Recurrent Sinusitis Research Articles

Management of Nasal Polyposis in Pediatric Patients With Cornelia de Lange Syndrome: A Case Series and Literature Review.

Cornelia de Lange syndrome (CdLS) is a rare genetic multiple malformation disorder with many otolaryngologic comorbidities. Patients with CdLS appear to have an increased prevalence of chronic rhinosinusitis (CRS) and chronic rhinosinusitis with nasal polyposis (CRSwNP), however, there is limited literature describing the presentation, evaluation, and management of CRSwNP within the CdLS population. Here we performed a literature review from Embase, PubMed, Cochrane Library, and Google Scholar and reported a case of CRSwNP with concomitant CdLS that was identified at our institution. We describe an 8-year-old male with CdLS and CRSwNP confirmed by history, physical exam, nasal endoscopy, and computed tomography. Symptoms of nasal obstruction were refractory to medical management and required repeat surgical management with improvement in nasal obstruction. Literature review identified 2 additional cases of nasal polyposis with similar management strategies. Additionally, one case series showed 33% of CdLS patients had recurrent sinusitis while a second series identified 39% of CdLS patients with CRS and 12% with CRSwNP. These data suggest that CRSwNP is more prevalent in patients with CdLS compared to the general public and can be both safely and effectively managed with a combination of medical and surgical therapy.

Dupilumab Improves Facial Pain and Reduces Rescue Treatments in Patients with CRSwNP and Recalcitrant Frontal Sinusitis.

Recalcitrant frontal sinusitis in patients with chronic rhinosinusitis and nasal polyps (CRSwNP) has a negative impact on their quality of life due to frontal pain and a high risk of sinus occlusion, thus necessitating antibiotics, systemic corticosteroids, and multiple surgeries. The aim of this study was to assess the efficacy of dupilumab in reducing frontal pain and the need for rescue treatments for recalcitrant frontal sinusitis in patients with CRSwNP. We enrolled a cohort of 10 patients with severe uncontrolled CRSwNP and concomitant recurrent frontal sinusitis associated with severe facial pain measured by MIDAS score who were treated with dupilumab 300 mg every 2 weeks and followed for at least 12 months. The mean MIDAS score decreased from 45.6 ± 10.7 at baseline to 1.3 ± 2.3 at 6 months (p < 0.05). VAS craniofacial pain decreased from 7.3 ± 1.6 at baseline to 1.2 ± 1.5 at 6 months (p < 0.05). No patient needed oral corticosteroids during treatment with dupilumab (p < 0.05), and the use of analgesics decreased from 9.6 ± 3.1 NSAID pills/week in the last 2 months at baseline to 0.6 ± 1.3 at 1 year of follow-up (p < 0.05). Our results demonstrated that use of subcutaneous dupilumab can improve symptom control, including recurrent severe cranio-facial pain, and reduce the need for rescue medical treatments (systemic steroids and NSAID) in patients with severe uncontrolled CRSwNP and concomitant recurrent frontal sinusitis.

ENT Manifestations of Sjögren’s Syndrome: A Comprehensive Narrative Review

Background: Sjögren's Syndrome (SjS) is a chronic autoimmune disorder predominantly affecting exocrine glands, leading to symptoms such as keratoconjunctivitis sicca and xerostomia. This review focuses on the ear, nose, and throat (ENT) manifestations of SjS, which significantly impact patient quality of life and pose diagnostic challenges. Aim: The review aims to consolidate current knowledge on the ENT manifestations of SjS, exploring pathophysiological underpinnings, clinical presentations, and treatment strategies, while addressing the diagnostic challenges associated with the disease. Review Summary: ENT manifestations in SjS include nasal dryness, recurrent sinusitis, otitis, and laryngeal dryness, which may precede other systemic manifestations, aiding in early diagnosis and management. This review highlights the importance of recognizing these symptoms for timely intervention, which can significantly improve disease prognosis. Future Implications: Understanding ENT manifestations can enhance multidisciplinary management approaches and foster development in diagnostic and therapeutic strategies, potentially improving patient outcomes and quality of life. Clinical Policy and Development: Enhanced awareness and training on the ENT aspects of SjS are recommended for healthcare professionals. Development of more sensitive diagnostic tools and personalized treatment plans could also address the variability in symptom presentation and response to treatment. Keywords: Sjögren's Syndrome, ENT Manifestations, Xerostomia, Keratoconjunctivitis Sicca, Multidisciplinary Management

Neurosarcoidosis-Induced Winging Scapula: Efficacy of Infliximab Treatment in Addressing Multifaceted Challenges.

