Colonoscopy in childhood.

A review was made of 139 fiberoptic colonoscopies performed between 1975 and 1982 on 113 patients aged 1 month to 20 years. General anesthesia was used in four procedures. All others were done under sedation with meperidine (mean dose 2.9 mg/kg) and diazepam (mean dose 0.5 mg/kg). Indications were rectal bleeding in 52 patients; assessment and surveillance of known inflammatory bowel disease in 33 patients; and diagnostic evaluation of abdominal pain, diarrhea, and/or fever in 28 patients. The cecum was reached in 84% of diagnostic examinations. Comparison of findings on colonoscopy with barium enema in 75 patients showed agreement in 46, colonoscopic superiority in 25, and barium enema superiority in four. Bleeding sufficient to cause anemia was seen in 10/26 patients with polyps. Five minor complications and no major complications occurred. Flexible fiberoptic colonoscopy and polypectomy may be done usefully in childhood by physicians well versed and experienced with these procedures. Colonoscopy and biopsy changed the radiographic diagnosis from ulcerative colitis to Crohn's disease in several cases and indicated greater extent of colonic disease in several cases of ulcerative colitis and Crohn's disease. Colonoscopy is usually the most sensitive and accurate diagnostic tool for the evaluation of colonic disease, but barium enema and colonoscopy are complementary tests and barium enema should usually precede colonoscopy, with certain exceptions.

Primary hemochromatosis in children: report of three newly diagnosed cases and review of the pediatric literature.

Hereditary hemochromatosis was diagnosed in three asymptomatic siblings following the unexpected finding of elevated serum iron concentrations. This diagnosis was confirmed by hepatic biopsy. Repeated phlebotomies resulted in a significant decline of serum iron and ferritin concentrations and a decrease of hepatic iron content. This report and a review of the literature indicate that the diagnosis of hereditary hemochromatosis must be considered more frequently in childhood. Organ dysfunction from iron overload may be minimized in children by the early commencement of regular phlebotomy.

Recurrence of extrahepatic biliary atresia in two half sibs.

Extrahepatic biliary atresia (EHBA) usually is a sporadic disorder. Familial cases, including occurrence in sibs and twins, have been reported. We report the first recurrence of EHBA in two half sibs born to a common father and unrelated mothers and suggest the possibility of gonadal mosaicism for a new dominant mutation.

Anomalous drainage of the common bile duct into the fourth portion of the duodenum. Clinical sequelae.

We report two cases of early-childhood anomalous drainage of the common bile duct (CBD) into the fourth portion of the duodenum, which caused recurrent abdominal pain and hyperamylasemia. In both patients, this anomaly was associated with a long common channel between the CBD and the pancreatic duct. The CBD was obstructed by compression from the first portion of the duodenum, which passed anteriorly. Both children required choledochoenteric anastomosis to achieve permanent resolution of the abdominal pain. Elevation of the first portion of the duodenum does not produce permanent relief from symptoms. We believe that these patients represent the first described to have this unusual malformation.

Nonsurgical treatments for portal hypertension in children.

The nonsurgical management of portal hypertension in infants and children has undergone dramatic changes with the advent of endoscopic injection sclerotherapy and variceal ligation. Additional newer therapeutic interventions include radiographic transhepatic obliteration of vessels and angiographic portosystemic shunts. Adjunctive drug therapy is helpful in selected cases. Physicians managing infants and children with varices must understand the nature of portal hypertension in younger patients and the limitations of the therapeutic modalities.

Pediatric gastrointestinal mucosal biopsy. Special considerations in children.

In most disorders of the gastrointestinal mucosa that occur in children and adults, the mucosal manifestations are the same. This article focuses on disorders and approaches to gastrointestinal procedures and mucosal biopsy that are of a particularly or peculiarly pediatric nature. This article discusses, in detail, the issues pertaining to endoscopy and other techniques of mucosal biopsy in children. Some approaches and techniques are considerably different than those in adults.

Esophageal stenosis in children.

This article focuses on the special features of esophageal stenosis which pertain to children. In order to focus on stenoses intrinsic to the esophagus, esophageal stenosis due to extrinsic compression is excluded. While the causes of esophageal stenosis may be grouped as either congenital or acquired, congenital causes account for less than 5% of cases.

