Spontaneous celiac artery aneurysms in 13-year-old and 10-year-old brothers with PLOD1-related kyphoscoliotic Ehlers-Danlos syndrome.

PLOD1-related kyphoscoliotic Ehlers-Danlos syndrome is a rare, autosomal recessive connective tissue disorder characterized by congenital hypotonia, early-onset, progressive kyphoscoliosis, and generalized joint hypermobility. PLOD1-kyphoscoliotic Ehlers-Danlos syndrome is also associated with heightened vascular fragility, resulting in an elevated susceptibility to recurrent vascular complications such as arterial aneurysms, dissection, and spontaneous arterial rupture. We report the cases of two affected brothers: a 13-year-old boy presenting with spontaneous rupture of a celiac artery aneurysm and a 10-year-old boy presenting with a rapidly enlarging celiac artery aneurysm requiring urgent repair.

How I treat nutcracker syndrome.

Anatomic compression of the left renal vein in the angle between the aorta and superior mesenteric artery may be asymptomatic or may result in symptoms, including flank pain, hematuria, or pelvic pain and/or congestion. Patients can be referred to a vascular surgeon due to symptoms and/or radiologic findings. Because symptoms of nutcracker syndrome can be vague and/or nondiagnostic, careful evaluation, assessment, and counseling with patients are required before undertaking intervention, which is often an open surgical procedure. The definitive diagnosis is ideally confirmed with diagnostic venography, including pressure measurements from the left renal vein and inferior vena cava. The optimal treatment includes open decompression of the left renal vein with renal vein transposition or gonadal vein transposition, with or without concomitant management of pelvic varicosities if symptomatic. Because most patients with nutcracker syndrome are young, long-term follow-up with scheduled ultrasound examinations should be maintained.

Current Approaches for Mesenteric Ischemia and Visceral Aneurysms.

This article provides an overview of acute mesenteric ischemia, chronic mesenteric ischemia, and visceral aneurysms, with a focus on treatment. Acute mesenteric ischemia can be a challenging diagnosis. Early recognition and adequate revascularization are key to patient outcomes. Chronic mesenteric ischemia is a more insidious process, typically caused by atherosclerosis. Various options for revascularization exist, which must be tailored to each patient. Visceral aneurysms are rare and the natural history is not well defined. However, given the risk of rupture and high mortality, treatment may be complex.

Systematic and scoping reviews: A comparison and overview.

In this article, we compare and contrast methods of reviewing, summarizing, and synthesizing the literature, including systematic reviews, scoping reviews, and narrative reviews. Review articles are essential to help investigators wade through the plethora of exponentially growing medical literature. In the era of evidence-based medicine, a systematic approach is required. A systematic review is a formalized method to address a specific clinical question by analyzing the breadth of published literature while minimizing bias. Systematic reviews are designed to answer narrow clinical questions in the PICO (population, intervention, comparison, and outcome) format. Alternatively, scoping reviews use a similar systematic approach to a literature search in order to determine the breadth and depth of knowledge on a topic; to clarify definitions, concepts, and themes; or sometimes as a precursor to a systematic review or hypothesis generator to guide future research. However, scoping reviews are less constrained by a priori decisions about which interventions, controls, and outcomes may be of interest. Traditional narrative reviews still have a role in informing practice and guiding research, particularly when there is a paucity of high-quality evidence on a topic.

Results of the North American Complex Abdominal Aortic Debranching (NACAAD) Registry.

