Rhinoplasty for Sinonasal Deformities in Granulomatosis with Polyangiitis: A Systematic Review and Meta-Analysis.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is a vasculitis that affects respiratory and kidney vessels. It primarily involves the nose and sinuses but can progress systemically. Granulomatosis with polyangiitis causes severe nasal deformities, impacting aesthetics and breathing. Literature focuses on rhinoplasty for saddle nose deformities in non-GPA patients, but its suitability for GPA patients remains uncertain. AIM: Our study aims to assess the effectiveness and safety of rhinoplasty in GPA patients by analyzing the existing literature. METHODS: Four databases were searched; 2 reviewers independently screened the retrieved references, then relevant data for our study were extracted. Categorical outcomes were analyzed using pooled proportions and 95% CI. Statistical heterogeneity was assessed using the I2 statistics χ 2 test with a P -value lower than 0.1 indicating heterogeneity. RESULTS: In our systematic review and meta-analysis, we incorporated 16 studies encompassing 136 patients who underwent nasal reconstruction as part of their treatment for GPA. The collective findings indicate a favorable success rate for rhinoplasty, with a pooled ratio of 89% (95% CI: 84%, 95%). The rate of revision surgeries was relatively low, with a pooled rate of 19% (95% CI: 12%, 26%). Furthermore, rhinoplasty demonstrated comparatively lower rates of recurrence (pooled rate: 22%, 95% CI: 3%, 42%) and complications (pooled rate: 12%, 95% CI: 7%, 18%). CONCLUSION: Rhinoplasty benefits GPA patients with saddle nose deformity and septal perforation with high success rates and low recurrence, revision, and complication outcomes. However, more research is needed for validation and refinement.

Facial Rejuvenation in a Patient With Granulomatosis With Polyangiitis-Treatment Protocol for Compromised Skin Flap.

Facial rejuvenation outcomes have not been well studied in patients with granulomatosis with polyangiitis (GP)-formerly Wegener granulomatosis. This report highlights a case of a 49-year-old female with a history of GP, presenting with facial aging and functional nasal concerns. The patient underwent facial rejuvenation and nasal reconstruction procedures, including primary open functional rhinoplasty, septal perforation repair with Alloderm, deep-plane rhytidectomy, platysmaplasty, nanofat grafting, and fractionated erbium laser to the face (sparing the nose and peri-incisional areas). Despite a routine operation, postoperative course was complicated by wound healing and vascular congestion issues related to her underlying autoimmune disease. This case highlights the risks associated with postsurgical healing in patients with GP undergoing esthetic surgery. To the best of our knowledge, this report is the first to discuss management considerations in a patient with GP undergoing facial rejuvenation surgery.

Nasal Reconstruction in Granulomatosis with Polyangiitis: A Two Decade Review.

Background: Granulomatosis with polyangiitis (GPA) leads to progressive destruction of the nasal tissues resulting varying degrees of saddle deformity and nasal obstruction. Reconstructive techniques are numerous, but there are no large series reporting their results. Objective: This study sought to measure complications and outcomes after rhinoplasty for GPA. Methods: We conducted a retrospective review of 42 patients with GPA who underwent nasal reconstruction of saddle nose deformity between 2005 and 2019 using primarily costal cartilage and soft tissue grafts. Results: Thirty-six patients met the criteria for inclusion. All were followed for a minimum of 12 months. Six patients required revision surgery due to infection or GPA flare ups. Five patients had complications. All patients were given a questionnaire at 12 months to rate their degree of satisfaction with their appearance and breathing. Conclusion: The findings of this study suggest that the use of strong cartilage grafts and the timing of surgery result in improvement in breathing and appearance after rhinoplasty in patients with GPA. Clinical Trial Registration number: REB # 21-125.

Remission of Granulomatosis with Polyangiitis Only After Resection of a Pulmonary Nodule.

Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous lesions and is classified as ANCA-associated vasculitis (AAV). We herein report a case of GPA that was remitted by resection of a pulmonary lesion without immunosuppressive therapy. We detected activated neutrophils and neutrophil extracellular traps (NET) formation in resected lung tissue by immunofluorescence. Activated neutrophils and NETs might be involved in the pathophysiology of AAV and induce the vicious cycle of ANCAs and NETs. In cases of GPA with no other severe lesions, the reevaluation of the disease activity after diagnostic resection is crucial for considering the need for immunosuppressive therapy.

[Surgical Treatment for Pneumothorax with Granulomatosis with Polyangiitis].

A 42-year-old man had been treated for granulomatosis with polyangiitis for eight years. He was referred to our department with the diagnosis of right pneumothorax on chest radiograph. After chest drainage, the surgical treatment was performed because of continuing air leak from chest tube. Under thoracoscopic approach, the pleural adhesions were carefully dissected and the air leak site was sutured and enforced by a polyglycolic acid sheet with fibrin glue. No recurrence of pneumothorax was observed six months after surgery.

Factors Affecting Dilation Interval in Patients With Granulomatosis With Polyangiitis-Associated Subglottic and Glottic Stenosis.

OBJECTIVE: Subglottic stenosis (SGS) is a known complication of granulomatosis with polyangiitis (GPA). We investigated the impact of medical and surgical interventions on the surgical dilation interval and characterized patients with glottic involvement. STUDY DESIGN: A retrospective chart review of patients with GPA-associated SGS was performed from 2010 to 2019. SETTING: Tertiary academic medical center. METHODS: The impact of medical and surgical interventions on dilation interval was assessed. The prevalence of glottic involvement was assessed, and clinical characteristics and outcomes were compared with patients without glottic involvement. RESULTS: A total of 39 patients with GPA-associated SGS were analyzed. Dilation intervals in patients receiving leflunomide (n = 4; median, 484 days; 95% CI, 405-1099) were greater than in those not receiving leflunomide (median, 155 days; 95% CI, 48-305; P = .033). The surgical technique used did not affect dilation interval. Patients with glottic involvement (n = 13) had a greater incidence of dysphonia (13/13 vs 15/26 [58%], P = .007) and a shorter dilation interval with involvement (median, 91 days; interquartile range, 70-277) versus without involvement (median, 377 days; interquartile range, 175-1148; hazard ratio, 3.38; 95% CI, 2.26-5.05; P < .001). Of 13 patients, 8 (62%) did not have glottic involvement on first presentation. CONCLUSION: Although GPA is classically thought to affect the subglottis, it also involves the glottis in a subset of patients. These patients have greater complaints of dysphonia and require more frequent surgery. Systemic therapy may increase dilation intervals. In this preliminary study, patients taking leflunomide demonstrated an improvement, highlighting the need for further study of immunosuppression regimens in the treatment of GPA-associated SGS.

Impact of Paranasal Sinus Surgery in Granulomatosis With Polyangiitis: A Longitudinal Computed Tomography Study.

OBJECTIVES/HYPOTHESIS: Severe chronic rhinosinusitis (CRS) in patients with granulomatosis with polyangiitis (GPA) failing medical therapies can be treated with paranasal sinus surgery. Whether this surgery protects from progressive sinonasal damage remains unknown. Here, we aimed to analyze time-dependent relations between sinus surgeries and computed tomography (CT) imaging features in the CRS of GPA. STUDY DESIGN: Longitudinal observational study. METHODS: We assessed CRS features including bone thickening by global osteitis scoring scale, bone erosions, and mucosal thickening by Lund-Mackay scores in serial paranasal sinus CT scans (742 CT scans in total) from a cohort of 127 well-characterized GPA patients. Data on sinonasal surgical procedures were from a mandatory national registry and from chart review. We defined the time from baseline CT to last CT as the study observation period in each patient. Datasets were analyzed by linear mixed models. RESULTS: We found that 23/127 cohort patients had one or more paranasal sinus surgical procedures, and 96% of these (22/23) had osteitis by CT after surgery. In patients with nasal surgery alone or no surgery, we identified osteitis in 7/11 (64%) and 45/93 (48%), respectively. During the observation period of a median of 5 years, 38 patients had progression of their sinus osteitis, with the highest annual osteitis progression rates observed around the time of surgery. CONCLUSIONS: In this cohort, paranasal sinus surgery was associated with prevalence, severity, and progression rate of sinus osteitis, indicating that sinus surgery does not reduce the bone damage development in the CRS of GPA. LEVEL OF EVIDENCE: 4 Laryngoscope, 130: E460-E468, 2020.

