The role of split-thickness skin grafting in the treatment of vasculitic and pyoderma gangrenosum ulcers in a multidisciplinary wound centre.

Vasculitic and pyoderma gangrenosum ulcers are traditionally treated with immunosuppressants, and the role of surgery in the treatment of these atypical ulcers remains unclear. This study aimed to investigate the need for surgical intervention as well as the outcome and safety of skin grafting in the treatment of 46 patients with vasculitic ulcers and 34 with pyoderma gangrenosum ulcers using data recorded in the validated Wound Registry. Of the 80 patients with atypical ulcers, 14% (n = 11) were treated surgically; these patients were older (p = 0.039), had lower mobility status (p = 0.002), and more often pulmonary diseases, rheumatoid arthritis, and previous arterial procedures (p = 0.007; p = 0.031; p = 0.031, respectively) than those treated conservatively. Of 181 ulcers, 15% (n = 27) were surgically treated, 78% once and 22% multiple times. During follow-up, 92.3% of both surgically and conservatively treated ulcers with available data healed. Of the surgically treated ulcers, median healing time after first surgical procedure was 96 days, and post-surgical complications were considered mild or unrelated to surgery. Our results suggest that if surgery is indicated, skin grafting is a safe and efficient treatment method provided that multidisciplinary approach is applied.

Rare Case of Abdominal Aortic and Multiple Visceral Aneurysms in a Pediatric Patient With PIK3CA Mutation and Vasculitis.

Abdominal aortic aneurysms (AAA) are most commonly observed in elderly male patients and are particularly rare in children. Among the pediatric population, they are usually diagnosed in the context of connective tissue disorders, genetic mutations, or vasculitis. The same is true of visceral arteries aneurysms. This case report describes the staged management of an 11-year-old patient presenting PIK3CA mutation and a 5.8 cm infrarenal AAA associated with bilateral common iliac arteries and multiple visceral aneurysms, the largest observed in the superior mesenteric artery (SMA = 3.2 cm). After careful evaluation, decision was made to first approach the most life-threatening lesion (the infrarenal AAA due to the large diameter) and the remaining aneurysms in secondary procedures, with special attention to the SMA aneurysm. The patient underwent a staged repair, with the first phase consisting of an aortobi-iliac graft with the distal anastomosis made at the left common iliac artery and right external iliac artery. The right hypogastric artery was ligated. The second procedure consisted of SMA aneurysm repair with a plication technique, as 7 branches were visualized coming off the aneurysm sac. Postoperative pathology analysis of the aortic and SMA aneurysms sac revealed vasculitis with a mixed inflammatory pattern and a COL3A1 gene heterozygote variant. He is currently in his 18th month after the last surgical intervention, receiving immunomodulatory therapy, with a planned follow-up by the interdisciplinary team to monitor the medications' side effects and the diameter of the remaining visceral aneurysms.

Silicone oil tamponade induced vasculitis- a rare manifestation of toxic posterior segment syndrome.

AIM: To report silicone oil tamponade induced vasculitis in the early post operative period - a rare manifestation of 'Toxic Posterior Segment Syndrome' after pars plana vitrectomy for rhegmatogenous retinal detachment. CASE DESCRIPTION: A 50-year-old gentleman presented with vasculitis after a pars plana vitrectomy with silicone oil tamponade on the first post-operative day. He was started on oral steroids (1 mg/ kg) tapered sequentially every week. All signs of vasculitis resolved over a period of one month after which the silicone oil was removed. The patient maintained a visual acuity of 20/90 even after 3 months. There was no recurrence of vasculitis observed thereafter. CONCLUSION: Silicone oil induced vasculitis is a rarely described entity, especially as a manifestation of toxic posterior segment syndrome. This differential must be kept in mind even on the first post-operative day in cases where silicone oil has been used for tamponade.

Clinical study of profile of retinal detachment in vasculitis and its management at tertiary eye care hospital.

