Single Center Experience Research Articles

Laparoscopic Sugarbaker repair of parastomal hernia following radical cystectomy and ileal conduit: a single-center experience

PurposeParastomal hernia (PH) is a frequent complication following radical cystectomy and ileal conduit. The purpose of this study was to summarize the clinical experience and technical characteristics of laparoscopic Sugarbaker repair of PH following radical cystectomy and ileal conduit.MethodsWe retrospectively evaluated all patients who underwent laparoscopic treatment of PH following radical cystectomy and ileal conduit at Huashan Hospital, Fudan University from May 2013 to December 2022.ResultsThirty-five patients were included in the study. Median follow up was 32months (IQR, 25–38 months). Three patients presented with a recurrence (8.6%), with a median time to recurrence of 14 months. Out of the 35 patients, Thirty-two underwent totally laparoscopic repair using the Sugarbaker technique, Three patients required open surgery to repair the intestinal injury after laparoscopic exploration. One patient died 9 months post-surgery due to COVID-19. During the follow-up period, two patients developed a peristomal abscess, and one patient experienced partial intestinal obstruction 10 days after surgery.ConclusionSurgical management of PH following radical cystectomy and ileal conduit is challenging. The laparoscopic Sugarbaker technique for repairing PH following radical cystectomy and ileal conduit has low complication and recurrence rate.

Evaluation of tocilizumab-induced hypofibrinogenemia in rheumatology practice: a retrospective, real-life, single-center experience.

Tocilizumab (TCZ) which is a humanized interleukin (IL)-6 receptor antibody has been increasingly widespread used in rheumatology practice. TCZ-related hypofibrinogenemia is a not well-described side effect, but awareness seems to be incresing as publications on this topic are also becoming more frequent. Our aim in this study was to determine the frequency, timing, and approach to TCZ-related hypofibrinogenemia in rheumatic diseases. We retrospectively screened our patients who received TCZ for inflammatory rheumatic diseases and studied serum fibrinogen at least once before and/or after drug administration. We recorded and analyzed demographic features (age, gender, diagnosis), comorbidities, laboratory parameters, management, and outcome data. 30 patients who received TCZ due to rheumatological diseases and had at least one fibrinogen level were included in this study. 73.3% were female and median age was found to be 65 (42-82) years, with median disease duration of 148 (36-500) months. 90% of the patients received TCZ for RA and 10% for GCA. We examined the fibrogen levels at an average of 24 months (min 1 max 108) following the start of TCZ treatment. The study group was divided into those with normal fibrinogen levels (normal fibrinogen group) and those with low fibrinogen levels (lowfibrinogen group). In total, hypofibrinogenemia was found in 14 (46.6%) patients. Median serum fibrinogen level in the low fibrinogen group after TCZ was calculated to be 151.5mg/dl (min 92 max 171). Most patients were asymptomatic (71.4%). Ecchymoses were in seen 4 (28,6%) patients. No major bleeding were seen. TCZ was discontinued in 5 out of 14 patients (35.7%), while 9 out of 14 patients (64.3%) were closely followed. Outcomes regarding fibrinogen levels (11 out of 14) were as follows: increase in 4 and not checked in one after cessation of the drug, 3 spontaneous increases under the drug, and 3 persistently low levels on TCZ treatment. We found no difference in terms of gender (p = 0.417), platelets (p = 0.343), ESR (p = 0.448), and CRP (p = 0.660) at the time of TCZ initiation between groups. TCZ-related hypofibrinogenemia is more frequent than expected with occurence in both the early and late stages of treatment. Further research is required to determine whether to regularly measure fibrinogen levels in patients using TCZ and how to treat patients with hypofibrinogenemia.

Real-World Use and Outcomes of Apixaban for Early Post-Surgical Fontan Thromboprophylaxis.

