Screwdriver-shaped incisors

MedGen UID:: 870607
•Concept ID:: C4025058
•: Anatomical Abnormality; Finding

Synonym:	Screwdriver shaped front teeth

HPO:	HP:0006346

Definition

An abnormality of morphology of the incisor tooth in which the tooth is shaped like a screwdriver blade, i.e., having a rhomboid shape. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVScrewdriver-shaped incisors

Abnormality of the mouth
- Abnormal oral morphology
  - Abnormal oral cavity morphology
    - Abnormality of the dentition
      - Abnormal dental morphology
        Abnormal incisor morphology
        Screwdriver-shaped incisors

Conditions with this feature

Nance-Horan syndrome

MedGen UID:: 208665
•Concept ID:: C0796085
•: Disease or Syndrome

Nance-Horan syndrome (NHS) is an X-linked disorder characterized by congenital cataracts, dental anomalies, dysmorphic features, and, in some cases, mental retardation (summary by Burdon et al., 2003).

See: Condition Record

Nance-Horan syndrome

Recent clinical studies

Etiology

A possible cranio-oro-facial phenotype in Cockayne syndrome.

Bloch-Zupan A, Rousseaux M, Laugel V, Schmittbuhl M, Mathis R, Desforges E, Koob M, Zaloszyc A, Dollfus H, Laugel V
Orphanet J Rare Dis 2013 Jan 14;8:9. doi: 10.1186/1750-1172-8-9. PMID: 23311583 Free PMC Article

Kabuki syndrome: oral and general features seen in a 2-year-old Chinese boy.

Atar M, Lee W, O'Donnell D
Int J Paediatr Dent 2006 May;16(3):222-6. doi: 10.1111/j.1365-263X.2006.00699.x. PMID: 16643546

See all (2)

Diagnosis

Nance-Horan Syndrome: characterization of dental, clinical and molecular features in three new families.

Guven Y, Saracoglu HP, Aksakal SD, Kalayci T, Altunoglu U, Uyguner ZO, Eraslan S, Borklu E, Kayserili H
BMC Oral Health 2023 May 23;23(1):314. doi: 10.1186/s12903-023-03029-4. PMID: 37221585 Free PMC Article

Nance-Horan syndrome-The oral perspective on a rare disease.

Gjørup H, Haubek D, Jacobsen P, Ostergaard JR
Am J Med Genet A 2017 Jan;173(1):88-98. Epub 2016 Sep 12 doi: 10.1002/ajmg.a.37963. PMID: 27616609

A possible cranio-oro-facial phenotype in Cockayne syndrome.

Cenani-Lenz syndactyly in a patient with features of Kabuki syndrome.

Elliott AM, Reed MH, Evans JA, Cross HG, Chudley AE
Clin Dysmorphol 2004 Jul;13(3):143-150. doi: 10.1097/01.mcd.0000127466.26888.15. PMID: 15194949

See all (4)