scholarly journals Frontonasal dysplasia-severe microphthalmia-severe facial clefting syndrome

2020 ◽  
Author(s):  
Keyword(s):  
Frontonasal Dysplasia

Frontonasal dysplasia: a review

2018 ◽  
pp. 66-76 ◽  
Author(s):  
Muhammad Umair ◽  
Farooq Ahmad ◽  
Muhammad Bilal ◽  
Muhammad Arshad
Keyword(s):  
Frontonasal Dysplasia

Frontonasal Dysplasia

2007 ◽  
pp. 431-435
Keyword(s):  
Frontonasal Dysplasia

Median Cleft Face Syndrome or Frontonasal Dysplasia: A Case Report with Associated Kidney Malformation

1979 ◽  
Vol 16 (1) ◽  
pp. 16-20
Author(s):  
Jaime Roizenblatt ◽  
Anita Wajntal ◽  
Aron J Diament
Keyword(s):  
Case Report ◽  
Frontonasal Dysplasia ◽  
Median Cleft ◽  
Median Cleft Face Syndrome ◽  
Kidney Malformation

Nasal Reconstruction of a Frontonasal Dysplasia via Septal L-Strut Reconstruction Using Costal Cartilage

2021 ◽  
pp. 105566562110366
Author(s):  
Yong Bae Kim ◽  
Seung Min Nam ◽  
Eun Soo Park ◽  
Chang Yong Choi ◽  
Han Gyu Cha ◽  
...  
Keyword(s):  
Postoperative Complications ◽  
General Anesthesia ◽  
Retrospective Review ◽  
Costal Cartilage ◽  
Point Of View ◽  
Nasal Reconstruction ◽  
Open Rhinoplasty ◽  
Frontonasal Dysplasia ◽  
Congenital Condition

Objective Frontonasal dysplasia (FND) is a rare congenital condition. Its major features include hypertelorism, a large and bifid nasal tip, and a broad nasal root. We present our technique of septal L-strut reconstruction using costal cartilage. Design Retrospective review from June 2008 and August 2017. Methods Under general anesthesia, 6 patients with FND underwent septal reconstruction using costal cartilage via open rhinoplasty. We reconstructed the nasal and septal cartilaginous framework by placing columellar struts and cantilever-type grafts. Results The patients ranged in age from 6 to 13 years old. All were female. The follow-up period ranged from 8 months to 2 years; we encountered no postoperative complications (infection, nasal obstruction, or recurrence). All patients were satisfied with their nasal appearance. Conclusions Although the results were not entirely satisfactory from an esthetic point of view, we found that FND can be treated via septal reconstruction with costal cartilage and that the clinical outcomes are reliable and satisfactory. Our approach is a useful option for FND patients.

Frontonasal and Craniofrontonasal Dysplasia: Preoperative Quantitative Description of the Cranio-Orbito-Zygomatic Region Based on Computed and Conventional Tomography

1994 ◽  
Vol 31 (2) ◽  
pp. 97-105 ◽  
Author(s):  
Stephanie M Moffat ◽  
Jeffrey C. Posnick ◽  
Gaylene E. Pron ◽  
Derek C. Armstrong
Keyword(s):  
Computed Tomography ◽  
Clinical Presentation ◽  
Matched Control ◽  
Quantitative Description ◽  
Frontonasal Dysplasia ◽  
Orbital Wall ◽  
Computed Tomography Scans ◽  
Conventional Tomography ◽  
The Mean ◽  
Medial Orbital Wall

The unoperated cranio-orbito-zygomatic complex of 18 children (mean 4.7 years) with frontonasal dysplasia (FND) and 12 children (mean 1.1 years) with craniofrontonasal dysplasia (CFND) was quantified by 15 standard measurements performed on either computed tomography scans or facial tomograms. The results were compared with age-matched control values. In the FND group, the mean anterior interorbital and mid-interorbital distances were significantly increased at 148% and 118% of normal, and in the CFND patients, at 177% and 140% of normal. Excessive medial orbital wall protrusion (mean, 145% of normal in FND and 177% in CFND), shortened zygomatic arch lengths (mean, 94% of normal In FND and 91% in CFND), and reduced cephalic lengths (mean, 96% of normal in FND and 83% in CFND) were all observed. An expanded interzygomatic buttress distance was documented only in the CFND group, at 111% of normal. The clinical presentation of craniofacial deformities such as FND and CFND can be objectively described by a numerical analysis of the bony pathology.

