Multidisciplinary Decision Research Articles

Single-Center Experience in Patients with Mixed Gonadal Dysgenesis.

Mixed gonadal dysgenesis (MGD) is an uncommon chromosomal Disorder of Sexual Development (DSD). There is insufficient information regarding clinical findings and growth patterns. This study aimed to provide more information about mixed gonadal dysgenesis, which has not yet been sufficiently defined. Data from 10 patients diagnosed with mixed gonadal dysgenesis were retrospectively reviewed. Clinical presentations, complaints at admission, imaging, genetic results, and treatments received by the patients were examined. Gonadal status and the gender of the patients were reared and evaluated by a multidisciplinary council decision. If received, growth hormone treatment doses and height gains were examined. The patients' ages at admission range from 6 months to 17.5 years. The median height SDS of the patients was -0.75 (2.73), the mean body weight SDS was -0.49 (±1.46), and the mean body mass index (BMI) SDS was 0.26 (±0.97). The complaints at admission varied, including ambiguous genitalia, short stature, and absence of menstruation. Some patients are completely in the female phenotype, while some are inadequately virilized male phenotype. External Masculinization Score (EMS) ranges from 1 to 6.5. The decision to raise 6 patients as female and 4 patients as male was made by a multidisciplinary council. Growth hormone treatment was administered to patients raised as female and diagnosed with short stature. The height SDS gain in treated patients was 0.42 (±0.49). Due to its rarity and varied clinical presentation, our knowledge about mixed gonadal dysgenesis is limited. Therefore, early diagnosis and individualized treatment plans are crucial for this patient group.

277. FLUORESCENCE-GUIDED ANASTOMOSIS DURING SINGLE-PORT VATS IVOR LEWIS ESOPHAGECTOMY

Abstract Background Minimally Invasive Esophagectomy (MIE) is the standard approach for esophageal cancer. The thoracic part is performed using multiple ports during video or robotic approach with CO2 inflation of 8-10 mmHg. There are recent publications of successful lung & thymic surgeries utilizing single port. This video describes our technique for performing ICG-guided intrathoracic anastomosis during single-port Ivor Lewis (IL) esophagectomy. Methods The surgery was performed on a 58-year-old male patient with Siwert type 1 adenocarcinoma (cT3, N0 M0). The patient underwent 4 cycles of neoadjuvant FLOT chemotherapy following a Multidisciplinary tumour board decision. Single-port Ivor Lewis (IL) esophagectomy was planned after three weeks of last cycle. Results Following the laparoscopic abdominal phase with extracorporeal gastric conduit formation and feeding jejunostomy, the patient was placed in a semiprone position. The thoracic phase was performed through single-port (4 cm in the 6th intercostal) VATS without CO2. The intrathoracic anastomosis was performed with a linear stapler in side-to-side manner under 1.5 ml indocyanine green (ICG) guidance. Thoracic phase time was 65 minutes. There were no intraoperative and postoperative complications. The pain score was three without opioid use. The patient was allowed orally on day five and discharged home on the eighth postoperative day. Conclusion Single port VATS IL MIE is a quick and safe surgical technique. Optics, instrumentations and surgical stapler maneuvering through a single port require a learning curve but are not difficult in our experience. A larger cohort and long-term follow-up are necessary for wider utilization of this approach. https://wetransfer.com/downloads/21555d68e61e7f962f2c33ed061b72dc20240319163828/b0b0feeea6f7a7ba47a04bb26ac96c2220240319163854/83de2d

Chest wall resection and reconstruction for primary chest wall sarcomas: Analysis of survival, predictors of outcome, and long-term functional status

ObjectivesWe aimed to analyze survival, predictors of outcome, and the long-term functional status of patients with a diagnosis of primary chest wall sarcoma who undergo chest wall resection and reconstruction. MethodsWe analyzed a prospectively maintained database, including all patients operated on between 2008 and 2021. The primary outcome measures were overall and disease-free survival and analyses were employed to determine the risk factors for poor survival and recurrence. ResultsOne hundred thirty-nine patients were included, 55% were men. The majority (96%) had an R0 resection and 75.1% had no postoperative complications up to 30 days postprocedure; median length of hospital stay was 7 days (range, 6-10 days). Median overall and disease-free survival was 58.8 and 53.6 months, respectively. For those alive, at long-term follow-up, 80% had a Medical Research Council dyspnea score of 0 and Karnofsky index >80%. Survival and mortality rates were better in chondrosarcomas compared with nonchondromatous sarcomas (P < .05). Previous history of radiotherapy, previous history of cancer, the type of sarcoma (Ewing's or soft tissue), the need for adjuvant treatment and tumor grade were significant predictors of mortality and recurrence on univariate testing. Extended resection, a higher number of ribs removed, and the incidence of postoperative complications were significantly associated with a worse postoperative Medical Research Council dyspnea score. ConclusionsCareful patient selection and multidisciplinary decision making is crucial. This leads to clear resection margins, good overall, and disease-free survival and good functional outcomes.

