Background/Aims: Various lumen-apposing metal stents (LAMS) have been used for the endoscopic ultrasound-guided transmural drainage (EUS-TD) of postoperative pancreatic fluid collections (POPFC) and peripancreatic fluid collections (PFC). In this study, we aimed to assess the efficacy and safety of novel electrocautery-enhanced LAMSs (Hot-Plumber with Z-EUS IT) with different inter-flange lengths (13 to 33 mm) for managing POPFC and PFC. Methods: We reviewed the interventional EUS database of Asan Medical Center to identify consecutive patients with POPFC or PFC who underwent EUS-TD with the novel LAMSs between April 2023 and December 2023. Technical success, clinical success, and adverse events were evaluated. Results: Ten patients (5 with POPFCs and 5 with PFCs) were included in the analysis. The technical and clinical success rates were 100% and 90%, respectively. The LAMS was placed using either the freehand technique (n=5) or the over-the-guide wire technique (n=5). One patient successfully underwent endoscopic necrosectomy for walled-off necrosis through a novel LAMS.Two patients experienced adverse events (one stent migration and one infection). The LAMS was removed in 7 out of 10 patients after resolution of the fluid collection at a median of 61 days (interquartile range, 31 to 69 days) post-LAMS placement. Conclusions EUS-TD using the novel LAMS for POPFC and PFC demonstrated high efficacy and an acceptable safety profile. This novel LAMS represents a viable option when selecting stents for EUS-guided drainage of the POPFC and PFC.

Background: Because of the low prevalence of inherited retinal diseases, reports on the distribution of retinitis pigmentosa (RP)-related genes in Korean patients are scarce. The aim of this study was to determine the mutation spectrum and allele frequency and observe the final diagnoses in a Korean cohort clinically diagnosed with RP. Methods: We used whole-exome sequencing (WES) to analyze a Korean cohort of 100 unrelated patients clinically diagnosed with RP. The possible pathogenicity of each variant was assessed based on the guidelines of the American College of Medical Genetics and Genomics and Association for Molecular Pathology, in-silico prediction tools, known clinical phenotypes, and inheritance patterns. Results: Definite causative genes were detected in 60/100 patients (60.0%). Of these 60 cases, USH2A was the most common causative gene (14/60, 23.3%), followed by EYS (13/60, 21.7%) and RP1 (6/60, 10.0%). The clinical diagnosis was redefined in 9 of the 60 probands (15.0%) with causative genes after WES. Five of the 60 patients (8.3%) carried a causative variant in CHM, and the clinical diagnosis was redefined as choroideremia. Leber congenital amaurosis was diagnosed in 2/60 probands (3.3%), and RDH12 and RPGRIP1 were the causative genes in each patient. One patient (1/60, 1.7%) was diagnosed with Bietti’s crystalline dystrophy, with CYP4V2 identified as the causative gene. In another patient (1/60, 1.7%), ABCA4 variants were detected with clinical findings suggestive of cone-rod dystrophy. Conclusion This study reports the mutational spectrum of a cohort of Korean patients with a clinical diagnosis of RP who were referred for genetic testing. This study adds valuable data regarding the frequency of genes as well as their relation to the age of symptom onset and relation to other inherited retinal degenerations.

