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Hirschsprung disease at a tertiary hospital in central South Africa: patient profile, management and outcomes
Elizabeth Brits 1, Laylaa Moosa 2, Muhammad Kola 2, Osman Cassim 2, Zafeerah Khan 2, Rummanah Cajee 2, Aslam Salie 2, Muhammed Peer 2, Mohammed-Sulaimaan Hoosen 2, Joseph Sempa 3
1 Paediatric Surgery, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa
2 Undergraduate medical student, School of Clinical Medicine, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa
3 Department of Biostatistics, Faculty of Health Science, University of the Free State, Bloemfontein, South Africa
Background: Hirschsprung disease (HD) is a congenital condition marked by absent ganglion cells in the colon, causing serious digestive problems. It affects approximately 1 in 5 000 newborns worldwide, predominantly males. Outcomes vary widely due to factors such as delayed diagnosis and limited resources. This study explored the clinical profile, management and outcomes of HD at Universitas Academic Hospital Complex, a tertiary care facility in central South Africa. The aim was to pinpoint healthcare challenges and enhance patient care in a resource-restricted setting.
Method: A retrospective cross-sectional study of paediatric surgical patients treated for HD from January 2010 to December 2021 was conducted. Data extracted from electronic medical records were analysed to evaluate demographics, disease presentation, diagnostic methods, treatment approaches and postoperative outcomes.
Results: The study analysed 65 patients, mostly male (83.1%), with a median diagnosis age of 87 days. Common symptoms included abdominal distension and difficulty passing stool, with 30.8% developing Hirschsprung-associated enterocolitis. Diagnostic methods mainly used rectal suction biopsies, while surgical treatments often required multi-stage procedures due to late diagnosis and extensive disease. Postoperative complications were common and many patients needed additional surgeries. Follow-up indicated a high survival rate but also significant loss to follow-up and long-term complications.
Conclusion: The study underscores major challenges in managing HD in resource-limited settings, where late diagnoses and scarce diagnostic resources influence outcomes. The findings emphasise the need for targeted research, improved diagnostics and enhanced surgical and postoperative strategies to improve the management and quality of life of patients affected by HD.