Detection of liver metastases in patients with pancreatic adenocarcinoma: MRI versus CT

Introduction. Detection of liver metastases is important to guide therapeutic management of pancreatic cancer. Data suggest that magnetic resonance imaging (MRI) is more sensitive than computed tomography (CT) for the diagnosis of liver metastases from pancreatic cancer. However, MRI is currently not recommended in several guidelines. Purpose. To compare the performance of CT vs MRI in detection of liver metastases in patients with pancreatic adenocarcinoma. Methods. This was a retrospective single-center study over 24 months from January 2020 to December 2021 of patients suspected of having PDAC. All patients underwent contrast-enhanced triple phase CT and contrast-enhanced MRI/MRCP. The CT and MRI assessment of resectability and detection of metastases were performed according to NCCN version 1.2024 criteria by one observer based on clinical report. Composite reference standard based on decision of operability (based on multidisciplinary tumor board decision), surgical findings (delay of 2 to 3 weeks after imaging) and histopathologic findings. Results. 117 patients (M/F 52/65, mean age 62±11y) were included. The mean tumor size was 43.6±15.2mm (add range). 42/46 patients (34.2%/39.3%) were diagnosed with metastatic disease on CT/MRI, 40/36 patients (34.2%/30.8%) were resectable, 20/16 patients (17.1%/13.7%) were borderline resectable and 15/19 patients (12.8%/16.2%) were locally advanced. The detection of liver metastases on CT and MRI was compared with surgical findings in 45 patients (38.5%). The analysis showed that MRI had superior performance to CT, with sensitivity at 100% vs 60% and specificity at 97.1% vs 97.1%, PPV at 90.9% vs 85.7% and NPV 100% vs 89.5% (MRI/CT). Conclusion. MRI is more sensitive than CT in detection of liver metastases in patients with pancreatic adenocarcinoma.

Chronic thromboembolic pulmonary disease.

Chronic thromboembolic pulmonary hypertension is a complication of pulmonary embolism and a treatable cause of pulmonary hypertension. The pathology is a unique combination of mechanical obstruction due to failure of clot resolution, and a variable degree of microvascular disease, that both contribute to pulmonary vascular resistance. Accordingly, multiple treatments have been developed to target the disease components. However, accurate diagnosis is often delayed. Evaluation includes high-quality imaging modalities, necessary for disease confirmation and for appropriate treatment planning. All patients with chronic thromboembolic pulmonary disease, and especially those with pulmonary hypertension, should be referred to expert centres for multidisciplinary team decision on treatment. The first decision remains assessment of operability, and the best improvement in symptoms and survival is achieved by the mechanical therapies, pulmonary endarterectomy and balloon pulmonary angioplasty. With the advances in multimodal therapies, excellent outcomes can be achieved with 3-year survival of >90%.

Achondroplasia current concept of orthopaedic management.

Achondroplasia, the most common form of inherited disproportionate short stature, is caused by mutations in the fibroblast growth factor receptor 3 gene. The typical clinical features of achondroplasia include short stature, rhizomelic disproportion, joint hyperlaxity, spinal deformity and deformity of the upper and lower limbs. The latter are among the challenges of state-of-the-art orthopaedic treatment plans and significantly contribute to the burden of the disease in individuals with achondroplasia. Multidisciplinary preoperative individual decision-making concerning surgical interventions should be considered. New medical treatments for achondroplasia have been developed and (some) have been approved for clinical use in several countries. While the number of research articles on achondroplasia is increasing rapidly, many unknown or controversial orthopaedic topics remain. Furthermore, in view of new medical developments with improvements in growth and potentially other effects, the timing and algorithms of orthopaedic treatments (e.g. guided growth, limb lengthening and deformity correction) need to be re-evaluated. While standing height is the primary research focus in medical therapy, it is crucial to comprehensively assess orthopaedic parameters in this multifactorial disease. The current treatment of patients with achondroplasia requires specialised multidisciplinary centres with transitional care and individual orthopaedic counselling.

