Dentofacial characteristics in a child with Meier –Gorlin syndrome: A rare case report

Publication date: Available online 16 May 2018 Source:The Saudi Dental Journal Author(s): Rahul G. Morankar, Ashima Goyal, Krishan Gauba, Aditi Kapur Meier–Gorlin syndrome (MGS) is a rare autosomal recessive disorder characterized by the triad of microtia, absent or small patellae and short stature. The other associated clinical features may include developmental delay, congenital pulmonary emphysema, gastro-esophageal reflux, urogenital anomalies, such as cryptorchidism and feeding problems. The facial characteristics during childhood are typical, comprising of a small mouth with full lips and micrognathia/retrognathia. The condition is rare affecting about one to nine individuals per million. Mutation in the genes of pre-replication complex involved in DNA-replication is detected in the majority of patients. This impedes the cellular proliferation resulting in a reduction of total cell number and thereby retardation of overall growth. This case report describe the typical dentofacial characteristics in a 5 years old child affected with Meier-Gorlin syndrome along with other associated anomalies and a multidisciplinary approach for their management.
Source: The Saudi Dental Journal - Category: Dentistry Source Type: research