Hirschsprung’s Disease (HD), characterized by congenital absence of ganglionic cells in the submucosal Meissner’s and myenteric plexus of the bowel wall, is often associated with a lack of peristalsis and subsequent functional bowel obstruction among paediatric patients [1, 2, 3]. A surgical approach, involving the removal of the aganglionic bowel segment and the restoration of bowel continuity with a ganglionic segment, is considered the mainstay for the treatment. Various surgical procedures exist for the management of HD. Some of the important procedures include Swenson’s procedure, which requires trans anal resection of the aganglionic distal colon [3]; Soave procedure, which involves removal of aganglionic rectal mucosa and connecting ganglionic colon to distal part of the rectum [4]; and Duhamel procedure that involves a retro-rectal pull through by bringing ganglionic colon behind the aganglionic rectum and connecting the two segments side-by-side [5].

The Duhamel operation is one of the commonly adopted procedures for the surgical correction of HD due to advantages like good visibility and limited anal stretching. A modified technique, wherein the ganglionic part of the lower colon is brought in anastomosis with the posterior part of the lower rectum, followed by creation of a rectal pouch, helps in preventing a blind rectal pouch that can be created in the regular Duhamel procedure [6]. The other advantages of the modified Duhamel procedure include a lower chance of anastomotic stricture, lower chances of injury to the anal canal and lesser soiling, avoidance of neurogenic bladder, and progressively improved sphincter control [5, 6].

Historically, the modified Duhamel procedure was a multistage procedure, which could be carried out in two or three stages. However, a single-stage procedure without a protective colonostomy is increasingly employed, owing to low morbidity, acceptable functional outcomes, and reduced hospital stay [7]. Moreover, the use of laparoscopic techniques in the procedure has made it popular among surgeons and the patients’ kin [8].

Scientific data and literature on the single-stage modified Duhamel procedure for the surgical management of HD remain limited. A previous study, published in 2012 [7], reports data from 48 patients treated for HD through a single-stage modified Duhamel procedure. However, this data needs supplementation, given the evolving surgical landscapes, advancements in perioperative care, and the need for larger datasets of evidence. Therefore, the present study aims to contribute to the existing literature by presenting the data on outcomes from a large cohort of paediatric HD patients undergoing a single-stage modified Duhamel procedure in a large Indian tertiary care hospital.

A retrospective cohort study was conducted at a paediatric surgical unit of a tertiary hospital in Mumbai, India. The study period extended from October 2008 to December 2024. After the required research and ethical clearance, the data of the patients who underwent a single-sitting modified Duhamel procedure for Hirschsprung’s Disease were obtained from hospital records. All the necessary administrative clearances were obtained before accessing the hospital records. The study was performed as per the ethical standards of the Declaration of Helsinki. Given the retrospective nature of the study and de-identification of the assessed data, the need for ethical consent from the individual patients was waived.

The inclusion criteria encompassed all patients diagnosed with Hirschsprung’s disease who had a single-stage modified Duhamel procedure. The procedure involves a retrorectal pull-through of the healthy colon, followed by a side-to-side anastomosis with the remaining rectum. This is often done using stapling devices to create the anastomosis and divide the spur (the dividing wall between the two bowel segments). The retrorectal plane is created just at the dentate line, not 1.5 cm above it, as the results obtained were better with the former approach. Additionally, over time, the procedure has been modified to incorporate techniques like using stapling instruments for the anastomosis and spur division, making the procedure potentially less complex and more efficient. As earlier, a Kellys clamp was used to divide the spur, which was more cumbersome [9]. Pre-diagnosed patients, as per the hospital records, were included. Only patients with a complete hospital record, including preoperative, intraoperative, and postoperative records, were included for analysis. Exclusion criteria from the study included patients who had undergone a staged surgical approach, a multi-stage Duhamel procedure, or any other surgical procedure. Other exclusion criteria included patients who presented with total colonic aganglionosis or had significant associated anomalies, due to which surgical management could not be instituted. Patients with unavailable follow-up data were also excluded from the study.

A manual data extraction from hospital records was performed by the researchers using a structured format. The data included demographic information, clinical presentation, intraoperative parameters, and post-operative outcomes. This included demographic data like age and gender, the anatomic extent of aganglionosis, intraoperative parameters such as weight at the time of surgery, blood loss, and duration of surgery, and the postoperative parameters such as hospital stay or postoperative complications. Each data point was cross-checked by two examiners to ensure consistency in the data and minimize reporting errors.

