Epidemiology of Congenital Cholesteatoma: Surveys of the Last 17 Years in Japan.

(1) Background: The incidence of congenital cholesteatoma (CC) has rarely been discussed, particularly from a demographic viewpoint. Therefore, we conducted an epidemiological study of CC using local medical characteristics. (2) Methods: The participants were 100 patients (101 ears) who underwent initial surgical treatment at university hospitals in two rural prefectures between 2006 and 2022. A total of 68% of the patients were males and 32% were females, with a median age of 5 years. We reviewed the medical records for the date of birth, date of surgery, stage of disease, and first symptoms of the disease. (3) Results: The total incidence of CC was calculated to be 26.44 per 100,000 births and tended to increase. No significant difference was found between the incidences in the two prefectures. The number of surgeries performed was higher in the second half of the study period. No difference in the stage of progress was observed based on age. (4) Conclusions: The incidence of CC was estimated to be 26.44 per 100,000 newborn births. The number of patients with CC tended to increase; however, this can be attributed to an increase in the detection rate rather than the incidence.

Otic Capsule Demineralization and Hearing Outcome of Stapes Surgery for Osteogenesis Imperfecta in Comparison With Otosclerosis.

OBJECTIVE: To assess the location/number of otic capsule demineralization and hearing outcomes of stapes surgery (SS) for osteogenesis imperfecta (OI) compared with otosclerosis (OS). PATIENTS: This study included 11 and 181 consecutive ears from 6 and 152 patients with OI and OS, respectively. INTERVENTIONS: Demineralization loci observed as hypodense area of the otic capsule were examined using high-resolution computed tomography. All patients underwent SS. MAIN OUTCOME MEASURES: Locations of the hypodense areas were classified into the anterior oval window, anterior internal auditory canal, and pericochlear area. The location/number of hypodense areas and preoperative/postoperative hearing parameters were correlated. Postoperative hearing outcome was evaluated 12 months after surgery. RESULTS: Hypodense area was more frequently observed in OI (9 of 11 ears [81.8%]) than in OS (96 of 181 ears [53.0%]), with significant differences. Multiple sites were involved in 81.8% OI and 18.8% OS patients, showing significant differences. Preoperative air conduction (AC), bone conduction, and air-bone gap (ABG) were 48.9 ± 17.8, 28.0 ± 11.3, and 20.7 ± 8.4 dB, respectively, in OI and 56.2 ± 13.5, 30.5 ± 9.9, and 26.4 ± 9.7 dB, respectively, in OS, demonstrating greater AC and ABG in OS than in OI. Postoperative AC (31.3 ± 20.5 dB), ABG (10.6 ± 10.0 dB), and closure of ABG (12.1 ± 4.7 dB), that is, preoperative ABG minus postoperative ABG of OI, were comparable to those of OS (AC, 30.9 ± 13.3 dB; ABG, 7.0 ± 7.4 dB; closure of ABG, 20.1 ± 11.6 dB). CONCLUSION: OI ears showed more severe demineralization of otic capsule than OS ears. However, favorable hearing outcomes could be obtained through SS for OI and OS ears.

Risk factors for early and late cholecystitis after covered metal stent placement for distal biliary obstruction.

BACKGROUND: Cholecystitis is a major adverse event after self-expandable metallic stent placement for distal biliary obstruction (DBO). Covered self-expandable metallic stent (CSEMS) is being increasingly used, but few studies have investigated risk factors for cholecystitis limited to CSEMS. The present study aimed to identify risk factors for cholecystitis after CSEMS. METHODS: Patients who underwent initial CSEMS placement for DBO between November 2014 and September 2021 were enrolled and followed-up until death, recurrent biliary obstruction, cholecystitis, or until March 2022. Cholecystitis within 30 days of CSEMS was defined as early cholecystitis and after 30 days as late cholecystitis. RESULTS: Cholecystitis occurred in 51 of 339 patients (15%) after CSEMS. Forty-one patients (80.4%) had early cholecystitis, and 10 (19.6%) had late cholecystitis. Multivariate logistic regression analysis revealed that the maximum diameter of the common bile duct (CBD) (per 1 mm increase) (odds ratio [OR]: 0.87; 95% confidence interval [CI]: 0.76-1.00; p = .044), gallbladder stones (OR: 3.63; 95% CI: 1.62-8.10; p = .002), and tumor involvement in the cystic duct (CD) (OR: 4.87; 95% CI: 2.16-11.00; p < .001) were significant independent risk factors associated with early cholecystitis. No significant risk factors were identified for late cholecystitis. CONCLUSIONS: A smaller CBD diameter, gallbladder stones, and tumor involvement in the CD were identified as risk factors for early cholecystitis development after CSEMS.

