Learning curve and technical nuances of endoscopic skull base reconstruction with nasoseptal flap to control high-flow cerebrospinal fluid leakage: reconstruction after endoscopic skull base surgery other than pituitary surgery.

PURPOSE: Although reconstruction techniques after endoscopic skull base surgery have been improved, there are difficulties in reconstructing the skull base with a nasoseptal flap (NSF), especially in the case of high-flow cerebrospinal fluid (CSF) leak. The aim of this study was to analyze risk factors for the development of postoperative CSF leaks in terms of less experienced surgeon practices. METHODS: Retrospective review of medical records was performed for 125 patients who underwent endoscopic skull base surgery for intradural pathology with intraoperative high-flow CSF leakage between Oct 2012 and Apr 2017. Basic demographic data were collected, including body mass index (BMI), tumor pathology, comorbidities, and outcomes. To assess the learning curve effect, patients were divided into early cohort (n = 30) and late cohort (n = 95) groups. RESULTS: Overall postoperative CSF leakage was 10.4% (13/125) in this series. There were no significant risk factors for postoperative CSF leakage among the demographic data including BMI, comorbidities, or radiation history. Postoperative CSF leakage was most prevalent in the transclival approach than in other approaches, but the difference was not statistically significant (20.8%, p = 0.351). When dividing the results by timetable, the patients who underwent skull base reconstruction in the early cohort experienced more postoperative CSF leakage (23.3%, 7 cases out of 30) than in the late cohort (6.3%, 6 cases out of 95, p = 0.014). The learning curve was steeper in the early cohort (30 early cases 23.3%, 31-60 10%, 61-90 6.7%, 91-125 2.9%). CONCLUSIONS: To improve the success rate of endoscopic skull base reconstruction, surgeons have to keep the basic technical details in mind to reduce the learning curve.

Age-associated changes in chronic rhinosinusitis endotypes.

BACKGROUND: Immunologic function in innate and adaptive immunity changes with the ageing process. Thus, age-related cytokine profiles in chronic rhinosinusitis (CRS) need to be investigated for precision medicine. OBJECTIVE: The objective of this study was to characterize age-related changes in immunologic profiles according to CRS subtypes. METHODS: Subjects in control (n = 29), CRS without nasal polyps (CRSsNP, n = 86), and CRS with nasal polyps (eosinophilic NP: ENP, n = 81; non-eosinophilic NP: NENP, n = 113) were enrolled in this study. Twenty markers for type 1/2/3 inflammation and other inflammatory processes were measured in homogenates of sinonasal tissues and statistically analysed. RESULTS: In control tissues, type 2/3 and proinflammatory mediators showed an inverse correlation with age. CRSsNP and NENP showed an age-related increase in type 2 cytokines and a decline in type 3 cytokines. Interestingly, the age-related decrease in type 3 mediators was associated with those of CT scores in NENP. ENP showed an age-related increase in type 3 cytokines with type 2 mediators sustained at high levels. Smokers with ENP demonstrated age-associated increases in type 1/2/3 mediators as well as CT scores. These age-related patterns in each CRS were confirmed by statistically adjusting atopy status, smoking history, and disease duration. CONCLUSION: Age-associated cytokine changes differed among CRS subtypes and control tissues. CRSsNP and NENP demonstrated a decline in type 3 mediators and increase in type 2 mediators, whereas type 3 mediators increased with age in ENP.

Beneficial effect of nasal saline irrigation in children with allergic rhinitis and asthma: A randomized clinical trial.

BACKGROUND: Asthma and allergic rhinitis (AR) are chronic inflammatory diseases of airway and affect the disease severity each other. OBJECTIVE: We performed this study to examine whether nasal saline irrigation (NSI) improves bronchial hyperresponsiveness and clinical parameters in children with asthma and allergic rhinitis (AR). METHODS: We enrolled 20 children with AR and asthma aged between 6-18 years. Patients were randomized into two groups: irrigation group (8 boys and 2 girls) and control group (8 boys and 2 girls). The irrigation group performed daily NSI. All patients received 12-week treatment with montelukast, levocetirizine, and inhaled glucocorticoids. Provocative concentrations of methacholine causing a 20% decrease in FEV1 (PC20), Asthma Control Test (ACT), the Questionnaire for Quality-of-Life Specific to Allergic Rhinitis in Korean Children (QQOL-ARK) and exhaled nitric oxide (FENO) were compared before and after the study. RESULTS: The PC20 at week 12 was higher than baseline measurements in the irrigation group (P = 0.017), while there was no difference in PC20 before and after treatment in the control group (P = 0.333). ACT score increased after 12 weeks of NSI (P = 0.007), while QQOL-ARK score decreased compared to baseline scores (P = 0.028) in the irrigation group. No differences in ACT and QQOL-ARK were found between weeks 0 and 12 in the control group. No differences were found in the median value of changes in PC20, ACT, QQOL-ARK and FENO between the irrigation and control groups. CONCLUSIONS: Our results suggest that NSI is beneficial for treatment of asthma and AR in children.

