Tertiary Referral Center Research Articles

Outcome measures analysis following total knee arthroplasty in patients with severe haemophilic arthropathy of the knee.

Total knee arthroplasty (TKA) has been the gold standard for treating severe haemophilic arthropathy of the knee when all conservative measures fail. However, performing a TKA in patients with haemophilic arthropathy is difficult due to severe joint deformity and destruction, and poor bone quality. The aim of the present study was to evaluate the short-term results of TKA in the treatment of knee haemophilic arthropathy in a tertiary referral centre, with an emphasis on health-related quality of life and knee function. A prospective study was conducted that included 19 male patients with end-stage haemophilic knee arthropathy who underwent TKA in a tertiary referral centre. Clinical outcome and health-related quality of life were assessed by the Western Ontario and McMaster Universities Arthritis (WOMAC) index and the Short Form-36 (SF-36) both pre-operatively and at 1-year post-operatively. The mean age of the patients was 50.37±7.63 years (range, 40-65 years). Pre-operative health-related quality of life was impaired in all patients in all SF-36 domains but was markedly improved after TKA. Knee function in all dimensions (pain, stiffness and physical function), as measured by the WOMAC questionnaire, significantly improved after TKA. Pre-operative pain, stiffness and function, along with total WOMAC score, were strongly and negatively correlated with pre-operative SF-36. Overall, the present study indicated a significant improvement in quality of life and clinical outcome after TKA in patients with advanced haemophilic arthropathy. More studies with longer follow-up periods in a larger population are needed to fully elucidate the mid- and long-term values of TKA in haemophilic patients.

Cholecystectomy in the red centre: a review of the surgical outcomes in Central Australia in a five-year period.

Despite the high rates of cholecystectomy in Australia, there is minimal literature regarding the outcomes of cholecystectomy in rural Central Australia within the Northern Territory. This study aims to better characterize the outcomes for patients undergoing cholecystectomy in Central Australia and review clinical and patient characteristics, which may affect outcomes. A retrospective case-control study was performed using data obtained from medical records for all patients undergoing cholecystectomy at Alice Springs Hospital in the Northern Territory from January 2018 until December 2022. Patient characteristics were gathered, and key outcomes examined included: inpatient mortality and 30-day mortality, bile duct injury, bile leak, return to theatre, conversion to open, duration of procedure, length of stay, and up-transfer to a tertiary referral centre. A total of 466 patients were included in this study. Majority of the patients were female and there was a large portion of Indigenous Australians (56%). There were no inpatient mortalities, or 30-day mortalities recorded. There were two bile leaks and/or bile duct injuries (0.4%) and two unplanned returned to theatres (0.4%). Indigenous Australians were more likely to require an emergency operation and had a longer median length of stay (P < 0.001). Cholecystectomy can be performed safely and to a high standard in Central Australia. Surgeons in Central Australia must appreciate the nuances in the management of patients who come from a significantly different socioeconomic background, with complex medical conditions when compared to metropolitan centres.

Peri- and post-operative outcomes of robot-assisted radical cystectomy after the implementation of the EAU guidelines recommendations for collecting and reporting complications at a high-volume referral center.

No studies relied on a standardized methodology to collect postoperative complications after robot-assisted radical cystectomy (RARC). The aim of our study was to evaluate peri- and post-operative outcomes of patients undergoing RARC adhering to the European Association of Urology (EAU) recommendations for reporting surgical outcomes and using a long postoperative follow-up. 246 patients who underwent RARC with intracorporal urinary diversion at a single tertiary referral center with a postoperative follow-up ≥ 1year for survivors. Postoperative outcomes were collected prospectively by interviews done by medical doctors. Complications were scored using the Clavien-Dindo classification (CD), grouped by type and severity (severe: CD score ≥ 3). We described peri- and post-operative outcomes and complication chronological distribution. Overall, 16 (6.5%) and 225 patients (91%) experienced intraoperative and postoperative complications, respectively. Moreover, 139 (57%) experienced severe complications. The most common any-grade and severe complications were infectious (72%) and genitourinary (35%), respectively. Overall, 52% of complications (358/682) occurred within 10days from surgery, and 51% of severe complications (106/207) occurred within 35days. However, 13% of complications (90/682) and 28% of severe complications (59/207) occurred 3months after surgery. The earliest complications were fever of unknown origins and paralytic ileus (median time-to-complication [mTTC]: 4days), the latest complications were urinary tract infection (mTTC: 40days) and hydronephrosis/ureteral obstruction (mTTC: 70days). The rate of postoperative complications after RARC is > 90% when a standardized collection method and a long follow-up is implemented. These results should be used to identify potential areas of improvement and for preoperative patient counseling.

Prospective study of hepatitis B and D epidemiology and risk factors in Romania: A 10-year update.

The global burden of hepatitis D virus (HDV) infection represents a major medical challenge and a public health crisis worldwide. However, there is a lack of accurate data on the epidemiology and risk factors for HDV. Hepatitis B virus (HBV) and HDV coinfection causes the most severe form of viral hepatitis, leading to a higher cumulative incidence of liver-related events compared with HBV monoinfection, including the need for liver transplantation and death. To investigate the epidemiology, natural history, risk factors and clinical management of HBV and HDV coinfection in Romanian patients. This prospective study was conducted between January and July 2022 in six tertiary gastroenterology and hepatology referral centres in Romania. All consecutive adults admitted for any gastroenterology diagnosis who were HBV-positive were enrolled. Patients with acute hepatitis or incomplete data were excluded. Of the 25390 individuals who presented with any type of gastroenterology diagnosis during the study period, 963 met the inclusion criteria. Testing for anti-HDV antibodies and HDV RNA was performed for all participants. Demographic and risk factor data were collected by investigators using medical charts and patient questionnaires. All data were stored in an anonymized online database during the study. The prevalence of HBV was 3.8%; among these patients, the prevalence of HBV/HDV coinfection was 33.1%. The median age of the study population was 54.0 years, and it consisted of 55.1% men. A higher prevalence of HBV/HDV coinfection was observed in patients 50-69 years old. Patients with HBV/HDV coinfection were significantly older than those with HBV monoinfection (P = 0.03). Multivariate multiple regression analysis identified female gender (P = 0.0006), imprisonment (P < 0.0001), older age at diagnosis (P = 0.01) and sexual contact with persons with known viral hepatitis (P = 0.0003) as significant risk factors for HDV. This study shows that HDV infection among those with HBV remains endemic in Romania and updates our understanding of HDV epidemiology and associated risk factors. It emphasizes the need for systematic screening for HDV infection and collaborative initiatives for controlling and preventing HBV and HDV infection.

A novel dosing approach for rituximab in glomerular diseases based on a population pharmacokinetic analysis

ObjectivesRituximab is being increasingly prescribed for the treatment of autoimmune glomerular diseases. While it is highly effective for some diseases, the response is less predictable for others, which may be due to differing requirements in terms of the dosing according to the disease type and variations concerning exposure to the drug. MethodsWe compiled novel rituximab dosing schedules according to pharmacokinetic analysis of data gathered from rituximab treated patients in a tertiary referral nephrology centre between May 2020 and June 2023. The population-pharmacokinetic analysis was based on the rituximab dosing, the patients’ characteristics, rituximab levels and anti-rituximab antibodies. ResultsThe analysis, which was based on data from 185 patients, clearly highlighted differing rituximab dosing requirements for patients with ANCA associated vasculitis and minimal change disease compared to those with membranous nephropathy, focal-segmental glomerulosclerosis and lupus nephritis. This corresponded to the good treatment response of the first two diseases and the unreliable efficacy for the others. The model predicts the rituximab pharmacokinetics with high degree of accuracy when body weight, proteinuria, type of glomerulonephritis, treatment length and anti-rituximab antibodies formation are used as covariates. We proposed a dosing schedule with shortened dosing intervals for difficult-to-treat diagnoses with high proteinuria. ConclusionIn order to ensure reliable and comparable exposure of rituximab with respect to the full range of glomerular diseases, the dosing schedule should be adjusted for membranous nephropathy, focal-segmental glomerulosclerosis and lupus nephritis. This is largely, but not solely, due to the enhanced level of unselective proteinuria in these diseases.

Choroidal manifestations of non-ocular sarcoidosis: an enhanced depth imaging OCT study

BackgroundAlthough choroidal thickening was reported as a sign of active inflammation in ocular sarcoidosis, there has been no research on the choroidal changes in non-ocular sarcoidosis (defined as systemic sarcoidosis without overt clinical signs of ocular involvement). Therefore, this study aimed to investigate choroidal structural changes in patients with non-ocular sarcoidosis.MethodsThis retrospective case–control study was conducted at Asan Medical Center, a tertiary referral center. We evaluated 30 eyes with non-ocular sarcoidosis and their age- and spherical equivalent-matched healthy control eyes. The subfoveal choroidal thickness, area ratio (Sattler layer-choriocapillaris complex [SLCC] area to Haller layer [HL] area), and choroidal vascularity index (CVI, luminal area to choroidal area) were analyzed using enhanced depth imaging in optical coherence tomography. Systemic and ocular factors associated with the choroidal thickness were investigated.ResultsCompared with the healthy control group, the non-ocular sarcoidosis group had significantly thicker subfoveal choroid (total and all sublayers [SLCC and HL]) and lower area ratio. There were no significant differences in the CVIs at all sublayers between groups. In the non-ocular sarcoidosis group, eyes under oral steroid treatment had thinner choroid than eyes under observation. In the control group, eyes with older age and more myopic spherical equivalent had thinner choroidal thickness.ConclusionTotal and all sublayers of the subfoveal choroid were significantly thicker without significant vascularity changes in non-ocular sarcoidosis eyes than in healthy control eyes. The degree of choroidal thickening was disproportionally greater at HL than at SLCC. These characteristic choroidal changes may be the subclinical manifestations in non-ocular sarcoidosis.

Specific Gravity Improves Identification of Clinically Significant Quantitative Proteinuria from the Dipstick Urinalysis.

CKD is often underdiagnosed during early stages when GFR is preserved due to underutilization of testing for quantitative urine albumin-to-creatinine ratio (UACR) or protein-to-creatinine ratio (UPCR). Semi-quantitative dipstick proteinuria (DSP) on urinalysis is widely obtained but not accurate for identifying clinically significant proteinuria. We identified all patients with a urinalysis and UACR or UPCR obtained on the same day at a tertiary referral center. The accuracy of DSP alone or in combination with specific gravity against a gold-standard of UACR ≥30 mg/g or UPCR ≥0.15 g/g, characterizing clinically significant proteinuria, was evaluated using logistic regression. Models were internally validated using 10-fold cross validation. The specific gravity for each DSP above which significant proteinuria is unlikely was determined. Of 11,229 patients, clinically significant proteinuria was present in 4,073 (36%). The area under the receiver operating characteristic curve (95% confidence interval) was 0.77 (0.76, 0.77) using DSP alone and 0.82 (0.82, 0.83) in combination with specific gravity (P<0.001), yielding a specificity of 0.93 (standard error, SE=0.02) and positive likelihood ratio of 9.52 (SE=0.85). The optimal specific gravity cut-offs to identify significant proteinuria were ≤1.0012, 1.0238, and 1.0442, for DSP of trace, 30, and 100 mg/dL. At any specific gravity, a DSP ≥300 mg/dL was extremely likely to represent significant proteinuria. Adding specific gravity to DSP improves recognition of clinically significant proteinuria and can be easily used to identify patients with early-stage CKD who may not have otherwise received a quantified proteinuria measurement for both clinical and research purposes.

The Positive Effect of Adenotonsillectomy on Drooling in Children.

(1) To investigate the prevalence and severity of drooling among healthy young children referred for adenotonsillectomy; (2) to evaluate the effect of adenotonsillectomy on drooling. Prospective study. Tertiary referral center. Healthy typically developed children aged 18 to 48 months referred to adenotonsillectomy for upper airway obstruction (UAO) were recruited. Age-matched children recruited from the community served as controls. Drooling frequency and severity were assessed at baseline and 2 months following surgery based on 2subjective scales: the Drooling Infants and Preschoolers Scale (DRIPS) and Thomas-Stonell and Greenberg Saliva Severity Scale (TSGS). Eighty-seven children aged 18 to 48 months were included in the study. Forty-three children referred to adenotonsillectomy (study group) and 44 age-matched controls. There were significant differences in almost all of the DRIPS items between children in the presurgery group compared to controls. Drooling severity and frequency were greater in the former compared to the latter (TGF-s severity: 1.4 ± 1.0 vs0.6 ± 0.8, P = .001; TGF frequency: 1.3 ± 0.9 vs0.5 ± 0.6, P < .001). After surgery, the scores for all DRIPS and TSGS items decreased significantly and were comparable to the control group. The frequency and severity of drooling among otherwise young children referred for adenotonsillectomy were greater than those for healthy controls. Following surgery, both the frequency and severity significantly improved and became comparable to those of controls. These findings suggest that a major improvement in drooling is one of the benefits of a surgical intervention in a child with UAO.

A novel classification of posterior pelvic exenteration to assess prognosis in female patients with locally advanced primary rectal cancer: a retrospective cohort study from China PelvEx collaborative