Purpose: The aim of the study was to understand the clinical profile and anatomical and visual outcome in Tractional/Combined (Tractional + Rhegmatogenous) Retinal Detachment due to vasculitis after surgical intervention. Methods: It was an interventional retrospective study of all cases who underwent surgery for RD with vasculitis at a single tertiary eye care center for over a period of 6 years. Patients with retinal detachment due to vasculitis were included in the study. All patients underwent the following surgical procedures: 240 belt buckle with three-port pars plana vitrectomy with membrane dissection and peeling with fluid gas exchange/with endolaser with silicon oil/C3 F8 gas injection. Results: In our study, 83.33% had preoperative vision of less than 6/60, whereas postoperatively 66.66% had vision of less than 6/60. Postoperatively 33.33% patients had vision better than 6/36. Of the six eyes operated for vasculitis with RD, retina was attached in five eyes following surgery. One patient had recurrent retinal detachment due to extensive proliferative vitreoretinopathy changes, and he was advised re-procedure but was lost for follow-up. The anatomical success was 83.33% on the first surgery. Conclusion: The overall anatomic success rate of retina reattachment surgery in vasculitis patient was good, and the visual outcome following the surgery can improve in majority of the cases. Hence, timely intervention is advocated.

[Our Experience with ICON Hip Resurfacing System]. / Nase zkusenosti s povrchovou náhradou kycelního kloubu ICON.

PURPOSE OF THE STUDY Our study aimed to assess the mid-term outcomes and complications with the ICON hip resurfacing system and to carry out a detailed analysis of pitfalls and risks associated with pairing the bearing surfaces of metal-on-metal hip implants. MATERIAL AND METHODS A total of 52 hip joints were assessed in 43 patients who received the ICON hip resurfacing system at our department between 2009 and 2013. The cohort included 34 men and 9 women. Their age at the time of primary surgery ranged from 34 to 67 years, with the mean age of 51.8 years. The mean follow-up was 7.6 years. The evaluation focused on the position and osseointegration of both components, bone remodelling around the implant, and signs of potential aseptic loosening. The functional status of the hip was assessed by Harris Hip Score. RESULTS The primary stability of both components was always good, there were no femoral neck fractures reported in our cohort. All the acetabular components were stable, showing appropriate osseointegration, with no radiolucent zones or signs of osteolysis around them. There was not a single case of the femoral component stem being in a biomechanically disadvantageous varus position. In zone I and III according to Beaulé, cancellous bone osteolysis developed in two patients. The narrowing of the femoral neck below the end of the femoral component, compared to postoperative X-rays, achieved the mean value of 1.3% according to Grammatopolouse. The HHS increased from 64 to 95.5 points. An excellent outcome was observed in 48 joints, whereas the outcome of the remaining 4 joints was very good. The mean survival rate of the resurfacing hip implant calculated using the Kaplan-Meier analysis achieved 100%. The cobalt and chromium levels in the blood of patients did not exceed the reference physiological value. DISCUSSION The resurfacing system enables to preserve the bone tissue of the metaphysis and a part of the femoral head. The reduced mechanical endurance of the peripheral part of femoral components smaller in size caused by implant malposition resulted in fatal consequences in the ASR system. Greater range of motion conditioned by the design of the resurfacing system led to a mechanical wear, with a significant increase in the concentration of metal particles in the effective joint space. The elevated levels of cobalt and chromium ions in some patients induced delayed-type hypersensitivity with subsequent development of aseptic lymphocyte-dominated vasculitis associated lesions presented as peri-acetabular changes (pseudotumors to osteolysis) with subsequent failure of implant fixation. We have not observed this complication in the ICON system as yet. In patients suffering from hip pain after the resurfacing hip arthroplasty and simultaneous high chromium and cobalt blood levels, pelvic CT/MRI is indicated with reduction of artefacts around the metal material. Surgical treatment of soft tissue affections, bone defects and reimplantation using conventional or revisioncementless components is a possible treatment option. CONCLUSIONS The ideal patient indicated for hip resurfacing is a physically active man under 60 years of age (with a femoral head size of 54-60 mm), with primary or secondary osteoarthritis, no joint deformity, with a good quality bone tissue in the femoral neck and head region. As for the functional performance, the resurfacing system allows the patients a large range of motion with very good joint stability immediately after surgery. Despite that, the metal-on-metal tribological pairing must be approached with caution. The risk of developing lesions associated with ALVAL is unpredictable. In our cohort of patients with ICON hip resurfacing system, mostly excellent outcomes with minimum complications were reported provided the indication criteria and the correct surgical procedure had been complied with. Key words: hip resurfacing system, metal articulating surfaces, adverse reaction to metal particles, aseptic lymphocytedominated vasculitis associated lesions, pseudotumor.

