Orvosi Hetilap
Volume/Issue:
Volume 150: Issue 45
Pentalogy of Cantrell: six new cases
Authors: Barbara Pete 1 , Júlia Hajdú 1 , Ágnes Harmath 1 , Zsolt Csapó 1 , Csaba Papp 1 , Zsanett Szigeti 1 , and János Rigó jr. 1
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  • 1 Semmelweis Egyetem, Általános Orvostudományi Kar I. Szülészeti és Nőgyógyászati Klinika Budapest Baross u. 27. 1088
DOI:
https://doi.org/10.1556/oh.2009.28744
Page Count:
2068–2073
Publication Date:
01 Nov 2009
Online Publication Date:
27 Oct 2009
Article Category:
Research Article
Open access

A praenatalis ultrahangvizsgálatok elterjedése óta számos próbálkozás történt a ritka Cantrell-pentalógia (elülső hasfali defektus, sternumdefektus, elülső diaphragmahernia, pericardiumhiány és szívfejlődési rendellenesség) intrauterin felismerésére. Célkitűzés és módszerek: Intézetünkben 1991. január és 2009. június között diagnosztizált Cantrell-pentalógiás magzatok praenatalis ultrahangvizsgálati eredményeit hasonlítottuk össze a boncolás során leírt fejlődési rendellenességeikkel. Saját és a nemzetközi szakirodalomban található Cantrell-pentalógiás magzatok prae- és postnatalis leletei alapján vizsgáltuk a pentalógia részét képező malformatiók és ectopia cordis előfordulási gyakoriságát, és ezek praenatalis felismerési arányát. Eredmények: A fenti időszakban 6 Cantrell-pentalógiás magzatot diagnosztizáltunk. Praenatalisan 3 esetben multiplex, 3 esetben izolált malformatiót észleltünk. A boncolás sternumdefektust minden magzatban igazolt. Összesen 49 Cantrell-pentalógiás eset adatainak elemzése azt mutatja, hogy a praenatalisan leggyakrabban felismert ectopia cordis (91%, 31/34) és hasfali defektus (83%, 38/46) mellett (prevalenciájuk 69%, 34/49 és 94%, 46/49) a pentalógiás magzatok jelentős részében sternumdefektus (80%, 39/49) és elülső diaphragmahernia (73%, 36/49) is megtalálható. Következtetések: A Cantrell-pentalógia részét képező, de izoláltnak vélt malformatio praenatalis észlelésekor érdemes keresni a tünetegyüttes további összetevőit a pontos diagnózis intrauterin felállítása érdekében.

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Cover Orvosi Hetilap
Orvosi Hetilap
Print ISSN:
0030-6002
Online ISSN:
1788-6120
Keywords:
Cantrell-pentalógia; ectopia cordis; sternumdefektus; praenatalis diagnózis; Pentalogy of Cantrell; ectopia cordis; sternal defect; prenatal diagnosis

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