Rare Extranodal Manifestation of Rosai-Dorfman Disease Presenting as Nasal Obstruction and its Management.

Rosai-Dorfman disease (RDD) is a rare and benign lymphoproliferative disorder that commonly presents as painless, bilateral neck swelling. Extranodal presentations are considered rare, but the most common extranodal locations involved include skin, subcutaneous followed by nasal/paranasal sinuses. Although it is a benign condition, it may be mistaken as a malignant lesion and requires a biopsy for diagnostic confirmation. In this study, we report a rare case of RDD with bilateral neck node and nasal/paranasal sinus involvement which initially presented with bilateral nasal obstruction. And, we reviewed the management in this unusual case and discussed the helpful role imaging studies play in the further workup and subsequent follow-up to treatment response.

Solitary Fibrous Tumour: A Rare Differential Diagnosis of Unilateral Nasal Mass.

Solitary fibrous tumors of the nasal cavity and paranasal sinuses are rarely encountered in clinical practice. These are unusual mesenchymal tumours initially described as primary spindle-cell neoplasms. Such tumours may manifest in pleural and extrapleural sites such as the liver, parapharyngeal space, sublingual and parotid glands, and thyroid but are seldom described in the nose and paranasal sinus region. Erosion of adjacent structures may occur, but the tumour itself does not metastasise. A young patient presented with a progressive unilateral nasal mass. The initial nasal biopsy reported it as a benign inflammatory nasal polyp. Imaging revealed a large, locally expansile mass within the right nasal cavity displacing the nasal septum. The patient underwent excision of the tumour and the diagnosis of solitary fibrous tumour was confirmed by immunohistochemistry staining. This case is intended to highlight the diagnosis and management of this rare tumour.

Fourth Branchial Pouch Sinus: A Rare, Often Misdiagnosed Congenital Anomaly.

Pathologies of the branchial apparatus originate from abnormal embryological development and frequently affects the second branchial cleft. Fourth branchial cleft lesions are exceptionally few and existing literature on it is scarce. We present a case of a fourth branchial pouch sinus, masquerading as a recurrent neck abscess.

A Rare Case of a Nasopharyngeal Pleomorphic Adenoma Mimicking a Nasopharyngeal Carcinoma.

Pleomorphic adenoma is the most common benign pathology of the major salivary gland but rare in the minor salivary gland, especially in the nasopharynx, with only a few cases reported in the literature. A 76-year-old lady presented with bilateral nasal blockage for one year secondary to a nasopharyngeal mass. Histopathological examination reported it to be nasopharyngeal carcinoma, but the mass persisted after a course of chemotherapy combined with radiotherapy. Upon repeat biopsy, the mass was found to be a pleomorphic adenoma. The patient underwent nasopharyngectomy without complications and no evidence of recurrence after 18 months of follow-up.

Knee measurements among children with normal alignment, physiologic and pathologic bowing aged 0-3 years old: a systematic review.

Distinguishing physiologic and pathologic genu varus is challenging among children below age 3. They can be assessed by measuring intercondylar distance (ICD), clinical tibiofemoral angle (cTFA) or radiologic TFA (rTFA). We aim to determine the knee measurement values among three groups of children. Medline (1946-) and EMBase (1947-) were searched until 31 July 2020 using a search strategy. Studies with original data which reported knee measurements among children with normal alignment, physiologic and pathologic bowing between the ages of 0-3 years were included. In total 1897 studies were identified, and 16 studies included after title and abstract screening. These studies involved 1335 patients with normal alignment, 286 with physiologic and 184 with pathologic bowing. Five studies provided data on ICD, seven on cTFA and eight on rTFA which were pooled for meta-analyses. Normal children had neither measurable ICD nor demonstrable varus on cTFA after 19 months old. The mean (95% confidence interval) ICD for children with pathologic genu varus at 18 months was 4.41 (4.19-4.63). The rTFA for children with pathologic compared to the physiologic bowing by age groups was; 11-20 months: 24.74°(23.22°-26.26°) vs. 19.44°(17.05°-21.84°), 21-30 months: 20.35°(18.13°-22.56°) vs. 14.72°(12.32°-17.12°) and 12-36 months: 32.60°(26.40°-38.80°) vs. 19.14°(17.78°-20.50°). Children above the age of 18 months with genu varus should be closely monitored clinically using ICD or cTFA. An ICD of more than 4 cm may warrant further investigation for pathologic cause. rTFA has limited use in the detection of pathologic varus.