Background: Because of the low prevalence of inherited retinal diseases, reports on the distribution of retinitis pigmentosa (RP)-related genes in Korean patients are scarce. The aim of this study was to determine the mutation spectrum and allele frequency and observe the final diagnoses in a Korean cohort clinically diagnosed with RP. Methods: We used whole-exome sequencing (WES) to analyze a Korean cohort of 100 unrelated patients clinically diagnosed with RP. The possible pathogenicity of each variant was assessed based on the guidelines of the American College of Medical Genetics and Genomics and Association for Molecular Pathology, in-silico prediction tools, known clinical phenotypes, and inheritance patterns. Results: Definite causative genes were detected in 60/100 patients (60.0%). Of these 60 cases, USH2A was the most common causative gene (14/60, 23.3%), followed by EYS (13/60, 21.7%) and RP1 (6/60, 10.0%). The clinical diagnosis was redefined in 9 of the 60 probands (15.0%) with causative genes after WES. Five of the 60 patients (8.3%) carried a causative variant in CHM, and the clinical diagnosis was redefined as choroideremia. Leber congenital amaurosis was diagnosed in 2/60 probands (3.3%), and RDH12 and RPGRIP1 were the causative genes in each patient. One patient (1/60, 1.7%) was diagnosed with Bietti’s crystalline dystrophy, with CYP4V2 identified as the causative gene. In another patient (1/60, 1.7%), ABCA4 variants were detected with clinical findings suggestive of cone-rod dystrophy. Conclusion This study reports the mutational spectrum of a cohort of Korean patients with a clinical diagnosis of RP who were referred for genetic testing. This study adds valuable data regarding the frequency of genes as well as their relation to the age of symptom onset and relation to other inherited retinal degenerations.

Background/Aims: Various lumen-apposing metal stents (LAMS) have been used for the endoscopic ultrasound-guided transmural drainage (EUS-TD) of postoperative pancreatic fluid collections (POPFC) and peripancreatic fluid collections (PFC). In this study, we aimed to assess the efficacy and safety of novel electrocautery-enhanced LAMSs (Hot-Plumber with Z-EUS IT) with different inter-flange lengths (13 to 33 mm) for managing POPFC and PFC. Methods: We reviewed the interventional EUS database of Asan Medical Center to identify consecutive patients with POPFC or PFC who underwent EUS-TD with the novel LAMSs between April 2023 and December 2023. Technical success, clinical success, and adverse events were evaluated. Results: Ten patients (5 with POPFCs and 5 with PFCs) were included in the analysis. The technical and clinical success rates were 100% and 90%, respectively. The LAMS was placed using either the freehand technique (n=5) or the over-the-guide wire technique (n=5). One patient successfully underwent endoscopic necrosectomy for walled-off necrosis through a novel LAMS.Two patients experienced adverse events (one stent migration and one infection). The LAMS was removed in 7 out of 10 patients after resolution of the fluid collection at a median of 61 days (interquartile range, 31 to 69 days) post-LAMS placement. Conclusions EUS-TD using the novel LAMS for POPFC and PFC demonstrated high efficacy and an acceptable safety profile. This novel LAMS represents a viable option when selecting stents for EUS-guided drainage of the POPFC and PFC.

Background/Aims: Various lumen-apposing metal stents (LAMS) have been used for the endoscopic ultrasound-guided transmural drainage (EUS-TD) of postoperative pancreatic fluid collections (POPFC) and peripancreatic fluid collections (PFC). In this study, we aimed to assess the efficacy and safety of novel electrocautery-enhanced LAMSs (Hot-Plumber with Z-EUS IT) with different inter-flange lengths (13 to 33 mm) for managing POPFC and PFC. Methods: We reviewed the interventional EUS database of Asan Medical Center to identify consecutive patients with POPFC or PFC who underwent EUS-TD with the novel LAMSs between April 2023 and December 2023. Technical success, clinical success, and adverse events were evaluated. Results: Ten patients (5 with POPFCs and 5 with PFCs) were included in the analysis. The technical and clinical success rates were 100% and 90%, respectively. The LAMS was placed using either the freehand technique (n=5) or the over-the-guide wire technique (n=5). One patient successfully underwent endoscopic necrosectomy for walled-off necrosis through a novel LAMS.Two patients experienced adverse events (one stent migration and one infection). The LAMS was removed in 7 out of 10 patients after resolution of the fluid collection at a median of 61 days (interquartile range, 31 to 69 days) post-LAMS placement. Conclusions EUS-TD using the novel LAMS for POPFC and PFC demonstrated high efficacy and an acceptable safety profile. This novel LAMS represents a viable option when selecting stents for EUS-guided drainage of the POPFC and PFC.

