Chronic Rhinosinusitis in Patients with Primary Immunodeficiency.

INTRODUCTION: Primary immunodeficiencies are a heterogeneous group of diseases associated with an increased incidence of infections, autoimmunity, autoinflammatory diseases, allergies, and cancer. Rhinosinusitis is one of the most common infections in these patients. In our study, we aimed to determine the presence of chronic rhinosinusitis in our patients with primary immunodeficiency and to investigate the etiology of chronic rhinosinusitis. METHODS: Forty-four patients (age range: 4-26 years) diagnosed with primary immunodeficiency were enrolled in our study. Patients were interviewed about the symptoms of chronic rhinosinusitis, and nasal endoscopic examinations were performed prospectively. The results of laboratory tests, medications, skin allergy tests, and the patients' lung computed tomography were retrospectively recorded from patient files. RESULTS: The distribution of patients' diagnoses included 38.6% (n = 17) primary antibody deficiencies, 6.6% (n = 3) combined immunodeficiencies, 27.3% (n = 12) combined immunodeficiencies with syndromic features, 6.8% (n = 3) phagocytic disorders, and 20.5% (n = 9) immune dysregulation disorders. There was no significant difference in the frequency of chronic rhinosinusitis among the different immunodeficiency groups. There were no significant differences between chronic rhinosinusitis and conditions such as atopy, hypogammaglobulinemia, and treatments with immunoglobulin and/or azithromycin. The incidence of chronic rhinosinusitis was 77.8% (n = 7) in patients with a history of acute sinusitis and 20% (n = 7) in patients without a history of sinusitis, with a statistically significant difference between them (p = 0.002). CONCLUSION: Chronic rhinosinusitis is more common in patients with primary immunodeficiencies than in the normal population. For effective treatment, it is necessary to identify the factors that cause chronic rhinosinusitis. Further studies involving larger patient populations are needed to explain the mechanisms of chronic rhinosinusitis.

Long-term efficacy of tonsillectomy/adenotonsillectomy in patients with periodic fever aphthous stomatitis pharyngitis adenitis syndrome with special emphasis on co-existence of familial Mediterranean fever.

Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is one of the most common autoinflammatory fever disorders in the childhood which may co-exists with familial Mediterranean fever (FMF) causing treatment complexity. As the role of surgery in PFAPA syndrome is still controversial, in this paper, our aim is to present our results of tonsillectomy/adenotonsillectomy in the treatment of PFAPA syndrome. Archives of a tertiary care hospital were investigated for patients who underwent tonsillectomy or adenotonsillectomy due to PFAPA Syndrome between 2010 and 2020. 344 patients were found but only 281 of them were accessible. Through phone call interview and chart review methods, preoperative and postoperative the number and severity of the attacks and general satisfaction after the operation were recorded and analyzed. Also, patients with concomitant FMF were analyzed separately. A total of 281 patients were included in the study. There was no improvement in 10 (3.55%) patients. Eight (2.84%) patients showed mild improvement, 29 (10.32%) patients had moderate improvement and 234 (83.27%) patients had full recovery after tonsillectomy. There were 266 PFAPA patients without FMF. No improvement, mild improvement, moderate improvement, and full recovery in this patient group were 5 (1.9%), 6 (2.3%), 25 (9.4%) and 230 (86.5%), respectively. FMF was present in 5.33% (15/281) of the patients. In PFAPA + FMF group 5 patients had no improvement (33.3%), 2 had mild improvement (13.3%), 4 had moderate improvement (26.7%) and 4 had full recovery (26.7%). Benefit of tonsillectomy was significantly lower in the patients with concomitant FMF when compared to the patients who did not have FMF (p < 0.001). Age of diagnosis, age of operation, severity of the disease, type of operation, and gender were found to have no significant relationship with the benefit from surgery (p < 0.05). According to the findings of this study, tonsillectomy is an effective long-term treatment for PFAPA syndrome with success rate of 83.27%. Also, preoperatively FMF should be considered in these patients, which dramatically reduces surgical efficacy.

Vestibular disorders in children: A retrospective analysis of vestibular function test findings.

