A Retrospective Review of All Children Treated for Hirschsprung's Disease from January 2014 to July 2019 in CWM Hospital

Introduction: No previous research study on Hirchsprungs Disease(HD) in Fiji. The objectives of this study were to describe the demographics, and clinical characteristics, and assess the surgical outcomes of HD in Fiji.
Method(s): This was a retrospective study, with a prospective follow-up design, study population was all children from birth to 14 years diagnosed with HD from January 2014- July 2019
Results: 32 confirmed cases of HD were reviewed in this study. The Incidence of HD in Fiji is 1 in every 1425 births and this is higher than in the rest of the world where it is 1 in 5000 births. HD affects the iTaukei (88%) more than the FOID population with the M: F ratio of 4:1 Children. More than 50% present with abdominal distension, constipation and delayed passage of meconium and 44% with enterocolitis. Patients waited an average of 5 months from presentation to rectal biopsy and another 8 months for histological confirmation. 11% were investigated with Barium Enema, but 100% of the patients had a rectal biopsy while 50% of the patients needed an emergency diverting stoma. Each patient had an average of 4 admissions and spent 35 days in the hospital, with more than 60% needing ICU admission. 16 patients underwent SOAVE Pull-through while 12 patients underwent SWENSON Pull-through. SOAVE operations had more post-operative complications compared to SWENSON. A total of 5 patients died post-pull-through, 3 SOAVE and 2 SWENSON. However, 2 had no complications, 23 survived, 1 of them had a Down Syndromes Case, and 1 needed a permanent Stoma.
Conclusion: Limitations of the study included a small number, some patients were difficult to follow up and poor documentation in folders. Results show that HD has a higher incidence in Fiji, and more in the male intake population, presenting in the neonatal stage with delayed passage of meconium or the older child, abdominal distension, and constipation. SOAVE pull-throughs have more complications than SWENSON. Our mortality rate is higher than in the developed world.