Risk factors for intraoperative saddle nose deformity in septoplasty patients.

INTRODUCTION: Septoplasty is one of the most common otolaryngologic procedures. Previous studies have reported that the overall rate of significant change in cosmetic appearance of the nose after septoplasty ranged from 0.4 to 3.4%, and saddle nose was the most commonly cited deformity. In this study, we evaluated the risk factors for intraoperative saddle nose in a group of septoplasty patients. METHODS: This case-control study (1:2 case:control) was conducted based on retrospective chart review. Intraoperative saddle nose was observed in 108 (5.1%) of 2106 patients who underwent septoplasty in our center between January 2008 and December 2017. The control group consisted of 216 randomly selected, hospital-matched septoplasty patients who had no intraoperative saddle nose deformity in the same period. The demographic data, preoperative endoscopic findings, and surgical procedures of the two groups were analyzed to identify possible risk factors of intraoperative saddle nose deformity. RESULTS: The mean ages of the two groups were 34.8 years (saddle group) and 33.2 years (control group). In multivariate logistic regression analysis, clinical risk factors associated with intraoperative saddle nose were female gender (OR 3.39; 95% CI 1.76-6.54; p < 0.01), severe caudal septal deviation (OR 2.22; 95% CI 1.30-3.79; p = 0.003), and intraoperative finding of septal cartilage fracture (OR 3.96; 95% CI 1.92-8.19; p < 0.01). CONCLUSIONS: Severe caudal septal deviation, intraoperative fracture of septal cartilage, and female gender were risk factors for intraoperative saddle nose deformity in our study population.

Clinical pattern of fungal balls in the paranasal sinuses: our experience with 70 patients.

PURPOSE: Many physicians recommend endoscopic sinus surgery (ESS) even when an asymptomatic paranasal sinus (PNS) fungal ball is detected incidentally. The aim of this study was to investigate the natural behavior of PNS fungal balls via sinus imaging techniques. METHODS: A follow-up study of 74 pathologically confirmed fungus balls was conducted in 70 patients who underwent multiple head and neck computed tomography (CT) scans or magnetic resonance imaging (MRI). We investigated the changes in symptoms and lesion size, as well as any new occurrences. RESULTS: Of the 74 fungus balls detected in 70 patients, we observed the renewed formation of a fungal ball in 21 patients, which was not present on initial imaging conducted over a period of 2-162 months. The fungal ball was already present in 53 patients on the initial screening, and the longest follow-up was 197 months (range 1-197 months). Of these 53 lesions, 29 lesions showed an increase in size (29/53, 54.7%), whereas 12 lesions were not associated with any clinical symptoms (12/53, 22.6%). In the 21 newly formed fungal balls, further development was observed in 10 lesions, with 4 lesions showing an increase in size. Thus, size increment occurred in 33 of the 57 fungus balls. CONCLUSIONS: The fungal balls can exist without local tissue invasion for up to 17 years and new formation of the fungal balls was observed even within 2 months, especially when accompanied by initial clinical symptoms of sinusitis.

Clinical significance of IgG4 in sinonasal and skull base inflammatory pseudotumor.