Management of periprosthetic joint infection of the shoulder: A narrative review

Evidence for management of shoulder periprosthetic joint infection (PJI) has traditionally originated from the hip and knee literature. The differing microbiome, anatomy and implants used in the shoulder mean this evidence is not always directly transferrable. The 2018 Philadelphia International Consensus Meeting for the first-time produced evidence-based guidelines and diagnostic criteria relating specifically to PJI of the shoulder. These guidelines and criteria recognize the pathogenicity of lower virulence organisms in the shoulder which often means clinical presentation is less obvious than other joints.The role of Cutibacterium acnes in shoulder PJI is the subject of increasing basic science and clinical research and advances in microbiological research may help to understand the pathology behind shoulder infections. There is new evidence that outcomes after revision shoulder arthroplasty are dependent on the virulence of the causative organism. An individualised approach to treatment considering host factors, organism, soft tissues and bone stock is recommended.Debate continues in the literature regarding the indications of one- or two-stage revision and the latest evidence is discussed and synthesized in this review article. We advocate careful multidisciplinary team decision making for cases of shoulder PJI and recognize a limited role for debridement and implant retention in acute shoulder PJI (<6 weeks). There appears to be a role for one-stage revision in lower risk cases with low virulence organisms but caution against its’ universal adoption. In higher risk or complex cases, there remains a clear role for two-stage revision arthroplasty, and we detail the specifics of this protocol and procedure from our tertiary shoulder and elbow unit.

Updating a conceptual model of effective symptom management in palliative care to include patient and carer perspective: a qualitative study

BackgroundA conceptual model of effective symptom management was previously developed from interviews with multidisciplinary healthcare professionals (HCP) working in English hospices. Here we aimed to answer the question; does a HCP data-derived model represent the experience of patients and carers of people with advanced cancer?MethodsSemi-structured interviews were undertaken with six patients with advanced cancer and six carers to gain an in-depth understanding of their experience of symptom management. Analysis was based on the framework method; transcription, familiarisation, coding, applying analytical framework (conceptual model), charting, interpretation. Inductive framework analysis was used to align data with themes in the existing model. A deductive approach was also used to identify new themes.ResultsThe experience of patients and carers aligned with key steps of engagement, decision making, partnership and delivery in the HCP-based model. The data aligned with 18 of 23 themes. These were; Role definition and boundaries, Multidisciplinary team decision making, Availability of services/staff, Clinician-Patient relationship/rapport, Patient preferences, Patient characteristics, Quality of life versus treatment need, Staff time/burden, Psychological support -informal, Appropriate understanding, expectations, acceptance and goals- patients, Appropriate understanding, expectations, acceptance and goals-HCPs, Appropriate understanding, expectations, acceptance and goals- family friends, carers, Professional, service and referral factors, Continuity of care, Multidisciplinary team working, Palliative care philosophy and culture, Physical environment and facilities, Referral process and delays. Four additional patient and carer-derived themes were identified: Carer Burden, Communication, Medicines management and COVID-19. Constructs that did not align were Experience (of staff), Training (of staff), Guidelines and evidence, Psychological support (for staff) and Formal psychological support (for patients).ConclusionsA healthcare professional-based conceptual model of effective symptom management aligned well with the experience of patients with advanced cancer and their carers. Additional domains were identified. We make four recommendations for change arising from this research. Routine appraisal and acknowledgement of carer burden, medicine management tasks and previous experience in healthcare roles; improved access to communication skills training for staff and review of patient communication needs. Further research should explore the symptom management experience of those living alone and how these people can be better supported.