Pyloric balloon dilation for delayed gastric emptying in children.

Delayed gastric emptying may manifest with symptoms of epigastric pain, early satiety and delayed vomiting, and at times may be associated with failure to thrive. These symptoms and signs may improve following surgical pyloroplasty. To determine whether pyloric balloon dilation (PBD) is an effective therapy for children with these symptoms, hospital records of all children who underwent endoscopic PBD between October 1991 and March 1994 at British Columbia's Children's Hospital were reviewed. Excluded were children with chromosomal abnormalities, neurological disorders and erosive esophagitis. Through-the-scope balloons of diameter 15 or 18 mm were positioned in the pyloric channel and inflated with air to 2334 or 1815 mmHg respectively, for 2 min. Nineteen children with a mean age of 3.75 years (range eight months to 10 years) who presented with symptoms for more than three months (mean 11 months) were identified. Eleven children presented with failure to thrive, 14 with delayed vomiting and 10 with early satiety. Results of gastric emptying tests at 90 min ranged from 8% to 75% (mean 32%). The pylorus was difficult to intubate in 11 of 19 children, and in two the pylorus could not be passed before PBD. No complications were experienced with PBD. Thirteen children had complete resolution of symptoms, and five had transient improvement lasting four to eight weeks after PBD with subsequent complete resolution of symptoms following surgical pyloroplasty. One child continued to have mild symptoms after PBD but did not have further treatment. This study suggests that PBD is a safe and effective therapeutic option in children with symptoms and signs associated with delayed gastric emptying.

Canadian Helicobacter Study Group Consensus Conference on the Approach to Helicobacter pylori Infection in Children and Adolescents.

Gastric infection with Helicobacter pylori is common in both children and adults, but children are considerably less susceptible to peptic ulcers and other pathological sequelae. As a result, the risk to benefit ratio of diagnostic studies and therapeutic regimens for H pylori in adults are likely different from those in pediatric populations. These guidelines for the management of pediatric H pylori infection, developed by the Canadian Helicobacter Study Group, are designed to identify when the diagnosis and treatment of H pylori may improve patient care. Given the low prevalence of this infection in Canada, it is important to recognize that indiscriminate testing and treatment programs in children are not recommended, and indeed may threaten the optimal care of children. Diagnostic tests should be employed judiciously and be reserved for children who are most likely to derive measurable benefit, such as those likely to have peptic ulcer disease. At this time a test and treat strategy in children cannot be considered prudent, evidence based or cost effective. It is appropriate to limit diagnosis and treatment to children and adolescents in whom H pylori has been identified during endoscopic investigation.

Long-term maintenance treatment with omeprazole in children with healed erosive oesophagitis: a prospective study.

BACKGROUND: Short-term studies show that PPIs heal erosive esophagitis in children. There are no prospective studies that examine long-term maintenance therapy of erosive esophagitis in children with and without underlying GERD-predisposing disorders. AIM: To determine prospectively the dose of omeprazole needed to maintain remission of erosive oesophagitis and reflux symptoms in children. METHODS: Patients aged 1-16 years with healed erosive reflux oesophagitis after omeprazole treatment (0.7-3.5 mg/kg/day) entered a 21-month maintenance phase where they initially received half the dose of omeprazole required to heal. Endoscopy was performed after 3, 12 and 21 months. The omeprazole dose was increased if erosive oesophagitis or reflux symptoms recurred. RESULTS: A total of 46 patients entered the study and 32 completed it. Of these, 17 (53%) remained on the maintenance dose, 12 (38%) returned to their healing dose and 3 (9%) ended the study on a dose higher than their healing dose. Three-quarters of the completers (24/32) had no erosive oesophagitis relapse. Four patients (13%) had relapse of only erosive oesophagitis, 4 (13%) had relapse of erosive oesophagitis and symptoms, and 10 (31%) had only symptomatic relapse. Of the 46 patients, 48% had GERD-predisposing disorders (neurological impairment or oesophageal atresia). Overall, 62.5% (5/8) of patients who had an erosive oesophagitis relapse had a GERD-predisposing disorder versus 33.3% (8/24) of those who did not. CONCLUSIONS: Remission of erosive oesophagitis is maintained with omeprazole treatment for at least 21 months in most children aged 1-16 years, and the drug is well tolerated. To maintain remission, some 60% of patients require more than half the dose required for healing. In children with GERD-predisposing conditions, GERD is often chronic and relapsing, and requires long-term management.