Multimodal Approach of Isolated Pulmonary Vasculitis: A Single-Institution Experience.

BACKGROUND: Isolated pulmonary vasculitis (IPV) is a single-organ vasculitis of unknown etiology and may mimic chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to review our clinical experience with pulmonary endarterectomy in patients with CTEPH secondary to IPV. METHODS: Data were collected prospectively for consecutive patients who underwent pulmonary endarterectomy and had a diagnosis of IPV at or after surgery. RESULTS: We identified 9 patients (6 women; median age, 48 years [range, 23-55]) with IPV. The diagnosis was confirmed after histopathologic examination of all surgical materials. The mean duration of disease before surgery was 88.0 ± 70.2 months. Exercise-induced dyspnea was the presenting symptom in all patients. Pulmonary endarterectomy was bilateral in 6 patients and unilateral in 3. No deaths occurred; however 1 patient had pulmonary artery stenosis, and stent implantation was performed. All patients received immunosuppressive therapies after surgery. Mean pulmonary artery pressure decreased significantly from 30 mm Hg (range, 19-67) to 21 mm Hg (range, 15-49) after surgery (P < .05). Pulmonary vascular resistance also improved significantly from 270 dyn/s/cm-5 (range, 160-1600) to 153 dyn/s/cm-5 (range, 94-548; P < .05). After a median follow-up of 41 months, all but 1 patient had improved to the New York Heart Association functional class I. CONCLUSIONS: IPV can mimic CTEPH, and these patients can be diagnosed with pulmonary endarterectomy. Furthermore surgery has not only diagnostic but also therapeutic value for IPV when stenotic and/or thrombotic lesions are surgically accessible. A multidisciplinary experienced CTEPH team is critical for management of these unique patients.

Cutaneous foreign body microemboli-induced occlusive vasculopathy: A complication of illicit intravenous injection of oral opioid tablets.

Occlusive nonvasculitic vasculopathy is a process characterized clinically by retiform purpura and potential ulceration and necrosis of affected areas, secondary to blockage of small vessels without associated inflammatory vasculitis. Intravascular injection of foreign material is known to cause distal ischemia and necrosis due to thrombosis, local vasoconstriction, or microemboli formation. A 27-year-old male presented with retiform purpura and worsening distal fingertip necrosis of the right upper extremity accompanied by suspicious intravascular polarizable foreign material identified on skin, muscle, and vascular biopsies. We report a case that highlights concerning complications and dermatopathologic findings of intravascular injection of oral opioid tablets.

Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea.

Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3-17.5), 7.7 (3.5-17.6), and 7.0 (1.6-16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud's phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2-32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

Surgical management of peripheral pulmonary artery pseudoaneurysm.

Pulmonary artery pseudoaneurysm is rare and may cause life-threatening haemoptysis. Coil or stent is the main treatment option. We present a case of intrapulmonary pseudoaneurysm located in the fissure between left upper and lower lobes surgically repaired due to the location and the broad base of the pseudoaneurysm.

A rare case of laparoscopy towards SLE with lupus mesenteric vasculitis induced ascites.

BACKGROUND: Diagnosis and management of acute abdomen secondary to systematic lupus erythematosus (SLE) has always been a clinical challenge. CASE PRESENTATION: A 21-year-old lady, with BMI 17.7, presented to our department with acute abdomen. Laparoscopy was carried out to exclude surgical emergency when conservative regimen failed. The patient revealed a history of purpuric changes and lupus test was positive for SLE. CONCLUSION: Based on our experience, early laparoscopy to alleviate acute abdomen has shown to improve the prognosis of the patient.

Contamination of revision procedures in patients with adverse tissues reaction to metal on metal implant.

