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Tessier number 7 facial cleft

MedGen UID:
1634567
Concept ID:
C4552111
Congenital Abnormality
Synonyms: Tessier cleft number 7; Tessier facial cleft number 7
 
HPO: HP:0031579

Definition

The temporozygomatic Number 7 cleft is found in both Treacher Collins syndrome and hemifacial microsomia. Soft tissue manifestations include macrostomia, malformations of the external and middle ear, temporalis muscle, variable involvement of the seventh cranial nerve (in hemifacial microsomia), and abnormalities of the preauricular hair in Treacher Collins syndrome. The skeletal cleft is through the pterygomaxillary junction, and vertical maxillary hypoplasia is present. In addition, abnormality of the mandibular ramus, coronoid, and condyle and absence of the zygomatic arch are typically present. A soft tissue furrow extends from the macrostomia laterally and superiorly across the cheek toward the preauricular hairline. The lower eyelids are intact. The anatomy of the external ear is normal, and there are no preauricular tags. Bony clefting is through the pterygomaxillary junction with hypoplasia of the alveolar process in the molar region, thereby producing a posterior open bite. The maxilla is hypoplastic, although the maxillary sinuses are symmetrically pneumatized. The hypoplastic zygomatic body arches upward, but then it takes a downward course and is severely malformed and displaced. The zygoma is continuous posteriorly with an apparently normal zygomatic process of the temporal bone. The mandibular condyle and coronoid process are hypoplastic and asymmetric. There is no antegonial notching of the mandible. Marked cranial base asymmetry, with tilting and asymmetric positioning of the temporomandibular articulations, is present. The anatomy of the sphenoid is abnormal, especially on the right where there is no recognizable medial or lateral pterygoid plate. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTessier number 7 facial cleft

Recent clinical studies

Etiology

Fijałkowska M, Antoszewski B
Pol Przegl Chir 2015 Aug;87(8):389-94. doi: 10.1515/pjs-2015-0077. PMID: 26495914
Nam SM, Kim YB
J Craniomaxillofac Surg 2014 Oct;42(7):1397-401. Epub 2014 Apr 13 doi: 10.1016/j.jcms.2014.03.032. PMID: 24837484
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

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
Cavaco-Gomes J, Duarte C, Pereira E, Matias A, Montenegro N, Merz E
J Obstet Gynaecol 2017 May;37(4):421-427. Epub 2017 Mar 13 doi: 10.1080/01443615.2017.1285274. PMID: 28287290
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
Presti F, Celentano C, Marcazzò L, Dolcetta G, Prefumo F
Ultrasound Obstet Gynecol 2004 Jun;23(6):606-8. doi: 10.1002/uog.1058. PMID: 15170805

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

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
Fijałkowska M, Antoszewski B
Pol Przegl Chir 2015 Aug;87(8):389-94. doi: 10.1515/pjs-2015-0077. PMID: 26495914
Nam SM, Kim YB
J Craniomaxillofac Surg 2014 Oct;42(7):1397-401. Epub 2014 Apr 13 doi: 10.1016/j.jcms.2014.03.032. PMID: 24837484
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
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
Cannistrà C, Bontemps C, Valero R, Iannetti G, Barbet JP
Plast Reconstr Surg 2006 Dec;118(7):1538-1542. doi: 10.1097/01.prs.0000240818.64018.2a. PMID: 17102725

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