BACKGROUND: Hybrid debranching repair of pararenal and thoracoabdominal aortic aneurysms was initially designed as a better alternative to standard open repair, addressing the limitations of endovascular repair involving the visceral aorta. We reviewed the collective outcomes of hybrid debranching repairs using extra-anatomic, open surgical debranching of the renal-mesenteric arteries, followed by endovascular aortic stenting. METHODS: Data from patients who underwent hybrid repair in 14 North American institutions during 10 years were retrospectively reviewed. Society of Vascular Surgery scores were used to assess comorbidity risk. Early and late outcomes, including mortality, morbidity, reintervention, and patency were analyzed. RESULTS: A total of 208 patients (118 male; mean age, 71±8 years old) were treated by hybrid repair with extraanatomic reconstruction of 657 renal and mesenteric arteries (mean 3.2 vessels/patient). Mean aneurysm diameter was 6.6±1.3 cm. Thoracoabdominal aortic aneurysms were identified in 163 (78%) patients and pararenal aneurysms in 45 (22%). A single-stage repair was performed in 92 (44%) patients. The iliac arteries were the most common source of inflow (n=132; 63%), and most (n=150; 72%) had 3 or more bypasses. There were 30 (14%) early deaths, ranging widely across sites (0%-21%). A Society of Vascular Surgery comorbidity score >15 was the primary predictor of early mortality (P<0.01), whereas mortality was 3% in a score ≤9. Early complications occurred in 140 (73%) patients and included respiratory complications in 45 patients (22%) and spinal cord ischemia in 22 (11%), of whom 10 (45%) fully recovered. At 5 years, survival was 61±5%, primary graft patency was 90±2%, and secondary patency was 93±2%. The most significant predictor of late mortality was renal insufficiency (P<0.0001). CONCLUSIONS: Mortality after hybrid repair and visceral debranching is highly variable by center, but strongly affected by preoperative comorbidities and the centers' experience with the technique. With excellent graft patency at 5 years, the outcomes of hybrid repair done at centers of excellence and in carefully selected patients may be comparable (or better) than traditional open or even totally endovascular approaches. However, in patients already considered as high-risk for surgery, it may not offer better outcomes.

Popliteal entrapment syndrome-The case for a new classification.

OBJECTIVES: To assess the ability of the current classification system for popliteal entrapment syndrome to accurately capture all patients, and if not, to design an all-inclusive new classification. METHODS: Retrospective review of all interventions performed for popliteal entrapment syndrome between 1994 and 2013 at our institution was performed. Preoperative imaging and intraoperative findings were used to establish the compressive morphology of popliteal entrapment syndrome. Patients were categorized, when possible, into six types of the current classification system (Rich classification, modified by Levien) and into seven types of a new classification. RESULTS: Sixty-seven limbs of 49 patients were operated on for unilateral (31) or bilateral (18) popliteal entrapment syndrome. The current classification system captured the anatomy of only 43 (64%) of 67 limbs with popliteal entrapment syndrome. Compressive morphologies without a defined class included aberrant insertion of the lateral head of gastrocnemius muscle, muscle slip originating from the lateral head of gastrocnemius or hamstrings, hypertrophied hamstring muscle, abnormal fibrous bands, perivascular connective tissue, and prominent lateral femoral condyle. The new classification captured 100% of the limbs with popliteal entrapment syndrome. CONCLUSIONS: Current classification of popliteal entrapment syndrome is inadequate as more than one-third of the cases reviewed fell outside of the standard classification system. Consideration of a more inclusive new anatomic classification system is warranted.

Society for Vascular Surgery clinical practice guidelines for management of extracranial cerebrovascular disease.

Management of carotid bifurcation stenosis in stroke prevention has been the subject of extensive investigations, including multiple randomized controlled trials. The proper treatment of patients with carotid bifurcation disease is of major interest to vascular surgeons and other vascular specialists. In 2011, the Society for Vascular Surgery published guidelines for the treatment of carotid artery disease. At the time, several randomized trials, comparing carotid endarterectomy (CEA) and carotid artery stenting (CAS), were reported. Since the 2011 guidelines, several studies and a few systematic reviews comparing CEA and CAS have been reported, and the role of medical management has been reemphasized. In the present publication, we have updated and expanded on the 2011 guidelines with specific emphasis on five areas: (1) is CEA recommended over maximal medical therapy for low-risk patients; (2) is CEA recommended over transfemoral CAS for low surgical risk patients with symptomatic carotid artery stenosis of >50%; (3) the timing of carotid intervention for patients presenting with acute stroke; (4) screening for carotid artery stenosis in asymptomatic patients; and (5) the optimal sequence of intervention for patients with combined carotid and coronary artery disease. A separate implementation document will address other important clinical issues in extracranial cerebrovascular disease. Recommendations are made using the GRADE (grades of recommendation assessment, development, and evaluation) approach, as was used for other Society for Vascular Surgery guidelines. The committee recommends CEA as the first-line treatment for symptomatic low-risk surgical patients with stenosis of 50% to 99% and asymptomatic patients with stenosis of 70% to 99%. The perioperative risk of stroke and death in asymptomatic patients must be <3% to ensure benefit for the patient. In patients with recent stable stroke (modified Rankin scale score, 0-2), carotid revascularization is considered appropriate for symptomatic patients with >50% stenosis and should be performed as soon as the patient is neurologically stable after 48 hours but definitely <14 days after symptom onset. In the general population, screening for clinically asymptomatic carotid artery stenosis in patients without cerebrovascular symptoms or significant risk factors for carotid artery disease is not recommended. In selected asymptomatic patients with an increased risk of carotid stenosis, we suggest screening for clinically asymptomatic carotid artery stenosis as long as the patients would potentially be fit for and willing to consider carotid intervention if significant stenosis is discovered. For patients with symptomatic carotid stenosis of 50% to 99%, who require both CEA and coronary artery bypass grafting, we suggest CEA before, or concomitant with, coronary artery bypass grafting to potentially reduce the risk of stroke and stroke/death. The sequencing of the intervention depends on the clinical presentation and institutional experience.