Gastric histology in children treated with proton pump inhibitors long term, with emphasis on enterochromaffin cell-like hyperplasia.

BACKGROUND: There are longstanding concerns that carcinoid tumours or atrophic gastritis might develop in children receiving proton pump inhibitors (PPIs) long term. In children, this has not been studied using stains sensitive and specific for enterochromaffin-like (ECL) cells. AIM: To evaluate gastric biopsies for ECL hyperplasia or gastric atrophy, in children treated long-term with PPIs. METHODS: Synaptophysin and chromogranin immunostaining, biopsies read anonymised, blinded. Endocrine cell numbers graded according to Rindi and Solcia. RESULTS: Of 130 children with gastro-oesophageal reflux disease (GERD), 65 had sequential gastric biopsies, starting at median 8.2 years (<1 to 17). Of the 65, 83% had GERD-predisposing conditions, mostly neurological impairment or repaired oesophageal atresia. Four hundred and fifty-eight tissue blocks (208 antrum, 250 body) were available from a mean of 5.8 endoscopies (2-14). Of 82 gastric body biopsies in 40 patients with ECL hyperplasia, 67 had grade 1 hyperplasia, 15 grade 2. Of the 40, nine had ECL hyperplasia before PPI use; all nine had received H2-receptor antagonists. Median duration of PPI use was 3.17 years in patients with ECL hyperplasia, 2.20 years in those without (P=0.16). Helicobacter pylori was present in four patients; two had ECL hyperplasia. PPI duration was >3 years in 24 patients. In nine patients who received H2-receptor antagonists, changes were present before PPI use. No patient had atrophic gastritis. CONCLUSIONS: A high percentage of children (61%) receiving long-term PPI continuously for up to 10.8 years (median 2.84 years) develop minor degrees of ECL hyperplasia. This has no known clinical significance. Children on PPIs for this duration do not appear to develop atrophic gastritis or carcinoid tumours.

Outcomes of fundoplication: causes for concern, newer options.

Antireflux surgery has been a mainstay of treatment for gastro-oesophageal reflux disease in children for some 40 years. In recent years, enthusiasm for antireflux surgery seems only to have increased, despite its often poor outcome, and the availability of highly effective medical therapy in the form of proton pump inhibitors (PPIs). Reports show that many children undergo surgery without reflux disease as the demonstrable cause of their symptoms/signs, and without evidence of having failed optimised medical management. Very few studies report objective testing postoperatively--those that do show high rates of failure within the first 1-3 years following surgery. Treatment with PPIs is an effective and safe alternative to surgery in many cases.

Barrett's esophagus: new definitions and approaches in children.

BE is a disorder that occurs in children likely as a consequence of prolonged GE reflux of gastroduodenal contents. It usually presents with complications of GE reflux, but it also may be relatively silent in childhood and then present with adenocarcinoma in childhood or present in adulthood. Although seldom recognized in children until relatively recently, it is being diagnosed with greater frequency but not always accurately. The diagnosis of BE can be made with certainty only if landmarks are carefully documented, and a detailed histologic map is made from multiple, large biopsies taken under direct vision at endoscopy; the diagnosis should be reserved for those patients where Barrett's specialized epithelium i.e., goblet cell metaplasia, is present. BE is a diagnosis that should be made with thorough documentation because of the implications for regression, cancer, and the need for follow-up and endoscopic biopsy surveillance. For treatment of complications and because there may be a chance for regression of a young (i.e., childhood) lesion, antireflux surgery or indefinite aggressive acid suppressing medical therapy is required. Because bile reflux may have a pathogenic role in BE, and because of the proven benefits of surgery in producing partial regression and prevention of dysphagia and cancer in some patients, the author's preference at present is for surgery. For children who are poor candidates for surgery, long-term omeprazole should be used (159). Adenocarcinoma does occur in childhood as a complication of BE. Because it can be recognized early, regular surveillance of children with bona fide BE is advisable.

