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Journal of Korean Cleft Palate-Craniofacial Association 2004;5(1):61-64.
Surgical Treatment of Choanal Stenosis Associated with Craniodiaphyseal Dysplasia.
Byeng June Jeon, Won Sok Hyon, So Young Lim, Ji Hyuck Lee, Goo Hyun Mun, Sa Ik Bang, Kap Sung Oh
Department of Plastic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. psdoc@samsung.co.kr
Abstract
Craniodiaphyseal dysplasia is a rare genetic disorder of bone due to modelling errors of long bones and skull bones. Facial and cranial thickening and distortion are particularly striking in this form. The genetic understanding of this disorder is unsatisfactory. We present one case of Craniodiaphyseal dysplasia involving facial bone, skull that causes nasal obstruction. This 3-year old female who was diagnosed of craniodiaphyseal dysplasia presented abnormal facial figuring, which is excessively thickened cortical bone resulting in very large craniofacial bony structure. The patient sufferred from respiratory difficulty due to complete obstruction of choana. We performed surgical recanalization of choanal atresia by transpalatal approach and gained good postoperative result.
Keywords: Craniodiaphyseal dysplasia; Choanal atresia
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