To determine whether children born with a cleft palate ± lip (CP ± L) and additional congenital differences (ACDs – including ‘Congenital malformations and deformations’ as coded in ICD-10), are less likely to meet the three national speech outcome standards at age five compared to children with CP ± L and no ACDs.
Design
An observational study, utilizing national data from the UK Cleft Registry and Audit NEtwork (CRANE) Database linked to national administrative data of hospital admissions.
Setting
National Health Service, England.
Patients
2191 children (993 female, 1198 male) with CP ± L, born 2006-2012 in England, with complete speech data and consent to data linkage.
Outcome Measure
Perceptual speech analysis utilised the Cleft Audit Protocol for Speech – Augmented (CAPS-A) rating and United Kingdom National Speech Outcome Standards (Speech Standard 1 (SS1), Speech Standard 2a (SS2a) and Speech Standard 3 (SS3)).
Results
Of 2191 children, 759 (35%) had at least one ACD. Presence of one ACD did not significantly impact speech outcomes but two or more ACDs reduced the odds of achieving all three speech standards: SS1 aOR 0.602 (CI 0.45-0.82, P = .002), SS2a aOR 0.563 (CI 0.41-0.77, P = .001), SS3 aOR 0.606 (0.43-0.84, P = .003). When exploring ACDs by ICD-10 groupings, congenital malformations of the ‘Eye, ear, face and neck’, ‘Circulatory system’, ‘Digestive system’, ‘Musculoskeletal system’ and ‘Other congenital malformations’ reduced a child's odds of achieving the speech standards.
Conclusions
ACDs, in the absence of a known syndrome, have a significant impact on speech outcome at age five. Incorporating these factors into risk-adjustment models for service level outcome reporting is recommended.
MosseyPAModellB. Epidemiology of oral clefts 2012: An international perspective. Front Oral Biol. 2012;16:1-18. doi: https://doi.org/10.1159/000337464
2.
SprangerJBenirschkeKHallJG, et al.Errors of morphogenesis: Concepts and terms. Recommendations of an international working group. J Pediatr. 1982;100(1):160-165. doi: https://doi.org/10.1016/s0022-3476(82)80261-8
3.
International classification of diseases tenth revision (ICD-10). 6th ed. World Health Organisation; 2019.
4.
CalzolariEPieriniAAstolfiG, et al.Associated anomalies in multi-malformed infants with cleft lip and palate: An epidemiologic study of nearly 6 million births in 23 EUROCAT registries. Am J Med Genet A. 2007;143A(6):528-537. doi: https://doi.org/10.1002/ajmg.a.31447
5.
FitzsimonsKJHamiltonMJvan der MeulenJ, et al.Range and frequency of congenital malformations among children with cleft lip and/or palate. Cleft Palate-Craniofac J: Off Publicat Am Cleft Palate-Craniofac Assoc. 2023;60(8):917-927. doi: https://doi.org/10.1177/10556656221089160[published Online First: 2022 Apr 5].
6.
ImpellizzeriAGiannantoniIPolimeniA, et al.Epidemiological characteristic of orofacial clefts and its associated congenital anomalies: Retrospective study. BMC Oral Health. 2019;19(1):290. doi: https://doi.org/10.1186/s12903-019-0980-5
7.
MileradJLarsonOHagbergC, et al.Associated malformations in infants with cleft lip and palate: A prospective, population-based study. Pediatrics. 1997;100(2Pt 1):180-186. doi: https://doi.org/10.1542/peds.100.2.180
8.
PereiraAVFradinhoNCarmoS, et al.Associated malformations in children with orofacial clefts in Portugal: A 31-year study. Plast Reconstr Surg Glob Open. 2018;6(2):e1635. doi: https://doi.org/10.1097/GOX.0000000000001635
9.
SekhonPSEthunandanMMarkusAF, et al.Congenital anomalies associated with cleft lip and palate-an analysis of 1623 consecutive patients. Cleft Palate-Craniofac J: Off Publicat Am Cleft Palate-Craniofac Assoc. 2011;48(4):371-378. doi: https://doi.org/10.1597/09-264
10.