Recurrent Nasal Septal Hematoma and Abscess: A Rare Manifestation of Leukemia.

Nasal septal abscess and hematoma are rare clinical entities. To the best of our knowledge, there have only been 2 cases of nasal septal abscess associated with haematological malignancy reported in the literature. Herein, we present a unique case of recurrent spontaneous nasal septal hematoma and abscess in a patient prior to and after the diagnosis of acute myelogenous leukemia. Its rarity in immunocompromised population, clinical presentation, treatment and complications are further discussed.

The Clinical and Radiological Characterizations of the Allergic Phenotype of Chronic Rhinosinusitis with Nasal Polyps.

PURPOSE: The allergic phenotype of chronic rhinosinusitis (CRS) and central compartment atopic disease (CCAD) have been described. The CCAD is a radiological phenotype in patients with CRS that presents as a central mucosal disease due to allergy. The subset of patients having chronic rhinosinusitis with nasal polyps (CRSwNP) has not been well characterized. We aim to describe the clinical and radiological characterizations of patients presenting with the allergic phenotype of CRSwNP. PATIENTS AND METHODS: A cross-sectional study at a tertiary hospital was performed. Adult patients diagnosed with CRSwNP who had both allergology and radiological assessments were enrolled. The symptoms of allergic rhinitis, Lund-Kennedy (LK) endoscopic scoring, Lund-Mackay (LM) computed tomography scan of paranasal sinuses (CTPNS) scoring, CCAD features, skin prick test (SPT) and level of specific IgE were assessed. All the patients underwent SPT for house dust mites. RESULTS: A total of 38 patients were enrolled. Symptoms, endoscopic and CTPNS scores were higher in the allergy and CCAD groups compared to the nonallergy and nonCCAD groups. The symptom of "need to blow nose" was statistically significant in allergy vs nonallergy (p=0.01) and CCAD vs nonCCAD (p=0.02). There were significant differences in the endoscopic scores in both allergy and CCAD (allergy vs nonallergy, p=0.01; CCAD vs nonCCAD, p=0.03), and CT scores in both allergy and CCAD (allergy vs nonallergy, p=0.02; CCAD vs nonCCAD, p=0.02). All patients with CCAD have worse scoring than nonCCAD (LK score, p=0.03; LM score, p=0.02). Patients with allergy have more polypoidal involvement of the middle turbinates (left middle turbinate, p=0.141; right middle turbinate, p=0.074) and CCAD (left middle turbinate, p=0.017; right middle turbinate, p=0.009) than nonallergy and nonCCAD patients. CONCLUSION: Allergic phenotype of CRSwNP has a worse clinical and radiological disease burden. Optimal treatment of allergy is essential for a better outcome.

Severe epistaxis in pregnancy due to nasal pyogenic granuloma: A case report.

Mild to severe epistaxis is common in pregnancy and often results from increased vascularity of the nasal mucosa and hormonal changes. Symptoms may occur in the absence of an obvious local cause or any systemic disorder; however, thorough otolaryngological (i.e., "ENT") evaluation is always warranted. Pyogenic granuloma or lobular capillary haemangioma is a benign fibrovascular proliferative tumour that is commonly found on the face, fingers, lips, and nasal mucosa. Pregnancy-induced pyogenic granuloma is not an uncommon entity and may result in torrential epistaxis if untreated. Managing a case of severe epistaxis during pregnancy usually requires multidisciplinary management. The authors present a case of severe epistaxis in pregnancy that necessitated examination of the nasal cavity under general anaesthesia. Intraoperative findings showed a bluish-red mass occupying the patient's right maxillary sinus. Bleeding was arrested and complete haemostasis was achieved. The nasal pyogenic granuloma completely resolved in the post-partum period.

