Perrault, Jean

SummaryAt our institution, proctocolectomy with rectal mucosectomy and ileoanal anastomosis with endorectal pull‐through is now the procedure of choice for young patients with chronic ulcerative colitis and familial polyposis. We have followed up 66 patients (37 male and 29 female, with a median age of 16 years) for at least 6 months after closure of the temporary ileostomy. The patients were seen regularly in follow‐up or were sent a comprehensive questionnaire to assess their condition. Twenty‐two patients had the construction of a J‐pouch. and 44 had a straight ileo‐anal pull‐through. Stool frequency, day (median 4 to 7 stools) and night (median 1 to 3 stools), was similar in the two groups. Voluntary continence was excellent in both groups during waking hours. Occasional night‐time incontinence in small volumes, mild perianal itching, and minimal bleeding from irritation were similar in both groups. Most of the patients could distinguish gas from stool. When medication was used, bulk agents were usually chosen, with a nonspecific anti‐diarrheal agent taken occasionally. A few patients with the straight ileo‐anal pull‐through expressed some dissatisfaction early during the follow‐up period: however, all but two patients expressed satisfaction later. Of the 66 patients, 41 had at least one complication during the postoperative period; however, in 26 the complications were minor. The more severe complications included small bowel obstruction (11 patients requiring surgical decompression), infection (one patient requiring surgery), and transient neuropathy (three patients).

ABSTRACTBackgroundInflammatory bowel disease (IBD) in adults with primary sclerosing cholangitis (PSC) is characterized by pancolonic involvement, a high frequency of rectal sparing, and an increased risk of pouchitis and colorectal neoplasia. The clinical features of IBD in pediatric patients with PSC have not been well described. The aim of this study was to characterize the frequency, clinical features, and natural history of IBD in pediatric patients diagnosed with PSC.MethodsA retrospective chart review was performed for all patients 18 years of age or younger diagnosed with PSC seen at the Mayo Clinic between 1975 and 1999. Endoscopic and histologic features and surgical and postsurgical outcomes were recorded.ResultsFifty‐two children with PSC were identified. Forty‐three patients (84%) were also diagnosed with IBD. In 36 of 43 cases, there was a sufficient diagnostic evaluation to allow a detailed review. Thirty‐two of 36 patients (89%) had ulcerative colitis and 4 of 36 patients (11%) had Crohn’s disease. In 4 of 36 patients (11%), IBD was asymptomatic. Although the most frequent endoscopic presentation of IBD was universal colitis, endoscopic rectal sparing was frequently noted (27% of colonoscopic studies). Of the four patients diagnosed with Crohn disease, in none did perianal, fistulizing, or stricturing disease develop. Proctocolectomy was performed in six patients (17%); three operations were performed for dysplasia. Pouchitis complicated four of the five ileal pouch–anal anastomoses procedures.ConclusionsAmong pediatric patients (1) PSC without IBD is uncommon; (2) asymptomatic IBD may be associated with PSC; (3) because the time to dysplasia may be accelerated, once the diagnosis of IBD is made in the setting of PSC, heightened endoscopic surveillance may be indicated; (4) pouchitis occurs frequently in these patients.

Objectives. To characterize the clinical features, results of diagnostic testing, and treatment outcomes for children and adolescents with rumination syndrome. Methods. Review of the medical records for all 147 patients ages 5 to 20 diagnosed with rumination syndrome at our institution between 1975 and 2000. Data are presented as mean ± the standard error of the mean. Results. Sixty-eight percent were female. Age at diagnosis was 15.0 ± 0.3 years. Symptom duration before diagnosis was 2.2 ± 0.3 years, 73% missed school/work, and 46% had been hospitalized because of symptoms. Before diagnosis, 16 (11%) underwent surgery for evaluation or management of symptoms. Twenty-four (16%) had psychiatric disorders; 3.4% had anorexia or bulimia nervosa. All patients described postprandial regurgitation after almost every meal (2.7 ± 0.1 meals per day). Weight loss was described by 42.2% (median: 7 kg). Additional symptoms included: abdominal pain, 38%; constipation, 21%; nausea, 17%; and diarrhea, 8%. Structural studies were normal. Gastric emptying of solids at 4 hours was delayed in 26 of 56 patients. Esophageal pH testing in 24 patients showed reflux/regurgitation in 54%. Gastroduodenal manometry in 65 patients showed characteristic rumination-waves in 40%. Outcome data (at median follow-up 10 months) were available for 54 patients. Symptoms resolved in 16 (30%) and improved in 30 (56%). Conclusions. Recognition of the clinical features of rumination syndrome in children and adolescents is essential; the diagnosis is often delayed and associated with morbidity. Extensive diagnostic testing is unnecessary. Early behavioral therapy is advocated, and patient outcomes are generally favorable.