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.

Objective Our objective was to determine the neurodevelopmental outcome at 18–24 months’ of corrected age (CA) in preterm infants with severe intraventricular haemorrhage (IVH). Methods This was a retrospective cohort study of all preterm infants who were <37 weeks’ gestation, had Grade 3–4 IVH. A comprehensive assessment including hearing, vision, neurological and developmental evaluation with Bayley Scales of Infant Development, Second edition (BSID II) was performed by the experienced researchers at 18 to 24 months’ CA. Results A total of 138 were diagnosed as severe IVH (Grade 3–4). Median Apgar score (p < 0.01) and resuscitation at birth (p < 0.01) were significantly different for group 1, group 2 and group 3. The use of catheterization, need for mechanical ventilation, need for phototherapy, retinopathy of premature and bronchopulmonary dysplasia were significantly higher in-group 1 compared to group 2 and 3 (p < 0.001, p < 0.001, p < 0.01, p < 0.01 and p = 0.014 respectively). The duration of hospitalisation and mortality rates consistent with the degree of prematurity and were significantly higher in-group 1 compared to group 2 and 3 (p = 0.03 and p = 0.01). Among the long-term outcomes; the rates of CP and NDI did not differ between the groups (p = 0.68 and p = 0.068). Conclusion Our results demonstrated that long-term outcomes of preterm infants did not differ between the groups classified according to the birth weight at 2 years of age. This has leaded to the conclusion that severe IVH is alone represents a significant risk factor for poor neurodevelopmental outcome in this already high-risk population.