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Tessier number 6 facial cleft

MedGen UID:
609365
Concept ID:
C0432120
Congenital Abnormality
Synonyms: Infraorbital facial cleft - Tessier cleft 6; Tessier facial cleft number 6
SNOMED CT: Infraorbital facial cleft - Tessier cleft 6 (254018004)
 
HPO: HP:0031578
Monarch Initiative: MONDO:0015417
Orphanet: ORPHA141265

Definition

A facial cleft extending from the zygomatic arch to the eye. This zygomaticomaxillary cleft is similar to that typically found in Treacher Collins syndrome. The overlying tissue shows a vertical sclerodermic furrow radiating from the labial commissure or the angle of the mandible across the cheek to a coloboma of the lower eyelid between the middle and lateral one-third. Microphthalmia is not observed. The skeletal cleft is between the maxilla and zygoma; it passes through the inferolateral orbital rim to enter the inferior orbital fissure. No alveolar cleft is present. The zygomatic arch is intact. The soft tissue furrow, which is more apparent on the right, radiates from the oral commissure toward the lateral two-thirds of the lower eyelid. The antimongoloid obliquity of the palpebral fissures is associated with laterally placed lower eyelid clefts and some ectropion. A left-sided anophthalmia is accompanied by adjacent soft tissue hypoplasia and is reflected in a short palpebral fissure, enophthalmos, and minor ptosis of the eyebrow. No abnormality is present in the alveolar arch except for some tilting of the occlusal plane secondary to hypoplasia of the left side of the maxilla. There is a vertical bony groove in the region of the zygomaticomaxillary suture that ends in the inferolateral portion of a small bony orbit. More laterally, the remainder of the zygomatic body and arch is normal in both shape and dimension. The lateral orbital floor is downslanting but intact, and it lacks direct communication with the temporal or infratemporal fossae. The hypoplasia of the left side of the maxilla and orbit is associated with a reduction in the transverse and anteroposterior dimensions of the anterior cranial fossa; mild asymmetry of the middle cranial fossa and calvarium is present. No significant asymmetry of size, shape, or position is present in the sphenoid. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTessier number 6 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
Fijałkowska M, Antoszewski B
Pol Przegl Chir 2015 Aug;87(8):389-94. doi: 10.1515/pjs-2015-0077. PMID: 26495914
Franchi G, Kadlub N, Diner PA, Bandini M, Vazquez MP, Picard A
Br J Oral Maxillofac Surg 2015 May;53(5):421-5. Epub 2015 Mar 21 doi: 10.1016/j.bjoms.2015.02.004. PMID: 25799958
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

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
Gao JL, Rogers GF, Myseros JS, Habeshian KA
Pediatr Dermatol 2022 Nov;39(6):990-991. Epub 2022 Jun 8 doi: 10.1111/pde.15059. PMID: 35677994
Deng Y, Tang W, Li Z
J Craniofac Surg 2018 Sep;29(6):e582-e585. doi: 10.1097/SCS.0000000000004599. PMID: 29771829
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
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

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
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
Bilkay U, Gundogan H, Ozek C, Gurter T, Akin Y
Ann Plast Surg 2000 Dec;45(6):654-7. doi: 10.1097/00000637-200045060-00016. PMID: 11128768

Clinical prediction guides

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
Van der Meulen JC, Moscona AR, Vaandrager JM, Hirshowitz B
Ann Plast Surg 1982 Jun;8(6):474-85. doi: 10.1097/00000637-198206000-00006. PMID: 7114757

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