INTRODUCTION: Inflammatory pseudotumor (IPT) in the sinonasal cavity and skull base region is benign non-neoplastic inflammatory process. However, IPT can mimic malignant tumor or infectious disease and there are difficulties in confirmation of diagnosis. The aim of study is to evaluate the clinical significance of immunoglobulin G4 (IgG4) in IPT in terms of steroid response and differential diagnosis with other skull base infiltrative lesions. METHODS: Medical records were reviewed retrospectively from 1998 to 2016. Subjects diagnosed with IPT by surgical biopsy were enrolled. IgG4 positivity was defined as IgG4/IgG ratio > 0.4. Additionally, IgG4/IgG ratio was calculated in eight skull base osteomyelitis (SBO) patients. RESULTS: Twenty-six IPT patients were included and the average age was 52.3 years, and 57.7% were male and 42.3% were female. Most lesions were involved in the sinuses (88.5%) and the incidence of extension beyond the sinuses itself was as follows: the cheek/hard palate/parapharynx (15.4%), orbit (61.5%), skull base (57.7%), and dura or brain (23.1%). All IPT cases revealed IgG4 + plasma cells and IgG4/IgG ratio over 0.4 was detected in 42.3% (11/26) of cases. In case of SBO, no patients had IgG4/IgG ratio exceed 0.4. Main treatment modality was systemic steroids (61.5%) and other modalities were used: surgery (3.8%), immunosuppressant (7.7%), radiotherapy (30.8%), or a combination of these modalities (15.4%). Steroid responses were not significantly different, but IgG4-positive group tended to have better response to steroid therapy. CONCLUSIONS: IgG4-positive and IgG4-negative IPT patients revealed no differences in involvement sites, clinical course, and steroid responses. However, IgG4/IgG ratio and IgG4 + plasma cell count can provide a diagnostic clue for infiltrative skull base lesions such as IPT and a differential diagnosis of SBO.

Air-conditioning characteristics in nasal cavity models exhibiting nasal cycle states.

The air-conditioning characteristics in nasal cavity models obtained from two subjects exhibiting different degrees of the nasal cycle states in terms of the airflow partition were investigated using computational fluid dynamics. A constant inspiratory flow rate of approximately 250 mL/s was considered, and the air temperature and relative humidity at the inlet were assumed to be 25 °C and 35%, respectively. The air-conditioning capacities of the congested and decongested sides were assessed by the amounts of epithelial heat and water vapor transferred to the inhaled air through the airway from the nostrils to the end of the septum. The results revealed that the air temperature and relative humidity near the end of the septum, respectively, reached approximately 31.4-32.5 °C and 81.4-88.0% in the decongested sides and 34.0-35.9 °C and 95.3-100% in the congested sides. The differences seen in the air temperatures and relative humidity between the congested and decongested sides were found to be larger in the cavity model that showed a larger degree of reciprocal change in the airflow rate. From a fluid mechanics perspective, while the congested side is in a rest period during the nasal cycle such that a lower amount of airflow is transported through it, this side, in effect, works to provide assistive air-conditioning capacity to the nasal cavity and aids when insufficiently conditioned airflow passes through the decongested side so that the inhaled air merging after the septum can approach the alveolar condition favorably through the nasopharynx.

Clinical course of rhinosinusitis and efficacy of sinonasal evaluation in kidney transplant recipients: review of 1589 patients.

INTRODUCTION: Rhinosinusitis in patients who undergo kidney transplantation (KT) might have specific clinical characteristics due to immune status of recipients. The aim of this study was to identify the clinical course of rhinosinusitis after KT and to evaluate the efficacy of routine sinonasal evaluation before KT. METHODS: The study included 1589 kidney transplant adult patients who underwent preoperative sinonasal evaluation including sinonasal symptoms, nasal endoscopy, and plain X-ray between November 1994 and December 2013 (19 years). Demographic data and clinical course of rhinosinusitis were evaluated retrospectively. RESULTS: The study population consisted of 897 men (56.5%) and 692 women (43.5%) with a mean age of 42.5 years (range 18-75 years). The prevalence of pre-KT rhinosinusitis was 4.2% (66/1589), and that of post-KT rhinosinusitis was 0.9% (13/1503). The prevalence of acute rhinosinusitis and chronic rhinosinusitis (CRS) was 0.13 and 2.33% in pre-KT patients and 0.2 and 0.6% in post-KT patients, respectively. The recurrence rate of CRS in pre-KT patients was 37.8%. Of the 27 asymptomatic patients, the recurrence rate was 11.1%. Symptomatic patients had more severe endoscopic findings and higher Lund-Mackay CT scores than asymptomatic patients. The prevalence of fungal ball (0.8% in pre-KT and 0.3% in post-KT patients) was similar to that in the general population, and only one patient experienced invasive fungal rhinosinusitis after KT. No patient with pre-KT rhinosinusitis experienced severe complications. CONCLUSIONS: The prevalence and recurrence rate of rhinosinusitis in KT patients was not increased after KT. Symptomatic patients needed surgical or medical treatment before KT, but asymptomatic patients showed no deterioration of clinical course after KT. Routine sinonasal evaluation for asymptomatic patients is not recommended before KT.