OBJECTIVE: The aim of this study is to evaluate the incidence of infections in MoM total hip replacement revisions and to propose a therapeutic algorithm that can reduce the onset of this complication. Total hip arthroplasty is one of the most successful procedures performed annually in the world. As the population ages, the number of primary arthroplasty procedures performed each year is rising in conjunction with an increasing revision burden. Metal on Metal (MoM) total hip arthroplasties were reintroduced in over the last fifteen years to meet these needs, larger diameters, improved lubrication, better stability, increased ROM and wear properties of the bearing couple. These advantageous features have led to an exponential diffusion of MoM. Since over last decade, it has become evident that hip replacements with MoM bearing have significantly higher revision rates compared to those with Metal on Polyethylene. The common pathway for this failure mode appears to be increased wear or corrosion with excessive release of metal ions and nanoparticles. Complications such as elevated serum metal ion levels, aseptic lymphocyte-dominated vasculitis-associated lesion (ALVAL) and pseudotumours have all been well documented, but recent studies suggest increased risk of infection with MoM bearing surfaces. PATIENTS AND METHODS: We collect data from a cohort of 44 patients who underwent revision of total hip arthroplasty between 2014 and 2017 for the complication of MoM bearing. Studied by radiological images, blood tests, and intraoperative clinical status, part of the population was treated with one stage revision, while the other was treated with a two-stage revision. RESULTS: Results showed a difference in the occurrence of infections in the two populations. CONCLUSIONS: We consider it appropriate to perform two-stage revision in all case of failure of MoM replacement so as to allow to minimize the likelihood of infection in patients with damaged tissues by ALVAL, pseudotumour, and necrosis that could create an ideal environment for bacterial development.

The Usefulness of Video Capsule Endoscopy in Evaluating Gastrointestinal Manifestations of Immunoglobulin A Vasculitis.

Objective Immunoglobulin (Ig) A vasculitis (IgAV) is a systemic vasculitis characterized by purpura and gastrointestinal involvement showing abdominal pain that usually occurs after the emergence of purpura. Criteria for evaluating gastrointestinal manifestations of IgAV are unavailable at present, so we conducted a study to investigate the usefulness of video capsule endoscopy (VCE) for detecting gastrointestinal manifestations of IgAV. Methods The clinical data of 10 patients who underwent VCE for IgAV at our hospital from 2012 to 2017 were collected. Nine patients underwent esophagogastroduodenoscopy, and five underwent colonoscopy. We compared the endoscopic severity determined by VCE to the findings of esophagogastroduodenoscopy and colonoscopy. Results The rates of positive findings of esophagogastroduodenoscopy, VCE, and colonoscopy were 56%, 100%, and 80%, respectively. In 70% of cases, the most severe gastrointestinal findings were detected by VCE. VCE was performed on average 16.8 days after the emergence of purpura, and in 8 of 10 patients, its result triggered the initiation of steroid therapy or the control of the steroid dose. Conclusion VCE is very useful for confirming gastrointestinal involvement in IgAV and may be used to determine the timing of steroid therapy initiation. We recommend performing VCE when IgAV is suspected in patients with gastrointestinal symptoms.

Five Hundred Fifty-five Retrieved Metal-on-metal Hip Replacements of a Single Design Show a Wide Range of Wear, Surface Features, and Histopathologic Reactions.

