Hirschsprung Disease
Conditions
Keywords
Aganglionic, Minimally invasive, Laparoscopic, Pediatric, Single incision, Hirschsprung, Hirschsprung disease
Brief summary
Hirschsprung's disease (HD) is a congenital disorder characterized by the absence of enteric ganglion cells in the distal bowel, leading to functional obstruction, delayed meconium passage in neonates, and chronic defecation difficulties. Surgical intervention is required to remove the aganglionic segment, with minimally invasive laparoscopic techniques increasingly preferred over open surgery due to reduced postoperative complications, shorter hospital stays, and faster recovery. Since 2012, the National Children's Hospital has pioneered the routine use of single-incision laparoscopic surgery (SILS) for HD in Vietnam. However, there is a lack of comprehensive analysis on the operative outcome in this large group of patients. This retrospective study aims to evaluate patient safety profiles, surgical effectiveness, and functional outcomes of minimally invasive laparoscopic procedures for HD performed at the National Children's Hospital between 2017 and 2023.
Detailed description
Hirschsprung's disease (HD) is a congenital disorder caused by the absence of enteric ganglion cells in the distal bowel, leading to functional obstruction, delayed meconium passage in neonates, and defecation difficulties. If left untreated, HD can result in life-threatening complications such as Hirschsprung-associated enterocolitis and severe bowel dysfunction. Surgical intervention is necessary to remove the aganglionic segment and restore normal bowel function. In recent years, minimally invasive laparoscopic techniques have gained preference over open surgery due to their potential to reduce intraoperative blood loss, postoperative pain, surgical site infections, and overall hospital stay, while promoting faster recovery and improved cosmetic outcomes. The conventional 3 ports laparoscopic rectal pull-through technique has been widely used as the standard treatment for HD since its introduction in 1995. In 2010, the first case series of single-incision laparoscopic-assisted rectal pull-through (SILS) surgery for HD was reported. The study demonstrated that SILS is safe, yielding comparable surgical outcomes while reducing surgical trauma and enhancing cosmetic results. Since 2012, the National Children's Hospital has been at the forefront of utilizing minimally invasive approaches for HD treatment in Vietnam, particularly through single-incision laparoscopic surgery (SILS) and conventional multi-port laparoscopic pull-through (CLP). However, despite the increasing adoption of these techniques, there remains a lack of comprehensive data on their surgical outcome, in a lower-middle-income country setting. Factors such as limited research funding, financial constraints preventing patients from accessing long-term follow-up care, and variability in postoperative management contribute to the gap in knowledge regarding these surgical approaches. This retrospective study aims to systematically evaluate the safety profiles, clinical effectiveness, and short-term functional outcomes of minimally invasive laparoscopic procedures for HD performed at the National Children's Hospital between 2017 and 2023. Key parameters of analysis will include perioperative complications, length of hospital stay, surgical success rates, incidence of postoperative enterocolitis, fecal continence, bowel function recovery, and cost-effectiveness of the intervention. By providing a detailed assessment of these factors, this study seeks to optimize surgical management strategies, enhance postoperative care, and contribute to the growing body of evidence supporting minimally invasive surgical techniques for HD in resource-limited settings.
Interventions
Pediatric patients diagnosed with Hirschsprung's Disease from 2017-2023 at the National Children's Hospital underwent two minimally invasive procedures: conventional laparoscopic pull-through (CLP) and single-incision laparoscopic pull-through (SILPS). CLP involves three to five small incisions for trocars, allowing laparoscopic visualization and instrument access. Pneumoperitoneum is established, and the aganglionic colon segment is identified and mobilized using laparoscopic energy devices. The rectum is dissected circumferentially to preserve mesenteric blood supply, and a transanal approach is used to excise the diseased segment, followed by coloanal anastomosis with absorbable sutures. SILPS follows the same principles but is performed through a single umbilical incision using a multi-port device for all instruments. This technique offers benefits like reduced scarring and less postoperative pain but requires advanced laparoscopic skills due to instrument crowding.