Impact of gender and sleep position on relationships between anthropometric parameters and obstructive sleep apnea syndrome.

OBJECTIVE: Considering the mechanisms by which obesity affects obstructive sleep apnea syndrome (OSAS) and the differences of fat distribution depending on gender, associations between anthropometric parameters, and OSAS may differ depending on gender or sleep position. We analyzed the impact of gender and sleep position on the relationship between fat distribution and development of OSAS. METHODS: One thousand thirty-two consecutive subjects were analyzed. Recorded anthropometric measurements and overnight polysomnographic data of the subjects were reviewed retrospectively. The presence of OSAS was defined by the respiratory disturbance index (RDI) ≥5 with documented symptoms of excessive daytime sleepiness. RESULTS: Eight hundred fifty-eight males and 174 females were included. Male subjects had significantly higher body mass index (BMI), larger waist circumference (WC), and lower percent of overall body fat (P < 0.0001, P < 0.0001, and P < 0.0001, respectively). The severity of OSAS was significantly higher in male subjects (RDI 26.9 ± 22.4 in males vs. 10.2 ± 13.8 in females, P < 0.0001). In male subjects, BMI, WC, and overall body fat were significantly associated with severity of OSAS and had larger impacts on supine RDI than lateral RDI. Overall body fat was not associated with severity of OSAS in female subjects, and there were no significant differences of the associations between all anthropometric parameters and RDIs depending on sleep position. CONCLUSIONS: Evaluation of the correlation of anthropometric data with severity of OSAS should consider sleep position as well as gender.

Effect of uvulopalatopharyngoplasty on CPAP compliance.

In this retrospective cohort study, we tried to evaluate the compliance of continuous positive airway treatment (CPAP) in the patients with or without previous surgical treatment to OSAS. Retrospective study with prospectively collected data was performed. Eighty-six consecutive OSAS patients who decided to try CPAP were enrolled, and data regarding polysomnography, history of previous surgical treatment and CPAP usage were collected. Among them, nine patients who had history of uvulopalatopharyngoplasty for OSAS were included in the surgery group and another 77 non-surgical patients were assigned to the untreated group. There was no statistical difference for demographics or polysomnographic parameters. They showed similar CPAP acceptance and air leak after the first trial. However, the surgery group (6.39 ± 1.49 h/day) used CPAP significantly longer than the untreated group (4.73 ± 1.64 h/day; p = 0.007) and less troubled with 'waking up during night'. The present study showed that the prior surgery for OSAS doesn't preclude the subsequent CPAP therapy.

Sinonasal evaluation and treatment before liver transplantation: a retrospective review of 982 patients.

Immunosuppression after liver transplantation (LT) increases complications from infection. The usefulness of sinonasal evaluation before LT has not been fully evaluated. The aim of this study was to determine the usefulness of routine sinonasal evaluation before LT and pre-transplant treatment of sinusitis. The clinical records of 982 adult patients (age ≥18 years) who underwent LT between January 1997 and June 2011 were retrospectively reviewed and analyzed. A total of 920 patients (93.7 %) underwent pre-LT sinonasal evaluation, including a sinonasal symptom questionnaire, nasal endoscopy and plain X-ray. Of these patients, 269 (29.2 %) had abnormal findings and underwent paranasal CT scans. Based on CT, 102 patients, including 21 with fungal ball, were diagnosed with sinusitis and 62 (60.8 %) underwent LT after medical or surgical treatment for sinusitis. Another 40 patients (33 with sinusitis and 7 with fungal ball) underwent LT without treatment for sinusitis. No difference in infectious complications was observed according to sinusitis treatment type. Among chronic rhinosinusitis patients, those who underwent LT (n = 48) after adequate treatment had a lower mortality rate than those who did not receive treatment (n = 33) (12.5 vs. 33.3 %; p = 0.024). Sinonasal evaluation preceding LT did not prevent infectious complications but untreated sinusitis might be correlated with increased mortality after LT. Routine pre-LT sinonasal evaluation would be considered for safer transplantation.

