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The phenotypic variability of variant Turner syndrome is wide, ranging from characteristic clinical features to those that are hardly distinguishable from the general population. A 4-year-old girl presented with multiple brownish macules and patches on the trunk and upper extremities as well as axillary freckles. Exome sequencing and chromosomal microarray testing revealed a microdeletion at Xp22.33p22.11 leading to a diagnosis of Turner syndrome. Here we describe an unusual case of variant Turner syndrome with multiple café-au-lait spots.
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Background@#Double-lumen endobronchial tube (DLT) displacement, defined as migration of the DLT > 15 mm from optimal position, may threaten patient safety. Lateral decubitus positioning (LDP) of the patient can induce DLT displacement; however, little is known regarding the predictors for DLT displacement after this maneuver. Obesity may further aggravate DLT displacement by distorting mediastinal anatomy, but no comprehensive data exist concerning the impact of obesity on DLT displacement after LDP. Therefore, we evaluated the predictive value of preoperative risk factors, including obesity, for DLT displacement after LDP in patients who underwent pulmonary resection. @*Methods@#Data of patients who underwent pulmonary resection between July 2020 and July 2021 were retrospectively reviewed. Age, sex, height, weight, body mass index, left main bronchus diameter, DLT size, lateral decubitus direction, DLT depth, patient height divided by the DLT depths in supine and lateral decubitus positions (H/Dsupine and H/Dlateral, respectively), and extent of DLT dislocation were assessed. Logistic regression analysis identified risk factors, and the optimal cutoff values for continuous variables were determined using receiver operating characteristic curve analysis. @*Results@#Data from 428 patients were analyzed. DLT displacement was observed in 52 patients (12.1%). Obesity and H/Dsupine were independent predictors for DLT displacement after LDP (odds ratio [OR]: 5.69, 95% CI [2.89, 11.23], P < 0.001 and OR: 8.28, 95% CI [2.92, 23.48], P < 0.001, respectively). @*Conclusions@#Obesity was significantly associated with DLT displacement after LDP. Pre-emptively advancing the DLT from its optimal position before LDP may be advantageous in patients with obesity.
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An irreducible ankle dislocation is a rare injury. The cause is a dislocation of the distal fibula anteriorly or posteriorly or the insertion of soft tissue, such as the deltoid ligament or posteromedial tendon. The tibialis posterior tendon can be dislocated through distal tibiofibular diastasis and prevent reduction of the ankle joint. The authors experienced anterolateral ankle fracture dislocation with a diastasis of the distal tibiofibular joint, and reduction was impossible because of impingement of the tibialis posterior tendon dislocated anteriorly through the distal tibiofibular diastasis. This paper reports the treatment of this injury.
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Background@#The coronavirus disease-2019 (COVID-19) pandemic has contributed to the change in the epidemiology of many infectious diseases. This study aimed to establish the pre-pandemic epidemiology of pediatric invasive bacterial infection (IBI). @*Methods@#A retrospective multicenter-based surveillance for pediatric IBIs has been maintained from 1996 to 2020 in Korea. IBIs caused by eight bacteria (Streptococcus pneumoniae, Haemophilus influenzae, Neisseria meningitidis, Staphylococcus aureus, Streptococcus agalactiae, Streptococcus pyogenes, Listeria monocytogenes, and Salmonella species) in immunocompetent children > 3 months of age were collected at 29 centers. The annual trend in the proportion of IBIs by each pathogen was analyzed. @*Results@#A total of 2,195 episodes were identified during the 25-year period between 1996 and 2020. S. pneumoniae (42.4%), S. aureus (22.1%), and Salmonella species (21.0%) were common in children 3 to 59 months of age. In children ≥ 5 years of age, S. aureus (58.1%), followed by Salmonella species (14.8%) and S. pneumoniae (12.2%) were common. Excluding the year 2020, there was a trend toward a decrease in the relative proportions of S. pneumoniae (rs = −0.430, P = 0.036), H. influenzae (rs = −0.922, P 3 months of age. These findings can be used as the baseline data to navigate the trend in the epidemiology of pediatric IBI in the post COVID-19 era.