PurposeSurgical techniques and the prognosis of posterior pelvic exenteration for locally advanced primary rectal cancer in female patients pose challenges that need to be addressed. Therefore, we investigated the short-term and survival outcomes of posterior pelvic exenteration in female patients using a novel Peking classification.MethodsWe retrospectively analysed a prospective database from China PelvEx Collaborative across three tertiary referral centres. A total of 172 patients who underwent combined resection for locally advanced primary rectal cancer were classified based on four subtypes (PPE-I [64/172], PPE-II [68/172], PPE-III [21/172], and PPE-IV [19/172]) according to the Peking classification; perioperative characteristics and short-term and oncological outcomes were analysed.ResultsDifferences were significant among the four groups regarding colorectal reconstruction (p < 0.001), perineal reconstruction (p < 0.001), in-hospital complications (p < 0.05), and urinary retention (p < 0.05). The R0 resection rates for PPE-I, PPE-II, PPE-III, and PPE-IV were 90.6%, 89.7%, 90.5%, and 89.5%, respectively. The 5-year overall survival rates of the PPE-I, PPE-II, PPE-III, and PPE-IV groups were 73.4%, 68.8%, 54.7%, and 37.3%, respectively. Correspondingly, their 5-year disease-free survival rates were 76.0%, 62.5%, 57.7%, and 43.1%, respectively. Notably, the PPE-IV group demonstrated the lowest 5-year overall survival rate (p < 0.001) and 5-year disease-free survival rate (p < 0.001).ConclusionThe Peking classification can aid in determining suitable surgical techniques and conducting prognostic assessments in female patients with locally advanced primary rectal cancer.

Unilateral cortical autoimmune encephalitis: A case series and comparison to late-onset Rasmussen's encephalitis

ObjectiveTo report a novel anatomical pattern of autoimmune encephalitis characterized by strictly unilateral cortical inflammation and a clinical picture overlapping with late-onset Rasmussen's encephalitis. MethodsWe retrospectively gathered data of patients identified at two tertiary referral academic centers who met inclusion criteria. ResultsWe identified twelve cases (average age 65, +/− 19.8 years, 58% female). All patients had unilateral cortical inflammation manifesting with focal seizures, cognitive decline, hemicortical deficits, and unilateral MRI and/or EEG changes. Six cases were idiopathic, two paraneoplastic, two iatrogenic (in the setting of immune checkpoint inhibitors), and two post-COVID-19. Serologically, ten patients were seronegative, one had high titer anti-GAD65, and one had anti-NMDAR. Five patients met Rasmussen's encephalitis criteria, and six did not fully meet the criteria but had symptoms significantly overlapping with the condition. Most patients had significant improvement with immunotherapy. DiscussionUnilateral cortical AE seems to be more prevalent in the elderly and more frequently idiopathic and seronegative. Patients with this anatomical variant of autoimmune encephalitis have overlapping features with late-onset Rasmussen's encephalitis but are more responsive to immunotherapy. In cases refractory to immunotherapy, interventions used in refractory Rasmussen's encephalitis may be considered, such as functional hemispherectomy.

Emergency embolization in locally advanced cervical cancer: a case series in a tertiary referral center in Argentina

Cervical cancer is the second most common cancer in women in South America. However, it represents the greatest impact on morbidity and mortality in women under 45. Among the most serious complications of cervical cancer, bleeding occupies a significant place, being the main symptom in advanced stages and leading to high morbidity and mortality. Uterine artery embolization is a minimally invasive method that consists of injecting an embolizing material into terminal vessels. The objective of this study is to describe cases of patients with advanced cervical cancer who required embolization of uterine arteries to control massive vaginal bleeding in a tertiary referral hospital. Materials and methods: This is an observational, retrospective study, conducted at Sanatorio Güemes, Buenos Aires between October 2017 and May 2023. We included patients that required embolization due to severe vaginal hemorrhage and diagnosis of locally advanced cervical cancer. Our Institution is one of the largest third-level centers in the city of Buenos Aires, receiving referrals for cervical cancer from other more disadvantaged regions of Argentina. Variables such as age, International Federation of Gynecology and Obstetrics stage, hemoglobin level, oncological treatment performed, embolization material, complications and re-embolization were analyzed. A univariate analysis was performed. The data are expressed in categorical variables in absolute numbers and percentages. Results: A total of 21 patients were included. Median age was 46 years (range, 24 - 83). The mean hemoglobin level was 7.6 g/dl. In our study, 95.2% of the patients responded to the first embolization, and 28.5% required a second intervention throughout the oncological treatment, and only one patient (4.76%) needed a third embolization. Regarding the material used, 66.6% of the patients were embolized with a gelatin sponge, while definitive material was used in the remaining patients (33.3%). Patients who underwent a second embolization, gelatin sponge was used in 83.33% and definitive material was used in only 1 (16.66%). The average survival time from diagnosis was 17.8 months, while the time from the first embolization was 9.8 months. 47.6% of the patients died after the first or second episode of embolization. 95.2% of the patients did not present complications and only 1 (4.76%) died two days after the procedure. Conclusion: Uterine artery embolization is a safe and effective procedure for the treatment of acute hemorrhage in patients with advanced cervical cancer. However, since it is a procedure that is performed only in tertiary centers, it makes it difficult to have universal access.

Acute diverticulitis with extraluminal air: is conservative treatment sufficient? A single-center retrospective study.

