Maxillonasal dysplasia

Binder syndrome is a rare developmental anomaly, affecting primarily the anterior part of the maxilla and nasal complex.



Input patient's signs and symptoms


Narrow down the case reports



Total: 22 (papers)

  


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
1
(42.7%)
2596747
Maxillonasal dysplasia, mandibular retrognathia and cleft palate.
Mossey P, Sandham J.
Angle Orthod. 1989;59(4):257-61; discussion 262.
Retrognathia
Cephalometry Child Child, Preschool Cleft Palate Females Homo sapiens Infant Maxilla Nose Pierre Robin Syndrome Retrognathia
2
(33.7%)
9558535
Binder syndrome: literature review and long-term follow-up on two cases.
McCollum AG, Wolford LM.
Int J Adult Orthodon Orthognath Surg. 1998;13(1):45-58.
Broad philtrum
Bone Transplantation Cartilage Cephalometry Child, Preschool Combined Modality Therapy Craniofacial Dysostosis Females Homo sapiens Maxilla Orthodontics, Corrective Syndrome
3
(32.1%)
21309654
Oral and craniofacial findings of Binder syndrome: two case reports.
Defraia E, Camporesi M, Conti G, Zoni V, Marinelli A.
Cleft Palate Craniofac J. 2012;49(4):498-503.
Cleft lip
Child Differential Diagnosis Females Homo sapiens Maxilla Maxillofacial Abnormalities Nose
4
(27.8%)
22675013
Binder's syndrome.
Jain U, Thakur G, Kallury A.
BMJ Case Rep. 2011;2011:.
Short nose Overbite
Females Homo sapiens Maxilla Maxillofacial Abnormalities Nose Orthodontics, Corrective Orthognathic Surgical Procedures
4
(27.8%)
15980645
Maxillo-nasal dysplasia (binder syndrome): antenatal discovery and implications.
Cuillier F, Cartault F, Lemaire P, Alessandri JL.
Fetal Diagn Ther. 2005;20(4):301-5.
Short nose Overbite
Adult Females Homo sapiens Infant, Newborn Maxillofacial Abnormalities Nose Pregnancy Ultrasonography, Prenatal
4
(27.8%)
3293247
Surgical approach in severe cases of maxillonasal dysplasia (Binder's syndrome).
Holmstrom H, Kahnberg KE.
Swed Dent J. 1988;12(1-2):3-10.
Open bite
Bone Transplantation Females Homo sapiens Male Malocclusion Maxilla Nose Osteotomy
7
(26.3%)
29068927
Orthognathic Surgery and Rhinoplasty to Address Nasomaxillary Hypoplasia.
Veeramani A, Sawh R, Steinbacher DM.
Plast Reconstr Surg. 2017;140(5):930-932.
Short columella
Females Homo sapiens Maxillofacial Abnormalities Orthognathic Surgical Procedures
7
(26.3%)
27162279
Prenatally Diagnosed Cases of Binder Phenotype Complicated by Respiratory Distress in the Immediate Postnatal Period.
Blumenfeld YJ, Davis AS, Hintz SR, Milan K, Messner AH, Barth RA, Hudgins L, Chueh J, Homeyer M, Bernstein JA, Enns G, Atwal P, Manning M.
J Ultrasound Med. 2016;35(6):1353-8.
Short columella
Adult Differential Diagnosis Females Homo sapiens Infant, Newborn Magnetic Resonance Imaging Male Maxillofacial Abnormalities Pregnancy Respiratory Insufficiency Ultrasonography, Prenatal
7
(26.3%)
22611639
A case of Keutel syndrome diagnosed in the neonatal period: associated with Binder phenotype.
Demirel G, Oguz SS, Celik IH, Erdeve O, Uras N, Dilmen U.
Genet Couns. 2012;23(1):25-30.
Respiratory insufficiency Short nose
Calcinosis Cartilage Diseases Chondrodysplasia Punctata Congenital Hand Deformities Differential Diagnosis Fatal Outcome Homo sapiens Infant, Newborn Maxilla Maxillofacial Abnormalities Nose Pulmonary Valve Stenosis
7
(26.3%)
20216441
Nasal reconstruction for maxillonasal dysplasia.
Holmes AD, Lee SJ, Greensmith A, Heggie A, Meara JG.
J Craniofac Surg. 2010;21(2):543-51.
Short columella
Age Factors Biomaterials Cartilage Child Child, Preschool Females Follow-Up Studies Graft Survival Homo sapiens Infant Longitudinal Studies Male Maxillofacial Abnormalities Nose Orthognathic Surgical Procedures Osteotomy, Le Fort Postoperative Complications Prosthesis Implantation Prosthesis-Related Infection Reconstructive Surgical Procedures Retrospective Studies Syndrome Tissue Expansion Young Adult
        

Phenotype(s) retrieved from Orphanet

    Total: 20

HPO ID Term Frequency
HP:0000327 Hypoplasia of the maxilla Very frequent (99-80%)
HP:0000457 Depressed nasal ridge Very frequent (99-80%)
HP:0002000 Short columella Very frequent (99-80%)
HP:0003196 Short nose Very frequent (99-80%)
HP:0005280 Depressed nasal bridge Very frequent (99-80%)
HP:0011800 Midface retrusion Very frequent (99-80%)
HP:0011892 Low levels of vitamin K Very frequent (99-80%)
HP:0012368 Flat face Very frequent (99-80%)
HP:0000175 Cleft palate Frequent (79-30%)
HP:0000691 Microdontia Frequent (79-30%)
HP:0001065 Striae distensae Frequent (79-30%)
HP:0002650 Scoliosis Frequent (79-30%)
HP:0004609 Patchy distortion of vertebrae Frequent (79-30%)
HP:0005288 Abnormality of the nares Frequent (79-30%)
HP:0008428 Vertebral clefting Frequent (79-30%)
HP:0009804 Reduced number of teeth Frequent (79-30%)
HP:0009882 Short distal phalanx of finger Frequent (79-30%)
HP:0010807 Open bite Frequent (79-30%)
HP:0000303 Mandibular prognathia Occasional (29-5%)
HP:0010185 Aplasia/Hypoplasia of the distal phalanges of the toes Occasional (29-5%)


Phenotype(s) retrieved from case reports

    Total: 5

HPO ID Term # of case reports
HP:0003196 Short nose 4
HP:0002000 Short columella 3
HP:0000278 Retrognathia 1
HP:0000289 Broad philtrum 1
HP:0000824 Growth hormone deficiency 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID