Early Experience with Variant Two-Stage Approach in Surgical Management of Inflammatory Bowel Disease Colitis in the Pediatric Population

Abstract

Introduction:

Multistaged surgical management of inflammatory bowel disease (IBD), culminating in ileal pouch-anal anastomosis (IPAA), can provide cure for refractory IBD symptoms while maintaining fecal continence. Surgical approaches to IPAA historically included a three-stage approach done by subtotal colectomy (STC) followed by IPAA with diversion. Recently, a variant two-stage approach without diversion at IPAA has become increasingly utilized, yet evidence of the efficacy of this approach is limited.

Methods:

Retrospective review of patients aged 5–21 years who underwent initial STC, followed by a total proctocolectomy with IPAA +/− diversion for medically refractory IBD from January 2010 to August 2018 (n = 25).

Results:

Majority of IPAA procedures were done laparoscopically (88.5%). Thirteen patients (52%) underwent two-stage variant IPAA. There were no differences in readmission rates (66.7% versus 53.8%, P = .5) or reoperation rates (50% versus 30.8%, P = .3) between groups. Forty percent of patients experienced a complication after IPAA. Complication rates were similar between two-stage and three-stage IPAA groups (38.5% versus 50%, P = .33). Complications within the two-stage group included anastomotic leak, pouchitis, wound infection, anastomotic stricture, and incarcerated hernia. Complications within the three-stage group included bloody ostomy output, dehydration, anastomotic stricture, small bowel obstruction, and pouch volvulus.

Conclusions:

Treatment of refractory IBD in children remains challenging, but STC followed by IPAA is an approach that provides symptom relief and preserves continence. Complication rates remained unchanged regardless of whether IPAA was conducted with or without diversion, demonstrating that the two-stage variant approach is a safe and feasible treatment that may reduce subsequent anesthesia exposure and trips to the operating room.

Introduction

The incidence of inflammatory bowel disease (IBD) is rising both in the United States and worldwide, and a significant proportion of these new diagnoses are occurring in children. Approximately 20%–30% of all new diagnoses of IBD occur in patients younger than the age of 20.^1–6 A pediatric diagnosis of IBD is associated with more severe disease manifestations, more rapid disease progression and extension, and a higher probability of needing surgical intervention.^2,7

Surgery is indicated in disease recalcitrant to medical management or after the development of disease sequelae such as stricture, abscess, bleeding, or perforation. Although the exact figure varies, the literature reports that between 15% and 20% of children diagnosed with IBD-related colitis will require colectomy within 5 years of their diagnosis.^3,6,8 Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) has become the procedure of choice for patients with ulcerative colitis (UC) and utilization of this procedure has extended to select patients with Crohn's disease (CD) and indeterminate colitis. However, the best path from active colitis to IPAA remains under debate.¹

The two most common surgical paths to IPAA are a three-stage approach, which involves an initial procedure of a subtotal colectomy (STC) with end ileostomy, subsequent IPAA creation with diversion, followed by eventual stoma closure, and a two-stage approach, which involves total proctocolectomy, IPAA creation with diversion, and, finally, subsequent stoma closure.¹ Other described approaches include a one-stage total proctocolectomy and IPAA. More recently, a variant two-stage approach, which involves an initial STC with end ileostomy, followed by completion proctectomy with IPAA creation without diversion has become increasingly utilized. The potential benefit of the variant two-stage approach is that it allows patients to recover from acute illness and allows for an opportunity for patients to be weaned from steroid or biologic medications before IPAA creation.^1,4,8–10 Despite the increasing utilization of the variant two-stage approach in the pediatric population, evidence as to the efficacy of this approach is limited and widely debated with strong proponents on either side. At the crux of this debate is the question of whether or not an ileal pouch should be routinely diverted with a loop ileostomy. Our aim of this study is to examine outcomes of pediatric patients undergoing variant two-stage approach IPAA for IBD-related colitis at our tertiary care children's hospital and compare these results with those children who underwent diverted pouches.

