pediatrics
Osteochondrodysplasias (Osteochondrodysplastic Dwarfism)
Treatment of Osteochondrodysplasias
Sometimes surgical limb-lengthening, surgical correction of leg bowing, or joint replacement (a C-type natriuretic peptide analog) is available and has shown encouraging results in studies ( Treatment reference ) in facilitating bone growth. An increase in adult height may be achieved by surgical limb-lengthening. Bowlegs can be corrected surgically. In achondroplasia and other nonlethal osteochondrodysplasias, surgery (eg, hip replacement) can help improve joint function. Hypoplasia of the odontoid process can predispose to subluxation of the first and second cervical vertebrae and compression of the spinal cord. Therefore, the odontoid process should be evaluated preoperatively and, if it is abnormal, the patient’s head should be carefully supported when hyperextended for endotracheal intubation during anesthesia. Because the inheritance pattern and gene mutations in most types are known, genetic counseling can be effective. Organizations such asLittle People of Americaprovide resources for affected people and act as advocates on their behalf. Similar societies are active in other countries. 1.Savarirayan R, Irving M, Bacino CA, et al: C-type natriuretic peptide analogue therapy in children with achondroplasia.N Engl J Med381(1):25–35, 2019. doi: 10.1056/NEJMoa1813446 