Best-Practice Biomarker Testing of Oesophago-Gastric Cancer in the UK: Expert Consensus Recommendations Developed Using a Modified Delphi

AimsOesophago-gastric cancers (OGCs) are amongst the most commonly diagnosed malignancies worldwide and are associated with high disease-related mortality. Predictive biomarkers are molecules that can be objectively measured and used to indicate a likely response to therapeutic intervention, thus facilitating individualised cancer therapy. However, there remains variation in uptake and implementation of biomarker testing across the UK. Materials and methodsWe conducted a modified Delphi study to formulate consensus recommendations for best-practice biomarker testing of OGC in the UK. We employed two rounds of online questionnaires followed by a virtual consensus meeting. Biomarkers for discussion included HER2, MSI/MMR, and PD-L1. Topics comprised the overall biomarker pathway, pre-analytical, analytical, and post-analytical considerations, including challenges in current practice. ResultsTwenty-six and eighteen participants completed the first and second round Delphi questionnaire, respectively, with an even split of pathologists and oncologists from across the UK. There was consensus (>80% agreement) across several topics, including the requirements for standardisation of the pathway, which must include coordination throughout the tissue journey, requirements for a quality-assured process to ensure accuracy and validity of testing, plus the need for clear, detailed information on the pathology report to support treatment decisions. There was consensus amongst oncologists regarding reflex testing of all biomarkers depending on histology; however, concerns over capacity in relation to workload and availability of pathologists were evident among the pathologists. Overall, participants were in the opinion that reflex testing improves the speed of treatment decisions and improves patient care. ConclusionThe recommendations reflect best-practices and should be implemented to support rapid multidisciplinary team decision-making within oesophago-gastric cancer. Results reflect the need for standardisation and demonstrate the challenges faced in clinical practice by those requesting and testing biomarkers for oesophago-gastric cancer, suggesting significant concerns relating to pathologist capacity.

72277 - Multidisciplinary treatment decision associated with improved survival in gastric cancer patients; a nationwide cohort study in 7818 patients

72277 - Multidisciplinary treatment decision associated with improved survival in gastric cancer patients; a nationwide cohort study in 7818 patients

Controlled Study of Multidisciplinary Team Decision-making Intervention to Prevent Falls in the Elderly

Controlled Study of Multidisciplinary Team Decision-making Intervention to Prevent Falls in the Elderly

Outcomes of Mitral Valve Regurgitation Management after Expert Multidisciplinary Valve Team Evaluation.

Background/Objectives: Mitral regurgitation (MR) affects millions worldwide, necessitating timely intervention. There are significant clinical challenges in the conservative management of MR, leaving a knowledge gap regarding the impact of multidisciplinary decision-making on treatment outcomes. This study aimed to provide insights into the impact of multidisciplinary decision-making on the survival outcomes of MR patients, focusing on conservative approaches. Methods: This study retrospectively analyzes 1365 patients evaluated by an expert multidisciplinary heart team (MDT) in a single center from 2015 to 2022. Treatments included surgery, catheter-based interventions, and conservative management. Propensity matching was utilized to compare surgery and conservative approaches. Results: Surgical intervention was associated with superior long-term survival outcomes compared to conservative and catheter-based treatments, particularly for degenerative MR (DMR). Survival rates of patients deemed by the MDT to have non-severe DMR were comparable to surgical patients (HR 1.07, 95% CI: 0.37-3.12, p = 0.90). However, non-severe functional MR (FMR) patients trended towards elevated mortality risk (HR 1.77, 95% CI: 0.94-3.31, p = 0.07). Pharmacological treatment for DMR was associated with significantly higher mortality compared to surgery (HR 8.0, 95% CI: 1.78-36.03, p = 0.001). Functional MR patients treated pharmacologically exhibited a non-significantly higher mortality risk compared to surgical intervention (HR 1.93, 95% CI: 0.77-4.77, p = 0.20). Conclusions: Survival analysis revealed significant benefits for surgical intervention, contrasting with elevated mortality risks associated with conservative management. "Watchful waiting" may be appropriate for non-severe DMR, while FMR may require closer monitoring. Further research is needed to assess the impact of regular follow-up or delayed surgery on survival rates, as pharmacological therapy has limited long-term efficacy for DMR.

Consistency of CSCO AI with Multidisciplinary Clinical Decision-Making Teams in Breast Cancer: A Retrospective Study.

