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Large ileal lipomas over 2 cm can cause symptoms, that may require a resection. Due to the narrow lumen and thin walls of the ileum, endoscopic treatments can have a high risk of adverse events and require technical expertise, thus surgical resection is currently the mainstay of treatment. To overcome the technical challenges, we developed a novel method to endoscopically resect terminal ileal lipomas. The technique involves extracting the lesion into the cecum, which creates sufficient space to maneuver, and a better field of view. The lipoma is resected with endoscopic mucosal resection or endoscopic submucosal dissection. The appearance of the lipoma protruding out of the ileocecal valve resembles that of a tongue sticking out of the mouth, thus we named this the "tongue out technique". To assess the technical feasibility of this method, we retrospectively analyzed seven cases of terminal ileal lipoma that were endoscopically resected using the "tongue out technique" at NTT Medical Center Tokyo between January 2017 and October 2023. Technical success was 100% and en bloc resection was achieved in all cases. The median size was 31 (14-55) mm. Three cases were resected with endoscopic mucosal resection while endoscopic submucosal dissection was performed on the other four cases. There was one case of delayed post-endoscopic mucosal resection bleeding, which was caused by clip dislodgement. There were no perforations. No recurrence of the lipoma or associated symptoms have been observed. This new technique can allow more ileal lipomas to be treated with minimally invasive and organ-preserving endoscopic procedures.
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Objectives: We aimed to identify independent factors for intraoperative endoscopic lens cloudiness during gastric and colorectal endoscopic submucosal dissections, investigate the effectiveness of Cleastay, an endoscope anti-fog solution, and examine factors associated with severe submucosal fat deposition. Methods: A total of 220 patients who underwent gastric or colorectal endoscopic submucosal dissections in two institutions between January 2022 and October 2023 were included. Significant factors related to cloudiness were determined using univariate and multivariate analyses. Patient background and tumor characteristics related to severe submucosal fat deposition were investigated, and the degree of intraoperative endoscopic lens cloudiness and outcomes were compared between the Cleash and Cleastay groups. Results: In the multivariate analysis, factors increasing lens cloudiness included long procedure time (odds ratio [OR], 17.51; 95% confidence interval [CI], 1.52-202.08), stomach (vs. colon; OR, 5.08; 95% CI, 1.99-12.96), and severe submucosal fat deposition (OR, 12.19; 95% CI, 5.02-29.60). Conversely, the use of Cleastay (vs. Cleash; OR, 0.066; 95% CI, 0.021-0.21) was identified as a factor reducing cloudiness. Location analysis revealed that severe submucosal fat deposition was more common in the upper stomach and right colon. Conclusions: It was suggested that Cleastay is more useful for endoscopic submucosal dissection of the upper stomach and right colon, where severe submucosal fat deposition is expected.
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A 73-year-old male patient was referred to us with a long Barrett's esophagus (BE). He had a history of pulmonary embolism under anticoagulant therapy. Esophagogastroduodenoscopy showed a C8M9 BE with no macroscopic lesions. Random biopsies from the BE revealed multifocal high-grade dysplasia. The case was discussed in a multidisciplinary team conference and the decision for full resection of BE with endoscopic submucosal dissection (ESD) was made. Considering the large ESD resection and the high risk of stricture, we developed a novel preventive technique: the "steroid lifting method" for submucosal injection during ESD. Complete circumferential ESD with en bloc resection was performed using the "steroid lifting method", without adverse events. Oral liquids were initiated on day 1 and the patient was discharged on day 4. Oral prednisolone (30 mg per day) was started and tapered for a total of 6 weeks. The pathological examination confirmed multifocal high-grade dysplasia, with radical and curative resection. The patient had neither stricture, dysphagia nor recurrence of Barrett's mucosa at the 2, 6, 12, and 24-month follow-up. International guidelines recommend oral prednisolone and triamcinolone injection to prevent stricture formation in large ESD of esophageal squamous cell carcinoma. However, there is no solid data on BE ESD. The risk factors for stricture formation and the optimal preventive management after large BE ESD is not known. The "steroid lifting method" might be an option in this context. Large prospective studies addressing stricture formation and preventive measures on BE ESD are necessary.
