Oesophageal Lichen Planus Successfully Treated with Budesonide Orodispersible Tablets: A Case Report.

Introduction: Lichen planus is a relatively common inflammatory condition of the nails, skin, and mucosal surfaces. Oesophageal involvement of lichen planus is thought to be very rare, mainly described in case reports, but is associated with a high risk of oesophageal stenosis as well as squamous cell carcinoma. No evidence-based treatment recommendations exist, with the majority of described treatment regimens involving systemic immunosuppression. Case Report: In this case report, we describe a novel approach in treating oesophageal lichen planus in a patient with budesonide orodispersible tablets, a treatment normally reserved for eosinophilic oesophagitis. The patient achieved complete relief of dysphagia, with a follow-up oesophagogastroduodenoscopy 2 months after treatment commencement being macroscopically and microscopically free of inflammatory activity. This case report is to our knowledge the first to report this treatment regimen in oesophageal lichen planus. Conclusion: We consider a trial of budesonide orodispersible tablets a reasonable initial management as it's a local therapy specific to the oesophagus with a more benign side effect profile than systemic immunosuppression, but further studies need to be undertaken to corroborate our findings. Also, based on the severity and malignant potential of oesophageal lichen planus, we suggest that physicians be liberal in ordering oesophagogastroduodenoscopy with biopsy taking as part of the workup of dysphagia in a patient with known lichen planus.

Cutaneous Hydrophilic Polymer Embolism in a Patient with Acute Kidney Injury Post Endovascular Procedure.

Background: Hydrophilic polymer gel coatings are used on different intravascular devices to prevent vasospasm and thrombosis. However, it may become dislodged from these devices, leading to ischaemic complications in various organs including the skin, kidneys, brain, heart or lungs. Hydrophilic polymer embolisation (HPE) is a rare complication following endovascular procedures that is currently not fully recognised. The current knowledge of this phenomenon is based on reports consisting of histologic evidence of foreign polymers in the affected organ. Case description: A 76-year-old male with a history of hypertension, type 2 diabetes, renal cell carcinoma and chronic kidney disease underwent endovascular stenting of the superficial femoral artery due to critical limb ischaemia of the right foot. The patient had an acute kidney injury following the procedure. Upon examining the legs, there were tender non-blanching macular lesions on the right lower limb. A skin biopsy of the lesion was performed and showed hydrophilic polymer embolisation. Unfortunately, a few weeks later the patient was readmitted due to a worsening of the right foot wound situation, which required below-knee amputation. Conclusion: HPE is a rarely reported complication after endovascular interventions, with the potential to embolise to multiple organs. By observing skin manifestations, it is possible to aid the early detection of ischaemic events in other organs and identify their underlying causes. Generally speaking, the course is benign and self-limiting when the skin is involved, but may be more sinister especially when other organs (e.g. brain) are involved. LEARNING POINTS: Meticulous skin examination is an essential part of the diagnostic armamentarium of an internist, aiding in formulating a differential diagnosis based on such findings.Hydrophilic polymer embolisation (HPE) is a rarely reported complication after endovascular procedures.Given the potentially attenuated histological findings of HPE, history details of recent endovascular intervention are key to the diagnosis.

A Case Report of a Challenging Disease: Immunoglobulin G4-Related Disease With Acute Kideny Injury.