Patients with Fontan physiology are at heightened risk of thrombosis related to passive venous return leading to increased stasis, as well as acquired thrombophilia from congestive hepatopathy. Variability exists for post-Fontan thromboprophylaxis, with no consensus on best practices. Direct oral anticoagulants offer advantages over conventional anticoagulants including fewer drug-drug interactions, no dietary restrictions, and less frequent monitoring. Herein, we report our single center experience utilizing apixaban thromboprophylaxis in children post-Fontan procedure. Single center, retrospective, cohort study evaluating apixaban thromboprophylaxis dosing strategies, efficacy, and safety in children admitted post-Fontan procedure at Boston Children's Hospital. Between September 2019 and December 2023, 62 children, median age 3.2years (2.1-10.5years), weight 13.9kg (9.5-56.3kg) received apixaban at a median of 93days post-Fontan (7-1421days). Over a total of 93days of apixaban exposure, there was 1 treatment-related thrombosis event (0.07 per 1000 person-days on apixaban) and 3 combined treatment-related clinically relevant non-major (CRNM) and major bleeding events (0.22 per 1000 person-days on apixaban). Apixaban for post-Fontan thromboprophylaxis was feasible with low rates of bleeding and thrombosis.

The impact of hemovigilance studies on transfusion practices: Single centre experience

Objective: Blood product transfusion is one of the most commonly used medical interventions worldwide. However, transfusion-related adverse events (TRAEs) can cause morbidity and rarely even mortality in patients. For this reason, hemovigilance studies have recently gained importance in terms of safe and effective implementation of transfusion. Material and methods: Transfusion practices issued in the period of 2016-2023 were analyzed retrospectively. All TRAE including adverse reactions (AR) and adverse events (AE) were recorded. The impact of hemovigilance practices on the incidence of TRAE was analyzed by comparing the numbers and types of events between years. Results: 135506 blood products were used for a total of 45571 patients for a total of 8 years between 2016 and 2023. 172 adverse reactions (AR) were reported in 170 patients. The overall AR incidence was found to be 126.8 (/100000). The highest yearly AR rate was reported in 2018 with 168.04 (/100000). Since hemovigilance measures were tightened, a statistically significant decrease was recorded in ARs from the beginning of 2020 to date (p

Infections Following Gender-Affirming Vaginoplasty: A Single-Center Experience.

We describe the epidemiology and incidence of infections following gender-affirming vaginoplasty. Urinary tract and surgical site infections were the most common infections with incidences of 17.5% and 5.5%, respectively. We also identified a significant gap in human immunodeficiency virus screening and prescription of preexposure prophylaxis.

Analysis of predictors of hemorrhagic transformation after reperfusion therapy with recombinant tissue plasminogen activator in patients with acute ischemic stroke: a single-center experience

BackgroundHemorrhagic transformation (HT) is a serious complication of thrombolytic therapy for acute ischemic stroke, limiting its indications and affecting treatment plans and clinical prognosis. Identifying risk factors for HT could help improve the risk–benefit ratio of thrombolytic therapy. We aimed to analyze the predictors of HT after reperfusion therapy with recombinant tissue plasminogen activator (rTPA) in patients with acute ischemic stroke. This study included 115 patients who received rTPA. All patients underwent history taking, clinical examination, neurological examination including Glasgow Coma Scale and National Institutes of Health Stroke Scale scores (NIHSS), radiological investigation, and cardiac investigation. Patients were followed up strictly every 2 h for 1st 24 h then for two weeks clinically using the NIHSS and radiologically using CT or MRI to detect HT.ResultsPatients with HT represented 21.7% of all patients receiving rTPA, while symptomatic intracranial hemorrhage (sICH) represented 6.9%. Patients with HT had significantly higher blood pressure, respiratory rate, atrial fibrillation rate, NIHSS score, INR, prothrombin time, neutrophil-to-lymphocyte ratio (NLR), and lower platelet count, LDL level, higher Fazekas score, lower ASPECT score, and prolonged onset-to-needle time.ConclusionPredicting HT in acute ischemic stroke patients is crucial for optimizing management and potentially improving outcomes. In our study, six predictors were associated with HT: higher respiratory rate, higher atrial fibrillation rate, higher NLR, lower LDL level, higher Fazekas score, lower ASPECT score, and onset-to-needle time greater than 180 min.

