Abstract:
Sirenomelia, also known as “mermaid malformation/
syndrome,” is a rare, serious congenital anomaly characterized
by variable degrees of fusion of the lower limbs and
associated with severe malformations of vertebral, genitourinary,
cardiovascular system and single umbilical artery.
The first pregnancy of a 25-year-old woman resulted in one
twin born by Cesarian section at 32 weeks’ gestation, who
was referred to our hospital with cyanosis, a congenital
anomaly and respiratory distress. On physical examination,
there was no urogenital region and anal fissure and
gender was indeterminate. The arms were in adduction and
wrist in flexion position with four fingers on the right hand
and two fingers on the left hand. There was a single lower
extremity with a webbed single foot and two toes consistent
with sirenomelia type IV radiologically. Abdominal
ultrasonography showed urogenital system agenesis and
echocardiography detected hypoplastic left heart. However,
the patient died 4 hours after birth. The other twin was followed
for 1 week for nutrition and respiratory support and
was then discharged without any problems.