Immunoglobulin G4-related disease (IgG4-RD), which was initially identified as a type of autoimmune pancreatitis around the year 2000, is now widely acknowledged to be a systemic sickness. Based on both general and organ-specific criteria, alongside laboratory measurements of IgG4-subtype, the diagnosis is made. The diagnosis requires, however, a heightened index of suspicion, especially given the nonspecific clinical presentation. In addition to this, the symptoms may be "disseminated" in time and the multitude of organ-system involvement may seem initially unrelated. Furthermore, IgG4 levels may be falsely normal especially during the first presentation of IgG4-RD. We report a case of a 33-year-old male who was referred by his general practitioner (GP) to the fast access nephrology clinic due to elevated creatinine and fatigue, which was found after the patient had undergone some investigations at the GP office. He had history of atopic dermatitis and a prior admission for acute pancreatitis of unknown cause and recent bilateral anterior uveitis treated with steroid eyedrops. His urinalysis showed one to two granular casts per high-power field (HPF), and his creatinine was 262 µmol/L (previously normal). Three main differential diagnoses were considered given the patient's history: sarcoidosis, tubulointerstitial nephritis with uveitis (TINU) and IgG4-related disorder. Investigations were undertaken in that regard showing elevated serum IgG4 levels (2.7 times upper-limit of normal). Renal biopsy demonstrated tubulointerstitial nephritis (TIN) with 30 IgG4-positive plasma cells per HPF. Given the patient's presentation over time, a diagnosis of IgG4-TIN was considered. The patient was treated with high-dose steroids and has shown signs of improvement of both his renal and ocular problems. The uniqueness of the case is reflected through the fact that IgG4-renal disease is usually diagnosed in patients with an already established manifestation of another organ, whilst in our patient the renal involvement led to establishing IgG4-RD. It is also important to note that, in spite of initially negative serum IgG4 levels, the diagnosis still needs to be considered especially if multisystem involvement is present (as in this case).

Simple diameter measurements with ultrasound can be safely used to follow the majority of patients after infrarenal endovascular aneurysm repair.

BACKGROUND: The optimal imaging follow-up after infrarenal EVAR is still undefined. The aim of this study was to analyze the outcome of a personalized follow-up program after infrarenal EVAR based on ultrasound AAA diameter measurements for low-risk patients. METHODS: All consecutive patients followed-up locally after elective and acute infrarenal EVAR between 2010 and 2015 were retrospectively reviewed. Patients underwent CTA at 1-month post-EVAR whereby the attending surgeon defined the subsequent follow-up. Patients considered at low risk were followed with ultrasound only assessing AAA diameter at 1, 2, 3 and every 5 years postoperatively (group A). Low risk required a favorable pre-operative anatomy especially regarding the aneurysm neck, satisfactory intraoperative result and uneventful 1 month CTA (type 2 endoleaks acceptable). Patients not fulfilling the criteria for group A were followed with yearly 3-phase-CTAs (group B). RESULTS: Two hundred twenty-two patients with a AAA median diameter of 58 (54-68) mm were included. One hundred ninety-one were allocated into group A and 31 in group B. Median follow-up time was 36 (24-59) months. Five-year primary and primary-assisted success was 82±5% and 93±3% for group A and 70±13% and 93±5% for group B, respectively (P=0.042 and P=0.504, respectively). Sixteen late aneurysm-related reinterventions were performed in 12 patients (7 in group A and 9 in group B). In group A, 5 reinterventions were rupture-preventing and 2 were symptomatic. All late reinterventions in group B were performed following findings on follow-up imaging. Five-year late reintervention-free survival was 95±2% and 84±7% for groups A and B, respectively (P=0.046). Five-year survival was 80±3% and 63±10% for group A and B, respectively (P=0.024). CONCLUSIONS: A customized follow-up program after infrarenal EVAR based on ultrasound AAA diameter measurements in low-risk patients seems to be effective in maintaining a very high mid-term clinical success rate.

Endovascular aneurysm repair for symptomatic abdominal aortic aneurysms has comparable results to elective repair in the long term.

