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Aplasia of the premaxilla

MedGen UID:
869286
•Concept ID:
C4023712
•
Anatomical Abnormality; Finding
Synonyms: Absence of the intermaxillary bone; Absence of the premaxilla; Absence of the primary palate bone; Aplasia of the intermaxillary bone; Aplasia of the primary palate bone; Failure of development of premaxilla; Failure of development of the primary palate bone; Missing premaxilla; Missing primary palate bone
 
HPO: HP:0010757

Definition

Absence of the premaxilla, which is the embryonic structure that forms the anterior part of the maxilla. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAplasia of the premaxilla

Conditions with this feature

Holoprosencephaly 2
MedGen UID:
322517
•Concept ID:
C1834877
•
Disease or Syndrome
A rare disorder characterized by the partial separation of the cerebral hemispheres. It is associated with mutations in the SIX3 gene.
See: Condition Record
Holoprosencephaly 2

Professional guidelines

PubMed

Cleft Lip and Palate Midfacial Hypoplasia: Criteria to Choose the Treatment.
Fariña R, Lolas J, Moreno E, Alister JP, Uribe MF, Pantoja R, Valladares S, Arrué C
J Craniofac Surg 2022 Mar-Apr 01;33(2):496-501. doi: 10.1097/SCS.0000000000007973. PMID: 34261964
Management of the protuberant premaxilla: where does it fit in?
Morris L
Curr Opin Otolaryngol Head Neck Surg 2019 Aug;27(4):302-309. doi: 10.1097/MOO.0000000000000558. PMID: 31219831
Management of the premaxilla in the treatment of bilateral cleft of lip and palate: what can the literature tell us?
Bittermann GK, de Ruiter AP, Janssen NG, Bittermann AJ, van der Molen AM, van Es RJ, Rosenberg AJ, Koole R
Clin Oral Investig 2016 Mar;20(2):207-17. Epub 2015 Sep 16 doi: 10.1007/s00784-015-1589-y. PMID: 26374747Free PMC Article

Recent clinical studies

Etiology

Repair of Protruding Bilateral Cleft Lip and Palate With Staged Premaxilla Setback Osteotomy, Cheiloplasty, and Palatoplasty in Trisomy 17p Patient: A Review of Syndromic Clinical Characteristic.
Bangun K, Halim J, Tania V
Cleft Palate Craniofac J 2023 May;60(5):621-626. Epub 2021 Dec 30 doi: 10.1177/10556656211069820. PMID: 34967231
Cleft Lip and Palate Midfacial Hypoplasia: Criteria to Choose the Treatment.
Fariña R, Lolas J, Moreno E, Alister JP, Uribe MF, Pantoja R, Valladares S, Arrué C
J Craniofac Surg 2022 Mar-Apr 01;33(2):496-501. doi: 10.1097/SCS.0000000000007973. PMID: 34261964
Lag screw fixation of the premaxilla during bilateral cleft lip repair.
Chauhan JS, Sharma S
J Craniomaxillofac Surg 2019 Dec;47(12):1881-1886. Epub 2019 Nov 28 doi: 10.1016/j.jcms.2019.11.015. PMID: 31812313
The arrhinias: Proboscis lateralis literature review and surgical update.
Galiè M, Clauser LC, Tieghi R, Kawamoto HK, Wolfe SA, Bianchi AE
J Craniomaxillofac Surg 2019 Sep;47(9):1410-1413. Epub 2018 Dec 27 doi: 10.1016/j.jcms.2018.12.013. PMID: 31420283
The Arrhinias.
Tessier P, Ciminello FS, Wolfe SA
Scand J Plast Reconstr Surg Hand Surg 2009;43(4):177-96. doi: 10.1080/02844310802517259. PMID: 19401938

Diagnosis

3D facial morphometry in Italian patients affected by Aicardi syndrome.
Masnada S, Gibelli D, Dolci C, De Giorgis V, Cappella A, Veggiotti P, Sforza C; Italian Aicardi Study Group
Am J Med Genet A 2020 Oct;182(10):2325-2332. Epub 2020 Aug 15 doi: 10.1002/ajmg.a.61791. PMID: 32798292
Incidence and morphology of the incisive suture in CT scanning of young children and human fetuses.
Nicol P, Elmaleh-Bergès M, Sadoine J, Slimani L, Vacher C
Surg Radiol Anat 2020 Sep;42(9):1057-1062. Epub 2020 Jun 20 doi: 10.1007/s00276-020-02521-8. PMID: 32564109
Is palato-premaxillary subduction a characteristic of Binder's syndrome?
de Saint Hilaire T, Nguyen DT, Jankowski R, Rumeau C
Eur Ann Otorhinolaryngol Head Neck Dis 2019 Sep;136(4):241-245. Epub 2019 Mar 21 doi: 10.1016/j.anorl.2019.03.002. PMID: 30904259
Premaxilla: an independent bone that can base therapeutics for middle third growth!
Trevizan M, Consolaro A
Dental Press J Orthod 2017 Mar-Apr;22(2):21-26. doi: 10.1590/2177-6709.22.2.021-026.oin. PMID: 28658352Free PMC Article
The Arrhinias.
Tessier P, Ciminello FS, Wolfe SA
Scand J Plast Reconstr Surg Hand Surg 2009;43(4):177-96. doi: 10.1080/02844310802517259. PMID: 19401938

