99 SURGICAL EXPERIENCE IN DOWN’S SYNDROME, A UNIQUE SINGLE CENTRE 10 YEAR EXPERIENCE.
Isaac Philip, Evelyn Ervine
Royal Belfast Hospital for Sick Children, Belfast, United Kingdom

Abstract

Aim of the Study

Down’s syndrome is associated with major congenital anomalies, often requiring paediatric surgical input as part of multidisciplinary care, with surgical outcomes thought to be worse than those in children without it. All children in our geographically isolated region requiring surgery come through a single paediatric surgical unit. Our birth rate for children with Down’s syndrome is 1.7 per 1000 live births, which is almost twice the UK average. This caseload represents a singular experience in their pre and post-natal diagnosis, antenatal counselling, subsequent surgery and follow-up.

We present our surgical experience in children with Down’s syndrome over a 10-year period along with immediate post-operative outcomes for major procedures.

Methods

A retrospective review of clinical notes, including electronic records of all children who had a post-natal diagnosis of Down’s syndrome, confirmed by chromosomal analysis; born between January 2006 and December 2015, was done.

Main Results

425 children with Down’s syndrome were identified over 10 years. 245 were male and 180 were female. 410 sets of notes could be reviewed (96 %).  67 (16%) children required surgery for 77 different conditions.  Figure 1 shows the incidence of surgical conditions. We present our early surgical outcome for   major conditions in this period.

Conclusion

 In our series, 16% required at least one surgical procedure. In comparison to other reported series, we report a higher incidence of Hirschsprung’s disease (2.35%, compared to 1 % in EUROCAT Registry) and duodenal atresia (5.6% compared to 2.9% in EUROCAT Registry). We present comparable short term outcomes for major operative procedures. The paper also provides a basis for antenatal counselling and allotment of resources while planning services, which is of benefit to hospitals, clinicians and parents.


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