International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia.

BACKGROUND: HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults. OBJECTIVE: The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease. METHODS: The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches. RESULTS: The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.

Endoluminal graft repair for abdominal aortic aneurysms in high-risk patients and octogenarians: is it better than open repair?

OBJECTIVE: To analyze the short-term and midterm results of open and endoluminal repair of abdominal aortic aneurysms (AAA) in a large single-center series and specifically in octogenarians. METHODS: Between January 1997 and October 2000, 470 consecutive patients underwent elective repair of AAA. Conventional open repair (COR) was performed in 210 patients and endoluminal graft (ELG) repair in 260 patients. Ninety of the patients were 80 years of age or older; of these, 38 underwent COR and 52 ELG repair. RESULTS: Patient characteristics and risk factors were similar for both the entire series and the subgroup of patients 80 years or older. The overall complication rate was reduced by 70% or more in the ELG versus the COR groups. The postoperative death rate was similar for the COR and ELG groups in the entire series and lower (but not significantly) in the ELG 80 years or older subgroup versus the COR group. The 36-month rates of freedom from endoleaks, surgical conversion, and secondary intervention were 81%, 98.2%, and 88%, respectively. CONCLUSION: The short-term and midterm results of AAA repair by COR or ELG are similar. The death rate associated with this new technique is low and comparable, whereas the complication rate associated with COR in all patients and those 80 years or older in particular is greater and more serious than ELG repair. Long-term results will establish the role of ELG repair of AAA, especially in elderly and high-risk patients.

Results of a new strategy for reconstruction of biliary injuries having an isolated right-sided component.

Poor results after repair of biliary injuries are most common when injuries are above the bifurcation of the left and right hepatic ducts or involve aberrant ducts. We have developed a novel approach to the right-sided component of such injuries. Preoperatively all isolated sections of the biliary tree are intubated percutaneously. At surgery the left duct is found by the Hepp-Couinaud approach. Dissection is continued to the right, staying within the coronal plane of the left hepatic duct, and continuing across the gallbladder plate into segment 5 between the hepatic parenchyma and the Wallerian sheath of the right portal pedicle. Hepatic parenchyma, anterior to the sheath, is resected. After a length of portal pedicle is exposed, right-sided bile ducts are opened on their anterior surface, using the percutaneous transhepatic stents as a guide, and hepaticojejunostomy is performed. Twenty-three patients were treated from May 1993 to February 1999. Injury types and (number of patients) were as follows: B (n = 2), C (n = 5), E4 (n = 10), and E5 (n = 6). There were no perioperative deaths. Follow-up ranged from 8 months to 7 years (median 3 years). There have been no cases of restricture, reoperation, or jaundice, and no interventional procedures. Serum bilirubin is normal in all patients. Alkaline phosphatase is normal or less than two times the normal value in 21 of 22 living patients. This novel approach brings the benefits of the Hepp-Couinaud approach to the right hepatic ducts. Very satisfactory results were obtained in the most severe types of biliary injury.

Recurrent gastrointestinal bleeding: use of thrombolysis with anticoagulation in diagnosis.

PURPOSE: To determine the safety and diagnostic accuracy of a provocative protocol with heparin and urokinase to induce bleeding and determine the source in patients with chronic gastrointestinal hemorrhage. MATERIALS AND METHODS: Nine patients had gastrointestinal bleeding from an indeterminate source and had negative results from esophagogastroduodenoscopy, colonoscopy, small-bowel examination, and angiography. Ten provocative bleeding studies were performed prospectively. Patients had no clinical evidence of bleeding within 2 days before the study. Intravenous administration of heparin and urokinase was performed systemically during a 4-hour period while scintigraphy was performed continuously. Mesenteric angiography was performed immediately in patients in whom substantial gastrointestinal bleeding was detected at scintigraphy. RESULTS: The provocative protocol was successful in inducing scintigraphically detectable hemorrhage in four (40%) studies within 4 hours. In two of these four studies, the source of hemorrhage was determined and treated with embolization or surgery. Three (30%) studies demonstrated scintigraphic evidence of hemorrhage only at delayed imaging (8-24 hours after initiation of the study). The remaining three (30%) studies did not show active bleeding. No complications occurred, including hemodynamic instability or uncontrollable decreases in hematocrit. CONCLUSION: Since this protocol with heparin and urokinase enabled determination of the bleeding source in only two of 10 studies, protocol modifications are necessary before this intervention is used widely.

