Medium-term symptom outcomes after paranasal sinus surgery in children and young adults with cystic fibrosis

OBJECTIVE: To determine the effectiveness of paranasal sinus surgery (PSS) for individual symptoms and recurrence of nasal polyposis in children and young adults with cystic fibrosis and chronic rhinosinusitis (CRS). DESIGN: Nonrandomized, prospective, clinical trial. INTERVENTION: Children and young adults with medically refractory CRS were examined before and after PSS. The extend of nasal polyposis was graded endoscopically and on computed tomography images. The severity of symptoms of CRS was recorded on a 6-point Likert scale by the investigator. RESULTS: In 26 patients, a complete rhinosinusitis symptom score (RSS) before and after surgery was available. The mean follow-up time after surgery was 23 months. Postoperatively, no polyp recurrence was seen in eight patients (of 26 patients). An improved polyp score was found in 14 patients. In four patients, polyp recurrence to the same extent as before surgery was seen. The mean RSS was significantly improved after surgery. Before surgery, "nasal obstruction" was the most common complaint. "Headache" was the second most common complaint before PSS. After PSS, although improved, "headache" was the most common complaint. During further follow-up, in two patients, endocranial complications caused by recurrent frontal mucopyoceles (1 frontal abscess, 1 subacute meningitis) were observed. After transfrontal revision surgery, both patients recovered without neurologic damage. CONCLUSIONS: Even in cases of polyp recurrence, PSS provides significant symptom relief for nasal and facial symptoms associated with CRS. The symptom "headache" requires special attention because it improves least after surgery and may indicate recurrence of frontoethmoidal mucopyoceles or endocranial complication of CRS.     

Extent of pathological changes in the paranasal sinuses of patients with cystic fibrosis: CT analysis.

The aim of the study was to define the characteristic changes revealed by computed tomography (CT) examination of the paranasal sinuses in patients with cystic fibrosis (CF). The group of 30 CF patients was evaluated in the outpatient clinic of the Department of Otorhinolaryngology, Medical University of Warsaw, from 1996 to 1998. The control group consisted of 30 patients with chronic rhinosinusitis (CRS). CT scans were obtained from both groups of patients, and findings revealed more advanced pathological changes in the CF than CRS group. The extensive inflammatory process observed in CT scans of CF patients resulted in the impairment of frontal and maxillary sinus development, destruction of bony structures, and medial projection of the lateral nasal wall.     

Eosinophilic cationic protein as a marker of nasal inflammation in patients with cystic fibrosis.

OBJECTIVES: Evaluation was made of eosinophilic cationic protein (ECP) in nasal secretion for measuring the degree of nasal inflammation and monitoring response to therapy in cystic fibrosis (CF) patients with chronic rhinosinusitis. Symptoms and findings in regard to ECP levels before and after treatment were described. STUDY DESIGN: Study was prospective, with 21 CF patients aged 4 to 19 years; 20 healthy volunteers served as controls. Collection of nasal secretion by a sponge was performed, and blood samples were obtained for serum. Cystic fibrosis (CF) patients were classified according to nasal symptoms and findings. METHODS: ECP was measured by fluoroimmunoassay. Age, sex, nasal symptoms, and endoscopic and histological findings were obtained, and examinations were conducted before and after treatment; recurrences were recorded. RESULTS: In CF patients with chronic nasal inflammation, increased nasal levels of ECP were detected when compared with asymptomatic CF patients or healthy nonatopic subjects. ECP concentrations were strongly related to the extent of nasal disease; patients with nasal polyps had higher levels than those without. Checked at 1 and 4 months after treatment, ECP levels declined with regression of symptoms, and in patients with exacerbation of nasal disease, ECP levels rose. CONCLUSIONS: According to our study, there is a relationship between levels of ECP in nasal secretions and the degrees of nasal inflammation. In addition, the measurement of ECP could be useful in monitoring nasal disease in CF patients.     

Nasal and paranasal sinus endoscopy, computed tomography and microbiology of upper airways and the correlations with genotype and severity of cystic fibrosis

OBJECTIVE: Many studies have assessed clinical and functional aspects of lower airway affections in cystic fibrosis. Conversely, few studies have been performed to assess the clinical and functional affections of upper airways. The objective of the present study was to correlate the variables obtained by nasal and paranasal sinuses endoscopy, paranasal sinus laboratory and computed tomography (CT) scan findings, and to check the association with severity and genotype of cystic fibrosis patients. METHODS: Clinical and laboratory study of 50 patients with cystic fibrosis at a university center. All patients were submitted to CT scan, nasal and paranasal endoscopy and bacterioscopy of maxillary sinus, trachea and oropharynx secretion. Severity of cystic fibrosis was assessed by Shwachman score and the most frequent genetic mutations were identified. RESULTS: The prevalence of polyposis in the studied population was 36% and it was greater among homozygote for DeltaF 508. Shwachman score was correlated with age (p=0.003). The genotype was correlated with presence of nasal polyposis (p=0.006). There was no association between affections in CT scan and severity of cystic fibrosis (CF). Patients presented high prevalence of early colonization of Pseudomonas aeruginosa. CONCLUSIONS: Sinus disease in CF patients presents several clinical, endoscopic and tomographic affections. Although most of them are not correlated with severity and disease genotype, severity of CF is correlated with age and presence of polyposis is genotype-dependent.     