Sarcoidosis, a systemic granulomatous disease primarily affecting the respiratory and lymphatic systems, can rarely manifest as neurosarcoidosis either in isolation or alongside other systemic symptoms. Here, we describe the case of a 45-year-old male with a history of recurrent sinusitis refractory to antibiotics, who presented to the emergency department with sinus congestion and dysphagia. Clinical examination revealed left lower motor neuron facial palsy and enlarged submandibular salivary glands. Despite obtaining negative results from various antibody panels, the patient exhibited elevated Angiotensin Converting Enzyme levels of 83 nmol/kg/min. Additionally, computed tomography chest scans revealed bilateral hilar and mediastinal lymph node enlargement, findings consistent with sarcoidosis. Otorhinolaryngology evaluation for dysphagia confirmed left vocal cord palsy. Following a negative infectious disease workup, submandibular salivary gland biopsy confirmed sarcoidosis. Treatment with mycophenolate mofetil and oral steroids led to gradual improvement in salivary gland swelling, dysphagia, and facial palsy. However, worsening left shoulder pain prompted further investigation, revealing winging of the left scapula on repeat examination. Magnetic resonance imaging (MRI) of the cervical spine revealed a six mm hyperintensity in the left dorsal cord at the C5 level, suggesting possible neurosarcoidosis vs. demyelinating disease. Subsequently, the patient was prescribed anti-tumor necrosis factor alpha inhibitor infliximab. Subsequent MRI of the cervical spine, conducted six months after initiating Infliximab therapy, indicated resolution of the lesions. This positive outcome was supported by the patient's report of symptom improvement, notably reduced shoulder pain and improvement in left scapular winging. This case underscores the unusual co-occurrence of Bell's palsy and vocal cord palsy in the same patient, along with the potential contribution of neurosarcoidosis to the winged scapula. Additionally, it sheds light on the positive response of neurosarcoidosis to Infliximab therapy.

Ameloblastic Carcinoma of the Maxilla With Uncommon Sinus Manifestations.

Ameloblastic carcinoma (AC) represents a distinct challenge in the realm of odontogenic malignancies due to its rarity and aggressive nature. We present a unique case of AC in a 70-year-old male, retired dry cleaner, with symptoms initially suggestive of chronic allergic rhinitis and recurrent acute sinusitis with asymmetric facial edema and paresthesia. Detailed evaluation revealed a prominent mass in the right maxillary sinus with extensive cortical destruction. Pathological assessment post-right maxillectomy identified a high-grade AC with malignant spindle cell transformation. The patient underwent subsequent interventions, including neck dissection and radiation therapy. Twelve months post-presentation, the patient was recovering appropriately without evidence of recurrence of malignancy. This case highlights the diagnostic challenges posed by AC as well as its unique presentations emphasizing the importance of a comprehensive approach and multidisciplinary management. It also raises considerations about potential chemical exposure implications in AC development.

Functional and Anatomical Features of the Nose Cavity in Children with Chronic, Acute Recurrent Sinusitis and Adenoid Hypertrophy of 1–3 Degrees

Functional and Anatomical Features of the Nose Cavity in Children with Chronic, Acute Recurrent Sinusitis and Adenoid Hypertrophy of 1–3 Degrees

ENT manifestations of Sjogren’s syndrome: A narrative review

Sjogren's syndrome (SS) is an autoimmune condition marked by dryness of the mouth and eyes, frequently manifesting as a range of ENT symptoms that have a major negative impact on the quality of life of patients. Dryness and discomfort in the mouth and eyes, brought on by lacrimal and salivary gland dysfunction are ENT symptoms in SS. However, ENT regions are involved in more areas than just the ocular surface and mouth cavity. Due to laryngopharyngeal involvement, patients may experience hoarseness, dysphagia, and the sensation that something foreign is in their throat. Common nasal symptoms include nasal dryness, congestion, and recurrent sinusitis, while Eustachian tube dysfunction can lead to middle ear infections and hearing problems. To improve the quality of life in SS patients, clinicians and healthcare professionals should be careful in identifying and treating these symptoms. More investigations and clinical trials are required to create tailored medicines that can reduce ENT symptoms and enhance long-term outcomes for SS patients.