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Endoscopic submucosal dissection (ESD) is a transformative advancement in the endoscopic management of superficial gastrointestinal lesions. Initially conceived for the treatment of early gastric cancer, ESD has demonstrated proficiency in achieving en-bloc resection of superficial gastrointestinal lesions. ESD has experienced widespread acceptance in Japan and East Asia; however, its adoption in the USA remains delayed. This initial hesitancy could be attributed to procedural complexity and training demands; nonetheless, recently, ESD has been gaining popularity in the USA. This is due to the advancements in endoscopic technology, tailored training programs, and cumulative evidence regarding the efficacy and safety of ESDs. This review aimed to deliberate the historical progress, current implementation, and prospective trajectory of ESDs in the USA. With ongoing clinical research, technological integration, and educational efforts, ESD is likely to become the gold standard for managing large gastrointesitinal lesions. This progress marks an imperative step toward less invasive, more precise, and patient-centric approaches regarding advanced therapeutic endoscopy in the USA.
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Objectives: For early gastrointestinal lesions, size is an important factor in the selection of treatment. Virtual scale endoscope (VSE) is a newly developed endoscope that can measure size more accurately than visual measurement. This study aimed to investigate whether VSE measurement is accurate for early gastrointestinal lesions of various sizes and morphologies. Methods: This study prospectively enrolled patients with early gastrointestinal lesions ≤20 mm in size visually. Lesion sizes were measured in the gastrointestinal tract visually, on endoscopic resection specimens with VSE, and finally on endoscopic resection specimens using a ruler. The primary endpoint was the normalized difference (ND) of VSE measurement. The secondary endpoints were the ND of visual measurement and the variation between NDs of VSE and visual measurements. ND was calculated as (100 × [measured size - true size] / true size) (%). True size was defined as size measured using a ruler. Results: This study included 60 lesions from April 2022 to December 2022, with 20 each in the esophagus, stomach, and colon. The lesion size was 14.0 ± 6.3 mm (mean ± standard deviation). Morphologies were protruded, slightly elevated, and flat or slightly depressed type in 8, 24, and 28 lesions, respectively. The primary endpoint was 0.3 ± 8.8%. In the secondary endpoints, the ND of visual measurement was -1.7 ± 29.3%, and the variability was significantly smaller in the ND of VSE measurement than in that of visual measurement (p < 0.001, F-test). Conclusions: VSE measurement is accurate for early gastrointestinal lesions of various sizes and morphologies.
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A 56-year-old male patient was diagnosed with a submucosal tumor in the fundus of the stomach. The conventional operation method is endoscopic submucosal dissection. We present a case of rapid tumor resection without employing traditional endoscopic submucosal dissection instruments such as a mucotomy knife and endoscopic injection needle, resulting in substantial cost savings for the patients.
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Objectives: A relationship between endoscopic submucosal dissection (ESD) and deep vein thrombosis has been recognized. We previously reported that a high corrected midazolam dose (total midazolam dose/initial dose of midazolam used to induce sedation) is related to elevated D-dimer levels after ESD. In this study, the effect of compression stockings (CSs) in preventing thrombosis following ESD under sedation was evaluated by measuring D-dimer levels before and after ESD. Methods: The participants were patients who underwent ESD for upper gastrointestinal tumors during the period between April 2018 and October 2022. Patients with pre-ESD D-dimer levels ≥1.6 µg/m and patients with corrected midazolam doses ≤3.0 were excluded. A retrospective investigation of the relationship between CS use and high post-ESD D-dimer levels (difference in D-dimer levels ≥1.0 µg/mL between before and after ESD) was conducted. Results: There were 27 patients in the non-CS group (NCS) and 33 patients in the CS group. The number of patients with high post-ESD D-dimer levels was 13 (48.2%) in the non-CS group and six (18.2%) in the CS group; the number in the CS group was significantly lower (p = 0.024). On logistic regression analysis, a relationship was seen between the wearing of CSs and a lower number of patients with high post-ESD D-dimer levels (odds ratio 0.24, 95% confidence interval 0.08-0.79, p = 0.019). Conclusion: Wearing CSs was related to a lower risk of high post-ESD D-dimer levels. This result suggests that thrombus formation is a cause of elevated D-dimer levels after ESD.
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Objectives: Endoscopic resection (ER) for gastric submucosal tumors (SMTs) has gained prominence in recent years, with studies emerging from various countries. However, there is a paucity of reports from Japan. We aimed to elucidate the efficacy and safety of ER for gastric SMT in Japan. Methods: In this retrospective observational study, we investigated the outcomes of consecutive patients who underwent ER for gastric SMT from January 2017 to May 2023. The outcome variables assessed included the complete resection rate, procedure time, closure-related outcomes, and the incidence of adverse events. Results: A total of 13 patients were included in the analysis. The median procedure time was 163 (55-283) min. Complete full-thickness resection was performed in seven cases, while in four cases, the serosa remained, and in two cases, the outer layer of the muscularis propria remained. In two cases where the SMT was located on the anterior side, conversion to laparoscopic surgery became necessary, resulting in a procedural success rate of 84.6% (11/13). Excluding these two cases, endoscopic closure of the defect was successfully accomplished in the remaining 11 cases. R0 resection was achieved in 12 out of 13 cases (92.3%). Although one patient had peritonitis, which was successfully treated conservatively, no other treatment-related adverse events were encountered. Conclusions: Although ER for SMT on the anterior side may be challenging, our experience revealed that ER is a safe and efficacious approach for gastric SMT.
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Objectives: Although color information is important in gastrointestinal endoscopy, there are limited studies on how endoscopic images are viewed by people with color vision deficiency. We aimed to investigate the differences in the visibility of blood vessels during endoscopic submucosal dissection (ESD) among people with different color vision characteristics and to examine the effect of red dichromatic imaging (RDI) on blood vessel visibility. Methods: Seventy-seven pairs of endoscopic images of white light imaging (WLI) and RDI of the same site were obtained during colorectal ESD. The original images were set as type C (WLI-C and RDI-C), a common color vision. These images were computationally converted to simulate images perceived by people with color vision deficiency protanope (Type P) or deutanope (Type D) and denoted as WLI-P and RDI-P or WLI-D and RDI-D. Blood vessels and background submucosa that needed to be identified during ESD were selected in each image, and the color differences between these two objects were measured using the color difference (ΔE 00) to assess the visibility of blood vessels. Results: ΔE 00 between a blood vessel and the submucosa was greater under RDI (RDI-C/P/D: 24.05 ± 0.64/22.85 ± 0.66/22.61 ± 0.64) than under WLI (WLI-C/P/D: 22.26 ± 0.60/5.19 ± 0.30/8.62 ± 0.42), regardless of color vision characteristics. This improvement was more pronounced in Type P and Type D and approached Type C in RDI. Conclusions: Color vision characteristics affect the visibility of blood vessels during ESD, and RDI improves blood vessel visibility regardless of color vision characteristics.
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Background: Transoesophageal echocardiography is rarely reported as a possible cause of aortic dissection during the transcatheter edge-to-edge repair procedure. Herein, we present a case of type B aortic dissection following the transcatheter mitral valve edge-to-edge repair procedure, most likely related to the transoesophageal echocardiography probe. Case summary: A 68-year-old Chinese man complained of exertional dyspnoea lasting over 2 years and had been diagnosed with severe mitral regurgitation. He was admitted to our hospital for the treatment of severe mitral regurgitation with transcatheter edge-to-edge repair. One MitraClip XTR (Abbott Vascular) was successfully implanted under the guidance of active transoesophageal echocardiography, and the mitral regurgitation became trace. However, the patient complained of persistent back pain after the treatment, and computed tomography angiography revealed a type B aortic dissection in the descending aorta. After 2 weeks of unsuccessful conservative treatment, he successfully underwent endovascular stenting and was discharged from the hospital. The patient recovered well and remained event free during the 6-month follow-up. Discussion: Herein, we presented a rare complication following transcatheter mitral valve edge-to-edge repair that was most likely related to the transoesophageal echocardiography probe-type B aortic dissection. We postulated that repetitive flexion of the transoesophageal echocardiography probe led to compression-induced injury to the descending aorta wall at the mid-oesophageal level, which was the most probable aetiology of type B aortic dissection. Although this complication is rare, it is potentially fatal and therefore needs attention.