Background: Because of the low prevalence of inherited retinal diseases, reports on the distribution of retinitis pigmentosa (RP)-related genes in Korean patients are scarce. The aim of this study was to determine the mutation spectrum and allele frequency and observe the final diagnoses in a Korean cohort clinically diagnosed with RP. Methods: We used whole-exome sequencing (WES) to analyze a Korean cohort of 100 unrelated patients clinically diagnosed with RP. The possible pathogenicity of each variant was assessed based on the guidelines of the American College of Medical Genetics and Genomics and Association for Molecular Pathology, in-silico prediction tools, known clinical phenotypes, and inheritance patterns. Results: Definite causative genes were detected in 60/100 patients (60.0%). Of these 60 cases, USH2A was the most common causative gene (14/60, 23.3%), followed by EYS (13/60, 21.7%) and RP1 (6/60, 10.0%). The clinical diagnosis was redefined in 9 of the 60 probands (15.0%) with causative genes after WES. Five of the 60 patients (8.3%) carried a causative variant in CHM, and the clinical diagnosis was redefined as choroideremia. Leber congenital amaurosis was diagnosed in 2/60 probands (3.3%), and RDH12 and RPGRIP1 were the causative genes in each patient. One patient (1/60, 1.7%) was diagnosed with Bietti’s crystalline dystrophy, with CYP4V2 identified as the causative gene. In another patient (1/60, 1.7%), ABCA4 variants were detected with clinical findings suggestive of cone-rod dystrophy. Conclusion This study reports the mutational spectrum of a cohort of Korean patients with a clinical diagnosis of RP who were referred for genetic testing. This study adds valuable data regarding the frequency of genes as well as their relation to the age of symptom onset and relation to other inherited retinal degenerations.

Background/Aims: Various lumen-apposing metal stents (LAMS) have been used for the endoscopic ultrasound-guided transmural drainage (EUS-TD) of postoperative pancreatic fluid collections (POPFC) and peripancreatic fluid collections (PFC). In this study, we aimed to assess the efficacy and safety of novel electrocautery-enhanced LAMSs (Hot-Plumber with Z-EUS IT) with different inter-flange lengths (13 to 33 mm) for managing POPFC and PFC. Methods: We reviewed the interventional EUS database of Asan Medical Center to identify consecutive patients with POPFC or PFC who underwent EUS-TD with the novel LAMSs between April 2023 and December 2023. Technical success, clinical success, and adverse events were evaluated. Results: Ten patients (5 with POPFCs and 5 with PFCs) were included in the analysis. The technical and clinical success rates were 100% and 90%, respectively. The LAMS was placed using either the freehand technique (n=5) or the over-the-guide wire technique (n=5). One patient successfully underwent endoscopic necrosectomy for walled-off necrosis through a novel LAMS.Two patients experienced adverse events (one stent migration and one infection). The LAMS was removed in 7 out of 10 patients after resolution of the fluid collection at a median of 61 days (interquartile range, 31 to 69 days) post-LAMS placement. Conclusions EUS-TD using the novel LAMS for POPFC and PFC demonstrated high efficacy and an acceptable safety profile. This novel LAMS represents a viable option when selecting stents for EUS-guided drainage of the POPFC and PFC.

Background: Because of the low prevalence of inherited retinal diseases, reports on the distribution of retinitis pigmentosa (RP)-related genes in Korean patients are scarce. The aim of this study was to determine the mutation spectrum and allele frequency and observe the final diagnoses in a Korean cohort clinically diagnosed with RP. Methods: We used whole-exome sequencing (WES) to analyze a Korean cohort of 100 unrelated patients clinically diagnosed with RP. The possible pathogenicity of each variant was assessed based on the guidelines of the American College of Medical Genetics and Genomics and Association for Molecular Pathology, in-silico prediction tools, known clinical phenotypes, and inheritance patterns. Results: Definite causative genes were detected in 60/100 patients (60.0%). Of these 60 cases, USH2A was the most common causative gene (14/60, 23.3%), followed by EYS (13/60, 21.7%) and RP1 (6/60, 10.0%). The clinical diagnosis was redefined in 9 of the 60 probands (15.0%) with causative genes after WES. Five of the 60 patients (8.3%) carried a causative variant in CHM, and the clinical diagnosis was redefined as choroideremia. Leber congenital amaurosis was diagnosed in 2/60 probands (3.3%), and RDH12 and RPGRIP1 were the causative genes in each patient. One patient (1/60, 1.7%) was diagnosed with Bietti’s crystalline dystrophy, with CYP4V2 identified as the causative gene. In another patient (1/60, 1.7%), ABCA4 variants were detected with clinical findings suggestive of cone-rod dystrophy. Conclusion This study reports the mutational spectrum of a cohort of Korean patients with a clinical diagnosis of RP who were referred for genetic testing. This study adds valuable data regarding the frequency of genes as well as their relation to the age of symptom onset and relation to other inherited retinal degenerations.