Endovascular stent grafts for aneurysmal and occlusive vascular disease.

BACKGROUND: This report details our initial experience with two types of endovascular grafts- one for the treatment of infrarenal abdominal aortic aneurysms and the other for the treatment of iliac artery occlusive disease. METHODS: An abdominal aortic aneurysm was repaired in 34 patients using 3 different types of Ancure endografts (Menlo Park, California). Control patients (n = 9) had a standard aneurysm repair. Patients with chronic lower extremity ischemia (n = 7) secondary to iliac artery occlusive disease were treated with a Hemobahn endograft (W. L. Gore & Associates, Flagstaff, Arizona). RESULTS: Ancure graft deployment was achieved in 33 of 34 (97.1%) patients. Perioperative mortality for the Ancure and control group patients was 2.9% and 0%, respectively. Periprosthetic leaks were identified within 48 hours of deployment in 6 (18.2%) Ancure graft patients. All but 2 of the leaks resolved on serial follow-up. Additional endovascular procedures were required in 11 (32.4%) Ancure graft patients at the initial procedure or during follow-up to correct graft or arterial stenoses. Patients treated with an endovascular graft had significantly less blood loss and shorter hospital stays than control group patients. For Hemobahn patients, the technical success for graft deployment was 100%. There were no perioperative deaths. The ankle/brachial index increased from a mean of 0.52 preoperatively to 0.86 postoperatively (P = 0.004). One patient required a Wallstent in follow-up to correct a graft wrinkle. Angiography at 6 months demonstrated mild intimal hyperplasia in the stent graft in 5 of 6 patients. CONCLUSIONS: These early results support the potential for endovascular grafts in the treatment of aneurysmal and occlusive vascular disease. Further modifications in the devices and deployment techniques are necessary to reduce the incidence of periprosthetic leaks, graft limb stenoses, and intimal hyperplasia.

Long-term outcome of flexible ureterorenoscopy in the diagnosis and treatment of lateralizing essential hematuria.

PURPOSE: We identified the long-term outcome of ureterorenoscopic diagnosis and treatment of patients with lateralizing essential hematuria. MATERIALS AND METHODS: We reviewed retrospectively 17 patients with lateralizing essential hematuria treated with flexible ureterorenoscopy (15) or flexible percutaneous nephroscopy (2) with or without biopsy and electrocoagulation. Followup was longer than 24 months (average 60, range 24 to 116) in all patients. RESULTS: At followup 9 of the 17 patients (59%) were cured and 7 (41%) had recurrent bleeding. Discrete lesions occurred in 11 patients (64%), who were treated with electrocoagulation with 9 (82%) cured. All 3 patients (18%) with diffuse lesions had recurrent bleeding as did 2 of 3 (66%, 18% overall) with negative examinations. CONCLUSIONS: Ureterorenoscopy is an effective means of diagnosis and treatment of lateralizing essential hematuria. The majority of patients with lateralizing hematuria will have a discrete lesion that will respond to ureterorenoscopic electrocoagulation in the long-term. However, if the lesions are diffuse or the diagnostic examination is unreliable recurrent bleeding is likely.

Prospective study of provocative angiograms to localize functional islet cell tumors of the pancreas.

BACKGROUND: Controversy exists concerning the use of preoperative imaging studies in patients with islet cell tumors. Since 1993 we have evaluated the use of provocative angiography in patients with insulinoma or Zollinger-Ellison syndrome (ZES). METHODS: Twelve patients with a working diagnosis of insulinoma (n = 4) or ZES (n = 8) were studied. Of the eight patients with ZES, four were known to have multiple endocrine neoplasia type 1. All patients underwent conventional imaging studies followed by provocative angiography. Angiograms were graded based on the ability to detect tumor and regionalize it within the pancreas. RESULTS: Of the three patients with a working diagnosis of ZES but equivocal results of biochemical studies, none had arteriographic imaging of an islet cell tumor or a positive provocative study result (true negative result). Of the nine patients with documented islet cell tumor, seven (78%) underwent arteriographic imaging of the tumor and eight (89%) had correct regional localization by provocative angiography. Sensitivity and specificity for imaging were 78% and 100%, respectively, and for regional localization 89% and 100%, respectively. CONCLUSIONS: Provocative angiography is the localization study of choice for both gastrinoma and insulinoma. Having few false-negative results, it can be used to corroborate the diagnosis and, having few false-positive results, it detects tumor and biochemically confirms localization in nearly every patient.