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Psoriasis is a common immune-mediated, inflammatory skin disease. However, unilateral psoriasis is rare and few cases have been reported. A 59-year-old male with a polio-affected right leg presented with exfoliative skin on the left leg for three months. He had been treated under the diagnosis of contact dermatitis with secondary infection. After the exfoliative skin lesions improved, psoriatic papules were noted, which was ultimately diagnosed as psoriasis and successfully treated with topical steroid and vitamin D agents. We propose that the Koebner phenomenon has acted as the triggering and aggravating factor of unilateral psoriasis in this patient.
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Cytomegalovirus (CMV) infection is common among immunocompromised hosts; however, its cutaneous manifestation is considered rare in comparison to internal organ involvement. Clinical manifestations of cutaneous CMV infection generally include perioral or perianal ulcerations. On the other hand, autoimmune bullous dermatosis can have bullae and ulcerations similar to those caused by cutaneous CMV infection. Autoimmune bullous dermatosis requires treatment with immunosuppressive agents for relatively long periods, which may cause reduction of immunocompetence. Because of this iatrogenic immunosuppression, patients with autoimmune bullous dermatosis subsequently acquire increased risk for opportunistic infections. However, cases of bullous pemphigoid (BP) complicated by cutaneous CMV infection are rarely reported. Herein, we report the case of an 88-year-old male who had BP and subsequently recalcitrant perianal skin lesions, which were eventually diagnosed as cutaneous CMV infections.
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Background@#Psoriasis localized to certain body areas, such as the scalp, nails, palms, soles, intertriginous regions, and genital regions, is reportedly difficult to treat. @*Objective@#To investigate the biologics-resistant areas in South Korean patients with psoriasis treated with biologics. @*Methods@#The study included 50 patients with chronic moderate to severe plaque psoriasis from the Pusan National University Hospital and Chosun University Hospital between October 2019 and September 2020. The patients had at least one psoriatic lesion, were treated with biologics for more than six months, and exhibited a partial or good response (reaching a Psoriasis Area and Severity Index [PASI] score of 1~5 after biologics treatment). @*Results@#A total of 50 patients with psoriasis (32 male, mean±standard deviation 47.8±11 years), with a median PASI score of 1.8, were included. The most common biologics-resistant areas were the anterior lower leg (56.0%), followed by the knee (48.0%) and posterior lower leg (42.0%). The proportion of biologics-resistant areas were obtained for body regions traditionally considered as difficult-to-treat entities, including the fingernails (10.0%), toenails (14.0%), scalp (38.0%), palm (12.0%), sole (14.0%), and genital areas (10.0%). @*Conclusion@#This study determined the biologics-resistant areas in South Korean patients, successfully treated with biologics, in a real-world clinical setting.
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Background@#Psoriasis imposes a significant treatment burden on patients, particularly impacting well-being and quality of life (QoL). The psychosocial impact of psoriasis treatments remains unexplored in most patient populations. @*Objective@#To assess the impact of adalimumab on health-related QoL (HRQoL) in Korean patients with psoriasis. @*Methods@#This 24-week, multicenter, observational study, assessed HRQoL in Korean patients treated with adalimumab in a real-world setting. Patient-reported outcomes (PROs) including European Quality of Life-5 Dimension scale (EQ-5D), EQ-5D VAS, SF-36, and DLQI were evaluated at week 16 and 24, versus baseline. Patient satisfaction was assessed using TSQM. @*Results@#Among 97 enrolled patients, 77 were assessed for treatment effectiveness. Most patients were male (52, 67.5%) and mean age was 45.4 years. Median baseline body surface area and Psoriasis Area and Severity Index (PASI) scores were 15.00 (range 4.00~80.00) and 12.40 (range 2.70~39.40), respectively. Statistically significant improvements in all PROs were observed between baseline and week 24. Mean EQ-5D score improved from 0.88 (standard deviation [SD], 0.14) at baseline to 0.91 (SD, 0.17) at week 24 (p=0.0067). The number of patients with changes in PASI 75, 90, or 100 from baseline to week 16 and 24 were 65 (84.4%), 17 (22.1%), and 1 (1.3%); and 64 (83.1%), 21 (27.3%), and 2 (2.6%), respectively. Overall treatment satisfaction was reported, including effectiveness and convenience. No unexpected safety findings were noted. @*Conclusion@#Adalimumab improved QoL and was well-tolerated in Korean patients with moderate to severe psoriasis, as demonstrated in a real-world setting. Clinical trial registration number (clinicaltrials.gov: NCT03099083).