Rapid Whole-Genome Sequencing in Acutely Ill Children: A Single Pediatric Center Experience

AbstractThe aim of this study was to describe the turnaround time, diagnostic yield, and clinical impact of rapid whole-genomic sequencing (rWGS). We conducted a prospective observational study in acutely ill children (0–21 years) with an undiagnosed, potentially genetic abnormality in a children's hospital. A phenotype-prioritized analysis approach for rWGS was utilized. The turnaround times, diagnostic yield, number of genes detected, inheritance pattern, zygosity, and the clinical impact of positive or negative tests were analyzed. Out of a total of 109 children, 92 abnormal (pathogenic or likely pathogenic) gene variants were detected in 60 (55%) patients. There were 45 neonates, 35 infants, and 29 children. The admission location was 49.5, 34.9, and 15.6% in the pediatric intensive care unit (PICU), neonatal intensive care unit (NICU), and cardiac intensive care unit (CICU), respectively. The median (interquartile range [IQR]) times for the return of preliminary and final results were 3 (2–5) and 10 (6–14) days, respectively. With ultra-rapid processing, the median time to final results was shorter (5 [3–7] vs. 12 [7.75–15] days). Neurologic issues were the most common underlying admission diagnoses. The diagnostic yield for a causative gene was 47.7%. The diagnostic yield was not different based on age group or location of admission but higher in metabolic issues (78.6 vs. 43.2%; odds ratio [OR]: 4.8; 95% confidence interval [CI]: 1.3–18.4). There was a change in clinical management in 39.4%. In acutely ill children with undiagnosed conditions and with clinical suspicion of a genetic disorder, rWGS detected gene variants in 55% with a diagnostic yield of 47.7% and resulted in a change in the management in 39.4%. The diagnostic yield in patients with metabolic conditions was the highest.

High-Definition 4K-3D Exoscope in Spine Surgery: A Single-Center Experience and Review of the Literature.

Background and Objectives: Binocular optical microscopy (OM) paved the way for a new era in brain and spine neurosurgery fields with the introduction of microsurgery. Despite its enormous contribution to modern neurosurgery, OM presents some intrinsic limitations that surgeons need to face during procedures such as prolonged non-ergonomic positions and decreased vision quality to the assistant eyepiece. To overcome these limitations, in recent years, new operative tools have been introduced, such as exoscopes. Here, we present our experience with exoscopes in spine surgery. Materials and Methods: In the period between January 2022 and December 2023, we gradually implemented the use of a high-definition 4K-3D exoscope (ORBEYETM, Olympus, Japan) in patients undergoing spinal surgery. Results: A total of 243 patients underwent spine surgery with exoscope magnification (47 intradural tumors, 99 lumbar degenerative cases, 79 cervical degenerative cases, 5 dorsal calcified disk herniations, 4 dural arteriovenous fistulas (dAVFs), and 9 others). We compared this cohort with a similar cohort of patients operated in the same period using OM based on different endpoints: operating time, complication rate, and infection rate. We did not find any statistically significant difference in any of the endpoints between these two groups. Conclusions: In our experience, the exoscope provides a better resolution of spinal anatomy and higher quality real-time images of the surgery for the entire OR team and improves the ergonomic posture of both surgeons, without lengthening the operating time and without increasing the rate of adverse events. Prospective studies with a larger cohort of patients are needed to further validate these findings.

Cell throughput and performance ratio as quality indicators on hematopoietic stem cell apheresis: A single-center experience

BackgroundBenchmarking in CD34+ cell apheresis is crucial for optimizing resources, ensuring consistent collection performance, and ultimately, decision-making algorithms to improve donor safety. Key performance indicators such as the “performance ratio” (PR) are applied routinely in some apheresis centers, whereas this report identifies the “cell throughput” (CT) as another quality indicator in apheresis. Material and methodsThis single-center study includes retrospective data from 117 aphereses. CT and PR were calculated based on the mononuclear cell collection (MNC) or continuous mononuclear cell collection (cMNC) protocols of the Spectra Optia® apheresis system, types of venous access, transplant settings, andmobilization regimens. ResultsCTs (× 106 CD34+ cells/min) were found to be greater in cMNC compared to MNC protocols (1.4 vs. 1.0, p = 0.0037), in allogeneic versus autologous (1.3 vs. 1.1, p = 0.0274), and in the mobilization regimen of G-CSF alone versus the G-CSF combined (1.3 vs. 1.0, p = 0.0249). In contrast, PR (%) was only statistically significant in favor of the cMNC protocol (213.0 vs. 186.8 for MNC). ConclusionsCT and PR are feasible quality indicators on CD34+ cell apheresis, are easy to calculate and implement, and have clinical and administrative implications. Analyzing CT and PR may strengthen the institutional criteria for selecting cMNC or MNC protocols; they may also be used to evaluate the performance of new personnel or cell separator devices or, eventually, trigger investigations for those aphereses under-collected by specific thresholds.

