گزارش يك مورد سيرنومليا، سندرم پري دريائي

A Rare case Report of Sirenomelia, the Mermaid Syndrome

ﺳﻴﺮﻧﻮﻣﻠﻴﺎ ﻳﺎ ﺳﻨﺪﺭﻡ ﭘﺮﻱ ﺩﺭﻳﺎﺋﻲ ﻳﮏ ﻧﺎﻫﻨﺠﺎﺭﻱ ﻣﺎﺩﺭﺯﺍﺩﻱ ﺑﺴﻴﺎﺭ ﻧﺎﺩﺭ ﻭ ﮐﺸﻨﺪﻩ ﺍﺳﺖ. ﺷﻴﻮﻉ ﺁﻥ 1 ﻣﻮﺭﺩ ﺩﺭ ﻫﺮ 60/000 ﺗﺎ 100/000 ﺣﺎﻣﻠﮕﻲ ﻣﻲ ﺑﺎﺷﺪ. ﺳﻴﺮﻧﻮﻣﻠﻴﺎ ﺑﺎ ﭼﺴﺒﻨﺪﮔﻲ ﮐﺎﻣﻞ ﺍﻧﺪﺍﻡ ﺗﺤﺘﺎﻧﻲ، ﻋﺪﻡ ﻭﺟﻮﺩ ﮊﻧﻴﺘﺎﻝ ﺧﺎﺭﺟﻲ ﻭ ﻋﺪﻡ ﻭﺟﻮﺩ ﮐﻠﻴﻪ ﻫﺎ ﻭ ﻧﺎﻫﻨﺠﺎﺭﻱ ﺁﻧﻮﺭﮐﺘﺎﻝ ﻣﺸﺨﺺ ﻣﻲ ﺷﻮﻧﺪ. ﺳﻴﺮﻧﻮﻣﻠﻴﺎ ﺷﺪﻳﺪﺗﺮﻳﻦ ﻭﺿﻌﻴﺖ ﻧﺎﻫﻨﺠﺎﺭﻱ ﺳﻨﺪﺭﻡ ﺭﮔﺮﺳﻴﻮﻥ ﮐﻮﺩﺍﻝ ﺍﺳﺖ. ﮔﺮﭼﻪ ﺗﺸﺨﻴﺺ ﭘﺮﻩ ﻧﺎﺗﺎﻝ ﺣﺘﻲ ﺩﺭ ﺍﻭﺍﺧﺮ ﺳﻪ ﻣﺎﻫﻪ ﺍﻭﻝ ﮔﺰﺍﺭﺵ ﺷﺪﻩ ﺍﺳﺖ ﻣﺎﻳﮏ ﻣﻮﺭﺩ ﺗﻮﻟﺪ ﻧﻮﺯﺍﺩ 33 ﻫﻔﺘﻪ ﺍﺯ ﻳﮏ ﻣﺎﺩﺭ 19 ﺳﺎﻟﻪ ﺭﺍ ﻣﻌﺮﻓﻲ ﻣﻲ ﮐﻨﻴﻢ ﮐﻪ ﺑﺎ ﺍﻧﺪﺍﻡ ﺗﺤﺘﺎﻧﻲ ﻭﺍﺣﺪ، ﻋﺪﻡ ﻭﺟﻮﺩ ﺩﺳﺘﮕﺎﻩ ﺍﺩﺭﺍﺭﻱ ﺗﻨﺎﺳﻠﻲ ﺧﺎﺭﺟﻲ ﻭ ﺁﺗﺮﺯﻱ ﺁﻧﻮﺭﮐﺘﺎﻝ ﻣﺘﻮﻟﺪ ﺷﺪ ﺑﺎ ﺍﻧﺠﺎﻡ Baby Gram ﻭ ﺍﺗﻮﭘﺴﻲ ﺳﺎﻳﺮ ﻧﺎﻫﻨﺠﺎﺭﻱ ﻫﺎ ﺭﺍ ﻣﻮﺭﺩ ﺑﺮﺭﺳﻲ ﻗﺮﺍﺭ ﺩﺍﺩﻳﻢ. ﺗﺸﺨﻴﺺ ﺯﻭﺩﺭﺱ ﺍﻳﻦ ﺳﻨﺪﺭﻡ ﺩﺭ ﺳﻪ ﻣﺎﻫﻪ ﺍﻭﻝ ﻳﺎ ﺩﻭﻡ ﺑﺎﺭﺩﺍﺭﻱ ﻭ ﺧﺎﺗﻤﻪ ﺯﻭﺩﺭﺱ ﺣﺎﻣﻠﮕﻲ ﺳﺒﺐ ﮐﺎﻫﺶ ﺗﺮﻭﻣﺎﻱ ﻧﺎﺷﻲ ﺍﺯ ﺧﺘﻢ ﺣﺎﻣﻠﮕﻲ ﻳﺎﺯﺍﻳﻤﺎﻥ ﺩﺭ ﻣﺎﻩ ﻫﺎﻱ ﺑﺎﻻﺗﺮ ﺧﻮﺍﻫﺪ ﺷﺪ.

Sirenomelia, the Mermaid syndrome is an extremely rare and lethal congenital anomaly with an incidence of one in 60.000 to 100.000 pregnancies. It is characterized by complete fusion of the lower limbs associated with external genitalia absence, renal agenesis and anorectal defects. Sirenomelia represents the most severe of caudal regression syndrome. Although prenatal diagnosis has been reported even as early as the first trimester, we present an infant delivered from a 19 - year- old primigravida at 33 week’s gestation, with a dysmorphic lower limb, agenesis of external urogenital and anorectol atresia. X- Ray (baby gram) and autopsy revealed other anomalies in the infant. The early detection of this syndrome during the first or the second trimester, helps us in the diagnosis of this rare anomaly and in terminating the pregnancy in a less traumatic period.