CODAS syndrome
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(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