Chronic rhinosinusitis in eosinophilic granulomatosis with polyangiitis: clinical presentation and antineutrophil cytoplasmic antibodies.

BACKGROUND: In this study we aim to describe presenting characteristics and identify prognostic factors for disease resolution in patients with chronic rhinosinusitis (CRS) in the setting of eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: Patients evaluated at a tertiary care center with diagnoses of EGPA and CRS were identified. Descriptive statistics were obtained. Univariate analysis was used to search for prognostic factors associated with higher Lund-Mackay score at presentation and disease resolution. RESULTS: Forty-four patients were included with a mean age of 52.7 (standard deviation, 14) years. Twenty-one patients (47.7%) were female, all had a diagnosis of asthma, and 36 (83.7%) had eosinophils >10%. Common presenting symptoms for CRS included nasal discharge (87.9%) followed by nasal congestion (83.9%) and facial pain and pressure (83.8%). Medical management of CRS included systemic corticosteroids (93.2%) and systemic antibiotics (75%). Surgical intervention occurred in 29 patients (67%). Nine patients (20.5%) had resolution of sinus symptoms, including 4 with imaging confirmation. Fourteen patients (31.8%) had continued CRS, but with improved symptoms, whereas 9 patients (20.5%) had continued CRS with no improvement in symptoms. Eleven patients (25%) were lost to follow-up and 4 (9.1%) died. Univariate analysis did not show antineutrophil cytoplasmic antibody positivity, presence of peripheral eosinophilia, gender, age, or absence of systemic therapy to be predictive of higher Lund-Mackay score at presentation or predictive of disease resolution. CONCLUSION: CRS in patients with EGPA is often refractory to medical and surgical management. Treatment of these patients should occur in a multidisciplinary setting.

Face Transplantation for Granulomatosis With Polyangiitis (Wegener Granulomatosis): Technical Considerations, Immunological Aspects, and 3-Year Posttransplant Outcome.

BACKGROUND: We report new data for a rare face transplant performed 3 years ago. Granulomatosis with polyangiitis (GPA) (Wegener) is a severe autoimmune necrotizing vasculitis and parenchymal inflammatory disease that can affect any organ including those of the craniofacial region. Skin involvement manifests as malignant pyoderma. This account (1) highlights the technical details of face transplantation for this unique indication, (2) reports the 3-year posttransplant outcome, and (3) describes relevant immunological aspects. METHODS: A Le Fort III near-total face and near-total scalp transplant was performed after extensive trauma and subsequent bone and soft tissue infection in a patient with GPA. Incisions were planned along facial aesthetic subunits. The vascular pedicle comprised the facial and superficial temporal arteries bilaterally. The functioning left eye was preserved and fitted into the donor tissues. RESULTS: The procedure took 21 hours, and transfusion was limited to 4 units of packed red cells. Early medical and surgical complications were successfully treated. At 3 years, acceptable aesthetic outcome was achieved with adequate color match and scalp hair growth. The patient has recovered light touch, temperature, and 2-point discrimination and has evidence of symmetric cheek elevation albeit with limited eyelid and frontalis function. GPA relapse did not occur. Four acute rejections were fully reversed. CONCLUSIONS: This case represents a new underlying disease (trauma + GPA) leading to face transplantation and a unique clinical scenario where allografting was indicated for potentially life-threatening and sight-preserving reasons and not for mere functional and aesthetic concerns. Despite complexity, 3-year clinical outcome is encouraging, and the patient is no longer at risk for dural exposure, meningitis, and related morbidity.

