A Case of Craniofacial Dysostosis (Crouzon’s Disease) |
Sung Soo Lee1, Jong Soo Kim1, Won Chull Kim1, Dong Shik Chin1, Kwang Kil Lee2, Chi Whan Kim2, Sung Ok Choi1 |
1Department of Pediatrics, Yonsei University College of Medicine Seoul, Korea 2Department of Pathology, Yonsei University College of Medicine Seoul, Korea 3Department of Radiology, Yonsei University College of Medicine Seoul, Korea |
Craniofacial Dysostosis(Crouzon 民病) 1 例 |
李晟守1, 金宗洙1, 金元澈1, 陳東植1, 李廣吉2, 金智煥2, 崔成旭1 |
1延世大學敎 醫科大學 小兒科學敎室 2延世大學敎 醫科大學 病理學敎室 3延世大學敎 醫科大學 放射線科學敎室 |
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Abstract |
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 day 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
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