Post-surgical Outcomes of Different Surgical Techniques in Hirschsprung’s Disease: A Literature Review

Hirschsprung's disease (HD) is a rare condition that affects newborns and is characterized by the lack of ganglion cells in the colon. Typical symptoms include difficulty passing stool, vomiting, and trouble feeding. Various surgical methods are available to manage the condition. The aim of the study is to investigate and compare the post-surgical outcomes of different surgical techniques used in the treatment of HD. A thorough literature search was conducted using various electronic databases to identify relevant studies to be referred to. Double-blinded screening of the identified articles led to the final selection of 40 out of 440 HD, including transanal endorectal pull-through (TERPT), laparoscopic approaches, and modified techniques. Several studies have investigated surgical procedures for HD, including TERPT, laparoscopic methods, and modified techniques. These have shown positive outcomes, with fewer complications, improved bowel function, and favorable cosmetic results. Individual patient characteristics and surgeon expertise should guide procedure selection. Surgery for HD aims to restore normal bowel function, but post-surgical outcomes can include constipation or fecal incontinence. Complications like enterocolitis, anastomotic stricture, and sphincter damage may occur. Laparoscopic approaches have shorter hospital stays. However long-term follow-up is essential to assess quality of life, psychological well-being, and potential side effects.


Introduction And Background
Hirschsprung's disease (HD) also referred to as aganglionosis, is distinguished by the lack of ganglion cells within the myenteric and submucosal plexuses found in the colon.The absence of ganglion cells in this particular segment leads to a condition where the intestinal contents cannot move effectively, causing stagnation or lack of contraction in that segment [1,2].HD is found in approximately one out of every 5,000 live births and is commonly diagnosed during the neonatal period.During this time, affected individuals exhibit symptoms such as a swollen abdomen, inability to pass meconium, expulsion of bile through regurgitation, vomiting, and difficulty with feeding.The most effective and used method for diagnosing HD is rectal biopsy, which can be performed by obtaining a full-thickness specimen or by suction technique obtaining a full-thickness specimen [3].This diagnostic approach is the gold standard as it is confirmed by demonstrating the absence of ganglion cells [4].
The surgical management of HD involves employing various techniques.These techniques are widely practiced worldwide and commonly include procedures such as Swenson, Duhamel, and Yancey-Soave.Currently, the two most frequently performed surgical procedures to treat rectosigmoid HD are endorectal pull-through (ERPT), which includes both total transanal (TERPT) and laparoscopic endorectal pull-through (LERPT) approaches.In this technique, our objective is to surgically remove the affected segment and bring down the normally innervated bowel [5].Pediatric surgeons follow children with HD from diagnosis to corrective surgery in infancy, to check for and manage short-term and long-term complications.Typically, HD primarily impacts a limited section of the large intestine or colon.The rectum is consistently implicated, with 80% of instances confined to the rectosigmoid colon.However, in rare cases, certain individuals may experience a complete absence of ganglion cells throughout the entire colon [6].
It has been reported that up to 60% of children have complications after corrective surgery [7].In the immediate postoperative period, patients report fecal soiling and diarrhea unrelated to obstruction, which typically normalizes over several months [8].During this transition, young children may also experience HD is a congenital condition of the gastrointestinal tract characterized by the absence of ganglion cells in the distal bowel and has been referred to as a congenital aganglionic megacolon.

Surgical techniques
Several surgical techniques have been developed in recent years.Removing the aganglionic portion of the gastrointestinal tract and restoring normal bowel continuity is an essential objective of surgery.
Factors such as the length of the affected bowel section, associated abnormalities, and surgeon experience shall be taken into account when choosing an approach.In recent years, minimally invasive techniques, such as laparoscopic and robotic-assisted approaches, have gained popularity due to their potential advantages, including reduced postoperative pain, shorter hospital stays, and improved cosmetic outcomes [2,[14][15][16][17][18][19].The TERPT and LERPT are the predominant surgical techniques utilized for the treatment of rectosigmoid HD [5].Accurate preoperative bowel preparation, correct trocar placement, and patient positioning proved to be crucial aspects of treatment [20].Surgical intervention to remove the affected portion of the stomach is an important aspect of the final treatment for HD to restore normal bowel function.The objective of this discussion is to present a comprehensive review of surgical outcomes in pediatric patients with HD.

