Clinical and cephalometric analysis of three cases with pycnodysostosis: case reports
نویسندگان
چکیده
The aim of this article is to present intra- and extra-oral and cephalometric findings of three patients with a rare disease: the pycnodysostosis. Two cases had skeletal Class III malocclusion due to maxillary retrognathia and one had bimaxillary retrusion with Class I relationship. Total circular crossbite, increased gonial angle and vertical facial proportions, deep-narrow palates and retruded upper lip were found in all cases. Maxillary expansion, face mask treatment or/and orthognathic surgery are treatment alternatives, considering the growth and development. Bone fragility and the risk of osteomyelitis after extractions should be considered in such cases before orthodontic treatment and orthognatic surgery.
منابع مشابه
Clinical, radiographic, diagnostic and cephalometric features of pycnodysostosis in comparison with Turkish cephalometric norms: A case report
Pycnodysostosis is a rare genetic disorder and was first described in 1962 by Maroteaux and Lamy. The incidence of this anomaly is estimated to be 1.7 per 1 million births. The principal characteristics of this disorder are short stature, prominent eyes with blue sclera, beaked nose, cranial dysplasia, exposed fontanelles and cranial sutures, clavicular dysplasia, total/partial dysplasia of the...
متن کاملThree Case Reports of Esophageal Tuberculosis
Rare cases of esophageal tuberculosis have been reported. In this report, 3 cases of tuberculosis-resulted ulcer in the esophageal mucosa are to be introduced among 29 cases of gastrointestinal tuberculosis. The first patient was presented with chills and fever and had a mild dysphagia and odynnophagia and a burning sensation behind the sternum. The second case admitted with neck fistula and e...
متن کاملClinical and radiographic features of pycnodysostosis: A case report
Pycnodysostosis is a rare disorder that was first described in 1962; however, it was only in 1996 that the defective gene was discovered, which led to a better understanding of this disease. This study reports and discuss a case of pycnodysostosis. In addition, a search of articles published in PubMed-Medline was performed. The case was a 13-year-old girl who was referred to a private clinic fo...
متن کاملPycnodysostosis with unusual findings: a case report
Pycnodysostosis is a rare clinical entity, first described in 1962 by Maroteaux and Lamy. The disease has also been named Toulouse-Lautrec syndrome, after the French artist Henri de Toulouse-Lautrec, who (it has been surmised) suffered from the disease. In 1996, the defective gene responsible for Pycnodysostosis was located, offering accurate diagnosis, carrier testing and a more thorough under...
متن کاملClinical and Radiographic Features of Pycnodysostosis with Emphasis on Dentofacial Problems
Pycnodysostosis (PDO) is a rare genetic disorder characterized by cathepsin K deficiency which plays an important role in bone metabolism. Among clinical features of this disease which are mainly caused by altered bone remodeling are craniofacial abnormalities such as hypoplastic maxilla and obtuse gonial angle which consequently lead to respiratory insufficiency in forms of pharyngeal narrowin...
متن کامل