Literature DB >> 8719727

Sirenomelia and situs inversus: case report and review of the literature.

B Langer1, C Stoll, R Nicolau, B Gasser, G Schlaeder.   

Abstract

We report a case of sirenomelia of the symelia dipus type which is associated with complete situs inversus. Sirenomelia is difficult to diagnose prenatally because of a frequently associated oligohydramnios. Symelia dipus type adds to the difficulty of the diagnosis, since the bones of the thighs and legs are fully formed. In fact, a close examination of the cases of sirenomelia previously reported in the literature shows that a prenatal diagnosis was achieved only when the oligohydramnios was no more than moderate and/or the associated malformation was of apus or unipus type. We propose a systematic amnioinfusion when one suspects symelia unipus type. Finally, the pathogenesis of sirenomelia is discussed, as the presence of a complete situs inversus in the case reported here could illuminate the debate concerning the origin of the malformation.

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Mesh:

Year:  1996        PMID: 8719727     DOI: 10.1159/000264284

Source DB:  PubMed          Journal:  Fetal Diagn Ther        ISSN: 1015-3837            Impact factor:   2.587


  4 in total

1.  Cebocephaly, alobar holoprosencephaly, spina bifida, and sirenomelia in a stillbirth.

Authors:  C P Chen; S L Shih; F F Liu; S W Jan
Journal:  J Med Genet       Date:  1997-03       Impact factor: 6.318

Review 2.  Sirenomelia: an epidemiologic study in a large dataset from the International Clearinghouse of Birth Defects Surveillance and Research, and literature review.

Authors:  Iêda M Orioli; Emmanuelle Amar; Jazmin Arteaga-Vazquez; Marian K Bakker; Sebastiano Bianca; Lorenzo D Botto; Maurizio Clementi; Adolfo Correa; Melinda Csaky-Szunyogh; Emanuele Leoncini; Zhu Li; Jorge S López-Camelo; R Brian Lowry; Lisa Marengo; María-Luisa Martínez-Frías; Pierpaolo Mastroiacovo; Margery Morgan; Anna Pierini; Annukka Ritvanen; Gioacchino Scarano; Elena Szabova; Eduardo E Castilla
Journal:  Am J Med Genet C Semin Med Genet       Date:  2011-10-14       Impact factor: 3.908

3.  Embryonic gut anomalies in a mouse model of retinoic Acid-induced caudal regression syndrome: delayed gut looping, rudimentary cecum, and anorectal anomalies.

Authors:  J E Pitera; V V Smith; A S Woolf; P J Milla
Journal:  Am J Pathol       Date:  2001-12       Impact factor: 4.307

4.  Mermaid syndrome: virtually no hope for survival.

Authors:  Nicolas Lutz; Blaise Julien Meyrat; Jean-Pierre Guignard; Judith Hohlfeld
Journal:  Pediatr Surg Int       Date:  2004-06-19       Impact factor: 1.827

  4 in total

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