Pediatric Colon Surgery: Challenges, Functional Outcome and Quality of Life

Clinical Presentation 

Children are infrequent visitors to specialists who treat ulcerative colitis. An estimated 20 to 30% of patients may experience their first symptom before 18 years of age, but the diagnosis is rare.3, 4, 5 Less than 5% of patients in population-based studies are diagnosed during childhood or adolescence.6, 7, 8 Furthermore, the incidence of CUC in children has remained stable at 1 to 5 per 100,000 over the past decade, while Crohn’s disease has increased considerably.6, 7, 9, 10, 11, 12 Crohn’s disease is now at least twice as common as CUC, while most studies of adult patients report an equivalent incidence.10, 11, 12, 13 Surgical relevance of these data becomes clear when evaluating modes of presentation. In both diseases, a higher proportion of children than adults present with pancolitis. Up to 65% of children with Crohn’s disease under age 8 will have pure colonic disease.4, 5, 11 The colonic predilection gradually decreases with age; however, there remains a higher likelihood a child with pancolitis undergoing an ileoanal procedure could have Crohn’s disease.2, 5, 14 This is especially relevant because Crohn’s disease is a significant risk factor for pouch failure in pediatric patients after ileal pouch anal anastomosis (IPAA).15 Surgeons may also exercise caution with female patients because of a Crohn’s disease gender predominance. However, multiple recent studies have placed males in the majority of the increase with pediatric Crohn’s disease.5, 7, 11, 12

Abdominal pain also does not help in discriminating the two conditions in childhood IBD. Fewer patients with CUC present with rectal bleeding, while more will simply complain of abdominal pain, increased stool frequency, and urgency.3, 4, 6, 16 Growth failure is often associated with Crohn’s disease but also occurs in CUC.3, 17 This and associated social factors can significantly influence the decision to operate. Genetic predisposition is found in 12 to 44%14 of children depending on age of presentation; the younger the child, the higher the familial incidence of IBD.3, 18, 19, 20 In the office, families of children with IBD resemble those with FAP, many of whom have the condition and have undergone surgery themselves. Parents often know which questions to ask in search for newer procedures that may benefit their child. Most remain touchingly optimistic that their child will do as well or better than they did. Nevertheless, their heightened anxiety and concern cannot be underestimated.

The rarity of CUC in childhood, its different presentation, and the relative lack of treatment experience prompt many pediatricians and pediatric specialists caring for these families to refer the child to a major pediatric center.3 This “institution bias” concentrates pediatric experience at larger centers where child-focused facilities and staff are readily available. Such age-specific care is entirely appropriate but can be isolating. Further efforts to share this experience with pediatric colonic surgery with other institutions are strongly encouraged.

Medical Treatment 

CUC is characterized by cyclic disease flares controlled by medication. Most surgical patients are resistant to the cornerstones of therapy: the aminosalicylates (5-ASA) and steroids. Immunomodulation with azathioprine, 6-mercaptopurine, and cyclosporin is often attempted before surgery. More targeted therapies such as Infliximab and Adalimumab21 are also seeing greater use in pediatric patients. The relatively strong association of the NOD2/CARD15 gene with Crohn’s disease and the greater understanding of the role of effector and regulatory T-cells promise to bring more effective medical therapies.22 The high rate of familial involvement with IBD in children makes this population ideal for future study. Environmental factors also play a role, especially the bacterial environment and the unusually protective association of appendectomy.6, 19, 23 The reproducible correlation of tobacco use and IBD is not only of great interest to parents but may figure prominently in amelioration of pouchitis.24, 25, 26

Surgical Indications 

Fulminant intractable colitis resistant to therapy is a dramatic presentation more common in childhood CUC,4, 5, 11 but other indications to operate include medication toxicity, growth retardation, carcinoma prophylaxis, and severe symptoms. Families acknowledge life-altering surgery as lifesaving, but have little time to absorb the consequences. Patients and practitioners alike may prolong the presurgical course in hope of an elusive remission that can often only be achieved in the operating room. Unfortunately, most of these desperately ill children will just tolerate total proctocolectomy alone; reconstruction is deferred for several months. Earlier identification of these patients is usually not possible, but perhaps genetic or immune stratification of risks will help identify which patients will likely fail medical therapy before they need urgent colectomy.