ShprintzenRJSiegel-SadewitzVLAmatoJ, et al.Anomalies associated with cleft lip, cleft palate, or both. Am J Med Genet. 1985;20(4):585-595. doi: https://doi.org/10.1002/ajmg.1320200404
Vallino-NapoliLDRileyMMHallidayJL. An epidemiologic study of orofacial clefts with other birth defects in Victoria, Australia. Cleft Palate Craniofac J. 2006;43(5):571-576. doi: https://doi.org/10.1597/05-123
ZhouQJShiBShiZD, et al.Survey of the patients with cleft lip and palate in China who were funded for surgery by the smile train program from 2000 to 2002. Chin Med J (Engl). 2006;119(20):1695-1700.
15.
LohmanderAPerssonCWilladsenE, et al.Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 4. Speech outcomes in 5-year-olds – velopharyngeal competency and hypernasality. J Plast Surg Hand Surg. 2017;51(1):27-37. doi: https://doi.org/10.1080/2000656X.2016.1254645
SellDSouthbyLWrenY, et al.Centre-level variation in speech outcome and interventions, and factors associated with poor speech outcomes in 5-year-old children with non-syndromic unilateral cleft lip and palate: The cleft care UK study. Part 4. Orthod Craniofac Res. 2017;20(Suppl 2):27-39. doi: https://doi.org/10.1111/ocr.12186
18.
WilladsenELohmanderAPerssonC, et al.Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 5. Speech outcomes in 5-year-olds – consonant proficiency and errors. J Plast Surg Hand Surg. 2017;51(1):38-51. doi: https://doi.org/10.1080/2000656X.2016.1254647
19.
BastaMNSilvestreJStranskyC, et al.A 35-year experience with syndromic cleft palate repair: Operative outcomes and long-term speech function. Ann Plast Surg. 2014;73(Suppl 2):S130-S135. doi: https://doi.org/10.1097/SAP.0000000000000286
20.
KlintoKSporreMBeckerM. Speech in a consecutive series of children born with cleft lip and palate with and without syndromes and/or additional malformations. BMC Pediatr. 2021;21(1):309. doi: https://doi.org/10.1186/s12887-021-02783-0
21.
PerssonCElanderALohmander-AgerskovA, et al.Speech outcomes in isolated cleft palate: Impact of cleft extent and additional malformations. Cleft Palate-Craniofac J: Off Publicat Am Cleft Palate-Craniofac Assoc. 2002;39(4):397-408. doi: https://doi.org/10.1597/1545-1569_2002_039_0397_soiicp_2.0.co_2
22.
StranskyCBastaMSolotC, et al.Do patients with pierre robin sequence have worse outcomes after cleft palate surgery?Ann Plast Surg. 2013;71(3):292-296. doi: https://doi.org/10.1097/SAP.0b013e3182898712
23.
Hospital Episode Statistics. HES user guide: Health and Social Care Information Centre. Accessed 14/07/23 2023. www.hscic.gov.uk/hes2010.
BrittonLAlberyLBowdenM, et al.A cross-sectional cohort study of speech in five-year-olds with cleft palate +/- lip to support development of national audit standards: Benchmarking speech standards in the United Kingdom. Cleft Palate-Craniofac J: Off Publicat Am Cleft Palate-Craniofac Assoc. 2014;51(4):431-451. doi: https://doi.org/10.1597/13-121[published Online First: 20140317].
26.
JohnASellDSweeneyT, et al.The cleft audit protocol for speech-augmented: A validated and reliable measure for auditing cleft speech. Cleft Palate-Craniofac J: Off Publicat Am Cleft Palate-Craniofac Assoc. 2006;43(3):272-288. doi: https://doi.org/10.1597/04-141.1
27.
SellDJohnAHarding-BellA, et al.Cleft audit protocol for speech (CAPS-A): A comprehensive training package for speech analysis. Int J Lang Commun Disord. 2009;44(4):529-548. doi: https://doi.org/10.1080/13682820802196815
28.
