A rare syndrome with unusual dental findings: Oculo-facio-cardio-dental syndrome.
A rare syndrome with unusual dental findings: Oculo-facio-cardio-dental syndrome.
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DOI：
10.4103/0973-029x.140928
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发表时间：
2014-05
期刊：
影响因子：
--
通讯作者：
Verma SL
中科院分区：
文献类型：
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作者：
Verma G;Singh GK;Tandon P;Verma SL
牙根肥大是一种罕见的疾病。当它与其他牙齿异常、面部畸形和先天性白内障相关联时，这种情况被称为眼面心动脉（OFCD）综合征。这是一个x连锁的显性特征，仅在女性中报道，这表明它在男性中是致命的。OFCD最一致和最具病理特征的牙齿发现是根状肥大（极长的根），特别是犬齿，偶尔也会出现其他牙齿，包括前磨牙和门牙，这很容易由牙医或正畸医生通过牙科全景x光片诊断出来。迄今为止，全世界已报告了21例病例。本报告的目的是提出一个新的病例24岁的女性受这种综合征的影响，并从牙科的角度来评估它，以呼吁牙医对这种罕见的异常的注意。
Radiculomegaly of a tooth is a rare condition. When it is associated with other dental abnormalities, facial dysmorphism and congenital cataracts, the condition is named as oculofaciocardiodental (OFCD) syndrome. This is an X-linked dominant trait, reported only in women, suggesting that it is lethal in males. The most consistent and pathognomic dental finding of OFCD is radiculomegaly (extremely long roots), particularly of the canines and occasionally of other teeth including premolars and incisors, which can easily be diagnosed on dental panoramic radiographs by a dentist or an orthodontist. Till date, 21 cases have been reported worldwide. The aim of this report is to present a new case of a 24 year old female affected by this syndrome and to evaluate it from a dental perspective to call the attention of dentists to this rare anomaly.