Long-term outcomes of below-the-knee bypass surgery using heparin-bonded expanded polytetrafluoroethylene grafts.

PURPOSE: To report the outcomes of below-the-knee (BK) bypass surgery using heparin-bonded expanded polytetrafluoroethylene (ePTFE) grafts, performed in two centers since its launch in Japan. METHODS: We conducted a retrospective analysis of databases from two medical centers, evaluating 51 limbs in 42 consecutive patients with peripheral arterial disease (PAD), who underwent BK bypass surgery using heparin-bonded ePTFE grafts between October, 2013 and April, 2023. RESULTS: Thirty-three limbs (64.7%) were classified as Rutherford category 4-6 and 33 limbs (64.7%) had a history of ipsilateral revascularization. Technical success was achieved in 98% of the patients. The 30 day mortality rate was 2.4% (n = 1) and the overall 30 day complication rate was 9.5% (n = 4). The median follow-up period was 38 (interquartile range 13-67) months. Three patients required major amputation and 14 died during follow-up. Primary patency rates at 1, 3, and 5 years were 67.8%, 57.5%, and 46.5%, respectively, while secondary patency rates for these periods were 84.6%, 70.0%, and 66.0%, respectively. Overall survival rates at 1, 3, and 5 years were 90.1%, 74.5%, and 70.9%, respectively. CONCLUSIONS: BK bypass surgery using heparin-bonded ePTFE graft is a viable and durable option for patients with PAD, who are deemed unsuitable for autologous vein bypass surgery.

Popliteal vein entrapment syndrome associated with an accessory slip of the lateral head of the gastrocnemius muscle.

Popliteal vein entrapment syndrome (PVES) is a rare subtype of popliteal entrapment syndrome that leads to symptoms of chronic venous stasis. We report a case of isolated PVES in a young patient associated with an accessory slip of the lateral head of the gastrocnemius muscle. The patient underwent resection of the anomalous muscle, and the symptoms were relieved postoperatively. PVES should be considered in young patients with unexplained symptoms or signs of venous stasis. Surgical resection of the causative lesion compressing the popliteal vein is indicated for selected patients.

Mucosal adjuvanticity and mucosal booster effect of colibactin-depleted probiotic Escherichia coli membrane vesicles.

There is a growing interest in development of novel vaccines against respiratory tract infections, due to COVID-19 pandemic. Here, we examined mucosal adjuvanticity and the mucosal booster effect of membrane vesicles (MVs) of a novel probiotic E. coli derivative lacking both flagella and potentially carcinogenic colibactin (ΔflhDΔclbP). ΔflhDΔclbP-derived MVs showed rather strong mucosal adjuvanticity as compared to those of a single flagellar mutant strain (ΔflhD-MVs). In addition, glycoengineered ΔflhDΔclbP-MVs displaying serotype-14 pneumococcal capsular polysaccharide (CPS14+MVs) were well-characterized based on biological and physicochemical parameters. Subcutaneous (SC) and intranasal (IN) booster effects of CPS14+MVs on systemic and mucosal immunity were evaluated in mice that have already been subcutaneously prime-immunized with the same MVs. With a two-dose regimen, an IN boost (SC-IN) elicited stronger IgA responses than homologous prime-boost immunization (SC-SC). With a three-dose regimen, serum IgG levels were comparable among all tested regimens. Homologous immunization (SC-SC-SC) elicited the highest IgM responses among all regimens tested, whereas SC-SC-SC failed to elicit IgA responses in blood and saliva. Furthermore, serum IgA and salivary SIgA levels were increased with an increased number of IN doses administrated. Notably, SC-IN-IN induced not only robust IgG response, but also the highest IgA response in both serum and saliva among the groups. The present findings suggest the potential of a heterologous three-dose administration for building both systemic and mucosal immunity, e.g. an SC-IN-IN vaccine regimen could be beneficial. Another important observation was abundant packaging of colibactin in MVs, suggesting increased applicability of ΔflhDΔclbP-MVs in the context of vaccine safety.