Nonsurgical management of gastric or duodenal perforation from a Wills-Oglesby-type gastrostomy tube.

PURPOSE: To describe the clinical and radiologic appearance of gastrointestinal perforation related to a Wills-Oglesby-type gastrostomy tube, as well as techniques for nonsurgical management. MATERIALS AND METHODS: Five patients with a previously placed 14-F modified Wills-Oglesby-type gastrostomy catheter experienced viscus perforation by the distal limb of the catheter during a 30-month period. RESULTS: The average interval between tube placement and perforation event was 4.3 months. Three patients had migration of the gastrostomy tube into the duodenum and subsequent duodenal perforation. One patient had posterior perforation of the stomach, and one patient developed a gastrocolic fistula. Generalized peritonitis was not present in any patient. All patients were treated successfully without surgery, and tube feedings were re-established in 4-14 days. CONCLUSIONS: Gastrostomy tube-related perforation is an uncommon, delayed complication of percutaneous gastrostomy with the modified Wills-Oglesby-type catheter. Nonsurgical management is feasible in select instances. Because of these gastrointestinal perforations, the gastrostomy tube has been modified (eliminating the distal tip), and no gastrostomy-associated gastrointestinal perforation has been experienced since.

Percutaneous gallstone removal: long-term follow-up.

PURPOSE: To evaluate gallstone and symptom recurrence rates, long-term complications, and life expectancy after percutaneous gallstone removal. PATIENTS AND METHODS: Medical records of 87 patients (mean age, 69 years +/- 14 [standard deviation]) undergoing percutaneous gallstone removal between 1987 and 1992 were reviewed. Physicians and patients (or their families) were contacted for clinical follow-up. Thirty-one patients returned for follow-up ultrasound (US). RESULTS: The final study group consisted of 65 patients. Mean survival from the time of initial gallbladder drainage was 33 months +/- 19. Over a mean clinical follow-up period of 33 months, eight of 65 patients (12%) developed recurrent symptoms; six of these eight had recurrent gallstones shown at US. Of 30 patients with technically adequate US images (mean follow-up, 14 months +/- 12), 12 (40%) had recurrent gallstones. Six of these 12 patients had recurrent symptoms. No long-term complications were identified. CONCLUSION: The risk of gallstone recurrence after percutaneous removal is notable, but the symptom recurrence rate is much lower. Percutaneous gallstone removal is beneficial for patients at prohibitive surgical or general anesthetic risk.

Anaerobic culture yield in interventional radiologic drainage procedures.

PURPOSE: This study was designed to determine the yield of anaerobic cultures from percutaneous radiologic drainage procedures. PATIENTS AND METHODS: Anaerobic culture results in 317 patients from June 1992 to May 1994 were retrospectively examined. Anaerobic specimens were placed in specially designed anaerobic culture tubes and not blood culture media. Patients had undergone the following procedures: percutaneous nephrostomy (105 patients), biliary drainage (65 patients), and abdominal abscess drainage (147 patients). Aerobic culture results were tabulated in those patients with positive anaerobic cultures. RESULTS: Overall, 10% of patients (n = 32) had positive anaerobic cultures (Bacteroides species, n = 25; Clostridium, n = 6; other organisms, n = 4). Anaerobes were isolated in 13% (n = 19) of abdominal abscess drainages, 8% (n = 8) of nephrostomy drainages, and 8% (n = 5) of biliary drainages. Aerobic isolates were present in 78% (n = 25) of patients with anaerobic infection. CONCLUSION: The yield for anaerobic cultures varies for different types of percutaneous drainage procedures from 8% to 13%. When isolated, anaerobic bacteria are frequently mixed with aerobic bacteria. Anaerobic culture usage is recommended with abdominal abscess and biliary drainages. Anaerobic bacterial cultures are not recommended for percutaneous nephrostomy unless the patient has a urinary tract malignancy or has undergone urinary instrumentation.

Accuracy of DSA in the evaluation of patency of infrapopliteal vessels.