A case report on spontaneous intracranial hypotension

A 38-year-old lady with bronchial asthma, psychiatric illness, and a suicidal attempt 6 years ago presented with persistent headache for more than 2 weeks. She was diagnosed with recurrent sinusitis and migraine and was on treatment at a local hospital and referred to a higher center for persistent headache which was orthostatic in nature. Clinically, the patient was alert, oriented, and systemic examination was unremarkable. MRI spine showed extensive extradural collection separating the anterior dural sac from the bony canal extending from C3 to D1. CT Myelogram showed cervical dural leak anteriorly at C7-D1 and bony osteophyte impinging on the dural sac – likely cause of leak. Diagnosis of Spontaneous Intracranial Hypotension was made. As no improvement was noted with trial of conservative management, we proceeded with advanced imaging-guided Epidural blood patch repair, following which the symptoms improved.

Granulomatosis with polyangiitis: The wily masquerader of cavitary pulmonary lesions

Background: Granulomatosis with Polyangiitis (GPA) is a small and medium vessel vasculitis with predominant respiratory and renal involvement that causes florid presentation of the disease entity with varying spectrum of illness at different time intervals. Case: We report the case of a 42-years-old female patient with symptoms of low-grade fever, productive cough and significant loss of weight with multiple cavitary lesions on Chest radiograph. The patient was initially suspected to have tuberculosis which was eventually ruled out. A diagnosis of vasculitis was suspected as patient gave history of repeated hospitalisations with symptoms of upper respiratory tract involvement, left facial nerve palsy and sensorineural hearing loss, A diagnosis of limited GPA was made when the cytoplasmic Antineutrophil Cytoplasmic Antibodies, (c-ANCA) and antiproteinase-3 antibodies (anti-PR3) was elevated. The patient was treated with combination immunosuppressive therapy of prednisolone and methotrexate which induced clinical remission within one month and complete radiological remission within 4 months. Conclusion: GPA is an important non infective cause of cavitary pulmonary lesions that presents with extrapulmonary clinical spectrum (recurrent sinusitis, ear infections, renal dysfunction and neurological disease). Multifocal presentation of the disease at different time intervals results in considerable delay in diagnosis and adds to the morbidity of the disease entity.

A Comparative Clinical Study on the Usefulness of Homoeopathic Medicines from Mineral Kingdom and Other Homoeopathic Medicines in the Management of Chronic Recurrent Sinusitis

It is a comparative clinical study to assess the usefulness of Homoeopathic mineral remedies and other Homoeopathic remedies in management of Chronic Recurrent Sinusitis.

Allergic Rhinitis as an Independent Risk Factor for Postoperative Recurrence of Children Chronic Sinusitis.

(1) Background: The recurrence rate of childhood recurrent sinusitis varies widely between 12% and 50%, with the postoperative recurrence risk factors remaining largely unidentified. We sought to enhance the understanding of chronic rhinosinusitis (CRS) via a retrospective observational childhood cohort. (2) Methods: The study recruited 125 cases. Demographic data and univariate and multivariate logistic regression analyses were conducted to investigate potential risk factors of childhood recurrent sinusitis following functional endoscopic sinus surgery (FESS). (3) Results: A postoperative recurrence rate of 21.6% was determined. Among the participants, 21 cases presented a history of allergic rhinitis (AR), with the remaining 104 cases being AR-free. A significantly heightened recurrence rate was noted in those bearing a history of AR compared to their counterparts devoid of such history (p < 0.000). The fully adjusted logistic regression model indicated a 21.04-fold increased risk of postoperative recurrence in childhood CRS bearing a history of AR compared to those without an AR history (p = 0.000), highlighting the history of AR as an independent risk factor for postoperative childhood recurrent sinusitis (p = 0.001); (4) Conclusions: The data implicate AR as an independent risk factor for postoperative childhood recurrent sinusitis.

