Case report of primary intestinal lymphangiectasia diagnosed in an octogenarian by ileal intubation and by push enteroscopy after missed diagnosis by standard colonoscopy and EGD.

RATIONALE: Primary intestinal lymphangiectasia (PIL) is a rare, presumably congenital lesion that is usually diagnosed in patients < 3 years old, is rarely first diagnosed in adulthood, and when first diagnosed in adulthood typically presents with symptoms for many years. Although PIL is often identified by endoscopic abnormalities, it must be emphasized that the jejunoileum/distal duodenum must be intubated for diagnosis because the lesions are present in these regions. This work demonstrates that 1)-PIL can occur in an octogenarian; 2)-shows that the characteristic endoscopic findings are not found at colonoscopy without terminal ileal intubation; and 3)-may be missed at standard EGD without distal duodenal intubation. DIAGNOSES: A patient initially presented at age 83 with symptoms of watery diarrhea, abdominal distention, 5-Kg-weight-gain, and weakness for one month, and had typical clinical findings of PIL including chylous ascites, pleural effusions, bilateral pitting leg edema, hypoalbuminemia, borderline lymphopenia, hypovitaminosis-D, and hypocalcemia. Protein-losing-enteropathy was demonstrated by positive stool tests for alpha-1-antitrypsin. Standard colonoscopy revealed no significant lesions, but terminal ileal intubation during colonoscopy demonstrated creamy-white, punctate, mucosal lesions in terminal ileum, characteristic of lymphangiectasia. EGD with intubation to mid-descending duodenum revealed no significant lesions, but subsequent enteroscopy demonstrated lesions in distal duodenum/proximal jejunum similar to those in terminal ileum characteristic of lymphangiectasia. Histopathologic analysis of lesions of terminal ileum/distal duodenum demonstrated dilated mucosal vessels, confirmed as lymphatic vessels by immunohistochemistry. PIL was diagnosed after excluding secondary causes of intestinal lymphangiectasia. INTERVENTIONS/OUTCOMES: Patient placed on standard PIL diet: oral supplements of medium-chain triglycerides, a high protein diet, supplements of fat-soluble vitamins, and avoiding long-chain fatty acids, with marked clinical improvement. LESSONS: This work shows that: 1)-standard EGD and colonoscopy may miss characteristic lesions of PIL, 2)-enteroscopy or terminal ileal intubation at colonoscopy may be required for the diagnosis because lesions are typically located in distal duodenum/jejunoileum; and 3)-PIL can first present in the very elderly even with symptoms of short duration.

Clinical Utility of Capsule Endoscopy and Double-Balloon Enteroscopy in the Management of Obscure Gastrointestinal Bleeding.

BACKGROUND: Obscure gastrointestinal bleeding (OGIB) is a common but embarrassing problem for gastroenterologists. Most bleeding lesions associated with OGIB are present in the small intestine and sometimes cannot be identified due to the difficulty associated with physical accessibility. Capsule endoscopy (CE) and double-balloon enteroscopy (DBE) have enabled in the process of diagnosing and have evolved to become approaches to treating OGIB. SUMMARY: CE is a minimally invasive procedure and has a high diagnostic yield in patients with OGIB. DBE offers additional advantage of biopsy collection for pathological diagnosis and therapeutic intervention, but it should be noted that it sometimes causes severe adverse events such as acute pancreatitis, intestinal bleeding, and intestinal perforation. CE should be performed early in the workup course of OGIB. Positive CE findings enhance the diagnostic yield of subsequent DBE, and the effective therapeutic intervention improves the clinical outcomes of OGIB patients. On the contrary, there are no clear guidelines for further investigation of patients with negative CE findings at the present. Although patients in stable general condition may only require follow-up, repeated CE is useful to detect positive findings in patients with evidence of sustained bleeding and progressing anemia. We have revealed that repeated CE has higher positive finding rate than DBE in OGIB patients with negative CE findings in a preliminary study. Key Messages: CE and DBE have complementary roles in the management of OGIB, and the precise timing and proper sequence may be important for the approach to treating OGIB.

