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Short-term outcomes of Hirschsprung disease in an LMIC: a retrospective review
Rafay Salman 1, Varisha Madni 1, Javeria Javed 2, Muhammad Osama Khan 2, Muhammad Aqil Soomro 2, Saqib Qazi 2, Saleem Islam 2
1 Medical College, Aga Khan University, Karachi, Pakistan
2 Section of Pediatric Surgery, Department of Surgery, Aga Khan University, Karachi, Pakistan
Purpose
Hirschsprung disease (HD) is a congenital disorder of the enteric nervous system that leads to functional intestinal obstruction. The children with HD in low- and middle-income countries (LMICs) face delayed diagnosis, limited access to care, and high complication rates. This study aimed to evaluate the short-term surgical outcomes of HD in an LMIC setting and identify factors associated with postoperative complications.
Methods
A retrospective cross-sectional study was conducted at a tertiary care hospital to evaluate the prevalence, risk factors, and outcomes associated with HD. Medical records of all patients diagnosed with HD from 2012- 2024 were reviewed. Data collected included prenatal history, birth details, birth weight, height, maternal risk factors, relevant laboratory investigations, imaging findings, surgical interventions and post-operative findings. These variables were analyzed to identify predictors of adverse clinical outcomes.
Results
We identified 60 patients who underwent definitive pull-through surgery between 2012 and 2024. The cohort was predominantly male (73%) with a median age at surgery of 23 weeks (IQR: 9-40 weeks). The majority underwent open Soave procedures (97%) and were admitted electively (90%). Postoperative complications occurred in 11 patients with postoperative autologous blood transfusion (7%) and surgical site infections (5.4%) being the most frequent. Patients with complications had previous steroid use (p = 0.037) or open wound infection (p = 0.037) and a longer length of stay (median: 9 vs 6 days, p = 0.003).
Conclusion
This study highlights the key short-term outcomes of HD in an LMIC. Postoperative complications were linked to prior steroid use, and wound infections. These findings emphasize the importance of preoperative risk stratification and standardized perioperative care.