Background: Modern neurosurgery has undergone significant evolution to include minimally inva-sive procedures, with the supraorbital approach (SOA) being a prime example. In this study, we aim to explore the surgical techniques and outcomes of this approach in the surgical treatment of frontal lobe, anterior skull base, and parasellar lesions. Methods: This study included 33 patients aged 36–83 years who underwent surgery using theSOA for lesions in the inferior frontal lobe, anterior skull base, and parasellar area between 2015 and 2024. There were 25 cases of meningioma, 2 cases of brain abscess, 2 cases of glioma, and one case each of craniopharyngioma, hemangioma, metastasis, and Rathke’s cleft cyst. The medical data and follow-up results were retrospectively analyzed. Results: The mean size of lesion was 3.38±3.05 cm. The mean follow-up period was 48.8months. Gross total resection was achieved in 25 patients (75.8%). There were no perioperative deaths, cases of cerebrospinal fluid rhinorrhea, or infections. Two cases of morbidity were reported as complications: one case of delayed intracerebral hemorrhage and one case of infarction due to vascular injury. All patients exhibited satisfactory cosmetic results. Conclusion In comparison to the conventional pterional approach, the SOA represents a safe and effective keyhole method for the removal of both extra-axial and intra-axial skull base tumors. This is particularly beneficial for lesions in the orbitofrontal region and parasellar area, as it allows for minimal disruption of normal brain parenchyma. Moreover, the SOA promotes a swift recovery and short hospital stay. Additionally, the SOA yields superior cosmetic results, including the prevention of temporalis muscle atrophy.

Background/Aims: Endoscopic papillectomy (EP) is increasingly used as an alternative to surgery for managing benign ampullary neoplasms. However, post-EP resection margins are often positive or indeterminate, and there is no consensus on the management of ampullary adenomas with positive or indeterminate margins after EP. This study was designed to compare the longterm outcomes between resected margin-negative (RMN) and resected margin-positive/indeterminate (RMPI) groups and to identify factors associated with clinical outcomes. Methods: This retrospective analysis included patients with ampullary adenoma without evidence of adenocarcinoma who underwent EP between 2004 and 2016. The RMN and RMPI groups were compared for recurrence rates and recurrence-free duration during a mean followup duration of 71.7±39.8 months. Factors related to clinical outcomes were identified using multivariate analysis. Results: Of the 129 patients who underwent EP, 82 were in the RMN group and 47 were in the RMPI group. The RMPI group exhibited a higher recurrence rate compared to the RMN group (14.6% vs 34.0%, p=0.019). However, the recurrence-free duration was not significantly different between the groups (34.7±32.6 months vs 36.2±27.4 months, p=0.900). Endoscopic treatment successfully managed recurrence in both groups (75% vs 75%). Submucosal injection was a significant risk factor for residual lesions (hazard ratio, 4.11; p=0.009) and recurrence (hazard ratio, 2.57; p=0.021). Conclusions Although ampullary adenomas with positive or indeterminate margins after EP showed a higher rate of recurrence at long-term follow-up, endoscopic treatment was effective with favorable long-term outcomes. Submucosal injection prior to resection was associated with increased risk of recurrence and residual lesions.