Pott's Puffy: First Shot is the Best Shot.

Low incidence of Pott's Puffy tumor (PPT) has caused studying risk factors and recurrences of the disease to be difficult. We used the comparatively increased incidence at our institution to evaluate potential risk factors for the disease process itself and prognostic factors for recurrence of the disease. Single institutional retrospective chart review identified 31 patients from 2010 to 2022 with PPT compared with a control group of 20 patients with either chronic rhinosinusitis or recurrent sinusitis. Patient mean age of PPT was 42 (range of 5 to 90) with the majority of the patient population as male (74%) and Caucasian (68%) in the setting of rural West Texas. Patient mean age of the control group was 50.7 (range of 30-78) with majority of patient population as male (55%) and Caucasian (70%). Interventions studied were functional endoscopic sinus surgery (FESS), FESS with trephination, and cranialization with or without FESS to compare prognostic factors for recurrence rates of PPT. These patients' prognostic risk factors for recurrence and risk factors to develop PPT were analyzed using Analysis of Variance (ANOVA) χ 2 statistical analysis with Fischer exact testing. Mean age was 42 years (range of 5-90) with the majority of the PPT patient population as male (74%) and Caucasian (68%) with an overall incidence of about 1 in 300,000. Pott's Puffy tumor patients were significantly favored in the younger and male population compared with the control patients. Risk factors of no prior allergy diagnosis, previous trauma, medication allergy to penicillin class or cephalosporin class, and lower body mass index were significant in the PPT population compared with the control group. Significant prognostic factors for recurrence of PPT were prior history of sinus surgery and operative treatment choice. Fifty percent (3/6) of patients with prior sinus surgery had recurrence of PPT. Of our 4 treatment options (FESS, FESS with trephination, FESS with cranialization, or cranialization alone), ;FESS had a recurrence of PPT of 0% (0/13), FESS with trephination had a recurrence of PPT of 50% (3/6), FESS with cranialization had a recurrence of PPT of 11% (1/9), and cranizalization alone had a recurrence of PPT of 0% (0/3). Of note, postop chronic rhinosinusitis was seen in 46% (6/13) of FESS alone, 17% (1/6) with FESS with trephination, 0% (0/9) with FESS with cranialization, and 33% (1/3) with just cranialization alone. Pott's Puffy tumor patients were younger and predominately male when compared to the control patients. No prior allergy diagnosis, previous trauma history, medication allergy to penicillin class or cephalosporin class, and lower body mass index are risk factors for PPT. There are 2 prognostic factors that predict recurrence of PPT: first operative treatment choice and prior sinus surgery. History of prior sinus surgery tends to increase the recurrence of PPT. The first operative treatment plan is the best shot at definitively treating PPT. Correct management surgically can prevent recurrence of PPT as well as long-term recurrence of chronic rhinosinusitis. With early diagnosis and mild disease, FESS is sufficient to prevent recurrence of PPT but chronic sinusitis may continue to occur if frontal sinus outflow track is not well opened. If considering trephination, a definitive cranialization may be more suited for more advanced disease since our study showed 50% of recurrence of PPT with trephination and FESS along with 17% chronic sinusitis long term. More advanced diseases with higher WBCs and intracranial extension do better with more aggressive surgical management with a cranialization with or without FESS which shows to reduce rates of PPT recurrence significantly.