Long-Term Outcomes of a Japanese Prospective Multicenter Registry Using a Heparin-Bonded Expanded Polytetrafluoroethylene Graft for Above-the-Knee Femoropopliteal Bypasses.

BACKGROUND: Despite the widespread use of PROPATEN®, a bioactive heparin-bonded expanded polytetrafluoroethylene graft, in bypass surgery, there are only a few reports of long-term results. We evaluated the long-term results of PROPATEN®use for above-knee femoropopliteal bypass (AKFPB).Methods and Results: After PROPATEN®-based AKFPB, patients were prospectively registered at 20 Japanese institutions between July 2014 and October 2017 to evaluate long-term results. During the median follow-up of 76 months (interquartile range 36-88 months) for 120 limbs (in 113 patients; mean [±SD] age 72.7±8.1 years; 66.7% male; ankle-brachial index [ABI] 0.45±0.27; lesion length 26.2±5.7 cm; chronic limb-threatening ischemia in 45 limbs), there were 8 major amputations; however, clinical improvement was sustained (mean [±SD] ABI 0.87±0.23) and the Rutherford classification grade improved in 105 (87.5%) limbs at the latest follow-up. At 8 years, the primary patency, freedom from target-lesion revascularization, secondary patency, survival, and amputation-free survival, as estimated by the Kaplan-Meier method, were 66.3±4.8%, 71.5±4.4%, 86.5±3.4%, 53.1±5.0%, and 47.4±5.3%, respectively. CONCLUSIONS: This multicenter prospective registry-based analysis showed sustained excellent clinical improvement and secondary patency for up to 8 years following PROPATEN®-based AKFPB. PROPATEN®constitutes a durable and good revascularization option for complex superficial femoral artery lesions, especially when endovascular treatment is inappropriate or an adequate venous conduit is unavailable.

Practicality and Hemodynamic Advantage of Microvascularly Augmented Pectoralis Major Musculocutaneous Flap: Technical Approach and Evidence Based on Indocyanine Green Angiography.

ABSTRACT: We have recently shown that including the blood flow from the lateral thoracic artery (LTA) in addition to the thoracoacromial artery in the pectoralis major muscle musculocutaneous (PMMC) flap (bipedicle PMMC flap) can increase the perfusion of the flap. We also developed the concept of the supercharged PMMC flap, in which the LTA included in the flap was once cut and anastomosed to a cervical artery under a microscope. It is an effective solution to maintain the additional blood flow from the LTA, when the length of the LTA is compromised for reconstruction. The mandibular reconstruction of an oral cancer patient was performed with a supercharged PMMC flap. Intraoperative indocyanine green angiography was performed in a single pedicle, bipedicle, and supercharged conditions, and the videos were analyzed with a quantitative assessment system of perfusion using some parameters. As a result, blood supply from the LTA was essential for flap survival in this patient, and supercharging from the cervical artery improved flap perfusion compared with the perfusion in the bipedicle condition. The supercharged PMMC flap can resolve the compromise of pedicle length and be also hemodynamically advantageous, thus making the reconstruction more reliable than the conventional technique.

Endovascular Treatment as an Alternative to Bypass Surgery for Juxtarenal Aortic Occlusion: Results from the CHAOS (CHronic Abdominal Aortic Occlusion, ASian Multicenter) Registry.

