A Review Of Referral Patterns For Sagittal Synostosis In Ireland: 2008-2013.

Sagittal synostosis (SS) is the commonest form of craniosynostosis. Children with sagittal synostosis in Ireland are treated in the National Paediatric Craniofacial Centre (NPCC) in Temple Street Children's University Hospital. This retrospective study analysed the correlation between referral patterns to the unit and age at operation. The notes of 81 patients referred over a 5-year period (April 2008 - April 2013) to the NPCC with non-syndromic SS were reviewed and demographics and referral information were recorded. Of 81 patients reviewed, 60 (74%) were referred before 6 months of age, while 21 (26%) had late referrals. Neonatologists referred 100% of infants before 6 months, paediatricians referred 71%, and GPs 64%. Later referral was associated with a more complex referral pathway, including multiple-steps of referral and unnecessary investigations. Improved clinician knowledge and emphasis on the importance of early referral may lead to a reduction in late referrals.

22q11 chromosome abnormalities and the cleft service.

Deletion of chromosome 22q11 gives rise to a spectrum of anomalies, including cleft palate. These are grouped together as the DiGeorge or velocardiofacial syndrome. Patients with this chromosomal abnormality account for a small, but noteworthy proportion of patients attending our cleft service. They frequently have other significant comorbidities consistent with their diagnosis. Over a ten-year period, 16 patients within our cleft service have been diagnosed, using chromosome analysis, as having deletions at 22q11. All had either a cleft palate and/or velopharyngeal incompetence, for which they underwent repair of the cleft palate or pharyngoplasty. Several have required secondary palate surgery following initial palate surgery. Poor quality of speech was the indication for secondary procedures in the majority of cases. Fourteen of the 16 have other comorbidities, ranging from congenital heart disease to ocular abnormalities. In addition, 15 of the 16 have developmental delays and/or learning difficulties. Other specialties, such as ENT, cardiology, genetics and ophthalmology have been involved in the care of all these patients. Although comprising only a small proportion of patients attending a cleft team, the diagnosis of this chromosomal abnormality is significant, as these patients may require substantial input of resources and the expertise of several specialties. Early recognition of features of this entity and diagnosis can aid more efficient intervention.

Sagittal synostosis: a review of 53 cases of sagittal suturectomy in one unit.

The role of sagittal suturectomy in the treatment of scaphocephaly remains controversial. The purpose of this study was to report the experience of the National Craniofacial Unit in Dublin. This unit is the referral centre for the Republic of Ireland and serves a population of approximately 3.5 million. Quantitative assessments were made using radiographs and the pre- and postoperative cephalic index (CI) was calculated. Patients were asked to score their head shapes in a questionnaire. The mean age at surgery for the 53 children was 4.9 months with a mean follow up of 8 years. Pre- and postoperative radiographs were available for analysis in 24 patients. The mean preoperative CI was 65.3. There was a statistically significant increase in the CI postoperatively to 73.7. Seventy five per cent of children achieved a 'normal' CI and 86% scored their head shapes as good to excellent. There was no correlation between the age at surgery and the CI achieved. Although only one child was not happy with the shape of the head, the impression was that the CI correlated poorly with the clinical assessment of the head shape. There were no significant complications. We therefore conclude that the sagittal suturectomy is a safe procedure that has a limited but definite potential to improve the CI. This procedure still has a place in the surgical repertoire of the craniofacial surgeon dealing with scaphocephaly.