Methods

Following Institutional Review Board approval, the records of all patients aged 5–21 years who underwent operative treatment for medically refractory IBD-related colitis with initial STC, followed by total proctocolectomy with IPAA +/− diversion from January 2010 to August 2018 at our tertiary care children's hospital, were obtained. Twenty-five unique patient records were identified as meeting these criteria. Both inpatient and outpatient records were reviewed when available. Patient characteristics, intraoperative details, and postoperative outcomes were collected and subsequently analyzed. Complications considered were only those occurring after completion proctectomy and IPAA creation. Statistics were calculated by using Fisher's exact test and the Student's t-test, as appropriate by using Prism 7. All means reported ± standard deviations.

Results

The average age of our patients at the time of initial STC was 13.3 ± 3.4 years. Patients were predominately male (52.0%) and Caucasian (56.0%). Thirteen of our 25 patients underwent the variant two-stage approach without diversion, and 12 underwent the traditional three-stage approach with diverting ostomy. Among the 12 patients undergoing conventional three-stage IPAA, reasons for diversion included family preference (75%), technical considerations including tension of the anastomosis and extensive pelvic adhesions (16.7%), and 1 patient diverted for pre-existing CD.

Patient preoperative characteristics, including BMI, preoperative albumin, steroid usage before IPAA surgery, and duration of IBD symptoms, were not significantly different among the two groups (Table 1). Indication for initial STC was UC in 84.6% of patients, CD in 4.0% of patients, and indeterminate colitis in 11.4% of patients. Based on dictated operative reports, as well as notations in inpatient and outpatient records when available, 86.5% of all patients were on steroids before initial STC. One patient initially diagnosed with indeterminate colitis had a change in diagnosis after IPAA creation to CD based on pathology and the formation of pouch vaginal fistula. All other patients had no change in postoperative diagnosis.

Table 1.

Preoperative Patient Characteristics

	Variant two-stage approach	Three-stage approach	P
n	13	12
Age (years)	12.5 ± 3.8	14.2 ± 2.6	.22
% Male	46.15	58.34	.70
% Caucasian	57.14	50.00	.70
Body mass index	19.95 ± 3.62	19.65 ± 2.39	.82
Preoperative albumin	3.708 ± 0.602	3.167 ± 1.005	.14
% of patients on preoperative steroids	84.62	58.33	.20
% patients with duration of symptoms >1 year	84.62	91.67	>.99

The majority of all procedures, regardless of approach, were preformed laparoscopically during the study period (88.5%). Length of hospital stay after IPAA creation was slightly, but significantly, longer in the two-stage variant group compared with the traditional three-stage group (7.8 ± 2.7 days versus 5.3 ± 1.1 days, P < .01). In addition, patients in the three-stage group stayed in the hospital an average of 3.3 ± 0.93 days after the operation for stoma closure.

Overall, 40% of patients experienced a complication after completion of proctocolectomy with IPAA. Complication rates were similar between two-stage variant and three-stage IPAA groups (38.5% versus 50%, P = .69) (Table 2). There were no mortalities in either group. There were no significant differences in overall unplanned readmission rates (53.8% versus 66.7%, P = .69) or unplanned reoperation rates (30.8% versus 50%, P = .43) between patients undergoing the two-stage variant approach and patients undergoing the three-stage approach.

Table 2.

<30-Day Postoperative Outcomes After Ileal Pouch-Anal Anastomosis

	Variant two-stage approach	Three-stage approach	P
Anastomotic leak (%)	1 (7.69%)	0	>.99
Wound infection (%)	1 (7.69%)	0	>.99
Bloody ostomy output (%)	0	1 (8.34%)	>.99
Dehydration (%)	0	1 (8.34%)	>.99

There were only two complications within the two-stage variant group occurring less than 30 days after IPAA, and two complications occurring within the three-stage group. Complications within the two-stage variant group within this timeframe were an anastomotic leak requiring drainage by interventional radiology and one abdominal wall port site infection, whereas complications in the three-stage group included hospitalizations for bloody ostomy output and dehydration secondary to intractable nausea and vomiting (Table 2). Greater than 30 days after IPAA, there were four total complications in the two-stage variant group, including 2 patients admitted for treatment of pouchitis, 1 patient with anastomotic stricture, and 1 patient requiring repair of ventral hernia at the previously close STC stoma site. One patient within the three-stage group also had anastomotic stricture requiring dilation at this time point.