Outcomes Following Inferior Mesenteric Artery Reimplantation During Elective Aortic Aneurysm Surgery.

BACKGROUND: The role of inferior mesenteric artery (IMA) reimplantation during open aortic reconstruction is debated. We assessed outcomes after inferior mesenteric artery reimplantation (IMAR) for aortic aneurysmal disease to help shed light on this question. METHODS: A single-center retrospective review of all IMARs performed during open aortic surgery over a 10-year period between 2000 and 2009 was carried out. The primary outcome was patency, while secondary outcomes included colonic ischemia and overall survival. Analysis was performed using Cox models and Kaplan-Meier estimates. RESULTS: Of 840 patients who underwent elective abdominal aortic aneurysm (AAA) reconstructions during this period, 70 underwent IMAR. Indications for IMAR included intraoperative colonic ischemia (n = 24), poor back bleeding (n = 52), large IMA (n = 5), internal iliac disease (n = 5), and prior colon surgery (n = 1). Follow-up imaging studies were available in 35 of 70 patients (computed tomography in 30 [86%] and duplex in 5 [14%]). Patency was confirmed in 32 of 35 patients (91%) over a median follow-up of 98 months. Both losses in patency were at 4 months and did not require an operation. One patient underwent left colon resection on postoperative day 9 because of ischemia. (Patency could not be confirmed.) No statistically significant predictor of patency was noted. Incidence of colonic ischemia was 1.4% in patients undergoing IMAR. The overall mortality was 51% in patients undergoing IMAR over the median follow-up period. The overall 10-year survival was 30% in patients undergoing IMAR for aortic aneurysmal disease. The nature of aneurysm (juxtarenal or higher juxta renal abdominal aortic aneurysm [JRAAA]) was associated with mortality, with a hazard ratio of 1.8 (P = 0.08) approaching significance. Ten-year survival was worse if IMAR was performed for intraoperative colonic ischemia (26% vs 34%) or in JRAAA (19.0% vs 38%; P = 0.03). Age per year at the time of repair was the only statistically significant predictor of survival (P < 0.001). CONCLUSION: IMAR for AAA remains necessary for select patients. Reimplantation is associated with excellent long-term patency and low risk of colonic ischemia.

Vascular surgery resident training in Canada.

The training of vascular surgeons in Canada has evolved over the past decade. Direct entry into a vascular surgery training program after medical school has been offered since 2012. At some institutions, it is the only option for surgery training. The smaller population of Canada and a single-payer health care system has resulted in unique opportunities and challenges for the training of vascular surgeons and providing opportunities for trainees to transition into clinical practice.

Outcomes of Transaxillary Approach to Cervical and First-Rib Resection for Neurogenic Thoracic Outlet Syndrome.