Outcomes of pituitary surgery for Cushing's disease: a systematic review and meta-analysis.

PURPOSE: Transsphenoidal surgery (TSS) is the first-line treatment for Cushing's disease (CD). This review aimed to synthesize the remission and recurrence rates following TSS for CD and identify predictors of these outcomes. METHODS: Medline (1946-) and Embase (1947-) were searched until 23rd January 2019 for original studies. A meta-analysis was performed of remission and recurrence rates. Studies were excluded if patients had prior radiosurgery/radiotherapy, mixed pathologies or interventions without separated data, follow-up not reported or population size < 20. For recurrence rate syntheses, studies with follow-up < 6 months were excluded. RESULTS: The search produced 2663 studies, of which n = 68 were included, involving 5664 patients. Remission rates after primary and revision TSS were 80% [77-82] and 58% [50-66] at last follow-up. After primary TSS, predictors of remission were micro- v macroadenomas (83% v 68%, p < 0.01), imaging-visible adenomas (81% v 69%, p < 0.01), adenomas confirmed on histopathology (87% v 45%, p < 0.01), absence of cavernous sinus invasion (80% v 30%, p < 0.01), postoperative serum cortisol (MSeC) nadir < 2 µg/dL (< 55 nmol/L; 95% v 46%, p < 0.01) and lower preoperative 24-h urine free cortisol (1250 nmol v 1726 nmol, p < 0.01). For revision TSS, predictors of remission were postoperative MSeC nadir < 2 µg/dL (< 55 nmol/L; 100% v 38%, p < 0.01) and operations for recurrence v persistence (80% v 54%, p < 0.01). Recurrence rates after primary and revision TSS were 18% [14-22] and 28% [16-42]. CONCLUSIONS: TSS is most effective in primary microadenomas, visible on preoperative imaging and without CS invasion, lower preoperative 24-h urine free cortisol and postoperative MSeC nadir < 2 µg/dL (< 55 nmol/L).

Nasal mucosal brushing as a diagnostic method for allergic rhinitis.

Background: Allergen specific immunoglobulin E (spIgE) in the nasal mucosa is a biomarker for local allergic rhinitis. Inferior turbinate tissue biopsy is a sensitive method to detect nasal spIgE but is invasive. Nasal brushing is a relatively noninvasive method to detect nasal spIgE that may be of comparable diagnostic utility. Objective: To assess the performance of nasal brushing to obtain a nasal spIgE sample compared with an inferior turbinate tissue biopsy among patients who underwent turbinate surgery. Methods: A diagnostic cross-sectional study that involved participants who were undergoing turbinate surgery was performed. Nasal brushing, inferior turbinate tissue biopsy, blood collection, and skin-prick test (SPT) were performed perioperatively and tested for house-dust allergens. A receiver operating curve was used to assess the performance of the nasal brushings to obtain nasal spIgE samples compared with the inferior turbinate tissue biopsy. The diagnostic utility of nasal brushings of spIgE compared with serum spIgE testing and SPT was also assessed. Results: A total of 157 patients (41.61 ± 14.83 years; 37.6% women) were included. Nasal brushing was an excellent method to sample for nasal spIgE compared with inferior turbinate tissue biopsy (Area under curve (AUC) 0.87 [95% confidence interval {CI}, 0.81-0.93], p < 0.01). Positive house-dust allergen spIgE results of nasal brushings was defined as > 0.1 kUA/L. Nasal brushings for spIgE sampling was also able to predict the presence of serum spIgE (AUC 0.93 [95% CI, 0.89-0.97], p < 0.01) and SPT (AUC 0.80 [95% CI, 0.72-0.87], p < 0.01). Conclusion: Nasal brushing constituted an easy and relatively noninvasive method to sample nasal epithelium. This sampling technique was comparable with an inferior turbinate tissue biopsy and may be developed as a diagnostic tool for the diagnosis of local allergic rhinitis.

Turbinate-Specific IgE in Normal and Rhinitic Patients.