Preoperative evaluation
A thorough preoperative evaluation, including a detailed medical history, physical examination, and diagnostic tests, is the first step in the management of HD.The evaluation of clinical characteristics, age at diagnosis, associated comorbidities, and nutrition status will be a major part of the assessment.There were no significant differences in the diagnosis or occurrence of enterocolitis, and the proportion of patients requiring bowel management for fecal incontinence was also similar [21,22].In a study by Lin et al., it was found that preoperative anal dilatation did not show any significant difference in outcome when compared to patients with no preoperative anal dilatation; hence, improving preoperative maneuvers might not help improve postsurgical outcomes for HD [23].A conclusion was drawn that there is no significant difference in effect on growth outcomes among different procedures [24].The study by Onishi et al. stressed the positive effects on children's quality of life by resolving post-operative issues through medical treatments or redo procedures [25].Moreover, to confirm the diagnosis, assess the size of the aganglionosis, and decide how to proceed with surgery, it is also necessary to use different diagnostic methods like contrast enemas, rectal suction biopsies, or anorectal manometry [26].In a study by Ralls et al., redo pull-through patients of HD have worse stooling results than initial operations [27].A study analyzed the growth outcomes of patients who underwent different pull-through methods; the Duhamel procedure (DP), Swenson procedure (SP), and TERPT showed the improvement of nutritional status was achieved in 21.2% of HD patients after TERPT, 14.3% post-Duhamel, and 5.9% following the Soave procedure [28].In a study by Urushiara et al., it was shown that laparoscopic Z-shaped colorectal anastomosis for HD appears feasible and safe to perform with good results [29].

Short-term outcomes
Short-term outcomes following HD surgery are crucial for immediate recovery.Key measures include surgical complications, pain, oral intake time, hospital stay, and bowel function.A study by Xu et al. comparing transumbilical enterotomy and conventional abdominal enterostomy found no significant difference in soiling and constipation rates.However, the transumbilical approach showed better cosmetic results [1].Surgical complications, although rare, can occur and range from wound infections and anastomotic leaks to enterocolitis and obstructive symptoms.In a study by Askarpour et al., children who underwent Saove's pullthrough procedure with oblique and circular anastomosis were followed up for soiling, and postoperative complications, such as wound infection, wound dehiscence, peritonitis, fecal soiling, and perianal excoriation, were recorded for each patient [17].The study found that perianal excoriation was the most common complication among patients in both groups.Oblique anastomosis yielded fewer complications than circular anastomosis, making it a viable choice for patients who underwent Soave's procedure.Also, enterocolitis was more frequent in the circular group than in the oblique group.The anastomotic stricture was also more frequent in the circular group [17].Anorectal function generally improves with age.In contrast, early postoperative complications may involve perianal excoriation, enterocolitis, and an anastomotic leak, while late postoperative complications can include issues such as anal stricture, constipation, and soiling problems [30][31][32].Using a transcolostomy, a single-incision laparoscopic approach provides a cosmetic benefit by reducing scarring.In addition, it provides surgical accuracy, reducing rates of intestinal perforation and, thus, reducing the incidence of postoperative intestinal obstruction caused by adhesions.Preventing tension between the anal stoma and colon is crucial to reduce complications like anal stenosis and enterocolitis, while a comparison between the SP and DP reveals that the SP has a higher frequency of bowel movements and more severe soiling but lower constipation severity [33][34][35].In a study by Kastenberg et al., patients who underwent primary endorectal pull-through for HD were analyzed, and the outcomes of neonatal and delayed pull-through cohorts were compared and found that delayed pullthrough was a safe alternative to neonatal surgery with similar functional outcomes [35].
A study by Dingemans et al. indicated that a primary laparoscopic endorectal pull-through procedure with a postoperative rectal tube reduces early-stage abdominal distension and Hirschsprung-associated enterocolitis (HAEC), offering beneficial postoperative management [36].
Postoperative complications in the surgical management of HD, such as HAEC, fecal incontinence, perianal excoriation, anastomosis stricture and leakage, anastomosis volvulus, and anovaginal fistula, vary in frequency based on the specific surgical procedure [21,37].TERPT has positive outcomes, with shorter hospital stays and fewer postoperative complications.
However, long-term complications such as constipation and soiling occur more frequently due to rectum mobilization and anal sphincter stretching.Certain measures such as leaving the rectal cuff short after TERPT and leaving the native rectum short after Duhamel pull-through do help to reduce the incidence of constipation postoperatively.Rectal stenosis, which occurs due to anastomotic ischemia, and anastomotic leakage were lower following the Duhamel pull-through [38].The significant factor for long-term complications was whether HD was associated with a syndrome or not.Between the two, the transanal pullthrough procedure is preferred over the Duhamel procedure.

