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Tessier number 8 facial cleft

MedGen UID:
1643447
Concept ID:
C4552113
Congenital Abnormality
Synonym: Tessier facial cleft number 8
 
HPO: HP:0031580

Definition

The frontozygomatic or Number 8 cleft is found in both Treacher Collins syndrome and the Goldenhar variant of hemifacial microsomia. Skeletal defects are more prominent in Treacher Collins syndrome, whereas the soft tissue clefting is more typical in cases of ''Goldenhar syndrome''. Soft tissue clefting presents as a dermatocele, a true lateral eyelid coloboma with absence of the outer canthus, and anomalies of the globe itself, especially epibulbar cysts in patients with Goldenhar syndrome. The frontozygomatic bony cleft produces absence of the lateral orbital rim; this border now is formed by the hypoplastic greater wing of the sphenoid. The absence of bony support for the outer canthus produces lateral canthal dystopia and the characteristic antimongoloid slant of the palpebral fissures. Secondary to the bony deficiency in the lateral orbital wall and floor, there is soft tissue continuity between the orbit, temporal fossa, and infratemporal region. Preauricular hairline indicators delineate the Number 8 cleft as the first of the northbound clefts. Complete absence of the bony lateral orbital wall and rim constitute the skeletal element of the Number 8 cleft. The lateral border of the orbit is formed by the greater wing of the sphenoid from which small spicules of bone, which represent the rudimentary zygoma, may be found in Treacher Collins syndrome. The symmetry of the facial anomalies is reflected in the apparently normal symmetric anterior and middle cranial fossae. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTessier number 8 facial cleft

Recent clinical studies

Etiology

Lessa S, Sebastiá R, Pontello J
Ophthalmic Plast Reconstr Surg 2019 Sep/Oct;35(5):491-494. doi: 10.1097/IOP.0000000000001353. PMID: 30865066
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
Fijałkowska M, Antoszewski B
Pol Przegl Chir 2015 Aug;87(8):389-94. doi: 10.1515/pjs-2015-0077. PMID: 26495914
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

Diagnosis

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
Morgan AL, Cason R, El Amm CA
J Craniofac Surg 2016 Nov;27(8):e785-e787. doi: 10.1097/SCS.0000000000003129. PMID: 28005824
Scheuerle AE, Good RA, Habal MB
J Craniofac Surg 1990 Apr;1(2):88-90. doi: 10.1097/00001665-199001020-00003. PMID: 2094477
Delaire J, Tessier P, Tulasne JF, Resche F
Head Neck Surg 1980 Nov-Dec;3(2):105-22. doi: 10.1002/hed.2890030205. PMID: 7440178

Therapy

Scheuerle AE, Good RA, Habal MB
J Craniofac Surg 1990 Apr;1(2):88-90. doi: 10.1097/00001665-199001020-00003. PMID: 2094477

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
Fijałkowska M, Antoszewski B
Pol Przegl Chir 2015 Aug;87(8):389-94. doi: 10.1515/pjs-2015-0077. PMID: 26495914
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

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
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
Fijałkowska M, Antoszewski B
Pol Przegl Chir 2015 Aug;87(8):389-94. doi: 10.1515/pjs-2015-0077. PMID: 26495914
Altuntas A, Yilmaz MD, Kahveci OK, Dereköy S, Yücel A
Int J Pediatr Otorhinolaryngol 2004 Aug;68(8):1081-5. doi: 10.1016/j.ijporl.2004.03.004. PMID: 15236897
Delaire J, Tessier P, Tulasne JF, Resche F
Head Neck Surg 1980 Nov-Dec;3(2):105-22. doi: 10.1002/hed.2890030205. PMID: 7440178

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