Sponsors
National Children's Hospital, Vietnam
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
No minimum to 18 Years
Healthy volunteers
No
Inclusion criteria
* All pediatric patients (under 18 years old) diagnosed with Hirschsprung's Disease, admitted to National Children's Hospital between January 2017 and December 2023. * Patients exhibiting clinical signs and symptoms consistent with Hirschsprung's Disease, with imaging findings confirming the diagnosis. If imaging was inconclusive but clinical suspicion remained high, patients were still considered for surgical evaluation and management. * Underwent minimally invasive laparoscopic pull-through surgery, either conventional multi-port (CLP) or single-incision (SILS). * Postoperative Follow-up Data Availability: Patients with documented short-term postoperative outcomes, including bowel function recovery, complications, or reoperation rates.
Exclusion criteria
* Clinical instability requiring emergency open laparotomy due to peritonitis, intestinal perforation, or other life-threatening conditions. * Biopsy findings inconsistent with Hirschsprung's Disease, ruling out the diagnosis. * Incomplete Medical Records: Patients with missing key data, such as operative details, pathology reports, or follow-up outcomes, that could compromise study integrity
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Length of Hospital Stay | Up to 24 weeks post-operation | The total number of days from admission to discharge after surgery, reflecting postoperative recovery and hospital resource utilization. |
| Rate of reoperation | Through study completion, an average of 5 years | The proportion of patients who require an additional surgical intervention related to the initial procedure. This includes cases of anastomotic complications, bowel obstruction, stricture formation, or other postoperative issues necessitating reoperation. |
| Fecal Continence and Bowel Function Recovery | Through study completion, an average of 5 years | Frequency of bowel movements, presence of soiling, need for enemas, and ability to maintain voluntary bowel control at follow-up visits. |
| Volume of Blood Loss | Perioperative | The amount of blood lost during surgery will be measured in milliliters (mL) by collecting blood from suction devices, counting soaked surgical sponges, and assessing any other visible loss |
| Conversion to open surgery | Perioperative | The proportion of cases in which single-incision laparoscopic surgery (SILS) could not be optimally completed and required conversion to open surgical techniques. Conversion may be necessitated by factors such as poor visualization, uncontrolled bleeding, adhesions, or patient-specific anatomical challenges. The outcome will be measured as the percentage of procedures requiring conversion during the perioperative period. |
| Operative time | Perioperative | Total duration of the surgical procedure from skin incision to closure, measured in minutes. |
| Mortality and severe morbidity | Through study completion, an average of 5 years | The incidence of mortality and severe postoperative complications following single-incision laparoscopic pull-through surgery. Severe morbidity includes life-threatening conditions such as sepsis, multi-organ failure, other major complications requiring intensive medical intervention, and death. |
| Early postoperative complications | Up to 8 weeks post-operation | The incidence of early postoperative complications, including but not limited to bowel obstruction, surgical site infections, and Hirschsprung-associated enterocolitis (HAEC). Additional complications such as anastomotic leakage, prolonged ileus, or unexpected reoperation will also be monitored. |
| Time to return of bowel function | Up to 4 weeks post-operation | The time from surgical intervention to the return of bowel function, defined as the first occurrence of spontaneous bowel movement or passage of flatus without the need for rectal stimulation or enemas |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Diagnostic predictive value of X-ray for Hirschsprung Disease | Perioperative | This outcome measures the diagnostic accuracy of X-ray in detecting Hirschsprung disease by comparing its findings to biopsy results. It evaluates whether the resected bowel contains the aganglionic segment and assesses the completeness of aganglionic bowel resection. Diagnostic performance will be analyzed using sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). |
| Total cost of the Surgical Approach | Through study completion, an average of 5 years | Total cost of hospitalization, medications, and additional treatments to assess the overall economic feasibility in a lower-middle-income setting. |
Countries
Vietnam
Outcome results
None listed