OBJECTIVE: This study was designed to describe the most common vestibular disorders in children and their associated findings on vestibular function testing. METHOD: Data from 203 children with a mean age of 11.16 ± 3.87 (range, 1-17) years were collected from among 3400 patients who underwent vestibular assessment at a vertigo center in a tertiary hospital over a 3-year period. A retrospective data analysis was performed for 203 children. RESULTS: Vestibular disorders were diagnosed in 78.3% (n = 159) of 203 children among 3400 patients, which revealed a 3-year incidence of 4.67% in our study. Benign paroxysmal positional vertigo (BPPV) was the most common diagnosis in our group of children (49%; n = 100), which involved both primary BPPV, and secondary BPPV that was associated with other vestibular pathologies. Vestibular migraine (VM) was the second most common diagnosis (41%; n = 83) followed by benign paroxysmal vertigo of childhood (BPVC; 4.5%, n = 9), vestibular neuritis (VN; 4.5%, n = 9), and psychogenic vertigo (4.5%, n = 9). Our study showed that Meniere's Disease (MD; 1.5%, n = 3) and central vertigo (1.5%, n = 3) were less commonly diagnosed in children. Perilymphatic fistula (PLF) was diagnosed and surgically confirmed in only one child. CONCLUSION: BPPV and VM were the most common pediatric vestibular disorders in our study. Clinicians should be aware of the prevalence, signs, and symptoms of the most common vestibular disorders in children to enable diagnosis, treatment, and rehabilitation. Vestibular function testing with age-appropriate adaptations results in improved differential diagnosis, which guides medical treatment and rehabilitation.

Relative proportions of stromal to cellular components of pleomorphic adenomas: determination with shear wave elastography.

OBJECTIVES: The purpose of this study was to evaluate whether the relative proportions of stromal to cellular components of pleomorphic adenomas have an effect on the shear elastic modulus determined by shear wave elastography (SWE). METHODS: This study was approved by the Ethics Committee of our institution and performed between September 2011 and December 2012. Thirty-five patients who underwent surgery for a parotid mass were included in the study. The maximum shear elastic modulus of the mass was measured by SWE. Pleomorphic adenomas were histopathologically subdivided into stroma-poor, intermediate-stroma, and stroma-rich tumors based on the relative proportion of stromal to cellular components. RESULTS: The maximum shear elastic modulus values of the pleomorphic adenomas ranged from 12.6 to 291.9 kPa (mean ± SD, 120.9 ± 92.8 kPa). The Friedman test revealed a statistically significant relationship between the stromal amount and maximum shear elastic modulus (P < .001). Stroma-poor adenomas (mean, 32.3 ± 27.7 kPa; range, 12.6-81.1 kPa) were softer on SWE than stroma-rich adenomas (mean, 174.1 ± 48.4 kPa; range, 92.7-217.2 kPa; P = .009). No such significant difference was detected between stroma-poor and intermediate-stroma tumors (mean, 146.3 ± 106.1 kPa; range, 19.1-291.9 kPa; P = .062) or between intermediate-stroma and stroma-rich tumors (P = .465). CONCLUSIONS: Shear wave elastography depicts pleomorphic adenomas with a variable appearance, which is due to the relative proportions of stromal to cellular contents in the tumors.

Unusual location of primary hydatid cyst: soft tissue mass in the parapharyngeal region.

Hydatid cyst is a cyclozoonotic infection caused by the cestode Echinococcus granulosus. Hydatid cyst in the head and neck region is very rare, even in countries where echinococcus infestation is endemic. We report the case of a 17-year-old male patient presenting with a hydatid cyst in the parapharyngeal and neck region. There was no pulmonary or hepatic involvement. The definitive therapy comprised excision of the cystic mass and postoperative medical treatment.

Congenital vocal cord paralysis.

Congenital stridor is rare. The most common presentation for congenital stridor is chronic loud breathing since birth. It is noticed in patients by the first 4 to 6 weeks of life. There are many causes of congenital stridor. Laryngomalacia is the most common cause of congenital stridor. Congenital vocal cord paralysis is the second most common cause of congenital stridor. Vocal cord paralysis in infants and children can be either congenital or acquired and either unilateral or bilateral. Although most commonly the paralysis is idiopathic, there are many causes of acquired vocal cord paralysis. Arnold-Chiari malformation is the most common congenital central nervous system abnormality resulting in vocal cord paralysis, and central nervous system abnormalities usually may cause bilateral vocal cord paralysis. In this article, we report 2 patients with idiopathic congenital vocal cord paralysis. One of them has the unilateral vocal cord paralysis, and the other, bilateral.

Lack of Chlamydophila pneumoniae and predominance of Alloiococcus otitidis in middle ear fluids of children with otitis media with effusion.

