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Isolated cleft palate(CP; CPI)

MedGen UID:
332392
•Concept ID:
C1837218
•
Congenital Abnormality
Synonym: nonsyndromic cleft palate
 
Monarch Initiative: MONDO:0007336
OMIM®: 119540

Definition

Cleft palate as an isolated malformation behaves as an entity distinct from cleft lip with or without cleft palate (see 119530). Dominantly inherited cleft soft palate in 4 generations has been reported (Jenkins and Stady, 1980); see 119570. [from OMIM]

Clinical features

From HPO
Seizure
MedGen UID:
20693
•Concept ID:
C0036572
•
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
See: Feature record | Search on this feature
Intellectual disability, profound
MedGen UID:
892508
•Concept ID:
C3161330
•
Mental or Behavioral Dysfunction
Profound mental retardation is defined as an intelligence quotient (IQ) below 20.
See: Feature record | Search on this feature
Micrognathia
MedGen UID:
44428
•Concept ID:
C0025990
•
Congenital Abnormality
Developmental hypoplasia of the mandible.
See: Feature record | Search on this feature
Anterior open-bite malocclusion
MedGen UID:
120566
•Concept ID:
C0266060
•
Finding
Anterior open bite is a malocclusion characterized by a gap between the anterior teeth (incisors), that is, by a deficiency in the normal vertical overlap between antagonist incisal edges when the posterior teeth are in occlusion.
See: Feature record | Search on this feature
Increased overbite
MedGen UID:
539656
•Concept ID:
C0266063
•
Disease or Syndrome
Maxillary teeth cover the mandibular teeth when biting to an increased degree. The feature is defined as a vertical overlap of the maxillary incisors over the mandibular incisors that exceeds 2 mm.
See: Feature record | Search on this feature
Gingival overgrowth
MedGen UID:
87712
•Concept ID:
C0376480
•
Finding
Hyperplasia of the gingiva (that is, a thickening of the soft tissue overlying the alveolar ridge. The degree of thickening ranges from involvement of the interdental papillae alone to gingival overgrowth covering the entire tooth crown.
See: Feature record | Search on this feature
Cleft palate
MedGen UID:
756015
•Concept ID:
C2981150
•
Congenital Abnormality
Cleft palate is a developmental defect of the palate resulting from a failure of fusion of the palatine processes and manifesting as a separation of the roof of the mouth (soft and hard palate).
See: Feature record | Search on this feature
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Professional guidelines

PubMed

Orofacial clefts: Reflections on prenatal diagnosis and family history based on a series of cases of a tertiary children hospital.
Moreira T, Dias M, Von Hafe M, Curval AR, Ramalho C, Maia AM, Moura CP; Orofacial Cleft Team of University Hospital Center of São João, EPE
Congenit Anom (Kyoto) 2023 Nov;63(6):195-199. Epub 2023 Aug 31 doi: 10.1111/cga.12538. PMID: 37653578
Cleft Lip and Palate in Newborns Diagnosed With Neonatal Abstinence Syndrome.
Danis DO 3rd, Bachrach K, Piraquive J, Marston AP, Levi JR
Otolaryngol Head Neck Surg 2021 Jan;164(1):199-205. Epub 2020 Jul 21 doi: 10.1177/0194599820944899. PMID: 32689883
The prenatal diagnosis and classification of cleft palate: the role and value of magnetic resonance imaging.
Zheng W, Li B, Zou Y, Lou F
Eur Radiol 2019 Oct;29(10):5600-5606. Epub 2019 Mar 18 doi: 10.1007/s00330-019-06089-9. PMID: 30887208