BACKGROUND: Juxtarenal aortic occlusion (JRAO), in which the occlusion of the aorta extends to just below the renal artery, is often treated by bypass surgery because of concerns about the risk of procedural failure and fatal embolization to abdominal organs when treated with endovascular treatment (EVT). This study assessed the outcome of EVT for JRAO compared with aorto-biiliac /aorto-bifemoral (AOB) or axillo-bifemoral (AXB) bypass. METHODS: A retrospective review of an international database created by 30 centers in Asia (CHronic Abdominal Aortic Occlusion, ASian Multicenter registry) was performed for patients who underwent revascularization for chronic total occlusion of the infrarenal aorta from 2007 to 2017. Of the 436 patients, 130 with JRAO (Forty-seven AOBs, 32 AXBs, and 51 EVTs) from 25 institutions were included in this study. RESULTS: Patients were significantly older in the AXB and EVT groups and more malnourished in the EVT group than the AOB group. EVT was attempted but failed in 1 patient. Seven patients (1 [2.1%] in the AOB group, 1 [3.1%] in the AXB group, and 5 [9.8%] in the EVT group) died during hospitalization, but most of the causes in the EVT group were not related to the revascularization procedure. No visceral embolism was observed, which had been concerned, even though protection was performed only in 2 cases of the EVT group. At the latest follow-up (median duration 3.0 years), the ankle-brachial pressure index was significantly higher in the order of AOB, EVT, and AXB. At 4 years, the estimated primary and secondary patency rates of the AOB group (87.5% and 90.3%, respectively) were significantly higher than the AXB group (66.7% and 68.6%, respectively). CONCLUSIONS: AOB remains the gold standard and should be the first choice for acceptable risk patients. For frail patients, EVT is a good option and likely preferable as a first-line treatment compared to AXB.

Refractory Deep Vein Thrombosis Caused by Femoral Vein Stenosis Due to Suture-Medicated Vascular Closure Device.

Vascular closing devices (VCDs) are widely used to replace manual compression at the femoral puncture site after catheter insertion. Perclose ProGlide is a suture-medicated VCD that is indicated for both arterial and venous access sites. However, there are few reports of complications related to venous use of ProGlide. Here, we describe a case of femoral vein stenosis caused by a suture-medicated VCD after an ablation procedure, which developed refractory deep vein thrombosis even after surgical vascular repair.

Clinical significance of Bacteroides fragilis as a potential prognostic factor in colorectal cancer.

INTRODUCTION: Bacteroides fragilis (B. fragilis) is considered to act in an anti-inflammatory manner on the intestinal tract. On the contrary, enterotoxigenic B. fragilis (ETBF), a subtype of B. fragilis, produces an enterotoxin (BFT; B. fragilis toxin), leading to asymptomatic chronic infections and colonic tumor formation. However, the impact of B. fragilis and ETBF on the clinical outcome of colorectal cancer (CRC) remains unclear. We aim to assess whether their presence affects the outcome in patients with CRC after curative resection. METHODS: We obtained 197 pairs of matched formalin-fixed paraffin-embedded samples from cancerous and adjacent non-cancerous tissues of patients with pathological stage (pstage) II and III CRC after curative resection. The presence of B. fragilis and ETBF were estimated using real-time polymerase chain reaction, and recurrence-free survival (RFS) and overall survival (OS) of the patients were analyzed. RESULTS: 16S rRNA for B. fragilis and bft DNA were detected in 120 (60.9%) and 12 (6.1%) of the 197 patients, respectively. B. fragilis-positive patients had better RFS than B. fragilis-negative patients, although that was not statistically significant. In subgroup analysis, better outcomes on RFS were observed in the presence of B. fragilis in pstage II and left-sided CRC. The association of B. fragilis positivity on OS was accentuated in the depth of T4 subgroup. No significant differences were observed in RFS and OS between ETBF and non-toxigenic B. fragilis. CONCLUSIONS: Our findings suggest that the presence of B. fragilis is associated with better outcomes in patients with pstage II and III CRC after curative resection.

Comparison of Clinical Outcomes in Patients Undergoing Common Femoral Thromboendarterectomy With or Without Patch Angioplasty.

