Multicenter Series Research Articles

Crooke Cell Adenoma Confers Poorer Endocrinological Outcomes Compared with Corticotroph Adenoma: Results of a Multicenter, International Analysis

Crooke cell adenomas (CCAs) are a rare, aggressive subset of secretory pituitary corticotroph adenomas (sCTAs) found in 5%-10% of patients with Cushing disease. Multiple studies support worse outcomes in CCAs but are limited by small sample size and single-institution databases. We compared outcomes in CCA and sCTA using a multicenter, international retrospective database of high-volume skull base centers. Patients surgically treated for pituitary adenoma from January 2017 through December 2020 were included. Among 2826 patients from 12 international centers, 20 patients with CCA and 480 patients with sCTA were identified. No difference in baseline demographics, tumor characteristics, or postoperative complications was seen. Microsurgical approaches (60% CCA vs. 62.3% sCTA) were most common. Gross total resection was higher in CCA patients (100% vs. 83%, P= 0.05). Among patients with gross total resection according to intraoperative findings, fewer CCA patients had postoperative hormone normalization of pituitary function (50% vs. 77.8%, P < 0.01) and remission of hypersecretion by 3-6 months (75% vs. 84.3%, P < 0.01). This was the case despite CCA having better local control rates (100% vs. 96%, P < 0.01) and fewer patients with remnant on magnetic resonance imaging (0% vs. 7.2%, P < 0.01). A systematic literature review of 35 studies reporting on various treatment strategies reiterated the high rate of residual tumor, persistent hypercortisolism, and tumor-related mortality in CCA patients. This modern, multicenter series of patients with CCA reflects their poor prognosis and reduced postsurgical hormonal normalization. Further work is necessary to better understand the pathophysiology of CCA to devise more targeted treatment approaches.

Multicentric case series of scuba diving fatalities: The role of intracardiac gaseous carbon dioxide in the forensic diagnosis

Scuba diving fatalities post-mortem diagnosis presents a higher level of forensic complexity because of their occurrence in a non-natural human life environment. Scuba divers are equipped with diving gas to breathe underwater. It is essential for them to be fully trained in order to be able to manage their dive safely despite the varying increase of ambient pressure and temperature decrease. Throughout the dive, the inhaled diving gas is dissolved in the diver’s tissues during the descent and if the decompression steps are not respected during the ascent, the balance between the dissolved gas and the tissues (including blood) is disrupted, leading to a gaseous release in the organism. Depending on the magnitude of this gaseous release, free gas can occur in blood and tissue. Venous or arterial gas embolism can also occur as a consequence of decompression sickness or barotraumatism. It can also induce drowsiness that consequently leads to drowning. As a result, the occurrence of gas in dead scuba divers is very complex to interpret, as is the difficulty to distinguish it from resuscitation maneuver artifacts or body decomposition. Although the literature is scarce in this domain, significant work has been done to provide a precise intracadaveric gas sampling method to enlighten the cause and circumstances of death during the dive. The aim of this study is to obtain higher statistical significance by collecting a number of cases to confirm the gas sampling protocol and analysis and gain more information about the cause of death and the events surrounding the fatality through the establishment of clear management guidelines.

What happens to endoscopic/arthroscopic tenotomies with iliopsoas impingement in the medium term? Review of a prospective cohort of 64 patients with a minimum follow-up of 5 years

IntroductionThe occurrence of iliopsoas impingement (IPI) after total hip arthroplasty (THA) is a proven risk factor for negative outcomes. Endoscopic or arthroscopic tenotomies of the iliopsoas offer a surgical solution with short-term results that have already been validated in prospective multicenter series. We carried out a review of the patients at more than 5 years of follow-up in order to assess the stability of the results over time. HypothesisOur main hypothesis was that endoscopic/arthroscopic tenotomies allow stable medium-term resolution of the painful symptoms of IPI. Our secondary hypothesis was that medium-term survival was satisfactory. Material and methodThis study is a continuation of a multicenter prospective series. Patients were contacted through multiple channels in order to: obtain an Oxford score, assess for satisfaction, psoas irritation, and daily pain on a visual analogue scale (VAS). ResultsOf 64 patients in the original study, 57 were contacted. The Oxford score at the last follow-up was 40.7±7.7 [12–48]. There was a significant difference between the Oxford scores preoperatively, at 8 months and at the last follow-up. The mean satisfaction out of 10 was 8.0±2.1 [1–10]. We found 84% satisfaction at 5 years against 83% at 8 months. The VAS was 2.1±2.3 [0–10]. A straight leg psoas sign was present in 19.6% (10/51) of patients at 5 years, compared to 15.6% (8/51) at 8 months. The sign disappeared in four cases, while it reappeared during the interval in six cases. Survival was 91.2% (95% CI: 80.2–96.3) at 5 years. ConclusionEndoscopic/arthroscopic iliopsoas tenotomies represent a permanent medium-term solution to treat IPI after THA. The existence of a force differential or an acetabular overhang does not seem, within a certain limit, to impact the results in the medium term. Level of evidenceIV; prospective series without control group.

