Minimally Invasive Surgical Intervention for Hirschsprung Disease: A Prospective Study on Efficacy and Long-term Functional Outcomes for Neonatal Patients
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 23
- 试验地点
- 1
- 主要终点
- Volume of Blood Loss
研究概览
简要总结
Hirschsprung's disease (HD) is one of the most common congenital conditions, with a global incidence of 1/5000 newborns; the prevalence in the Vietnamese population is even higher. The absence of enteric ganglia in the distal bowel causes intestinal obstruction and delayed meconium passage in newborns, as well as failure of normal defecation later in life. If left untreated, HD can lead to life complications such as enterocolitis and even death. Standard treatment involves surgical resection of the affected bowel segment, with minimally invasive laparoscopic techniques offering reduced postoperative complications, shorter hospital stays, and faster recovery compared to open surgery. Since 2012, the National Children Hospital has been the first institution in Vietnam to routinely use the minimally invasive surgical approach for HD. However, due to a lack of research funding and patients' financial constraints to travel to post-operative treatment centers, there has yet to be a publication addressing the long-term outcomes and associated abnormalities of all patients treated with SILS. Thus, the purpose of this study is to report on the safety, efficacy, and long-term functional outcomes and cosmesis results of minimally invasive surgeries performed on HD neonatal patients at The National Children's Hospital from 2020 to 2021, thus optimize surgical management and improve patient outcomes in a lower-middle-income country setting.
详细描述
Hirschsprung's disease (HD) is one of the most common congenital disorders affecting the gastrointestinal tract, with a global incidence of approximately 1 in 5,000 live births. However, studies suggest that the prevalence is notably higher in the Vietnamese population. The condition arises due to the absence of enteric ganglion cells in the distal colon, leading to a functional obstruction that prevents normal bowel motility. This defect results in delayed meconium passage in newborns, severe constipation, abdominal distension, and feeding intolerance. If left untreated, HD can progress to life-threatening complications such as Hirschsprung-associated enterocolitis (HAEC), bowel perforation, sepsis, and even death.
Surgical intervention remains the mainstay of treatment for HD, with the goal of resecting the aganglionic bowel segment and restoring normal intestinal function. Traditionally, open pull-through procedures were the standard approach, but advancements in minimally invasive surgery (MIS) have led to the widespread adoption of laparoscopic techniques. Georgeson's traditional three-port laparoscopic rectal pull-through technique, first introduced in 1995, has been widely used as the standard treatment for HD. The first case of single-incision laparoscopic assisted rectal pull-through (SILS) surgery for HD was published in 2010, in which six patients who underwent SILS all had positive outcomes and excellent cosmesis. Compared to conventional open surgery, laparoscopic approaches offer several benefits, including reduced postoperative pain, fewer wound-related complications, shorter hospital stays, improved cosmetic outcomes, and faster overall recovery. Despite the promising outcomes of minimally invasive surgical intervention, 15 years after the first reported case, only a few articles (a total of 9 articles reported in Pubmed) have been published to evaluate SILS in HD.
Since 2012, the Vietnam National Children's Hospital (NCH) has been a pioneer in the routine use of the minimally invasive surgical technique for the treatment of Hirschsprung's disease in Vietnam. However, despite over a decade of experience with this method, research on long-term outcomes in Vietnamese patients remains scarce. A major challenge has been the limited availability of research funding and the financial difficulties faced by many families, which prevent regular postoperative follow-ups and comprehensive long-term studies. As a result, there is currently no published data evaluating the long-term safety, efficacy, and potential postoperative complications associated with minimally invasive intervention for HD in this specific patient population.
The primary objective of this study is to bridge this knowledge gap by systematically analyzing the outcomes of pediatric patients who underwent minimally invasive surgical intervention for Hirschsprung's disease at NCH between 2020 and 2021. The study aims to assess key factors such as perioperative outcomes, postoperative bowel function, the incidence of complications such as anastomotic stricture or incontinence, associated abnormalities/ comorbidities, cosmesis, and quality of life improvements. By providing robust clinical evidence, this research will contribute to optimizing surgical management strategies for Hirschsprung's disease in resource-limited settings, ultimately improving patient outcomes and guiding future surgical practices in Vietnam and other LMICs.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- — 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Age: From 1 to 28 days old
- •Diagnosis: Hirschsprung disease was diagnosed based on clinical presentation, intraoperative frozen section biopsy, and postoperative histopathological findings showing absence of ganglion cells.
- •Surgical Approach: Patients underwent SILEP through a single periumbilical incision, with no additional abdominal incisions. All surgeries were conducted by the same team, and postoperative care was provided according to a standardized protocol.
- •Parents or guardians provided consent to participate in the study and committed to following the scheduled follow-up visits.
排除标准
- •Patients exhibiting severe HAEC, bowel obstruction, or peritonitis at the time of operation
- •Those with a history of failed Hirschsprung disease surgery, prior abdominal operations, or colostomy
- •Individuals with contraindications to laparoscopic procedures, such as bleeding disorders or significant congenital heart defects
- •Cases where parents or legal guardians refused consent for study participation
结局指标
主要结局
Volume of Blood Loss
时间窗: 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
时间窗: 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
时间窗: Perioperative
Total duration of the surgical procedure from skin incision to closure, measured in minutes.
Mortality and severe morbidity
时间窗: Through study completion, an average of 4 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. This outcome will be assessed throughout the study period.
Early postoperative complications
时间窗: 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.
Rintala score
时间窗: Through study completion, an average of 4 years
The Rintala Score assesses postoperative bowel function in pediatric Hirschsprung disease (HD) patients, ranging from 0 to 20. It evaluates stool frequency, incontinence, constipation, and social continence. Scores of 18-20 indicate excellent function with normal bowel habits and no incontinence. Scores of 10-17 suggest moderate function, with occasional issues like constipation or minor incontinence. Scores below 10 reflect poor function, often requiring medical or surgical intervention.
Manchester Scar Scale
时间窗: Through study completion, an average of 4 years
The Manchester Scar Scale (MSS) evaluates the aesthetic and physical characteristics of scars, scoring from 4 to 28. It assesses scar color, contour, texture, distortion, and patient discomfort. Lower scores (4-8) indicate minimal scarring with good cosmetic outcomes. Moderate scores (9-18) suggest noticeable but acceptable scars with some textural or color irregularities. Higher scores (19-28) reflect severe scarring, often associated with significant discoloration, contour defects, or functional impairment.
次要结局
- Associated conditions(Through study completion, an average of 4 years)
研究者
Nguyen Thanh Quang
Principle Investigator
National Children's Hospital, Vietnam