BACKGROUND: Cervical rib can often be symptomatic causing neurogenic thoracic outlet syndrome (nTOS). Surgical treatment involves rib resection through a supraclavicular, transaxillary or combined approach. We review outcomes of different approaches and describe our technique of transaxillary resection through a video. METHODS: A single-center retrospective review of perioperative and short-term outcomes in subjects undergoing cervical rib resection for nTOS between 1994 and 2013 was performed. RESULTS: Of the 75 operations performed for nTOS, 40% (30 procedures in 29 patients) required resection of cervical ribs. The first and cervical ribs were removed in 24 operations, whereas only the cervical rib was resected in 6. Scalenectomy was performed in all patients. Thirteen (43%) procedures were performed with a supraclavicular-only (SC group) approach, 9 (30%) with a transaxillary-only (TA group) approach, and 8 (27%) with a combined approach (TA + SC group). Incidence of persistent nTOS symptoms occurred in 3 (23%) of SC patients, 1 (13%) TA patient, and 2 (25%) TA + SC patients (P > 0.05). Recurrence of symptoms was noted in one patient (8%) in the SC group at 1-year follow-up. No patient required operative reintervention. CONCLUSIONS: Resection of cervical ribs and/or first ribs in the treatment of nTOS can be safely performed through SC, TA, or a combined approach. In young patients, a TA incision should be considered to avoid a neck incision, with outcomes similar to alternate approaches.

Comparison of open surgical techniques for repair of types III and IV thoracoabdominal aortic aneurysms.

OBJECTIVE: The objective of this study was to compare outcomes after repair of type III and type IV thoracoabdominal aortic aneurysms (TAAAs) by three different open surgical techniques at a tertiary care institution. METHODS: Consecutive patients who underwent elective repair of type III and type IV TAAAs at our institution between 1999 and 2011 were retrospectively reviewed. Patients were divided into three groups according to surgical technique: clamp and sew (CS), left-sided heart bypass (LHB), and visceral branching (VB) followed by aortic reconstruction. Primary end points were early mortality and complications; secondary end points were need for blood transfusion, duration of operation, and long-term survival. RESULTS: Between 1999 and 2011, there were 121 consecutive patients (83 men, 38 women) with 52 type III and 69 type IV TAAAs who underwent elective repair (CS, 65 patients; LHB, 31 patients; VB, 25 patients). Perioperative spinal drainage was used in 84%. Procedure duration was longest in the VB group (mean, 9.1 hours vs 7.7 hours and 5.7 hours for CS and LHB; P < .001), but transfusion requirement was largest in the LHB group (mean, 3.5 L vs 1.7 L and 2.1 L for CS and VB; P = .015). Mean duration of mesenteric ischemia was significantly shorter in the VB group vs CS and LHB (18 minutes vs 35 minutes for CS and 30 minutes for LHB; P < .0001). Mean intensive care unit and hospital stays were the same (9, 10, and 8 days [P = .82]; 18, 20, and 18 days [P = .76]). Overall 30-day mortality was 6.6%, not different between groups (6%, 10%, and 4%; P = .68). Mean follow-up was 45 ± 42 months, and actuarial overall survival at 3 and 5 years was 70% and 64%, with no difference between groups (P = .36). CONCLUSIONS: For repair of type III and type IV TAAAs, the sequential VB technique has the longest duration, but it has the advantage of the shortest mesenteric and visceral ischemia times without improvement in early outcomes. Irrespective of the techniques used, complications, early mortality, risk of spinal cord injury, and survival were the same.

Outcomes after early and delayed carotid endarterectomy in patients with symptomatic carotid artery stenosis.

