Sirenomelia: a review of embryogenic theories and discussion of the differences from caudal regression syndrome

Abstract

Objective: To discuss the pathological features of sirenomelia in the light of our 10 cases and review the current theories.

Methods: We identified 10 patients with sirenomelia from our hospital database. All clinical details and the autopsy features of 10 cases were noted.

Results: Of the 10 children with sirenomelia seven had bilateral renal agenesis, three had bladder agenesis and one had a renal hypoplasia. Single umbilical artery was found in 60% of children with sirenomelia. External genitalia was ambiguous in seven of 10 patients.

Conclusions: Even though the etiology of caudal regression syndrome (CRS) and sirenomelia remains unknown we tend to believe that sirenomelia and CRS might be different entities.

• Caudal regression syndrome
• single umbilical artery
• sirenomelia

Declaration of Interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.