Presentation Description
Institution: Queensland Children's Hospital - Queensland, Australia
Purpose:
Despite advances in surgical technique, children with Hirschsprung disease (HD) continue to experience significant morbidity. This study aimed to review contemporary practice patterns and outcomes in the management of HD at a tertiary paediatric surgical centre, with a focus on identifying areas for quality and practice improvement.
Methodology:
A retrospective cohort audit was conducted of all children diagnosed with HD and managed between 2019 and 2024. Demographic, clinical, operative, and follow-up data were collected using a standardised proforma. Key measures included age at diagnosis, disease extent, operative approach, levelling strategy, stoma use, Hirschsprung-associated enterocolitis (HAEC), perioperative complications, long-term bowel function, and comorbidities. Descriptive statistics were used.
Results:
Fifty-one patients were included (male:female ratio 2:1). While most patients were diagnosed neonatally (median 6 days), a wide diagnostic range was observed. Short-segment disease predominated (n=41), though extensive disease was present in a significant minority. Stoma formation was common, with 41 patients requiring a pre–pull-through stoma. Median age at definitive pull-through was 146 days, most commonly via laparoscopic Swenson (n=18) or Soave (n=16). HAEC occurred both preoperatively (13%) and postoperatively (27.5%). Frequent practice-related challenges included high-output stomas, stoma prolapse, sphincter dysfunction, and persistent bowel dysfunction, often necessitating further interventions such as Botox therapy, washouts, laxatives, or additional surgery. Syndromic and developmental comorbidities were common.
Conclusion:
This audit highlights ongoing morbidity in children with Hirschsprung disease and identifies several modifiable practice areas, including diagnostic timeliness, stoma selection and management, levelling strategies, and structured long-term bowel care. Standardised pathways, enhanced multidisciplinary follow-up, and targeted parental education may reduce complications and improve long-term outcomes.
Presenters
Authors
Authors
Dr Eamon Clanchy - , Dr Carly Perry - , Ass. Prof Bhaveshkumar Patel - , Prof Craig Mcbride -
