Sinonasal manifestations of granulomatosis with polyangiitis: A retrospective analysis.

OBJECTIVE: This study aimed to examine the characteristics of nasal and imaging findings of sinonasal lesions in granulomatosis with polyangiitis (GPA) patients and how these lesions change over time in both the active and remission phases of the disease. METHODS: We retrospectively reviewed GPA patients with sinonasal lesions who were followed up at our department between January 2005 and December 2020. The following data were collected: age, sex, symptoms at initial presentation, anti-neutrophil cytoplasmic antibody (ANCA) type, and histopathological, nasal (initial and follow-up), and imaging (initial and follow-up) findings. RESULTS: This study included 17 patients with GPA aged 30 to 79 years. Computed tomography (CT) of the sinuses showed mucosal thickening in 16 patients, bone thickening in 12, bone destruction in 4, and an orbital invasion mass in 3 at the time of diagnosis. After initiating treatment, mucosal thickening of the sinuses improved in 3 of 16 patients and remained unchanged in 13. Bone thickening at the time of diagnosis remained unchanged in 10 of 12 patients and worsened in 2; 1 patient displayed newly developed bone thickening. Destructive nasal findings on CT were positive for proteinase 3-ANCA. CONCLUSIONS: Our study revealed that mucosal thickening, bone thickening, bone destruction, and orbital invasion mass were major CT findings in patients with GPA. Intranasal findings such as granulations, crusting, and necrosis were seen in the active phase; moreover, saddle nose, loss of turbinate, and nasal septal perforation were subsequently seen in the course of the disease. Sinonasal findings of GPA vary depending on the disease stage and period.

Prognostic value of pretreatment inflammatory biomarkers in patients with laryngeal cancer.

OBJECTIVES: The systemic inflammatory response is strongly involved in the progression of malignant tumors, and it is useful for predicting survival time and determining therapeutic effects. The inflammatory biomarkers, neutrophil-to-lymphocyte ratio (NLR), lymphocyte-to-monocyte ratio (LMR), and platelet-to-lymphocyte ratio (PLR) are used to assess post-treatment survival and recurrence in various malignant tumors.(Walsh et al., 2005; Burt et al., 2011; Smith et al., 2009) 1,2,3 These indicators may be effective as predictive markers for head and neck malignancies. METHODS: The participants were 125 glottic laryngeal and supraglottic cancer cases who received primary treatment in our department from 2010 to 2016. The NLR, LMR, and PLR for each patient were calculated in addition to the association with overall survival (OS) rate, disease-specific survival (DSS) rate, and laryngeal preservation rate for tumor location, T and N classification, TNM stage classification, treatment, and smoking. We investigated whether inflammatory biomarkers are useful for predicting prognosis. RESULTS: The cutoff values for NLR, LMR, and PLR on the ROC curve were 1.88, 5.57, and 108, respectively. Multivariate analysis with LMR 5.57 as the cutoff value showed significant differences in OS, DSS, and laryngeal preservation. However, setting the cutoff values for NLR 1.88 and PLR 108 showed significant differences only in OS and laryngeal preservation. CONCLUSION: LMR may be a total survival predictor of laryngeal cancer, including OS, DSS, and laryngeal preservation.

Reconstruction using a free jejunal patch flap in salvage head and neck surgery after radiotherapy.

BACKGROUND: Chemoradiotherapy is a standard treatment for functional preservation in patients with advanced head and neck carcinoma. However, chemoradiotherapy increases the risk of postoperative complications. AIMS/OBJECTIVES: We report the usefulness of reconstruction using a free jejunal patch flap in treating recurrence or residual head and neck carcinoma after radiotherapy. Furthermore, we investigated the factors for the occurrence of postoperative complications in patients who underwent salvage surgery using a free flap transfer. MATERIAL AND METHODS: This study included 41 patients with head and neck carcinoma who underwent salvage surgery using a free flap transfer, including 11 patients who underwent reconstruction using a free jejunal patch flap. Prognostic analysis was performed for the development of complications. RESULTS: Ten jejunal patch flaps survived without microvascular problems. One patient underwent revision reconstructive surgery because of flap failure. However, no patient had a pharyngocutaneous fistula. Oral intake could be resumed in all patients at a median 14 days postoperatively. Multivariate logistic regression analysis indicated that the use of cutaneous flaps was significantly associated with the development of complications. CONCLUSIONS AND SIGNIFICANCE: Free jejunal patch flaps can be considered useful for head and neck reconstruction after radiotherapy for early intake resumption and complication prevention.

A laser-assisted endoscopic approach to pyriform sinus fistula via fibrin glue cauterization.

In the past, the general treatment method for pyriform sinus fistula was its removal by open surgery; however, in recent years, endoscopic surgery has become more common. We report two cases where laser surgery was performed using an endoscope and recurrence was prevented using fibrin glue. Both cases involved 3-year-old girls who underwent laser ablation of a pyriform sinus fistula under an endoscope, after which the site was closed with fibrin glue. No recurrence was observed in either case, and the postoperative course was uneventful. This approach is presented as a non-invasive and effective treatment for pyriform sinus fistula.

