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Range and Frequency of Congenital Malformations Among Children With Cleft Lip and/or Palate
K J Fitzsimons1, M J Hamilton2, J van der Meulen3
1Clinical Effectiveness Unit, Royal College of Surgeons of England, London, UK.
Insights
Congenital malformations are frequent in children with cleft lip and palate (CL/P), impacting nearly 40% of cases. Specific cleft types, like cleft palate (CP), show higher rates, necessitating targeted screening and integrated care.
Area of Science:
- Pediatric Health
- Medical Genetics
- Public Health
Background:
- Cleft lip and/or palate (CL/P) are common birth defects.
- Additional congenital malformations frequently co-occur with CL/P, impacting long-term health outcomes.
- Understanding the spectrum and frequency of these co-occurring conditions is crucial for comprehensive patient care.
Purpose of the Study:
- To determine the range and frequency of additional congenital malformations in live-born infants with CL/P.
- To analyze the prevalence of these malformations based on specific cleft types.
- To identify common co-occurring malformation types and affected systems.
Main Methods:
- Analysis of patient-level data from a national cleft birth registry linked to hospital admission records in England.
- Inclusion of children born between 2000 and 2012 who received cleft care within the NHS.
- Assessment of ICD-10 codes for additional congenital malformations and their correlation with cleft type.
Main Results:
- The study included 9403 children; 38.8% had documented additional congenital malformations.
- Prevalence varied by cleft type: Cleft palate (CP) at 53.0%, bilateral cleft lip and palate (BCLP) at 33.5%, unilateral cleft lip and palate (UCLP) at 26.3%, and cleft lip with or without alveolus (CL±A) at 22.2%.
- Skeletal and circulatory system malformations were most common (10.5% and 10.2% respectively); 16.8% had malformations affecting multiple systems.
Conclusions:
- Congenital malformations are common in children with CL/P, particularly in certain subgroups.
- Clinicians should consider standardized screening for specific structural malformations based on prevalence data.
- Enhanced collaboration with pediatric and genetic services is recommended for optimal management.
Abstract:
To assess the range and frequency of additional congenital malformations identified among children born alive with CL/P.
Abstract:
Analysis of patient-level data from a national registry of cleft births linked to national administrative data of hospital admissions.
Abstract:
National Health Service, England.
Abstract:
Children born between 2000 and 2012 receiving cleft care in English NHS hospitals.
Abstract:
The proportion of children with ICD-10 codes for additional congenital malformations, according to cleft type.
Abstract:
The study included 9403 children. Of these 2114 (22.5%) had CL±A, 4509 (48.0%) had CP, 1896 (20.2%) had UCLP, and 884 (9.4%) had BCLP. A total of 3653 (38.8%) children had additional congenital malformations documented in their hospital admission records. The prevalence of additional congenital malformations was greatest among children with CP (53.0%), followed by those with BCLP (33.5%), UCLP (26.3%), and then CL±A (22.2%) (P < .001). Among those with UCLP, children with right-sided clefts were more likely to have additional malformations than those with left-sided clefts (31.6% vs 23.0%, P < .001). Malformations of the skeletal system and circulatory system were most common, affecting 10.5% and 10.2% of the included children, respectively. A total of 16.8% of children had additional congenital malformations affecting 2 or more structural systems.
Abstract:
Congenital malformations are common among children born alive with a cleft, affecting over half of some cleft subgroups. Given the frequency of certain structural malformations, clinicians should consider standardized screening for these children. Establishing good links with pediatric and genetic services is recommended.
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