Awakening ECMO During Pediatric Extracorporeal Membrane Oxygenation: A Single-center Experience

Awakening ECMO During Pediatric Extracorporeal Membrane Oxygenation: A Single-center Experience

Initial Experience with the New DERIVO® Mini Embolisation Device for the Treatment of Intracranial Aneurysms.

The aim of this study is to present the outcomes of cerebral aneurysm treatment with the DERIVO® mini Embolisation Device (DMD), which is compatible with microcatheters with 0.021-inch inner diameters. Consecutive patients treated with DMD were identified retrospectively. Patient and aneurysm characteristics, procedural findings, clinical outcomes and follow-up imaging results were evaluated. A total of 44 target aneurysms in 30 patients were treated with DMD. The mean age of the patients was 49.9 (range, 4-77 years). Four patients with five aneurysms presented with acute subarachnoid hemorrhage. The mean aneurysm size was 6.8 mm (range, 1.5-22 mm). In 29 (65.9%) aneurysms, adjunctive devices were used for endovascular treatment. The overall mortality rate was 3.3% and procedure-related mortality was 0%. Overall neurologic morbidity was 6.6% and none of the patients had a permanent sequela secondary to the procedure. The mean clinical follow-up period was 20.9 months (range, 3 days-46 months) and the mean DSA follow-up period was 10.9 months. A total of 37 (84.1%) aneurysms demonstrated total occlusion (Raymond-Roy [RR 1]); 3 (6.8%) aneurysms had a neck remnant or infundibular filling at the origin of the jailed side branch (RR 2), 4 (9.1%) aneurysms had residual aneurysm filling (RR 3). For those aneurysms treated with bare DMD, the total occlusion rate was 73.3% at a mean follow-up of 16.1 months. In this initial clinical single-center experience, DMD had a good safety profile and efficacy comparable with the currently used flow diverters.

FP7.2 - Single centre experience of laparoscopic management of acute small bowel obstruction

Abstract Objectives The objective of this article is to present a single-centre experience of laparoscopic emergency surgery reporting outcomes by laparoscopic management of acute small bowel obstruction (ASBO). Methods A retrospective review of case notes to evaluate postoperative outcomes. 119 patients were included in this study of laparoscopic management of ASBO and data analysis was performed with SPSS software. Results There were 119 patients with primary aetiologies of adhesion bands (49.5%/59) and hernia (31.9%/38) were noted. Mortality and morbidity outcomes – 15 patients had minor complications, 37 had major complications and 3 patients had post-operative mortality. The average length of stay (LOS) was 10.86 days. The LOS Subgroup analysis showed that 13.8 days were average for adhesiolysis and 6.7 days for the hernia. Discussion On comparison with the published data global data suggest minor complications of 12.6 % (14% in this single-centre experience), major complications of 34 % (31 % in this single-centre experience) and mortality of 0.6% (2.5 % in this single-centre experience). Conclusion This study shows that this centre has comparable mortality and morbidity outcomes when compared to the global trend, but these outcomes.

SP8.2 - Single centre experience in establishing a robotic colorectal surgery programme in a UK district hospital