Clinical characteristics, the diagnostic criteria and management recommendation of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) proposed by Japan Otological Society.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a form of necrotizing vasculitis with few or no immune deposits. It primarily affects small and medium blood vessels. AAV is classified into three categories, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangitis (EGPA), and two major ANCAs, proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA are involved in their pathogenesis. Intractable otitis media frequently occurs in patients with GPA, MPA or EGPA, although all patients show similar clinical features, regardless of the type of AAV. Furthermore, approximately 15% patients with otitis media caused by AAV do not show ANCA positivity, histopathological evidence, or any other AAV-related lesions at the initial visit; therefore, these patients do not fulfill the ordinary diagnostic criteria for systemic AAV. Thus, we first proposed that this condition could be categorized as "otitis media with AAV (OMAAV)". Subsequently, the Japanese Otological Society (JOS) conducted a nationwide survey between December 2013 and February 2014 and identified 297 patients with OMAAV. The survey revealed that OMAAV is a disease that initially occurs in the middle ear and subsequently spreads to other organs such as the lungs and kidneys, with eventual involvement of all body organs. Severe sequelae such as facial palsy, hypertrophic pachymeningitis, complete deafness, and subarachnoid hemorrhage resulting in death can also occur. In this review, we introduce the clinical features, diagnostic criteria, and treatment strategies recommended by JOS for early diagnosis and treatment of OMAAV.

A novel strategy with combined assays for detection of anti-neutrophil cytoplasmic antibody (ANCA) in clinically ANCA-negative granulomatosis with polyangiitis patients.

OBJECTIVE: Granulomatosis with polyangiitis (GPA) that is localized to the upper airway presents a diagnostic challenge because of a tendency towards anti-neutrophil cytoplasmic antibody (ANCA)-negativity. The purpose of this study was to investigate whether positivity of ANCA detection might be elicited with combined use of enzyme-linked immunosorbent assay (ELISA) kits. METHODS: Twenty-nine serum samples obtained from GPA patients were used in this study. In addition to routine biochemical investigation for ANCA, tests for detecting PR3-, MPO-ANCAs, and minor ANCAs were performed with commercially available ELISA kits. Cytoplasmic (C)-ANCA and perinuclear (P)-ANCA were evaluated using the indirect immunofluorescence (IIF) technique. RESULTS: Twelve patients were positive for PR3- or MPO-ANCA in the clinical laboratory test, and 17 patients were negative for both ANCAs. Of the 17 ANCA-negative patients, four were positive for PR3- or MPO-ANCA, and three were positive for minor ANCA according to results obtained from six different ELISA kits. These findings indicated that performing detection tests with six different ELISA kits might improve the positivity of ANCA and might contribute to establishing the diagnosis of ANCA-associated vasculitis. Together with results from IIF, the samples of eight patients with clinically ANCA-negative results (8/17, 47.1%) were converted to ANCA-positive results, and the ANCA detection rate was significantly improved from 12/29 (41.4%) to 20/29 (69.0%, p=0.03). CONCLUSIONS: Additional detection techniques should be used to confirm the results of clinically ANCA-negative samples, particularly when vasculitis is suspected. Minor ANCAs should also be evaluated with detection tests when PR3- and MPO-ANCA are negative.

Clinical features and treatment outcomes of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV): A retrospective analysis of 235 patients from a nationwide survey in Japan.

OBJECTIVE: We aimed to analyze clinical features and treatment outcomes of otitis media caused by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), i.e. otitis media with AAV (OMAAV). METHODS: This survey was performed between December 2013 and February 2014. The study began with a preliminary survey to 123 otolaryngology institutions in Japan to inquire about their experiences with OMAAV patients during the past 10 years, and was followed by a questionnaire survey to investigate clinical and laboratory findings. OMAAV was defined using the criteria described in the text. RESULTS: Two hundred and thirty-five patients classified as OMAAV were enrolled in this study. They were characterized as follows: (1) disease onset with initial signs/symptoms due to intractable otitis media with effusion or granulation, which did not respond to ordinary treatments such as antibiotics and insertion of tympanic ventilation tubes, followed by progressive hearing loss; (2) predominantly female (73%) and older (median age: 68 years); (3) predominantly myeloperoxidase (MPO)-ANCA-positive (60%), followed by proteinase 3 (PR3)-ANCA-positive (19%) and both ANCAs-negative (16%); (4) frequently observed accompanying facial palsy (36%) and hypertrophic pachymeningitis (28%); and (5) disease often involving lung (35%) and kidney (26%) lesions. Four factors associated with OMAAV were found to be related to an unfavorable clinical course threatening the patient's hearing and/or lives, namely facial palsy, hypertrophic pachymeningitis, both ANCAs-negative phenotype, and disease relapse. The occurrence of hypertrophic pachymeningitis was associated with facial palsy (p < 0.05), both ANCAs-negative phenotype (p < 0.001), and headache (p < 0.001). The administration of corticosteroid together with an immunosuppressant was an independent predicting factor for lack of disease relapse (odds ratio [OR] = 1.90, p = 0.03) and an improvement in hearing loss (OR =2.58, p = 0.0002). CONCLUSION: Since OMAAV has novel clinical features, the disease may be categorized as a subentity for the classification of AAV.

