SIRENOMELIA ASSOCIATED WITH HYPOPLASTIC LEFT HEART IN A NEWBORN
Küçük Resim Yok
Tarih
2017
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Macedonian Acad Sciences Arts
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
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.
Açıklama
Anahtar Kelimeler
Caudal regression syndrome (CRS), Hypoplastic left heart, Sirenomelia
Kaynak
Balkan Journal of Medical Genetics
WoS Q Değeri
Q4
Scopus Q Değeri
Q4
Cilt
20
Sayı
1
