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Submucous cleft of soft and hard palate

MedGen UID:
1384667
•Concept ID:
C4477069
•
Congenital Abnormality
HPO: HP:0410031

Definition

Soft and hard-palate submucous clefts are characterized by bony defects in the midline of the soft and hard palate that are covered by the lining (ie mucous membrane) of the roof of the mouth. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSubmucous cleft of soft and hard palate

Conditions with this feature

Mullegama-Klein-Martinez syndrome
MedGen UID:
1683985
•Concept ID:
C5193008
•
Disease or Syndrome
Mullegama-Klein-Martinez syndrome (MKMS) is an X-linked recessive disorder with features of microcephaly, microtia, hearing loss, developmental delay, dysmorphic features, congenital heart defect, and digit abnormalities. Females are generally affected more severely than males (Mullegama et al., 2019).
See: Condition Record
Mullegama-Klein-Martinez syndrome

Recent clinical studies

Etiology

Tissue adjuncts in primary cleft palate reconstruction: A systematic review.
Ku YC, Al-Malak M, Mulvihill L, Deleonibus A, Maasarani S, Bassiri Gharb B, Rampazzo A
J Plast Reconstr Aesthet Surg 2023 Nov;86:300-314. Epub 2023 Sep 16 doi: 10.1016/j.bjps.2023.09.038. PMID: 37797378
Incidence of Symptomatic Submucous Cleft Palate in the Netherlands: A Retrospective Cohort Study Over a Period of 22 Years.
Smit JA, Mulder PP, de Graaf F, de Bakker BS, Breugem CC
Cleft Palate Craniofac J 2021 Sep;58(9):1121-1127. Epub 2020 Dec 3 doi: 10.1177/1055665620977760. PMID: 33267609Free PMC Article
Analysis of human soft palate morphogenesis supports regional regulation of palatal fusion.
Danescu A, Mattson M, Dool C, Diewert VM, Richman JM
J Anat 2015 Oct;227(4):474-86. Epub 2015 Aug 24 doi: 10.1111/joa.12365. PMID: 26299693Free PMC Article
Submucous cleft palate.
Chen KT, Wu J, Noordhoff SM
Changgeng Yi Xue Za Zhi 1994 Jun;17(2):131-7. PMID: 8069735
The occult submucous cleft palate.
Kaplan EN
Cleft Palate J 1975 Oct;12:356-68. PMID: 1058746

Diagnosis

A Pitfall in Prenatal Ultrasonic Detection of Submucous Cleft Palate.
Hu JQ, Zhang YG, Feng W, Shi H
Ear Nose Throat J 2022 Nov;101(9):NP369-NP372. Epub 2020 Nov 23 doi: 10.1177/0145561320974867. PMID: 33226853
Incidence of Symptomatic Submucous Cleft Palate in the Netherlands: A Retrospective Cohort Study Over a Period of 22 Years.
Smit JA, Mulder PP, de Graaf F, de Bakker BS, Breugem CC
Cleft Palate Craniofac J 2021 Sep;58(9):1121-1127. Epub 2020 Dec 3 doi: 10.1177/1055665620977760. PMID: 33267609Free PMC Article
Bony defect of palate and vomer in submucous cleft palate patients.
Ren S, Ma L, Zhou X, Sun Z
Int J Oral Maxillofac Surg 2015 Jan;44(1):63-6. Epub 2014 Oct 23 doi: 10.1016/j.ijom.2014.09.001. PMID: 25442739
Submucous cleft palate.
Chen KT, Wu J, Noordhoff SM
Changgeng Yi Xue Za Zhi 1994 Jun;17(2):131-7. PMID: 8069735
The occult submucous cleft palate.
Kaplan EN
Cleft Palate J 1975 Oct;12:356-68. PMID: 1058746