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Background@#Topical medications play a crucial role in the treatment of atopic dermatitis (AD). Topical corticosteroids (TCSs) remain the main treatment of choice and topical antibiotics have also been used. However, with the new topical calcineurin inhibitors (TCIs), the prescription patterns of topical agents have changed over time. @*Objective@#To characterize the prescription patterns of topical medications in Korean patients with AD. @*Methods@#We investigated topical medications prescribed to Korean patients with AD using the National Health Insurance Sharing System (NHISS) database over a 14-year period (2002~2015). Additionally, the potency of prescribed TCSs was compared with AD and psoriasis patients. @*Results@#The annual prescription of TCSs showed a slightly decreasing trend without significant change. In particular, in terms of steroid class, prescription of moderate-to-low potency TCSs were increased and the use of high potency TCSs were decreased. TCSs were the most commonly prescribed topical medications for AD. Tertiary hospitals had a higher prescription rate for TCIs than secondary or primary hospitals (16.2%, 3.1%, and 1.9%, respectively). Additionally, dermatologists prescribed TCIs more frequently than pediatricians and internists (4.3%, 1.2%, and 0.6%, respectively). Among TCSs, Class 5 was prescribed the most (40.6%) followed by Class 7, 6, 4, 3, 1, and 2. When we compared the potency of TCSs prescribed for AD with psoriasis patients, moderate-to-low-potency TCSs were more commonly prescribed in AD. @*Conclusion@#Prescription patterns of topical medications had changed from 2002 to 2015 and differed according to the type of institution and specialty of the physician.
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Giant cellulitis-like Sweet syndrome (GCS) is the most recently defined variant of Sweet syndrome (SS) which could clinically mimic wide-spreading cellulitis. Although there has been only paucity of reports in the literature, it mostly appears at lower half of the body and histologically shows dense infiltration of neutrophils with occasional histiocytoid mononuclear cells. Although its exact etiology has not been clarified, abnormal conditions (e.g., infection, malignancy and drugs) could be related triggering factors and trauma itself can be one of the causative elements as a ‘pathergy phenomenon’. GCS could be confusing manifestation especially when appeared in postoperative condition. A 69-year-old female presented with an erythematous edematous papules and plaques on the right thigh after varicose vein surgery. Skin biopsy revealed diffuse neutrophilic infiltrates that was consistent with SS. To our knowledge, there has been no report of GCS as a postoperative complication after varicose vein surgery. Physicians should be aware of this uncommon reactive neutrophilic dermatoses mimicking infectious cutaneous disease.
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The development of drug-resistant influenza and new pathogenic virus strains underscores the need for antiviral therapeutics. Currently, neuraminidase (NA) inhibitors are commonly used antiviral drugs approved by the US Food and Drug Administration (FDA) for the prevention and treatment of influenza. Here, we show that vitisin B (VB) inhibits NA activity and suppresses H1N1 viral replication in MDCK and A549 cells. Reactive oxygen species (ROS), which frequently occur during viral infection, increase virus replication by activating the NF-κB signaling pathway, downmodulating glucose-6-phosphate dehydrogenase (G6PD) expression, and decreasing the expression of nuclear factor erythroid 2-related factor 2 (Nrf2) antioxidant response activity. VB decreased virus-induced ROS generation by increasing G6PD expression and Nrf2 activity, and inhibiting NF-κB translocation to the nucleus through IKK dephosphorylation. In addition, VB reduced body weight loss, increased survival, decreased viral replication and the inflammatory response in the lungs of influenza A virus (IAV)-infected mice. Taken together, our results indicate that VB is a promising therapeutic candidate against IAV infection, complements existing drug limitations targeting viral NA. It modulated the intracellular ROS by G6PD, Nrf2 antioxidant response pathway, and NF-κB signaling pathway. These results demonstrate the feasibility of a multi-targeting drug strategy, providing new approaches for drug discovery against IAV infection.
