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Angeborene Pylorusagenesie Typ C

Eine sehr seltene Fehlbildung des Gastrointestinaltrakts

Congenital pyloric atresia type c

Rare malformation of the gastrointestinal tract

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Zusammenfassung

Die Pylorusagenesie ist mit einer Prävalenz von 1:100.000 Neugeborenen eine sehr seltene angeborene Fehlbildung des Gastrointestinaltrakts. Im vorliegenden Beitrag wird erstmalig über den seltenen Fall einer Pylorusagenesie Typ C mit Nierenagenesie und Polydaktylie berichtet. Es wird das klinisch-diagnostische und therapeutische Management unter Berücksichtigung der aktuellen Literatur dargestellt. Bei bestehendem Hydramnion war bereits pränatal sonographisch der Verdacht auf eine Stenosierung im Bereich des oberen Magen-Darm-Trakts gegeben. In der postpartal angefertigten Röntgenübersichtsaufnahme des Abdomens zeigte sich eine deutlich vergrößerte Magenblase („single bubble“-Phänomen) ohne nachfolgende Luftfüllung im Dünndarmbereich. Das chirurgische Vorgehen bestand in einer direkten Antroduodenostomie am 2. Lebenstag. Der postoperative Verlauf gestaltete sich unkompliziert.

Abstract

Congenital pyloric atresia is an extremely rare congenital malformation of the gastrointestinal tract with a prevalence of 1 per 100,000 newborns. The present paper reports on a child with a congenital pyloric atresia type C with renal agenesis und polydactyly. The clinical diagnostic and therapeutic management is outlined considering the few relevant medical scientific references. A stenosis of the upper gastrointestinal tract was suspected during prenatal ultrasound screening in addition to an already existing hydramnios. The X-ray of the abdomen taken postpartum showed a clearly enlarged gastric bubble (single-bubble phenomenon) without accumulation of air in the small intestine. The intraoperative result confirmed pyloric atresia type C (pyloric atresia). A direct antroduodenostomy was performed surgically on the second day of life. No problems appeared during the postoperative course.

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Authors and Affiliations

  1. Fehlbildungsmonitoring Sachsen-Anhalt, Medizinische Fakultät, Otto-von-Guericke-Universität Magdeburg, Leipziger Straße 44, 39120, Magdeburg, Deutschland

    J. Hoyer-Schuschke & S. Pötzsch

  2. Arbeitsbereich Kinderchirurgie, Klinik für Allgemein-, Viszeral- und Gefäßchirurgie, Universitätsklinikum, Otto-von-Guericke-Universität Magdeburg, Magdeburg, Deutschland

    H-J Haß & H. Krause

  3. Universitätsfrauenklinik, Otto-von-Guericke-Universität Magdeburg, Magdeburg, Deutschland

    C. Gerloff

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  1. J. Hoyer-Schuschke
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  2. H-J Haß
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  3. S. Pötzsch
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  4. C. Gerloff
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  5. H. Krause
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Correspondence to J. Hoyer-Schuschke.

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Hoyer-Schuschke, J., Haß, HJ., Pötzsch, S. et al. Angeborene Pylorusagenesie Typ C. Monatsschr Kinderheilkd 160, 579–581 (2012). https://doi.org/10.1007/s00112-011-2576-2

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  • DOI: https://doi.org/10.1007/s00112-011-2576-2

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