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Poor tissue quality of adventitia and intima makes aortic root repair complex in patients with acute type A aortic dissection. The management of aortic root repair remains controversial. Internal aortic annuloplasty devices provide a standardized aortic valve repair technique to reduce and prevent annular dilation, while the modified Florida sleeve (mFS) procedure is an aortic root remodeling technique that does not require coronary artery reimplantation. However, little is known about the long-term durability of internal aortic annuloplasty with the hemispheric aortic annuloplasty remodeling ring (HARRT) combined with a mFS procedure in acute type A aortic dissection repair. A 52-year-old man had initial type A aortic dissection repair with an internal aortic annuloplasty ring and a mFS technique. He presented with dyspnea on exertion and intermittent chest pain one year later. Transesophageal echocardiogram revealed malposition of aortic annuloplasty ring and severe aortic insufficiency. He underwent a redo sternotomy with aortic valve replacement. Intraoperative findings demonstrated the aortic annuloplasty ring had dislodged from under the left and right coronary annulus and was adherent to the base of the noncoronary leaflet. The annuloplasty ring and aortic valve leaflets were excised and replaced with a mechanical aortic valve.
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Blunt trauma is a major cause of death in children, with renal arterial injuries occurring in less than 1% of cases. Traumatic renal artery occlusion (RAO) in children is rare and results in the loss of ipsilateral renal perfusion. Clinical signs are often nonspecific, and there is a lack of information on the exact incidence and management outcomes of these rare cases in children. We report a case of unilateral RAO in a 13-year-old boy with polytrauma. He was diagnosed with right RAO and hepatic laceration after a fall. A conservative approach yielded favorable outcomes despite the loss of function in the right kidney. In stable polytrauma patients, prompt diagnosis of RAO is crucial for treatment optimization and potential kidney salvage.
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Background: The occurrence of type A aortic dissection (TAAD) during transoesophageal echocardiography (TEE) has only been reported once. We present another case of pre-procedural type B AD with retrograde TAAD or de novo TAAD during the TEE procedure. Case summary: An 81-year-old man with a pre-existing infrarenal abdominal aortic aneurysm and highly tortuous aorta was referred to our ward for acute decompensated heart failure (ADHF) with New York Heart Association functional class II. On hospital Day 2, the patient complained of intermittent dull pain over chest and back; ADHF or acute coronary syndrome was suspected. On Day 3, we transferred the patient to the intensive care unit due to ADHF with cardiogenic shock attributed to fluid overload, atrial fibrillation with rapid ventricular response, and severe mitral regurgitation with severely impaired left ventricular ejection fraction. Given the heightened surgical risk, TEE was performed to evaluate the eligibility of mitral transcatheter edge-to-edge repair. The first mid-oesophageal long-axis view showed no evidence of dissection. After 20â min, the same view showed the occurrence of TAAD. Urgent contrast CT confirmed a TAAD extending from the aortic root to the infrarenal abdominal aorta. Due to the prohibitive risk for surgical repair of TAAD, the patient received palliative care and unfortunately passed away on hospital Day 6. Discussion: Albeit rare, TAAD could progress or de novo occur during TEE, especially in high-risk patients. Therefore, high alertness during TEE procedures is imperative. Moreover, in patients with AD and poor renal function, the risk of using TEE as an alternative diagnostic modality should be carefully considered.
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Chest pain is a common yet complex presentation in the emergency department, often requiring the exclusion of life-threatening conditions such as aortic dissection. Stanford type B aortic dissection, which affects the descending aorta, poses significant diagnostic and therapeutic challenges but can often be managed medically without immediate surgery. This case underscores the necessity of having a vigilant mindset, performing a detailed clinical examination, and including aortic dissection in the differential diagnosis, especially when typical symptoms are observed. The challenging part of this case was the investigation, as computed tomography angiography couldn't be performed, necessitating the use of magnetic resonance imaging for diagnosis. It highlights the importance of individualized patient care, vigilant monitoring, and comprehensive management strategies in the treatment of aortic dissection.