Steroid-dependent refractory disease has added importance for growing children.3, 15 Middle and high school adolescents are acutely aware of their social limitations and look to surgery to restore a more normal life. Preoperative discussions with these patients center on the quality of life and psychosocial issues as well as postoperative details and specific bowel functional outcome.

Overall, most patients with Crohn’s disease will eventually require surgery, while less than half of those with ulcerative colitis will have colectomy in their lifetime. The increased incidence of pancolitis in childhood produces relatively more treatment resistance, but durable remissions and good control by medication can still be expected. The promise of new therapy is a tantalizing alternative to surgery; however, early consultation with the surgical team and the discussion of the procedure and outcomes are essential for an informed decision. Experienced nurse practitioners are essential to guide patients and families through the process and coordinate a large team of healthcare providers and other patients who have undergone the same procedure.1, 27

Preoperative Evaluation 

Laparoscopy is possible for most children requiring surgery. The extent of reconstruction will depend on disease morbidity, especially the overall patient condition, the presence of toxic colitis, anemia, hypoalbuminemia, elevated CRP levels, and coagulopathic states.28, 29, 30 Correction of mild abnormalities should not preclude complete reconstruction; however, it is unsafe to proceed with major restoration of function in seriously ill children.

Bowel preparation when possible includes a mechanical washout and antibiotic administration. Oral antibiotics the night before are traditional, while intravenous perioperative dosing is essential. Laparoscopy is size-independent and is preferred for large steroid-dependent children.28 When truncal obesity precludes safe pouch construction, weight reduction is recommended following laparoscopic colectomy.

Currently, many centers utilize a laparoscopic-assisted approach and a portion of the procedure is done through small incisions.31, 32 Preoperative evaluation of the child’s abdomen helps determine whether a Pfannenstiel or periumbilical incision is most appropriate. Our preferred method for almost all patients is through suprapubic transverse Pfannenstiel incision.

Selection of Procedure 

Surgery for CUC is performed laparoscopically, laparoscopic-assisted, or open in one, two, or three stages with or without a variety of pouches that are stapled or handsewn to the anus. With such tremendous variation in the pediatric population, greater flexibility is required for treatment recommendations.

The higher incidence of childhood refractory pancolitis makes laparoscopic colectomy and end ileostomy an attractive option. The procedure can be performed without a major incision as the colon is removed through the ileostomy site. Newer intracorporeal ligating devices have reduced the amount of mesenteric manipulation that can be so dangerous in these critically ill patients. Postoperative recovery is still prolonged due to patient condition, but the procedure is effective and safe. The ensuing months before reconstruction also allow for recovery, tapering of steroids, and differentiation from Crohn’s disease.

Laparoscopic colectomy and immediate reconstruction are recommended for children with steroid-dependent or refractory disease. The diverting loop ileostomy is a major concern for children and families. Teens are especially resistant to the concept, prolonging treatment that may in turn make it more likely that they will need one. Our approach is to divert all patients except highly selected patients with normal nutritional parameters. The informed family, perhaps with prior experience with disease, particularly FAP patients will make a strong case for single-stage procedures by avoiding debilitation and medication dependence. Nevertheless, anastomotic leak and pelvic sepsis are still concerning complications that could have major future implications for children.1, 31, 32, 33

