Sirenomelia: A Detailed Fetal Autopsy Study.
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Date
2016-10
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Abstract
Sirenomelia also known as mermaid syndrome, characterized by fused lower limbs, is a lethal and rare congenital
abnormality. This is an extreme example of caudal regression syndrome caused by vascular steal of single
umbilical artery. Sirenomelia is found approximately one in 100,000 live births and is usually fatal because of
complications associated with pulmonary hypoplasia, abnormal development of the kidneys and urinary
bladder. Most of the cases are associated with maternal diabetes and single umbilical artery.
We report a case of sirenomelia terminated at 21weeks of gestation due to multiple congenital abnormalities.
Antenatal scan of a25-year old primi revealed a single live fetus of 21 weeks gestation with severe olighydramnios,
non-visualization of kidneys and bladder and lower part of the spine. This pregnancy was terminated and the
specimen sent to the department of anatomy for fetal autopsy. On external examination,a tail like rudimentary
single midline lower limb without foot was noted. There were no external genitalia and anal orifice. Internal
examination revealed hypoplastic lungs, atrial septal and ventricular septal defects, aberrant right subclavian
artery, hypoplastic abdominal aorta distal to the single umbilical artery, complete agenesis of urinary system,
rectal agenesis and rudimentary indeterminate gonads.
We would like to discuss the clinical features, etio- pathogenesis and review of literature of sirenomelia. Early
diagnosis and termination of this lethal congenital anomaly results in minimizing the trauma related to the
termination of pregnancy at advanced gestation.
Description
Keywords
Sirenomelia, Caudal regression syndrome, Vascular steal, Maternal diabetes, Single umbilical artery
Citation
Sirikonda Parimala, Himabindu Nalluri. Sirenomelia: A Detailed Fetal Autopsy Study.International Journal of Anatomy and Research. 2015 Oct-Dec; 3(4): 1669-1674.