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Background and Objectives@#Fewer studies are available on geriatric patients’ gustatory dysfunction than on their olfactory dysfunction. Here we aimed to evaluate the relationship between subjective gustatory dysfunction and subjective or objective olfactory dysfunction according to cognitive function in geriatric patients.Subjects and Method We prospectively enrolled patients who underwent both cognitive function test and olfactory function test between August 2018 and May 2019. The correlation between subjective gustatory dysfunction and subjective olfactory dysfunction or conventional olfactory function scores was evaluated for geriatric patients with or withhout cognitive dysfunction. Participants with a threshold-discrimination-identification (TDI) score (<21) on the YSK olfactory function test were diagnosed with olfactory dysfunction. Subjective gustatory function and olfactory function were evaluated using the visual analog scale. The Korean version of the Consortium to Establish a Registry for Alzheimer’s Disease Assessment Packet and Mini-Mental State Examination were administered to all participants. Overall, 120 patients (27 male, 93 female; mean age, 73.00±7.50 years) were enrolled. @*Results@#We found that the subjective gustatory function score did not correlate with the threshold, discrimination, identification, or the summation of TDI scores of the olfactory function test but was significantly associated with the subjective olfactory function score (p<0.001). Further, there was no significant correlation between the subjective gustatory function score and cognitive function. @*Conclusion@#The subjective olfactory function score was the only factor significantly correlated with the subjective gustatory function score. Based on these results, we suggest evaluating gustatory function in geriatric patients with olfactory dysfunction.
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Purpose@#Hypoxaemia is a significant adverse event during endoscopic retrograde cholangiopancreatography (ERCP) under monitored anaesthesia care (MAC); however, no model has been developed to predict hypoxaemia. We aimed to develop and compare logistic regression (LR) and machine learning (ML) models to predict hypoxaemia during ERCP under MAC. @*Materials and Methods@#We collected patient data from our institutional ERCP database. The study population was randomly divided into training and test sets (7:3). Models were fit to training data and evaluated on unseen test data. The training set was further split into k-fold (k=5) for tuning hyperparameters, such as feature selection and early stopping. Models were trained over k loops; the i-th fold was set aside as a validation set in the i-th loop. Model performance was measured using area under the curve (AUC). @*Results@#We identified 6114 cases of ERCP under MAC, with a total hypoxaemia rate of 5.9%. The LR model was established by combining eight variables and had a test AUC of 0.693. The ML and LR models were evaluated on 30 independent data splits. The average test AUC for LR was 0.7230, which improved to 0.7336 by adding eight more variables with an l 1 regularisation-based selection technique and ensembling the LRs and gradient boosting algorithm (GBM). The high-risk group was discriminated using the GBM ensemble model, with a sensitivity and specificity of 63.6% and 72.2%, respectively. @*Conclusion@#We established GBM ensemble model and LR model for risk prediction, which demonstrated good potential for preventing hypoxaemia during ERCP under MAC.
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Purpose@#To examine the refractive errors, retinal manifestations, and genotype in tuberous sclerosis complex (TSC) patients in a Korean population. @*Materials and Methods@#A total of 98 patients with TSC were enrolled in Severance Hospital for a retrospective cohort study. The number of retinal astrocytic hamartoma and retinal achromic patch within a patient, as well as the size, bilaterality, and morphological type were studied. In addition, the refractive status of patients and the comorbidity of intellectual disability and epilepsy were also examined. @*Results@#Retinal astrocytic hamartoma was found in 37 patients, and bilateral invasion was observed in 20 patients (54%). TSC1 mutation was associated with myopia (p=0.01), while TSC2 mutation was associated with emmetropia (p=0.01). Retinal astrocytic hamartoma was categorized into three morphological types and examined as follows: type I (87%), type II (35%), and type III (14%). Single invasion of retinal astrocytic hamartoma was identified in 32% of the patients, and multiple invasions in 68%. The TSC1/ TSC2 detection rate was 91% (41/45). Among them, TSC1 variant was detected in 23 patients (54%), whereas TSC2 variant was detected in 18 patients (40%). The results showed that TSC2 mutations are correlated with a higher rate of retinal astrocytic hamartoma involvement (all p<0.05), and multiple and bilateral involvement of retinal hamartomas (all p<0.05). However, the size of retinal astrocytic hamartomas, comorbidity of epilepsy, or intellectual disability did not show correlation with the genetic variant. @*Conclusion@#TSC1 variant patients were more myopic, while TSC2 variant patients showed association with more extensive involvement of retinal astrocytic hamartoma.