[Modern management of otologic consequences of Wegener's granulomatosis - a case report and review of the literature]. / Wegener-granulomatosis talaján kialakult krónikus gennyes középfülgyulladás komplex kezelése.

Wegener's granulomatosis - or, in other words, granulomatosis with polyangiitis - is an anti-neutrophil cytoplasmic antibody associated granuloma forming vasculitis, mainly affecting the respiratory tract and the renal system. Otologic manifestations of Wegener's disease can be otitis media with effusion or chronic silent mastoiditis with conductive hearing loss, but sensorineural hearing loss can also evolve. The diagnosis is based on the clinical appearance as well as the immunoserological and histopathological results. It is of paramount importance to begin a combined immunosuppressive treatment immediately, besides eradicating the otologic manifestations. The intractable cases of chronic otitis media due to Wegener's granulomatosis are challenging any ear surgeons. Subtotal petrosectomy has proved to be an effective solution in such cases to create a dry ear and to provide a safe surgical field for hearing restoration. The authors reviewed the literature and report a case history to present the modern management of Wegener's granulomatosis with otologic manifestation. Orv Hetil. 2019; 160(4): 151-157.

Head and neck manifestations of granulomatosis with polyangiitis: a retrospective analysis of 19 patients and review of the literature

Introduction: Granulomatosis with Polyangiitis (GPA) is a small vessel vasculitis characterized by a necrositing granulomatous inflammation of the upper and lower respiratory tracts and focal/proliferative glomerulonephritis. In more than 70% of the cases, the presenting symptoms are head and neck manifestations that are often misdiagnosed as infectious or allergic in etiology. Objective: The present study provides an analysis of head and neckmanifestations in a series of patients diagnosed with GPA. It also evaluates their medical and surgical treatment and provides a review of the relevant literature. Methods: A retrospective analysis of 19 patients diagnosed with GPA at a public tertiary care hospital between 2006 and 2017 was performed. Results: A total of 19 patients were included in the present study, and 16 of them presented head and neck manifestations. Sinonasal symptoms were the most common, affecting 56% of the patients, followed by laryngotracheal (31.25%) and ear (25%) symptoms. In 7 patients, sinonasal symptoms were the first manifestation of the disease (43.75%). Four patients underwent surgery at some stage of the disease. Conclusions: Head and neck involvement is common in GPA and may stand for the first or the onlymanifestation of the disease. The otolaryngologists play a central role in the diagnosis and long-term treatment of these patients, and they have to keep this pathology in mind when treating patients with ENT symptoms that do not respond as expected to the treatment (AU)

Granulomatosis eosinofílica con poliangitis: informe de un caso / Eosinophilic granulomatosis with polyangiitis: A case report

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Reconstruction of Wegener Granulomatosis Nose Deformity Using Fascia Lata Graft.

Wegener granulomatosis (WG) is a necrotizing vasculitis that involves the upper respiratory tract, the lungs, and the kidneys. It also causes deformities of the nose in practically all patients, altering the cartilaginous and bony structure as well as the inner lining of the nose.The surgical repair of these nasal distortions is a challenge mainly due to the impairment of wound healing and graft take that is caused both by the disease itself and by the prolonged immunosuppressant treatment.Many different reconstruction plans have been developed, using costal or calvarial bone grafts, cartilage grafts and local skin and mucosa flaps. Results have been diverse.The authors present the case of a 56-year-old patient who suffered from WG and consulted for nasal sequelae. She initially underwent several failed attempts at reconstruction; finally, the authors performed a nasal repair using a double strip of fascia lata as a dorsal graft, with no complications and a good aesthetic result.Fascia lata graft is a new and good option for nasal reconstruction in patients with WG because it is easily obtainable, it can be set in place through closed rhinoplasty and it can achieve good vascularization with low risk of resorption or disease reactivation.