Acute diverticulitis with extraluminal air constitutes a heterogeneous condition whose management is controversial. The aims of this study are to report the failure rate of conservative treatment for diverticulitis with extraluminal air and to report risk factors of conservative treatment failure. A retrospective study was performed from an institutional review board-approved database of patients admitted with acute diverticulitis with extraluminal air from 2015 to 2021 at a tertiary referral center. All patients managed for acute diverticulitis with covered perforation (without intraabdominal abscess) were included. The primary endpoint was failure of medical treatment, defined as a need for unplanned surgery or percutaneous drainage within 30days after admission. Ninety-three patients (61% male, mean age 57 ± 17years) were retrospectively included. Ten patients had failure of conservative treatment (11%). These patients were significantly older than 50years (n = 9/10, 90% versus n = 47/83, 57%, p = 0.007), associated with cardiovascular disease (n = 6/10, 60% versus n = 10/83, 12%, p = 0.002), American Society of Anesthesiologists (ASA) score of 3-4 (n = 4/7, 57% versus 6/33, 18%, p = 0.05), under anticoagulant and antiplatelet (n = 6/10, 60% versus n = 11/83, 13%, p = 0.04) and steroid or immunosuppressive therapy (n = 3/10, 30% versus 5/83, 6%, p = 0.04), and with distant pneumoperitoneum location (n = 7/10, 70% versus n = 14/83, 17%, p = 0.001) compared with those with successful conservative treatment. On multivariate analysis, only distant pneumoperitoneum was an independent risk factor of failure (odds ratio (OR) 6.5, 95% confidence interval (CI) [2-21], p = 0.002). Conservative treatment with antibiotics for acute diverticulitis with extraluminal air is safe with a success rate of 89%. Patients with distant pneumoperitoneum should be carefully monitored.

Treatment with (chemo)-radiation in old patients (≥76 years of age) with newly diagnosed non-metastatic squamous cell cancer of the head and neck region: real-world data from a tertiary referral center.

Treatment of patients with cancer of the head and neck region is in focus in a multitude of studies. Of these patients, one patient group, those aged 76 and more, is mostly underrepresented despite requiring thorough and well-reasoned treatment decisions to offer curative treatment. This study investigates real-world data on curative treatment of old (≥76 years) patients with newly diagnosed squamous cell carcinoma of the head and neck region (HNSCC). Between January 2010 and December 2021, we identified 71 patients older than 76 years with newly diagnosed HNSCC and cM0 at the Department of Radiation Oncology of the University Hospital of Erlangen-Nuremberg. Using electronic medical records, we analyzed treatment patterns and outcomes in terms of overall survival (OS), progression-free survival (PFS), and locoregional control (LRC) rate. Additionally, we performed univariate risk analysis and Cox regression in order to identify predictive factors associated with the abovementioned treatment outcomes. The median follow-up was 18 months. OS was 83%, 79%, and 72% after 1 year, 2 years, and 3 years, respectively. PFS was 69%, 54%, and 46% after 1 year, 2 years, and 3 years, respectively. A total of 34 (48%) patients were treated with standard therapy according to current guidelines. The reasons for deviation from standard therapy before or during treatment were as follows: unfitness for cisplatin-based chemotherapy (n = 37), reduction of chemotherapy (n = 3), and dose reduction/interruption of radiotherapy (n = 8). Carboplatin-based systemic therapy showed improved PFS compared to cisplatin or cetuximab (60 vs. 28 vs. 15 months, p = 0.037) but without impact on OS (83 vs. 52 vs. 38 months, p = 0.807). Oropharyngeal tumor localization (p = 0.026) and combined treatment (surgery and postoperative treatment) (p = 0.008) were significant predictors for a better OS. In multivariate analysis, oropharyngeal tumor localization (p = 0.011) and combined treatment (p = 0.041) showed significantly increased PFS. After 1 year, 2 years, and 3 years, the cumulative incidence of locoregional recurrences (LRRs) was 13%, 24%, and 27%, respectively, and was significantly decreased in patients with oropharyngeal tumor localization (p = 0.037). Adherence to treatment protocols for radiotherapy alone in old patients with HNSCC is good, whereas the application of concurrent chemotherapy often deviates from guidelines in terms of de-escalation. An important risk factor for decreased OS, PFS, and a higher rate of LRR appears to be non-oropharyngeal tumor location in old patients.