Isolated tracheo-esophageal fistula in children: Analysis of a large multicentric series and proposal of a comprehensive treatment algorithm

BackgroundIsolated tracheo-esophageal fistulas (TEF) are rare and challenging, possibly leading to severe complications. We evaluated the outcomes in a large series of patients treated in 9 referral Centers for airway surgery, and suggested an ideal management algorithm. MethodsA multicentric retrospective study on TEF was performed. Results186 cases were collected. Among 101 patients with recurrent TEF after EA repair, 1 was treated successfully with thoracoscopy, 23 with open surgery with TEF division and tissue interposition, with 15 complete resolution (65 %), 2 TEF recurrence (9 %) and 6 other complications (26 %), and 77 received TCA endoscopic cauterization, with complete resolution in 61 (79 %). Among 54 patients with congenital H-type fistula, 1 received conservative treatment with resolution; 4 underwent thoracoscopy (50 % resolution); 6 had endoscopic cauterization (33 % resolution); 44 received open surgery, with 43 (98 %) successes. In the last group, the majority of TEF was ligated and divided with tissue interposition. Among 26 post-traumatic fistulas (mechanical trauma, battery or caustic ingestion), 6 patients received endoscopic procedure and 5 of them (83 %) reached complete resolution. The other 19 received open surgery (trans-tracheal direct FTE closure with tracheal resection), and 12 had complete resolution (63 %). Two patients eventually died and complication rate was 23 %. Other 5 cases (3 Bronchial-Esophageal Fistulas and 2 TEF after cleft repair) were treated endoscopically or with open approach. ConclusionEndoscopic cauterization can be the preferred treatment for recurrent TEF after EA, while for the congenital H-type and post-traumatic TEF the open approach remains our first choice.

Clinical outcomes of non-COVID-19 orthopaedic patients admitted during the COVID-19 pandemic: a multi-centre interrupted time series analysis across hospitals in six different countries

ObjectivesTo assess across seven hospitals from six different countries the extent to which the COVID-19 pandemic affected the volumes of orthopaedic hospital admissions and patient outcomes for non-COVID-19 patients admitted...

P-198 - Sustained complete remission in Sézary syndrome using extracorporeal photopheresis: a multicentric case series

P-198 - Sustained complete remission in Sézary syndrome using extracorporeal photopheresis: a multicentric case series

Cardiovascular risk factors in coeliac disease (ARCTIC): a protocol of multicentre series of studies

IntroductionThere is only limited and controversial information available on the cardiovascular (CV) risk in coeliac disease (CD). In this study, we plan to investigate the body composition and CV risk-related...

Predictors of mid-term functional outcomes for robot-assisted Madigan simple prostatectomy: results of a multicentric series according to the BPH-6 achievement.

BPH-6 achievement remains an objective far to be evaluated for every technique currently available for the surgical management of bladder outlet obstruction (BOO) with the goal of preserving ejaculatory function. The aim of this study was to evaluate predictors of BPH-6 achievement of urethral-sparing robot assisted simple prostatectomy (us-RASP) on a large series performed at two tertiary-care centers. Two institutional us-RASP datasets were merged, considering eligible all patients with a follow-up >12 months. Baseline, perioperative and functional data according to BPH-6 endpoint were assessed. Descriptive analysis was used. Frequencies and proportions were reported for categorical variables while medians and interquartile ranges (IQRs) were reported for continuously coded variables. A logistic regression model was built to identify predictors of BPH-6 achievement. For all statistical analyses, a two-sided P<0.05 was considered significant. Study cohort consisted of 94 eligible patients. The median follow-up was 40.7 months (IQR 31.3-54.2). Overall BPH-6 achievement was 54.7%. Compared to baseline, reduction of ≥30% in IPSS was observed in 93.6% of patients, reduction of <6 points for SHIM in 95.7% and response to MSHQ-EjD question 3 indicating emission of semen in 72.6%, respectively. On multivariable analysis, prostate volume between 110-180 mL (OR 0.09; 95% CI 0.01-0.92; P=0.043) and higher preoperative SHIM score (OR 1.18; 95% CI 1.05-1.32; P<0.01) were independent predictors of BPH-6 metric achievement. us-RASP may provide a complete resolution of BOO and preservation of ejaculatory function in sexually active men with a prostate volume ranging 110-180 mL.

Retrospective study on the association of human herpesvirus reactivation with severe DRESS: A description of blood and skin reactivations.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a severe adverse event (mortality of 10%). Its pathophysiology involves herpesviruses, particularly HHV-6, but the exact mechanisms are still poorly understood. To describe severe cases of DRESS and especially their association with herpesvirus reactivation. This study was a multicentre case series conducted between 2007 and 2021 at five University Hospital Centres in France. The study included patients who had severe DRESS, which was defined as death, transfer to the intensive care unit (ICU), or severe damage to internal organs. We excluded patients without blood PCR sample, without a drug formally attributed or with RegiSCAR score < 6. We collected data on severity, causative drug, associated visceral damage and results of viral blood PCRs. HHV-6 reactivation was studied in skin biopsies by detection of small non-coding transcripts (HHV-6 miR-aU14) and a late viral protein (GP82/105). Fifty-two patients were included (29 female, median age 62, interquartile range (IQR) [37;72]). Eight patients (15%) died, 13 (27%) were admitted to ICU. Most patients (n = 34; 65%) had multisystem involvement: most frequent was liver (n = 46; 88%), then renal failure (n = 24; 46%). Forty patients (77%) had at least one blood viral reactivation among HHV-6, EBV or CMV, of which 21 (53%) had at least two. Median time of blood HHV-6 reactivation was 24 days (IQR [20;35]). HHV-6 reactivation was demonstrated in 15 out of 20 skin biopsies, with a median time of 11 days [9;17]. We confirmed the high rate of HHV-6 reactivation in severe DRESS and demonstrated cutaneous HHV-6 reactivation using small non-coding transcripts (HHV-6 miR-aU14), which preceded viral PCR positivity in blood. These results suggest that HHV-6 reactivation during DRESS may start in skin. Furthermore, search for miR-aU14 in skin biopsy could become a useful diagnostic tool for early detection of HHV-6 reactivation.

Cenobamate and Clobazam Combination as Personalized Medicine in Autoimmune-Associated Epilepsy With Anti-Gad65 Antibodies.

Autoimmune-associated epilepsy (AAE) with antiglutamic acid decarboxylase 65 (GAD65) antibodies is considered a T-cell-mediated encephalitis that evolves to drug-resistant epilepsy. We do not have an effective therapeutic strategy for these patients. Because the GAD enzyme is primarily responsible for the conversion of glutamate to GABA, the mechanism of epileptogenesis in this condition predicts decreased levels of GABA content in synaptic vesicles. Cenobamate (CNB) acts as a positive allosteric modulator at synaptic and extra synaptic GABAA receptors, producing increased inhibitory neurotransmission in the brain. This mechanism could be especially beneficial in AAE with anti-GAD65 antibodies because it would be able to correct the imbalance due to the GABAergic stimulation deficit in postsynaptic neurons. We recruit a retrospective multicentric consecutive case series of AAE with anti-GAD65 antibodies from 5 epilepsy units in Spain who have received treatment with CNB. A total of 8 patients were recruited. This cohort of highly refractory patients have failed a mean of 9.50 (SD = 3.20) ASM without control of seizures for sustained periods of time. The average number of seizures per month during the previous 3 months before CNB treatment was 19.63 (SD = 17.03). After the introduction of CNB improvement was achieved in all our patients, with a median reduction in the number of seizures of 92.22% (interquartile range [IQR]: 57.25-98.75). The mean follow-up was 156.75 days (SD = 68.23). In patients with concomitant treatment with clobazam (CLB), the median percentage of seizure reduction was higher than those not taking CLB: 94.72% (IQR: 87.25-100) vs 41.50% (p = 0.044) and also higher than the control group of patients with refractory epilepsy not related to anti-GAD65 treated with the same combination: 94.72% (IQR: 87.25-100) vs 45.00% (IQR: 25.00-87.00) (p = 0.019). Treatment with the combination CNB + CLB could be a type of personalized medicine in patients with AAE with anti-GAD65. Our preliminary data will need to be endorsed with new prospective and controlled studies.

