Oro-facial-digital syndrome Type 1: A case report

Abstract

Oro-Facial Digital Syndrome (OFDS) is a generic term for group of apparently distinctive genetic diseases that affect the development of the oral cavity, facial features, and digits. One of these is OFDS type I (OFDS-I) which has rarely been reported in Asian countries. This is the case report of a 13 year old patient with OFDS type I who reported to the Department of Pedodontics and Preventive Dentistry, with the complaint of discolored upper front teeth.

Oral-Facial-Digital Syndrome Type 1: Oral Findings in a 6-Year-Old Girl

“…OFDS1 is a syndrome that includes missing and supernumerary teeth. 9 Only cases with supernumerary canines have been reported in the literature 10,11 ; a case with an excessive number of both primary and permanent incisors has also been reported. 5 In our case, the upper bilateral supernumerary canines and lower, supernumerary canines were present.…”

“…Similar findings are observed in patients with Opitz trigonocephaly syndrome, in addition to short stature and skin laxity. 12 Although there are many articles in the literature that show the oral findings of OFDS1, 5,8,10,11,13,14 the articles reporting changes in oral findings as a result of long follow-up of patients are limited. 15,16 Also, the oral and facial changes have been reported after orthodontic treatment of patients 21 and 14 years of age with OFDS1.…”