Management of chronic sinusitis in cystic fibrosis

Chronic rhinosinusitis is extremely common in patients with cystic fibrosis. It causes numerous problems in these patients and can put them at risk for life-threatening illness. Potential problems include nasal obstruction, congestion, sinus pain and pressure, infection (usually with Pseudomonas organisms), hyposmia or anosmia, and the seeding of bacteria into the lower respiratory tract. Cystic fibrosis patients with chronically infected sinuses are at increased risk for pneumonia following lung transplantation. A prophylactic protocol has been developed for the management of chronic sinusitis in patients with cystic fibrosis. These patients are fully evaluated at the Nasal Dysfunction Clinic of the University of California, San Diego (UCSD), Medical Center. Based on the results of the evaluation, they are treated with endoscopic sinus surgery, partial middle turbinectomy, septoplasty, and a large middle meatal maxillary antrostomy. Surgery is followed by a rigorous regimen of pulsatile hypotonic saline nasal irrigation to wash away tenacious cystic secretions. Tobramycin (Nebcin®) is given once daily in the nasal irrigant to inhibit the growth of Pseudomonas organisms. At the USCD Nasal Dysfunction Clinic, this prepulmonary transplantation protocol is now used in all cystic fibrosis patients with chronic sinusitis.     

Dornase alfa as postoperative therapy in cystic fibrosis sinonasal disease

OBJECTIVE: To determine the benefit of nasally inhaled dornase alfa in patients with cystic fibrosis and nasal symptoms. DESIGN: Double-blind placebo-controlled trial. SETTING: Cystic Fibrosis Regional Center of Campania at the University of Naples "Federico II." PATIENTS: A total of 24 patients with cystic fibrosis and chronic sinusitis. INTERVENTIONS: Patients underwent sinonasal surgery during a 3-year period and received once-daily doses of either dornase alfa (2.5 mg) or hypotonic saline solution (5 mL) beginning 1 month after surgery and for a 12-month period. MAIN OUTCOME MEASURES: Primary outcomes were nasal-related symptoms and nasal endoscopic appearance; secondary outcomes were forced expiratory volume in 1 second, nasal computed tomography findings, and saccharine clearance test results. Patients were evaluated before and after treatment. RESULTS: After surgery, all outcomes were significantly improved for each treatment at 1 month (P<.05); primary outcomes were improved at 24 and 48 weeks in the group receiving dornase alfa (P<.05), and at 12 weeks in the group receiving placebo. Secondary outcomes were better in the dornase alfa group (P<.01) than in the placebo group at 12 months except for the saccharine clearance test results. In particular, median relative difference in forced expiratory volume in 1 second between dornase alfa and placebo was significantly improved in the dornase alfa group (P<.01). CONCLUSIONS: Nasally inhaled dornase alfa can be effective in patients with cystic fibrosis and sinonasal disease who do not respond to conventional therapy after surgical treatment. Further studies should be carried out to determine the long-term effect on sinus disease, recurrence of polyps, and quality of life.     

Abnormal expression of the cystic fibrosis transmembrane regulator in chronic sinusitis in cystic fibrosis and non-cystic fibrosis patients.

Cystic fibrosis (CF) patients commonly suffer from chronic sinusitis. Mutations of a single gene, the cystic fibrosis transmembrane conductance regulator (CFTR) gene, have been associated with CF. Functional CFTR protein is localized to the apical cell membrane, while dysfunctional CFTR is commonly found in the cytoplasm. We undertook a preliminary immunocytochemical study of CFTR subcellular localization in CF and non-CF pediatric and adult patients using a newly developed murine monoclonal antibody, TAM. Immunostaining was evaluated for subcellular localization (cytoplasmic versus membranous) and for epithelial layer (basal versus luminal). Analysis of the predominant CFTR distribution patterns demonstrated significant differences in adult versus pediatric groups independent of whether the latter were CF or non-CF (p<.0001 and p<.008, respectively), and no significant difference between the 2 pediatric groups (p = .70). This suggests that the pathophysiology of pediatric sinusitis differs from that of adult sinusitis at the level of secretion production.     

Cytokine profile of chronic sinusitis in patients with cystic fibrosis

BACKGROUND: The inflammatory-cell and cytokine profiles of chronic sinusitis (CS) are well documented in the literature. In contrast, little is known about the pathogenesis of this condition in patients with cystic fibrosis (CF). OBJECTIVE: To determine whether patients with CF have inflammatory-cell and cytokine profiles that differ from other patients with CS. METHODS: Patients with CF (n = 7) and adults with CS (n = 7) undergoing functional endoscopic sinus surgery were recruited for the study. Patients with no allergies or sinus disease (n = 6) were used as controls. Using immunohistochemical analysis, we assessed sinus mucosal specimens for the presence of T lymphocytes, eosinophils, macrophages, and neutrophils. Using in situ hybridization, we assessed the expression of interleukin (IL) 4, IL-5, IL-8, IL-10, and interferon gamma. RESULTS: There was a higher number of neutrophils, macrophages, and cells expressing messenger RNA for interferon gamma and IL-8 in patients with CF than in patients with CS or in controls (P<.01). The number of eosinophils and cells expressing messenger RNA for IL-4, IL-5, and IL-10 was higher in patients with CS than in those with CF and controls (P<.01). CONCLUSIONS: Sinus disease in patients with CF presents different inflammatory-cell and cytokine profiles than that seen in other patients with CS. These results may explain the difference in response to treatment in the CF group.     