A 4-year-old girl with chronic nonspecific multiple ulcers of the small intestine diagnosed by double-balloon endoscopy.

We present the youngest patient reported to date with chronic nonspecific multiple ulcers of the small intestine (CNSU) diagnosed by double-balloon endoscopy (DBE). A 3-year-old girl was referred to our department with a 2-year history of iron-deficiency anemia. Failure to thrive and hypoproteinemia were also noted, and stool occult blood tests had been persistently positive. However, the C-reactive protein level and erythrocyte sedimentation rate were not elevated. Esophagogastroduodenoscopy and double-contrast enema revealed no abnormality in the colon and terminal ileum. Retrograde DBE was performed when the patient was 4 years old. Linear ulcerations arranged in an oblique or circular pattern were present at 3 sites between 55 and 65 cm from the ileocecal valve. Microscopic examination showed nonspecific inflammatory changes, and no granuloma was present. Based on the clinical and endoscopic findings, the patient was diagnosed as having CNSU. The youngest previously reported patient with CNSU was 7 years old, whereas our present patient was diagnosed at the age of 4 years. In pediatric cases of obscure gastrointestinal bleeding, it may be necessary to be aware of small bowel disease.

Comparison of double balloon enteroscopy in adults and children.

AIM: To compare results of double balloon enteroscopy (DBE) procedures in pediatric and adult patients. METHODS: The medical files of patients who underwent DBE at Gazi University School of Medicine, Ankara, Turkey between 2009 and 2011 were examined retrospectively. Adult and pediatric patients were compared according to DBE indications, procedure duration, final diagnosis, and complications. DBE procedures were performed in an operating room under general anesthesia by two endoscopists. An oral or anal approach was preferred according to estimated lesion sites. Overnight fasting of at least 6 h prior to the start of the procedure was adequate for preprocedural preparation of oral DBE procedures. Bowel cleansing was performed by oral administration of sennosides A and B solution, 2 mL/kg, and anal saline laxative enema. The patients were followed up for 2 h after the procedure in terms of possible complications. RESULTS: DBE was performed in 35 patients (5 pediatric and 30 adult). DBE procedures were performed for abdominal pain, chronic diarrhea, bleeding, chronic vomiting, anemia, and postoperative evaluation of anastomosis. Final diagnosis was diffuse gastric angiodysplasia (n = 1); diffuse jejunal angiodysplasia (n = 1); ulceration in the bulbus (n = 1); celiac disease (n = 1); low differentiated metastatic carcinoma (n = 1); Peutz-Jeghers syndrome (n = 1); adenomatous polyp (n = 1) and stricture formation in anastomosis line (n = 1). During postprocedural follow-up, abdominal pain and elevated amylase levels were noted in three patients and one patient developed abdominal perforation. CONCLUSION: With the help of technological improvements, we may use enteroscopy as a safe modality more frequently in younger and smaller children.

Small intestinal adenocarcinoma: rarely considered, often missed?

Adenocarcinoma of the small intestine is rare in comparison with other gastrointestinal malignancies but its incidence is rising. It often presents at an advanced stage due to the non-specific symptomatology. More recent advances in small intestinal visualisation including video capsule endoscopy and double balloon enteroscopy may facilitate diagnosis in patients with suspected small intestinal neoplasia. At present aggressive surgical resection provides the best chance of cure of small intestinal adenocarcinoma. Despite apparent curative resection the long-term outlook remains poor. The role of adjuvant chemotherapy is not well defined due to the rarity of the disease and lack of randomised controlled trials; however, there appears to be a survival benefit in advanced disease with the use of oxaliplatin and 5-fluorouracil. We reviewed the clinical aspects of this aggressive condition focusing on the pathological associations, available diagnostic modalities and current management options. Three cases are included to illustrate the review.