Cystic Fibrosis as a Rare Cause of Apple Peel Syndrome

Abstract

Apple peel atresia is a special form of intestinal atresia with absence of mesentery. It is most likely due to an intrauterine intestinal vascular accident and has been described with other anomalies. Meconium ileus can compromise blood supply causing intestinal atresia. Therefore, cystic fibrosis needs to be ruled out in apple peel syndrome.

Zusammenfassung

Die ‚Apple-Peel‘-Atresie ist eine Sonderform der Dünndarmatresie mit einer Mesenteriallücke. Sie wird am ehesten durch einen intrauterinen intestinalen Gefäßverschluss verursacht und ist beschrieben worden mit weiteren Anomalien. Ein Mekoniumileus kann die Blutversorgung beeinträchtigen, welcher eine Dünndarmatresie verursachen kann. Deshalb sollte beim Vorliegen eines ‚Apple-Peel‘-Syndroms eine Mukoviszidose ausgeschlossen werden.

Key words

absence of mesentery - F508del mutation - intestinal atresia - meconium ileus - microcephaly - ocular anomalies

Schlüsselwörter

fehlendes Mesenterium - F508del-Mutation - Dünndarmatresie - Mekoniumileus - Mikrozephalie - Augenerkrankung

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