Abstract Aim To describe our centre’s experience in establishing a robotic programme for colorectal surgery procedures. Method Demographic, peri-operative and follow-up data from a prospectively maintained database were collected for all laparoscopic and robotic assisted colorectal procedures carried out at our centre. Results 38 colorectal resections were completed with the DaVinci Xi (Intuitive Surgical, USA) robotic platform between April-December 2023. These were compared to a laparoscopic cohort of 57 consecutive colorectal resections. 37% (n=14) of the robotic cases were proctored and all were resections for malignancy. There were 57.9% (n=22) males in the robotic cohort and 50.9% (n=28) in the laparoscopic cohort. There was no statistical difference between the demographics of the two cohorts in terms of age, body mass index and ASA grade. 21/38 (55.3%) robotic resections were left sided compared to 35/57 (61.4%) laparoscopic resections. No robotic cases were converted to open or laparoscopy, but 8.8% (n=5) of laparoscopic cases were converted to open. Laparoscopic procedures were shorter (median operative time 277 min compared to 412 min, p<0.001). A stoma was formed in 13/38 (34.2%) robotic cases and 16/57 (28.1%) laparoscopic cases. All bowel anastomoses performed in the robotic cohort were stapled with 16/32 (50.0%) being intra-corporeal. The median length of stay was 5 days for both cohorts. 5.3% (n=2/38) Vs 12.3% (n=7/57) had a significant post-operative complication (Clavien-Dindo ³3). No mortalities were recorded within 90 days of the procedures. Conclusions Other than longer operative times, robotic colorectal resections in a district hospital setting is comparable to laparoscopic resections.

WP3.4 - Partial gastrectomy as a day case procedure for the treatment of Gastrointestinal Stromal Tumours – a single centre experience

Abstract Aims Gastrointestinal stromal tumours (GISTs) are malignant growths that may be managed effectively with radical surgery if diagnosed at an early stage. The majority of GISTs are found in the stomach and many may be managed effectively via a laparoscopic partial gastrectomy (PG). Day case surgery is becoming a more feasible option for patients undergoing laparoscopic PG. This study examined the outcomes of patients who underwent laparoscopic PG as a day case procedure at a UK tertiary centre. Methods The records of patients who underwent a partial gastrectomy to treat extra-luminal gastric GISTs between January 2021 to December 2023 were retrospectively reviewed. The rates of readmission and post-operative complications were noted for the patients were assessed. Patients with tumours near the gastro-oesophageal junction, lesser curve or with a significant intraluminal component were excluded. Results 42 patients aged between 52 - 81 years were ultimately included in this analysis and 31 (73.8%) underwent surgery as a day case procedure. There were no readmissions, peri- or post-operative complications and none of the patients required a conversion from a laparoscopic to an open approach. Conclusions This single centre analysis suggests that laparoscopic day case PG is a safe option for selected patients. Overall this experience suggests that day case surgery should be carefully considered in those deemed likely to be able to recover well post-operatively. Additional larger studies are required to further evaluate the safety of day case laparoscopic PG.

SP4.12 - A single centre experience of managing patients admitted for elective Nissen’s Fundoplication as a day case procedure

Abstract Aims Laparoscopic Nissen’s fundoplication (LNF) is an elective procedure used to treat gastro-oesophgeal reflux which cannot be managed effectively with medical and conservative methods. LNF is the most common surgical procedure used to treat reflux in the UK and optimising the discharge process for these patients can therefore prevent excessive admission times for many patients. This study aimed to analyse the success of LNF as a day case procedure in a tertiary centre. Methods The notes from patients who underwent LNF between January 2020 and December 2023 were retrospectively reviewed. The demographics, co-morbidities and the rates of readmission and complications of the patients included were noted. All patients were managed following local and national surgical, anaesthetic and nursing guidelines. Results 67 patients underwent LNF within the time frame studied, each of which were initially planned to be kept in hospital overnight. 41 (61.2%) cases however, were considered to be medically safe for discharge as a day case operation. None of the patients studied required readmission or suffered any complications despite this change in their care plan. Conclusions This single centre experience showed that patient safety was not compromised by encouraging LNF as a day case procedure in selected patients. Day-case protocols should therefore be considered for patients undergoing LNF who have no/few major co-morbidities and can be looked after at home post-operatively in order to reduce healthcare costs and maximise bed space availability.

Vascular Access Related Infections in Hemodialysis Children and the Impact of Implementation of CDC Guidelines and Tools on the Control of these Infections: A Single Centre Experience from Egypt