Nasal and paranasal sinus surgery in children with cystic fibrosis

Cystic fibrosis (mucoviscidosis; fibrocystic disease of the pancreas) is an inherited autosomal recessive disorder that results in generalized dysfunction of exocrine glands. Chronic pansinusitis with nasal polyposis occurs frequently in this disease. Polypectomy and sinus drainage procedures are often required in those cases refractory to medical therapy. Recurrence of nasal polyps following removal is common. Controversy exists regarding the timing and extent of surgery. The management of 40 children with cystic fibrosis who underwent nasal polypectomy, intranasal ethmoidectomy, and Caldwell-Luc procedures to treat extensive sinusitis and nasal polyposis forms the basis for this report. Follow-up data indicate that fewer recurrences and longer symptom-free intervals result when intranasal ethmoidectomy and Caldwell-Luc procedures are combined with polypectomy.     

Clinical characteristics and genotype analysis of patients with cystic fibrosis and nasal polyposis1

Clinical characteristics and genotype analysis of patients with cystic fibrosis and nasal polyposis The prevalence of nasal polyps in a group of paediatric patients with cystic fibrosis was prospectively studied in comparison with a control group with cystic fibrosis but without polyps. Clinical variables, including pulmonary function tests, skin testing and mucociliary transport, were carried out in both groups, as well as genotype analysis. Endoscopic intranasal evaluation identified polyps in 29 of 89 patients (33%). Statistical analysis revealed that patients with nasal polyposis had better pulmonary function, a higher rate of Pseudomonas aeruginosa colonization, more hospitalizations, and more prevalence of allergy to Aspergillus fumigatus than did the comparison group. We found no statistically different genotype distribution between the polyposis and the control group. However, it can be emphasized that the prevalence of the compound heterozygous genotype is higher in the nasal polyposis group than in controls. Our observations suggest that other genetic and environmental factors could play an important role in the development of nasal polyposis.     

鼻腔鼻窦腺样囊性癌15例临床分析

探讨鼻腔鼻窦腺样囊性癌的临床特点、诊断、治疗方式及预后。     

慢性鼻窦炎骨炎患者病情程度的危险因素

目的 探讨慢性鼻窦炎骨炎患者病情程度的危险因素及预防对策.方法 对2011年4月～2014年4月期间本院收治的120例慢性鼻窦炎患者的手术治疗及临床资料进行回顾性分析,对影响患者病情程度的危险因素进行Logistic回归分析.结果 本组慢性鼻窦炎病例中,有65例（54.17％）合并骨炎,与未合并骨炎患者比较,其慢性鼻窦炎病程、合并哮喘比例、伴发鼻息肉比例、手术次数、Lund Kennedy评分、Lund Mackay评分、GOSS评分等指标差异有统计学意义（P＜0.05）,尤其慢性鼻窦炎病程、Lund Kennedy评分、LundMackay评分及手术次数等,均是影响慢性鼻窦炎骨炎患者全球骨炎评分（GOSS）的独立危险因素（P＜0.05）.结论 慢性鼻窦炎患者骨炎的发生与手术次数、慢性鼻窦炎病程、Lund-Kennedy评分、Lund-Mackay评分等因素密切相关,应规范鼻窦手术操作,预防骨炎的发生。     

鼻腔鼻窦腺样囊性癌88例临床分析

目的 总结鼻腔鼻窦腺样囊性癌的临床特征和治疗方法,探讨影响鼻腔鼻窦腺样囊性癌患者预后的因素.方法 总结中山大学肿瘤防治中心头颈外科1975年11月至2003年8月收治的88例原发于鼻腔鼻窦的腺样囊性癌患者的临床及病理资料,回顾性分析单纯手术、单纯放疗与放疗结合手术、化疗等治疗方式的疗效.对其治疗与预后进行统计分析.生存分析采用Kaplan-Meier法,组间比较采用Log-rank检验,多因素分析采用Cox比例风险模型.结果 就诊时56例鼻腔鼻窦腺样囊性癌患者为Ⅲ、Ⅳ期病变,治疗多采用手术+放疗.全组患者的5年、10年、15年生存率分别为0.640、0.341、0.190;Ⅲ期患者的5年、10年生存率分别为0.833、0.221;Ⅳ期患者的5年、10年生存率分别为0.323、0.145.手术+放疗组、单纯手术组及单纯放疗组的5年、10年生存率分别为0.761、0.415,0.750、0.367,0.286、0.143.结论 鼻腔鼻窦腺样囊性癌的治疗应以综合治疗为主,临床分期及治疗方式是影响鼻腔鼻窦腺样囊性癌患者预后的独立因素.     