Complications within the three-stage group occurred predominantly after the third operation where stoma reversal occurred. These complications included 1 patient requiring two hospital admissions for small bowel obstruction treated with NGT decompression, 1 patient requiring four hospital admissions, 2 for anastomotic stricture, and 2 for pouch volvulus requiring laparoscopy. One patient in the three-stage group who was initially diagnosed with indeterminate colitis before STC was ultimately diagnosed with CD presenting as a pouch-vaginal fistula subsequent to stoma closure (Table 3). The duration of outpatient postoperative follow-up was not significantly different between the two groups (31.4 ± 31.2 months versus 32.3 ± 18.7 months, P = .93).

Table 3.

>30-Day Postoperative Outcomes After Ileal Pouch-Anal Anastomosis

	Variant two-stage approach	Three-stage approach	P
Pouchitis (%)	2 (15.38%)	0	.79
Anastomotic stricture (%)	1 (7.69%)	2 (16.67%)	.59
Ventral hernia (%)	1 (7.69%)	0	>.99
Small bowel obstruction (%)	0	2 (16.67%)	.22
Pouch volvulus (%)	0	1 (8.34%)	>.99
Postoperative diagnosis of Crohn's disease (%)	0	1 (8.34%)	>.99

Discussion

Restorative proctocolectomy with IPAA creation is the surgical gold standard for children with IBD-related colitis who require colectomies. One of the primary benefits of this procedure is that it allows for removal of the diseased colon and maintenance of fecal continence.^1,4,9,11,12 Surgical approaches to this procedure include a one-stage, two-stage, three-stage, and a more recently popularized “variant two-stage approach.” There is currently no consensus as to which of these approaches produces the best outcomes, or whether one approach should be utilized in specific clinical scenarios. An integral part of this discussion is whether or not a diverting stoma is necessary at the time of IPAA creation. Previously, studies in both the adult and pediatric literature suggested that creation of diverting ostomy is protective against the development of pelvic sepsis after anastomotic leak.^1,13,14 More recently, this thinking has been called into question and innovative surgical approaches that reduce the need for diverting ostomy are now increasing in popularity as ostomies themselves can cause significant morbidity secondary to skin infections, obstruction, prolapse, and negative body image.^4,9,12,15

Reducing unnecessary placement of diverting ostomies, and thus reducing their associated morbidities, is especially important in the pediatric population. Therefore, it is the aim of this study to add to the body of literature examining the need for diverting ostomy at the time of IPAA creation in the pediatric population. In the adult literature, Samples et al. conducted a 7-year retrospective review of 248 patients comparing patients undergoing the variant two-stage approach with those undergoing the classical two-stage approach. This study found no significant difference in 3-year cumulative leak rates between the two groups, and overall morbidity was equivocal, despite the fact that the patients in the two-stage variant group tended to be sicker and with more severe symptomology. The authors explain this finding by noting that the two-stage variant approach allows for longer healing time between initial STC and IPAA creation. In addition to demonstrating equivocal outcomes, these authors also reported reduced hospital stay and overall reduced hospital cost for the two-stage variant group.⁴

Similarly, Zittan et al. conducted an 11-year retrospective review of 460 adult patients undergoing either the classical two-stage approach or the variant two-stage approach. This study again demonstrated that patients undergoing the variant two-stage approach had more severe IBD manifestations. Despite having more severe disease, patients in this study undergoing the variant two-stage approach had a significantly lower leak rate than those undergoing the classical approach. Again, these authors cite the ability of patients to heal and potentially be weaned from steroid or immunomodulatory medications in the time between STC and IPAA as the cause of improved outcomes following the variant approach, despite being utilized in a sicker patient population.¹

In the pediatric literature, Chen et al. and Bismar et al. attempt to address this same question, although ultimately coming to differing conclusions. In the Chen et al. study, 37 pediatric patients undergoing either variant two-stage or classical two-stage IPAA after STC are retrospectively reviewed. These authors demonstrate a significantly increased length of stay and 30-day complication rate (64.71% versus 15%, P = .003), in the variant two-stage group. Although it did not achieve significance, they also demonstrated a strong trend toward increased anastomotic leak rate within the variant group (30% versus 5%). Despite these differences, there was no difference in long-term functional outcomes, including number of daily bowel movements or symptoms of incontinence, among the groups.⁸