OBJECTIVE: Surgical thromboendarterectomy (TEA) is the standard treatment for an occlusive lesion of the common femoral artery (CFA). However, there is limited knowledge on the need for patch angioplasty in CFA TEA. The objective of this study was to compare the peri-operative and two year results of CFA TEA with or without patch angioplasty. METHODS: A multicentre retrospective observational study was performed at 34 Japanese centres. Comparisons were made between patients undergoing CFA TEA with or without patch angioplasty after propensity score matching (PSM). The primary endpoints were primary patency and freedom from target lesion revascularisation (TLR) of the TEA lesion. The secondary endpoints were hospital outcomes, limb salvage, and overall survival. RESULTS: Between 2018 and 2020, 428 TEA procedures (237 with patch angioplasty and 191 with primary closure) were performed. PSM extracted 151 pairs with no significant intergroup differences in baseline characteristics. Peri-operative death and complications occurred in 0.7% vs. 1.3% (p = 1.0) and 6.0% vs. 6.6% (p = 1.0), respectively. The follow up rate was 96% over a median follow up of 14.9 months (interquartile range 8.3, 24.3). Loss of primary patency occurred in 18 patients. The two year primary patency of patch angioplasty cases was statistically significantly higher than that of primary closure cases (97.0% vs. 89.9%; p = .021). TLR was performed in 14 patients. The two year freedom from TLR in patch angioplasty cases was also statistically significantly higher than in primary closure cases (98.6% vs. 92.9%; p = .003). During follow up, seven limbs required major amputation and 40 patients died. There was no statistically significant difference in limb salvage and survival between the two groups after PSM. CONCLUSIONS: This is the first report to show that patch angioplasty may decrease re-stenosis and target lesion revascularisation of CFA TEA lesions.

Development of a Non-Invasive Local Heating Load Test to Detect Severe Limb Ischemia Within 200 seconds.

PURPOSE: To establish a non-invasive test method for the rapid detection of severe ischemia (SI) in the limbs in patients with peripheral arterial disease (PAD). METHODS: Between November 2019 and May 2021, 22 patients admitted for PAD to 2 hospitals agreed to participate in the study. All patients underwent a local heating load (LHL) test. SI was defined as at least 1 ankle-brachial index value of <.4 and/or transcutaneous oximetry value of <30 mmHg. The other cases were classified as mild-to-moderate ischemia (MMI). The LHL test was performed simultaneously with 15 minutes of heating and measurement by attaching a blood flow meter measuring probe combined with a warmer to the patient's dorsal foot. Evaluation consisted of 200-s periods from the start of heating to 800 seconds. For each period, perfusion value (PV) was evaluated, and slope was calculated graphically based on linear regression as PV fluctuation per minute. Test accuracy was calculated using a receiver operating characteristic curve. RESULTS: Twenty-four limbs of 18 patients were finally evaluated; 4 patients (2 with missing data, 1 with collagen disease, and 1 with embolism) were excluded, with 13 and 11 limbs in the SI and MMI groups, respectively. The SI group showed a significantly lower slope value in the first 200 seconds and lower PV at 200 seconds and thereafter. From the slope value, it was possible to detect SI with 85% sensitivity and 73% specificity at 200 seconds. PV could be determined with higher accuracy in periods after 200 seconds, with 85% sensitivity and 82% specificity at 800 seconds. CONCLUSIONS: Our non-invasive LHL test could be used as a rapid screening test to detect SI in limbs within 200 seconds, as well as a more accurate test to detect ischemia within 800 seconds.

Failed endovascular abdominal aortic aneurysm repair due to Mycobacterium bovis infection following intravesical bacillus Calmette-Guérin therapy.

A mycotic aneurysm after intravesical instillation of bacillus Calmette-Guérin (BCG) for early-stage bladder cancer is a rare, but life-threatening, complication. In the present report, we have described the case of a patient who had undergone endovascular aneurysm repair for a rapidly growing saccular abdominal aortic aneurysm after BCG therapy. Three months after endovascular aneurysm repair, the patient had developed an abscess that required open surgery. Cultures from a blood sample and the abscess revealed Mycobacterium bovis BCG. A mycotic aneurysm due to BCG therapy should be suspected in patients with a history of BCG treatment. Such patients should immediately start antitubercular therapy.

