Minimally Invasive Techniques for Hirschsprung Disease

Introduction

In 1889, Harald Hirschsprung first described an infant with congenital intestinal obstruction. The diagnosis was congenital aganglionic megacolon, also known as Hirschsprung disease (HD). ¹ In the past, first surgical step in these patients was laparotomy, intestinal biopsies, and colostomy creation. In 1948, Swenson and colleagues proposed a full-thickness dissection of the rectum and end-to-end anastomosis, ² while Duhamel suggested a retrorectal pull-through. ³ Few years later, Soave first described an extramucosal dissection of the rectum, aimed to preserve the pelvic innervation at best. ⁴ Surgical treatment of HD evolved in the last decades with the introduction of new technologies, such as one-step minimal access surgery. Nowadays minimal access can be performed with two different approaches, total transanal endorectal pull-through (TEPT also known as De Latorre and Ortega) and laparoscopic-assisted transanal pull-through (LA-TAPT also known as Georgeson).^5,6

The aim of present study is to compare the results of two Italian Centers, each applying one of these different approaches.

Materials and Methods

Data of all patients admitted between January 2011 and December 2016 in two Italian referral hospitals for HD were retrospectively collected and analyzed. We compared the two hospitals as the surgical approach for HD was different (TEPT in one and LA-TAPT in the other), with the choice defined a priori by surgeon's experience and preference. In LA-TAPT center, patients with major cardiac anomalies undergo open approach, different from TEPT; therefore, we excluded these patients from the study. In both hospitals, the surgical team was composed by a senior surgeon and 5 assistant surgeons.

Patients with redo surgery, with total colonic aganglionosis (ileostomy), lost at follow-up, or with missing data were excluded from the present study.

Patients who underwent TEPT and LA-TAPT were compared for gestational age, birth weight, age and weight at surgery, associated malformations, genetic syndrome, length of hospital stay, and early postoperative complications (within 30 days).

We included the following in postoperative complications: redo surgery, episodes of pre- or postoperative enterocolitis, mild and severe dermatitis, stenosis, or other infections. We defined severe perianal dermatitis as skin lesions, characterized by punched-out erosions or ulcerations and easy bleeding. Mild dermatitis was defined as rash presenting as perianal redness.

Study population was stratified in three groups according to the age: group A, infants aged within 3 months of life; group B, patients aged between 3 and 12 months; and group C, children older than 1 year of age.

Results

During the study period (2011–2016) data of 145 patients were collected from the two pediatric centers; 2 patients required a redo surgery (1 had anastomotic leakage and 1 suffered intestinal perforation during postoperative calibration by parents) and were excluded from the study. In addition, 36 with total colonic aganglionosis or lost at follow-up/missing data were excluded from the study. Therefore, 107 patients were included, 61 who underwent TEPT and 46 LA-TAPT.

Age distribution in the two Centers was statistically different with a prevalence of patients below 3 months of age in TEPT group, while LA-TAPT more commonly included patients aged >12 months (P = .01).

No differences were found between TEPT and LA-TAPT groups in terms of median gestational age and median body weight at birth (P = 1 and .98, respectively), while median body weight at surgery and median age at surgery were significantly higher in LA-TAPT group (P < .0001 and <.001, respectively). Not surprisingly, patients who underwent TEPT had a significantly higher prevalence of associated major cardiac anomalies (hemodynamically significant ventricular or atrial septum defects, complete atrioventricular canal defect, aortic coarctation, transposition of great vessels, patent ductus arteriosus requiring medical or surgical closure) (P = .006), while the incidence of genetic syndromes was similar in the two groups. Median length of hospital stay was significantly longer (24 hours, P = .0007) in TEPT group. Median length of cumulative hospital stay was also higher in TEPT group (P < .0001). One patient for each group required redo surgery within 30 days. The prevalence of both pre- and postoperative enterocolitis was similar in the two groups. TEPT group suffered from mild perianal dermatitis significantly more frequently than LA-TAPT group (P = .001), while the prevalence of severe dermatitis was comparable in the two groups. No difference in terms of stenosis, infections, or other complications was observed. Table 1 summarizes main findings.

Discussion

In this study comparing TEPT and LA-TAPT, we found that patients who underwent the former had a younger age and weight, higher prevalence of associated cardiac anomalies, and mild perineal dermatitis. Patients who underwent LA-TAPT had a shorter length of hospital stay.