DEBRIDEMENT, ANTIBIOTICS, AND IMPLANT RETENTION IS SUCCESSFUL IN THE MANAGEMENT OF ACUTELY INFECTED UNICOMPARTMENTAL KNEE ARTHROPLASTY: A CASE SERIES

AbstractBackgroundInfections are rare and poorly studied complications of unicompartmental knee athroplasty (UKA) surgery. They are significantly less common compared to infections after total knee arthroplasties (TKAs). Optimal management of periprosthetic joint infections (PJIs) after a UKA is not clearly defined in the literature. We present the results of a multicentre retrospective series of UKA PJIs treated with Debridement, Antibiotics and Implant Retention (DAIR).MethodologyPatients presenting between January 2016 and December 2019 with early UKA infection were identified at three specialist centres using the Musculoskeletal Infection Society (MSIS) criteria. All patients underwent a standardized treatment protocol consisting of the DAIR procedure and antibiotic therapy comprising two weeks of intravenous (IV) antibiotics followed by six weeks of oral therapy. The main outcome measure was overall survivorship free from reoperation for infection.ResultsA total of 3225 UKAs (2793 (86.2%) medial and 432 (13.8%) lateral UKAs) were performed between January 2016 and December 2019. Nineteen patients had early infections necessitating DAIR. The mean follow-up period was 32.5 months. DAIR showed an overall survivorship free from septic reoperation of 84.2%, with an overall survivorship free from all-cause reoperation of 78.95%. The most common bacteria were Coagulase-negative Staphylococci, Staphylococcus aureus and Group B Streptococci. Three patients required a second DAIR procedure but remained free from re-infection at follow-up obviating the need for more demanding, staged revision surgery.ConclusionsIn infected UKAs, the DAIR procedure produces a high rate of success, with a high survivorship of the implant.

Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series

IntroductionThere are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly. Study ObjectiveTo describe another variant of UD and compare the presentation and management across different institutions MethodsThis was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used. ResultsFive patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain. ConclusionDiagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.

Diagnostic Accuracy and Safety of Endoscopic Ultrasound-Guided End-cutting Fine-needle Biopsy needles for tissue sampling of Abdominal and Mediastinal Lymphadenopathies: a prospective multicenter series.

The role of the newer EUS-fine needle biopsy (FNB) needles in lymphadenopathies (LA) is still under evaluation. We aimed to evaluate the diagnostic accuracy and the adverse event rate of EUS-FNB in diagnosing LA. From June 2015 to 2022, all patients referred to 4 institutions for EUS-FNB of mediastinal and abdominal LA were enrolled. 22G Franseen tip or 25G Fork tip needles were used. The gold standard for positive results was surgery or imaging and clinical evolution over a follow-up of at least one year. A total of 100 consecutive patients were enrolled, consisting of those with a new diagnosis of LA (40%), presence of LA with a previous history of neoplasia (51%), or suspected lymphoproliferative disease (9%). EUS-FNB was technically feasible in all LA patients with 2 to 3 passes (mean 2.62±0.93). The overall EUS-FNB sensitivity, positive predictive value (PPV), specificity, negative predictive value (NPV), and accuracy were 96.20%, 100%, 100%, 87.50%, and 97.00%, respectively. Histological analysis was feasible in 89% of cases. Cytological evaluation was performed in 67% of specimens. There was no statistical difference between the accuracy of the 22G or 25G needle (p=0.63). A sub-analysis on lymphoproliferative disease revealed a sensitivity and accuracy of 89.29% and 90.0%. No complications were recorded. EUS-FNB with new end-cutting needles is a valuable and safe method to diagnose LA. The high quality of histological cores and the good amount of tissue allowed a complete immunohistochemical analysis of metastatic LA and precise subtyping of the lymphomas.