BACKGROUND: In 2010, a widely used metal-on-metal hip implant design was voluntarily recalled by the manufacturer because of higher than anticipated failure rates at 5 years. Although there was a large published range of revision rates, numerous studies had reported a higher risk of revision for excessive wear and associated adverse tissue reactions when compared with other metal-on-metal total hips. The reasons for this were suggested by some to be related to cup design features. QUESTIONS/PURPOSES: From retrievals of ASR metal-on-metal implants and tissue samples obtained at revision surgery, we asked the following questions: (1) What were the common and uncommon surface features? (2) What were the common and uncommon linear and volumetric wear characteristics? (3) Were there common taper corrosion characteristics? (4) What aseptic lymphocytic vasculitis-associated lesion (ALVAL) features were present in the tissues? METHODS: Five hundred fifty-five ASRs, including 23 resurfacings, were studied at one academic research center. Features of wear (eg, light and moderate scratching), damage (eg, deposits, gouges), and bone attachment on the porous coating were semiquantitatively ranked from 0 (none) to 3 (> 75%) based on the amount of a feature in each region of interest by the same experienced observer throughout the study. Visible features of head taper corrosion were ranked (Goldberg score) from 1 (none) to 4 (severe) by the same observer using a previously published scoring method. An experienced tribologist measured component wear depth using a coordinate measuring machine and quantified wear volume using previously validated methods. All available tissues were sampled and examined for features of ALVAL and scored from 0 to 10 by a single observer using a method they previously developed and published. A score from 0 to 4 is considered low, 5 to 8 is considered moderate, and 9 or 10 is considered high with regard to the risk of metal hypersensitivity features in the tissues. RESULTS: The most common bearing surface features were light and moderate scratches and removal or postremoval damage. Discoloration and deposits were commonly observed on femoral heads (55% [305 of 553]) and less commonly on cups (30% [165 of 546]). There was no evidence of impingement or dislocation damage. There was typically a small amount of bone attachment in at least one of eight designated regions of interest (84% [460 of 546]); extensive or no bone attachment was uncommon. Edge wear was highly prevalent. The maximum wear of 469 cups (88%) occurred near the edge, whereas the maximum wear of 508 femoral heads (94%) occurred between the pole and 45° from the pole. The median combined head-cup wear volume was 14 mm (range, 1-636 mm). One hundred sixty-nine pairs (32%) had a combined wear volume of < 10 mm, 42 pairs (8%) had volumetric wear of > 100 mm, and 319 pairs (60%) had wear volume between 10 and 100 mm³. Seventy-four percent of tapers (390 of 530) received a Goldberg score of 4, 22% (116 of 530) a score of 3, < 5% (24 of 530) a score of 2, and none received a score of 1. The most frequent ALVAL score was 5 out of 10 (35 of 144 hips [24%]) and ranged from 2 (one hip) to 10 (nine hips); 92 of 144 (64%) had a moderate score, 17 of 144 (12%) had a high score, and 35 (24%) had a low score. CONCLUSIONS: Although edge wear was prevalent, in most cases, this was not associated with high wear. The increased diameter and decreased coverage angle of the ASR design may have resulted in the observed high incidence of edge wear while perhaps decreasing the risk for impingement and dislocation. CLINICAL RELEVANCE: The role of bearing wear in the revisions of metal-on-metal implants is controversial, because it is known that there is a large range of in vivo wear rates even within the same implant type and that patient variability affects local tissue responses to wear debris. The observations from our study of 555 retrieved ASR implant sets indicate that there was a wide range of wear including a subset with very high wear. The results suggested that the failure of the ASR and ASR XL was multifactorial, and the failure of different subgroups such as those with low wear may be the result of mechanisms other than reaction to wear debris.

Outcome of Renal Transplant in Recipients With Vasculitis.

OBJECTIVES: End-stage renal disease develops in a high percentage of patients with vasculitis, in whom kidney transplant has become a therapeutic option. However, limited data are available on the prognosis and outcomes after kidney transplant in these patients. We aimed to compare the long-term graft survival and graft function in 8 renal transplant recipients with vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, Goodpasture syndrome, and Henoch-Schonlein purpura) with the other kidney recipients at a single center. MATERIALS AND METHODS: We conducted a retrospective study of patients followed for chronic renal failure associated with vasculitis before renal transplant. We excluded patients with no biopsy-proven nephropathy. RESULTS: There was no difference in the occurrence of metabolic and cardiovascular complications in our case group compared with the other graft recipients. Infections were frequent and included cytomegalovirus and urinary tract infection. The rates of bacterial and viral infection were equivalent in our population. The incidence of allograft loss was estimated at 1.8%, less than that seen in our entire transplant population. The presence of vasculitis was not significantly related to renal failure (P = .07). Extrarenal relapse occurred in 1 patient with microscopic polyangiitis. Antineutrophil cytoplasmic antibody levels in patients with granulomatosis with polyangiitis and microscopic polyangiitis did not seem to influence the renal outcome (P = .08). Circulating antineutrophil cytoplasmic antibodies were associated with the development of vascular lesions in the graft but were not significantly correlated with graft survival (P = .07). CONCLUSIONS: This study supports the theory that renal transplant is an effective treatment option for patients with end-stage renal disease secondary to vasculitis. These patients fare similarly to, if not better than, other patients.