The application of low echo reduction in endoscopic ultrasound-guided tissue acquisition for pancreatic mass lesions.

Toyonaga and colleagues demonstrate, with accompanying video, the use of low echo reduction, which is a novel endoscopic ultrasound function provided by a new endoscopic ultrasound processor that increases contrast without white-out. Low echo reduction might be useful in improving lesion boundaries and needle visibility during endoscopic ultrasound-guided tissue acquisition.

Tip-in endoscopic papillectomy for ampullary adenoma near diverticulum to minimize complications.

Toyonaga and colleagues present a novel "tip-in endoscopic papillectomy" approach for resecting ampullary tumors, aiming to minimize complications like perforation and residual tumor by adapting the colonic polyp endoscopic mucosal resection tip-in method. The technique is described with accompanying video in a case of ampullary tumor near a diverticulum.

Changes in functional connectivity among vestibulo-visuo-somatosensory and spatial cognitive cortical areas in persistent postural-perceptual dizziness: resting-state fMRI studies before and after visual stimulation.

Introduction: Persistent postural-perceptual dizziness (PPPD) is a functional chronic vestibular syndrome with symptom exacerbation by upright posture, motion, and complex visual stimuli. Among these exacerbating factors, visual exacerbation is the most specific characteristic of PPPD requiring further investigation. We hypothesized that stimulus-induced changes occur in the functional connectivity (FC) rather than simple neural activation that is involved in visual stimulation. The present study aimed to identify the neural basis of PPPD by investigating FC before and after visual stimulation. Methods: Eleven patients with PPPD and 11 age- and sex-matched healthy controls (HCs) underwent resting-state fMRI (rs-fMRI) before and after task-based fMRI with visual stimuli. Results: At pre-stimulus, FC between the vestibular cortex and visual areas was low, while that between the somatosensory and visual areas was high in PPPD compared with that in HCs. FC between the visuospatial (parahippocampal gyrus) and spatial cognitive areas (inferior parietal lobule) was elevated in PPPD even in the pre-stimulus condition, which no longer increased at post-stimulus as observed in HCs. In the post-stimulus condition, FC between the visual and spatial cognitive areas and that between the visual and prefrontal areas increased compared with that in the pre-stimulus condition in PPPD. Task-based fMRI demonstrated that no brain regions showed different activities between the HC and PPPD groups during visual stimulation. Discussion: In PPPD, vestibular inputs may not be fully utilized in the vestibulo-visuo-somatosensory network. Given that the FC between visuospatial and spatial cognitive areas increased even in HCs after visual stimuli, elevated status of this FC in combination with the high FC between the somatosensory and visual areas would be involved in the visual exacerbation in PPPD. An increase in FC from the visual areas to spatial cognitive and prefrontal areas after visual stimuli may account for the prolonged symptoms after visual exacerbation and anxious status in PPPD.

Hearing Outcome and Predictors after Implanting Bone Conduction or Middle Ear Implants in Ears with Refractory Otitis Media.

Bone conduction implants (BCIs) and middle ear implants (MEIs) are promising options for individuals with persistent chronic inflammation of the middle or outer ear. However, the structure of the middle ear is often altered in patients who undergo mastoidectomy or posterior wall removal for refractory otitis media, leaving uncertainty regarding the efficacy of hearing devices. Only a few studies have examined auditory outcomes based on the etiology of hearing impairment. We investigated hearing outcomes, including speech audiometry, in patients who underwent implantation after surgery for refractory otitis media. Our findings indicated that patients who received BCIs or MEIs achieved favorable hearing outcomes. Furthermore, a correlation was observed between the preoperative bone-conduction threshold at 1 kHz in the better ear and the sound-field threshold at 1 kHz with BCIs, whereas no correlation was observed between the preoperative bone-conduction threshold and the sound-field threshold with MEIs. This study highlights the positive impact of BCIs and MEIs in patients who undergo implantation after surgery for refractory otitis media. Additionally, our study identified parameters that predict postoperative efficacy.

