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Tessier number 9 facial cleft

MedGen UID:
1635323
Concept ID:
C4703422
Congenital Abnormality
HPO: HP:0031581

Definition

This is an upper lateral orbital cleft. The soft tissue deformity is in the lateral one-third of the upper eyelid, and the bony cleft is through the superolateral orbital angle. Microphthalmia is present. The superolateral bony deficiency of the orbits allows a lateral displacement of the globes. The lateral one-third of the upper eyelid and the outer canthus are distorted, thus preventing apposition to the globe. The upper eyelid does not have a true cleft. A soft tissue furrow radiates superiorly and posterisphenoid is symmetric and normal. Mild cranial base asymmetry is reflected in the pterygoid plates. The left pair is more laterally displaced from the midline. Skull vault plagiocephaly is evident with an apparent reduction in the anteroposterior dimension of the anterior cranial fossa. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTessier number 9 facial cleft

Recent clinical studies

Etiology

da Silva Freitas R, Alonso N, Busato L, Ueda WK, Hota T, Medeiros SH, Kunz RT
J Craniofac Surg 2010 Mar;21(2):390-5. doi: 10.1097/SCS.0b013e3181cfa6b1. PMID: 20186077
da Silva Freitas R, Alonso N, Shin JH, Busato L, Ono MC, Cruz GA
J Craniofac Surg 2008 Sep;19(5):1348-52. doi: 10.1097/SCS.0b013e318184326e. PMID: 18812861
Stelnicki EJ, Hoffman W, Foster R, Lopoo J, Longaker M
J Craniofac Surg 1998 Nov;9(6):557-62; discussion 563. doi: 10.1097/00001665-199811000-00012. PMID: 10029770

Diagnosis

Bajaj A, Dyke P, Zaleski C, Cava J, McPherson E
Am J Med Genet A 2011 Sep;155A(9):2298-301. Epub 2011 Aug 10 doi: 10.1002/ajmg.a.34166. PMID: 21834042

Therapy

Stelnicki EJ, Hoffman W, Foster R, Lopoo J, Longaker M
J Craniofac Surg 1998 Nov;9(6):557-62; discussion 563. doi: 10.1097/00001665-199811000-00012. PMID: 10029770

Prognosis

da Silva Freitas R, Alonso N, Busato L, Ueda WK, Hota T, Medeiros SH, Kunz RT
J Craniofac Surg 2010 Mar;21(2):390-5. doi: 10.1097/SCS.0b013e3181cfa6b1. PMID: 20186077

Clinical prediction guides

Kalantar-Hormozi A, Noori M, Karimi A
J Craniofac Surg 2023 Nov-Dec 01;34(8):e790-e793. Epub 2023 Aug 25 doi: 10.1097/SCS.0000000000009679. PMID: 37622550

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