Long-term outcomes
Long-term surgical outcomes in HD focus on restoring normal bowel function.Most patients achieve satisfactory results, improving their quality of life.However, some may experience issues like fecal incontinence and constipation.Factors such as segment length, genetic syndromes, comorbidities, and surgical technique can influence outcomes.Fecal soiling is a common concern during long-term follow-up, which can be minimized by proper positioning of the mucosectomy incision on the anal canal [24].
Modified laparoscopic Swenson (MLSw) and laparoscopic Soave (LS) procedures when compared showed that early postoperative outcomes like soiling and constipation were much better in the MLSw group, but long-term outcomes were similar in both procedures [18,26].Reoperations to treat complications showed symptomatic improvement.

Quality of life
Constipation was stated as the predominant concern by almost 15% of patients having TERPT [15].The transanal approach was praised for being less intrusive and having fewer problems than the Swenson-Denda (SD) treatment [16].The study by Oh et al. investigated stool frequency and soiling in individuals with HD [34].The findings showed that shorter aganglionic segments were correlated with reduced bowel movements and milder soiling.However, as patients aged, these distinctions became less prominent [34].
Surgical intervention remains crucial in treating HD, providing relief from symptoms, promoting growth, and improving function.However, long-term follow-up is essential to assess the quality of life, psychological well-being, and potential side effects.It is important to investigate factors influencing outcomes and establish standardized follow-up protocols for patients with HD and other conditions.

Significant findings from specific studies
Pini Prato et al. substantiated the minimally invasive redo pull-through technique's appropriateness for treating postoperative obstructive problems [20] (Table 1).Oblique anastomosis in the Soave pull-through method has the potential to reduce postoperative problems as compared to circular anastomosis.When compared to the Duhamel procedure, the Swenson procedure had a higher frequency of bowel movements and a higher severity of soiling [22].In a two-stage technique, Xu et al. indicated good outcomes and enhanced cosmetic effects of transumbilical enterostomy (TUE) compared to conventional abdominal enterostomy (CAE) [1].The preoperative anal dilatation contributed to decreased operational time, postoperative HAEC, and obstructive symptoms.The majority of children spent between 5 and 8 days in the hospital.The most prevalent postoperative complications were enterocolitis, followed by fecal incontinence, constipation, and other difficulties such as anastomotic leak, perianal excoriations, blockage, and more.

Specific conclusions came from the remaining studies
There was no significant difference in long-term bowel function between TERPT and LERPT in research comparing the two procedures [5,10,11].In terms of hospitalization length, postoperative incontinence, and constipation, TERPT surpassed transabdominal pull-through (TAPT).Overall, the quality of life of patients postoperatively depends on the level of self-esteem in children, which is influenced by the parental response to the child's illness [39].Follow-up after redo surgery showed no complaints of constipation, stenosis, or intestinal obstruction, but soiling and fecal incontinence were reported at higher rates [29,40].In a study by Giuliani et al., lighter impact of the surgical procedure on infants, a lower incidence of complications, and a better long-term outcome of the transanal pull-through compared to the Duhamel approach [41].Zhang et al. showed that HAEC, which occurs due to intestinal obstruction and abnormal microbial flora, is another common complication that can be reduced by using a postoperative rectal tube after a primary laparoscopic endorectal pull-through procedure [42].There is, however, a higher risk of incontinence after laparoscopy with fewer signs of short-term improvement [43].The disorder predominantly impacts males with a ratio of about 4 males to 1 female being affected [44].Congenital malformations contribute to around 7% of neonatal fatalities and result in a global burden of 25.3-38.8 million disability-adjusted life-years (DALYs) [45].The relationship between HD and Down's syndrome is well-known, demonstrating an occurrence of 7.32% [46].
Importantly, even after a successful operation, individuals might have consequences such as enterocolitis, night-time soiling, constipation, and others.Impaired bowel control, infections, scar tissue development, and a weakened blood supply can all lead to these issues.Regular follow-up exams, imaging investigations, and coordination with specialists are required to address these difficulties.