Outcomes of Immediate Total Thyroidectomy in First-Side Loss of Neuromonitoring Signal

Use of intraoperative neuromonitoring (IONM) during thyroidectomy can nearly eliminate the risk of postoperative bilateral vocal cord palsy (VCP) by indicating staged surgery in cases of loss of signal (LOS) on the first side of planned total thyroidectomy. However, aborting planned total thyroidectomy may lead to persistence of symptoms, delay in adjuvant treatment, and patient inconvenience and distress. There are few data to guide a selective approach to total thyroidectomy in patients with first-side LOS. To investigate outcomes of immediate bilateral surgery in patients undergoing total thyroidectomy with first-side LOS. This cohort study was a retrospective review of outcomes for patients undergoing thyroidectomy between January 2016 and July 2023 at an academic tertiary referral center for thyroid surgery. Consecutive patients scheduled for total thyroidectomy using IONM were included. Exclusion criteria were preoperative VCP, deliberate sacrifice of recurrent laryngeal nerve (RLN), inadvertent RLN resection, and surgery performed without IONM. Total thyroidectomy performed using IONM. Vocal mobility on first postoperative day as assessed by flexible laryngoscopy; secondary outcome measures included subjective voice assessment, other postoperative complications, and long-term vocal mobility. Among 400 patients undergoing planned total thyroidectomy (mean age, 50.5 years [range, 4-88 years]; 318 female [79.5%]), 51 (12.8%) had first-side LOS, of whom 37 (9.3%) had persistent LOS. Twenty-nine patients (56% of procedures with first-side LOS, including 18 with persistent LOS) proceeded to immediate total thyroidectomy. Postoperatively, 16 patients (55% of patients undergoing total thyroidectomy following first-side LOS, including 14 of 18 with persistent LOS) had impaired vocal mobility. One patient had bilateral VCP that did not require tracheostomy, and 2 had postoperative hypoparathyroidism. Of those whose surgery was aborted after first-side LOS, 8 of 22 (36%) underwent completion thyroidectomy at a later stage. In those undergoing completion thyroidectomy, 2 of 8 (25%) had temporary VCP after the second surgery, 2 (25%) had permanent hypoparathyroidism, and 1 (12.5%) developed inoperable cancer. Postoperative VCP was fully reversible in all but 1 patient. Among patients planned for total thyroidectomy who develop first-side LOS, immediate total thyroidectomy may be considered among those who have pressing reasons for same, and where surgical difficulties might be anticipated in a secondary surgery.

Clinical Outcomes of Salvage Microsurgical Resection after Radiation Therapy for Sporadic Vestibular Schwannomas

Abstract Background The purpose of this study was to evaluate clinical outcomes after salvage microsurgery for vestibular schwannoma (VS) treated initially with modern radiotherapy techniques as compared to those treated with primary microsurgical resection. Methods Patients who underwent microsurgical resection of sporadic VS undergoing a translabyrinthine approach at a single academic skull base tertiary referral center were included. Baseline and postoperative dynamic gait index, functional gait assessment, House–Brackmann facial nerve grading, and completeness of resection were prospectively recorded. Results Of the 265 patients reviewed, 21 (7.9%) patients underwent prior radiation. Median age of the cohort was 55 years (interquartile range: 51–63). The likelihood of achieving a gross total resection was significantly lower for radiated as compared to nonradiated patients (odds ratio: 0.18, 95% confidence interval: 0.05–0.53, p = 0.004) when controlling for tumor size. Radiated patients had better postoperative facial nerve function on the first postoperative day, but this difference was not significant at long-term follow-up. Radiated patients had lower preoperative postural stability scores than nonradiated patients on FGA (26 vs. 23, p = 0.035). Postoperatively, radiated patients had comparable outcomes compared to nonradiated patients when controlling for age and tumor size. Conclusions Compared to patients with VS treated with surgery alone, previously radiated patients are less likely to achieve gross total resection in the salvage setting. Radiated patients scored better on facial nerve outcomes compared to nonradiated patients in the initial postoperative period but demonstrated similar long-term outcomes.