BACKGROUND: Specific immunoglobulin E (sIgE) within the nasal airway is likely to be the most ideal marker of allergic status, but little is known of the normative values in asymptomatic patients and those with rhinitis. OBJECTIVE: The aim of this study was to assess the diagnostic characteristics of inferior turbinate tissue biopsy sIgE in asymptomatic and rhinitic patients. METHODS: A diagnostic cross-sectional study was undertaken, involving patients who underwent inferior turbinate surgery with or without other surgical interventions. Inferior turbinate tissue biopsy was performed during surgery and was assessed for allergen sIgE (dust mite, grass [temperate or subtropical], and animal epithelium) using an automated immunoassay. Tissue sIgE was assessed among asymptomatic patients and those with nasal symptoms. Data were presented as median (interquartile range). A receiver operating curve was used to predict the diagnostic utility of turbinate tissue sIgE in determining allergic rhinitis. RESULTS: A total of 160 patients (41.89 ± 14.65 years, 36.9% females) were included. The median tissue sIgE concentration among the asymptomatic nonatopic group of patients was 0.09 (0.08-0.10) kUA/L and tissue sIgE > 0.10 kUA/L was determined as a positive threshold. Inferior turbinate tissue sIgE was shown to be a predictive test for allergic rhinitis (area under curve: 0.87, 95% confidence interval: 0.84-0.90) with 90% sensitivity and 89% negative predictive value. CONCLUSION: Inferior turbinate tissue biopsy sIgE is a sensitive tool to predict allergic rhinitis. The threshold value of 0.1 kUA/L corresponded well with the asymptomatic nonatopic group of patients. This method detects sIgE in the nasal mucosa and may be a useful test for allergic rhinitis in future research.

Finding the Petroclival Carotid Artery: The Vidian-Eustachian Junction as a Reliable Landmark.

Introduction Identifying the internal carotid artery (ICA) when managing petroclival and infratemporal fossa pathology is essential for the skull base surgeon. The vidian nerve and eustachian tube (ET) cartilage come together at the foramen lacerum, the vidian-eustachian junction (VEJ). The ICA position, relative to the VEJ is described. Methods Endoscopic dissection of adult fresh-frozen cadaver ICAs and a case series of patients with petroclival pathology were performed. The relationship of the VEJ to the ICA horizontal segment, vertical segment, and second genu was assessed. The distance of the ICA second genu to VEJ was determined in coronal, axial, and sagittal planes. The length of the vidian nerve and ET was measured from the pterygopalatine fossa (PPF) and nasopharyngeal orifice to the VEJ. Results In this study, 10 cadaver dissections (82.3 ± 6.7 years, 40% female) were performed. The horizontal petrous ICA was at or behind VEJ in 100%, above VEJ in 100%, and lateral to VEJ in 80%. The vertical paraclival segment was at or behind VEJ in 100%, above in 100%, and medial in 100%. The second genu was at or behind VEJ in 100% (3.3 ± 2.4 mm), at or above in 100% (2.5 ± 1.6 mm), and medial in 100% (3.4 ± 2.0 mm). The VEJ was successfully used to locate the ICA in nine consecutive patients (53.3 ± 13.6 years, 55.6% female) where pathology was also present. The VEJ was 15.0 ± 6.0 mm from the ET and 17.4 ± 4.1 mm from the PPF. Conclusion The VEJ is an excellent landmark as it defines both superior and posterior limits when isolating the ICA in skull base surgery.

Systemic Predictors of Eosinophilic Chronic Rhinosinusitis.

