Second international guidelines for the diagnosis and management of hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications. The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The guidelines expert panel included expert physicians (clinical and genetic) in HHT from 15 countries, guidelines methodologists, health care workers, health care administrators, patient advocacy representatives, and persons with HHT. During the preconference process, the expert panel generated clinically relevant questions in 6 priority topic areas. A systematic literature search was done in June 2019, and articles meeting a priori criteria were included to generate evidence tables, which were used as the basis for recommendation development. The expert panel subsequently convened during a guidelines conference to conduct a structured consensus process, during which recommendations reaching at least 80% consensus were discussed and approved. The expert panel generated and approved 6 new recommendations for each of the following 6 priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver VMs, pediatric care, and pregnancy and delivery (36 total). The recommendations highlight new evidence in existing topics from the first International HHT Guidelines and provide guidance in 3 new areas: anemia, pediatrics, and pregnancy and delivery. These recommendations should facilitate implementation of key components of HHT care into clinical practice.

Left hepaticogastrostomy for biliary obstruction: long-term results.

PURPOSE: To evaluate the long-term results of peripheral biliary diversion by means of anastomoses of the left lobe of the liver to the stomach. MATERIALS AND METHODS: Transhepatic perforation of the left lobe of the liver into the lesser curvature of the stomach was performed in 35 patients with a presumed diagnosis of malignant obstructive jaundice. Jaundice was found to be caused by a malignant stricture in 32 patients and a benign stricture in three. Perforation was performed under fluoroscopic, endoscopic, and laparoscopic guidance in 33 patients and without laparoscopy in the other two. The hepaticogastric anastomosis was secured with a gastrostomy tube; patency of the tract was maintained with placement of a metallic stent. Kaplan-Meier analysis was used to evaluate survival, anastomosis patency rate, and jaundice recurrence. RESULTS: Technical success was achieved in all patients. Two (6%) patients had anastomotic obstruction. The actuarial survival rate was 91%, 80%, 59%, and 26% at 1, 3, 6, and 12 months. The mean patency was 234 days +/- 252. The jaundice-free rate among surviving patients was 100%, 96%, 93%, and 80% at 1, 3, 6, and 12 months. The reintervention rate was 14%. Late cholangitis occurred in seven (20%) patients. CONCLUSION: This peripheral diversion procedure appears to be safe and shows good long-term patency.

Percutaneous stent placement for treatment of stenosis of a portacaval H-graft shunt.

Surgical treatment of an occluded or stenotic portacaval shunt carries a high risk of mortality, but the rate of restenosis after transluminal angioplasty is also high. The authors report high-grade stricture of a portacaval H-graft shunt in a 51-year-old man, who presented with hematemesis and melena. The patient was treated with concomitant balloon angioplasty and placement of a metallic stent through a percutaneous venous approach. The procedure was tolerated well by the patient, and stenosis had not recurred at follow-up 1 year later.

Malignant biliary obstruction: preliminary results of palliative treatment with hepaticogastrostomy under fluoroscopic, endoscopic, and laparoscopic guidance.

PURPOSE: To report a technique of peripheral biliary decompression by means of anastomosis of a bile duct in segment II of the liver to the lesser curvature of the stomach. MATERIALS AND METHODS: Seven patients with unresectable biliary neoplasm were treated. After transhepatic catheterization of a segment II bile duct, the left lobe of the liver and the lesser curvature of the stomach were perforated under fluoroscopic and laparoscopic guidance. Anastomosis between the biliary tree and the stomach was maintained with a gastrostomy tube placed across the tract. After 2 weeks, the tube was removed and patency of the tract was preserved with a metallic stent. RESULTS: Three patients died, at 3, 6, and 9 months, respectively, without reocclusion; the other four were alive at 5 months without jaundice. One patient had an episode of cholangitis, which was resolved with antibiotic therapy. CONCLUSION: This method yields a good patency rate with few problems. Further investigation is required to evaluate long-term patency and the necessity of laparoscopic guidance.

Routine operative cholangiography during laparoscopic cholecystectomy: feasibility and value in 107 patients.

OBJECTIVE: Laparoscopic cholecystectomy is becoming a popular alternative to open cholecystectomy. However, the technical aspects of this new procedure increase the risk of injuring the bile ducts. The purpose of this study was to determine the feasibility and value of performing cholangiography during laparoscopic cholecystectomy. MATERIALS AND METHODS: We retrospectively reviewed the clinical and operative cholangiographic findings of the first 107 patients undergoing laparoscopic cholecystectomy at Hôtel-Dieu de Montréal between August 1990 and August 1991. RESULTS: Operative cholangiography was attempted in 98% of patients; the success rate was 71%. Eight anatomic anomalies of the biliary tract that were of surgical importance were found as well as 10 cases of stones in the common bile duct, eight of which were unsuspected. No biliary tract injuries occurred. With experience, surgical cannulation of the cystic duct for injection of contrast material can be done quickly without major difficulty, and cholangiograms of excellent diagnostic quality can be obtained. CONCLUSION: Our results show that operative cholangiography is feasible and useful in patients undergoing laparoscopic cholecystectomy.