Cervicofacial tissue infarction in patients with acute invasive fungal sinusitis: prevalence and characteristic MR imaging findings.

INTRODUCTION: Tissue infarction is known as one of the characteristic features of invasive fungal sinusitis (IFS). The purpose of this study was to investigate the prevalence and characteristic MR imaging findings of cervicofacial tissue infarction (CFTI) associated with acute IFS. METHODS: We retrospectively reviewed MR images in 23 patients with histologically or microbiologically proven acute IFS. CFTI was defined as an area of lack of enhancement in and around the sinonasal tract on contrast-enhanced T1-weighted images. We divided CFTI into two groups, i.e., intrasinonasal and extrasinonasal. Particular attention was paid to the location of extrasinonasal CFTI and the signal intensity of CFTI on T1- and T2-weighted images. The presence of bone destruction on CT scans was also recorded. RESULTS: CFTI was found in 17 (74%) of 23 patients. All of these 17 patients had intrasinonasal CFTI, and 13 patients also had extrasinonasal CFTI. All 13 patients with extrasinonasal CFTI died of disease directly related to IFS. Various locations were involved in the 13 patients with extrasinonasal CFTI, including the orbit (n = 8), infratemporal fossa (n = 7), intracranial cavity (n = 3), and oral cavity and/or facial soft tissue (n = 4). Various signal intensities were noted at the area of CFTI on T1- and T2-weighted images. Bone destruction was found on CT scans in only 3 of 17 patients with CFTI. CONCLUSION: CFTI with preservation of the bony wall of the involved sinonasal tract may be a characteristic MR imaging finding of acute IFS. The mortality is very high once the lesion extends beyond the sinonasal tract.

Dynamic characteristics of heat capacity of the human nasal cavity during a respiratory cycle.

The dynamic characteristics of air-conditioning in the human nasal cavity during a respiratory cycle were investigated using unsteady numerical simulations to assess whether inhaled air is sufficiently conditioned by the nasal cavity. Variations in the epithelial surface temperature, surface heat, and water vapor fluxes were found to vary significantly during inspiration while providing substantial air conditioning to the inhaled air, but variations and magnitudes were significantly reduced during the expiration period. Air temperature (31.3-35.3 °C) and relative humidity (85.1-100 %) in the nasopharynx exhibited significant variations during inspiration. Flow rate-weighted average values of the air temperature and relative humidity during inspiration were estimated to be 32.0 °C and 89.1 %, respectively. Inhaled air did not attain alveolar conditions before reaching the nasopharynx, and was therefore thought to be insufficiently conditioned by the nasal cavity alone. A steady flow of approximately 250 mL/s appears to be useful for evaluating the accumulated thermal state of air in the nasopharynx during inspiration.

Hypoxemia During Sleep and the Progression of Coronary Artery Calcium.

The aim of this study was to evaluate the associations between objective sleep parameters of obstructive sleep apnea (OSA) and progression of subclinical cardiovascular disease as measured by the coronary artery calcium (CAC) score. We reviewed the medical records of 196 patients who underwent both polysomnography (PSG) and repeated coronary artery computed tomography (CT) for screening purposes. For each participant, the first coronary CT scan was conducted within 12 months of PSG. Follow-up CT was performed voluntarily. The CAC score was log-transformed to obtain normally distributed data. We evaluated potential associations between various sleep parameters by analyzing overnight-attended PSG and CAC score progression over time. ST90 (total sleep time of SaO2 < 90%), CT90 (percentage of time of SaO2 < 90%), and degree of mean oxygen desaturation were significantly correlated with CAC score progression even after adjustment for confounders (age, sex, DM, HTN, hypercholesterolemia, BMI, and smoking status) (estimate = 0.004, p = .010; estimate = 0.009, p < .001; estimate = 0.027, p = .001; respectively). We also performed subgroup analysis and found that the progression of CAC score over time showed higher tendency when CT90 value was 2.73 or more (CT90 ≥ 2.73 group; estimate = 0.336, CT90 < 2.73 group; estimate = 0.194, p < .001 each). ST90, CT90 and mean oxygen desaturation are significant predictors of cardiovascular disease progression. Coronary artery status should be monitored repetitively in patients with hypoxemia during sleep.

Potential Therapeutic Effect of Continuous Positive Airway Pressure on Laryngopharyngeal Reflux in Obstructive Sleep Apnea Patients.