The Chinese Society of Clinical Oncology Artificial Intelligence System (CSCO AI) serves as a clinical decision support system developed utilizing Chinese breast cancer data. Our study delved into the congruence between breast cancer treatment recommendations provided by CSCO AI and their practical application in clinical settings. A retrospective analysis encompassed 537 breast cancer patients treated at the Second Affiliated Hospital of Anhui Medical University between January 2017 and December 2022. Proficient senior oncology researchers manually input patient data into the CSCO AI system. "Consistent" and "Inconsistent" treatment categories were defined by aligning our treatment protocols with the classification system in the CSCO AI recommendations. Cases that initially showed inconsistency underwent a second evaluation by the Multi-Disciplinary Treatment (MDT) team at the hospital. Concordance was achieved when MDTs' treatment suggestions were in the 'Consistent' categories. An impressive 80.4% concurrence was observed between actual treatment protocols and CSCO AI recommendations across all breast cancer patients. Notably, the alignment was markedly higher for stage I (85.02%) and stage III (88.46%) patients in contrast to stage II patients (76.06%, P=0.023). Moreover, there was a significant concordance between invasive ductal carcinoma and lobular carcinoma (88.46%). Interestingly, triple-negative breast cancer (TNBC) exhibited a high concordance rate (87.50%) compared to other molecular subtypes. When contrasting MDT-recommended treatments with CSCO AI decisions, an overall 92.4% agreement was established. Furthermore, a logistic multivariate analysis highlighted the statistical significance of age, menstrual status, tumor type, molecular subtype, tumor size, and TNM stage in influencing consistency. In the realm of breast cancer treatment, the alignment between recommendations offered by CSCO AI and those from MDT is predominant. CSCO AI can be a useful tool for breast cancer treatment decisions.

Rectal metastasis arising from breast cancer: a case report.

Breast cancer is the most common cancer among women worldwide. Breast cancer often metastasizes to the regional lymph nodes, bone, brain, liver, and lungs, whereas gastrointestinal tract metastases are rare. Herein, we present a rare case of rectal metastasis from breast cancer that occurred during palliative chemotherapy. A 69-year-old female with a history of invasive ductal carcinoma, negative for hormonal receptors and positive for human epidermal growth factor receptor 2 (HER2) receptor, underwent various treatments, including neoadjuvant chemotherapy, breast-conserving surgery, and adjuvant therapy. Eight months postoperatively, the patient experienced axillary lymph node recurrence, requiring palliative chemotherapy. Despite ongoing treatment, metastatic lesions were confirmed in the lungs and pleura. During palliative chemotherapy, the patient developed anal pain, and subsequent examination revealed an infiltrating rectal lesion. Despite histological confirmation of metastatic breast carcinoma and tubular adenoma, a multidisciplinary decision was made regarding palliative chemotherapy over surgical intervention. Eribulin was administered, but due to the patient's inability to tolerate the treatment, she passed away 3 months after rectal lesion diagnosis. Although breast cancer metastasis to the rectum is rare, clinicians should consider the possibility of rectal involvement and perform a digital rectal examination if anal symptoms are present.

Synchronous Carcinoma Cervix with Renal Cell Carcinoma: An Incidental Finding

Background In India, renal cell carcinoma (RCC) is the most frequent kidney cancer with an increasing tendency, whereas cervical cancer is the second most common disease among women. Synchronicity of RCC with other malignancies is not uncommon, but with the cervical cancer is rare.  Case description A 64-year-old, postmenopausal female, presented with complaint of bleeding per vagina and was diagnosed with carcinoma cervix and later on metastatic work-up also diagnosed with right RCC. After multidisciplinary discussion, patient planned for management for cervix then followed by renal lesion. Patient received radical external beam radiotherapy followed by brachytherapy for carcinoma cervix and underwent nephrectomy for right RCC.  Conclusion Synchronous cases of cervical cancer and renal cell carcinoma are very rare. Advancement in diagnostic protocol makes these kind of cases common and multidisciplinary team decisions are required for their management.

Multidisciplinary Management of Isocitrate Dehydrogenase-Mutated Gliomas in a Contemporary Molecularly Defined Era.

Mutations in isocitrate dehydrogenase (IDH) genes, an early step in the ontogeny of lower-grade gliomas, induce global epigenetic changes characterized by a hypermethylation phenotype and are critical to tumor classification, treatment decision making, and estimation of patient prognosis. The introduction of IDH inhibitors to block the oncogenic neomorphic function of the mutated protein has resulted in new therapeutic options for these patients. To appreciate the implications of these recent IDH inhibitor results, it is important to juxtapose historical outcomes with chemoradiotherapy. Herein, we rationally evaluate recent IDH inhibitor data within historical precedents to guide contemporary decisions regarding the role of observation, maximal safe resection, adjuvant therapies, and the import of patient and tumor variables. The biological underpinnings of the IDH pathway and the mechanisms, impact, and limitations of IDH inhibitors, the actual magnitude of tumor regression and patient benefit, and emergence of resistance pathways are presented to guide future trial development. Management in the current, molecularly defined era will require careful patient selection and risk factor assessment, followed by an open dialog about the results of studies such as INDIGO, as well as mature data from legacy trials, and a discussion about risk-versus-benefit for the choice of treatment, with multidisciplinary decision making as an absolute prerequisite.

