Diarrheal disease was described in the early writings of Hippocrates (c. 400 BC) as a significant health problem (4). Bailey, a British pathologist who lived from 1761 to 1823, first reported diarrheal disease attributed to what would later be termed ulcerative colitis (5). He described autopsy reports of intestinal pathology, suggesting that UC was responsible for mortality in the late eighteenth century. It was not until the nineteenth century that a differentiation emerged between the more common infectious diarrhea and UC. Crohn (6), in his historical treatise on UC from 1962, noted autopsy descriptions of 200 cases of diarrhea and dysentery from the Union Army during the Civil War. These cases were likely attributed to UC, and herald the time when the term “ulcerative colitis” was first applied. Wilks and Moxon published their classic anatomic description of UC 16 years later in 1875, differentiating it from dysentery (6). In 1907, Lockhart-Mummery (7) first reported an increased incidence of colon carcinoma in 7 of 36 UC patients. Kirsner (8) later recommended chronic surveillance of UC patients with an illuminated proctosigmoidoscope.

Medical management in this early era consisted mainly of dietary restrictions and homeopathic medications. The original surgical therapy was a sigmoid colostomy described by Pennel in 1850 (9). Subsequently, an irrigation appendicostomy was described in 1902, followed by the use of a completely diverting ileostomy in 1913. These diversions were later used in conjunction with partial resection of the diseased colon with antegrade irrigations initiated either through the appendicostomy or ileostomy.

Total colectomy, with or without a proctectomy, and an end ileostomy were considered definitive therapy in the 1940s. The resultant high-output ileostomy was associated with significant peristomal complication rates until Brooke described surgical maturation of the stoma in 1952 (10). Brooke’s technique permitted effective appliance application, therefore abrogating many of these local complications.

The modern era of surgical therapy for UC was heralded by a novel anus-saving technique using a rectal mucosectomy and intestinal pull-through procedure reported by Ravitch and Sabiston in 1947 (11). However, the resultant acute and chronic postoperative complications
rendered this technique initially unacceptable, being favored by the subsequently developed Kock continent ileostomy. The Koch pouch permitted reasonable fecal continence via the catheterized stoma and potential freedom from a chronic appliance (12). However, a stoma was still necessary, and the procedure was accompanied by significant complications.

This undesirable situation led to a reevaluation of the intestinal pull-through procedure initially described by Ravitch and Sabiston. A modification of the Soave technique commonly used for the correction of Hirschsprung’s disease was proposed for the treatment of UC by Martin and LeCoultre (13). Emphasizing extensive preoperative preparation, precise operative technique, and the addition of a temporary diverting ileostomy, this modification resulted in an acceptable complication rate and functional results. However, significant postoperative morbidity persisted with an undesirable high stool frequency rate and nighttime incontinence following ileostomy closure (14).

These complications prompted the further addition of several types of surgically constructed ileal reservoirs or pouches that could be used in conjunction with the endorectal ileal pull-through procedure (ERIPT) (15,16,17,18,19). Indeed, pediatric surgeons were leaders in developing the contemporary surgical options now used in the management of UC. The addition of a pouch offered faster postoperative recovery, less frequent stooling, and facilitated an earlier return to a more normal pattern of daily living. The introduction of an ileal pouch had its negative effect, however, creating a new entity of reservoir inflammation termed pouchitis. Pouchitis manifests as diarrhea, bleeding, and pain, and requires chronic antibiotic therapy in 26% to 40% of patients (18,19,20,21). Pouch use is still debated, because outcomes following the ERIPT with or without a pouch are relatively equivalent after the first year. This has prompted some pediatric surgeons to avoid pouch use in the younger pediatric patients who seem to have less morbidity in the early postoperative period.

The etiology of UC remains undetermined. UC occurs with an equal gender distribution and is four times more frequent in the white population. The incidence of UC is increased in the United States and Europe, especially in Scandinavia and England, demonstrating regional variation (22,23,24). UC also occurs four times more frequently in Jewish populations (25), with a higher incidence in Jewish immigrants to North America and Europe compared with Jews living in Israel. There is a lower incidence in Mediterranean regions, Africa, and Asia. These variations suggest a strong genetic risk for UC coupled with potential environmental and/or socioeconomic factors. Although the incidence of UC exceeded that of Crohn’s disease in the first half of the twentieth century, these inflammatory bowel diseases (IBDs) are now equivalent.

