Frontonasal and fibrous dysplasia in a patient with unilateral cleft lip and palate

J Craniofac Surg. 2013 Jul;24(4):e422-4. doi: 10.1097/SCS.0b013e3182942d27.

Abstract

Frontonasal dysplasia is a rare entity. It has characteristic physical deformities: hypertelorism, broad nasal root, median facial cleft of the upper lip or palate, clefting of the nasal alae, poorly formed nasal tip, cranium bifidum occultum, and a widow's peak hairline. Fibrous dysplasia is a benign bone tumor in which normal bone is replaced by fibrous, poorly formed osseus tissues. We present a patient with frontonasal dysplasia who desired correction of her hypertelorism. Incidentally, fibrous dysplasia was found in her left orbit complicating surgical correction. In addition, the patient has velopharyngeal insufficiency and a class III malocclusion. The interplay of all these craniofacial defects makes the sequencing and timing of surgery important in this unique patient.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple*
  • Adolescent
  • Cleft Lip / surgery*
  • Cleft Palate / surgery*
  • Craniofacial Abnormalities / diagnosis*
  • Face / abnormalities*
  • Female
  • Fibrous Dysplasia of Bone / diagnosis
  • Humans
  • Hypertelorism / diagnosis
  • Malocclusion, Angle Class III / diagnosis
  • Orbital Diseases / diagnosis
  • Velopharyngeal Insufficiency / diagnosis

Supplementary concepts

  • Frontonasal dysplasia