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Tessier number 10 facial cleft

MedGen UID:
1643789
Concept ID:
C4703423
Congenital Abnormality
HPO: HP:0031582

Definition

In a Number 10 Tessier cleft there is an upper central orbital cleft with a cleft of the middle one-third of the upper eyelid, which often results in total ablepharia. The eyebrow is disrupted, being virtually absent medially, whereas the lateral portion angles upward toward the frontal hairline. There may be ocular anomalies, including colobomata of the iris. The skeletal cleft is through the midportion of the supraorbital rim, the adjacent frontal bone, and the orbital roof lateral to the supraorbital nerve. A frontal encephalocele frequently occupies the frontal bony cleft. The palpebral fissure is grossly elongated with an amblyopic eye displaced inferiorly and laterally. There is also a divergent squint of the right eye. The eyebrow is deficient medially and becomes thinned out laterally , where it is contiguous with a broad downward and forward projection of the frontotemporal hairline (this may be seen in both the Number 9 and 10 clefts.) A broad frontal encephalocele bulges forward from the middle one-third of the right forehead, supraorbital ridge, and orbital roof. The bony cleft, through which the frontal encephalocele presents, involves the anterior half of the orbital roof, the supraorbital rim, and two-thirds of the vertical height of the frontal bone lateral to the supraorbital nerve. The bony orbit is inferiorly displaced and widened with the lateral orbital wall shortened and laterally deviated. Similar distortion of the anterior cranial fossa is evident, being broader and more flattened on the affected side. The calvarium above the level of the cleft and the cranial base below is symmetric. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTessier number 10 facial cleft

Recent clinical studies

Etiology

Tonello C, Martins DANDP, Baptista MAFB, Mondelli F, Kokitsu Nakata NM, Feitosa LB, Alonso N
Cleft Palate Craniofac J 2022 Oct;59(10):1228-1232. Epub 2021 Sep 13 doi: 10.1177/10556656211042172. PMID: 34514882
Shao C, Lu W, Li J, Chen J, Yao Q, Fan X, Fu Y
Eye (Lond) 2017 Aug;31(8):1140-1145. Epub 2017 Mar 17 doi: 10.1038/eye.2017.39. PMID: 28304389Free PMC Article
Allam KA, Lim AA, Elsherbiny A, Kawamoto HK
J Plast Reconstr Aesthet Surg 2014 Aug;67(8):1055-62. Epub 2014 May 10 doi: 10.1016/j.bjps.2014.04.020. PMID: 24933239
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
Fan X, Shao C, Fu Y, Zhou H, Lin M, Zhu H
Ophthalmology 2008 Dec;115(12):2290-2294.e3. Epub 2008 Jun 26 doi: 10.1016/j.ophtha.2008.05.008. PMID: 18584875

Diagnosis

James O, Sabo VY, Adamson OO, Otoghile B, Adekunle AA, Adeyemo WL, Ladeinde AL, Ogunlewe MO
Cleft Palate Craniofac J 2023 Feb;60(2):133-141. Epub 2021 Nov 17 doi: 10.1177/10556656211055012. PMID: 34787018
Tonello C, Martins DANDP, Baptista MAFB, Mondelli F, Kokitsu Nakata NM, Feitosa LB, Alonso N
Cleft Palate Craniofac J 2022 Oct;59(10):1228-1232. Epub 2021 Sep 13 doi: 10.1177/10556656211042172. PMID: 34514882
Bubanale SC, Kurbet SB, De Piedade Sequeira LMG
Indian J Ophthalmol 2017 Jul;65(7):610-612. doi: 10.4103/ijo.IJO_914_16. PMID: 28724820Free PMC Article
Shao C, Lu W, Li J, Chen J, Yao Q, Fan X, Fu Y
Eye (Lond) 2017 Aug;31(8):1140-1145. Epub 2017 Mar 17 doi: 10.1038/eye.2017.39. PMID: 28304389Free PMC Article
Corona-Rivera JR, Zapata-Aldana E, Bobadilla-Morales L, Corona-Rivera A, Peña-Padilla C, Solis-Hernández E, Guzmán C, Richmond E, Zahl C, Zenker M, Sukalo M
Am J Med Genet A 2016 Jun;170(6):1495-501. Epub 2016 Mar 17 doi: 10.1002/ajmg.a.37630. PMID: 26989884

Prognosis

Shao C, Lu W, Li J, Chen J, Yao Q, Fan X, Fu Y
Eye (Lond) 2017 Aug;31(8):1140-1145. Epub 2017 Mar 17 doi: 10.1038/eye.2017.39. PMID: 28304389Free PMC Article
Allam KA, Lim AA, Elsherbiny A, Kawamoto HK
J Plast Reconstr Aesthet Surg 2014 Aug;67(8):1055-62. Epub 2014 May 10 doi: 10.1016/j.bjps.2014.04.020. PMID: 24933239
Toriyama K, Hatano H, Yagi S, Saito K, Kamei Y
J Craniofac Surg 2013 May;24(3):e202-3. doi: 10.1097/SCS.0b013e31826469a8. PMID: 23714958
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

Yu BF, Wei SY, Dai CC, Wei J
J Craniofac Surg 2022 Jan-Feb 01;33(1):183-186. doi: 10.1097/SCS.0000000000008032. PMID: 34320576
Shao C, Lu W, Li J, Chen J, Yao Q, Fan X, Fu Y
Eye (Lond) 2017 Aug;31(8):1140-1145. Epub 2017 Mar 17 doi: 10.1038/eye.2017.39. PMID: 28304389Free PMC Article

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