Interdisziplinäre Behandlung von Patienten mit angeborenen Fehlbildungen – Zusammenhang zwischen Dysfunktion und Dysgnathie

Zusammenfassung

Der vorliegende Artikel erklärt den Zusammenhang von Dysfunktion und Dysgnathie am Beispiel von kranio- und orofazialen Fehlbildungen. Einerseits verhindert eine gestörte Form eine normale Funktion: bei Patienten mit Lippen-Kiefer-Gaumenspalte beeinträchtigt die gestörte Morphologie Aussprache, Gehör, Zahn-/Kieferstellung, Ästhetik und soziales Verhalten. Die Rekonstruktion der gespaltenen Strukturen, d. h. der Form, steht im Zentrum der Rehabilitation und stellt eine wichtige Voraussetzung für das Erlangen einer ungestörten Funktion dar. Andererseits verschlechtert eine gestörte Funktion die Form: die Einlagerung der Zunge in den Spaltbereich verhindert ein sich Annähern der Spaltsegmente. Eine Gaumenplatte normalisiert die Zungenfunktion und durch gezieltes Einschleifen der Apparatur wird das Wachstum der Spaltsegmente gesteuert. Ausgeprägte Atemstörungen können bei Neugeborenen mit Pierre-Robin-Sequenz lebensbedrohlich sein. Eine modifizierte Gaumenplatte verhindert die Glossoptose und sichert den Atemweg; über die Normalisierung der Funktion wird, bei angeborener mandibulärer Retrognathie, ein koordinierter Ablauf von Ober- und Unterkieferwachstum ermöglicht. Das Zusammenspiel zwischen Form und Funktion zeigt sich in der Verstärkung der Dysmorphie durch Dysfunktion, aber auch in der wechselseitigen Verbesserung während der sehr frühen Therapie. Übertragen auf Patienten ohne angeborene Fehlbildung erklärt sich, weshalb gewisse Dysgnathien früh behandelt werden sollten.

Abstract

This article deals with the interaction of dysfunction and dysgnathia using the example of craniofacial and orofacial malformations. First, it lays out how abnormal form impedes a proper function: In patients with cleft lip and palate, the malformation negatively affects pronunciation, hearing, tooth and jaw position, aesthetics, and social behavior. To regain proper function, the form needs correction in terms of surgical reconstruction of the cleft tissues in the first year of life. Secondly, it shows that abnormal function can also impede a proper form: In patients with cleft lip and palate, the tongue penetrates the cleft area, preventing the cleft segments from converging. A palatal plate helps to normalize the function and steers the growth of the cleft segments. In a similar vein, in newborns with Pierre Robin sequence, a therapy with a pre-epiglottic baton plate can prevent life-threatening respiratory disorders. Born with a mandibular retrognathia, these patients profit from the recovered function providing a coordinated physiological upper and lower jaw growth. To summarize, the interaction between form and function becomes manifest in the reinforcement of the dysmorphia through dysfunction, but also in the reciprocal improvement during very early therapy. Applied to patients without congenital malformations, it explains why certain forms of dysgnathia should be treated at a very early stage.

Schlüsselwörter

kranio-/orofaziale Dysmorphien - Lippen-Kiefer-Gaumenspalten - Pierre-Robin-Sequenz - (prächirurgische) kieferorthopädische Frühbehandlung

Key words

cranio-/orofacial malformations - cleft lip - cleft palate - Pierre Robin sequence - presurgical infant orthopedics

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