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Purpose@#This study aimed to investigate the association between sedentary behavior and generalized anxiety disorder (GAD) according to changes in physical activity after coronavirus disease 2019 (COVID-19) in Korean adolescents. @*Methods@#This study included 52,723 South Korean middle and high school students who had obtained a complete Korea youth risk behavior survey, 2021. To identify the association between sitting time and GAD (GAD-7 scale≥10), odds ratio (OR) and 95% confidence interval (CI) were calculated using complex sample logistic regression analysis (p＜0.05). @*Results@#After adjusting for confounding variables, higher sitting time was associated with higher GAD risk (OR [95% CI]: 5–10 hours, 1.15 [1.04–1.27]; 10–15 hours, 1.23 [1.12–1.35]; ≥15 hours, 1.28 [1.15–1.43]). In adolescents whose physical activity has not changed or has increased after COVID-19, as the sitting time increased, the OR (95% CI) of GAD increased (5–10 hours, 1.15 [1.02–1.31]; 10–15 hours, 1.20 [1.07–1.35]; ≥15 hours, 1.35 [1.17–1.54]). But, not in adolescents whose physical activity has decreased after COVID-19. @*Conclusion@#We observed the independent associations between sedentary behavior and GAD, and sedentary behavior and GAD are associated by change in physical activity after COVID-19 among adolescents. Therefore, in the pandemic era reducing sedentary behavior and increasing physical activity is necessary for adolescents to enhance mental health and decrease the case of GAD.
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Objective@#Mental health is a global concern and needs to be studied more closely. We aimed to estimate the prevalence of mental disorders and their associated factors among the general population in Korea. @*Methods@#The National Mental Health Survey of Korea 2021 was conducted between June 19 and August 31, 2021 and included 13,530 households; 5,511 participants completed the interview (response rate: 40.7%). The lifetime and 12-month diagnosis rates of mental disorders were made using the Korean version of the Composite International Diagnostic Interview 2.1. Factors associated with alcohol use disorder (AUD), nicotine use disorder, depressive disorder, and anxiety disorder were analyzed, and mental health service utilization rates were estimated. @*Results@#The lifetime prevalence of mental disorders was 27.8%. The 12-month prevalence rates of alcohol use, nicotine use, depressive, and anxiety disorders were 2.6%, 2.7%, 1.7%, and 3.1%, respectively. The risk factors associated with 12-month diagnosis rates were as follows: AUD: sex and age; nicotine use disorder: sex; depressive disorder: marital status and job status; anxiety disorder: sex, marital status, and job status. The 12-month treatment and service utilization rates for 12-month AUD, nicotine use disorder, depressive disorder, and anxiety disorder were 2.6%, 1.1%, 28.2%, and 9.1%, respectively. @*Conclusion@#Approximately 25% of adults in the general population were diagnosed with mental disorders during their lifetime. The treatment rates were substantially low. Future studies on this topic and efforts to increase the mental health treatment rate at a national level are needed.
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Water beads are dangerous foreign bodies causing intestinal obstruction in young children because the beads absorb water and are radiolucent. Although the features lead to progressive intestinal obstruction, it is difficult to diagnose ingestion of the beads by imaging studies. For the diagnosis, ultrasonography is safe, fast, and accurate. The imaging modality can show intestinal water beads as spherical, anechoic, smoothly demarcated cysts. This characteristic finding may be more useful in rapid and accurate diagnosis than computed tomography scan. We report a case of an 8-month-old boy who obtained a timely sonographic diagnosis of water bead-induced small bowel obstruction in the emergency department.