Septicemia after cyanoacrylate glue closure of varicose veins.

Cyanoacrylate glue closure (CAC) is being increasingly used as a minimally invasive procedure for the treatment of symptomatic incompetent saphenous veins. The most common adverse event associated with CAC has been phlebitis, including hypersensitivity phlebitis, superficial thrombophlebitis, and granulomatous phlebitis. This complication can be serious and debilitating. In the present report, we have described a case of symptomatic septicemia after CAC that required surgical excision of the treated saphenous veins.

Safety and Ease of Sheath Insertion During Endovascular Treatment Via the Common Femoral Artery After Endarterectomy With Autologous Repair: A Retrospective Cohort Study.

Background Endovascular treatment (EVT) using the common femoral artery (CFA) for access after endarterectomy (EA) may result in sheath insertion difficulties because of subcutaneous scar tissue, as well as difficulties with hemostasis. We evaluated the safety of CFA access and the ease of sheath insertion over time after EA. Method We included 19 patients (21 limbs, 40 cases) in whom the CFA was used after EA with autologous repair as an access route in EVT for peripheral arterial disease in our institution from January 2013 to December 2020. Nine limbs underwent simple closure repair and 12 underwent autologous patch repair. Difficult sheath insertions were defined as those in which additional devices (stiff guidewire or a smaller diameter sheath for dilation) were used for scheduled sheath insertion. The inability to insert a sheath with the scheduled diameter was defined as a failed sheath insertion. We evaluated the EVT timing after EA for difficult sheath insertions, and whether the CFA was repaired with simple closure or autologous patch repair during EA surgery. Results There were 10 (25%) difficult sheath insertions, with one (2.5%) failure. The rate of difficult sheath insertions peaked from 6 months to 1 year after EA and gradually decreased (47% from 6 months to 3 years, 14% thereafter). There were more statistically significant difficult sheath insertions with simple closure repair (50%) than with autologous patch repair (12%) (P = 0.018). Hemostasis devices were used in 90% of EVT cases. The median maximum sheath diameter was 6 Fr (mean = 5.8 Fr). None of the cases required surgical procedures to achieve hemostasis after EVT. Conclusion EVT may be performed safely using the CFA after EA. The difficulty of sheath insertion may differ depending on the EVT timing after EA; it was more difficult with simple closure than with autologous patch repair, possibly related to scar formation.

Upper limb gangrene due to a subclavian arterial thrombosis associated with thoracic outlet syndrome following cervical spondylotic amyotrophy.

Thoracic outlet syndrome (TOS) refers to an abnormal compression of the subclavian vessels and the brachial plexus at the base of the neck or thoracic outlet. The authors described a novel case of a 66-year-old male with arterial TOS and unilateral upper rib dysplasia following cervical spine disease. Owing to the comorbidity of cervical spondylotic amyotrophy and a history of sloping shoulder due to rib dysplasia, TOS occurred, which subsequently led to subclavian arterial thrombosis and eventually resulted in the gangrene of the hand. Thrombectomy and surgical resection of the first and second ribs avoided the progression of further neurovascular disorder. When treating a cervical spondylotic patient, concurrent TOS can be obscured by symptoms caused by cervical lesions, leading to difficulty in identifying the origin of these neurovascular findings. The possibility of subsequent thromboembolic events based on TOS should be considered, especially in cases with dysplasia of the upper ribs.

A novel technique of reimplantation of a radial artery that makes a hairpin turn to reduce the excessive vascular access flow in a dialysis patient.