P133 Anti-synthetase syndrome: an experience from a tertiary referral center

Abstract Background/Aims Anti-synthetase syndrome (ASS) is a subset of Idiopathic inflammatory myositis (IIMs). It is a syndromic diagnosis classified by Connors et al. and Solomon et al. criteria. Raynaud’s phenomenon (RP), fever, arthritis, mechanics hands, ILD and myositis constitute this syndrome along with the classical Anti-aminoacyl tRNA synthetase antibodies (ARS). About 25% of IIMs patients are classified as ASS. This study was planned to evaluate the prevalence of ASS; their phenotype, antibody subtypes, treatment responsiveness and outcomes. Methods All consecutive ASS patients were enrolled in the study fulfilling Connors et al. criteria during January, 2016 to August2023. Their demographic details, clinical features, laboratory and imaging features, treatment were analyzed. ARS were considered positive on a Line Immunoassay with cutoff &amp;gt;6U/ML using BlueDriverQuantrix-ANA25 Screen IgG kit (includes Jo1, Pl-7, Pl-12) from D-tek, Belgium including three ASS antibodies (Jo-1, PL-12, PL-7). Anti EJ, OJ antibodies were not present in the kit. Descriptive analysis were used to describe disease characteristics. Results Among 139 total IIM patients, 21(15%) patients were diagnosed with ASS. All patients fulfilled Connors et al. criteria while 66.6% patients fulfilled stricter criteria by Solomon et al. Mean(±SD) age group was 43(±16.4) years. 71.4% were Females. Anti Jo-1 was the predominant antibody seen in 66.6% while Anti PL-12 and Anti Pl-7 antibodies were present in 19% and 14.3% respectively. 66.6% had ANA positivity and MAA antibodies were seen in 52.3%. Rash was seen in 61.9%, mechanics hands in 38%, RP in 52.3% fever in 38% ,arthritis in 57%, myositis 62% and ILD in 80.9%. NSIP (66.6%) was the predominant type, followed by OP (9.5%) and UIP (4.7%). Median (IQR) CPK level was 736 (210-4556)U/L. Anti Jo-1 patients had higher prevalence of mechanics hands and rash whereas ILD was observed in all cases of Anti PL-7 and Anti PL-12 antibodies. Malignancy was seen in two patients (Malignant fibrous histiocytoma, Metastatic Pancreatic carcinoma). Mean (SD) MMT8 score was 62.8(±22.2) at presentation . Treatment is with steroids in all cases followed by maintenance with azathioprine in 57.1%, Methotrexate in 14.2%. Rituximab was used in 14.2%, cyclophosphamide in 9.5% and mycophenolate mofetil in 23.8%. Mortality was seen in one patient with respiratory failure. Lung involvement was severe in patients with both MSA and MAA positivity. 62% patients were in remission. Conclusion ILD and Arthritis were the main presenting complaints in our cohort. Myositis is relatively less prevalent in ASS. NSIP was the predominant ILD subtype. Non Jo-1 group had high MAA positivity. Jo-1 was associated with mechanic’s hands and rash. Malignancy was less prevalent in ASS. All patients with PL-7 and PL-12 antibodies had lung involvement. Biologics were required in few refractory cases. Disclosure P. Khatri: None. J.R. Yadavalli: None. S. Dhuria: None. N. Negalur: None. K. Shivam: None. R. Gupta: None.

Diagnostic accuracy of pocket-size lung ultrasound for etiological definition of pneumonia and surveillance of complications in children hospitalized: a prospective diagnostic cohort study

Background: Pneumonia, the leading infectious cause of death in children worldwide, often requires a chest radiograph (CXR) for diagnosis, involving radiation exposure. Point-of-Care Ultrasound (POCUS) offers a radiation-free alternative and, specifically the pocket-size variant, enhances convenience at the patient's bedside. While evidence supports ultrasound's accuracy in detecting community-acquired pneumonia (CAP) in children comparable to CXR, few studies have explored its ability to distinguish pneumonia etiology, especially utilizing pocket-size POCUS devices. Methods: In this prospective diagnostic cohort study conducted over a year in a tertiary pediatric referral center, we aim to assess the diagnostic accuracy of a pocket-size POCUS device compared to CXR for determining the etiology of CAP in pediatric patients (aged &gt;6 months and &lt;18 years). At least 76 participants diagnosed with CAP will undergo independent POCUS examinations at various intervals, complemented by CXRs when necessary, independently classified by a third investigator. The General Electrics Vscan AirTM®, featuring Bluetooth connectivity to smartphone/tablet, will be employed for POCUS. Data collection will include systematized POCUS and CXR descriptions, alongside sociodemographic, clinical, and therapeutic variables. Statistical analysis using SPSS® version 28 will evaluate the diagnostic accuracy of the POCUS device. Conclusions: This trial's outcomes hold significant promise in unveiling unknown data about the diagnostic accuracy of pocket-size POCUS for pediatric CAP etiological diagnosis. Utilizing a device meeting technical recommendations, featuring a dual-headed probe and Bluetooth connectivity, this study has the potential to bring innovation to clinical practice, improving patient care and creating scientific value. Trial Registration Number: NCT06296693.

E018 Prophylactic co-trimoxazole prescribing practice in patients who received rituximab therapy: comparison across indications and specialties