The postoperative follow-up in the hospital is scheduled twice weekly for 1 month, followed by 1 monthly for 3 months, and thereafter 6 monthly for 1 year. The data for complications included in this study were recorded at the first six monthly visit, which was 6 months from the date of discharge. This made the data consistent and comparable with earlier studies that have also taken 6 monthly visit as the benchmark for short term data collection. The complications assessed included anastomotic leak, which was defined by clinical evidence of leakage confirmed by radiological evidence. Surgical site infection was also included in the study, which encompassed superficial incisional, deep incisional, or space infection within 30 days of the surgery. Another complication studied was residual aganglionosis, which was confirmed by rectal biopsy or contrast imaging. Post-operative adhesive internal obstruction and enterocolitis, which presented as abdominal distension, fever, and diarrhoea, were also included. Finally, mortality was also included as a study outcome.

Descriptive statistics were utilised to summarise the data. Categorical variables such as sex, level of aganglionosis, and complication rates were expressed as absolute numbers and percentages. Continuous variables including weight at surgery, operative time, blood loss, and hospital stay were summarised using means, medians, and ranges.

Mean values were derived by computing the arithmetic average of all observations. Median values represented the central tendency and were calculated by arranging data in ascending order and identifying the middle value.

All statistical analyses were performed using IBM SPSS Statistics for Windows, Version 26.0 (IBM Corp., Armonk, NY, USA). A p-value of < 0.05 was considered statistically significant, where applicable, although the present study primarily employed descriptive analysis.

The present study analysed 282 paediatric patients who were diagnosed with Hirschsprung disease and underwent a single-stage modified Duhamel surgery. The study focused on demographic characteristics, perioperative characteristics, and postoperative complications observed among these patients. Out of the 282 patients, 165 (58.5%) were male and 117 (41.5%) were female. The male-to-female ratio for this study was approximately 1.4:1.

Regarding the level of aganglionosis, the rectosigmoid segment was most commonly affected, observed in 175 patients (62.1%). Long-segment aganglionosis was present in 82 cases (29.0%), and ultrashort-segment disease was comparatively rare, noted in only 25 patients (8.9%). These distributions are reflective of classical anatomical predilections of the disease, with rectosigmoid involvement being most prevalent.

When stratified by age at clinical presentation, the majority of patients were diagnosed during infancy. Specifically, 188 children (66.7%) presented between 1 and 12 months of age, followed by 62 children (22.0%) who presented after their first birthday. Neonatal presentation (within the first 30 days of life) accounted for 32 cases (11.4%). Delayed diagnoses beyond infancy may reflect referral patterns, regional disparities in healthcare access, or milder initial symptomatology in longer-segment variants.

The age at which surgical intervention was performed ranged from 6 months to 10 years, with a mean age of 4.2 years and a median of 2.5 years. The lower median relative to the mean indicates a slightly right-skewed distribution, with a substantial number of children undergoing surgery within the first three years of life. Patients had body weights between 5 and 20 kilograms, with a mean operative weight of 7.85 kg and a median of 7.10 kg. Intraoperative blood loss was modest, ranging from 35 to 60 ml, with a mean volume of 45.2 ml and an estimated median of 45 ml. Blood loss noted was less than 5ml/kg which is less than significant blood loss requiring intra op transfusion. This is especially significant in paediatric patients where small-volume losses can be clinically relevant.

Duration of surgery varied from 130 to 250 minutes, with a mean operative time of 174.5 minutes and a median of 175 minutes, indicating a relatively standardised approach and operative learning curve. Postoperatively, the length of hospital stay ranged between 6 and 10 days, with an average stay of 5.17 days and a modal stay of 6 days.

The most common complication of the procedure was enterocolitis. Hischsprung’s associated enterocolitis occurs due to stasis of stool, followed by bacterial overgrowth that leads to infection and sepsis if not controlled [10, 11]. A total of 20 patients (7.09%) developed enterocolitis in the present study. Surgical site infection (SSI) occurred in 6.03% patients. Anastomotic leak occurs when the anastomosis between the two bowel segments fails after surgery. It is one of the major complications of modified Duhamel surgery [12]; however, in the present study, 3 patients (1.06%) had anastomotic leak. Abdominal surgeries, including modified Duhamel Surgery, stand a risk of post-operative adhesive intestinal obstruction [13, 14]. In the present study, post-operative adhesive intestinal obstruction occurred in 5 (1.77%) patients. Of the other complications while residual aganglionosis was seen in 2 patients (0.71%), while mortality was 1.06%. Further, as we transacted the aganglionic bowel for side-to-side anastomosis just below the peritoneal reflection, pouchitis was not observed, as there was a small residual pouch. No soiling, structure, or incontinence was observed.

