Buttiens-Fryns syndrome
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Form of SHSF (acronym for Split Hand Split Feet) malformation with micrognathia. Autosomal dominant transmission of 10q24 microduplication.
Association of:
- severe shortening of the four distal extremities of the limbs (oligodactyly),
- micrognathia,.
- microstomia with or without cleft palate
Anesthetic implications:
difficult intubation, difficult peripheral venous access
References:
Updated: October 2018