We report a new technique called "reimplantation of an artery with a hairpin turn (RAHT)" to reduce excessive vascular access flow. A 73-year-old woman on dialysis consulted us for vascular surgery because of an increased cardiac preload. Chest radiography and echocardiography revealed an excessive shunt flow in the brachial artery (flow rate, 2336 mL/min). Vascular echo-Doppler of the left upper limb showed that the radial artery made a hairpin turn at the arteriovenous fistula (diameter, 9 mm). Diameters of the radial artery proximal and distal to the arteriovenous fistula were 5.4 and 3.7 mm, respectively. We ligated and divided the juxta-anastomosis proximal radial artery and subsequently created an end-to-side anastomosis between the proximal radial artery and the distal radial artery. The anastomosis ostium in the distal radial artery (the recipient) was formed with a 4-mm longitudinal and gently curved incision. We performed RAHT so that the small anastomosis between both arteries and the small diameter of the distal radial artery juxta-anastomosis segment could reduce the vascular access flow. The flow rates in the brachial artery were 500 mL/min just after surgery and 560 mL/min at 2 months after surgery. Postoperative chest radiography and echocardiography confirmed a decrease in cardiac preload. We believe that this RAHT technique could be useful as one of the options to reduce the flow in patients who have excessive vascular access flow with a radial artery that makes a hairpin turn.

Successful conversion surgery of distal pancreatectomy with celiac axis resection (DP-CAR) with double arterial reconstruction using saphenous vein grafting for locally advanced pancreatic cancer: a case report.

BACKGROUND: Pancreatic cancer is a disease with a poor prognosis, requiring multidisciplinary treatment combining chemotherapy and surgery for effective management. Distal pancreatectomy with celiac axis resection (DP-CAR) is a surgical intervention performed for locally advanced pancreatic cancer, but the benefit of arterial reconstruction in DP-CAR is unclear. CASE PRESENTATION: A 49-year-old man with pancreatic cancer was referred to our hospital. Imaging revealed a 54-mm tumor mainly in the pancreatic body, but with arterial infiltration including into the celiac, common hepatic, left gastric, splenic and gastroduodenal arteries. Distant metastases were not detected. The patient was diagnosed with unresectable locally advanced pancreatic cancer and chemoradiotherapy was planned. Three cycles of gemcitabine (1000 mg/m2) plus nab-paclitaxel (125 mg/m2) every 4 weeks were followed by irradiation (2 Gy/day, total 50 Gy over 25 days) together with S-1 administration (80 mg/m2/day). A partial response (PR) according to Response Evaluation Criteria in Solid Tumors (RECIST) was achieved, so surgical intervention was considered. Because the tumor had invaded the root of the gastroduodenal artery, we performed DP-CAR with resection of the gastroduodenal artery, followed by arterial reconstruction of the proper hepatic and left gastric arteries, anastomosed with the abdominal aorta using a great saphenous vein graft in the shape of a "Y". Histopathology showed that 60% of tumor cells were destroyed by the chemoradiotherapy, defined as grade IIb in the Evans classification. No malignancy was detected at the surgical margin, including the celiac artery, gastroduodenal artery or pancreatic stump; thus R0 surgery was successful. S-1 (80 mg/day) was administered as adjuvant chemotherapy for 6 months. The patient is now doing well without recurrence for > 2 years after the initial treatment (more than 16 months after surgery). CONCLUSION: For locally advanced pancreatic cancer, multidisciplinary treatment combining gemcitabine/nab-paclitaxel-based chemoradiotherapy and then DP-CAR surgery with gastroduodenal artery resection and arterial reconstruction using saphenous vein grafting enabled R0 resection in this patient and led to a favorable long-term prognosis.

The Comparison between Axillofemoral Bypass and Endovascular Treatment for Patients with Challenging Aortoiliac Occlusive Disease as Alternative Treatment to Aortofemoral Bypass.