OBJECTIVE: The objective of this study was to define outcomes after carotid endarterectomy (CEA) in patients with symptomatic carotid artery stenosis (CAS) when patients are operated on within 14 days after onset of symptoms. METHODS: Clinical data of consecutive patients who underwent CEA between 2003 and 2012 for symptomatic CAS were reviewed. Patients were classified into group 1, CEA ≤14 days of minor stroke or transient ischemic attack, and group 2, CEA >14 days. Primary end point was stroke/death; secondary end points were stroke, death, and myocardial infarction. RESULTS: There were 233 patients (32% female; mean age, 72 ± 9.1 years) who underwent 238 CEAs. Group 1 included 57 CEAs in 56 patients; 11 CEAs were performed at 0 to 2 days, 23 at 3 to 7 days, and 23 at 8 to 14 days. Group 2 included 181 CEAs in 177 patients. One death (group 2) and five strokes (group 1, four; group 2, one) occurred at 30 days (stroke/death, 2.6%), more in group 1 vs group 2 (7.1% vs 1.1%; P = .03). In group 1, three strokes occurred when the patients were operated on within 2 days (27% [3/11]), more than at 3 to 7 days (0% [0/22]) or 8 to 14 days (4.3% [1/23]; P = .008). Patients operated on between days 3 and 14 had similar stroke/death rate to those operated on after 14 days (2.2% vs 1.1%; P = .49). Myocardial infarction occurred in six patients (2.5%; group 1, 0% [0/57]; group 2, 3.3% [6/177]; P = .34). Median follow-up was 7.0 years (interquartile range, 4.6-9.9 years). Freedoms from stroke/death were similar between groups (hazard ratio [HR], 1.22; 95% confidence interval [CI], 0.75-1.99; P = .42), 69% for group 1 and 76% for group 2 at 5 years. Age ≥80 years, high surgical risk, and no preoperative P2Y12 antagonist use predicted stroke/death. Freedoms from any stroke were similar in groups (HR, 2.46; 95% CI, 0.95-6.41; P = .06); survivals were also similar (HR, 1.12; 95% CI, 0.67-1.87; P = .67) at 5 years. CONCLUSIONS: In this single-center study, CEA in symptomatic patients had a 30-day stroke/death rate of 2.6%. Age ≥80 years and high surgical risk predicted late stroke or death; taking P2Y12 antagonists was associated with late stroke. High stroke rates when patients were operated on immediately support CEA after 2 days in symptomatic patients with CAS.

Management of refractory chylous ascites with peritoneovenous shunts.

OBJECTIVE: The purpose of this study was to define outcome of treatments of refractory chylous ascites using peritoneovenous shunts (PVSs). METHODS: Clinical data of patients with refractory chylous ascites treated with PVSs between 1992 and 2015 were retrospectively reviewed. The primary end point was clinical benefit, defined as cured, improved, or poor results; secondary end points were complications and reinterventions. RESULTS: Seventeen patients (eight female [47%]; median age, 47 years [range, 19-78 years]) with refractory chylous ascites were studied. This group represented 6% of 284 patients treated for chylous ascites during the study period. The etiology was primary lymphangiectasia in 10 patients (59%) and secondary chylous ascites due to previous surgery, lymphatic obstruction with associated portal hypertension, or malignant tumor in 7 (41%). Eleven patients were treated with LeVeen shunts and six with Denver shunts. Thirty-day mortality, morbidity, and reintervention rates were 5.9%, 18%, and 12%, respectively. Reintervention rate at 6 months was 9.1% with LeVeen shunt, significantly lower than 100% with Denver shunt (P = .001). During a mean follow-up of 5.1 years (range, 17 days-22.7 years), 7 of 11 patients with LeVeen shunt and all 6 patients with Denver shunt required shunt replacement. Median duration of patency was 215 days (range, 2 days-9.0 years) of a total of 25 LeVeen shunts placed in 11 patients and 44 days (range, 6-91 days) of 20 Denver shunts placed in 6 patients. At last follow-up, patency of the LeVeen shunt was 36% (4/11); symptoms improved in 64% of the patients (7/11). Patency rate of Denver shunts was 33% (2/6), and symptoms improved in 33% (2/6). CONCLUSIONS: Treatment of refractory chylous ascites continues to be a major challenge. The only currently available PVS, the Denver shunt, had a median patency period of <2 months; it required frequent replacements and resulted in intermittent short-term clinical benefit in one-third of the patients. Improvements in technology to design new shunts, to develop new therapies, or to adopt new techniques to treat chylous ascites are urgently needed.

Thoracic outlet syndrome as a consequence of isolated atraumatic first rib fracture.

Neurogenic thoracic outlet syndrome (nTOS) resulting from an isolated first rib fracture is extremely infrequent. We report a case of performance limiting nTOS in a college athlete who was initially evaluated and treated for upper extremity ligamentous injury with only transient improvement. Subsequent noninvasive studies were consistent with TOS physiology and MRA showed a large hypertrophic callus on the first rib adjacent to the brachial plexus. With continued athletic limitations and radiographic findings consistent with TOS, surgical decompression was performed resulting in resolution of symptoms. Although apparent atraumatic isolated first rib fractures are infrequently reported etiologies for TOS in athletes, they are a reasonable consideration in this population with corresponding presentations.