Bismar et al. also examined outcomes of pediatric patients undergoing either traditional two-stage IPAA or variant two-stage IPAA after STC. This retrospective study included 41 children over a 13-year period. These authors found no difference in postoperative complications, return of continence, or return of appetite between groups. The classical two-stage group did require significantly more antidiarrheal agents (1.48 versus 0.71, P = .01) than the variant group, but overall there was no significant difference in outcomes among the groups.⁹

In our series, preoperative characteristics, including severity of IBD-related colitis symptoms, were equivalent between our two groups. We also demonstrated a significantly increased length of stay in our variant two-stage group; however, there was no significant difference in overall complications, unplanned readmissions, unplanned reoperations, or anastomotic leak rates. It is unclear what contributed to the increased length of stay for our variant patients given that there was no significant difference in their preoperative characteristics or postoperative outcomes, but likely this increased length of stay is related to enhanced caution on the part of the surgical team to ensure that the undiverted variant patients show no signs of possible leak or associated sepsis before discharge. Importantly, this difference in length of stay was neutralized when including the subsequent length of stay after ileostomy reversal for those children who underwent diverted pouches.

We did not observe that the types of complications seen between the two-stage variant group and the three-stage group in our study cohort were specifically related to the presence or absence of a stoma. The complications incurred were complications intrinsic to surgical intervention generally for this disease process. There was 1 patient within the three-stage group who was hospitalized less than 30 days after IPAA for bloody ostomy output. This complication is directly related to the presence of a stoma and could have been avoided if this patient was not diverted. The other complications experienced by patients in our study, such as anastomotic stricture, anastomotic leak, hernia formation, and pouchitis, are not related to stoma creation. Therefore, not only were the complication rates the same among both groups but also the reasons for these complications remained unchanged whether diversion was chosen or not. This observation further supports the idea that creation of a stoma adds additional procedures, anesthesia exposures, and potential risks without a robust benefit in our study population.

Although IPAA creation for UC is widely implemented, IPAA for CD is more controversial. IPAA for CD remains strongly debated as there is concern that risk for disease recurrence within the ileal pouch is high and will ultimately necessitate pouch excision.¹⁶ Recent literature suggests that IPAA for CD is feasible if patients with appropriate disease characteristics are identified preoperatively and families are counseled properly.¹⁶ Two patients in our study cohort had CD and both were in the three-stage group. One patient had a preoperative diagnosis of CD and IPAA was chosen for this patient based on favorable preoperative disease characteristics, including the absence of active perianal disease or small bowel disease. This patient did not have recurrence of CD within the pouch and pouch functionality has been maintained. The second CD patient within our study cohort was preoperatively diagnosed with indeterminate colitis, but was subsequently diagnosed with CD postoperatively after formation of a pouch-vaginal fistula requiring diverting loop ileostomy. This patient continues to require diversion, and is the only patient within our study who did not maintain a functional pouch. All patients with UC or indeterminate colitis have maintained functionality of their pouch as of the end of follow-up for this study, and thus showed no difference in this important long-term functional outcome based on type of IBD diagnosis.

Our study is limited by its retrospective nature and small sample size, thus limiting the power of the study. Ideally, we would like to conduct a prospective randomized controlled study examining this question, but conducting such a study may prove difficult to achieve surgeon buy-in. In addition, our study was limited by access to complete patient records as many of the patients included in our study were seen at our institution before implementation of our electronic medical record system and access to paper charts was limited. All inpatient records, outpatient records, and medication lists were analyzed as available. Despite its limitations, our study results contribute to the body of knowledge on IPAA surgical approaches, and will hopefully help to inform both physician and family decision making regarding the appropriate surgical approach for a child requiring surgical intervention for IBD. In our experience, complication rates remained unchanged whether IPAA was conducted with or without diversion. Our experience is congruous with other published results in both the pediatric and adult literature, demonstrating that adoption of the two-stage variant approach is a safe and feasible surgical treatment plan.

Footnotes

Disclosure Statement

No competing financial interests exist.

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