PURPOSE: To evaluate the accuracy of intraarterial digital subtraction angiography (DSA) in the demonstration of patent infrapopliteal vessels. PATIENTS AND METHODS: One-hundred sixty-five arteriograms were obtained in 153 consecutive patients prospectively enrolled to evaluate lower extremity ischemia. In 86 cases a follow-up angiogram of the infrapopliteal vessels was obtained during surgery or after endovascular intervention (n = 57). Twenty-nine arteriograms were followed by surgical exploration of the infrapopliteal vessels. Standard angiographic technique was performed with intraarterial DSA of the most symptomatic foot. Visualization of distal vessels was compared with intraoperative or postintervention imaging or with the results of surgical exploration. RESULTS: Of the 57 procedures after which either intraoperative or post-endovascular intervention angiography was performed, DSA results were equivalent in 47 (82%) and worse in five (9%). When individual vessels were evaluated, the sensitivity of DSA in the identification of patent named vessels was 95%, and the specificity was 92%. Among 29 cases with a surgical standard of reference, 28 patients underwent bypass to a vessel correctly identified as patent at DSA; one patient was incorrectly identified as having no patent named vessels. CONCLUSION: Intraarterial DSA is accurate and reliable in the assessment of patency in infrapopliteal vessels before surgery or endovascular intervention in patients with infrainguinal atherosclerotic disease.

Percutaneous biliary endoscopy.

The development of small-diameter flexible endoscopes has expanded the role of biliary endoscopy to include percutaneous interventions. Percutaneous biliary endoscopy is a technique that is easily learned. The equipment for percutaneous biliary endoscopy is readily available since most hospitals have an appropriate-sized choledochoscope and light source for intraoperative use. Therefore, the initial capital costs associated with flexible biliary endoscopy are minimal. Percutaneous biliary endoscopy in the interventional radiology suite is an ideal arrangement to facilitate a wide variety of biliary diagnostic and interventional procedures.

Subcutaneous urinary diversion utilizing a nephrovesical stent: a superior alternative to long-term external drainage?

OBJECTIVES: The use of external percutaneous nephrostomy drainage in patients with end-stage ureteral obstruction in whom ureteral stenting has failed presents significant compromises in the patient's quality of life. Toward this end, we present the initial experience in the United States with an intracorporeal nephrovesical stent. METHODS: We performed successful subcutaneous urinary diversion in 2 patients with malignant, metastatic periureteral obstruction. Both patients had previously been managed with a chronic percutaneous nephrostomy that was both painful and inconvenient. The nephrovesical stent was inserted utilizing percutaneous access to both the kidney and bladder followed by creation of a subcutaneous tunnel between the two sites. RESULTS: The nephrovesical stents are patent at 6 and 9 weeks postoperatively and both patients have had their nephrostomy tubes removed. Both patients have noted a marked improvement in their overall comfort and quality of life since the stent has been in place. CONCLUSIONS: Subcutaneous urinary diversion with a nephrovesical stent provides effective urinary drainage and may improve the quality of life of patients with malignant metastatic ureteral obstruction. Further long-term studies are needed.

Bloodstream infections after interventional procedures in the biliary tract.

PURPOSE: To assess the adequacy of prophylaxis for interventional radiologic biliary procedures and the etiologic organisms of subsequent bloodstream infections. MATERIALS AND METHODS: Data from 148 patients who underwent 480 interventional radiologic biliary procedures were evaluated for evidence of bloodstream infection. Data analyzed included type of procedure performed, whether an antibiotic was used, and evidence of infectious complications occurring during and within 72 hours after the procedure. All culture data obtained before and after the procedure were recorded. RESULTS: Seven cases of new bloodstream infection were identified, five of which were caused by Enterococcus species. No substantial risk factors for bloodstream infection were identified, although it occurred only in patients who had recently undergone biliary surgery or underwent manipulations other than simple cholangiography. Microbial colonization of the bile was associated with older age. Evidence of possible or proved infection after the first interventional procedure was more common in patients with positive bile cultures. CONCLUSION: Although the importance of enterococcal bacteremia is uncertain, current recommendations for cephalosporin prophylaxis for interventional radiologic biliary procedures should be reevaluated.

Angiographic evaluation and therapy of ureteroarterial fistulas.