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Background@#A total of 8,303 individuals (4.3%) with adverse reactions (n=191,860) after vaccination developed serious conditions or died. Such health developments could cause people not vaccinated yet or waiting for a booster shot to become fearful of the vaccination. @*Methods@#The 3-month (July–September 2021) clinical data of 41 patients from the family medicine department of a single medical center were analyzed retrospectively to determine risk factors and to investigate the clinical course to identify the cause of symptoms in detail. @*Results@#A significant number of older adults aged over 50 years reported experiencing general weakness (P=0.026) but fewer incidences of fever than patients aged 50 years or younger (P=0.011). Eighteen of the 41 patients were requested to visit more than twice or consult a specialist. In 14 patients, the symptoms were explained by other medical causes. @*Conclusion@#The primary physician has a pivotal role in thoroughly evaluating patients who complain of adverseeffects after vaccination, considering the broad multitude of symptoms and medical conditions presented. To thoroughlyevaluate and appropriately advise patients with adverse reactions to their chosen vaccine, taking detailedmedical history and nutritional counseling are required to identify possible underlying causes, resolve symptoms,and educate them on self-care and regarding vaccines.
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Generally, an induction agent is chosen based on the conditions of the deceased donor and the recipient. Antithymocyte globulin (ATG) is preferred in relatively high-risk conditions. No clear evidence indicates which induction agent is safer or more efficient for deceased donor kidney transplantation (DDKT). This study compares the efficacy and safety of basiliximab (BSX) and ATG according to donor characteristics in DDKT. Methods: A total of 724 kidney transplant recipients from three transplant centers were enrolled, and propensity score matching was performed. Based on a donor age of 60 years, donor kidney with acute kidney injury (AKI), and Kidney Donor Profile Index (KDPI) score of 65%, we investigated how the choice of induction therapy agent affected the posttransplant clinical outcomes of delayed graft function (DGF), acute rejection (AR), infectious complications, and allograft and patient survival. Results: AR and DGF did not differ significantly according to induction agent in elderly/young donor, AKIon-AKI, and high-KDPI/ low-KDPI subgroups. The infection rate did not show meaningful differences. The differences in death-censored allograft survival and patient survival rates between induction agents were not statistically significant. Conclusion: Our study suggests that BSX can produce clinical outcomes similarly favorable to those of ATG even in DDKT cases with relatively poor donor conditions. Nonetheless, the donor and recipient conditions, immunological risk, and infection risk must be all taken into consideration when choosing an induction agent. Therefore, clinicians should carefully select the induction therapy agent for DDKT based on the risks and benefits in each DDKT case.
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Although hanging is the most common method of suicide in Korea, there are few reports of dysphagia following laryngeal injuries due to near-hanging. This is a case report of a patient who complained of dysphagia after a complex fracture of the neck structures caused due to attempted suicide by hanging. A 51-year-old male visited our hospital with neck injuries after a near-hanging episode. Laryngoscopy suspected dislocation of the right arytenoid cartilage. A computed tomography (CT) scan revealed fractures of the right hyoid bone and thyroid cartilage, as well as soft tissue emphysema. A closed reduction for the dislocation of the right arytenoid cartilage was performed. After surgery, oral feeding was attempted five days after surgery but stopped due to a large amount of aspiration. After two weeks, a videofluoroscopic swallowing study (VFSS) revealed a decreased laryngeal elevation and severe impairment of the upper esophageal sphincter opening. The patient was then given rehabilitation for oropharyngeal dysphagia to restore swallowing function. Four months after the injury, VFSS showed that the residue in the pyriform sinus after multiple swallowing attempts decreased compared to the previous studies. However, oral diet and enteral feeding were combined due to the patient experiencing early fatigue due to swallowing difficulties.Our observations, in this case, show that it was necessary to provide treatment through a multidisciplinary approach coupled with long-term follow-up for patients with dysphagia after complex fractures by near-hanging.