Multiplex Digital PCR Assay to Detect Multiple KRAS and GNAS Mutations Associated with Pancreatic Carcinogenesis from Minimal Specimen Amounts.

Digital PCR (dPCR) allows for highly sensitive quantification of low-frequency mutations and facilitates early detection of cancer. However, low-throughput targeting of single hotspots in dPCR hinders variant specification when multiple probes are used. We developed a dPCR method to simultaneously identify major variants related to pancreatic carcinogenesis. Using a two-dimensional plot of droplet fluorescence under the optimized concentration of two fluorescent probe pools, the absolute quantification of different KRAS and GNAS variants was determined. Successful detection of the multiple driver mutations was verified in 24 surgically resected tumor samples from 19 patients and 22 fine-needle aspiration samples from patients with pancreatic ductal adenocarcinoma. Precise quantification of the variant allele frequency was optimized by using template DNA at a concentration as low as 1 to 10 ng. Furthermore, amplicons targeting multiple hotspots were successfully enriched with fewer false-positive findings using high-fidelity polymerase, allowing for the detection of various KRAS and GNAS mutations with high probability in small amount of cell/tissue specimens. Using this target enrichment, mutations at a rate of 90% in small residual tissues, such as the fine-needle aspiration needle flush and microscopic lesions in resected specimens, were successfully identified. The proposed method allows for low-cost, accurate detection of driver mutations to diagnose cancers, even with minimal tissue collection.

Prophylactic endoscopic gallbladder stent placement for cholecystitis after covered metal stent placement for distal biliary obstruction (with video).

BACKGROUND AND AIMS: Acute cholecystitis is occasionally observed after biliary drainage using a covered self-expandable metal stent (CSEMS) for distal biliary obstruction (DBO). Gallbladder drainage before CSEMS placement may reduce cholecystitis. This study aimed to examine the preventive effect of endoscopic gallbladder stent placement (EGBS) on cholecystitis with CSEMSs. METHODS: We retrospectively analyzed patients with DBO who underwent CSEMS placement across the orifice of the cystic duct between November 2014 and October 2021 and were negative for cholecystitis on biliary drainage. Prophylactic EGBS was attempted before CSEMS placement. The incidence of cholecystitis was compared between patients with and without EGBS. RESULTS: In total, 286 patients (128 men; median age, 75 years) were included in this study. EGBS was attempted in 32 patients before CSEMS placement, and technical success was achieved in 24 patients (75%). Adverse events were noted in 3 patients (9.4%; penetration of cystic duct in 1 and acute pancreatitis in 2). The cumulative incidence of cholecystitis was significantly lower in patients with EGBS than in those without EGBS (1 [4.2%] vs 56 [21.4%], P = .045). In multivariable analysis, EGBS was a significant protective factor against cholecystitis (hazard ratio, .11; 95% confidence interval, .01-.79; P = .028). CONCLUSIONS: Although the transpapillary approach to the gallbladder is not easy for patients with DBO, EGBS is effective in preventing cholecystitis associated with CSEMS placement.

Efficacy of gel immersion endoscopic ultrasonography for delineating the duodenal papilla and pancreatobiliary ducts: A retrospective study with video.

