Craniofacial and Dental Manifestations of Proteus Syndrome: A Case Report
| Autor: | Jonas P. Becktor, Karin Binner Becktor, Eugene E. Keller, P. S. Karnes |
|---|---|
| Rok vydání: | 2002 |
| Předmět: |
medicine.medical_specialty
Hemifacial hypertrophy business.industry Tooth eruption Mandible Mandibular canal Inferior alveolar nerve medicine.disease Dermatology Proteus syndrome Surgery Gigantism stomatognathic diseases medicine.anatomical_structure Otorhinolaryngology medicine Oral Surgery Craniofacial business |
| Zdroj: | The Cleft Palate-Craniofacial Journal. 39:233-245 |
| ISSN: | 1545-1569 1055-6656 |
| Popis: | The Proteus syndrome is a rare congenital hamartomatous condition that is characterized by a wide range of malformations, sometimes involving the face. Common manifestations include partial gigantism, congenital lipomas, and plantar hyperplasia. In this report we describe the craniofacial clinicopathological development in a girl with Proteus syndrome from age 6 to 20 years. The patient had pronounced hemifacial hypertrophy, exostoses in the left parietal region, and enlargement of the inferior alveolar nerve and mandibular canal in the affected region. The dental development of the affected left mandible and maxilla was characterized by extremely premature development and eruption of the primary and permanent teeth and by pronounced idiopathic root resorptions. The multidisciplinary management of the patient and the treatment outcome is reported. A review of the Proteus patients in the literature who exhibited manifestation in the craniofacial region is presented. |
| Databáze: | OpenAIRE |
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