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Tessier number 2 facial cleft

MedGen UID:
1634409
Concept ID:
C4703418
Congenital Abnormality
HPO: HP:0031573

Definition

As is typically seen in isolated cleft cases, a cleft of the lip is present. There is hypoplasia, but not true notching of the ala nasi with flattening of the lateral part of the nose. The nasal root is broadened, with lateral displacement of the inner canthus. The palpebral fissure and lacrimal drainage system are not disturbed. The alveolar cleft is through the lateral incisor area and extends to the pyriform aperture. There is normal septation between the nasal cavity and maxillary sinus. Notching at the junction between the nasal bone is present, as is a broad, flat frontal process of the maxilla. Transverse ethmoid enlargement produces orbital hypertelorism. Above the cleft of the lip and palate is a true broad cleft of the nostril that is medial to the intact, but laterally displaced, tail of the alar cartilage. A shallow soft tissue groove extends superiorly to the asymmetrically widened nasal root. The lacrimal system, palpebral fissures, and eyebrows remain intact. The alveolar cleft extends posteriorly as a complete unilateral cleft of the hard and soft palate. The nasal septum is intact but deviated to the opposite side. The nasal cavity remains separated from the normally pneumatized, although hypoplastic, maxilla on the cleft side. Above the nasomaxillary notching, the ethmoid sinus is less well developed, and there is no pneumatization of the frontal sinus on this side. Anterior rotation of the greater and lesser wings of the sphenoid occurs on the cleft side in relation to the narrower orbit and smaller ethmoid sinus. There is mild asymmetry of the anterior cranial fossa, which is narrower on the cleft side. The cranium is brachycephalic with marked occipital flattening. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTessier number 2 facial cleft

Recent clinical studies

Etiology

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
Chapchay K, Zaga J, Billig A, Adler N, Margulis A
Ann Plast Surg 2019 Mar;82(3):289-291. doi: 10.1097/SAP.0000000000001688. PMID: 30562204
Racz C, Dakpé S, Kadlub N, Testelin S, Devauchelle B, Rachwalski M, Picard A
J Craniomaxillofac Surg 2018 Jan;46(1):22-27. Epub 2017 Oct 27 doi: 10.1016/j.jcms.2017.10.012. PMID: 29239768
Fijałkowska M, Antoszewski B
Pol Przegl Chir 2015 Aug;87(8):389-94. doi: 10.1515/pjs-2015-0077. PMID: 26495914
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

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
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
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
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

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

Prognosis

Raposo-Amaral CE, Oliveira YM, Denadai R, Ghizoni E, Raposo-Amaral CA
J Craniofac Surg 2021 Oct 1;32(7):e638-e642. doi: 10.1097/SCS.0000000000007648. PMID: 33770026
Racz C, Dakpé S, Kadlub N, Testelin S, Devauchelle B, Rachwalski M, Picard A
J Craniomaxillofac Surg 2018 Jan;46(1):22-27. Epub 2017 Oct 27 doi: 10.1016/j.jcms.2017.10.012. PMID: 29239768
Fijałkowska M, Antoszewski B
Pol Przegl Chir 2015 Aug;87(8):389-94. doi: 10.1515/pjs-2015-0077. PMID: 26495914
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
Fijałkowska M, Antoszewski B
Pol Przegl Chir 2015 Aug;87(8):389-94. doi: 10.1515/pjs-2015-0077. PMID: 26495914
Savastano CP, Bernardi P, Seuánez HN, Moreira MÂ, Orioli IM
Birth Defects Res A Clin Mol Teratol 2014 Apr;100(4):300-6. Epub 2014 Feb 12 doi: 10.1002/bdra.23216. PMID: 24677696
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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