OBJECTIVE: Fistulas between the iliac artery and the ureter are extremely uncommon, life-threatening conditions usually seen in patients who have had pelvic irradiation or have indwelling ureteral stents. We describe our experience in the angiographic evaluation and therapy of these fistulas. MATERIALS AND METHODS: We retrospectively reviewed medical records for diagnoses of ureteroarterial fistulas. Patients' records were evaluated for potentially associated etiologic factors, clinical features and course, radiographic evaluation and findings, and therapy. RESULTS: Our review showed that four patients treated at our institution (all since 1990) had ureteroarterial fistulas. All four patients had indwelling ureteral stents and had had irradiation for pelvic cancer. Three had spontaneous brisk hemorrhage in the urinary tract. The fourth had hemorrhage after balloon dilatation of a ureteral stricture. Initial diagnosis was based on findings on iliac arteriography in three patients and on findings on retrograde ureterography in one. Angiographic techniques required to visualize the fistulas included selective arterial catheterization, use of multiple projections, and provocative maneuvers. Treatment of the ureteroarterial fistulas involved surgery in one case, isolated embolotherapy in one case, and a combination of embolotherapy and surgery in two cases. CONCLUSION: Specific angiographic maneuvers are often required to identify ureteroarterial fistulas. Transcatheter embolotherapy (with or without surgical bypass) is an effective form of treatment for this rare abnormality.

Diagnosis and management of biliary complications of laparoscopic cholecystectomy.

Laparoscopic cholecystectomy has become the operation of choice for symptomatic cholelithiasis. However, this operation may result in serious biliary complications. Our aims were to review our experience with biliary complications of laparoscopic cholecystectomy and to document the mechanisms of the injuries and the techniques of managing these complications. We treated 20 patients with biliary complications of laparoscopic cholecystectomy. Symptomatic collections of bile (bilomas) were present in five patients. One of these patients underwent operative ligation of an accessory bile duct in the gallbladder bed, whereas the others had percutaneous or endoscopic therapy. In the remaining 15 patients (of whom 13 were referred from other hospitals), injuries to the major bile ducts were managed by combined radiologic, endoscopic, and operative therapies. In 10 of these patients (67%), the mechanism of injury was the misidentification of the common bile duct as the cystic duct. In 3 of 15 patients, a noncircumferential injury to the lateral aspect of the common bile duct occurred. The Bismuth levels of the remaining bile duct injuries were type I in 3, type II in 4, type III in 3, and type IV in 2. Early outcome of therapy for these bile duct injuries has been favorable. One patient was lost to follow-up, and 2 died of nonbiliary causes, whereas 12 patients are alive and well with normal serum liver enzyme levels at 4 to 19 months postoperatively (mean: 14 months). The most common cause of major bile duct injury during laparoscopic cholecystectomy is mistaking the common bile duct for the cystic duct. Most bilomas can be managed successfully with noninvasive methods. Coordinated efforts by radiologists, endoscopists, and surgeons are necessary to optimize the management of patients with major bile duct injury, suggesting that patients with biliary complications of laparoscopic cholecystectomy should be referred to specialty centers for optimal care.

Intrahepatic portosystemic vascular stents: a bridge to hepatic transplantation.

Refractory esophageal variceal hemorrhage (EVH) remains a formidable problem in patients awaiting liver transplantations. Transjugular intrahepatic portosystemic shunts (TIPS) have provided an alternative approach for managing EVH that may obviate the need for portosystemic shunt surgery. Experience with TIPS placement and subsequent successful hepatic transplantation in patients without previous portosystemic shunt surgery has not been previously reported. Two patients are reported who underwent TIPS placement and subsequent successful hepatic transplantation without previous portosystemic shunt surgery. This experience indicates that (1) TIPS can provide effective control of EVH for at least several weeks, (2) TIPS placement decreases portal hypertension, thus facilitating technical performance of the transplant procedure and minimizing blood loss, (3) TIPS may undergo vascular incorporation, thus requiring that they be accurately positioned so that the lengths of suprahepatic inferior vena cava and portal vein are not compromised at the time of transplantation, (4) TIPS thrombosis can be effectively treated and prolonged patency may be observed, and (5) deterioration in hepatic function and exacerbation of hepatic encephalopathy were not observed after TIPS placement. In summary, TIPS provide an attractive, effective means for managing refractory EVH in patients awaiting liver transplantation.