Fraser's syndrome clinical spectrum and anaesthetic considerations in neonatal period

ABSTRACT

Fraser's syndrome which is a rare autosomal recessive disorder characterised by cryptophthalmos, defect of auricle, genital anomaly and syndactyly was seen in a neonate. This baby had all the findings originally described by Fraser and earlier known as cryptophthalmos syndactyly syndrome. The baby was taken for coloboma repair and then subsequently for syndactyly release after one month. On both occasions, anaesthetists had difficulty in intubating the baby due to laryngeal stenosis which is a rare association of Fraser's syndrome. The ultimate prognosis of such babies is poor due to multisystem involvement and as there is defect not only of eyelid formation but also associated ocular anomaly, the likelihood of achieving adequate visual perception is small