OBJECTIVE: To investigate the presence of Chlamydophila pneumoniae and other bacterial pathogens in middle ear effusion samples obtained from children with otitis media with effusion (OME). MATERIALS AND METHODS: Twenty-eight children (mean age 7.03; standard deviation 2.18) with OME unresponsive to medical therapy were included in the study. All of the children underwent ventilation tube insertion under general anesthesia. Eighteen patients were bilaterally affected whereas 10 children had unilateral disease. The middle ear fluids (46 samples in total) were collected during ventilation tube insertion, and were evaluated subsequently for the presence of C. pneumoniae and other bacterial pathogens using polymerase chain reaction (PCR). RESULTS: Although all samples were negative for C. pneumoniae, bacterial DNA was detected in 21 of 46 samples. Overall 40% of the patients (4/10) with unilateral involvement, and 61% of the patients (11/18) with bilateral involvement were positive for bacterial DNA. In 6 patients with bilateral OME bilateral samples were positive, whereas 5 patients with bilateral OME showed only unilateral positivity. According to the results of DNA sequencing analysis, all of the positive samples harbored only one bacterial species. In 12 of 46 samples Alloiococcus otitidis DNA (26%), in 7 Haemophilus influenzae DNA (15%), in one Streptococcus pneumoniae DNA (2%) and in one Moraxella catarrhalis DNA (2%) were present. CONCLUSIONS: Our findings support that C. pneumoniae does not seem to have a role in OME in children whereas A. otitidis was found to be more frequent than the other common pathogens. Further studies are required to elucidate the exact pathogenetic role of these microorganisms in OME.

Current management of isolated sphenoiditis.

Isolated sphenoiditis (IS) is a relatively rare clinical entity which might present with serious complications. The clinical records of ten patients with IS were reviewed. The presenting symptoms, the findings, and the treatments given were noted. Eight patients were female and two were male, and their age varied between 9 and 65 years (mean 31 years). The main presenting symptom was headache in five patients, diplopia in four patients, and postnasal drainage in one patient. The duration of the symptoms ranged between 48 h and 1 year. The diagnosis was accomplished by history, nasal endoscopy and radiological examination (computed tomography and/or magnetic resonance imaging). Two patients had fungus ball. One patient was a scuba diver as a possible predisposing factor. All of the patients underwent medical treatment consisting of intravenous antibiotics or oral antibiotics, and endoscopic sinusotomy was performed in nine patients additionally. Complete resolution was obtained for all patients except one who had diplopia for one year. IS may present with headache and orbital symptoms. Timely diagnosis and treatment are substantial in order to avoid serious complications, and to obtain a complete recovery. Medical treatment does not avoid surgery in majority of cases. Surgery is indicated from the very beginning specifically for the cases starting with diplopia which might be suggestive of a progression of the infection. Currently the most frequently used approach is endoscopic transnasal sphenoidotomy. This technique seems to be effective and less traumatic compared to other approaches.

Infantile supraglottic hemangioma: a case report.

Hemangiomas of the airway are benign vascular lesions that can involve any site from the nares to the tracheobronchial tree. Most of these lesions are seen in the subglottic area in infants. Supraglottic infantile hemangiomas are very rare. We report a case of supraglottic hemangioma in a 2-month-old boy who had been admitted to our hospital with inspiratory stridor and dyspnea. The hemangioma involved the left arytenoid and aryepiglottic fold. A tracheostomy was performed, and the patient was followed up endoscopically every 6 months thereafter The hemangioma disappeared when the child was 30 months old. Subsequently, a Montgomery T-tube was placed for 6 months to assist in maintaining normal breathing. The patient remains disease-free during ongoing follow-up. We also discuss the management strategies for infantile laryngeal hemangiomas.

Hoarseness and laryngopharyngeal reflux: a cause and effect relationship or coincidence?

