J Korean Soc Plast Reconstr Surg.  2009 Jul;36(4):481-484.

Correction of Frontal Bone Defect in Cleidocranial Dysostosis with Porous Polyethylene(Medpor(R)): A Case Report

• Moh JS
• Na YC

Abstract

PURPOSE
Cleidocranial dysostosis is a rare hereditary disorder affecting bones that develop by intramembranous formation. The typical features include excessive growth of transverse diameter of the skull, hypoplastic clavicles, low height and characteristic facial features.
METHODS
A 28-year-old female patient visited by frontal area depression. The diagnosis was performed by computed tomographic study and radiographic imaging. The patient had widely opened anterior fontanelle, partial fused metopic suture, multiple wormian bone and supernumenary impacted teeth. Under the coronal incision, we exposed depressed frontal area and corrected with Medpor block carving. RESULTS: Postoperatively frontoparietal skull was aestheticlly improved and satisfied the patient. CONCLUSION: Authors report a case of cleiodocranial dysostosis who underwent correction of abnormal skull shape by Medpor(R) insertion.

Keyword

Cleidocranial dysostosis; Medpor(R)

MeSH Terms

Adult
Clavicle
Cleidocranial Dysplasia
Cranial Fontanelles
Depression
Dysostoses
Female
Frontal Bone
Humans
Polyethylenes
Skull
Sutures
Tooth, Impacted
Polyethylenes