Background: Vascular access-related infection (VARI) is a serious issue in pediatric end-stage kidney disease (ESKD) patients on regular hemodialysis. Objective: To estimate the frequency of vascular access-related infections in hemodialysis (HD) children and to assess the effect of the implementation of CDC guidelines and tools on the prevention of VARI. Subjects and Methods: This study was conducted on 110 patients with ESKD on regular hemodialysis at the Pediatric Dialysis Unit, Ain Shams University, from August 2019 to September 2021, retrospectively for one year and prospectively for another year after implementing the CDC infection control procedures. Besides full history taking, vascular access history, and thorough clinical examination, lab investigations for VARI diagnosis and echocardiography were performed. CDC infection control procedures were implemented for one year, and VARI was re-evaluated. Results: The total number of patients enrolled in the study was 110, they were 58 males (52.7%) and 52 females (47.3%). The incidence of infection per 1000 vascular access days in both years was highest in patients with NTVCs (10.447 /1000 NTVC days, 6.861 /1000 NTVCs days), followed by patients with TVCs (1.051/1000 TVC days, 0.604 /1000 TVC days) then patients with AVF, (0.141/1000 AVF days, 0.046 /1000 AVF days). The mortality from vascular access-related infections in the first year was higher than in the second year, with 5 patients (10.2%) and 1 patient (3.8%), respectively. The average duration of hospital stays (PPY) due to VARI (total admission days 8.09 and ward admission days 6.41) in the first year were significantly higher than in the second year, 4.04, 3.20 days/PPY, respectively. Conclusion: The incidence of vascular access infection (VAI) is higher with non-tunneled catheters and lowest with Arteriovenous fistulae (AVF). To reduce the risk of VARI and hospitalization, proper infection control procedures must be implemented.

EP609 - Factors affecting the recurrence rate of acute uncomplicated diverticulitis: Single-center experience

Abstract Introduction Acute diverticulitis (AD) is a prevalent condition, and the use of antibiotics for uncomplicated cases remains a topic of debate. Understanding the risk factors for recurrence is crucial for guiding treatment strategies. This study aimed to investigate the risk factors for recurrence within one year following hospital admission for AD. Methods A retrospective analysis of patients admitted to a District General Hospital (DGH) with acute diverticulitis in 2022 was conducted. The study examined patient demographics, initial observations, admitting investigations, care methods, and recurrence within a year. Statistical analysis was performed using SPSS 22 and the Chi-square test. Results The results revealed that out of 105 patients, 39 (37.1%) experienced a recurrence within a year. Notably, age, gender, and diabetes did not significantly impact recurrence (P=0.2,0.06,0.6 respectively), while a history of previous diverticulitis and white cell count (WCC) between 10-15 were associated with higher recurrence rates (P=0.032,0.011 respectively). Furthermore, Hinchey grade, use of antibiotics, and the method of care, whether ambulatory or inpatient, did not show significant differences in recurrence rates (P=0.5,0.8,0.92 respectively). Surgical intervention or interventional radiology (IR) drainage also did not affect the occurrence of recurrence (P=0.91,0.92, respectively). Conclusion The findings suggest that the use of antibiotics for uncomplicated AD may not reduce the risk of recurrence. Additionally, a history of previous admission with AD and elevated WCC were identified as significant risk factors for recurrence within one-year post-hospitalization. However, age, gender, diabetes, Hinchey grade, and interventions did not demonstrate statistical significance in predicting recurrence.

Pediatric spinal arachnoid cysts with compressive myelopathy: single-center experience and update on surgical management.

This study describes the surgical outcome of pediatric primary spinal arachnoid cysts (SACs) presenting with compressive myelopathy and gives an update on the classification and management of these rare lesions. We performed a single-center retrospective analysis of pediatric patients operated for primary spinal arachnoid cysts. The clinical and radiologic profiles and surgical outcomes of these children were analyzed. Subgroup analysis was done in the laminoplasty vs laminectomy groups to see for the development of spinal deformity. There were 10 males and seven females with a mean age of 10.4years (range:6-14years). The cysts extended to an average of 5.2 levels (range:2-8). They were extradural in seven (41%) and intradural in 10 (59%). Six intradural and four extradural cysts underwent laminectomy (n = 10) while four intradural and three extradural cysts underwent laminoplasty (n = 7). Although three out of 10 cases in the laminectomy group and none in the laminoplasty group had post-operative spinal deformity, this result was not statistically significant (p = 0.110). There was a moderate negative correlation between post-operative cord occupancy ratio (COR) and post-operative McCormick grade (Pearson correlation coefficient = -0.453, p = 0.068), suggesting that higher CORs are associated with lower McCormick grades. Symptomatic pediatric primary spinal arachnoid cysts are safely and effectively managed by marsupialization or microsurgical excision. Considering the growing age group, laminoplasty rather than laminectomy should be the standard surgical procedure to prevent late postoperative spinal deformity. Clinically significant recurrences are rare in the setting of adequate cord expansion and restored subarachnoid CSF flow following surgery.