scholarly journals Crucial and Overlapping Roles of Six1 and Six2 in Craniofacial Development

2019 ◽  
Vol 98 (5) ◽  
pp. 572-579 ◽  
Author(s):  
Z. Liu ◽  
C. Li ◽  
J. Xu ◽  
Y. Lan ◽  
H. Liu ◽  
...  
Keyword(s):  
Neural Crest ◽  
Ectopic Expression ◽  
Mandibular Condyle ◽  
Neural Crest Cell ◽  
Messenger Rnas ◽  
Developmental Defects ◽  
Facial Cleft ◽  
Frontonasal Dysplasia ◽  
Morphogenetic Protein ◽  
Crest Cell

SIX1 and SIX2 encode closely related transcription factors of which disruptions have been associated with distinct craniofacial syndromes, with mutations in SIX1 associated with branchiootic syndrome 3 (BOS3) and heterozygous deletions of SIX2 associated with frontonasal dysplasia defects. Whereas mice deficient in Six1 recapitulated most of the developmental defects associated with BOS3, mice lacking Six2 function had no obvious frontonasal defects. We show that Six1 and Six2 exhibit partly overlapping patterns of expression in the developing mouse embryonic frontonasal, maxillary, and mandibular processes. We found that Six1 –/– Six2 –/– double-mutant mice were born with severe craniofacial deformity not seen in the Six1 –/– or Six2 –/– single mutants, including skull bone agenesis, midline facial cleft, and syngnathia. Moreover, whereas Six1 –/– mice exhibited partial transformation of maxillary zygomatic bone into a mandibular condyle-like structure, Six1 –/–Six2 +/– mice exhibit significantly increased penetrance of the maxillary malformation. In addition to ectopic Dlx5 expression at the maxillary-mandibular junction as recently reported in E10.5 Six1 –/– embryos, the E10.5 Six1 –/– Six2 +/– embryos showed ectopic expression of Bmp4, Msx1, and Msx2 messenger RNAs in the maxillary-mandibular junction. Genetically inactivating 1 allele of either Ednra or Bmp4 significantly reduced the penetrance of maxillary malformation in both Six1 –/– and Six1 –/– Six2 +/– embryos, indicating that Six1 and Six2 regulate both endothelin and bone morphogenetic protein-4 signaling pathways to pattern the facial structures. Furthermore, we show that neural crest–specific inactivation of Six1 in Six2 –/– embryos resulted in midline facial cleft and frontal bone agenesis. We show that Six1 –/– Six2 –/– embryos exhibit significantly reduced expression of key frontonasal development genes Alx1 and Alx3 as well as increased apoptosis in the developing frontonasal mesenchyme. Together, these results indicate that Six1 and Six2 function partly redundantly to control multiple craniofacial developmental processes and play a crucial neural crest cell–autonomous role in frontonasal morphogenesis.

Pentalogy of cantrell, ectopia cordis, and frontonasal dysplasia

1992 ◽  
Vol 44 (4) ◽  
pp. 540-540 ◽  
Author(s):  
Miguel Reyes-Mugica
Keyword(s):  
Ectopia Cordis ◽  
Pentalogy Of Cantrell ◽  
Frontonasal Dysplasia

Frontonasal Dysplasia

2017 ◽  
pp. 1173-1182
Author(s):  
Harold Chen
Keyword(s):  
Frontonasal Dysplasia

Frontonasal dysplasia: oral features, restorative and orthodontic dental treatment in a child

2017 ◽  
Vol 18 (2) ◽  
pp. 127-133
Author(s):  
R. A. Valério ◽  
C. Scatena ◽  
F. R. R. Santos ◽  
F. L. Romano ◽  
A. M. Queiroz ◽  
...  
Keyword(s):  
Dental Treatment ◽  
Frontonasal Dysplasia
Sign in / Sign up

Export Citation Format

Share Document