Sinusitis in patients with cystic fibrosis

The management of sinus disease in children with cystic fibrosis is reviewed, based on a literature review and clinical experience in The Hospital for Sick Children, Toronto. Diagnostic and treatment approaches are discussed, with emphasis given to indications for surgical therapies. Great importance is given to the preservation of normal nasal anatomy whenever possible to minimize the possibility of iatrogenic injuries occurring, especially when revision surgery is required.     

Safety of paranasal sinus surgery in patients with cystic fibrosis

Objective: Document the safety of paranasal sinus surgery in cystic fibrosis patients and review the changing trends in paranasal sinus surgery in the cystic fibrosis population. Study Design: Retrospective review. Materials and Methods: Chart review of cystic fibrosis patients who underwent paranasal sinus surgery from 1955 to 1997. Results: Indications for surgery included chronic sinusitis, nasal obstruction, purulent rhinorrhea, head pain, and pyocele. Average duration of anesthesia was 2.1 hours. The complication rate from general anesthesia was zero. Excessive bleeding and significant hypoxia did not occur. Conclusions: Paranasal sinus surgery and general anesthesia can be safely performed in cystic fibrosis patients. The indications for paranasal sinus surgery are changing from symptomatic nasal obstruction to pre-lung transplantation care. Today, treatment has evolved to include placement of sinus catheters for direct topical instillation of antibiotics and consideration of maxillary and frontal sinus obliteration.     

CT of the paranasal sinuses and functional endoscopic surgery: a critical analysis of 100 symptomatic patients

CT scans of 100 patients from the Rhinology Clinic at the Royal National Throat, Nose and Ear Hospital were reviewed in order to test the precepts forming the basis of functional endoscopic sinus surgery, especially as they relate to the radiological investigation. These were: (a) the site of origin of sinus infection, (b) The relevance of certain anatomical variants in the middle meatus to sinus infection and (c) The use of CT as the radiological method of diagnosis in all cases. Obstruction in the middle meatus and ostiomeatal complex was associated with an increased incidence of opacity in the sinuses but the primary site of disease was not established: the concept that sinus disease takes origin in the middle meatus was not proven. Anatomical variants in the middle meatus were not associated with an increase in sinus opacity and there was no evidence that these anomalies have any effect on sinus disease by causing middle meatal stenosis. The radiological assessment of patients with inflammatory naso-sinus disease should start with plain X-ray. CT is unnecessary as a routine examination. It should be reserved for the pre-operative assessment of patients for endoscopic surgery, its main function being to show important anatomical landmarks.     

Negative hearing effects of a single course of IV aminoglycoside therapy in cystic fibrosis patients

Abstract

Objective: Identify hearing effects of a single course of intravenous (IV) aminoglycoside antibiotics (AGs) therapy in adult cystic fibrosis (CF) patients. Determine whether the change is large enough to enable a proof-of-concept study of a new drug preventing AG-associated hearing loss.

Design: Retrospective case review of CF patients with sequential audiograms?±?an intervening course of IV AG therapy.

Study sample: 84 patients with no intervening IV AG treatment, 38 patients undergoing a single course of IV AGs.

Results: Using ASHA ototoxicity metrics, 45% of adult CF patients in the Single-IV group met the criteria for ototoxicity compared to 23% of the No-IV patients. Other hearing metrics including the average maximal threshold shift (TS) and average high frequency TS showed highly significant differences between groups. Testing only participants with mild or greater pre-therapy high frequency hearing loss further increased the differences between the two groups by every metric tested.

Conclusion: Adult CF patients exposed to a single course of IV AGs have significantly greater TS than patients without IV AG exposure. Patients with mild to moderate hearing loss prior to AG-IVs are at increased risk of developing ototoxicity from subsequent parenteral AG therapy.     

Survey anatomy of the paranasal sinuses in the normal mouse

OBJECTIVE: To provide researchers with a survey atlas of normal paranasal sinus anatomy in the mouse as well as to standardize the reporting of data within the murine nose and sinuses. STUDY DESIGN: Histologic and radiographic study in mice. METHODS: C57BL/6 mice were killed and their heads sectioned in the axial and coronal planes as well as imaged using a small animal micro-computed tomography (CT) scanner. Distinctive regions within the nose and paranasal sinuses were delineated and labeled A to G for identification. RESULTS: Definable regions within the normal murine nose and paranasal sinuses include A) the nasal airway, B) the superior nasal vault, C) the osteomeatal complex, D) the anterior ethmoid sinuses, E) the posterior ethmoid sinuses, F) the true maxillary sinus, and G) the secondary maxillary sinus. Mice also possess discernible sphenoid sinuses. CT scans confirmed the histologic plane of section. CONCLUSIONS: A survey atlas of normal murine sinonasal anatomy shall provide laboratories seeking to use mice in sinus research a reference for beginning their work. As new transgenic and gene knockout mice become available, phenotypic changes in sinonasal architecture can be more easily discerned using such a reference. Defining specific regions (A-G) within the sinuses will standardize the nomenclature used for reporting data.