The Prevalence and Clinical Profile of Hirschsprung’s Disease at a Tertiary Hospital in Bahrain

Abstract

Background Hirschsprung’s disease (HD) is a rare but important congenital pediatric disease of the colon, and its incidence varies widely between ethnic groups. Its incidence was first studied in Bahrain in 1980 using hospital-based data. Over a 16-month period, 10 cases were reported, representing a relatively high incidence rate: 1 per 4000 births. Even though the number of live births in Bahrain has increased dramatically over the last four decades (doubling from 10,000 to 20,000 per year), published studies about the incidence of HD are uncommon. In this research, we aimed to determine both the prevalence and the clinical characteristics of HD at a tertiary hospital in Bahrain. Methods This retrospective observational cross-sectional study included children diagnosed with HD at a tertiary hospital in Bahrain over the last seven years (2014-2020). Children over 10 years were excluded. Clinical data collected included gestational age, birth weight, gender, associated anomalies, clinical features at presentation, disease management, and complications. Results The prevalence of HD in Bahrain was found to be 1.3 per 10,000 live births, according to 18 patients qualified for inclusion in this study. The median age at diagnosis was 18 days; approximately 72% were males, and nearly 94% were Bahraini nationals. Three patients (16.7%) had associated anomalies including cardiac and urogenital malformations. Abdominal distention was the most common clinical presentation (83.3%) followed by constipation (77.8%). Half of the patients (50%) passed meconium within 48 hours of birth. Transanal full-thickness rectal biopsy was the method of diagnosis in most patients (83.3%). Seven patients (38.9%) were diagnosed comparatively late (beyond the neonatal period), at a mean age of 1-2 years. Significant associations between age at diagnosis and clinical presentation, initial management, or surgical intervention were not found. A total of 17 patients (94.4%) underwent the definitive surgery (transanal pull-through procedure). In this surgical group, initial colonic decompression was performed via rectal washout in six patients (35.3%) and via temporary stoma in three patients (17.8%). Laparotomy combined with the definitive surgery was necessary for six patients (35.3%). Most of the patients treated surgically had a short segment disease (70.5%). Conclusions The awareness of HD is highly important, especially with more than one-third of cases diagnosed outside the neonatal period and half of them passing meconium within 48 hours of birth. In addition, early detection of HD in the neonatal period would result in a less complicated course by reducing the number of patients requiring a multi-stage surgery and further laparotomies.