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NJBMS - Volume 3, Issue 4, April - June 2013

Pages: 301-305

SIRENOMELIA SEQUENCE –A CASE REPORT

Author: SARITHA .S, SUPRIYA .G, BHEEMESH, PRAVEEN KUMAR M

Category: Anatomy

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Abstract:

Sirenomelia sequence (Symelia) or “Mermaid syndrome” is a rare lethal congenital anomaly characterized by fusion, rotation, hypotrophy or atrophy of the lower limbs and severe urogenital abnormalities (usually absence of bladder and agenesis or dysgenesia of kidneys) leading to oligohydramnios. It is also always associated with agenesis or hypoplasia of diverse organs. It is a mermaid syndrome, a fact that is named so because the two fused lower limbs look like a mermaid's tail. The infants resemble the mythical siren. It occurs as a primary defect of blastogenesis affecting multiple midline primordia during the final stages of gastrulation at the caudal eminence. This leads to insufficient migration and differentiation of mesoderm which is responsible for the array of defects in the caudal region. This rare malformation is incompatible with life. These infants usually do not survive for more than 24 hours. Very few survive even after surgery. Only four known cases of children with the affliction are reported to be alive in the world today. The aim of the study is to present a rare congenital malformation. Here we present such an interesting case of Sirenomelia. A 27 year old primi at 24 weeks of gestation was admitted to the hospital. Prenatal ultrasound demonstrated severe oligohydramnios and fusion of the lower limbs suggesting the diagnosis of Sirenomelia. Pregnancy was terminated and the fetus was submitted for detailed autopsy. The findings and review of the literature are presented in this report.

Keywords: Sirenomelia, teratogenesis, caudal regression, congenital malformations, vascular steal theory.

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Editor in Chief of NJBMS Dr.Deepti Shastri, Professor and Head, Department of Anatomy, Academic Co-ordinator for Pre and Paraclinical Sciences, VMKV Medical College & Hospital, Salem.


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