Endoscopic ultrasound-guided drainage of pancreatic collections with dedicated metal stents: A nationwide, multicenter, propensity score-matched comparison

BackgroundThe new dedicated stents for endoscopic ultrasound (EUS)-guided transluminal drainage of peri‑pancreatic fluid collections (PFCs) demonstrated optimal efficacy and safety profiles. AimsThis study aimed to evaluate the safety, technical and clinical success, and recurrence rate of PFCs drained with Lumen Apposing Metal Stent (LAMS) or Bi-Flanged Metal Stent (BFMS). MethodsData from a multicenter series of PFCs treated with LAMS or BFMS at 30 Italian centers during a 5-year period were retrieved. The rate of adverse events (AEs), technical success, clinical success, PFC recurrence were evaluated. To overcome biases, a 1-to-1 match was created using propensity score analysis. ResultsOut of 476 patients, 386 were treated with LAMS and 90 with BFMS, with a median follow-up of 290 days (95% CI 244 to 361). Using propensity score matching, 84 patients were assigned to each group. The incidence of AEs did not differ between the two stents (13.1% versus 15.5%, p = 0.29), mainly bleeding or recurrence rate (4.7% versus 3.5%, p = 1). Technical and clinical success in the BFMS and LAMS groups were 92% versus 95% (p = 0.36) and 91% versus 94% (p = 0.64), respectively. ConclusionOur study demonstrates that LAMS and BFMS have comparable safety profiles with similar technical and clinical success rates for EUS-guided PFC drainage.

Radical cystectomy in bladder cancer patients previously treated for prostate cancer: Insights from a large European multicentric series

IntroductionPrevious radical prostatectomy (RP) for prostate cancer (PCa) might impair feasibility of radical cystectomy (RC) for bladder cancer (BCa). The current study addressed morbidity, operative time (OT), and length of stay (LOS) of RC, within the largest available series of patients with history of previous RP. Materials and methodsAll patients previously submitted to RP for PCa and subsequently submitted to RC for BCa, at six high-volume European institutions between 2010 and 2019, were identified. Presence of either PCa or BCa metastases, RT as primary treatment for PCa, and palliative RC represented exclusion criteria.The quality criteria for accurate and comprehensive reporting of intra- and post-operative surgical outcomes, recommended by the European Association of Urology guidelines, were fulfilled. Multivariable logistic and Poisson regression analyses were performed. ResultsOverall, 140 RC patients with history of RP were identified. After RP, 69 (49%) patients received radiotherapy (RT) for PCa, either in adjuvant (n = 50, 36%) or salvage setting (n = 19, 13%). Median age-adjusted Charlson comorbidity index was 6 (IQR 5, 7). Median OT, estimated blood loss and LOS were, respectively, 300 min, 500 ml, and 16 days. Intra-operative transfusions rate was 47% (n = 65). One intra-operative complication occurred (EAUiaiC grade 2, perforation of the rectum managed with immediate repair). Eighty-two (59%) patients experienced a total of 107 post-operative complications during the hospital stay, and seven (5%) patients required hospital readmission. In multivariable regression analyses, RT for PCa was associated with higher risk of post-operative complications (odds ratio 1.82, p = 0.039), longer OT (incidence rate ratio 1.09, p < 0.001), and longer LOS (incidence rate ratio 1.24, p < 0.001). ConclusionsRC in patients with history of RP is feasible, albeit burdened by remarkable morbidity, even in centers of excellence. RT after RP for PCa portends worse surgical outcomes.

Not All Proximal Humerus Fractures Do Well Without Surgery: Anterior Translation Predicts the Need for Surgery.

To evaluate the effect of translation on a large series of low energy proximal humerus fractures initially treated nonoperatively. Retrospective multicenter analysis. 5 level-one trauma centers. 210 Patients (152F;58M) average age 64 with 112 left and 98 right sided low energy proximal humerus fractures (OTA/AO 11-A-C). All patients were initially treated nonoperatively and were followed for an average 231 days. Radiographic translation in the sagittal and coronal planes was measured. Patients with anterior translation were compared to those with posterior or no translation. Patients with ≥80% anterior humeral translation were compared to those with < 80% anterior translation, including those with no or posterior translation. The primary outcome was failure of nonoperative treatment resulting in surgery and the secondary outcome was symptomatic malunion. Nine patients (4%) had surgery, 8 for nonunion and 1 for malunion. All 9 patients (100%) had anterior translation. Anterior translation as compared with posterior or no sagittal plane translation was associated with failure of nonoperative management requiring surgery (p = 0.012). Additionally, of those with anterior translation, having ≥80% anterior translation as compared with < 80% was also associated with surgery (p = 0.001). Finally, 26 patients were diagnosed with symptomatic malunion, of whom translation was anterior in 24 and posterior in 2 (p = 0.0001). In a multicenter series of proximal humerus fractures anterior translation of >80% was associated with failure of nonoperative care resulting in non-union, symptomatic malunion, and potential surgery.

