Daniel Picus

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.

Two-dimensional contrast echocardiography in the detection and follow-up of congenital pulmonary arteriovenous malformations

Pulmonary arteriovenous (A-V) malformation is frequently a manifestation of Osler-Weber-Rendu syndrome (hereditary hemorrhagic telangiectasia). We identified 14 patients (9 men and 5 women) with A-V malformation by contrast echocardiography; 10 patients with atrial right-to-left shunt served as control subjects. Agitated saline solution (10 ml) was injected through a peripheral vein during echocardiographic imaging. The delay in the appearance of microcavitations in the left atrium was measured (in number of frames) after right atrial appearance. The degree of left ventricular opacification was graded 1 to 4+ (where 4+ = intense left ventricular endocardial outline, and 1+ = minimal opacification). Results indicated patients with A-V malformation had a significant delay (p less than 0.001) in left atrial appearance of microcavitations compared with those with atrial right-to-left shunt (66 +/- 27 vs 21 +/- 7 frames, mean +/- 1 standard deviation). In the group with A-V malformation, abnormal blood gases were present in only 6 of 14 patients and chest x-ray was positive in 7. Pulmonary angiography was performed in 11 of 14 patients with positive contrast echocardiography, and all 11 had A-V malformation identified. In patients with 3 to 4+ left ventricular opacification (n = 8), large (greater than 5 mm feeding vessel) or multiple malformations were present, whereas patients with small or isolated malformation had 1 to 2+ left ventricular opacification. Balloon occlusion of malformations was performed in all 11 of these patients; repeat contrast echocardiography revealed significant diminution of right-to-left shunt in 9, and 2 required repeat embolotherapy for an additional previously undetected A-V malformation.(ABSTRACT TRUNCATED AT 250 WORDS)

Lower Extremity Venous Thrombolysis with Adjunctive Mechanical Thrombectomy

PURPOSE To evaluate the use of adjunctive mechanical thrombectomy (MT) with pharmacologic catheter-directed lower extremity venous thrombolysis. MATERIALS AND METHODS Catheter-directed thrombolysis with adjunctive MT was used to treat 28 symptomatic limbs in 20 patients (22 procedures) with lower extremity deep vein thrombosis (DVT) between August 1997 and July 2001. Procedural success, major bleeding, thrombolytic infusion time, and total thrombolytic agent dose were recorded. RESULTS Procedural success was achieved in 23 of 28 limbs (82%). Fifteen patients (18 limbs) received iliac vein stents. Major bleeding was observed after three of 22 procedures (14%) and resulted in transfusion in two patients and endometrial ablation in the third patient. Mean per-limb infusion time was 16.8 hours +/- 12.8. Mean per-limb total doses were lower than those reported in published studies of DVT thrombolysis: 2.67 million U +/- 1.60 urokinase, 18.4 mg +/- 10.7 tissue plasminogen activator, and 13.8 U +/- 6.9 reteplase. Venographic analysis demonstrated minimal thrombus removal (26.0% +/- 24.1) when using MT alone, compared with substantial thrombus removal (62.0% +/- 24.9) when using MT after pharmacologic thrombolytic agents had been administered (P =.006). CONCLUSION The use of adjunctive MT to augment pharmacologic catheter-directed DVT thrombolysis provides comparable procedural success and may reduce the required thrombolytic dose and infusion duration.

Pharmacomechanical thrombolysis and early stent placement for iliofemoral deep vein thrombosis

PURPOSE To evaluate an approach to the treatment of iliofemoral deep vein thrombosis (DVT) that included pharmacomechanical catheter-directed thrombolysis with reteplase and the Helix mechanical thrombectomy device, followed by early stent placement. MATERIALS AND METHODS During 3-year period, 23 symptomatic limbs in 18 patients with iliofemoral DVT were treated with reteplase catheter-directed thrombolysis. After an initial infusion of 8 to 16 hours, any residual acute thrombus over a long segment (> 10 cm) was treated by maceration with use of the Helix thrombectomy device. Residual short-segment (< 10 cm) iliac vein thrombus and/or stenosis were treated with stent placement. Technical success, clinical success, complications, thrombolytic infusion time, total thrombolytic agent dose, fibrinogen level changes, and late limb status were retrospectively analyzed. RESULTS Technical success was achieved in 23 of 23 limbs (100%). Clinical success was achieved in 22 of 23 limbs (96%). Complete or partial thrombolysis was observed in 19 of 23 limbs (83%). Major bleeding was observed in one patient (6%) and necessitated blood transfusion. Mean per-limb thrombolytic infusion time and total dose were 19.6 hours +/- 8.1 and 13.8 U +/- 5.3 reteplase, respectively. Mean serum fibrinogen nadir and percentage drop in serum fibrinogen were 282 mg/dL +/- 167 and 47% +/- 24%, respectively. Late (mean, 19.8 +/- 11.6 months) modified Venous Disability Scores were 0 (none) for six limbs, 1 (mild) for 10 limbs, 2 (moderate) for two limbs, and 3 (severe) for no limbs. CONCLUSION In a preliminary experience, pharmacomechanical catheter-directed iliofemoral DVT thrombolysis with early stent placement was safe and effective.