The single-stage modified Duhamel procedure has garnered considerable attention due to its potential to simplify the surgical management of Hirschsprung’s disease [7]. This study provided insight into the outcome of the procedure in terms of its post-operative complications and associated mortality. The results of this study provide evidence from the largest retrospective cohort of patients undergoing this procedure so far. The study establishes single-stage modified Duhamel procedure as an effective and viable surgical option for HD management, especially in resource-constrained settings, where minimizing the number of surgical stages can benefit the patient as well as the health care system immensely.

Granström and Wester (2017) [15] have shown that the mortality rate of Swedish patients with HD in a cohort of 739 individuals was 3% in 2017. A systematic review and meta-analysis by Thomson et al. (2015) [16] of 5 studies and 405 patients did not report any mortality in the two studies where it was taken as an outcome variable. Granström and Wester (2017) have quoted the mortality after one-stage transanal pull-through procedures in HD as ranging from 0 to 2%. The mortality rate of 1.06% seen in the present study is comparable to this range. The other surgical complications, especially the major complications, were also low. Therefore, the single-stage modified Duhamel procedure for HD seems to be a viable surgical method for the management of HD.

Another key finding in the present study was the age of presentation of the disease. Majority of the patients in the present study (66%) presented between 1 to 12 months of age. This was different from the earlier findings by Kothari et al., [7] where the majority of patients (62.5%) presented within the first month. This could be attributed to an exponential increase in the number and diversity of the population in Mumbai over the past decade, as well as an improvement in referral facilities from the secondary and primary centres. While this may have led to an increased number of patients reporting to our hospital, the time taken to report might have increased owing to differences in awareness levels of the community and time taken to identify and refer the patients. A detailed study is warranted on this subject to determine the reasons for this difference in the age of reporting. Similarly there was a significant age gap between reporting age, and the age in which surgery was performed. While the reporting age is 1 to 12 months, the median age at which surgery was performed was 2.5 years. This gap was due to nutritional and personal reasons. Sometimes the children were not nutritionally fit to undergo surgery, while many a times, the family deferred the surgery due to personal and financial reasons.

The level of aganglionosis in the present study was similar to the previous literature. The pattern seen in the present study was that the rectosigmoid colon was involved in 62.1% of cases, followed by long-segment disease in 29.0% and ultrashort variants in 8.9%. However, a slight difference in percentage distribution was seen as compared to the earlier study by Kothari et al [7], which aligned more closely to the data provided by Lotfollahzadeh et al (2023). According to Lotfollahzadeh et al, the retrosigmoid colon was affected in about 80% of the cases of HD [2].

Operative data from our cohort were largely consistent with previously published findings [17, 18]. Surgical times ranged from 130 to 250 minutes, and mean blood loss during the procedure was modest, averaging 45 milliliters. A single-stage approach was employed in the majority of cases. Only a small fraction of patients (11.3%) required the creation of a leveling nstoma, most often due to intraoperative assessments or the need for bowel decompression that precluded primary reconstruction. Postoperative hospital stays fell within a typical range; most individuals were discharged between six and ten days following surgery, depending on the pace of recovery and the presence or absence of complications.

Of the complications, enterocolitis was the most common one, which occurred in 7.09% of the patients. Hirschsprung’s disease is commonly associated with enterocolitis [19, 20], which is known to occur in about 15 to 26 percent of the cases [11]. As compared to the present study, study by Peters et al, [13] showed a higher percentage of patients with enterocolitis in short term (14.5%), while the percentage for long term was 5.7% which was higher than the percentage of patients with enterocolitis in the present study. The same study showed higher percentage of anastomotic leaks (2.9%), and a lower percentage of wound infections (2.9%) as compared to the present study (6.03%).

Owing to its large sample size and a long observation period of 16 years, the present study was able to provide a robust analysis of demographic details and outcomes of the single-stage modified Duhamel procedure. The study was also able to determine the merits of a uniform surgical technique in the success of treatment amidst a changing diagnostic and care environment. However, as a retrospective study, this analysis is subject to certain inherent biases that should be considered as a limitation of this study. We recommend that future prospective studies incorporate standardized follow-up metrics and patient-reported outcomes. Such studies will be valuable in complementing the findings of the present study.

The single-stage modified Duhamel procedure remains a reliable and effective surgical option for the treatment of Hirschsprung’s disease in children, particularly in settings where staged procedures may not be feasible or ideal. Our findings support its use across a wide age range and disease extent, with acceptable complication rates and surgical outcomes. With careful patient selection, adherence to operative principles, and standardized perioperative care, this approach offers the potential to streamline the management of HD while maintaining surgical safety and efficacy.