Impact of regulatory t cells in peripheral blood on pathological complete response in patients with locally advanced gastric or gastroesophageal junction cancer undergoing neoadjuvant chemoimmunotherapy.

e16081 Background: Neoadjuvant chemotherapy has become the standard treatment for locally advanced gastric or gastroesophageal junction (G/GEJ) cancer. With the widely clinical application of immune checkpoint inhibitors, represented by anti-PD-1 antibodies, an increasing number of patients with locally advanced G/GEJ cancer are undergoing neoadjuvant chemoimmunotherapy. However, there is currently no effective predictive marker for neoadjuvant chemoimmunotherapy in locally advanced G/GEJ cancer. We explored the role of peripheral blood regulatory T (Treg) cells in patients with locally advanced G/GEJ cancer. Methods: We retrospectively analyzed lymphocyte subpopulations before and after treatment in patients with locally advanced G/GEJ cancer who received neoadjuvant chemotherapy combined with anti-PD-1 antibodies and curative surgery. Inclusion criteria were: 1) Pathologically confirmed adenocarcinoma of the G/GEJ; 2) Clinically staged as gastric cT3-4 or N+ without distant metastasis by enhanced CT and endoscopic ultrasound examination; 3) Patients requiring neoadjuvant treatment according to gastric cancer multidisciplinary team decisions; 4) Receipt of neoadjuvant treatment with chemotherapy combined with PD-1 antibodies; 5) Completion of 3-4 cycles of treatment; 6) Underwent curative resection; 7) Lymphocyte subpopulation testing performed before the first and second cycles. Results: From July 1, 2020, to May 31, 2023, 85 patients were included. All 85 patients underwent D2 radical surgery and achieved R0 resection, with 21 patients achieving pathological complete response (pCR), resulting in a pCR rate of 24.7% (95% confidence interval (CI) 15.3-34.1%). There were no statistically significant differences in baseline characteristics between the pCR and non-pCR groups. There were no statistically significant differences in lymphocyte subpopulations between the baseline pCR and non-pCR patient groups. After one cycle of treatment (before the second cycle), there were statistically significant differences in lymphocytes, T lymphocytes, and Treg cells between the two groups. Further analysis of the absolute changes in lymphocyte subpopulations before and after one cycle of treatment revealed a greater decrease in Treg cells in the pCR group. Based on the increase or decrease in Treg cells after one cycle of treatment, the 85 patients were divided into Treg cell increase and decrease groups. The pCR rate was 11.4% (4/35, 95% CI 0.3-22.5%) in the Treg cell increase group and 34% (17/50, 95% CI 20.4-47.6%) in the Treg cell decrease group, with a statistically significant difference between the two groups ( P= 0.022). Conclusions: In locally advanced G/GEJ cancer, changes in Treg cells after one cycle of neoadjuvant treatment may serve as a predictive marker for achieving pCR.

Total Neoadjuvant Therapy for Rectal Cancer: Which Regimens to Use?

Total neoadjuvant therapy (TNT) is a novel strategy for rectal cancer that administers both (chemo)radiotherapy and systemic chemotherapy before surgery. TNT is expected to improve treatment compliance, tumor regression, organ preservation, and oncologic outcomes. Multiple TNT regimens are currently available with various combinations of the treatments including induction or consolidation chemotherapy, triplet or doublet chemotherapy, and long-course chemoradiotherapy or short-course radiotherapy. Evidence on TNT is rapidly evolving with new data on clinical trials, and no definitive consensus has been established on which regimens to use for improving outcomes. Clinicians need to understand the advantages and limitations of the available regimens for multidisciplinary decision making. This article reviews currently available evidence on TNT for rectal cancer. A decision making flow chart is provided for tailor-made use of TNT regimens based on tumor location and local and systemic risk.