Maximal aortic diameter affects outcome after endovascular repair of abdominal aortic aneurysms.

OBJECTIVE: The purpose of this study was to evaluate whether maximal aortic diameter affects outcome after endovascular aneurysm repair (EVAR) of abdominal aortic aneurysm (AAA). METHODS: Clinical data of patients undergoing EVAR between 1997 and 2011 for nonruptured asymptomatic AAAs in a tertiary center were reviewed. Patients were classified according to diameter of AAA: group 1, <5.0 cm; group 2, 5.0 to 5.4 cm; group 3, 5.5 to 5.9 cm; and group 4, ≥6.0 cm. The primary end point was all-cause mortality; secondary end points were complications, reinterventions, and ruptures. RESULTS: There were 874 patients studied (female, 108 [12%]; group 1, 119; group 2, 246; group 3, 243; group 4, 266); mean age was 76 ± 7.2 years. The 30-day mortality rate was 1.0%, not significantly different between groups (P = .22); complication and reintervention rates were 13% and 4.1%, respectively, similar between groups (P < .05). Five-year survival was 68%; freedom from complications and reinterventions was 65% and 74%, respectively; rupture rate was 0.5%. Multivariate analysis revealed that factors associated with all-cause mortality included maximal aortic diameter, age, gender, surgical risk, cancer history, and endograft type (P < .05). Group 4 had increased risks of mortality (hazard ratio [HR], 2.0; 95% confidence interval [CI], 1.38-2.85; P = .002) and complications (HR, 1.6; 95% CI, 1.2-2.7; P = .009) relative to group 1. Reinterventions were more frequent for aneurysms ≥6.0 cm (HR, 2.0; 95% CI, 1.2-3.3; P = .01). Late rupture rate after EVAR was not different between groups. CONCLUSIONS: Maximal aortic diameter is associated with long-term outcomes after elective EVAR. Patients with large AAAs (≥6.0 cm) have higher all-cause mortality, complication, and reintervention rates after EVAR than those with smaller aneurysms. We continue to recommend that AAAs be repaired when they reach 5.5 cm as recommended by the guidelines of the Society for Vascular Surgery. On the basis of our data, EVAR should be considered even in high-risk patients with a maximal aortic diameter between 5.5 and 6.0 cm because surgical risk with aneurysm size above 6.0 cm will increase significantly.

Outcomes of Women Treated for Popliteal Artery Aneurysms.

BACKGROUND: Popliteal artery aneurysms (PAAs) in women are rare and their outcomes compared with men with PAA are unknown. The purpose of this study was to compare the surgical outcomes of PAA of women with men. METHODS: All patients who underwent PAA repair at a single institution from 1985 to 2013 were reviewed. All women with degenerative PAA treated during that time frame were matched on year of repair to men. Presentation, mode of repair, and outcomes were reviewed. Survival and amputation-free survival were evaluated by life table analysis. RESULTS: During the study interval, 8 women with degenerative PAA underwent surgical treatment (1.6% of 485 total PAA repairs). The overall median follow-up was 5 years (range 1 month to 19 years), but the median follow-up was shorter for women than men (1.6 vs. 6 years, P = 0.04). At the time of repair, women were of similar age compared with men (73.5 vs. 71.7 years) and had similar aneurysm size (2.7 vs. 2.9 cm). Women had similar urgency (25 vs. 17.5% emergent) and symptomatic status (50% vs. 55% acute) even though 7 of the 8 women had a thrombosed PAA at the time of repair. Operative time, approach, graft type, and inflow and outflow sources were similar between genders. No women received endovascular repair (0% vs. 10%, P = 0.5). One patient of each gender underwent major amputation (one woman on post-operative day 158 and one man on post-operative day 3). Overall, women had lower survival and amputation-free survival at 2 years (51% vs. 100% and 20% vs. 94%, P < 0.01 for both, standard error 0.2). CONCLUSIONS: PAA requiring intervention in women is a rare clinical occurrence. Although our series is limited, women requiring PAA repair had higher long-term mortality compared with men with a similar pathology and treatment strategy.