Columnar-lined esophagus in children.

Children with columnar-lined esophagus (CLE) become adults with CLE. Therefore, an understanding of the disorder as it occurs in children may be important for physicians caring for adults with CLE and for pediatric gastroenterologists and surgeons. Although there are many features of CLE common to children and adults, this article focuses on those special features of CLE in children and the issues that pertain particularly to children.

Wrap session: is the Nissen slipping? Can medical treatment replace surgery for severe gastroesophageal reflux disease in children?

For over 20 yr, antireflux surgery has been the treatment of choice for severe gastroesophageal (GE) reflux disease in children, and antireflux operations are said to be the commonest major surgical procedures performed by pediatric surgeons in North America. Yet, only recently have the results of surgery been more closely examined; both the surgical morbidity and operative failure rates have been found to be particularly high in children with neurological impairment, repaired esophageal atresia, and chronic lung disease. Of interest, these groups of children are among those most at risk for developing severe GE reflux disease in the first place. Close examination of surgical reports also raises some questions about the indications for surgery in some children, specifically whether the presence of severe GE reflux disease had been established before surgery and whether a trial of appropriate medical management had been given. Failure of medical management has always been an accepted indication for surgery. However, in the past the medical management that was available for children was ineffective because drug dosages were not optimized (H2-receptor antagonists), the drugs had side effects precluding their use long term or in high doses (bethanechol, metoclopramide), or they were simply insufficiently potent to treat severe GE reflux disease (all the above drugs plus cisapride). Thus, in the past, failure of medical management did not mean failure of very much. In contrast, the proton pump inhibitor omeprazole has recently been shown to be effective and safe for the treatment of severe childhood GE reflux disease refractory to other medical treatments and where antireflux surgery has failed. The issues of why certain groups of children are at highest risk for severe GE reflux disease are discussed as are the outcomes and roles of surgical and medical treatment for all groups of children with severe GE reflux disease. The options of antireflux surgery or omeprazole should be reserved for those children with severe GE reflux disease, e.g., GE reflux accompanied by a complication.

Barrett's esophagus: congenital or acquired?

Barrett's esophagus (BE) is a premalignant condition in which metaplastic specialized columnar epithelium with goblet cells is present in the tubular esophagus. BE is much more prevalent in adults than in children, but largely because of its occurrence in children, a congenital etiology for BE has been proposed by some. However, there is extensive, compelling evidence to indicate that Barrett's specialized metaplasia is an acquired disorder in children and adults, resulting from both a severe mucosal injury and an abnormal intraesophageal milieu during mucosal repair. Acid reflux has been emphasized as being the usual inciting and ongoing injurious factor, but more recently the additional importance of refluxed duodenal contents has been recognized. Despite recent advances in our understanding, it remains unclear why pathologic gastroesophageal reflux results in squamous esophagitis in some persons and Barrett's specialized metaplasia in others. Although the evidence cited for a purely congenital cause of BE can be readily refuted, a congenital component in combination with severe mucosal injury cannot be ruled out.

Familial inflammatory bowel disease in a paediatric population.

Familial aspects of inflammatory bowel disease (IBD) were assessed as part of an age and sex matched case control study of 91 children with IBD and 131 controls. The prevalence of IBD in family members of the children was studied, the affected side (mother's side or father's side) of the family was documented and the type of inflammatory bowel disease was traced among relatives. Data were collected from children in out-patient clinics at a large urban tertiary-care facility. All family data were verified with the affected relatives and/or their physicians. The children with IBD (the cases) had significantly (P = 0.0000385) more IBD in their families than the controls. Among all children with IBD in their family, IBD was found significantly (P = 0.0073) more often on the mother's side of the family than on the father's side. Patterns of disease varied within families. A mixture of ulcerative colitis and Crohn's disease was found within families. The implications of the findings are discussed. Directions for prevention, screening, early intervention and further study are given.