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Tessier number 4 facial cleft

MedGen UID:
1642051
Concept ID:
C4703420
Congenital Abnormality
Synonyms: facial clefting, oblique, 1; facial clefting, oblique, type 1; OBLFC1; oculomaxillofacial dysplasia with oblique Facial clefts
 
HPO: HP:0031576
Monarch Initiative: MONDO:0010850
Orphanet: ORPHA141258

Definition

The cleft lip is midway between the philtral ridge and the commissure of the mouth. The cleft is lateral to the normally shaped and placed nasal ala and passes onto the cheek. The cleft extends through the lower eyelid lateral to the punctum. The lacrimal system and inner canthus are normal. Microphthalmia may be present. The alveolar cleft passes between the lateral incisor and canine, as in the Number 3 cleft. The cleft passes around the pyriform aperture and continues through the portion of the maxillary sinus medial to the infraorbital foramen. The cleft terminates at the medial end of the inferior orbital rim. There is severe vertical soft tissue deficiency in a Number 4 cleft, with the medial margins of the cleft lip extending directly into the medially placed cleft of the lower eyelid. Within the medial segment of the right-sided cleft lip, muscle elements are apparently absent. Muscle bunching is noted in the ipsilateral lateral lip segment, as is seen in a typical unilateral cleft lip. The anatomically normal nasal ala is superiorly displaced in association with a severe deficiency in the overall nasal length. Marked dystopia of the right globe results in its inferior displacement into the medially deficient orbital floor and inferior rim. Both globes are otherwise normal. The complete palatal cleft passes through the maxilla medial to the infraorbital foramen and extends to the medial portion of the inferior orbital rim without evidence of an intact maxillary sinus. Bony septation persists medially, thereby separating the nasal cavity from the orbit, maxillary sinus, and mouth, which are contiguous. Marked midfacial hypoplasia is present. The cleft is manifest as asymmetry of the body of the sphenoid; it is smaller on the right, with asymmetric placement of the pterygoid plates relative to the midline. The orbital floor cleft has no communication with the inferior orbital fissure. The cleft does not extend to the skull base, but there is marked facial asymmetry associated with plagiocephaly. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTessier number 4 facial cleft

Conditions with this feature

Oculomaxillofacial dysostosis
MedGen UID:
333072
Concept ID:
C1838348
Disease or Syndrome
Oblique facial clefts are a rare form of orofacial clefting, comprising about 0.25% of all facial clefts. Two major types have been described classically: nasoocular and oroocular, the latter of which can be subdivided into oromedial-canthal and orolateral-canthal (summary by Dasouki et al., 1988).

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
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
Assouan C, Ambroise B, de Boutray M, Labbé D, Bénateau H, Veyssiere A
J Stomatol Oral Maxillofac Surg 2018 Jun;119(3):208-211. Epub 2017 Dec 12 doi: 10.1016/j.jormas.2017.12.005. PMID: 29246756
Laure B, Picard A, Bonin-Goga B, Letouze A, Petraud A, Goga D
J Craniomaxillofac Surg 2010 Jun;38(4):245-7. Epub 2009 Jul 8 doi: 10.1016/j.jcms.2009.06.008. PMID: 19586777
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

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
Deng Y, Tang W, Li Z
J Craniofac Surg 2018 Sep;29(6):e582-e585. doi: 10.1097/SCS.0000000000004599. PMID: 29771829
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
Morgan AL, Cason R, El Amm CA
J Craniofac Surg 2016 Nov;27(8):e785-e787. doi: 10.1097/SCS.0000000000003129. PMID: 28005824

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

Watanabe A, Yoshida S, Ishii T, Saito C, Shibahara T
Bull Tokyo Dent Coll 2019 Dec 10;60(4):291-296. Epub 2019 Nov 22 doi: 10.2209/tdcpublication.2018-0070. PMID: 31761879
Assouan C, Ambroise B, de Boutray M, Labbé D, Bénateau H, Veyssiere A
J Stomatol Oral Maxillofac Surg 2018 Jun;119(3):208-211. Epub 2017 Dec 12 doi: 10.1016/j.jormas.2017.12.005. PMID: 29246756
Laure B, Picard A, Bonin-Goga B, Letouze A, Petraud A, Goga D
J Craniomaxillofac Surg 2010 Jun;38(4):245-7. Epub 2009 Jul 8 doi: 10.1016/j.jcms.2009.06.008. PMID: 19586777

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
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
Fearon JA
J Craniofac Surg 2008 Jan;19(1):110-2. doi: 10.1097/SCS.0b013e31815ca1ba. PMID: 18216674
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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