Nonsyndromic Craniosynostosis: Referral Patterns For Metopic, Coronal And Lambdoid Craniosynostosis In Ireland; 2009-2018, A 10-Year Review

Background Craniosynostosis is a congenital condition resulting from premature fusion of the cranial sutures resulting in a phenotypic headshape. Nonsyndromic craniosynostosis, involving a single suture only; is the most common presentation involving the fusion of one of the following sutures; sagittal, metopic, coronal or lambdoid. The National Paediatric Craniofacial Centre in Temple Street Children’s University Hospital is the designated craniofacial centre in the Republic of Ireland. The treatment of craniosynostosis involves appearance-changing surgery designed to normalise the headshape. As with most other designated craniofacial centres the aim is to perform surgery as close to 12 months of age as possible for these children. The clinical presentation of craniosynostosis as with most rare diseases can result in an over-reliance on different types of radiology imaging, a subsequent later referral and surgery being performed at a later age. The aim of this review was to report the referral patterns of children with metopic, coronal and lambdoid nonsyndromic craniosynostosis presenting to the NPCC. Methods A retrospective chart review of children who underwent surgery for metopic, coronal or lambdoid craniosynostosis between January 2009 and December 2018 was undertaken. Data collected included: patient demographics, hospital of birth, referrer specialty, date of referral and any radiology investigations performed. Children subsequently diagnosed with a syndrome were excluded. Results 130 patients were included in the review. 80% (n=104) were referred before 10 months of age. There was a high use of radiology imaging with 71% (n= 92) of all children having at least one radiology investigation prior to referral. 15% (n=19) had multiple radiology investigations performed. Paediatricians and Neonatologists referred the largest number of children; 58% (n=75) and 22% (n=28) respectively. Surgery was performed before 15 months of age for 44% (n=57) of children and before 18 months of age for a further 26% (n=34) of all children. Conclusion The incidence of metopic, coronal and lambdoid craniosynostosis is in keeping with reported rates in Europe. Although most children were referred before 10 months of age complex referral patterns and use of radiology imaging were associated with later referrals, leading to subsequent delays in surgery performed. It is hoped that an increased awareness of recognising and referring children with craniosynostosis will lead to subsequent treatment as early as possible. The NPCC encourages that referral letters for children with suspected craniosynostosis are accompanied by photographs of the child’s headshape and not radiology investigations.