The etiology of IBD has been related to environmental factors, but no conclusive evidence implicates diet, as previously suggested. The effect of cigarette smoking on the incidence of IBD has been widely studied. Harries and colleagues first linked a lower incidence of UC with smoking in 1982 (26). Although smoking doubles one’s risk for Crohn’s disease, the incidence of UC is reduced by 50%. At least one adult case has shown remission of UC related to the use of nicotine gum. The risk of UC remains increased in former smokers as compared with those who have never smoked (27).

Appendectomy appears to protect against the development of UC (28). Patients having undergone prior appendectomy developed symptoms of UC at a significantly later age. However, there was no impact on the extent of disease, disease course, use of immunosuppressive therapy, or ultimate need for surgical treatment. Prior appendectomy is also associated with the development of primary sclerosing cholangitis. The performance of an appendectomy is unlikely to be of benefit in cases of established UC (28).

UC and Crohn’s disease are distinct entities with a significantly different clinical prognosis that can be difficult to differentiate using anatomic and histologic criteria. UC is a chronic mucosal inflammation of the large intestine invariably involving the rectum, and extending proximally in continuity to involve all or part of the colon. Children are most likely to develop pancolitis, with the rectosigmoid remaining the most severely involved area. In 10%, there is mild terminal ileal inflammation and edema. This backwash ileitis is a misnomer because no evidence supports the etiologic reflux of luminal contents (52).

Crohn’s disease, in distinction to UC, is a transmural granulomatous disease that typically involves the small intestine. The seldom seen Crohn’s colitis can, however, be initially confused with UC. Frank distal ileal involvement should implicate Crohn’s disease. Cases of indeterminate colitis should be thoroughly investigated prior to undertaking surgical treatment, when possible.

UC is typically diagnosed in young adults, although 18% of cases present between 10 and 20 years of age, and 4% in children younger than 10 years of age (52). Symptoms associated with pediatric UC are listed in Table 87-1. Presentation is usually insidious, with persistent diarrhea and rectal bleeding, commonly with pus or mucous. In 15% of children, however, the onset is fulminant with profuse bloody diarrhea, severe crampy abdominal pain, fever, and occasionally sepsis. The distribution of UC in children is usually more extensive compared with adults and indices
of disease severity can be unreliable. Most children respond to the initiation of medical therapy, but 5% will develop toxic megacolon (Fig. 87-2) necessitating emergent surgery.

The significant rectal bleeding that occurs is predictable when the gross and microscopic picture of UC is considered. The mucosal edema and ulcerations combined with the exuberant granulation tissue preclude normal colonic absorption. The resultant diarrhea irritates and produces further disruption of the friable mucosa. Crampy lower abdominal pain or tenesmus can be presenting symptoms, but tend to occur more commonly with chronic disease. Other chronic symptoms include anemia, anorexia, weight loss, growth retardation, and chronic fatigue. Often, these chronic symptoms manifest in children as the lack of desire to participate in social and athletic activities.

Growth retardation is predominantly related to the degree of intestinal inflammation and resultant poor nutrition in combination with the side effects of medical therapy (54). Approximately 14% of pediatric UC patients have decreased height at the time of diagnosis (55). Many more manifest decreased growth velocity, as well as delayed sexual maturation (56). Short stature UC patients have normal growth hormone levels, but lower urinary gonadotropins (57). There is a linear relationship between growth and disease activity, which is independent of steroid use (54). This growth delay persists throughout puberty and is not reversed after surgery, implicating that growth failure is a “prepatterned” manifestation of IBD (54).

Most children develop quiescent colitis punctuated by periodic exacerbations, often precipitated by stress or infections. Although not causative, psychological factors and stress do play a role in relapse rates. Permanent remission after a single attack occurs in fewer than 10% of children with UC. Many progress to chronic colitis with shorter and less frequent remissions. Nearly 50% of those diagnosed in childhood will need a colectomy before the age of 18 (58) as will nearly one-half of all children with pancolitis. Predictive factors for colectomy also include steroid use. Nearly 77% of those who are refractory to medical management came to operation within 3 years. Falcone and colleagues provide a review on predicting the need for colectomy in the pediatric UC patient (58).

Colorectal carcinoma is reported in 3% of children with UC patients within 10 years of disease presentation and increases by 10% to 15% with each subsequent decade (3).
Cancer risk is associated with pancolitis, earlier symptom development, and frequent flares. Dysplasia on endoscopic biopsy indicates a high risk for subsequent carcinoma. Colonoscopic surveillance may be associated with a decreased risk from death from colorectal carcinoma in patients with long-standing UC (59). As the time to dysplasia in IBD may be accelerated, earlier consideration for definitive surgery is encouraged (60).