An Indonesian child with orofacial duplication and neurocristopathy anomalies: case report

J Craniomaxillofac Surg. 2001 Aug;29(4):195-7. doi: 10.1054/jcms.2001.0221.


The parents of an Indonesian patient were concerned with the facial appearance of their child. A medical, family and social history was taken for the patient with the detection of no specific, systemic abnormalities or unusual conditions, although it was noted that the family lived in an area associated with pollutants. On examination, the facial appearance was asymmetric with inferior displacement of the right eye. The zygomatic-maxillary region appeared to be enlarged and deformed. There was unilateral macrostomia with the upper lip appearing elongated. Intra-oral examination revealed that there was duplication of the maxilla and associated structures. The maxilla had a normal complement of deciduous teeth but gross caries was noted. A supernumerary maxilla appeared to be responsible for the extra-oral, zygomatic-maxillary deformation, and its deciduous dentition was incomplete intra-orally. For the mandibular structures, the tongue was abnormally small, giving the appearance of microglossia. The mandibular dentition appeared to be incomplete with gross caries. Further posteriorly, there appeared to be clefting of the individual soft palates. Photographs and radiological examinations were undertaken but CAT scans were not possible. For patient management, a comprehensive surgical strategy is indicated with full family support, education and cooperation.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Craniofacial Abnormalities / complications
  • Craniofacial Abnormalities / pathology*
  • Dental Caries / complications
  • Female
  • Humans
  • Indonesia
  • Neural Crest / abnormalities*