Therapy

Tissue adjuncts in primary cleft palate reconstruction: A systematic review.
Ku YC, Al-Malak M, Mulvihill L, Deleonibus A, Maasarani S, Bassiri Gharb B, Rampazzo A
J Plast Reconstr Aesthet Surg 2023 Nov;86:300-314. Epub 2023 Sep 16 doi: 10.1016/j.bjps.2023.09.038. PMID: 37797378
Validation of the Dutch registry of common oral clefts: quality of recording specific oral cleft features.
Rozendaal AM, Luijsterburg AJ, Mohangoo AD, Ongkosuwito EM, de Vries E, Vermeij-Keers C
Cleft Palate Craniofac J 2012 Sep;49(5):609-17. Epub 2010 Nov 23 doi: 10.1597/10-109. PMID: 21091368
Palatal fistulas after primary repair of clefts of the secondary palate.
Andersson EM, Sandvik L, Semb G, Abyholm F
Scand J Plast Reconstr Surg Hand Surg 2008;42(6):296-9. doi: 10.1080/02844310802299676. PMID: 18991171
Frequency of pharyngoplasty after primary repair of cleft palate.
Bicknell S, McFadden LR, Curran JB
J Can Dent Assoc 2002 Dec;68(11):688-92. PMID: 12513937
Partial adenoidectomy.
Shapiro RS
Laryngoscope 1982 Feb;92(2):135-9. doi: 10.1002/lary.1982.92.2.135. PMID: 7162308

Prognosis

Incidence of Symptomatic Submucous Cleft Palate in the Netherlands: A Retrospective Cohort Study Over a Period of 22 Years.
Smit JA, Mulder PP, de Graaf F, de Bakker BS, Breugem CC
Cleft Palate Craniofac J 2021 Sep;58(9):1121-1127. Epub 2020 Dec 3 doi: 10.1177/1055665620977760. PMID: 33267609Free PMC Article
Palatal fistulas after primary repair of clefts of the secondary palate.
Andersson EM, Sandvik L, Semb G, Abyholm F
Scand J Plast Reconstr Surg Hand Surg 2008;42(6):296-9. doi: 10.1080/02844310802299676. PMID: 18991171
Influence of the Furlow palatoplasty for patients with submucous cleft palate on facial growth.
Cho BC, Kim JY, Yang JD, Lee DG, Chung HY, Park JW
J Craniofac Surg 2004 Jul;15(4):547-54; discussion 555. doi: 10.1097/00001665-200407000-00004. PMID: 15213528
Submucous cleft palate with a discontinuous bony deformity.
Malata CM, Cooter RD, Batchelor AG
Cleft Palate Craniofac J 1993 Nov;30(6):590-2. doi: 10.1597/1545-1569_1993_030_0590_scpwad_2.3.co_2. PMID: 8280740

Clinical prediction guides

Relationship between palate-vomer development and maxillary growth in submucous cleft palate patients.
Ren S, Ma L, Sun Z, Qian J
Cleft Palate Craniofac J 2014 May;51(3):314-9. Epub 2012 Sep 20 doi: 10.1597/12-051. PMID: 22994963
Clefts of the secondary palate referred to the Oslo Cleft Team: epidemiology and cleft severity in 994 individuals.
Andersson EM, Sandvik L, Abyholm F, Semb G
Cleft Palate Craniofac J 2010 Jul;47(4):335-42. doi: 10.1597/07-230.1. PMID: 19860491
Dental arches in six-year-old children with operated and unoperated submucous cleft palate and isolated cleft palate.
Heliövaara A, Rautio J
Acta Odontol Scand 2005 Apr;63(2):123-6. doi: 10.1080/00016350510019766. PMID: 16134552
Middle ear disease in children with congenital velopharyngeal insufficiency.
Sheahan P, Miller I, Earley MJ, Sheahan JN, Blayney AW
Cleft Palate Craniofac J 2004 Jul;41(4):364-7. doi: 10.1597/03-085.1. PMID: 15222782
Influence of the Furlow palatoplasty for patients with submucous cleft palate on facial growth.
Cho BC, Kim JY, Yang JD, Lee DG, Chung HY, Park JW
J Craniofac Surg 2004 Jul;15(4):547-54; discussion 555. doi: 10.1097/00001665-200407000-00004. PMID: 15213528

Recent systematic reviews

Tissue adjuncts in primary cleft palate reconstruction: A systematic review.
Ku YC, Al-Malak M, Mulvihill L, Deleonibus A, Maasarani S, Bassiri Gharb B, Rampazzo A
J Plast Reconstr Aesthet Surg 2023 Nov;86:300-314. Epub 2023 Sep 16 doi: 10.1016/j.bjps.2023.09.038. PMID: 37797378

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