Limitations and drawbacks
The possibility of publication bias exists in this literature review, wherein studies that demonstrate positive or significant outcomes are more likely to be published, while studies with negative or no significant findings might be underrepresented.This bias has the potential to influence the overall findings and could lead to an overestimation of the effectiveness of surgical outcomes.The included studies in the systematic review may have utilized different study designs, including retrospective and prospective designs, leading to heterogeneity in the data.Variability in patient populations, surgical techniques, follow-up durations, and outcome measures can affect the ability to draw definitive conclusions The quality of the individual studies included in the review can vary.Some studies may have a higher risk of bias due to methodological flaws, inadequate sample sizes, or incomplete reporting of outcomes.The inclusion of lower-quality studies may introduce uncertainty or bias into the overall findings.Some studies may have incomplete or insufficient reporting of relevant data, such as complications, long-term outcomes, or patient characteristics.The lack of standardized reporting across studies can make it challenging to obtain a comprehensive overview of surgical outcomes.
The duration of follow-up in the included studies may vary, and there might be a lack of long-term followup data.This limitation hinders the assessment of late complications, functional outcomes, and the durability of surgical interventions.The review might be limited to studies published in English, which are available only in indexed journals.This restriction can introduce language and publication bias, potentially excluding relevant studies that were not accessible or included.
The review may not always account for confounding factors that could influence surgical outcomes, such as patient co-morbidities, surgeon experience, or the presence of additional congenital abnormalities.The absence of comprehensive data on these factors can limit the accuracy of the conclusions drawn.The review may be limited to studies published between 2023 and 2010.Older studies may not be included, potentially affecting the relevance of the findings.

Conclusions
Surgery for HD aims to restore normal bowel function, and most patients achieve good or excellent bowel control post-surgery.TERPT is a preferred technique for HD, replacing staged procedures.TERPT and LERPT are commonly performed for rectosigmoid HD.Post-surgical outcomes include issues like constipation or fecal incontinence.Complications such as perianal excoriation, enterocolitis, anastomotic stricture, leakage, wound infection, and anal stenosis can occur but vary in frequency.Prompt recognition and treatment of enterocolitis are crucial.Achieving continence is important, but some patients may have persistent problems.The length of hospital stay varies depending on the procedure, with laparoscopic approaches generally associated with shorter stays.Enterocolitis is a common complication.Long-term sphincter damage may occur with TERPT.Single-incision laparoscopic surgery (SILS) with Soave TERPT offers cosmetic benefits and faster recovery.Post-surgical outcomes are influenced by various factors, and personalized information from healthcare professionals is essential.

FIGURE 1 :
FIGURE 1: PRISMA flow chart with the description of studies inclusion PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analyses

1 :
pull-through is a safe and feasible option in pediatric patients with a considerably low risk of complications 3 weeks Enterocolitis, night-time soiling, constipation Laparoscopic-assisted pull-through is a safe and feasible option in pediatric patients with a considerably low risk of complications Patients may need 2023 Munnangi et al.Cureus 15(10): e47012.DOI 10.7759/cureus.470124 of 12 Review study NA Evaluation and treatment of the post-HD pull-through patient who is not doing well 1) Fecal incontinence, (2) obstructive symptoms, and (3) recurrent episodes of enterocolitis NA A thoughtful, systematic approach will help the clinician to determine the cause of the problem and adopt a successful therapeutic plan to longer for the LATP group (OR 1.59, 95% CI 1.21-1.96,p<0.001) , 95% CI 0.76-4.04,p=0.19) or length of stay (OR 0.33, 95% CI -0.41 to 1.08, p=0.38).Soave, Duhamel, Sphincteromyectomy, Ileostomy, Sigmoid colostomy, For patients older than ten years with an HD surgical history, the endorectal pull-through In the SD group, anastomotic leakage (4.3%), cuff stenosis (14.5%) enterocolitis (17.4%) were observed.In In the SD group, In children above two years of age, Soave transanal one-stage endorectal pull-through was found to be safe and with low morbidity (PT) NA NA Compared to the DP, the SP was associated with an increased frequency of bowel movements and soiling severity; however, the constipation severity was lower Table showing the postoperative outcome in HD in different studies HD: Hirschsprung's disease LoHs: Length of hospital stays TUE: Transumbilical enterostomy LERPT: Laparoscopic endorectal pull-through TERPT: Transanal endorectal pull-through CS: Corrective surgery DD: Defecation disorder TA: Transanal LAPT: Laparoscopic-assisted pull-through LATP: Laparoscopic-assisted transanal pull-through CTP: Complete transanal pull-through RSPT: Robotic Soave pull-through MIRPT: Minimally invasive redo pull-through TRSPT: Totally robotic soave pull-through HAEC: Hirschsprung-associated enterocolitis SD: Soave-Denda 2023 Munnangi et al.Cureus 15(10): e47012.DOI 10.7759/cureus.