The role of laryngopharyngeal reflux (LPR) on hoarseness is widely assumed and accepted. Few articles present objective information on the subject. One of the main problems in establishing a relationship is our lack of knowledge of what is normal. We compared patients with chronic hoarseness and healthy controls to establish three goals: (1) to demonstrate the presence of additional symptoms and signs of LPR in patients with hoarseness; (2) to find the prevalence of LPR by utilizing 24 h double-probe in patients with hoarseness and compare it with that of the control group; (3) to contribute data to establish prevalence of LPR in healthy population. Forty-three patients with hoarseness and 20 healthy volunteers were enrolled in the study. All the patients in the study group had hoarseness of more than 3 months duration. The subjects recieved videolaryngoscopic evaluation and 24 h double-probe pH monitoring, and the results were compared. In the study group, 27 patients (62.8%) out of 43 had LPR episodes, compared to 6 (30%) of 20 healthy volunteers. Mean number of LPR episodes recorded by the pharyngeal probe was 7.0 [standard deviation (SD): 8.8] in 24 h, and this was significantly higher than that of the healthy controls [0.9/24 h (SD: 1.9)] (P = 0.003). Mean number of LPR episodes of the study group in upright position was 5.8 (SD: 7,0) and in supine position was 1.2 (SD: 3.3). These numbers were also significantly higher from the controls (P = 0.005 and P = 0.014 respectively). The results of this study show that: (1) most common additional symptoms were heartburn and chronic throat clearing and most common finding is pachydermia; (2) LPR incidence in patients with chronic hoarseness is significantly higher than the LPR incidence in healthy controls; (3) LPR is present in healthy people. However, the severity of LPR seems to be the causative factor rather than its presence.

Tonsillar metastasis of primary signet-ring cell carcinoma of the cecum.

Metastatic carcinoma of the palatine tonsil is unusual. The most common primary sites of the tonsillar metastasis are the kidney, cutaneous melanoma, lung and breast. Primary manifestation of these tumors as a tonsillar mass is extremely rare in the literature. We present a case with a tonsillar metastasis from unusually localized primary signet ring-cell carcinoma of the cecum.

Unusual extension of the first branchial cleft anomaly.

First branchial cleft is the only branchial structure that persists as the external ear canal, while all other clefts are resorbed. Incomplete obliteration and the degree of closure cause the varied types of first branchial cleft anomalies. They were classified based on the anatomical and histological features. We present an unusual type of first branchial cleft anomaly involving the external auditory canal, the middle ear and the nasopharynx through the eustachian tube.

Isolated sphenoid sinusitis presenting with unilateral VIth nerve palsy.

Isolated sphenoid sinusitis is a rare disorder. There are some difficulties in its diagnosis and the first presentation of this disorder might be with complications. These complications are essentially due to the anatomical location of the sinus and its proximity to the intra-cranial and orbital contents, to which infection may easily spread. In this paper, we report a case of isolated sphenoid sinusitis in a 12-year-old girl who was presented with unilateral VIth nerve palsy. She was initially treated with parenteral antibiotherapy, the abducens nerve palsy recovered but as sphenoiditis persisted she underwent an endoscopic sphenoidotomy. We discuss the clinical features, the diagnostic tools, and the treatment options for this entity.

[Hyperbaric oxygen treatment in sudden hearing loss after unsuccessful medical treatment]. / Medikal tedavinin basarisiz oldugu ani isitme kayipli olgularda hiperbarik oksijen tedavisi.

OBJECTIVES: We evaluated the efficacy of hyperbaric oxygen (HBO) treatment in patients with sudden hearing loss unresponsive to medical treatment. PATIENTS AND METHODS: Fifty-one patients (37 males, 14 females; mean age 37 years; range 9 to 63 years) with sudden hearing loss were treated with HBO following unsuccessful medical treatment. Time elapsed from the occurrence of sudden hearing loss ranged between 15 to 45 days. Treatment included two sessions daily for the first three days, followed by a single daily session, to make 20 sessions of 90 minutes. Treatment was discontinued because of early recovery in two patients. Audiometric examinations were made at the end of every five sessions. The patients were classified according to age, gender, and audiogram curves. RESULTS: The mean hearing thresholds were 75.3 dB and 65.6 dB before and after treatment, respectively. Recovery was rated as complete in two patients (3.9%), moderate in two patients (3.9%), mild in 19 patients (37.25%), and as no recovery in 28 patients (54.9%). No significant differences were found between the patient groups in terms of improvement and hearing gain (p>0.05). CONCLUSION: We propose that HBO be resorted to when other means of medical treatment prove unsuccessful in patients with sudden hearing loss.

[A case of sinonasal lymphoma]. / Sinonazal lenfoma: Olgu sunumu.

Sinonasal lymphoma is a rare disease that poses difficulties in diagnosis. A fifty-year-old male patient presented with a mass in the nasal vestibulum that destroyed the left nasal ala. Nocardiosis was considered in the light of the initial biopsy findings; however, microbiologic studies were not consistent with pathologic examination. A subsequent biopsy was obtained, which resulted in the diagnosis of peripheral T-cell lymphoma. Following the diagnosis, the patient was referred to the oncology clinic to receive chemotherapy. He died during the second course of chemotherapy.