The Incidence and Outcomes for Children with Cleft Palate and/or Lip and CHARGE Syndrome.

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.