It is probable that there are between 500 and 1 000 persons with achondroplasia in South Africa and it is inevitable that they will seek consultation and care in general and specialized dental practices.
Methods: Ten children with a firm diagnosis of achondroplasia were documented at the Genetic Clinic of Red Cross Children's Hospital, Cape Town, in 2006 for detailed oro-dental assessment and the implementation of ongoing specialized dental management.
Results: The affected children all breathed by mouth because of upper-airway obstruction. Short limbs, stunted stature and occasional spinal malalignment made it difficult for the children to sit comfortably in a conventional dental chair. Upper airway obstruction due to depression of the nasal bridge, variable choanal atresia and enlarged adenoids resulted in mouth breathing. A small nasal pharynx and larynx posed additional problems for anaesthesia.
The possible presence of cranio-cervical instability necessitated special precautions in head control during dental intervention.
Conclusion: The large heavy head, the small foramen magnum and the possible abnormality of the cranio-cervical junction pose a potential threat of spinal cord compression. It is likely that this special risk applies to dental procedures, with or without general anaesthesia. In particular, hypoplasia and relative mandibular prognathism were consistent features.