Proficiency score as a predictor of early trifecta achievement during the learning curve of robot-assisted radical prostatectomy for high-risk prostate cancer: Results of a multicentric series.

Recently, an innovative tool called "proficiency score" was introduced to assess the learning curve for robot-assisted radical prostatectomy (RARP). However, the initial study only focused on patients with low-risk prostate cancer for whom pelvic lymph node dissection (PLND) was not required. To address this issue, we aimed to validate proficiency scores of a contemporary multicenter cohort of patients with high-risk prostate cancer treated with RARP plus extended PLND by trainee surgeons. Between 2010 and 2020, 4 Italian institutional prostate-cancer datasets were merged and queried for "RARP" and "high-risk prostate cancer." High-risk prostate cancer was defined according to the most recent European Association of Urology guidelines as follows: prostate-specific antigen >20 ng/mL, International Society of Urological Pathology ≥4, and/or clinical stage (cT) ≥ 2c on preoperative imaging. The selected cohort (n = 144) included clinical cases performed by trainee surgeons (n = 4) after completing their RARP learning curve (50 procedures for low-risk prostate cancer). The outcome of interest, the proficiency score, was defined as the coexistence of all the following criteria: a comparable operation time to the interquartile range of the mentor surgeon at each center, absence of any significant perioperative complications Clavien-Dindo Grade 3-5, no perioperative blood transfusions, and negative surgical margins. A logistic binary regression model was built to identify the predictors of 1-year trifecta achievement in the trainee cohort. For all statistical analyses, a 2-sided p < 0.05 was considered significant. A proficiency score was achieved in 42.3% patients. At univariable level, proficiency score was associated with 1-year trifecta achievement (odds ratio, 8.77; 95% confidence interval, 2.42-31.7; p = 0.001). After multivariable adjustments for age, nerve-sparing, and surgical technique, the proficiency score independently predicted 1-year trifecta achievement (odds ratio, 9.58; 95% confidence interval, 1.83-50.1; p = 0.007). Our findings support the use of proficiency scores in patients and require extended PLND in addition to RARP.

CPC14 A truly VEXing diagnosis

Abstract A 50-year-old man with a background of prostate cancer was admitted with fever, weight loss, polyarthritis, symptomatic anaemia and raised inflammatory markers. Multiple tender indurated violaceous plaques on the trunk and limbs were suggestive of Sweet syndrome. Skin biopsies revealed a neutrophilic dermal infiltrate and a pandermal vasculitis affecting small vessels. Laboratory screening (including rheumatoid factor, anticyclic citrullinated peptide, antinuclear antibody, antineutrophil cytoplasm antibodies, HIV, hepatitis B and C serology, antiphospholipid antibodies and cryoglobulins) was negative. Upper and lower endoscopies and gastrointestinal biopsies were normal. Biopsy of an axillary lymph node revealed reactive changes only. Computed tomography (CT) of the thorax, abdomen and pelvis showed sacroiliitis. A diffuse low signal on T1-weighted magnetic resonance imaging of his spine was suggestive of conversion of fatty marrow to red marrow. His cutaneous findings resolved fully with oral prednisolone 0.5 mg kg−1; however, he had ongoing transfusion-dependent anaemia and arthritis. Infliximab was started for presumed ankylosing spondylitis by rheumatology without improvement. Six months later, he was readmitted with multifocal ulceration of both feet. Histology demonstrated a leucocytoclastic vasculitis. He developed haemoptysis and chest pain. CT of the thorax showed multifocal, bilateral, ground–glass change consistent with pulmonary vasculitis. Bone marrow biopsy revealed hypercellular marrow with markedly increased granulopoiesis. A pathogenic missense mutation in UBA1 (c121A.G; pMet41Val) was found on next-generation sequencing of the bone marrow aspirate (Leeds Cancer Centre). Somatic variants at this codon have been implicated in vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic (VEXAS) syndrome (Beck DB, Ferrada MA, Sikora KA et al. Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease. N Engl J Med 2020; 383:2628–38). First described in 2020, VEXAS syndrome is a somatic monogenic autoinflammatory disorder of adulthood. Affected patients develop predominantly inflammatory and haematological symptoms. Cutaneous findings have been reported in up to 89% of cases (Georgin-Lavialle S, Terrier B, Guedon AF et al. Further characterization of clinical and laboratory features in VEXAS syndrome: large-scale analysis of a multicentre case series of 116 French patients. Br J Dermatol 2022; 186:564–74) and include neutrophilic dermatosis, vasculitis, urticaria, angioedema, erythematous papules and chondritis. Systemic manifestations are protean, but fever of noninfectious origin, weight loss, and ocular and pulmonary manifestations are most prevalent. Fifty per cent of patients with VEXAS have myelodysplastic syndrome (Georgin-Lavialle et al.). In our case, persistent haematological abnormalities coupled with multisystem inflammation (arthritis, vasculitis) prompted testing for VEXAS syndrome. This novel condition should be considered when assessing adult patients with neutrophilic dermatosis or cutaneous vasculitis who manifest other autoinflammatory phenomena. He was commenced on tocilizumab but, despite this, has ongoing acral vasculopathy.