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A 65-year-old male presented with progressive swelling and difficulty in walking due to a right foot sprain. Initial treatments were conducted in Chandrapur, followed by referral to Acharya Vinoba Bhave Rural Hospital for further evaluation and management. The patient, a known case of diabetes mellitus and hypertension, reported an insidious onset of right foot swelling over two months. A physical examination revealed stable vital signs; no significant abnormalities were observed during the systemic examination. Laboratory investigations indicated mild anemia and slightly elevated liver enzymes. Imaging studies, including MRI and CT scan, identified an ill-defined lesion on the medial aspect of the right foot, consistent with dermatofibroma. The patient underwent a below-knee amputation with inguinal lymph node dissection on 31st May 2024. The procedure, performed under spinal and epidural anesthesia, involved meticulous dissection and ligation, with the posterior flap sutured using Ethilon 2-0 (Ethicon, Cincinnati, OH, USA). Post-operative management included IV antibiotics and supportive care. The patient's postoperative course was uneventful, with a healthy suture line and stable vitals upon discharge. Histopathological evaluation of the resected specimen confirmed melanoma, with immunohistochemistry revealing HMB-45 and S-100 negativity. The patient was discharged with advice on local hygiene, physiotherapy, dietary recommendations, and a follow-up schedule. This case highlights the importance of comprehensive multidisciplinary management in treating malignancies complicated by chronic conditions. Early diagnosis, appropriate surgical intervention, and diligent post-operative care are crucial for favorable outcomes in complex oncological cases.
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BACKGROUND: Aortic dissection with malperfusion necessitates emergency surgery and is associated with poor outcomes. Therefore, in this study, we analyzed patients' treatment courses from the initial management to hospital discharge in cases of acute aortic dissection (AAD) with malperfusion and investigated the risk factors associated with mortality. METHODS: We evaluated cases of AAD with malperfusion treated at our institution over a 16-year period from 2007 to 2022. The primary endpoint was in-hospital mortality. The study's primary outcome measure was mortality during hospitalization. We collected and analyzed data encompassing preoperative patient characteristics, Stanford classification, sites of preoperative malperfusion, surgical techniques employed, and postoperative complications. These variables were examined to identify factors associated with in-hospital mortality. RESULTS: During the study period, 366 patients were admitted with AAD, 102 of whom had malperfusion. There were 62 men (61%) and 40 women (39%), with a mean age of 64 ± 13 years (range: 28-87 years). According to the Stanford classification, 75 (74%) and 27 (26%) patients had type A and B aortic dissection, respectively, and 29 patients (28%) presented with shock. Preoperative malperfusion sites included the brain, coronary arteries, abdominal viscera, limbs, and spinal cord in 40 (39%), 10 (10%), 34 (33%), 52 (51%), and six (6%) patients, respectively. Eleven (11%) patients required immediate intervention in the emergency department. The treatments administered to the patients were as follows: ascending aortic replacement, 30 (29%) patients; aortic arch replacement, 34 (33%) patients; root replacement, three (3%) patients; thoracic endovascular aortic repair (TEVAR), 12 (12%) patients; non-anatomic bypass, five (5%) patients; and conservative management, five (5%) patients. In-hospital mortality occurred in 23 (23%) patients. Multivariate logistic regression analysis identified preoperative coronary malperfusion as an independent risk factor of mortality. CONCLUSION: Preoperative coronary malperfusion is an independent risk factor for in-hospital mortality in patients with AAD presenting with malperfusion.
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BACKGROUND: With the development of minimally invasive surgical techniques, the use of laparoscopic D2 radical surgery for the treatment of locally advanced gastric cancer (GC) has gradually increased. However, the effect of this procedure on survival and prognosis remains controversial. This study evaluated the survival and prognosis of patients receiving laparoscopic D2 radical resection for the treatment of locally advanced GC to provide more reliable clinical evidence, guide clinical decision-making, optimize treatment strategies, and improve the survival rate and quality of life of patients. AIM: To investigate the survival prognosis and influencing factors of laparoscopic D2 radical resection for locally advanced GC patients. METHODS: A retrospective cohort study was performed. Clinicopathological data from 652 patients with locally advanced GC in our hospitals from December 2013 to December 2023 were collected. There were 442 males and 210 females. The mean age was 57 ± 12 years. All patients underwent a laparoscopic D2 radical operation for distal GC. The patients were followed up in the outpatient department and by telephone to determine their tumor recurrence, metastasis, and survival. The follow-up period ended in December 2023. Normally distributed data are expressed as the mean ± SD, and normally distributed data are expressed