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.

Endovascular abdominal aortic aneurysm repair in 144 patients: correlation of aneurysm size, proximal aortic neck length, and procedure-related complications.

PURPOSE: During endovascular abdominal aortic aneurysm (AAA) repair, larger aneurysms often present formidable anatomic challenges to the insertion of the delivery catheter and graft deployment. The authors sought to evaluate whether large-diameter aneurysms and those with short proximal aortic necks might be associated with a higher frequency of insertion-related and short-term complications. MATERIALS AND METHODS: From October 1999 to August 2000, 144 patients underwent elective endovascular graft placement for infrarenal AAA disease at the authors' institution. These patients were treated with use of the AneuRx bifurcated endoprosthesis. AAA size (maximum aneurysm diameter) and proximal aortic neck length were compared to estimated blood loss, operative time, accuracy of graft placement, presence of endoleak, intraoperative and postoperative complications (such as limb occlusion or vascular injury), length of hospital stay, and mortality. Statistical methods included correlation analysis and logistic regression. RESULTS: There were 121 men and 23 women whose aneurysms ranged in size from 3 cm to 9.8 cm (mean, 5.6 cm; 95% CI, 5.4-5.8 cm). Endograft insertion was successful in all cases. There were three deaths within 30 days (2.1%) and seven deaths overall (4.9%). There were 43 intraoperative complications (29.9%) in 31 patients (21.5%), most of them minor. Patients with major intraoperative complications had significantly longer procedure times than those without complications (337 vs. 149 min; P <.0001). In the postoperative period (within 30 days), 31 complications (21.5%) occurred in 28 patients (19.4%), again most of them minor. AAA size was unrelated in any way to the rate of complications, but short proximal aortic neck length was associated with more serious intraoperative and postoperative complications (P =.0404 and P =.0230, respectively), and decreased 30-day and overall survival (P =.0240 and P =.0152, respectively). CONCLUSIONS: Endovascular repair of large AAAs can be challenging; however, the size of the AAA does not influence the rate of complications. A short proximal aortic neck is the only significant risk factor for more serious complications.

Use of aortic cuffs to exclude iliac artery aneurysms during AneuRx stent-graft placement: initial experience.

PURPOSE: As many as 39% of patients who undergo aortic endografting for abdominal aortic aneurysm disease will have ectasia of the iliac arteries that will require intervention. Coil embolization of the internal iliac artery and extension of the graft to the external iliac artery is one solution to this problem. However, 19%-41% of these patients experience buttock claudication, which may be permanent, after unilateral embolization. The authors examined an alternative: the use of larger-sized aortic cuffs to seal the iliac limb. Outcomes and short-term results are presented in this article. MATERIALS AND METHODS: From October 1999 to August 2000, 144 AneuRx stent-grafts were placed at the authors' institution. Among the population receiving stent-grafts, 14 patients had 15 aortic cuffs placed across the distal iliac graft limbs to seal them and preserve flow to the internal iliac artery. One patient had bilateral cuffs placed. Five patients had embolization of the contralateral internal iliac artery because of bilateral disease. Patients were followed with computed tomography (CT) at 1, 6, and 12 months to evaluate for endoleaks. RESULTS: One- and 6-month endoleak rates, determined from only those patients with follow-up CT, were 0% and 10%, respectively. One type II endoleak was first discovered 9 months after graft placement. It sealed spontaneously at 15-month follow-up. One patient among the five who had internal iliac artery embolization had claudication. Mean CT follow-up was 7.8 months (range, 1-15). One patient declined CT but was alive and well 11 months after endografting. One patient moved across the country and declined follow-up. CONCLUSION: Placement of aortic cuffs in dilated iliac arteries can preserve flow to the ipsilateral internal iliac artery and provide an adequate seal. Additionally, the option of later treatment is maintained. Patients with bilateral iliac ectasia can undergo stent-graft placement without bilateral internal iliac artery embolization. Longer-term follow-up in larger numbers of patients will be important to determine the ultimate durability of this technique.

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.

Tc-99m sulfur colloid scintigraphy for detecting perigraft flow following endovascular aortic aneurysm repair: A feasibility study.

PURPOSE: To determine if scintigraphy with Tc-99m sulfur colloid can be used to detect perigraft flow after stent-graft repair of abdominal aortic aneurysm (AAA). METHODS: Twenty-three men and two women aged 56-84 years (mean 71 years) underwent endoluminal AAA repair as part of the EVT Phase II trial [EVT = Endovascular Technologies (Menlo Park, CA, USA)]. Aneurysm size averaged 5.4 cm (range 3-8 cm). Sixteen bifurcated, seven tube, and two aorto-uniiliac grafts were placed. Two days after stent-graft placement, patients underwent both contrast-enhanced computed tomography (CT), including delayed views, and Tc-99m sulfur colloid scintigraphy. RESULTS: Perigraft flow was found in only one patient at completion of angiography. Four additional patients had perigraft flow, discovered during their postoperative follow-up CT. Four patients had leaks at an attachment site and one had retrograde branch flow. Tc-99m sulfur colloid scintigraphy failed to diagnose any of the five leaks prospectively. In two of these patients, however, some abnormal paraaortic activity was noted in retrospect. CONCLUSION: Tc-99m sulfur colloid scintigraphy was unable to demonstrate endoleak with either rapid flow (attachment site leak) or slow filling (branch flow).

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.

Interventional radiologic placement of chest wall ports: results and complications in 161 consecutive placements.

PURPOSE: To review the technical results and long-term follow-up of the first 157 consecutive patients undergoing placement of 161 chest wall ports at the authors' institution. PATIENTS AND METHODS: All ports were placed in the interventional radiology suite with use of fluoroscopic and/or ultrasound guidance with a combination of standard interventional radiology and surgical techniques. The hospital records of all patients were reviewed, and telephone contact made when possible. Patients ranged in age from 21 to 87 years (average, 57 years). The most common indication for port placement was chemotherapy (88%). RESULTS: Placement was technically successful in all 161 procedures. Minor procedural complications occurred in eight patients (5%). There were no major complications related to the procedure. Cumulative follow-up to date includes 35,992 port-days (average, 226 days per patient). The long-term complication rate was 8.7% or .39 per 1,000 access days, which includes nine infections and five migration-related complications. CONCLUSION: Interventional radiologic placement of chest wall ports is safe and has a high technical success rate, in large part due to the integration of interventional radiology techniques to the procedure. The short and long-term complication rates are equal to or less than those of current surgical series.

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.

Evaluation of coagulation tests as predictors of angiographic bleeding complications.

PURPOSE: To determine whether prothrombin time (PT), partial thromboplastin time (PTT), and platelet count are useful predictors of postangiographic hematoma. MATERIALS AND METHODS: The authors prospectively studied 1,000 consecutive patients who underwent femoral arterial puncture for a diagnostic or therapeutic vascular procedure. Demographic and procedural variables were recorded, including patient age and sex, history of medications and bleeding, procedure type and length, catheter size, and experience level of radiologist applying compression for hemostasis. RESULTS: Abnormal results of coagulation tests were not correlated with an increased occurrence of hemorrhagic complications, but bleeding complications did occur more often in patients with thrombocytopenia. Hematomas occurred in 8.1% (10 of 123) of patients with any abnormal coagulation test results and 9.7% (85 of 877) of patients with normal test results. A platelet count of less than 100 X 10(9)/L was correlated with a higher occurrence of hematoma (P = .002). CONCLUSION: Abnormal PT and PTTs do not correlate with an increased risk of postangiographic hematoma, but a low platelet count is associated with more bleeding complications.

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.