Rhabdomyosarcoma of the Biliary Tract in Children: Analysis of Single Center Experience.

Rhabdomyosarcoma (RMS) of the biliary tract is a rare tumor in children, constituting 0.5-0.8% of all pediatric RMS. Still, it is the most common malignancy in this location in children. Due to its rarity and location, it may cause diagnostic and treatment difficulties. Above all, there are no therapeutic guidelines specific for this tumor location. The aim of the study was to present an analysis of our experience with the treatment of children with biliary tract rhabdomyosarcoma (RMS) and discuss clinical recommendations for this specific location published in the literature. A retrospective analysis of medical records of eight children with biliary tree RMS treated in one center between 1996-2022 was performed. Records of eight children, five boys and three girls aged 2 yrs 6 mo to 16 yrs 9 mo (median-6 yrs) were analyzed. All patients presented with jaundice as the first symptom. In two patients, initial diagnosis of a tumor was established. For the remaining six, the primary diagnoses were as follows: choledochal cyst-one, malformation of the biliary ducts-one, choledocholithiasis-one, cholangitis-three. In four patients, the extrahepatic bile ducts were involved; in four patients, both the intrahepatic and extrahepatic bile ducts were involved. Embryonal RMS was diagnosed in seven patients (three botryoides type). Alveolar RMS was found in one patient. Biopsy (three surgical, four during endoscopic retrograde cholangiopancreatography (ERCP)) was performed in seven patients. One child underwent primary partial tumor resection (R2). Seven patients received neoadjuvant chemotherapy, followed by delayed resection in five, including liver transplantation in one (five were R0). Two patients did not undergo surgery. Radiotherapy was administered in four patients (two in first-line treatment, two at relapse/progression). Six patients (75%) are alive with no evidence of disease, with follow-up ranging from 1.2 yrs to 27 yrs (median 11 yrs. and 4 mo.). Two patients died from disease, 2 y 9 mo and 3 y 7 mo from diagnosis. Children presenting with obstructive jaundice should be evaluated for biliary tract RMS. The treatment strategy should include biopsy and preoperative chemotherapy, followed by tumor resection and radiotherapy for residual disease and in case of relapse.

The Indispensable Role of Cardiac Catheterization Before Surgical Intervention of Pediatrics Undergoing Tetralogy of Fallot Total Correction; A Single Center Experience

Background: Inadequate pulmonary blood flow in the tetralogy of Fallot (ToF) can lead to the development of major aortopulmonary collaterals, interfering with surgical repair. Objectives: We aimed to depict the crucial role of cardiac catheterization before the surgical intervention of pediatrics undergoing ToF total correction. Methods: This retrospective cohort study was conducted on consecutive pediatric patients under two years of age who underwent ToF total correction surgery. All of the patients were diagnosed with ToF with doubled times trans-thoracic echocardiography, and the patients with high suspicion of the presence of major aortopulmonary collateral arteries (MAPCAs) underwent cardiac catheterization before ToF total correction surgery. Conversely, if the catheterization approach failed, they underwent ToF total correction surgery simultaneously with MAPCA closure. On the other hand, the patients with no evidence of MAPCA in the echocardiographic findings (as considered undiagnosed patients) underwent ToF total correction surgery. Results: Two hundred fifty eligible pediatrics who underwent ToF total correction surgery participated. The ICU stay (P < 0.001), hospital stay (P < 0.001), and weaning time from mechanical ventilation (P < 0.001) in patients with correct diagnosis before the ToF surgery were statistically lower than misdiagnosed open MAPCA patients who underwent the surgical approach of the ToF group. Some intra-operative and postoperative parameters showed no statistical differences between patients in the two groups. We had three mortality in the patients whose trans-thoracic echocardiography (TTE) findings were undiagnosed as there was no evidence to rely on the presence of open MAPCA. Conclusions: Based on this study's findings, we strongly recommend that all ToF patients undergo cardiopulmonary catheterization before the surgical approach.