Background Eosinophilic chronic rhinosinusitis (eCRS) is linked with skewed T-helper 2 or immunoglobulin E (IgE)-mediated allergic responses, with differing diagnosis, prognosis, and management to non-eCRS. Objective The association between biomarkers and eCRS was investigated to assess the predictors of eCRS. Methods A cross-sectional study of adult patients with chronic rhinosinusitis (CRS) undergoing endoscopic sinus surgery was conducted. eCRS was defined by histopathological assessment showing >10 eosinophils/high-power field on sinus mucosal biopsy. Blood tests were performed preoperatively and assessed for a full blood count including eosinophils and a white cell count (WCC) as well as biochemical markers of inflammation and atopy including Immunoglobulin E (IgE), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and ImmunoCAP testing for serum-specific IgE. Comparisons between eCRS and non-eCRS patients were performed. Results 345 patients (48.1% female, age 48.72 ± 15.06 years) were recruited, with 206 (59.7%) identified as eCRS, 41% with asthma and 47% CRS with nasal polyps. eCRS patients were more likely to have asthma ( P < .01) and nasal polyps ( P < .01). Blood eosinophils were significantly elevated in eCRS (0.42±0.34 vs 0.17±0.13 × 109/L, P < .01) as were eosinophils as a ratio of WCC (6.21 ± 4.48 vs 2.55 ± 1.84, P < .01). ESR was decreased when compared with non-eCRS (8.1±7.87 vs 10.65±11.91, P = .03). Receiver operating characteristic curve analysis predicted high tissue eosinophilia at blood eosinophil levels above 0.24 × 109/L (sensitivity 70.9%, specificity 78.4%, area under the curve [AUC]: 0.792, P < .01). eCRS was predicted at eosinophil above 4.27% of total WCC (sensitivity 64.1%, specificity 88.5%, AUC 0.797; P < .01; positive predictive value 89.2%, negative predictive value 62.4%, positive likelihood ratio 5.57, and diagnostic odds ratio 13.71). There was no significant association among WCC, CRP, IgE, or ImmunoCAP testing. Conclusion eCRS is associated with elevated blood eosinophils (>0.24 × 109/L), eosinophil ratio (>4.27% of total WCC), and lower ESR when compared with non-eCRS.

Optimizing Protein Harvest From Nasal Brushings for Determining Local Allergy Responses.

Background Rhinitis is a highly prevalent yet often misdiagnosed condition. Patients who have local allergic rhinitis are regularly mislabeled as having a nonallergic etiology. Thus, a highly accurate, reproducible, and noninvasive assessment, which can be performed quickly and with minimal discomfort to the patient, is required. Objective The aim of this research was to identify the efficiency of various nasal brushes as tools for harvest and collection of epithelial proteins and its suitability for identification of rhinitis. Methods Nasal epithelial mucosa samples were taken from patients undergoing turbinate surgery using a cytology brush, a dental brush, and a nasal curette in random order. After washing in phosphate-buffered saline, the suspended cells were sonicated. Total protein content was assessed for all samples by bicinchoninic acid assay measured using a Nanodrop machine. Identification of nasal-specific immunoglobulin E (spIgE) was then assessed using immunoassay and compared to the patient's allergic status from epicutaneous and serum testing. The lower threshold limit for the spIgE in nasal brushings was determined using the results of serum spIgE tests as the reference. The diagnostic accuracy of this new established cutoff value was determined. Results The cytology brush was found to be the optimal tool for maximal nasal mucosa protein collection followed by dental brush and nasal curette (0.75 ± 0.45 mg/mL vs 0.43 ± 0.24 mg/mL vs 0.071 ± 0.55 mg/mL, respectively; P < .01). The optimal cutoff value of nasal spIgE from the cytology nasal brushings was 0.14 kUA/L to predict allergic status from serum testing. This gave a sensitivity of 75%, specificity of 86%, positive predictive value of 74%, likelihood ration positive of 5.40, and diagnostic odds ratio of 18.62. Conclusion The cytology brush is the optimal tool for protein collection. This is an easy and direct method to sample the nasal mucosa for assessment of nasal allergy or future biomarkers.

Allergic phenotype of chronic rhinosinusitis based on radiologic pattern of disease.