OBJECTIVE: Endovascular aneurysm repair (EVAR) has been extensively study regarding elective and ruptured abdominal aortic aneurysm (AAA) repair. However, much less is known about EVAR of symptomatic nonruptured AAA, especially concerning the long-term results. The aim of this study was to assess the outcomes of EVAR of symptomatic AAA compared with asymptomatic AAA at a tertiary center using a single graft. METHODS: All consecutive patients treated for symptomatic and asymptomatic AAAs from 1998 to 2012 at our institution, using the Cook Zenith stent graft (Cook Europe A/S, Bjaeverskov, Denmark), were included in the study. Ruptured AAAs were excluded. Patients' charts were reviewed to obtain preoperative, intraoperative, and postoperative data. All available imaging was reviewed. Life tables were constructed to assess for overall and late AAA-related survival, clinical success, and endoleak freedom. RESULTS: There were 680 patients included (137 symptomatic AAAs). No difference in technical success rate (96.1% for asymptomatic AAAs vs 94.9% for symptomatic AAAs) was present (P = .477). Thirty-day mortality was more common in symptomatic AAAs (6.6% vs 1.5% for asymptomatic AAAs; P = .002). Freedom from reinterventions was 72% ± 3% for asymptomatic AAAs vs 73% ± 5% for symptomatic AAAs (P = .785) at 10 years postoperatively. There was no difference in primary (P = .300) or secondary (P = .099) clinical success between groups, although there was higher assisted clinical success (P = .023) for asymptomatic AAAs compared with symptomatic AAAs. Persistent late clinical failure was similar in both groups (14.2% for asymptomatic AAAs vs 15.3% for symptomatic AAAs; P = .732). Freedom from late AAA-related death was higher (P = .016) for asymptomatic AAAs compared with symptomatic AAAs, but the differences disappeared when the first 30 days were disregarded. Overall survival (P = .687) was similar in both groups. An adequate aneurysm neck preoperatively conferred a better outcome in end points including overall survival. CONCLUSIONS: Symptomatic AAAs have an almost quadrupled 30-day mortality compared with asymptomatic AAAs, but the outcome differences fade in the long term. An adequate aneurysm neck was associated with better outcomes including overall survival independent of the initial presentation of the AAA. These results suggest the need of improving the identification of symptomatic patients requiring preoperative medical optimization. However, this is often limited by the acute need of the procedure, and more intensive postoperative monitoring may have greater potential. Independently, a strict anatomic selection for infrarenal EVAR is of paramount importance for the long-term outcome.

Low Iliofemoral Calcium Score May Predict Higher Survival after EVAR and FEVAR.

BACKGROUND: Abdominal aortic aneurysm is associated with an increased mortality, mostly cardiovascular events. Moreover, aortoiliac calcification is associated with increased mortality in patients with peripheral occlusive disease. The aim of this study is to assess the potential association between iliofemoral calcification, assessed by calcium score, in patients undergoing infrarenal (endovascular aneurysm repair [EVAR]) or fenestrated endovascular aortic repair (FEVAR) and long-term mortality, particularly caused by cardiac events. METHODS: All patients with preoperative noncontrast-enhanced computed tomographic scans who underwent infrarenal EVAR and FEVAR of nonruptured abdominal aortic aneurysm between 2004 and 2012 at a single tertiary center were screened for inclusion. Agatston calcium score was measured from the aortic bifurcation to common femoral arteries using a dedicated postprocessing software. The values are presented as median and interquartile range. RESULTS: About 404 (62.05%) of 651 patients who underwent EVAR and FEVAR had sufficient imaging quality to be included. There was no difference in survival between included and excluded patients (P = 0.33). Nine patients (2.2%) died within 30 days of the operation, whereas the remaining were followed up for 6.3 (4.7-8.4) years. The iliofemoral calcium score was 8348 (3830-14,179). Estimated overall survival at 5 years was 73 ± 2%. Patients within the lowest quartile of iliofemoral calcium score had significantly higher overall survival (5 years: 79 ± 4% vs. 71 ± 3%; P = 0.01) and cardiac event-free survival (5 years: 95 ± 2% vs. 91 ± 2%; P = 0.033) when compared with the remaining ones. Calcium score was associated with neither univariate regression analysis with survival (odds ratio, 1.016 [0.988-1.045]; P = 0.268) nor cardiac event-free survival (odds ratio, 1.024 [0.986-1.063]; P = 0.222). CONCLUSIONS: Low iliofemoral calcium score may be associated with lower incidence of fatal cardiac events and all-cause long-term mortality after EVAR and FEVAR. This may be partially a reflection of aging and cardiovascular comorbidity but needs to be studied further.