A COMPARISON OF TOPICAL STEROIDS AND ANTIBIOTICS TO IMPROVE DRUG THERAPY FOR RECURRENT SINUSITIS

Objective: this study is to assess the efficacy of antibiotics against topical steroids in the treatment of recurrentsinusitis, with an emphasis on symptom relief, recurrence rates, and patient satisfaction.Study Design: a prospective study.Duration and Place of Study: This study was conducting at Department of ENT at Hayatabad MedicalComplex between Jan 2020 to Jan 2021.Materials and Methods: This study included 175 patients between the ages of 18 and 65 who had recurrentsinusitis. Patients were randomly assigned to two groups: Group A got antibiotics, whereas Group B received topicalsteroid treatment. Validated rating methods were used to measure symptom intensity, recurrence rates, and patientsatisfaction at baseline, 4 weeks, and 12 weeks following therapy beginning. The following information was gathered:medical history, clinical examination, endoscopic results, and radiographic examinations. To compare the results ofthe two treatment groups, the obtained data were analyzed using relevant statistical techniques such as t-tests andchi-squared tests.Results: Of 175 patients, 88 got antibiotics and 87 topical steroids. The average age of antibiotic patients was 44.2± 8.1 years, whereas topical steroid patients averaged 43.9 ± 7.8 years. Both groups had identical baseline scores:antibiotics scored 7.2±1.5 and topical steroids scored 7.4±1.4. Both treatment groups’ 12-week patient satisfactionand recurrence rates were examined. 65.9% of antibiotic patients were pleased, 26.1% moderately satisfied, and7.95% unhappy. In contrast, 85.05% of topical steroids patients were satisfied, 13.8% moderate, and 1.14% poor.These data demonstrate topical steroids may reduce chronic sinusitis patients’ symptoms, satisfaction, and recurrence.Conclusion: This comparative research shows that topical steroids relieve symptoms quicker and longer, have a reducedrecurrence rate, and increase patient satisfaction. These findings suggest that topical steroids may help treat recurrentsinusitis and suggest that further study and clinical recommendations are needed to improve therapy.Keywords: recurrent sinusitis, antibiotics, topical steroids, drug therapy, comparative study, symptom improvement,recurrence rates, patient satisfaction.

Combined Radiological and Endoscopic Evaluation of Sino Nasal Anatomical Variations in Patients of Chronic Rhinosinusitis: A North Indian Study.

To determine the prevalence of anatomical variations of nasal cavity and paranasal sinuses of patients with chronic rhinosinusitis (CRS) on CT scan imaging. To correlate the Anatomical variations with the extent of CRS. 100 patients attending the ENT outpatient department with clinically diagnosed CRS were selected for study based on inclusion and exclusion criteria. Patients were subjected to CT scan and diagnostic nasal endoscopy. The correlation of anatomical variation with severity of CRS based on radiological score and endoscopic score was observed. Deviated nasal septum was the most common anatomical variation observed in 71% cases. Followed by Agger nasi (68%), concha bullosa (55%), Onodi cell (25%), Haller cell (14%), frontal sinus hypoplasia (2%) and uncinate bulla (1%) respectively. Statistically significant relationship of radiological score with left side Bullous Concha Bullosa and highly statistically significant relationship with Haller Cell was observed. While statistically significant relationship between Deviated Nasal Septum on left side with endoscopic score was also observed. Correlation of anatomical variation with CRS concludes on the notethat some variations cause impaired sinus drainage and ventilation leading to recurrent sinusitis. Also, incidence of these variations was comparable to other studies done in asymptomatic population therefore, simply detection of a solitary anatomical variant itself does not determine predisposition to disease or the pathogenesis of the CRS and that we should have a critical look out for these anatomical variations from point of view of surgical management.

ACase of GATA2 Deficiency with Pancytopenia and Recurrent Infections: To Transplant or Not to Transplant?