Therapy

Bilateral Cleft Lip and Palate, With Facial Dysplasia: Interdisciplinary Treatment and Long-Term Follow-Up.
Bennun RD, Harfin JH
J Craniofac Surg 2018 Sep;29(6):1441-1444. doi: 10.1097/SCS.0000000000004756. PMID: 30015740
Advantages and limits of 3-segment (paramedian) versus 2-segment (median) surgically assisted rapid maxillary expansion (SARME).
Landes CA, Laudemann K, Petruchin O, Revilla C, Seitz O, Kopp S, Ludwig B, Sader RA
Oral Surg Oral Med Oral Pathol Oral Radiol 2012 Jan;113(1):29-40. Epub 2011 Mar 31 doi: 10.1016/j.tripleo.2011.01.013. PMID: 22677689
The origin and development of the upper lateral incisor and premaxilla in normal and cleft lip/palate monkeys induced with cyclophosphamide.
Wei X, Senders C, Owiti GO, Liu X, Wei ZN, Dillard-Telm L, McClure HM, Hendrickx AG
Cleft Palate Craniofac J 2000 Nov;37(6):571-83. doi: 10.1597/1545-1569_2000_037_0571_toadot_2.0.co_2. PMID: 11108527
Presurgical columellar elongation and primary retrograde nasal reconstruction in one-stage bilateral cleft lip and nose repair.
Cutting C, Grayson B, Brecht L, Santiago P, Wood R, Kwon S
Plast Reconstr Surg 1998 Mar;101(3):630-9. doi: 10.1097/00006534-199803000-00009. PMID: 9500379
A devitalized tooth as a complication of septorhinoplasty.
Sykes JM, Toriumi D, Kerth JD
Arch Otolaryngol Head Neck Surg 1987 Jul;113(7):765-7. doi: 10.1001/archotol.1987.01860070079021. PMID: 3580160

Prognosis

Incidence and morphology of the incisive suture in CT scanning of young children and human fetuses.
Nicol P, Elmaleh-Bergès M, Sadoine J, Slimani L, Vacher C
Surg Radiol Anat 2020 Sep;42(9):1057-1062. Epub 2020 Jun 20 doi: 10.1007/s00276-020-02521-8. PMID: 32564109
Treatment algorithm for bilateral alveolar cleft based on the position of the premaxilla and the width of the alveolar gap.
Koh KS, Kim H, Oh TS, Kwon SM, Choi JW
J Plast Reconstr Aesthet Surg 2013 Sep;66(9):1212-8. Epub 2013 May 21 doi: 10.1016/j.bjps.2013.04.060. PMID: 23702194
The Arrhinias.
Tessier P, Ciminello FS, Wolfe SA
Scand J Plast Reconstr Surg Hand Surg 2009;43(4):177-96. doi: 10.1080/02844310802517259. PMID: 19401938
Long-term evaluation of bilateral cleft lip and palate patients after early secondary closure and premaxilla repositioning.
Geraedts CT, Borstlap WA, Groenewoud JM, Borstlap-Engels VM, Stoelinga PJ
Int J Oral Maxillofac Surg 2007 Sep;36(9):788-96. Epub 2007 Jul 6 doi: 10.1016/j.ijom.2007.04.010. PMID: 17618084
Management of the prominent premaxilla in bilateral cleft lip and palate.
Aburezq H, Daskalogiannakis J, Forrest C
Cleft Palate Craniofac J 2006 Jan;43(1):92-5. doi: 10.1597/04-114r.1. PMID: 16405381

Clinical prediction guides

3D facial morphometry in Italian patients affected by Aicardi syndrome.
Masnada S, Gibelli D, Dolci C, De Giorgis V, Cappella A, Veggiotti P, Sforza C; Italian Aicardi Study Group
Am J Med Genet A 2020 Oct;182(10):2325-2332. Epub 2020 Aug 15 doi: 10.1002/ajmg.a.61791. PMID: 32798292
Subphenotyping and Classification of Cleft Lip and Alveolus in Adult Unoperated Patients: A New Embryological Approach.
Vermeij-Keers C, Rozendaal AM, Luijsterburg AJM, Latief BS, Lekkas C, Kragt L, Ongkosuwito EM
Cleft Palate Craniofac J 2018 Oct;55(9):1267-1276. Epub 2018 Apr 13 doi: 10.1177/1055665618767106. PMID: 29652537
A re-evaluation of the premaxillary bone in humans.
Barteczko K, Jacob M
Anat Embryol (Berl) 2004 Mar;207(6):417-37. Epub 2004 Feb 4 doi: 10.1007/s00429-003-0366-x. PMID: 14760532
Development of the facial midline.
Carstens MH
J Craniofac Surg 2002 Jan;13(1):129-87; discussion 188-90. doi: 10.1097/00001665-200201000-00032. PMID: 11887012
Clinical observations of cleft lip and palate.
Precious DS, Delaire J
Oral Surg Oral Med Oral Pathol 1993 Feb;75(2):141-51. doi: 10.1016/0030-4220(93)90084-h. PMID: 8426712

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