Craniofacial morphology of the tricho-dento-osseous syndrome

Katherine Kula, Kathie Hall, Thomas Hart, J. Tim Wright

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Tricho-dento-osseous syndrome (TDO) is characterized by abnormal bone, hair, and tooth morphology. However, the reported craniofacial abnormalities are not well characterized. The purpose of this study was to compare the craniofacial parameters of 25 subjects affected with TDO (A) with those of 15 unaffected relatives (U). Standardized lateral cephalograms were traced and digitized. Each subject's data were compared by age and sex to cephalometric standards (Bolton, Behrents); severity was scored by standard deviations from the standard mean, and then grouped into A vs. U. All cephalograms were evaluated for frontal sinuses, mastoid pneumatization, diploe, and bone density, and cranial thickness was measured. Cranial base length (SN; NBa), cranial base angle (BaSN), and mandibular body length (GoPg) were greater in A than in U (p ≤ 0.05). Both groups had longer total and lower facial heights (NMe; ANSMe) compared with normal standards. Frontal sinuses, mastoid pneumatization and diploe were visible less often in A than in U (p ≤ 0.05). Parietal bone and bone at lambda was significantly thicker (p ≤ 0.05) in A than in U. Variability was substantial in many measures in both A and U. The major TDO craniofacial features involve the cranial base, mandibular body length, absence of visible pneumatized mastoids, frontal sinuses and diploe, and thicker cranial bone.

Original languageEnglish (US)
Pages (from-to)446-454
Number of pages9
JournalClinical Genetics
Volume50
Issue number6
StatePublished - Dec 1996
Externally publishedYes

Fingerprint

Frontal Sinus
Mastoid
Skull Base
Bone and Bones
Craniofacial Abnormalities
Cephalometry
Parietal Bone
Bone Density
Tooth
Tricho-dento-osseous syndrome

Keywords

  • Bone
  • Cephalometric
  • Craniofacial
  • Cranium
  • Genetics
  • Radiology
  • Variability

ASJC Scopus subject areas

  • Genetics(clinical)
  • Genetics

Cite this

Kula, K., Hall, K., Hart, T., & Wright, J. T. (1996). Craniofacial morphology of the tricho-dento-osseous syndrome. Clinical Genetics, 50(6), 446-454.

Craniofacial morphology of the tricho-dento-osseous syndrome. / Kula, Katherine; Hall, Kathie; Hart, Thomas; Wright, J. Tim.

In: Clinical Genetics, Vol. 50, No. 6, 12.1996, p. 446-454.

Research output: Contribution to journalArticle

Kula, K, Hall, K, Hart, T & Wright, JT 1996, 'Craniofacial morphology of the tricho-dento-osseous syndrome', Clinical Genetics, vol. 50, no. 6, pp. 446-454.
Kula K, Hall K, Hart T, Wright JT. Craniofacial morphology of the tricho-dento-osseous syndrome. Clinical Genetics. 1996 Dec;50(6):446-454.
Kula, Katherine ; Hall, Kathie ; Hart, Thomas ; Wright, J. Tim. / Craniofacial morphology of the tricho-dento-osseous syndrome. In: Clinical Genetics. 1996 ; Vol. 50, No. 6. pp. 446-454.
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