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.

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

ObjectiveTo 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. MethodsBetween 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. ResultsPatient 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. ConclusionThe 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.

Comparison of Endoscopic and Radiological Residual Fragment Rate following Percutaneous Nephrolithotripsy

The stone-free rate after extracorporeal shock wave lithotripsy (ESWL) is primarily determined by a plain abdominal radiograph alone. However, the accuracy of that assessment can be challenged by renal tomography and flexible nephroscopy. We report on 29 patients with large renal calculi treated with a combination of percutaneous nephrolithotripsy and ESWL. Stone-free rates were determined by plain abdominal radiograph, renal tomography and flexible nephroscopy. The plain abdominal radiograph and renal tomography overestimated stone-free rates by 35% and 17%, respectively, compared to flexible nephroscopy. To determine stone-free rates after ESWL renal tomography is superior to a plain abdominal radiograph alone. Also, among patients with large renal calculi the use of the flexible nephroscope markedly improves the stone-free rate.

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

Poor results after repair of biliary injuries arc 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 intuhated 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 uf 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 phosphatasc 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.

Outcomes of percutaneous endovascular intervention for type II endoleak with aneurysm expansion.

OBJECTIVE Type II endoleak (T2EL) with aneurysm expansion is believed to place patients at risk for aneurysm-related mortality (ARM). Treatment with glue and/or coil embolization of the aneurysm sac, inferior mesenteric artery (IMA), and lumbar branches via translumbar or transarterial approaches has been utilized to ablate such endoleaks, and thus decrease ARM. We evaluated the midterm results of percutaneous endovascular treatment of T2EL with aneurysm expansion. METHODS Single-institution, 5-year (January 2003 to August 2008) retrospective study of all endovascular interventions for T2EL with sac expansion. Blinded, independent review of all available pre- and post-T2EL intervention computed tomography (CT) scans was performed. Aneurysm sac maximal transverse diameters and aneurysm sac growth rates prior to and following T2EL intervention were analyzed. RESULTS Forty-two patients (34 male, eight female; mean age, 75) underwent T2EL intervention at 26 ± 20 months after endovascular aneurysm repair (EVAR) and were subsequently followed for 23 ± 20 months. Seven out of 42 patients (17%) underwent repeat T2EL intervention. Interventions included 44 translumbar sac embolizations, and transcatheter embolizations of nine IMAs and seven lumbar/hypogastric arteries. Aneurysm diameter was 6.1 ± 1.6 cm at EVAR, 6.6 ± 1.5 cm at initial T2EL treatment, and 6.9 ± 1.7 cm at last follow-up. There were no significant differences in the rates of aneurysm sac growth pre- and post-T2EL treatment. At last follow-up imaging, recurrent or persistent T2EL was noted in 72% of patients. Of 42 patients, nine (21%) received operative endoluminal correction of occult type I or type III endoleaks that were diagnosed during the T2EL angiographic intervention. There were no aneurysm ruptures or ARMs during follow-up; overall mortality for the 5-year study period was 24%. CONCLUSIONS In this series, percutaneous endovascular intervention for type II endoleak with aneurysm sac growth does not appear to alter the rate of aneurysm sac growth, and the majority of patients display persistent/recurrent endoleak. However, diagnostic angiographic evaluation may reveal unexpected type I and III endoleaks and is therefore recommended for all patients with T2EL and sac growth. While coil and glue embolization of aneurysm sac and selected branch vessels does not appear to yield benefit in our series, the diagnosis and subsequent definitive treatment of previously occult type I and III endoleaks may explain the absence of delayed rupture and ARM in our series.

Endourological Treatment of Ureteroenteric Anastomotic Strictures: Long-Term Followup

In 1987 we reported our initial experience with an endosurgical incisional approach to ureteroenteric anastomotic strictures (that is endoureterotomy). We have extended that initial report to encompass 15 patients with 19 ureteroenteric strictures followed for an average of 2.5 years. In all cases an endosurgical approach was well tolerated, blood loss was less than 50 cc and hospital stay averaged 3.5 days. A 16 to 22F external stent was left in place for 4 to 6 weeks postoperatively in 14 strictures. A permanent external 12F catheter was left in 5 strictures due to the presence of metastatic disease (4) and a complete dense stricture (1). The only major complication was a ureteroenteric fistula that healed over a ureteral stent without any open surgical intervention. Among 14 ureteroenteric strictures in which the stent was removed, the ureteroenteric area has remained patent in 8 (57%) with an average followup of 28.6 months (range 9 to 57 months). Failures were associated with metastatic disease in 2 cases, generalized debility in 2 and unexplained in 2. In this series an endosurgical approach to ureteroenteric strictures provided long-term satisfactory management of the problem in the majority of patients with benign and malignant disease. By current standards, only 1 patient (7%) would have been considered a candidate for open surgical repair.