Hypertrophic Pachymeningitis and Hydrocephalus-The Role of Neuroendoscopy: Case Report and Review of the Literature.

BACKGROUND: Hypertrophic pachymeningitis (HP) is a rare primary or secondary inflammatory disorder that manifests with thickening of dura mater involving predominantly the tentorium and falx in the focal form. Hydrocephalus has not been reported in association with secondary HP. CASE DESCRIPTION: A 61-year-old woman presented with intracranial HP of the posterior fossa secondary to Wegener granulomatosis not responsive to corticosteroid therapy. Owing to the association of noncommunicating hydrocephalus, endoscopic third ventriculostomy was performed. There was immediate improvement of neurologic symptoms and no postoperative complications. The patient had very good clinical and radiologic outcome at 1-year follow-up. CONCLUSIONS: In a case of noncommunicating hydrocephalus related to focal HP of the posterior fossa, endoscopic third ventriculostomy was effective in resolving symptoms related to increased intracranial pressure. The physiopathogenetic mechanisms and therapeutic strategies were discussed along with a review of the most relevant literature.

Holmium Laser for Endoscopic Treatment of Benign Tracheal Stenosis

Abstract Introduction Laryngotracheal stenosis is a difficult problem with varied etiology and various treatment options. The holmium laser represents another tool for the treatment of benign tracheal stenosis. Objectives To determine the utility of holmium laser treatment for benign tracheal stenosis with regards to safety and efficacy. Methods This was a retrospective case study examining patients with benign tracheal stenosis from 1998-2016 who underwent holmium laser treatment. Determining the safety of this procedure was the primary goal, and complications were monitored as a surrogate of safety. Results A total of 123 patients who underwent holmium laser treatment for benign tracheal stenosis were identified. In total, 123 patients underwent 476 procedures, with follow-up ranging from 1 month to 14 years. No intraoperative or post-operative complications were identified as a direct result of the use of this particular laser. Conclusions The holmium laser is an effective and safe laser to use for tracheal stenosis treatment. It is a contact laser with a short acting distance, which reduces the risk of injury to distal airway structures. Given the favorable experience reported here, the holmium laser should be considered when tracheal surgery is attempted.

Rinosinusitis recalcitrante por granulomatosis eosinofílica con poliangeítis: reporte de un caso clínico y revisión de la literatura / Chronic recalcitrant rhinosinusitis secondary to eosinophilic granulomatosis with polyangiitis: report of a case and literature review

RESUMEN Se expone el caso de un paciente de 40 años con diagnóstico de granulomatosis eosinofílica con poliangeítis subyacente a una rinosinusitis crónica recalcitrante. Se describe el caso y se discuten aspectos relevantes de la literatura al respecto.

ABSTRACT We report the case of a 40-year-old man with diagnosis of chronic recalcitrant rhino-sinusitis secondary to eosinophilic granulomatosis with polyangiitis. We described the case and discuss relevant aspects of the literature about it.

Granulomatosis With Polyangiitis Involving the Ascending Aorta.

A 58-year-old man visited the emergency department with vague anterior chest discomfort. From the computed tomographic findings, our initial suspicion was intramural hematoma in the ascending aorta. After a comprehensive discussion with relevant departments, emergent operation was planned. Intrapericardial space, however, was severely adhered, suggesting chronic inflammation. Biopsies were performed, and additional surgical procedures were discontinued. The patient was finally diagnosed as having granulomatosis with polyangiitis. Careful approach with high-degreed suspicion for granulomatosis with polyangiitis, although rare, is required when preoperative findings are similar to the present case.