Objective: Although aortofemoral bypass (AoFB) is the standard treatment for challenging aortoiliac occlusive disease (AIOD), less-invasive treatments, such as axillofemoral bypass (AxFB) or endovascular treatment (EVT) have been conducted for patients with severe comorbidities. In this study, we compared the clinical outcomes between AxFB and EVT for AIOD. Materials and Methods: We retrospectively reviewed 9 patients with AxFB and 10 with EVT for challenging AIOD. The patients' information and operative results were evaluated. The rates of patency and limb salvage were analyzed according to the Kaplan-Meier method. Results: In the EVT group, 5 of 10 (50%) patients had aortic stenting alone, 3 (30%) received aorto-uniiliac stenting, and 2 (20%) received aorto-biiliac stenting. In the AxFB group, 2 cases (22.2%) showed acute graft thrombosis; however, in the EVT group, no acute thrombotic complications were seen. The primary patency rates in the AxFB and EVT groups at 5 years were 53.6% and 81.2%, respectively (log rank P=0.225), and the assisted primary patency rates at 5 years were 53.6% and 100%, respectively (log rank P=0.012). Conclusion: EVT exhibited a more durable, better long-term patency rate than AxFB. EVT may, therefore, be a viable treatment alternative to AoFB for challenging AIOD.

The Comparison of Long-Term Results between Aortofemoral and Axillofemoral Bypass for Patients with Aortoiliac Occlusive Disease.

PURPOSE: We evaluated the clinical outcomes of aortofemoral bypass (AoFB) and axillofemoral bypass (AxFB) surgeries for complex aortoiliac occlusive disease (AIOD) and compared them from the perspectives of safety and efficacy. METHODS: We retrospectively reviewed 21 patients with AoFB grafting and 9 patients with AxFB grafting. The demographic information of the patients was examined, and the intra-, peri-, and postoperative results as well as long-term outcomes were evaluated. RESULTS: In the AoFB, 2 of 21 (9.5%) cases had intra- and perioperative complications, and 4 of 21 (19.0%) cases had postoperative complications; however, there were no postoperative mortalities. In the AxFB, two of nine (22.2%) cases had postoperative graft thrombosis; however, again there were no postoperative mortalities. According to Kaplan-Meier analysis, the primary patency rates in the AoFB and AxFB groups at 5 years were 94.8% and 53.6%, respectively (P = 0.001), while the limb salvage rates at 5 years were 96.4% and 92.9%, respectively (P = 0.320). CONCLUSIONS: Even though the patency rates with AxFB grafting were inferior to those with AoFB grafting, AxFB was able to achieve equivalent limb salvage rates and should thus be considered as an alternative treatment method, especially when limb salvage is a goal.

De Novo Renal Artery Stenosis Developed in Initially Normal Renal Arteries during the Long-Term Follow-Up of Patients with Moyamoya Disease.

BACKGROUND: The de novo occurrence of renal artery stenosis in renal arteries that were angiographically confirmed to be normal in the past has never been reported before in patients with moyamoya disease. CASE DESCRIPTION: During the long-term follow-up of pediatric patients with moyamoya disease, we observed 3 patients who developed de novo renal artery stenosis in arteries that had been angiographically confirmed to be normal 1 year after the surgery (7 years on average, ranging from 4 to 11 years). All of these patients were neurologically stable after successful indirect bypass surgery during childhood. However, more than 10 years after the surgery (15 years on average, ranging from 14 to 23 years), they developed hypertension and were found to have de novo renal artery stenosis, which was ameliorated by endovascular angioplasty. During the follow-up after angioplasty, 1 patient experienced a recurrence of hypertension and required a second and third angioplasty for restenosis. Another patient died of intracranial hemorrhage 2 years after angioplasty. In the 2 surviving patients, gene analysis of the ring finger protein 213 (RNF213; p.R4810K) point mutation, the susceptibility gene for moyamoya disease in the Asian population, was positive for the heterozygous variant. CONCLUSIONS: De novo renal artery stenosis might develop in initially normal arteries during long-term follow-up, particularly among pediatric patients with moyamoya disease. Considering the extracranial manifestations of moyamoya disease, clinicians should keep in mind that de novo renal artery stenosis could emerge later in their life. Thus, it is crucial to continue to follow these patients for decades, even if the patients are neurologically stable after bypass surgery. Monitoring for blood pressure and the de novo occurrence of renal artery stenosis is important to prevent hypertension-related morbidity and mortality, such as intracranial hemorrhage, in this disease population.