Recent clinical studies

Etiology

Timing of Primary Surgery for Cleft Palate.
Gamble C, Persson C, Willadsen E, Albery L, Soegaard Andersen H, Zattoni Antoneli M, Appelqvist M, Aukner R, Bodling P, Bowden M, Brunnegård K, Cairns G, Calladine S, Campbell L, Clayton-Smith J, Cooper R, Conroy E, El-Angbawi A, Kildegaard Emborg B, Enfält Wikman J, Fitzpatrick B, Fukushiro AP, Guedes de Azevedo Bento Gonçalves C, Havstam C, Hvistendahl AK, Jorgensen LD, Klinto K, Berntsen Kvinnsland M, Larham C, Lemvik J, Leturgie L, Liljerehn E, Lodge N, Lohmander A, McMahon S, Mehendale F, Miguel HC, Moe M, Nielsen JB, Nyberg J, Pedersen NH, Phippen G, Alvarez Piazentin-Penna SH, Patrick K, Pliskin L, Rigby L, Semb G, Southby L, Sporre M, Björkman Taleman AS, Tangstad J, Trindade IEK, Underwood I, van Eeden S, Westberg LR, Williamson PR, Paciello Yamashita R, Munro K, Walsh T, Shaw W; TOPS Study Group
N Engl J Med 2023 Aug 31;389(9):795-807. doi: 10.1056/NEJMoa2215162. PMID: 37646677Free PMC Article
Do Infants With Robin Sequence Have Improved Feeding and Weight Gain After Mandibular Distraction?
Harris JA, Caprio RM, Resnick CM
J Oral Maxillofac Surg 2021 Jun;79(6):1331-1338. Epub 2021 Jan 28 doi: 10.1016/j.joms.2021.01.029. PMID: 33631135
Use of ondansetron during pregnancy and the risk of major congenital malformations: A systematic review and meta-analysis.
Kaplan YC, Richardson JL, Keskin-Arslan E, Erol-Coskun H, Kennedy D
Reprod Toxicol 2019 Jun;86:1-13. Epub 2019 Mar 5 doi: 10.1016/j.reprotox.2019.03.001. PMID: 30849498
Cleft lip and palate.
Mossey PA, Little J, Munger RG, Dixon MJ, Shaw WC
Lancet 2009 Nov 21;374(9703):1773-85. Epub 2009 Sep 9 doi: 10.1016/S0140-6736(09)60695-4. PMID: 19747722
Audiologic findings in Pierre Robin sequence.
Gould HJ
Ear Hear 1989 Jun;10(3):211-3. doi: 10.1097/00003446-198906000-00013. PMID: 2744260

Diagnosis

Timing of Primary Surgery for Cleft Palate.
Gamble C, Persson C, Willadsen E, Albery L, Soegaard Andersen H, Zattoni Antoneli M, Appelqvist M, Aukner R, Bodling P, Bowden M, Brunnegård K, Cairns G, Calladine S, Campbell L, Clayton-Smith J, Cooper R, Conroy E, El-Angbawi A, Kildegaard Emborg B, Enfält Wikman J, Fitzpatrick B, Fukushiro AP, Guedes de Azevedo Bento Gonçalves C, Havstam C, Hvistendahl AK, Jorgensen LD, Klinto K, Berntsen Kvinnsland M, Larham C, Lemvik J, Leturgie L, Liljerehn E, Lodge N, Lohmander A, McMahon S, Mehendale F, Miguel HC, Moe M, Nielsen JB, Nyberg J, Pedersen NH, Phippen G, Alvarez Piazentin-Penna SH, Patrick K, Pliskin L, Rigby L, Semb G, Southby L, Sporre M, Björkman Taleman AS, Tangstad J, Trindade IEK, Underwood I, van Eeden S, Westberg LR, Williamson PR, Paciello Yamashita R, Munro K, Walsh T, Shaw W; TOPS Study Group
N Engl J Med 2023 Aug 31;389(9):795-807. doi: 10.1056/NEJMoa2215162. PMID: 37646677Free PMC Article
Incidence of New-Onset Obstructive Sleep Apnea After Posterior Flap Pharyngoplasty in Children.
Trabelsi I, Amaddeo A, Michel B, Khirani S, Picard A, Fauroux B
Ann Plast Surg 2022 Aug 1;89(2):180-184. Epub 2021 Oct 8 doi: 10.1097/SAP.0000000000003026. PMID: 34670983
Cleft Lip and Palate in Newborns Diagnosed With Neonatal Abstinence Syndrome.
Danis DO 3rd, Bachrach K, Piraquive J, Marston AP, Levi JR
Otolaryngol Head Neck Surg 2021 Jan;164(1):199-205. Epub 2020 Jul 21 doi: 10.1177/0194599820944899. PMID: 32689883
Radiology of Cleft Lip and Palate: Imaging for the Prenatal Period and throughout Life.
Abramson ZR, Peacock ZS, Cohen HL, Choudhri AF
Radiographics 2015 Nov-Dec;35(7):2053-63. doi: 10.1148/rg.2015150050. PMID: 26562237
Children with cleft lip/palate and mental retardation: a subpopulation of cleft-craniofacial team patients.
Strauss RP, Broder H
Cleft Palate Craniofac J 1993 Nov;30(6):548-56. doi: 10.1597/1545-1569_1993_030_0548_cwclpa_2.3.co_2. PMID: 8280732