To investigate the potential therapeutic effect of continuous positive airway pressure (CPAP) treatment on laryngopharyngeal reflux in obstructive sleep apnea (OSA) patients, we performed a retrospective analysis of data prospectively collected from patients who underwent CPAP therapy after being diagnosed with moderate to severe OSA between January 2019 and May 2020. Subjects were asked to complete the reflux symptom index (RSI) questionnaire before and after CPAP. Additionally, a laryngoscopic examination was performed to evaluate objective endoscopic findings and determine reflux finding score (RFS). A total of 46 patients were included in the analysis. Overall, significant decreases in mean RSI score (10.85 ± 6.40 vs. 8.80 ± 7.99, p < 0.001) and RFS (7.41 ± 3.32 vs. 4.65 ± 2.12, p < 0.001) were observed after CPAP treatment. Within subdomains of the RSI, throat clearing, postnasal drip, breathing difficulty, troublesome cough, and foreign body sensation were significantly improved by CPAP treatment. All subdomains of RFS, with the exception of posterior commissure hypertrophy and granuloma, showed significant differences after CPAP treatment. There were no differences between subgroups according to body mass index or severity of OSA. CPAP treatment in OSA potentially reduces laryngeal reflux symptoms and improves laryngeal examination findings.

The Potential Esthetic Effect of Endonasal Septoplasty on the Cartilaginous Dorsum in Asian Population.

Importance: Septoplasty is used to correct nasal obstructions caused by a deviated septum. In some patients, septal deviation is also associated with external nasal deformity, which suggests that proper septoplasty can improve external deformities as well. However, objective studies of the esthetic outcome of septoplasty are limited. Objective: To investigate the potential esthetic outcome of correction of septal in addition to its functional purpose of treating nasal obstructions. Design, Setting, and Participants: Retrospective chart analysis of prospectively collected data was conducted for patients who underwent septoplasty performed by a single surgeon at our clinic. Ninety-five patients who had preoperative and postoperative medical photographs were enrolled. Main Outcomes and Measures: We measured the mid-vault dorsum angle (MDA) from a bird's eye view to evaluate the objective esthetic outcome. The nasofrontal angle and the nasolabial angle were also included in our analysis. Differences in preoperative anthropometric measurements and application of specific surgical techniques were additionally analyzed. Results: The mean age of the participants was 39.1 years (male: 85.2%, female: 14.8%). The average preoperative MDA was 3.41° ± 3.05°, and the postoperative MDA was 1.88° ± 1.09° (p < 0.001). In the severe dorsum deviation group (MDA >4°, N = 31), angle measure improvement was prominent (3.82° ± 2.97°, p < 0.001). The correlation analysis revealed that a higher preoperative MDA had a significant correlation with a higher angle improvement postoperatively (r = 0.7, p < 0.001); this outcome was also observed in the severe dorsal deviation group (r = 0.6, p = 0.003). In addition, we found a positive correlation between the bony septal deviation angle on computed tomography and preoperative MDA (r = 0.51, p = 0.003). Conclusions and Relevance: In some cases with significant external dorsum deviation, septoplasty via an endonasal approach can yield positive esthetic outcomes in addition to functional improvements. This finding will benefit patient counseling and also provide important surgical concepts in rhinoplasty with an emphasis on a gradual surgical approach to correcting external nasal deformity.

Long-Term Clinical Course of Benign Fibro-Osseous Lesions in the Paranasal Sinuses.