BackgroundGATA2 deficiency is an autosomal dominant disorder arising from various mutations in the GATA2 gene, which encodes a zinc finger transcription factor that is required for hematopoiesis and angiogenesis. Patients with GATA2 deficiency can present with a range of hematologic and immunologic manifestations. We present a case of GATA2 deficiency with recurrent transient pancytopenia who is also heterozygous for C9 deficiency. Case presentationOur 30-year-old female patient presented with febrile neutropenia (ANC 850) and rash. Interestingly, she reported a history of recurrent viral upper respiratory infections since childhood and had mononucleosis at the age of 5. In her mid-twenties, she started having recurrent sinusitis and pneumonias, requiring hospitalization and antibiotic therapy 1–2 times per year. She also had persistent pancytopenia for at least 5 years that transiently improved with transfusions. CBC ranged from Hgb 5.5–10.1 g/dL (mean 8), platelets 30–102 K/uL (mean 67), WBC 1.4–5.6 K/uL (mean 2.87), neutrophils 0.75–4.35 K/uL (mean 2), monocytes 0–0.07 K/uL (mean 0.02), lymphocytes 0.38–1.18 K/uL (mean 0.8). Bone marrow biopsy showed severely hypocellular bone marrow with trilineage hypoplasia without dysplasia or neoplasia. We initiated an immune workup that showed normal IgG, IgA and IgM levels; low B cell numbers; absent NK cells (CD56+); and preserved T cell numbers on flow cytometry. Strep pneumoniae IgG levels were nonprotective with less than 0.1 ug/mL for all serotypes. Genetic panel testing for primary immunodeficiency revealed a heterozygous pathogenic mutation in GATA2 (c.1081C>T (p.Arg361Cys). Upon multidisciplinary discussion with Hematology, decision was made to begin immunoglobulin replacement. Bone marrow transplant workup was initiated, but the team is hesitant to proceed with transplant at this time. DiscussionHematopoietic stem cell transplantation is the only treatment option with curative potential for this patient, but there are significant associated morbidity and mortality risks. Identifying the appropriate timing for transplantation remains a central focus of this patient's care. Close monitoring of the patient's overall clinical evolution including peripheral cell counts, bone marrow findings, and infection frequency and severity could help approximate the optimal transplantation timing after careful consideration of risks and benefits.

P031 Montelukast antagonising underlying systemic illness

Abstract Background/Aims Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, multisystemic, autoimmune vasculitis involving small to medium-sized blood vessels. It has a well-established affiliation with atopic syndromes, typically adult-onset asthma and sinusitis. More rare associations are linked with drug side effects such as Montelukast. According to the ‘American College of Rheumatology 1990’, the following criteria yield a sensitivity of 84% in identifying a patient with EGPA. The criteria include: asthma, eosinophilia &amp;gt;10% on differential white cell count, mononeuropathy or polyneuropathy, transient pulmonary infiltrates on chest radiography, paranasal sinus abnormality, and blood vessel biopsy demonstrating extravascular eosinophilia, in a patient with symptoms of systemic vasculitis. The multisystemic involvement of EGPA makes both diagnosis and management difficult as presentations can vary. Methods We report the complex case of a 61 year-old gentleman diagnosed with EGPA. His relevant past medical history includes a diagnosis of Asthma in 2002 (managed with inhaled bronchodilators, corticosteroids, and montelukast), recurrent sinusitis, and lower respiratory tract infections. The patient presented with a one month history of abdominal cramping and bloating. This was associated with nausea, constipation, anorexia, and a week of ‘drenching’ night sweats. His full physical examination during this time was unremarkable. Results He had a normal CT Abdomen Pelvis and colonoscopy. His admission chest radiograph demonstrated bilateral parenchymal lung changes. This was followed by a CT Thorax showing bilateral upper and lower lobe bronchiectasis. Mediastinal and hilar adenopathy consistent with a diagnosis of allergic bronchopulmonary aspergillosis with associated reactive adenopathy. His laboratory investigations showed eosinophilia (21.68) and leucocytosis (32.7). In view of these results, an autoantibody screen was sent showing positive ANCA and positive anti-Myeloperoxidase antibodies(MPO titre 55). Two days into his admission he developed pain and paralysis of his right upper limb. He reported marked fatigue followed by a petechial, non-blanching, vasculitic rash on both ankles. Neurological examination reflected a subacute symmetrical sensory and motor polyneuropathy predominantly involving the right side with bilateral foot drop. MRI Brain demonstrated an established infarct in the left external capsule. He was commenced on high dose aspirin. Due to high suspicion for EGPA, methylprednisolone was commenced for 5 days, followed by tapering oral corticosteroid therapy and Rituximab therapy. A skin punch biopsy showed ‘ a vasculopathic type reaction with small vessel vasculitis, associated mixed inflammatory infiltrate which includes some eosinophils’ confirmed his diagnosis. Conclusion A topic of particular relevance to this case is the association between EGPA and montelukast. It has been hypothesised that there may be a relationship between Leukotriene Receptor Antagonist Therapy, specifically Montelukast, with the pathogenesis of EGPA. Some research suggests that treatment with Montelukast permits the decreased need for oral corticosteroids for asthma control. Therefore, suggesting that EGPA could have been masked by corticosteroid therapy. Disclosure Á. Connerton: None.

