The Use of Distraction Osteogenesis in the Treatment of Rickets-Associated Craniosynostosis

Background: Craniosynostosis has a known association with rickets. Because of abnormal bone development and a tendency for patients with rickets to present at an older age than most patients with craniosynostosis, repair may be complicated by inadequate cranial expansion and greater risk of sutural re-fusion. We present 2 cases of rickets-associated sagittal craniosynostosis and show the effectiveness of distraction osteogenesis in the surgical management of this condition.

Case description: Two 3-year-old boys with rickets presented with sagittal synostosis and marked scaphocephalic deformity. Cranial osteotomies were performed followed by placement of 2 internal distractors. The devices were activated daily for 30 days to achieve a target transverse cranial distraction of 30 mm. A subsequent period of 10-12 weeks allowed for adequate bone consolidation. At the time of device removal, the final breadth of distraction and the quality of new bone formed was assessed. Distraction distance in both cases was verified intraoperatively to be 30 mm. Clinical examination confirmed that the distraction gaps were bridged by solid bone. The treatment protocol resulted in a significant improvement in cranial proportion in both patients, which was maintained at long-term follow-up. Estimated blood loss was high in both cases, presumably related to the hyperemic nature of rickets bone.

Conclusions: Distraction osteogenesis promotes bone growth and cranial remodeling in patients with craniosynostosis caused by rickets. The technique allows for continuous incremental expansion of both bone and scalp tissue. We recommend consideration of distraction osteogenesis in the treatment of older children with severe deformity related to craniosynostosis, including those with rickets.