Case report
SIRENOMELIA AND CAUDAL REGRESSION SYNDROME – THE RARE CONGENITAL ANOMALIES
Žana Žegarac
; Klinika za ženske bolesti i porode, KB »Merkur«, Ivana Zajca 19, 10000 Zagreb
Željko Duić
; Klinika za ženske bolesti i porode, KB »Merkur«, Ivana Zajca 19, 10000 Zagreb
Jasenka Zmijanac Partl
; Klinika za ženske bolesti i porode, KB »Merkur«, Ivana Zajca 19, 10000 Zagreb
Sandra Stasenko
; Klinika za ženske bolesti i porode, KB »Merkur«, Ivana Zajca 19, 10000 Zagreb
Danka Mirić-Tešanić
; Klinika za ženske bolesti i porode, KB »Merkur«, Ivana Zajca 19, 10000 Zagreb
Ivan Fistonić
; Privatna ginekološka ordinacija »Ivan Fistonić« u Zagrebu
Abstract
Sirenomelia and caudal regression syndrome present a rare congenital malformation. Although sirenomelia was thought to be severe type of the caudal regression syndrome in the previous years, it is known today that the sirenomelia and the caudal regression syndrome are, in fact, two mutually diverse states. Both states appear during the early embryogenic development of the fetus due to the different pathogenic mechanisms. The sirenomelia is considered to appear due to the vascular steal theory, whereas the caudal regression syndrome appears due to the disorder of the mesoderm differentiation. The sirenomelia and caudal regression syndrome come into existence in the early embryologic development of the fetus during the fourth postconceptional week at the period of gastrulation. The two reported case studies show the distinction between the pathogenesis, as well as the associated anomalies based upon the antenatally diagnosed malformations. The antenatal diagnostics and prognosis are discussed, especially emphasizing the antenatal screening of the malformations which enables the early diagnosis, as well as the indications for the early and less traumatic pregnancy termination.
Keywords
sirenomelia; caudal regresion syndrome; congenital malformations
Hrčak ID:
70299
URI
Publication date:
1.3.2011.
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