[Serological Study of Otitis Media with ANCA Associated Vasculitis (OMAAV)].

Otitis media related with ANCA associated vasculitis is an intractable condition conditions that is resistant to conventional conservative treatment or surgical treatments. The name "otitis media with ANCA associated vasculitis (OMAAV)" has been proposed as a new concept for this disease. In the present study, we examined the clinical feature of 14 cases of OMAAV. We also investigated the usefulness of combined tests for ANCA detection. There were 11 females and 3 males, with a median age of 68.5 years. PR3-ANCA was positively detected in 4 (28.6%) patients, MPO-ANCA was detected in 6 (42.9%), and 4 (28.6%) were both ANCAs negative. During the course 6 patients developed pachymeningitis and 5 patients developed facial palsy. Plasma samples from 14 OMAAV patients were tested for the presence of ANCAs with five detection methods (3 PR 3-ANCA kits (direct ELISA, capture ELISA, anchor ELISA), 2 MPO-ANCA kits (direct ELISA, capture ELISA)) and the indirect immunofluorescence (II) technique. Six patients (42.9%) were positive for ANCA in clinical examination (single EIA method), while ANCA was positively detected in 9 patients (64.3%) with combined methods. In ANCA negative cases, ANCA should also be tested with a different technique such as new generation ELISA and IIF.

[Closed Septorhinoplasty for Caudal Septum Deviation: A Japanese Rhinologic Perspective].

Caudal septum deviation is one of the most difficult tasks in nasal surgeries even for the experienced surgeons. Correction of the deviated nose presents a challenge because frequently a functional as well as an aesthetic problem must be addressed. In patient with caudal septum deviation, a proper correction of the deviated septal cartilage is a critically important factor for surgical success. For successful correction of caudal septum deviation, the skill to precisely execute the surgical steps is required to alter and control the septum thorough a thorough understandings of nasal anatomy and physiology, accurate preoperative analysis and intraoperative diagnosis, together with an understanding of the physiology of cartilage and its healing. The closed (endonasal) septorhinoplasty approach provides a wide surgical filed that enables the surgeon to implement various surgical procedures. For correction of the caudal septal strut, placement of a batten graft on the concave side after scoring incisions can also be used, resulting in successful correction of caudal deviation. A functional septorhinoplasty with the ultimate purpose of improving nasal obstruction is expected to develop from the Japanese rhinologic perspective. In the current study, closed septorhinoplasty proved to be one of the most effective methods for correcting caudal septum deviation.

Single-stage reconstruction of oral and hypopharyngeal double cancer with a free jejunal flap.

Multiple primary tumors are a known phenomenon in head and neck cancer. However, the incidence of simultaneous oral and hypopharyngeal double cancer is extremely rare. In light of this, the surgical treatment for oral and hypopharyngeal double cancer has not been established. Here we present a case of oral and hypopharyngeal double cancer in which we successfully used a free jejunal flap to reconstruct an oral and hypopharyngeal defect. When the oral tumor is limited to the mucosal surface, a single-stage reconstruction with a free jejunal flap is a suitable option because it is simple and causes less morbidity than using additional flap reconstruction.

[Acute onset Wegener's granulomatosis presenting as otitis media; a case report].

We report the case of a 57-year-old woman with Wegener's granulomatosis who presented with otitis media. The patient presented with a 2-month history of bilateral hearing loss and dizziness. Antibiotic treatment was not effective, and the patient was confirmed to have bilateral sensorineural hearing loss. Serum was positive for cytoplasmic anti-neutrophil cytoplasmic antibody [C (PR3)-ANCA] but negative for P(MPO)-ANCA (perinuclear ANCA). While the test results were pending, the patients' general condition worsened ; in particular, signs of active inflammation, cardiorespiratory failure and scleritis developed suddenly. Emergency chest computed tomography revealed evidence of lung involvement and hydrothorax; thus, the patient was diagnosed to have the generalized form of Wegener's granulomatosis. The patient was started on pulse methylprednisolone therapy at 1000mg/day for 3 days, which resulted in marked clinical improvement, and then, the drug therapy was switched to prednisolone 60mg/day and cyclophosphamide 50mg/day and gradually tapered. Early diagnosis of Wegener's granulomatosis is often difficult because of atypical manifestations of the disease; particular attention must be paid to acute onset of the disease, such as in our case. ANCA is a very useful marker for early diagnosis, but about one week is needed to obtain the test results. We believe that early steroid and cyclophosphamide therapy is an effective therapeutic option for patients with signs of severe inflammation and generalized involvement.