Therapy

Timing of Primary Surgery for Cleft Palate.
Gamble C, Persson C, Willadsen E, Albery L, Soegaard Andersen H, Zattoni Antoneli M, Appelqvist M, Aukner R, Bodling P, Bowden M, Brunnegård K, Cairns G, Calladine S, Campbell L, Clayton-Smith J, Cooper R, Conroy E, El-Angbawi A, Kildegaard Emborg B, Enfält Wikman J, Fitzpatrick B, Fukushiro AP, Guedes de Azevedo Bento Gonçalves C, Havstam C, Hvistendahl AK, Jorgensen LD, Klinto K, Berntsen Kvinnsland M, Larham C, Lemvik J, Leturgie L, Liljerehn E, Lodge N, Lohmander A, McMahon S, Mehendale F, Miguel HC, Moe M, Nielsen JB, Nyberg J, Pedersen NH, Phippen G, Alvarez Piazentin-Penna SH, Patrick K, Pliskin L, Rigby L, Semb G, Southby L, Sporre M, Björkman Taleman AS, Tangstad J, Trindade IEK, Underwood I, van Eeden S, Westberg LR, Williamson PR, Paciello Yamashita R, Munro K, Walsh T, Shaw W; TOPS Study Group
N Engl J Med 2023 Aug 31;389(9):795-807. doi: 10.1056/NEJMoa2215162. PMID: 37646677Free PMC Article
Cleft Lip and/or Palate in Infants Prenatally Exposed to Opioids.
Proctor-Williams K, Louw B
Cleft Palate Craniofac J 2022 Apr;59(4):513-521. Epub 2021 May 7 doi: 10.1177/10556656211013687. PMID: 33960243
Cleft Lip and Palate in Infants With Prenatal Opioid Exposure.
Langa O, Cappitelli AT, Ganske IM
Cleft Palate Craniofac J 2022 Apr;59(4):497-504. Epub 2021 Apr 28 doi: 10.1177/10556656211011896. PMID: 33906463
Use of ondansetron during pregnancy and the risk of major congenital malformations: A systematic review and meta-analysis.
Kaplan YC, Richardson JL, Keskin-Arslan E, Erol-Coskun H, Kennedy D
Reprod Toxicol 2019 Jun;86:1-13. Epub 2019 Mar 5 doi: 10.1016/j.reprotox.2019.03.001. PMID: 30849498
A revised method of conservative palatoplasty.
Ohishi M, Miyanoshita Y, Suzuki A, Goto K
J Craniomaxillofac Surg 1992 Jul;20(5):198-202. doi: 10.1016/s1010-5182(05)80315-4. PMID: 1401090

Prognosis

Incidence of New-Onset Obstructive Sleep Apnea After Posterior Flap Pharyngoplasty in Children.
Trabelsi I, Amaddeo A, Michel B, Khirani S, Picard A, Fauroux B
Ann Plast Surg 2022 Aug 1;89(2):180-184. Epub 2021 Oct 8 doi: 10.1097/SAP.0000000000003026. PMID: 34670983
Do Infants With Robin Sequence Have Improved Feeding and Weight Gain After Mandibular Distraction?
Harris JA, Caprio RM, Resnick CM
J Oral Maxillofac Surg 2021 Jun;79(6):1331-1338. Epub 2021 Jan 28 doi: 10.1016/j.joms.2021.01.029. PMID: 33631135
Cleft Lip and Palate in Newborns Diagnosed With Neonatal Abstinence Syndrome.
Danis DO 3rd, Bachrach K, Piraquive J, Marston AP, Levi JR
Otolaryngol Head Neck Surg 2021 Jan;164(1):199-205. Epub 2020 Jul 21 doi: 10.1177/0194599820944899. PMID: 32689883
Cleft lip and palate: review.
Ciminello FS, Morin RJ, Nguyen TJ, Wolfe SA
Compr Ther 2009 Spring;35(1):37-43. PMID: 19351103
Cleft palate: confirmation of prenatal diagnosis by colour Doppler ultrasound.
Sherer DM, Abramowicz JS, Jaffe R, Woods JR Jr
Prenat Diagn 1993 Oct;13(10):953-6. doi: 10.1002/pd.1970131009. PMID: 8309902