Genito-urinary Reconstruction in Female Children With Congenital Adrenal Hyperplasia: Favorable Surgical Outcomes can be Achieved by Contemporary Techniques and a Dedicated Multidisciplinary Management

IntroductionCongenital adrenal hyperplasia (CAH) is the most common cause of genital atypia in females. A dedicated multidisciplinary team (MDT) should be included for an optimal management. Here, we aimed to review our surgical experience and to assess long-term urinary, gynecological and endocrine outcomes after primary genitoplasty in this specific cohort. MethodsPatients born with CAH and who underwent feminizing genitoplasty in our institution were retrospectively identified (2001–2021). We analyzed patients’ characteristics, intraoperative details, and postoperative urinary, gynecological, and endocrine outcomes. ResultsForty patients were included and followed-up for a median (IQR) time of 7 (1–19) years. Thirty-eight (95%) had 21-hydroxylase deficiency. After multidisciplinary decision and written consent from patient and/or family, a single-stage reconstructive surgery was performed at a median age of 10 (3–165) months. Median length of hospital stay was 5 (1–7) days. Procedures were: PUM (N = 35 (87.5%)), TUM (N = 3 (7.5%)), urogenital mobilization was unnecessary in 2 (5%). Reduction clitoroplasty was done in 33 (82.5%) patients. Only 3 (7.5%) experienced significant Clavien-Dindo complications requiring additional surgery during the follow-up period. Recurrent urinary tract infections (UTI) occurred in 6 (15%), one required ureteric reimplantation for symptomatic high-grade vesicoureteric reflux. All patients over 3 years were toilet-trained without incontinence. Severe vaginal stenosis occurred in 1 (2.5%) patient. In patients who achieved puberty, 6/9 had vaginal calibration at a median age of 17.3 (16–21) years without detected stenosis. One (2.5%) had major hypertrophy of the right labia minora requiring labiaplasty. Nine (22.5%) reached puberty. Two (5%) patients developed acne/hirsutism. Short stature was noted in 11 (27.5%) and obesity in 18 (45%). ConclusionBased on our contemporary series, genitourinary reconstructive surgery for female patients born with CAH is technically feasible and safe with a low complication rate. A regular follow-up with a MDT to assess long-term complications is necessary, and it is vital to inform patients and families about the different management options with all the risks and benefits of surgery. Type of the studyoriginal research, clinical research. Level of evidenceLevel 3 retrospective study.

Total Knee Arthroplasty in Patients Who Have Skeletal Dysplasia: A Center’s Experience With a Mean 9-Year Follow-Up

BackgroundTotal knee arthroplasty (TKA) in patients who have skeletal dysplasia (SD) is a technically challenging surgery due to deformity, joint contracture, and associated comorbidities. Patients presenting with this condition have traditionally been treated with conservative measures, leading to poor outcomes. The aim of this study was to follow-up on patients who had SD following TKA, specifically with regards to clinical outcomes. MethodsA total of 31 knees (22 patients) with SD that had undergone TKA in our institution were included in our study. The mean follow-up from index surgery was 110.3 months (range: 20 to 291). The type of dysplasia, implant used, and clinical outcomes with patient-reported outcome measures are presented. ResultsThere were 8 patients (36.3%) who had a diagnosis of achondroplasia, followed by multiple epiphyseal dysplasia (31.8%) and spondyloepiphyseal dysplasia (22.7%). There were 14 men and 8 women who had a mean age of 51 years (range: 28 to 73). Custom implants were required in 12 cases (38.7%), custom jigs were used in 6 cases (19.4%), and robotic-assisted surgery was used in 2 (6.5%) TKAs. Hinged prostheses were used in 17 cases (54.8%), posterior-stabilized in 9 (29.0%), and cruciate-retaining implants in 5 (16.1%). There was 1 patient who sustained an intraoperative medial tibial plateau fracture treated with concomitant open reduction and internal fixation. There was 1 revision that occurred during the follow-up period with a patella resurfacing for continued anterior knee pain. Postoperatively, Oxford Knee Scores improved on average by 12.2 points. The 10-year and 20-year all-cause revision-free survival was 96.8, respectively. ConclusionsDespite the technical challenges and complexity associated with this unique patient cohort, we demonstrated excellent implant survivorship and clinical outcomes post-TKA with mid-term to long-term follow-up of more than 20 years. We recommend preoperative cross-sectional imaging for precise planning and implant templating with multidisciplinary team decision-making. Despite our results, functional outcomes remain inferior to primary arthroplasty within the general population, although we still recommend this treatment modality to appropriately counseled patients. Level of EvidenceIII.