Long-term outcomes of infrarenal endovascular aneurysm repair with a commercially available stent graft.

OBJECTIVE: To assess the long-term results of elective abdominal aortic aneurysm (AAA) repair with a single stent graft system. METHODS: Patients undergoing elective, infrarenal endovascular aneurysm repair (EVAR) with a single-stent graft system between 1998 and 2012 were analyzed retrospectively registering pre-, intra-, and postoperative data. All imaging was reviewed. Data are presented as median and interquartile range unless otherwise stated. RESULTS: A total of 543 patients were included (476 males; 74 [69-79] years). Technical success was achieved in 522 (96.1%) patients. Eight (1.5%) patients died perioperatively. Median imaging follow-up was 5.0 (2.8-8.0) years. Freedom from reinterventions was 72 ± 3% at 10 years. Primary and secondary clinical success rates were 58 ± 3% and 78 ± 2% at 10 years, respectively. Freedom from late AAA-related death was 96 ± 1% and overall survival was 32 ± 2% at 10 years. Late persistent secondary clinical failure occurred in 77 (14.2%) patients. Most of the patients with late failures did not undergo reinterventions (83.1%) because they were unfit (39 patients), refused (5 patients), or had stable mild findings (20 patients). Five patients were converted to open repair (most recent, 2008). Favorable neck anatomy was more common in the second half of the study and was associated with less proximal intraoperative adjuncts. Importantly, it also conferred higher primary and secondary clinical success (P = .001 and P < .0001) and primary and assisted freedom from type I/III endoleaks (P = .002 and P < .0001); along with lower AAA-related mortality rates (P = .008). CONCLUSIONS: Elective infrarenal EVAR of asymptomatic AAA with single-stent graft has sustainable long-term results, especially when the aneurysm neck anatomy is favorable. AAA-related death and conversion to open repair are very rare events, but the overall survival after 10 years is quite low. The majority of the patients with persistent failure were unfit or refused further reinterventions or had stable findings. Good patient selection and technical developments may continue to improve the results in the future.

Midterm results of laser generated in situ fenestration of the left subclavian artery during thoracic endovascular aneurysm repair.

OBJECTIVE: To analyze the midterm result of in situ fenestration (ISF) of the left subclavian artery (LSA) during thoracic endovascular aneurysm repair (TEVAR). METHODS: In this clinical study, between 2014 and 2016, ISF for LSA revascularization was attempted during TEVAR in 10 patients (7 males; median age, 68 years). An excimer laser, placed from the left brachial artery, was used to create a fenestration and all fenestrations were stented with covered stent grafts. Follow-up included computed tomography scans 1 month postoperatively and annually thereafter. Survival was analyzed according to Kaplan-Meier. RESULTS: Nine of the 10 laser-assisted ISF were successful. No 30-day mortality occurred. One patient had a transient ischemic attacked in the left carotid territory. After a median follow-up of 27 months, all fenestrations were patent. At 1 month, computed tomography follow-up showed nonspecific endoleaks of unknown origin in five of the nine patients. At 27 months follow-up, only two endoleaks remained. One reintervention was done after 24 months owing to a type Ic endoleak from the LSA. Overall TEVAR success, defined as survival with no aneurysm expansion, was eight of nine. One patient showed aneurysm expansion after 14 months. Two deaths occurred (at 33 and 31 months postoperative), one of unknown reason and one aneurysm related. CONCLUSIONS: Laser fenestration might be an option for LSA revascularization during emergent or semiurgent TEVAR and electively in patients with hostile neck anatomy (eg, previous neck radiation, short and adipose necks) that might make a carotid-subclavian bypass difficult. The LSA fenestration has excellent patency and TEVAR success was not affected by nonspecific endoleaks around the LSA.