Case Report of Crouzon's Syndrome

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  • Crouzon症候群の一症例について

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Abstract

Crouzon's syndorome is a disease which presents the three symptoms of (1)craniostosis, (2) hypoplasia of the middle face, and (3) exopthalmus. This report presents the systemic and dental findings concerning a 5-year-6-month-old girl with Crouzon's syndorome whom we encountered in our practice.<BR>1) The patient was the first child of a 26-year-old woman and weighed 2,080 g when born during the 34th week of pregnancy.<BR>2) The family included the parents and a younger sister, all of whom where healthy and had no notable abnormalities.<BR>3) The following findings were noted in the patient:<BR>(1) The child was deaf.<BR>(2) No abnormalities were observed in the hair or skin, and, the eyes were slightly protruded, altough otherwise normal.<BR>(3) The middle region of the face was not fully developed, and the patient had a "parrot's beak" nose and short upper lip.<BR>(4) Clinical tests showed a high level of serum phosphorus and alkaline phosphatase, indicating the possibility of osteopathosis. No particular abnormalities were noted in the other blood, urine, and chromosomal tests.<BR>(5) The cephalometoric radiograph showed conical and several fingerlike indentations in the bone. Underdevelopment of the maxilla and anterioinferior development of the mandible were observed. The patient had poor facial development, presenting a long lower face. Deformation of the mandible was also observed.<BR>(6) The patient had 20 primary teeth. Examination of the occlusion showed an anterior open bite, mesial deviation of the mandible in the molar region, and a narrow mandibular arch. The patient had a high palate.<BR>(7) All of the permanent tooth buds up to the second molars were observed in panoramic and dental radiographs, and the calcification age was judged to be 5 years and 4 months.

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