as M (Q1, Q3) or M (range). Statistical data are expressed as absolute numbers or percentages; the χ 2 test was used for comparisons between groups, and the Mann-Whitney U nonparametric test was used for comparisons of rank data. The life table method was used to calculate the survival rate, the Kaplan-Meier method was used to construct survival curves, the log rank test was used for survival analysis, and the Cox risk regression model was used for univariate and multifactor analysis. RESULTS: The median overall survival (OS) time for the 652 patients was 81 months, with a 10-year OS rate of 46.1%. Patients with TNM stages II and III had 10-year OS rates of 59.6% and 37.5%, respectively, which were significantly different (P < 0.05). Univariate analysis indicated that factors such as age, maximum tumor diameter, tumor differentiation grade (low to undifferentiated), pathological TNM stage, pathological T stage, pathological N stage (N2, N3), and postoperative chemotherapy significantly influenced the 10-year OS rate for patients with locally advanced GC following laparoscopic D2 radical resection for distal stomach cancer [hazard ratio (HR): 1.45, 1.64, 1.45, 1.64, 1.37, 2.05, 1.30, 1.68, 3.08, and 0.56 with confidence intervals (CIs) of 1.15-1.84, 1.32-2.03, 1.05-1.77, 1.62-2.59, 1.05-1.61, 1.17-2.42, 2.15-4.41, and 0.44-0.70, respectively; P < 0.05]. Multifactor analysis revealed that a tumor diameter greater than 4 cm, low tumor differentiation, and pathological TNM stage III were independent risk factors for the 10-year OS rate in these patients (HR: 1.48, 1.44, 1.81 with a 95%CI: 1.19-1.84). Additionally, postoperative chemotherapy emerged as an independent protective factor for the 10-year OS rate (HR: 0.57, 95%CI: 0.45-0.73; P < 0.05). CONCLUSION: A maximum tumor diameter exceeding 4 cm, low tumor differentiation, and pathological TNM stage III were identified as independent risk factors for the 10-year OS rate in patients with locally advanced GC following laparoscopic D2 radical resection for distal GC. Conversely, postoperative chemotherapy was found to be an independent protective factor for the 10-year OS rate in these patients.
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Acute coronary syndrome due to a non-atherosclerotic, non-traumatic, or iatrogenic-induced spontaneous coronary artery dissection (SCAD) is a rare clinical condition that affects mostly young women of reproductive age. In this case, we present a 36-week-pregnant, 35-year-old G2P1 woman, with no previous medical history, who was admitted to our hospital with premature pre-labor contractions. During her hospitalization, she underwent a coronary artery percutaneous angiography revealing SCAD of the three coronary vessels, after an episode of acute-onset chest pain, tachypnea, EKG alterations, cardiac enzyme elevation, and bilateral pleural effusions. An emergency cesarean delivery was performed and the patient was transferred to the cardiology intensive care unit. Conservative management was decided and the woman was discharged a few days later.
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The urethra is a long muscular tubular organ that changes its internal configuration as it traverses different regions. Therefore, the pathology of each region is distinctive, and regional characteristics may influence exploration and treatment. We set out to perform detailed dissections of the male urethra and illustrate the regional specificity for each urethral segment. Therefore, we dissected the vesical cervix, showing both the normal appearance and the appearance of prostatic middle lobe adenoma. We dissected the prostatic urethra, showing the seminal colliculus, ejaculatory ducts, and prostatic utricle. We revealed the membranous urethra surrounded by the striated sphincter. We have shown the appearance of the spongy urethral bulb with its cul-de-sac appearance, the aspect of the urethral lacunae, the navicular fossa, and the external urethral meatus. We performed cross-sections through the prostatic urethra as well as dissection of the bulbourethral glands at the level of the urogenital diaphragm. All the information obtained through dissection is illustrated in high-quality graphic form and correlated with clinical applications, making this work valuable teaching material.
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Aortic dissection is a critical and life-threatening condition that can present with atypical symptoms, often leading to misdiagnosis and delayed treatment. The report presents a case of a 65-year-old male who initially exhibited fever, right-sided chest pain, and a productive cough, resulting in an initial diagnosis of pneumonia. Despite empirical antibiotic therapy, his symptoms persisted, prompting further investigation. A computed tomography (CT) scan ultimately revealed a Type B aortic dissection. The patient was then transferred to a specialized tertiary care facility for successful endovascular intervention. This case underscores the importance of considering aortic dissection in patients presenting with persistent, atypical symptoms that do not respond to standard treatments, such as unexplained fever and chest pain. It highlights the crucial role of advanced imaging techniques, such as CT scans, in achieving an accurate and timely diagnosis. Clinicians must maintain a high index of suspicion and ensure prompt referral to specialized centers to improve patient outcomes in this potentially fatal condition.