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[Cantrell's syndrome].

Z Zachariou1, R Daum, H Roth

  • 1Abteilung für Kinderchirurgie des Chirurgischen Zentrums der Universität Heidelberg.

Zeitschrift Fur Kinderchirurgie : Organ Der Deutschen, Der Schweizerischen Und Der Osterreichischen Gesellschaft Fur Kinderchirurgie = Surgery in Infancy and Childhood
|August 1, 1987
PubMed
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Congenital diaphragmatic hernia, often fatal with other defects, may indicate a specific syndrome. This review and case series explore Cantrell's syndrome, a rare condition with multiple congenital abnormalities.

Area of Science:

  • Embryology
  • Pediatric Surgery
  • Medical Genetics

Background:

  • Congenital diaphragmatic hernia (CDH) is a severe condition with high mortality, particularly when associated with other congenital anomalies.
  • The recurrence of specific congenital defects suggests an underlying syndromic cause, possibly related to embryonic mesodermal development.

Purpose of the Study:

  • To summarize clinical data on Cantrell's syndrome.
  • To report three cases of Cantrell's syndrome with varying presentations.
  • To highlight the spectrum of congenital defects associated with this syndrome.

Main Methods:

  • Literature review of clinical data on Cantrell's syndrome.
  • Case series reporting three patients with Cantrell's syndrome.
  • Analysis of the pattern of congenital defects in the reported cases.

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Main Results:

  • Cantrell's syndrome involves a specific constellation of defects: supraumbilical abdominal wall defects, lower sternal defects, anterior diaphragmatic deficiency, diaphragmatic pericardial defects, and intracardiac defects.
  • The three reported cases exhibited different combinations of these anomalies.
  • The findings support the hypothesis of a specific syndrome affecting organs derived from the embryonic mesoderm.

Conclusions:

  • Cantrell's syndrome is a complex congenital condition with a characteristic set of defects.
  • Understanding the spectrum of anomalies is crucial for diagnosis and management.
  • Further research into the embryological origins may elucidate the pathogenesis of this syndrome.