OBJECTIVES/HYPOTHESIS: Polypoid edema of the middle turbinate is a marker of inhalant allergy. Extensive edematous changes may result in limited central nasal and sinus disease, which has been called central compartment atopic disease (CCAD). Radiologically, this is seen as soft tissue thickening in the central portion of the sinonasal cavity with or without paranasal sinus involvement. When the sinuses are involved, the soft tissue thickening spares the sinus roof or lateral wall (centrally limited). This centrally limited radiological pattern was assessed among chronic rhinosinusitis (CRS) patients and compared to allergy status. STUDY DESIGN: Diagnostic cross-sectional study. METHODS: This study included consecutive CRS patients without prior sinus surgery. Computed tomography (CT) scans of the paranasal sinuses were blindly assessed and allergy status was confirmed by serum or skin testing. Individual sinus cavities were defined as either centrally limited or diffuse disease. The radiological pattern that may predict allergy was determined, and its diagnostic accuracy was calculated. RESULTS: One hundred twelve patients diagnosed to have CRS, representing 224 sides, were assessed (age 46.31 ± 13.57 years, 38.39% female, 41.07% asthma, Lund-Mackay CT score 15.88 ± 4.35, 56.25% atopic). The radiological pattern defined by centrally limited changes in all of the paranasal sinuses was associated with allergy status (73.53% vs. 53.16%, P = .03). This predicted atopy with 90.82% specificity, 73.53% positive predictive value, likelihood positive ratios of 2.16, and diagnostic odds ratio of 4.59. CONCLUSIONS: A central radiological pattern of mucosal disease is associated with inhalant allergen sensitization. This group may represent a CCAD subgroup of patients with mainly allergic etiology. LEVEL OF EVIDENCE: 3b Laryngoscope, 128:2015-2021, 2018.

Central compartment atopic disease.

BACKGROUND: Isolated polypoid changes of the middle turbinate were recently reported as having a high association with inhalant allergy. A more advanced manifestation of this association may present as polypoid changes of the entire central sinonasal compartment (i.e., the middle and superior turbinates, and the posterosuperior nasal septum), while the lateral sinus mucosa remains relatively normal. OBJECTIVE: To introduce and describe this newly recognized variant of chronic rhinosinusitis (CRS), termed central compartment atopic disease (CCAD). METHODS: A case series of 15 patients from two institutions who presented with sinonasal symptoms and demonstrated central compartment polypoid mucosal changes on computed tomography (CT). The endoscopic appearance of central compartment edema was assessed. Allergy status was determined by skin or serum in vitro testing. RESULTS: The mean ± standard deviation patient age was 42.4 ± 14.8 years, and 47% of the patients were women. All 15 patients had a diagnosis of allergic rhinitis symptomatically, and those who underwent allergy assessment (14/15) tested positive. All the patients had central compartment polypoid edema on endoscopy and central nasal soft-tissue thickening with peripheral clearing on CT. Even with more severe sinus disease, a central focus of inflammatory change existed. CONCLUSION: CCAD may represent a local inhalant allergy process that affects the central nasal structures of ethmoid origin. Although inhalant allergy changes mainly appear within the nasal cavity, medial-to-lateral progression to involve the sinuses can occur as a simple obstructive phenomenon. This is a pattern of CRS distinct from the more diffuse sinonasal inflammatory disease and likely requires allergy management as a core component.

Middle turbinate edema as a diagnostic marker of inhalant allergy.

BACKGROUND: Middle turbinate edema could be a characteristic feature of aeroallergen sensitization. In this study we sought to determine the diagnostic characteristics of middle turbinate edema as a marker of inhalant allergy. METHODS: A cross-sectional diagnostic study was performed on patients who had undergone nasal endoscopy and allergy testing. Allergy status was determined by positive serology or epicutaneous testing. Endoscopy was reviewed by blinded assessors for middle turbinate head edema. Appearance was graded as either normal, focal, multifocal, diffuse, or polypoid edema. Receiver-operator (ROC) analysis, likelihood ratio (LR), sensitivity, specificity, and positive predictive value (PPV) were determined. RESULTS: One hundred eighty-seven patients representing 304 nasal cavities were assessed (42% female, age 39.74 ± 14.7 years, 57% allergic). Diffuse edema (PPV 91.7%/LR = 8) and polypoid edema (PPV 88.9%/LR = 6.2) demonstrated the strongest association with inhalant allergy. Multifocal edema was used as a cut-off to represent inhalant allergy from ROC analysis, which demonstrated 94.7% specificity and 23.4% sensitivity. The PPV for multifocal was 85.1% and LR = 4.4. CONCLUSION: Middle turbinate edema is a useful nasal endoscopic feature to predict presence of inhalant allergy and, although not sensitive, has excellent PPV.