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Tessier number 14 facial cleft

MedGen UID:
Concept ID:
Congenital Abnormality
Synonym: Tessier facial cleft number 14
HPO: HP:0031586


This midline cranial cleft usually occurs with a midline facial cleft that completes a median craniofacial dysraphia. A broad nasal root and bifid nose are associated with orbital hypertelorism and a median frontal encephalocele. The frontal bone abnormality varies from a minor flattening to a large midline defect. There is an increased distance between the olfactory grooves. The crista galli is widened, duplicated, or in some cases absent. Marked inferior prolapse of the enlarged ethmoid bone occurs with orbital hypertelorism. The severe orbital hypertelorism is associated with a broad flattening of the glabella and extreme lateral displacement of the inner canthi. The periorbita, including the eyelids and eyebrows, are otherwise normal. A long midline projection of the frontal hairline marks the superior extent of the soft tissue features of this midline cranial cleft. The median frontal defect delineates the region through which the frontal encephalocele herniates. The lateral segments of the frontal bone sweep upward from the region of the intact glabella and are flattened laterally. No pneumatization of the frontal sinus is evident. The crista galli and the perpendicular plate of the ethmoid are bifid. Just as the ethmoid, including the cribriform plate, is widened and caudally displaced, the sphenoid sinus is broadened and extensively, but symmetrically pneumatized. The lateral rotation of the greater and lesser wings of the sphenoid results in a relative shortening of the anteroposterior dimension of the middle cranial fossa. The floor of the anterior cranial fossa is upslanting from its medial aspect to its lateral aspect, with a harlequin appearance on the coronal scan. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTessier number 14 facial cleft

Recent clinical studies


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, Shin JH, Busato L, Ono MC, Cruz GA
J Craniofac Surg 2008 Sep;19(5):1348-52. doi: 10.1097/SCS.0b013e318184326e. PMID: 18812861


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


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

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