Dental malpositions such as rotations, eruption failures and ankylosis are among other anomalies complicating Seliciclib supplier this dental condition.[64,65] CONCLUSION The etiology of dental anomalies is partly environmental and partly genetic. Because of the polygenic nature of dental characteristics, it is very challenging to identify one single defective gene responsible for a specific dental anomaly. However, recent studies provide new data about the candidate genes. Further studies are required and the rapid progress in the field of genetics may help the clinicians to more accurately discern the environmental and genetic factors contributing to the development of dental anomalies.

Currently, the orthodontist, probably the first to diagnose hereditary dental anomalies and malocclusion of an individual, will remain responsible for the detection of any additional defects in the same patient in order to provide the best treatment. The clinician should always keep in mind that some of those dental anomalies can coexist with certain syndromes and other family members might also have been affected. Whenever it seems necessary, a genetic consultation should be added as part of the orthodontic treatment. Finally, this interdisciplinary approach may help to reveal any risk of recurrence in subsequent generations. Footnotes Source of Support: Nil. Conflict of Interest: None declared
Nevoid basal cell carcinoma syndrome (NBCCS), also known as basal cell nevus syndrome, multiple basal cell carcinomas (BCC) syndrome, Gorlin syndrome and Gorlin-Goltz syndrome, is an inherited medical condition involving defects within multiple body systems such as the skin, nervous system, eyes, endocrine system and bones.

People with this syndrome are particularly prone to developing a common and usually non-life-threatening form of non-melanoma skin cancers.[1] The absence of major diagnostic criteria such as BCC or palmar or plantar pits in young patients delay the early diagnosis and the correct screening for medulloblastoma, BCC and cardiac fibromas.[1] The odontogenic keratocyst (OKC) has significant growth capacity and recurrence potential and is occasionally indicative of the NBCCS. The NBCCS is inherited as an autosomal-dominant trait that consists principally of multiple OKC, multiple BCCs, skeletal anomalies and cranial calcifications.

Syndrome-associated OKC have the highest recurrence rate and represent approximately 5% of all OKC patients.[2] The BCCs develop early in life and may number in the tens or hundreds. The most frequently cited skeletal anomaly is bifid rib. Early calcification of falx cerebri is also relatively frequently seen on skull radiograms.[2] Batimastat This syndrome has been linked to mutations in the PATCHED tumor-suppressor gene that encodes a receptor protein that is a component of the hedgehog (Hh) signaling pathway. Mutations of this gene have been found in syndrome-associated BCCs and OKC.