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Tessier number 13 facial cleft

MedGen UID:
1633782
Concept ID:
C4703426
Congenital Abnormality
HPO: HP:0031585

Definition

There is a paramedian frontal encephalocele and a soft tissue cleft that passes medial to an intact eyebrow. The frontal bone shows a paramedian bony cleft with an associated encephalocele. The olfactory groove, cribriform plate, and ethmoid sinus are all increased in transverse diameter, resulting in hypertelorism. The cleft extends medially to the undisturbed eyebrow to end in a short paramedian frontal widow's peak. The bony cleft begins in the region of the nasal bone and extends superiorly through the full height of the frontal bone. Posteriorly, the cleft extends through the cribriform plate and ethmoid sinus as far as the lesser wing and body of the sphenoid. The pterygoid processes are anatomically normal, but they are displaced laterally from the midline on the cleft side. There is orbital hypertelorism below and asymmetry of the floor of the anterior cranial fossa above. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTessier number 13 facial cleft

Conditions with this feature

Frontonasal dysplasia with alopecia and genital anomaly
MedGen UID:
462053
Concept ID:
C3150703
Disease or Syndrome
Frontonasal dysplasia-2 (FND2) is an autosomal recessive disorder characterized by variable degrees of alopecia, skull defects, hypertelorism, depressed nasal bridge and ridge with notched alae nasi, and abnormal central nervous system findings (summary by Kariminejad et al., 2014).

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
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

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

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

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

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
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

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