E033 The evolution of neurosarcoidosis on infliximab: a case report

Abstract Background/Aims We report a case of neurosarcoidosis (NS) 16 years after initial diagnosis of systemic sarcoidosis in a patient treated with methotrexate, infliximab and corticosteroids. Methods In 2015 a 47-year-old male presented with florid synovitis in MCPs, PIPs, in a pattern reminiscent of rheumatoid arthritis (rheumatoid factor, anti-CCP antibodies were negative; CRP 46g/l). Previously in 2006 a diagnosis of sarcoidosis had been made due to systemic symptoms, arthralgia, bilateral hilar lymphadenopathy (BHL) and confirmed on biopsy of bone and mediastinal nodes, for which he was currently prescribed prednisolone 10mg. So in 2015, considering this to be an inflammatory arthritis (IA), in the presence of sarcoid, methotrexate 15mg subcutaneously was added with good response initially. Though his BHL had regressed and he has was systemically well his IA subsequently flared, and after liaising with his respiratory consultant, adalimumab was added in 2017. Subsequently, as he was well, prednisolone was weaned to 3mg daily. In May 2020 his IA flared again, this time with DIP involvement and dactylitis (his father has psoriasis). Adalimumab was switched to baricitinib, and prednisolone increased to 15mg daily. Baricitinib was inefficacious and linked to recurrent sinusitis so this was switched to infliximab in January 2022 and methotrexate increased to 25mg weekly with resolution of his IA. The patient presented in September 2022 with confusion, slurred speech and unsteady gait with a background of recurrent headaches for six months since starting infliximab. Results Routine blood tests were normal: full blood count, biochemistry, serum ACE, calcium and CRP 2mg/L. Chest X-ray was normal. Neuroimaging: MRI (non-contrast) and CT head were normal. CSF fluid: protein 1.75(0.15-0.4)g/l; glucose 1.8(2.2-3.9)mmol/L; Lymphocytes 98cmm; bacterial and viral culture negative. A diagnosis of neurosarcoidosis was made. Prednisolone was increased to 30mg daily with resolution of neurological symptoms and headache. Conclusion In the UK the incidence of NS is 1/100,000. NS is reported in 5-10% of patients with sarcoidosis and typically presents within 2 years of diagnosis. Presentation of NS includes: cranial neuropathies (50-75%), aseptic meningitis (10-20%), parenchymal disease (&amp;lt;50%), hydrocephalus (10%), sellar disease (2-8%), myopathy (10%), myelopathy (5-26%), neuropsychiatric illness (depression 60%, psychosis 20%) and peripheral neuropathy (2-86%). Typical CSF examinations in NS are: a mild to moderate pleocytosis (usually &amp;lt;100cells/μL), with lymphocyte predominance and elevated protein. Though Infliximab is reported as a treatment option for NS, we reflect that anti-TNF alpha therapies, though they reduce systemic inflammation, do not cross the blood brain barrier. We speculate that introduction of infliximab unmasked NS in this patient. This case also highlights the importance of probing and investigating neurological symptoms in patients with sarcoidosis. Disclosure H.S. Jayasekera: None. S.A. Tabassum: None. K.M.J. Douglas: None.

Otorhinologic Disorders in 22q11.2 Deletion Syndrome.