Clinical prediction guides

Timing of Primary Surgery for Cleft Palate.
Gamble C, Persson C, Willadsen E, Albery L, Soegaard Andersen H, Zattoni Antoneli M, Appelqvist M, Aukner R, Bodling P, Bowden M, Brunnegård K, Cairns G, Calladine S, Campbell L, Clayton-Smith J, Cooper R, Conroy E, El-Angbawi A, Kildegaard Emborg B, Enfält Wikman J, Fitzpatrick B, Fukushiro AP, Guedes de Azevedo Bento Gonçalves C, Havstam C, Hvistendahl AK, Jorgensen LD, Klinto K, Berntsen Kvinnsland M, Larham C, Lemvik J, Leturgie L, Liljerehn E, Lodge N, Lohmander A, McMahon S, Mehendale F, Miguel HC, Moe M, Nielsen JB, Nyberg J, Pedersen NH, Phippen G, Alvarez Piazentin-Penna SH, Patrick K, Pliskin L, Rigby L, Semb G, Southby L, Sporre M, Björkman Taleman AS, Tangstad J, Trindade IEK, Underwood I, van Eeden S, Westberg LR, Williamson PR, Paciello Yamashita R, Munro K, Walsh T, Shaw W; TOPS Study Group
N Engl J Med 2023 Aug 31;389(9):795-807. doi: 10.1056/NEJMoa2215162. PMID: 37646677Free PMC Article
Do Infants With Robin Sequence Have Improved Feeding and Weight Gain After Mandibular Distraction?
Harris JA, Caprio RM, Resnick CM
J Oral Maxillofac Surg 2021 Jun;79(6):1331-1338. Epub 2021 Jan 28 doi: 10.1016/j.joms.2021.01.029. PMID: 33631135
Cleft Lip and Palate in Newborns Diagnosed With Neonatal Abstinence Syndrome.
Danis DO 3rd, Bachrach K, Piraquive J, Marston AP, Levi JR
Otolaryngol Head Neck Surg 2021 Jan;164(1):199-205. Epub 2020 Jul 21 doi: 10.1177/0194599820944899. PMID: 32689883
Use of ondansetron during pregnancy and the risk of major congenital malformations: A systematic review and meta-analysis.
Kaplan YC, Richardson JL, Keskin-Arslan E, Erol-Coskun H, Kennedy D
Reprod Toxicol 2019 Jun;86:1-13. Epub 2019 Mar 5 doi: 10.1016/j.reprotox.2019.03.001. PMID: 30849498
Analysis of the Relationship Between Micrognathia and Cleft Palate: A Systematic Review.
Price KE, Haddad Y, Fakhouri WD
Cleft Palate Craniofac J 2016 Mar;53(2):e34-44. Epub 2015 Feb 6 doi: 10.1597/14-238. PMID: 25658963

Recent systematic reviews

A systematic review of differences in outcome between one and two stage palate repair in cleft lip and palate.
Cornefjord M, Arnebrant K, Guné H, Holst J, Klintö K, Stiernman M, Svensson H, Wiedel AP, Becker M
J Plast Surg Hand Surg 2023 Dec 14;58:132-141. doi: 10.2340/jphs.v58.13368. PMID: 38095226
Growth and prevalence of feeding difficulties in children with Robin sequence: a retrospective cohort study.
Paes EC, de Vries IAC, Penris WM, Hanny KH, Lavrijsen SW, van Leerdam EK, Rademaker MM, Veldhoen ES, Eijkemans RMJC, Kon M, Breugem CC
Clin Oral Investig 2017 Jul;21(6):2063-2076. Epub 2016 Nov 21 doi: 10.1007/s00784-016-1996-8. PMID: 27868158Free PMC Article
Assessment of Chromosome 22q11.2 Deletion in Patients with Isolated Cleft Palate: A Systematic Review of Prospective Studies.
Panamonta V, Wichajarn K, Wongswadiwat Y, Panamonta M, Pradubwong S, Chowchuen B
J Med Assoc Thai 2016 Aug;99 Suppl 5:S194-8. PMID: 29906081
Analysis of the Relationship Between Micrognathia and Cleft Palate: A Systematic Review.
Price KE, Haddad Y, Fakhouri WD
Cleft Palate Craniofac J 2016 Mar;53(2):e34-44. Epub 2015 Feb 6 doi: 10.1597/14-238. PMID: 25658963
A systematic review comparing Furlow double-opposing Z-plasty and straight-line intravelar veloplasty methods of cleft palate repair.
Timbang MR, Gharb BB, Rampazzo A, Papay F, Zins J, Doumit G
Plast Reconstr Surg 2014 Nov;134(5):1014-1022. doi: 10.1097/PRS.0000000000000637. PMID: 25347635

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