The stapled anastomosis is now standard therapy for adults and older adolescents.34, 35, 36 Smaller children still require a handsewn procedure with its attendant concern for later development of cuff adenocarcinoma.37 To date, 19 cases have been reported.36 Pediatric surgeons caring for children with Hirschsprung’s disease generally have more experience with handsewn coloanal anastomosis. Rectal dissection is routinely performed laparoscopically and completed with a transanal mucosectomy. The success of straight ileoanal procedures is a testimony to tremendous long-term adaptability of children.28, 38, 39 Relatively equivalent stool frequencies are achieved with fewer reports of pouchitis. The time taken to achieve these results, however, is generally longer than those children with pouch construction. Ileal J-pouches have therefore become the standard for pediatric reconstruction. Other methods, such as the S-, W-, and lateral pouches have also been recommended by some leading pediatric centers.30 While each approach has its advocate and may be necessary in certain situations, the J-pouch is the benchmark; it is easy to construct and can be done laparoscopically.23, 31, 34, 38, 40

Staged reconstruction following emergency colectomy may be performed without ileostomy provided there are no technical concerns, such as the anastomosis is not under tension and the patients are in excellent health. Three-stage procedures have become less common,32, 34 but remain an option in some patients where emergent colectomy has been performed and on pouch reconstruction there is concern about the anastomosis and a diverting loop ileostomy is performed. Hand-assisted laparoscopy is an attractive option not available to the pediatric surgeon caring for younger patients. The discrepancy between the size of the surgeon’s hand and the small volume of a child’s abdomen precludes its effectiveness in most pediatric patients. With experience, intracorporeal mobilization and vascular ligation becomes more facile and the extra time required for dissection is counterbalanced by the smaller size of the patients.41 Our laparoscopic approach utilizes a Pfannenstiel incision as the best tolerated and most cosmetic approach. Short midline scars are also acceptable but usually not necessary. Also, the authors prefer to reperitonealize the pelvis at the time of surgery in an effort to prevent entrapping the tubes and ovaries in the pelvic dissection.

Indeterminate colitis presents a special problem for pediatric patients and their surgeons. The incidence increases with younger age patients who also have an increasing incidence of Crohn’s disease. The safest approach is to perform a Brooke ileostomy, but data from several adult studies demonstrate excellent results with pouch procedures in the treatment of indeterminate colitis despite a higher complication rate.42, 43 Even the development of Crohn’s disease in the pouch or at the anastomosis may not be the dreaded complication once feared.21 Few patients lose their pouch, but temporary ileostomy for fistulization is more common. The addition of targeted medical therapy may also be important for this unexpected success.

Postoperative Care 

Perioperative total parenteral nutrition is continued, especially for children with high metabolic rates that may not be a tolerating full diet for many days. Ileostomies can be particularly troublesome with prolapse, stenosis, and bleeding. There is a relatively high rate of reoperation for stoma complications but these procedures are preferred over re-exploration for anastomotic leak and pelvic sepsis. Of great concern to children and adolescents is the prospect of returning to school with a stoma. Most children are capable of engaging in school activities and sports within 3 to 4 weeks of surgery but are reluctant to do so. Once again, encouragement and preoperative teaching from the surgical team and experienced nurse practitioners are highly valuable. Often, patient stories and contact with others can allay their concerns.

Perioperative Complications 

The greatest concern for patients and surgeons alike is the high incidence of perioperative morbidity, which overall occurs between 30 and 66% for proctocolectomy with IPAA.15 Infection, obstruction, and anastomotic strictures predominate along with the temporary but sometimes emergent complications associated with the ileostomy. Patients who travel from a distance for care may need to return on multiple occasions for further evaluation and treatment. These episodes can be extremely disappointing to families who have not been informed up front. The practice of at-home anal dilation is also more difficult in children, many of whom opt for outpatient procedures under sedation. Long-term complications center on the high rate of pouchitis and the less common chronic problems with the ileoanal anastomosis. Most children respond well to medication but continued close contact with families is necessary for long-term success. Importantly, we strongly feel that educating parents is vital in the long-term well-being of these children.

The somewhat surprising end result is that these complications of the procedure, with the exception of pelvic sepsis and chronic pouchitis, do not adversely affect quality of life and bowel functional outcome. Laparoscopy is still a relatively newer technique, but with more experience, the benefits of reduced perioperative morbidity may be realized.

