolated, non-syndromal craniosynostosis

Current concepts of diagnostics and therapy

  • Leitthema
  • Published:
Der MKG-Chirurg Aims and scope

Zusammenfassung

Die Kraniosynostose ist ein vorzeitiger Verschluss einer oder mehrerer Schädelnähte aufgrund einer Störung innerhalb der osteogenen Prozesse, die während der Ossifikation des Schädels ablaufen. Diese bedingen ein für die jeweils betroffene Schädelnaht typisches klinisches Erscheinungsbild. Die Standarddiagnostik umfasst die klinische Untersuchung mit zephalometrischen Messungen (analog, 3-D-photometrisch) und eine Sonographie der Schädelnähte. Der Einsatz von ionisierender Strahlung innerhalb der Routinediagnostik sollte ebenso wie die MRT-Untersuchung in Sedierung vermieden und nur in Ausnahmefällen in Betracht gezogen werden. Für die operative Korrektur des Neuro- und Viszerokraniums stehen an das Alter der Patienten angepasste, standardisierte Verfahren mit guten, vorhersagbaren Ergebnissen zur Verfügung.

Abstract

Isolated single-suture craniosynostosis, which derives from a premature fusion of one of the cranial sutures, is the most common form of craniosynostosis with a prevalence of approximately 1 in 2,500 live births. As the characteristic clinical manifestations of single-suture craniosynostosis are usually sufficient for an accurate diagnosis and with respect to the biological effects of ionizing radiation and risks of sedation especially in infants, preoperative imaging should be reduced to a minimum and should be reserved for complex craniofacial anomalies. Each deformity requires a specific surgical procedure tailored to the individual case. Although the appropriate timing or method of surgical treatment is still controversially discussed, it is widely accepted that surgery before the age of 1 year results in minimal impairment of brain development and optimal bone regeneration. The objective of this treatment is to induce normal brain development, to prevent increased intracranial pressure and to achieve an acceptable cranial morphology.

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Danksagung

In dankbarer Erinnerung Herrn Prof. Dr. Dr. Joachim Mühling (10.04.1948 bis 02.05.2009), Ordinarius für Mund-, Kiefer- und Gesichtschirurgie am Universitätsklinikum Heidelberg von 1991 bis 2009, – dem Wegbereiter der kraniofazialen Chirurgie in Deutschland – gewidmet.

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Authors and Affiliations

  1. Klinik und Poliklinik für Mund-, Kiefer- und Gesichtschirurgie, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Deutschland

    C. Freudlsperger, J. Hoffmann &  M. Engel

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  1. C. Freudlsperger
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  2. J. Hoffmann
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  3. M. Engel
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Correspondence to C. Freudlsperger or M. Engel.

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Interessenkonflikt

C. Freudlsperger, J. Hoffmann und M. Engel geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben. Im Falle von nicht mündigen Patienten liegt die Einwilligung eines Erziehungsberechtigen oder des gesetzlich bestellten Betreuers vor.

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Dieser Beitrag ist eine aktualisierte und neu bearbeitete Fassung des Beitrags: Freudlsperger C, Castrillón-Oberndorfer G, Hoffmann J, Engel M (2013) Isolierte, nichtsyndromale Kraniosynostosen. MKG-Chirurg 6:301–313. https://doi.org/10.1007/s12285-013-0364-9

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Freudlsperger, C., Hoffmann, J. & Engel, M. Isolierte, nichtsyndromale Kraniosynostosen. MKG-Chirurg 11 (Suppl 1), 15–22 (2018). https://doi.org/10.1007/s12285-018-0154-5

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