Investigate incidence and natural history of otologic and sinonasal disease associated with 22q11.2 deletion syndrome. Case series. Tertiary care children's hospital. Charts from consecutive children born 2000 to 2018 with a diagnosis of 22q11.2 deletion, DiGeorge, or velocardiofacial syndrome based on the International Classification of Diseases (ICD)-9 and ICD-10 codes were reviewed. Otologic and rhinologic diagnoses and surgeries and immune and microbiologic laboratory findings were collected from the medical record. After the exclusion of patients with no 22q11.2 deletion (n = 101), otologic care at an outside hospital (n = 59), and loss to follow-up prior to 3 years of age (n = 22), 128 were included. Males comprised 80 (62.5%) patients, 115 (89.8%) were white, and the median age at genetic confirmation of 22q11.2 deletion was 119 days (range 0 days to 14.6years). Recurrent acute otitis media (RAOM), chronic otitis media with effusion, chronic rhinosinusitis, and recurrent acute sinusitis were diagnosed in 54 (42.2%), 37 (28.9%), 10 (7.8%), and 8 (6.3%), respectively. Tympanostomy tubes were placed in 49 (38.3%). Adenoidectomy and sinus surgery were performed in 38 (29.7%) and 4 (3.1%), respectively. Neither immunoglobulin nor cluster of differentiation deficiency increased the odds of RAOM diagnosis, tympanostomy tube placement, or chronic/recurrent sinusitis. Methicillin-resistant Staphylococcus aureus was the most common organism in sinus cultures (4/13, 30.8%). Streptococcus pneumonia dominated otorrhea cultures (11/21, 52.4%). Approximately half of children with 22q11.2 deletion may experience otologic disease that often requires surgical management. Future studies will utilize a larger cohort to examine the role of immunodeficiency in otologic and rhinologic disease in this population.

Efficacy functional endoscopic sinus surgery (FESS) for the treatment of chronic rhinosinusitis

Objective: To assess the efficacy and quality of life after functional endoscopic sinus surgery (FESS) in individuals who have chronic rhinosinusitis. Methods: This prospective study was held in the department of ENT of Aziz Bhatti Shaheed Teaching Hospital and ENT, Head and Neck Surgery department, Pak International Medical College and Peshawar Institute of Medical Sciences (PIMS), Hayatabad Peshawar for the duration from September 2021 to September 2022. The study had 80 patients in total who underwent FESS surgery. Inclusion criteria: radiographically and clinically suggested disease of the paranasal sinuses and nose, such as rhinosporidiosis, nasal polyposis, chronic recurrent acute sinusitis and patients who are not improving after receiving the proper surgical care (antral lavage) and medical care. Exclusion criteria: Nasal and paranasal sinus Malignant conditions. Patients with acute sinusitis who have been diagnosed with intracranial complications and patients with intracranial complications. The data was analyzed with SPSS 21.0. Results: There were 80 patients in total. In this study, ethmoid polyps were operated in 30 (37.5%) patients, chronic rhinosinusitis in 23 (28.8%), Antrochoanal polyps in 12 (15%) patients, rhinosporidiosis in 10 (12.5%) patients, and in 5 (6.2%) patients with inverted papilloma’s were operated.

Assessment of low immunoglobulin levels and clinical manifestations in patients with mastocytosis.

Patients with a low IgG level alone or with low IgA or IgM levels have been reported to be susceptible to respiratory tract infections and recurrent sinusitis. Patients diagnosed with CVID have a higher prevalence of autoimmune diseases and lymphoid malignancies. Mastocytosis is a myeloproliferative disease, not typically associated with autoimmune disease or frequent infections. We sought to determine the distribution of immunoglobulins in children and adults with mastocytosis. Evaluate the impact of low immunoglobulins on the clinical management of patients with mastocytosis. We performed a 10-year retrospective analysis on 320 adult and pediatric patients with mastocytosis for immunoglobulins using an electronic medical query. We identified 25 adults and 9 children with one or more low immunoglobulins. Patient records were examined for a history of infections and autoimmune disorders. Serum immunoglobulins in children and adults with mastocytosis fell within a normal range. Among patients with low IgG levels alone or with low IgM and /or IgA, 20% had a history of infections and 20% of adults had autoimmune disorders. The most common infection was recurrent otitis media (OM). Patients with mastocytosis typically have normal immunoglobulins. With few exceptions, those with low immunoglobulins did not have frequent infections or autoimmune diseases. This data supports the conclusion that routine determination of immunoglobulins in patients with mastocytosis is not required and reserved for patients with clinical conditions, which might be related to an immunoglobulin deficiency.