OBJECTIVES: Fibro-osseous lesions of the paranasal sinuses can present various clinical manifestations. This study aimed to report the long-term clinical course of benign fibro-osseous lesions (BFOLs) in the paranasal sinuses, including clinical and radiologic features. METHODS: Radiologically confirmed BFOLs between 1994 and 2016, with the exclusion of osteoma cases, were retrospectively reviewed. We compared demographic characteristics between the surgery and observation groups. The reasons for the imaging study, radiographic features, histopathology, and clinical course based on serial image scans were analyzed. RESULTS: In total, 183 subjects were selected from a thorough review of head and neck radiologic tests (n=606,068) at a tertiary referral hospital over 22 years. Patients' mean age was 28.6±18.1 years, and 56.3% were males. A diagnostic imaging workup was performed in 55.7% of patients due to facial asymmetry, headache, skull mass, or other symptoms related to BFOLs. In other patients (37.7%), BFOLs were found incidentally on computed tomography or magnetic resonance imaging. The most common diagnosis was fibrous dysplasia, followed by ossifying fibroma, based on both radiologic exams and histopathologic results. In total, 42.6% of the patients underwent surgery because of subjective symptoms or esthetic concerns. The patients who underwent surgery were younger (P<0.001) and had a longer follow-up duration (P<0.001) than those who underwent observation. Patients who experienced lesion growth (11.5%) were younger (P<0.001) and had more lesion sites (P=0.018) than those who did not, regardless of surgical treatment. Five patients underwent optic nerve decompression, and one patient experienced malignant transformation. CONCLUSION: BFOL in the paranasal sinuses is a rare disease, and most cases were observed without specific treatment. Surgical treatment should be considered in symptomatic patients with aggressive clinical features. Regular observation and management are needed, particularly in younger patients in their teens.

Genetic Variants and Clinical Phenotypes in Korean Patients With Hereditary Hemorrhagic Telangiectasia.

OBJECTIVES: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by recurrent epistaxis, telangiectasia, and visceral arteriovenous malformations (AVMs). Activin A receptor-like type 1 (ACVRL1/ALK1) and endoglin (ENG) are the principal genes whose mutations cause HHT. No multicenter study has yet investigated correlations between genetic variations and clinical outcomes in Korean HHT patients. METHODS: Seventy-two members from 40 families suspected to have HHT based on symptoms were genetically screened for pathogenic variants of ACVRL1 and ENG. Patients with genetically diagnosed HHT were also evaluated. RESULTS: In the HHT genetic screening, 42 patients from 24 of the 40 families had genetic variants that met the pathogenic criteria (pathogenic very strong, pathogenic strong, pathogenic moderate, or pathogenic supporting) based on the American College of Medical Genetics and Genomics Standards and Guidelines for either ENG or ACVRL1: 26 from 12 families (50%) for ENG, and 16 from 12 families (50%) for ACVRL1. Diagnostic screening of 42 genetically positive HHT patients based on the Curaçao criteria revealed that 24 patients (57%) were classified as having definite HHT, 17 (41%) as having probable HHT, and 1 (2%) as unlikely to have HHT. Epistaxis was the most common clinical presentation (38/42, 90%), followed by visceral AVMs (24/42, 57%) and telangiectasia (21/42, 50%). Five patients (12%) did not have a family history of HHT clinical symptoms. CONCLUSION: Only approximately half of patients with ACVRL1 or ENG genetic variants could be clinically diagnosed as having definite HHT, suggesting that genetic screening is important to confirm the diagnosis.

Nasal polyps with metaplastic ossification: CT and MR imaging findings.

INTRODUCTION: Metaplastic ossification is a rare event in nasal polyps. The purpose of this study was to review the computed tomography (CT) and magnetic resonance (MR) imaging findings of nasal polyps with metaplastic ossification. METHODS: CT (n=5) and MR (n=3) images of five patients (four men and one woman; mean age, 59 years) with surgically proven nasal polyp with metaplastic ossification were retrospectively reviewed. The location and morphologic characteristics of metaplastic ossification were documented as well. RESULTS: All lesions were seen as lobulated (n=3), ovoid (n=1), or dumbbell-shaped (n=1) benign-looking masses with a mean size of 3.7 cm (range, 2.4-6.5 cm), located unilaterally in the posterior nasal cavity and nasopharynx (n=2), posterior nasoethmoidal tract (n=2), and maxillary sinus and nasal cavity (n=1). Compared with the brain stem, the soft tissue components of all lesions demonstrated isoattenuation on precontrast CT scans, slight hypointensity on T1-weighted MR images, and hyperintensity on T2-weighted MR images. On contrast-enhanced MR images, heterogeneous enhancement with marked peripheral enhancement was seen in two and homogeneous moderate enhancement in one. All lesions contained centrally located radiodense materials on CT scans, the shape of which was multiple clustered in three, single nodular in one, and single large lobulated in one. CONCLUSION: Although rare, metaplastic ossification can occur within nasal polyps. The possibility of its diagnosis may be raised when one sees a benign-looking sinonasal mass with centrally located radiodense materials on CT scans. MR imaging may be useful when mycetoma or inverted papilloma cannot be ruled out on CT scans.