Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome.

Primary intestinal lymphangiectasia (PIL) or Waldmann's disease is a rare protein-losing gastroenteropathy of unknown etiology. Less than 200 cases have been reported globally. Patients may be asymptomatic or present edema, lymphedema, diarrhea, ascites and other manifestations. We report two pediatric cases with PIL with extremely different outcome in a 3-year follow-up period. The first patient presented with persistent diarrhea, hypoalbuminemia and failure to thrive, while the second patient presented with an abrupt eyelid edema. Hypoproteinemia was the common laboratory finding for the two patients and upper gastrointestinal endoscopy established the diagnosis. The first patient relapsed five times during the follow-up period after the diagnosis had been made and required intravenous albumin administration and micronutrient supplementation. The second patient revealed normal gastrointestinal endoscopy 4 months after the diagnosis had been established; he followed an unrestricted diet and remained asymptomatic throughout the follow-up period. PIL can be either severe, affecting the entire small bowel, leading to lifetime disease, or sometimes affects part of the small bowel, leading to transient disorder.

Symptom-focused results after laparoscopic fundoplication for refractory gastroesophageal reflux disease--a prospective study.

BACKGROUND AND AIMS: Symptoms of gastroesophageal reflux disease (GERD) are common in the general population. Although the results of laparoscopic fundoplication are well documented, there have been no reports on the operative outcome in patients refractory to or with only partial response to medical therapy for GERD. PATIENTS-METHODS: Thirty-two patients with GERD, whose continuous high doses of medical treatment with proton-pump inhibitors produced no or only partial symptom relief, underwent laparoscopic Nissen fundoplication. Symptoms were evaluated with a standardized questionnaire preoperatively and 12 months after surgery. RESULTS: The complete follow-up evaluation was obtained in 30 out of the 32 patients. The main symptoms before surgery were regurgitation (93%), heartburn (60%), epigastric pain (47%), and globus sensation (47%). All patients were relieved from heartburn, vomiting, and globus sensation. Dysphagia was relieved in 75% of the patients and regurgitation in 86%. Dysphagia as a new symptom occurred in 9%. The overall morbidity rate was 16%. Patient satisfaction rate was 87%. CONCLUSION: Laparoscopic fundoplication seems to be an effective treatment for severe, drug-resistant GERD. The high patient satisfaction rate and the positive therapeutic response in 95% of patients justify this procedure in this strictly selected group of patients.

MR cholangiography in the evaluation of patients with duodenal periampullary diverticulum.

OBJECTIVE: This retrospective study evaluates the relationship between periampullary duodenal diverticula and the common bile duct (CBD). MATERIALS AND METHODS: The patients were examined with HASTE breath hold MR cholangiography (MRC) using a 1 T Siemens Expert Plus Scanner. Axial T2-weighted images were also obtained in all patients. RESULTS: From the 66 patients, only 23 clearly depicted to have deverticula, while 31 patients were suspected to have diverticula and for the rest 12 patients, the MRC results were negative. From these 66 patients, the 18 patients proved to have stones in the CBD. CONCLUSION: MRC has very low sensitivity in the detection of duodenum diverticula, but is the method of choice to evaluate their relationship with the distal part of the CBD.