Functional Outcome 

Chronic pouchitis and pouch failure occur anywhere from 3 to 10% in large adult series in patients who underwent proctocolectomy with IPAA.44 Two studies have specifically investigated, in a retrospective manner, the functional outcomes of proctocolectomy with IPAA in children.1, 15 Overall, children had excellent bowel health. In fact, children had lower incontinence rates and stooling frequency than most adult studies.45, 46 This finding may be related to the pediatric age group, as others have found functional outcome to be better in younger patients. Importantly, while most postoperative complications did not have a negative effect, chronic pouchitis may have a detrimental effect on functional outcome.10, 44

Randomized trials investigating laparoscopic surgery in adults have demonstrated similar efficacy and safety, with some benefits, to open colonic procedures but no trials in the pediatric age group have been performed. We started performing laparoscopic and laparoscopic-assisted proctocolectomy with IPAA in 1998 for CUC and FAP patients, and our preliminary data suggest that our functional outcomes were similar to our open group. Though there is a sharp learning curve with this technically demanding laparoscopic pelvic procedure, the complication rate did not affect the excellent functional outcomes. Moreover, psychosocial health, which correlates significantly and predicted overall quality of life, was rated to be excellent.

Quality of Life 

To our knowledge there is only one article that has looked at pediatric quality of life after ileal anal J-pouch anastomosis.1 This study also discussed the bowel functional outcomes and correlated these analyses. Data were obtained using the Child Health Questionnaire-28 (CHQ-28; Fig 1). This tool is a measure of the psychological and physical well-being of children age 5 years and older. The CHQ assesses 14 health concepts and derives a summary score to the psychological and physical well-being. This population was compared with the general US population of healthy children. Body image changes, prolonged illness, physical limitations, and bodily pain were reported as normal. The General Health score, which is a subjective assessment of how parents viewed their children’s past, current, and future health and illness, scored lower than the US norm. Once a child had undergone a major procedure, parents viewed their child’s health to be worsened overall. Importantly, the psychosocial assessment of emotional behavior, mental health, self-esteem, family cohesion, and social function were normal. However, parental anxiety regarding their child’s ongoing health remained higher than norms. This heightened level of concern becomes important when dealing with parental concerns during the follow-up period.

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Figure 1. Comparison of Child Health Questionnaire (CHQ) scores for children after ileal pouch-anal anastomosis (IPAA) and for “normal” (ie, healthy) children in the United States. The CHQ evaluates qualify of life from the perspective of a parent or guardian who responds to questions about 14 health concepts. Reprinted with permission from Stavlo PL, et al: Pediatric ileal pouch-anal anastomosis. J Pediatr Surg 38:935-939, 2003.

The psychological and physical summary scores, which measure quality of life, were normal despite the alteration in the general health and parental anxiety scores. These findings argue that while the patient and family have accommodated to the stressors and have a normal quality of life, there remains a high level of parental concern for the child’s well-being that does not dissipate over time.

Future Considerations 

Families often wonder if the excellence achieved in children will decline with age. Studies of adult patients have refuted this concern by showing a steady level of function even in elderly patients. Success in childhood therefore gives promise of life-long relief from symptoms. Follow-up studies of this pediatric group that has undergone ileoanal surgery will be important to determine the real incidence of pouch failure and long-term quality of life. Fertility, pregnancy, and childbirth are of particular concern. Adult studies have demonstrated a decreased level of fertility after proctocolectomy with IPAA in CUC patients. For this reason, the authors prefer to reperitonealize the pelvis to prevent entrapment of tubes and ovaries.47

Summary 

Colon and rectal surgery including open and laparoscopic proctocolectomy with ileal J-pouch anal anastomosis provide excellent bowel function and a normal quality of life in pediatric-aged patients. Special consideration of presentation, anatomic differences, and genetic influences play a unique role in childhood disease. Nonetheless, despite the perioperative complications and some chronic morbidity, the long-term outcome is regularly excellent and quality of life measurements show near-normal indices.