CODAS syndrome

[MIM 600 373]

(cerebro-oculo-dento-auriculo-skeletal syndrome)

Acronym for Cerebral-Ocular-Dental-Auricular-Skeletal. 

Extremely rare, most cases have been reported in the Mennonite populations in Pennsylvania. Autosomal recessive transmission. 


Symptoms:

-         psychomotor disorders

-         cataract

-         abnormal dentition: delayed dental eruption, projections of enamel at the level of the cusps

-         malformation of the ears ("wrinkled") with sensorineural hearing loss

-         short stature due to epiphyseal dysplasia; delayed bone age, vertebral and pelvic abnormalities

-         a particular facies: ptosis, slanting eyes,  anteverted nostrils with a median nasal groove

-         sometimes: paralysis of the vocal cords


Anesthetic implications: 

short stature and mental retardation; dorsolumbar column RX before making a neuraxial block; preoperative fibroscopy to check the mobility of the vocal cords.


References : 


Updated: October 2019