The role of upper gastrointestinal endoscopy in the diagnosis of pediatric inflammatory bowel disease.

OBJECTIVES: Upper gastrointestinal (UGI) tract involvement is frequently reported in pediatric Crohn disease (CD) and ulcerative colitis (UC). Aside from granulomas, most findings are nonspecific. The aims of this study were to review the prevalence of UGI tract findings in pediatric patients with CD or UC at diagnosis and to describe differences in endoscopic and histologic features. METHODS: Patients with CD and UC aged 2 to 17 years diagnosed between 2000 and 2015 who had upper and lower endoscopy at diagnosis were randomly chosen from the BC Children's Hospital inflammatory bowel disease (IBD) registry. Pathology review of the UGI biopsy specimens was blinded to IBD diagnosis. RESULTS: Of the 198 patients, 102 with CD and 96 with UC were included, with a mean age of 11.7 years (range, 2.3-17 years). Patients with CD were more likely to have aphthous ulcers (20.4% vs 3.5%, P = .002) and erosions (16.3% vs 3.5%, P =.018), most commonly affecting the antrum. Macroscopically normal UGI endoscopy was present in 60% of patients. Microscopic disease was reported in 100% of patients with CD and 87% of patients with UC. In both groups, nonspecific inflammation was the most common finding. Chronic deep, superficial, and diffuse inflammation were more frequent among patients with CD than UC (42% vs 4%, P < .001; 60% vs 17%, P < .001; 50% vs 34%, P = .04, respectively). CONCLUSIONS: The UGI tract macroscopic changes were common in pediatric IBD, especially in CD. Despite macroscopically normal endoscopy, histologic abnormalities were frequent. Although chronic inflammation was more often reported in patients with CD, aside from granulomas there were no unique histologic abnormalities unique to CD.

Abdominal wall desmoid tumors associated with pregnancy: current concepts.

The desmoid tumors (DTs) are unusual soft-tissue tumors that have a propensity for aggressive local growth and may develop during, or soon after pregnancy. Pregnancy-associated DTs are uncommon and optimal management of this tumor has yet to be defined. Currently, controversy centers on the timing of surgical resection and is influenced by the potential for tumor growth and the effects of a gravid uterus. A review of current literature in which DTs were managed either during pregnancy or in the postpartum period, was carried out. Surgical resection of these tumors has been performed successfully both during and soon after delivery, and the role of postpartum radiotherapy, chemotherapy and other medical intervention remains controversial. Management of DTs diagnosed during pregnancy is complex and treatment must be individualized.

Gastrointestinal manifestations of systemic mastocytosis.

Systemic mastocytosis (SM) is a rare disease with abnormal proliferation and infiltration of mast cells in the skin, bone marrow, and viscera including the mucosal surfaces of the digestive tract. Gastrointestinal (GI) symptoms occur in 14%-85% of patients with systemic mastocytosis. The GI symptoms may be as frequent as the better known pruritus, urticaria pigmentosa, and flushing. In fact most recent studies show that the GI symptoms are especially important clinically due to the severity and chronicity of the effects that they produce. GI symptoms may include abdominal pain, diarrhea, nausea, vomiting, and bloating. A case of predominantly GI systemic mastocytosis with unique endoscopic images and pathologic confirmation is herein presented, as well as a current review of the GI manifestations of this disease including endoscopic appearances. Issues such as treatment and prognosis will not be discussed for the purposes of this paper.

Incidental littoral cell angioma of the spleen.

BACKGROUND: Littoral cell angioma (LCA) is a recently described primary vascular neoplasm of the spleen that may be associated with other malignancies and may itself also have malignant potential. CASE PRESENTATION: We present a case of LCA that was discovered incidentally in a 52-year-old woman who presented with biliary colic at the time of consultation for cholecystectomy. This vascular neoplasm was evaluated by ultrasound, CT, MRI, Tc-99m labelled red blood cell scintigraphy, and core biopsy. A splenectomy revealed LCA by pathological evaluation. Post-operative outcome was favourable with no evidence of complication or recurrent disease. Following this case presentation, clinical, radiographic, and pathological features of LCA will be reviewed as well as recent advances in our understanding of this uncommon splenic lesion. CONCLUSION: LCA is a rare, generally benign, primary vascular tumour of the spleen that typically is discovered incidentally. Individuals diagnosed with this tumour must be carefully evaluated to exclude primary, secondary, and synchronous malignancies.