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The Incidence and Outcomes for Children with Cleft Palate and/or Lip and CHARGE Syndrome
Mohammed Farid1, Louise Cafferky1, Jeremy Kirk2
1West Midlands Cleft Centre, Birmingham Children's Hospital, Birmingham, UK.
Insights
Children with cleft lip/palate and CHARGE syndrome often have severe medical issues, leading to delayed surgeries. Outcomes for feeding and communication were better in older children.
Area of Science:
- Pediatric surgery
- Genetics
- Craniofacial anomalies
Background:
- Literature on children with cleft palate and/or lip (CP +/-L) and CHARGE syndrome (CS) is limited.
- This population presents unique challenges in surgical management and developmental outcomes.
Purpose of the Study:
- To investigate cleft characteristics, surgical timing, and feeding/communication outcomes in children with CP +/-L and CS.
- To identify factors influencing outcomes in this complex patient group.
Main Methods:
- Retrospective cross-sectional review of patients diagnosed with CP +/-L and CS between 1989-2019.
- Data collected included cleft type, timing of surgical repair, feeding methods, and communication modalities.
Main Results:
- Twenty-two children with CP +/-L and CS were identified, with bilateral cleft lip and palate (BCLP) being the most common type (50%).
- Cleft repair was significantly delayed compared to non-syndromic controls, with median ages of 9 months for lip repair and 21 months for palate repair.
- Only 27% achieved full oral feeding and verbal communication; outcomes improved in children over ten years old.
Conclusions:
- Children with CP +/-L and CS exhibit severe cleft types and significant medical comorbidities, contributing to delayed surgical interventions.
- Optimizing feeding and communication requires long-term, multidisciplinary management, with better outcomes observed in older children.
Objectives:
Published literature on children with cleft palate and/or lip (CP + /-L) and CHARGE syndrome (CS) is limited. This study investigated cleft characteristics including surgery, and feeding and communication outcomes in children identified with CP + /-L and CS.
Design:
Retrospective cross-sectional review.
Setting:
Regional Referral Centre for Paediatric Cleft Surgery.
Patients:
All children diagnosed with CP + /-L and CS (based on clinical features and/or CHD7 mutation testing) between 1989-2019.
Main Outcome Measures:
Cleft type, timing of CP + /-L repair, reasons for 'delayed' repair, feeding methods and communication modality.
Results:
Twenty-two children with CP + /-L and CS were identified. Cleft sub-types (%) were: Eleven (50%) had bilateral cleft lip and palate (BCLP), six (27%) had unilateral cleft lip and palate (UCLP) and five (23%) had cleft palate (CP). Cleft repair was delayed compared to protocol care for non-syndromic children with CP + /-L. Median age for lip repair + /- vomerine flap was 9 months (range 4-22 months), and palate repair was 21 months (range 11-40 months). Median age for isolated CP repair was 13 months (range 7-23). Surgery for cardiac anomalies (36%) before cleft repair, and (59%) were classed as having severe systemic disease at the time of cleft surgery. Only 27% of the children in this study had both full oral feeding and verbal communication.
Conclusions:
Children with CP + /-L and CS had severe cleft types and complex medical problems leading to delayed cleft surgery. Feeding and speech outcomes were better in the children aged over ten years.
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