TEPT is worldwide accepted as the first choice in patients affected by HD. ⁷ Endorectal pull-through can be performed either using a completely transanal approach or with previous laparoscopic biopsies and preparation of the colonic vessels. To compare these two approaches, we analyzed two high volume Italian pediatric centers. The concept of minimally invasive technique for patients with HD was born to reduce the risks of bowel obstruction, laparocele, and surgical site infections and reduce postoperative pain associated to laparotomy. ⁸ Moreover, avoiding abdominal incision reduces the length of hospital stay.^9,10 Since 1995, when Georgeson et al. first described the laparoscopic procedure for HD also in infants, a large number of authors reported the benefits of this technique in terms of reducing postoperative pain, length of hospital stay, and the risk of postoperative intestinal obstruction due to adhesions. ¹¹ In 1998, De La Torre and Ortega-Salgado proposed a single-stage total transanal approach, reaching similar results to laparoscopic technique with shorter operative time and with no transperitoneal access or pneumoperitoneum. De La Torre and Ortega-Salgado also underlined similar outcome, as esthetic results, postoperative pain, and shortness of length of hospital stay. ⁶ Therefore, a minimally invasive approach that avoids a laparotomy, whether open or laparoscopic, seems to be associated with better postoperative outcomes.

In our study, we analyzed the outcomes in two Italian centers that adopt the two different approaches to compare the outcomes of those two techniques. We found no statistical differences in terms of outcomes such as redo surgery, enterocolitis, anastomotic stenosis, and infectious complications. Our findings are similar to those from Guerra et al. who reported a comparison between TEPT and LA-TAPT in 139 patients. ¹² They analyzed major postoperative complications (such as dehiscence of the anastomosis, stenosis, postoperative episodes of enterocolitis, fecal incontinence, and bowel obstruction symptoms) and length of hospital stay, and they did not find any difference. The main differences were as follows: a shorter operative time and lower costs for TEPT. ¹³ Similarly, in a recent meta-analysis, Thompson and colleagues found no differences between the two techniques, in terms of incidence of enterocolitis, constipation, and episodes of postoperative obstruction. ¹⁴

Other authors reported a higher incidence of fecal incontinence in TEPT group, possibly due to prolonged stretching of anal sphincter during surgery. ¹⁵ Contrarily to Guerra et al., we found a longer length of stay for patients who underwent TEPT compared to those treated with LA-TAPT. This may be due to several reasons. First, patients treated with TEPT were younger than those who underwent LA-TAPT, with the majority below 3 months of age. This difference may at least, in part, be responsible for the longer hospital stay, for a more cautious postoperative observation. Second, patients who underwent TEPT had a higher prevalence of associated cardiac anomalies that may also be responsible for a prolonged length of stay, both pre- and postoperatively. It must be also underlined that the difference in postoperative stay between the two groups is only 24 hours, that is, however statistically significant. Patients who underwent TEPT also experienced more frequently mild perineal dermatitis. These findings may relate to the younger age in this group, with the reduced period of contact between the stools and the perineal skin that is not yet used to its chemical “aggression.”

Our study has some limitations. The first is the retrospective nature of the study, without a purposely designed protocol, leading to potential differences in treatment over time. In addition, for its retrospective nature, the analysis was limited to available data, and we could not identify a priori the variables that may have been of interest. In addition, the comparison of different institutions, with different management protocols, may participate to the differences found between patients who underwent TEPT and LA-TAPT. Furthermore, we had no complete data about operative time in both groups, which could impact postoperative outcome (e.g., the time of anal canal stretching).

Conclusion

In conclusion, despite all limitations of our study due to the heterogeneity of our population and the lack of standardized protocol for HD patients, we found no marked differences related to the surgical approach. We conclude that the longer hospital stay and the higher prevalence of mild dermatitis in TEPT group are likely related to the higher prevalence of hemodynamically significant cardiac diseases and the significantly younger age of this patient group. Both TEPT and LA-TAPT are minimally invasive approaches, safe and feasible in children with HD. The surgeon may choose the procedure based on his/her preference, maybe except for patients with severe cardiac anomalies. TEPT allows the same surgical advantages and outcomes as LA-TAPT even in very young infants, avoiding pneumoperitoneum and the transperitoneal approach that would otherwise be required in less than 20% of HD patients, and maybe allow earlier surgery for HD. The laparoscopic-assisted approach may have the advantage to allow intestinal biopsies earlier during the operation. This could optimize the time for perioperative histological examination to define the level of aganglionosis and simplify the preparation of the colon for pull-through that would be performed through the anal canal, being technically more demanding and possibly requiring anal sphincter stretching.