Where to Draw the Line in Ex-Situ Split Liver Transplantation for Adult Recipients? – A Benchmarking Analysis

Abstract Background A comprehensive outcome analysis of ex situ right split liver graft transplantation (RSLT) is currently lacking. Aims Benchmarking outcomes of RSLT against best achievable results from whole LT offers a novel comparative approach. Methods This retrospective study includes all consecutive ex-situ RSLT for adult recipients performed from 2014-2019 at 4 high volume transplant centers (&amp;gt;70 LT/year). Outcomes including overall morbidity expressed by the comprehensive complication index (CCI) and graft related complications were compared to the published benchmark cutoffs for whole graft LT. Results We included 129 ex-situ RSLT with 76% H45678 (n=98) and 24% (n=31) H5678 grafts. The median follow-up was 4.8 years. Overall and severe 1-year morbidity expressed by the CCI were within established whole graft LT benchmarks. Biliary complication rates were slightly higher than the benchmark due to the occurrence of cut-surface leaks. The arterial thrombosis rate was outside the benchmark cutoff (7% vs ≤4.4%) leading to a higher retransplantation rate. However, overall 1-year graft and recipient mortality were well within the benchmark cutoffs, resulting in a 5-year overall recipient survival of 88.5%. Selecting benchmark cases for RSLT defined by low-risk recipient-donor criteria including static cold storage time ≤10h and recipient MELD≤20 points, did not significantly reduce overall morbidity nor arterial complication rate. In contrast, the type of split graft (H45678 vs.H5678) did impact on both arterial and biliary complications. Conclusions This large multicenter series of ex-situ right split grafts disclosed an overall 1-year morbi-mortality comparable to the best achievable results in whole graft LT. The results show that the main morbidity burden in adult RSLT is related to technical criteria rather than graft-recipient matching.

Calcium-stimulated calcitonin test for the diagnosis of medullary thyroid cancer: results of a multicenter study and comparison between different assays.

A basal serum calcitonin (Ct) increase >100 pg/mL in patients with a thyroid nodule is consistent with the diagnosis of medullary thyroid cancer (MTC). In cases where the CT test have a slight to moderate increase, the calcium gluconate stimulation test is helpful to increase diagnostic accuracy. However, reliable cut-offs for calcium-stimulated Ct are still lacking. The aim of this study was to evaluate the sex-specific calcium-stimulated Ct cutoffs for the diagnosis of MTC in a multicenter series. A comparison between different Ct assays has been also performed. 90 subjects undergone calcium-stimulated Ct for a suspected MTC in 5 Endocrine Units between 2010-2021 were retrospectively analyzed. Serum Ct concentrations were assessed by immunoradiometric (IRMA) or chemiluminescence (CLIA) assays. MTC was diagnosed in 37 (41.1%) and excluded in 53 (58.9%) patients. The best calcium-stimulated Ct cut-off to identify MTC was 611 pg/mL in males (AUC =0.90, 95% CI (0.76;1) and 445 pg/mL in females (AUC=0.79, 95% CI (0.66;0.91). Logistic regression analysis showed that both basal (OR 1.01, P=0.003) and peak Ct after stimulation (OR 1.07, P=0.007) were significantly associated with MTC, together with sex (OR=0.06, P<0.001). The "Ct assay" variable was also considered in the logistic regression model, but it was not significantly associated with MTC (OR=0.93, P=0.919). This study indicates that calcium test could be helpful to identify patients with early-stage MTC and those without MTC. A Ct value of 611 pg/mL in males and 445 pg/mL in females are proposed as the optimal Ct cut-offs at the stimulation test.