Akademik Veri Yönetim Sistemi
Journal of Advanced Oral Research, cilt.14, sa.2, ss.218-222, 2023 (ESCI)
Initially defined by Robinow et al. in 1969, Robinow syndrome is an exceptionally uncommon (1:500,000) hereditary disorder showing five distinct phenotypes. Although skeletal system symptoms are intense in all phenotypes, maxillofacial indications and genital organ hypoplasia are also present. Maxillofacial appearances can be listed as follows: hypertelorism, midface hypoplasia, wide and flat nose, low nasal bridge, downturned oral commissures, low-set ears, and micrognathia. In oral manifestations, gingival hyperplasia, tongue, and dental abnormalities are frequently encountered. The detection of intraoral and extraoral findings by the dentist plays a significant role in the diagnosis of this disease. Additionally, every new case documented will help dentists plan treatment approaches. Therefore, in this case report we aimed to describe the manifestations of a patient with Robinow syndrome who was referred to us by a pediatric cardiologist for her dental treatments and also the treatment approach under general anesthesia.