OSTEOPETROSIS Tarda : CASE REPORT

,; ,

Journal of the Korean Academy of Pediatric Dentistry 1999;26(3):473-478.
Published online August 31, 1999.

OSTEOPETROSIS Tarda : CASE REPORT

만발형 골화석증에 대한 증례보고

권종필, 양규호

전남대학교 치과대학 소아치과학교실

Abstract

Osteopetrosis is an uncommon hereditary bone condition characterized by a generalized symmetric increase in skeletal density and abnormalities of bone resorption remodeling. In 1904, the first case of generalized sclerosis of the skeleton was reported by Albers-Schonberg. Osteopetrosis is generally divided into two main type. The infantile(malignant, congenita) type is the most severe form of the disease; It is characterized by skeletal and hematologic abnormalities. The adult(benign, tarda) type which is usually diagnosed in the third or fourth decade of life is limited predominantly to skeletal anomalies and it carries a more favorable prognosis. The recently recognized intermediate form with its mild and variable clinical recessive trait. There is no reported gender or racial predilection. The characteristic feature of osteopetrosis which is an abscence of physiologic bone resorption results in accumulation of bone mass and mainfests skeletal disturbance. Dental finding of osteopetrosis includes delayed eruption, congenitally absent teeth, unerupted and malformed teeth, and enamel hypoplasia. Our report involves a patient with a chief complaint of tooth mobility and delayed eruption. After clinical and radiologic examination, this patient was referred to dept. of pediatrics under the suspicion of osteopetrosis and it was confirmed.

Key Words: Osteopetrosis, Bone resorption, Delayed eruption