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KMID : 0367419740150100059
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Journal of Korean Pediatric Society
1974 Volume.15 No. 10 p.59 ~ p.63
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A Case of Craniofacial Dysostosis (Crouzon´s Disease)
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李晟守/Lee, Sung Soo
金宗洙/金元植/陳東植/李廣吉/金智煥/崔成旭/Kim, Jong Soo/Kim, Won Chull/Chin, Dong Shik/Lee, Kwang Kil/Kim, Chi Whan/Choi, Sung Ok
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Abstract
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Craniofacial dysostosis(Crouzon´s disease) is a rare disease and there is no report of craniofacial dystosis in children in Korea.
We experienced a case of Crouzon´s disease in 4 days old male infant with chief complaints of respiratory distress and bilateral exophthalmos since birth.
Physical examination showed craniosynostosis, bilateral exophthalmos, external strabismus, bird-like beaked nose, bilateral choanal atresia, high arched palate, hypoplasia of maxilla, prognathism of mandible and caudal appendage.
On 2nd hospital day patient was died and autopsy was done.
Autopsy findings were as follows;
1) craniosynostosis of sagittal suture
2) bilateral exophthalmos
3) bird-like beaked nose
4) bilateral posterior choanal atresia
5) high arched palate
6) hypoplasia of maxilla and prognathism of mandible
7) overgrowth of cartilage of coccygeal bone
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