Objectives: Endoscopic ultrasonography is an important examination for periampullary diseases. The duodenum is filled with water to ensure a clear image and distend the duodenal wall without burying the papilla within duodenal folds; however, peristalsis frequently makes it difficult to maintain water within the duodenum. The gel immersion method (intestine is filled with viscosity gel) has recently been attracting attention. We evaluated the usefulness of using this method for endoscopic ultrasonography to detect and delineate the major duodenal papilla. Methods: Fifty-nine consecutive patients who underwent gel immersion-endoscopic ultrasonography between February and March 2021 were included retrospectively. The papilla was observed by filling the duodenum with clear viscosity gel. Outcomes were the rate of duodenal distention, delineation rates of the papilla, the time required for delineation, volume of the gel used, and adverse events. Results: Duodenal distention was excellent, good, and poor in 58%, 34%, and 7% of cases, respectively. The delineation rates of the papilla in the axial and longitudinal views were 98% and 66%, respectively. The median time required to delineate the papilla in each view was 3.1 (range, 1.0-1.4) and 7.9 (1.9-28.6) min; the median volume of the gel used was 80 (30-150) ml and 100 (50-200) ml, respectively. No adverse events were noted. Conclusions: Gel immersion-endoscopic ultrasonography provided sufficient duodenal distention, leading to high rates of detection and delineation of the papilla using a small volume of gel within a short time. This method may be useful for the evaluation of the ampullary region.

Re-intervention with 10-mm vs 12-mm covered self-expandable metallic stent for recurrent unresectable distal biliary obstruction in patients with previous stent implantation.

BACKGROUND: Treatment for unresectable distal malignant biliary obstruction (DMBO) involves placing a 10-mm covered self-expandable metallic stent (CSEMS). In recurrent biliary obstruction (RBO), replacement of the CSEMS is recommended; however, the appropriate stent type remains unknown. We speculated that large-bore CSEMSs would provide longer patency. We compared the efficacy of 10-mm and 12-mm CSEMS in patients with RBO. METHODS: We retrospectively retrieved data on patients who underwent CSEMS replacement from the endoscopic database. We investigated the time to RBO (TRBO) after CSEMS replacement. The hazard ratio (HR) for the the second TRBO was estimated using the Cox proportional hazard model. The estimated median second TRBO was determined using the Kaplan-Meier method. RESULTS: Among the 493 patients with DMBOs treated with 10-mm CSEMS, 48 and 29 patients underwent re-intervention with 10-mm and 12-mm CSEMS, respectively. The use of 12-mm CSEMS was inversely associated with second TRBO (multivariable-adjusted HR, 0.62; 95% CI, 0.39-0.98; P = .042). The estimated median second TRBO was greater with the 12 mm CSEMS than with the 10 mm variety (562 days vs 207 days; P = .019). CONCLUSION: The second intervention with 12-mm CSEMS was associated with a longer TRBO.

Efficacy of pancreatic duct stenting to prevent postendoscopic retrograde cholangiopancreatography pancreatitis after covered self-expandable metal stent deployment.

OBJECTIVES: Although covered self-expandable metal stents (CSEMSs) are associated with the risk of postendoscopic retrograde cholangiopancreatography (ERCP) pancreatitis due to pancreatic duct (PD) orifice obstruction, they are often used for biliary drainage treatment in malignant biliary obstruction (MBO). This study aimed to investigate the efficacy of PD stenting in preventing post-ERCP pancreatitis after CSEMS implantation. METHODS: This retrospective cohort study analyzed 554 patients with transpapillary CSEMS for MBO. Patients with noninitial deployment, benign disease, CSEMS deployment above the papilla, surgically altered anatomy, uncovered self-expandable metal stents, multiple thin self-expandable metal stents, and unavailable procedure videos were excluded. Logistic regression analysis estimated the association between PD stenting and post-ERCP pancreatitis incidence. We adjusted for age, sex, pancreatitis history, prophylactic rectal nonsteroidal anti-inflammatory drug use, naïve papilla, MBO etiology, and prolonged biliary cannulation time. RESULTS: Among 554 patients, 67 (12.1%) experienced post-ERCP pancreatitis. Post-ERCP pancreatitis was recorded in 13.7% of patients in the non-PD stenting and 4.3% in the PD stenting groups. Pancreatic duct stenting was associated with lower risks of post-ERCP pancreatitis (odds ratio [OR] 0.28; 95% confidence interval [CI] 0.099-0.79; P = 0.028). In multivariable analysis, the association between PD stenting and lower post-ERCP pancreatitis incidence was consistent (OR 0.19; 95% CI 0.062-0.58; P = 0.0034). CONCLUSIONS: Pancreatic duct stenting could reduce the risk of post-ERCP pancreatitis after CSEMSs.