Aplasia cutis congenita

Zusammenfassung

Hintergrund: Beim Erkrankungsbild der Aplasia cutis congenita handelt es sich um eine seltene kongenital bedingte Anlagestörung der Haut. Diese manifestiert sich zumeist an der Kopfhaut, jedoch können auch tiefer liegende Gewebsschichten wie Knochen oder Dura mater betroffen sein. Blutungen aus dem Sinus sagittalis sowie Infektionen stellen die häufigsten Komplikationen dar. Es existieren zahlreiche Fallberichte, jedoch besteht bisher kein eindeutiger Konsens über das therapeutische Management, welches sowohl konservative als auch chirurgische Maßnahmen beinhaltet.

Methoden: Wir berichten über 3 Neugeborene mit Aplasia cutis congenita mit isolierter Hautmanifestation ohne Beteiligung tieferliegender Gewebsschichten. In allen 3 Fällen wurden die Patienten konservativ mit Fettgaze als Wundauflage behandelt und engmaschig kontrolliert.

Ergebnisse: Bei allen 3 Neugeborenen konnte innerhalb weniger Monate eine komplikationslose Abheilung sämtlicher Hautdefekte erzielt werden.

Schlussfolgerungen: Die konservative Therapie mit Fettgaze stellt eine verlässliche therapeutische Option bei isolierter Hautmanifestation dar.

Abstract

Background: Aplasia cutis congenita is a rare congenital disorder characterised by the absence of skin. It mostly affects the scalp, but may also involve deeper tissue layers such as bone or dura mater. Bleeding from the sagittal sinus and infections are the most common complications. Numerous case reports have been published, but there is no consensus on therapeutic management, which includes both conservative and surgical treatment.

Methods: We report on 3 newborns with aplasia cutis congenita solely affecting the skin without involvement of deeper tissue layers. All 3 patients were treated conservatively with fatty gauze being used as a wound dressing. The patients were monitored in tight intervals.

Results: In all 3 newborns, the skin defects healed within a few months without any complications.

Conclusion: Conservative treatment with fatty gauze represents a reliable therapeutic option in case the disease solely affects the skin.

Schlüsselwörter

Aplasia cutis congenita - angeborene Kopf-Hals-Fehlbildungen - Haut - Adams-Oliver Syndrom

Key words

aplasia cutis congenita - congenital malformations of the head and neck - skin - Adams-Oliver syndrome

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