SellDMildinhallSAlberyL, et al.The cleft care UK study. Part 4: Perceptual speech outcomes. Orthod Craniofac Res. 2015;18(Suppl 2):36-46. doi: https://doi.org/10.1111/ocr.12112
29.
ButterworthSFitzsimonsKJMedinaJ, et al.Investigating the impact of patient-related factors on speech outcomes at 5 years of age in children with a cleft palate. Cleft Palate Craniofac J. 2023;60(12):1578-1590. doi: https://doi.org/10.1177/10556656221110094[published Online First: 2022 Jun 22].
30.
Hovels-GurichHHBauerSBSchnitkerR, et al.Long-term outcome of speech and language in children after corrective surgery for cyanotic or acyanotic cardiac defects in infancy. Eur J Paediatr Neurol. 2008;12(5):378-386. doi: https://doi.org/10.1016/j.ejpn.2007.10.004[published Online First: 20080124].
31.
RamananSSundaramSGopalakrishnanA, et al.Intermediate-term neurodevelopmental outcomes and quality of life after arterial switch operation beyond early neonatal period. Eur J Cardiothorac Surg. 2021;60(6):1428-1436. doi: https://doi.org/10.1093/ejcts/ezab223
32.
BellingerDC. Are children with congenital cardiac malformations at increased risk of deficits in social cognition?Cardiol Young. 2008;18(1):3-9. doi: https://doi.org/10.1017/S104795110700176X[published Online First: 2007].
FitzsimonsKJDeaconSACopleyLP, et al.School absence and achievement in children with isolated orofacial clefts. Arch Dis Child. 2021;106(2):154-159. doi: https://doi.org/10.1136/archdischild-2020-319123[published Online First: 2020 Aug 19].
35.
RawashdehMAJawdat Abu-HawasB. Congenital associated malformations in a sample of Jordanian patients with cleft lip and palate. J Oral Maxillofac Surg. 2008;66(10):2035-2041. doi: https://doi.org/10.1016/j.joms.2008.01.009
36.
SunTTianHWangC, et al.A survey of congenital heart disease and other organic malformations associated with different types of orofacial clefts in eastern China. PLoS One. 2013;8(1):e54726. doi: https://doi.org/10.1371/journal.pone.0054726[published Online First: 20130121].
37.
GerdesMSolotCWangPP, et al.Cognitive and behavior profile of preschool children with chromosome 22q11.2 deletion. Am J Med Genet. 1999;85(2):127-133.
BeckerDBPetersenJDKaneAA, et al.Speech, cognitive, and behavioral outcomes in nonsyndromic craniosynostosis. Plast Reconstr Surg. 2005;116(2):400-407. doi: https://doi.org/10.1097/01.prs.0000172763.71043.b8
40.
NaranSMillerMShakirS, et al.Nonsyndromic craniosynostosis and associated abnormal speech and language development. Plast Reconstr Surg. 2017;140(1):62e-69e. doi: https://doi.org/10.1097/PRS.0000000000003423
41.
PenningtonLGoldbartJMarshallJ. Direct speech and language therapy for children with cerebral palsy: Findings from a systematic review. Dev Med Child Neurol. 2005;47(1):57-63. doi: https://doi.org/10.1017/s0012162205000101
42.
JiangDMorrisonGA. The influence of long-term tracheostomy on speech and language development in children. Int J Pediatr Otorhinolaryngol. 2003;67(Suppl 1):S217-S220. doi: https://doi.org/10.1016/j.ijporl.2003.08.031
43.
de SouzaCDRPadovaniLFFerreira-DonatiGC, et al.Babies with pierre robin sequence: Neuropsychomotor development. Pediatr Neurol. 2023;141:72-76. doi: https://doi.org/10.1016/j.pediatrneurol.2023.01.010[published Online First: 20230121].
44.
CorbettSSDrewettRF. To what extent is failure to thrive in infancy associated with poorer cognitive development? A review and meta-analysis. J Child Psychol Psychiatry. 2004;45(3):641-654. doi: https://doi.org/10.1111/j.1469-7610.2004.00253.x
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