Abstract Background/Aims Rituximab is a chimeric anti-CD20 monoclonal antibody targeting B-cells used as part of treatment algorithms for malignancy and autoimmune diseases. As is the case of other immunosuppressive agents, rituximab increases the risk of infections. Primary prophylaxis with co-trimoxazole may be considered in high-risk cohort of patients to prevent opportunistic infection, particuarly Pneumocystis jerovecii pneumonia (PJP). To investigate the prescribing practice for prophylactic co-trimoxazole in patients who received rituximab infusion in a large tertiary referral centre. To compare practices between rheumatology and nephrology department. To compare the incidence of hospitalisation for infection (particularly PJP) between those prescribed prophylactic co-trimoxazole and those who did not. Methods This was a single centre retrospective observational study over a 3 year period assessing prophylactic co-trimoxazole prescribing practice in 185 patients attending rheumatology or renal services in Cork University Hospital (CUH) and receiving rituximab. Co-trimoxazole prophylactic treatment was defined as prophylaxis dose of co-trimoxazole (either 480mg daily or 960mg three times per week) received during rituximab treatment and at least 6 months post completion of treatment. Results 185 patients’ medical charts were reviewed. Only 28 patients received prophylaxis with co-trimoxazole. The majority of patients (n = 24, 85%) who received prophylaxis with co-trimoxazole had an underlying diagnosis of small vessel vasculitis (SVV). Co-trimoxazole was more commonly prescribed by nephrologists (n = 17, 71% primarily for SVV) as compared to rheumatologist (n = 7, 29% primarily for SVV). 156 patients were not prescribed prophylactic co-trimoxazole. The primary indication for rituximab in these patients was inflammatory arthropathy (IA) (n = 72,46%), SVV (n = 26, 16%), systemic lupus erythematosus (n = 19, 12%), with the remainder accounted for by scleroderma, undifferentiated connective tissue disease, membranous nephropathy (MN), myositis, focal segmental glomerulosclerosis and amyloid renal disease. No patient was admitted over the course of the study period with PJP. 59 patients required hospital admissions due to other infections, most commonly patients with SVV. The most common infections were respiratory tract and urinary tract infections. Overall infections requiring hospitalization were more common in the group receiving prophylaxis, perhaps highlighting the vulnerable nature and greater prednisolone prescribing in the SVV cohort. No difference was seen between groups in the prevalence of UTIs. Conclusion In this cohort co-trimoxazole prophylaxis was more commonly prescribed for patient with small vessel vasculitis and differences in prescribing patterns were seen between nephrologists and rheumatologists. With greater awareness of the heightened risk of PJP in patents receiving rituximab consideration should be given to use of antibiotic prophylaxis for other indications. Disclosure S. Tio: None. J. Morrow: None. S. Cowley: None. M. Clarkson: None. G. Murphy: None.

Medical photography and the reconstructive urologist: A 6-month prospective study.

Medical photography has multiple, important roles. The education of medical practitioners, documentation of disease, response to treatment, research, publication, intraoperative recording and trauma documentation all rely on medical photography. Additionally, there are important medicolegal implications pertaining to medical photography across many medical disciplines. Other than specific image use to document cases, there remains a paucity of urological literature regarding the use of medical photography in Urology. The aims of this 6-month study were to document the use of medical photography by a Reconstructive Urological Service in a tertiary referral centre and to assess the range of urological conditions photographed. A secondary aim was to specifically document intraoperative use of the medical photography.

Paediatric cutaneous lymphomas including rare subtypes: A 40-year experience at a tertiary referral centre.

Primary cutaneous lymphomas are neoplasms of the immune system with a distinct tropism for the skin and an absence of extracutaneous manifestations at the time of diagnosis. Studies focusing on cutaneous lymphomas in children and adolescents remain scarce and often do not encompass the rare subtypes. To address this knowledge gap by describing the clinical, histological and molecular characteristics of a large group of paediatric patients affected by primary cutaneous lymphoma. We also provided the Paediatric Primary Cutaneous Lymphoma Atlas that illustrates the clinicopathological spectrum of observed presentations, in the hope of supporting other physicians in the diagnostic process. Retrospective chart review of paediatric patients diagnosed with primary cutaneous lymphomas between 1980 and 2022 at the Paediatric Dermatology Unit of Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan. A total of 101 patients (58 males, 43 females) met the inclusion criteria. The most common subtypes were lymphomatoid papulosis (n = 48) and mycosis fungoides (n = 31). These were followed by primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders (n = 7), primary cutaneous anaplastic large-cell lymphomas (n = 5), primary cutaneous marginal zone B-cell lymphomas (n = 3), primary cutaneous follicle centre lymphomas (n = 2), subcutaneous panniculitis-like T-cell lymphomas (n = 2), primary cutaneous peripheral T-cell lymphoma not otherwise specified (n = 1), primary cutaneous precursor B-lymphoblastic lymphoma (n = 1) and Sézary syndrome (n = 1). Clinical follow-up data covering a median of 70.8 months (range 1-324) were available for 74 patients, of whom three died due to cutaneous lymphoma. Our findings shed light on the peculiar aspects and long-term outcomes of paediatric cutaneous lymphomas, particularly emphasizing their distinctive features in comparison to their adult counterparts and exploring the less common subtypes. Further larger-scale studies are warranted to better characterize these entities and to achieve a more rapid and accurate diagnosis.