<i>ALX</i>-Related Frontonasal Dysplasias: Clinical Characteristics and Surgical Management.

Vargel I., Canter H. I., Kucukguven A., Aydin A., Ozgur F.

The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association, vol.59, no.5, pp.637-643, 2022 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 59 Issue: 5
  • Publication Date: 2022
  • Doi Number: 10.1177/10556656211019621
  • Journal Name: The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, CINAHL, Educational research abstracts (ERA), EMBASE, Linguistics & Language Behavior Abstracts, MEDLINE, Veterinary Science Database
  • Page Numbers: pp.637-643
  • Keywords: ALX gene, cleft lip, cleft palate, facial clefts, frontonasal dysplasia, hypertelorism, median cleft, CNS STRUCTURAL ANOMALIES, MENTAL-RETARDATION, EYELID COLOBOMAS, EAR ANOMALIES, MACROBLEPHARON, HYPERTELORISM, MACROSTOMIA, DYSOSTOSIS, MUTATIONS, DEFECTS
  • Hacettepe University Affiliated: Yes

Abstract

Aim: The term frontonasal dysplasia (FND) represents a spectrum of anomalies and its genetics have not been well defined. Recently, the critical role of the aristaless-like homeobox (ALX) gene family on the craniofacial development has been discovered. In the present study, we aimed to propose a systematic surgical treatment plan for the ALX-related FNDs according to the genotypic classification as well as demonstrating their clinical characteristics to help surgeons diagnose the underlying pathology accurately.