The usefulness of computed tomography scans in cystic fibrosis patients with chronic sinusitis

BACKGROUND: This study was performed to evaluate the usefulness of computed tomography (CT) scans of the sinuses in children with cystic fibrosis (CF) as an outcomes measurement for medical and surgically treated chronic rhinosinusitis. METHODS: Patients with CF who had sinus scans performed at the study institution from January 1999 to September 2003 were identified. The scans and any available prior scans were scored according to the Lund-MacKay system. A retrospective chart review determined if hospitalization with i.v. antibiotic therapy or operative intervention had occurred between scans and if the scans showed improvement secondary to the intervention. RESULTS: One hundred thirty-four patients were identified who met inclusion criteria. A total of 290 scans were reviewed, with the average number of CT scans per patient of 2.24 (range, 1-10). The average Lund-MacKay score for scans closest in time to the first sinus surgery was 14.5. (range, 7-24), and for the first postoperative scans, the score was 14.7 (8-24). There was no significant difference between the preoperative and postoperative Lund-MacKay score after initial surgery (p = 0.99) or in subsequent scans despite medical or surgical interventions (p = 0.90). CONCLUSION: CT scanning of the sinuses does not appear to be useful in determining outcomes of intervention in CF patients.     

Immunomorphological features of chronic cystic sinusitis

Morphogenesis of cystic lesions of the paranasal sinuses is described. Immunohistochemical studies were conducted using a panel of monoclonal and polyclonal antibodies. A comparative analysis of the immunohistochemical parameters of the cystic lesions suggested the presence of chronic inflammation with immune component in the wall of false cysts.     

Nasal polyps and sinusitis in children with cystic fibrosis

Eighteen children suffering from cystic fibrosis and nasal polyps were studied. Two patients presented with nasal polyps at two years of age; the remaining patients had an initial polypectomy at any age up to fifteen years (mean age of seven and a half years). There were no obvious correlations between the cultures of the sinus washouts and the corresponding sputum samples. Organisms were only grown from five of the fifteen wash outs and from nine of the sputum samples. The patients with severe polyposis were not obviously more atopic.     

Preventing postoperative complications in the adult cystic fibrosis patient

Herein we report on a 27-year-old cystic fibrosis (CF) patient who developed bronchospasm, secretory plugging of the trachea, and pneumothorax following general endotracheal anesthesia for intranasal polypectomy and bilateral Caldwell-Luc procedures at an adult facility. Intranasal polypectomy and paranasal sinus procedures are the most common surgical procedures performed on cystic fibrosis patients, making the otolaryngologist a frequent member of the cystic fibrosis team [8]. As survival improves, the pediatric otolaryngologist will find himself following CF patients into their 20's and 30's. Adult care facilities may not be as familiar with these patients as the surgeon would like. The otolaryngologist's familiarity with the unique perioperative requirements of the CF patient can prove invaluable in such a setting. Preoperative assessment should identify any acute pulmonary changes, assess nutritional status, assure good control of blood glucose levels, and rule out clotting abnormalities. Good perioperative hydration and meticulous attention to pulmonary toilet are of foremost importance in the surgical care of the cystic fibrosis patient. Chest physiotherapy and suctioning of the tracheobronchial tree should precede arousal from anesthesia and extubation. By insisting on appropriate anesthetic and perioperative care, the knowledgeable otolaryngologist may circumvent potential postoperative complications in the cystic fibrosis patient.     

Surgical management of chronic sinusitis in children

OBJECTIVES/HYPOTHESIS: The objective was to compare three common surgical modalities in children for the treatment of chronic sinusitis that is refractory to medical management. STUDY DESIGN: Prospective nonrandomized study in a pediatric otolaryngology tertiary service. METHODS: Two hundred two children who satisfied criteria for surgery and were referred over a 10-year period were studied. Children were divided into three surgical groups. Group 1 had both endoscopic sinus surgery and adenoidectomy, group 2 had endoscopic sinus surgery alone, and group 3 had adenoidectomy. After a follow-up period of 12 months, improvement of symptoms was assessed. RESULTS: One hundred eighty-three children had adequate follow-up. Eighty seven percent of children in group 1 had improved symptoms, compared with 75% in group 2 and 52% in group 3 (P < .0001). Multivariate analysis showed that surgical procedure was a predictor of success. Asthma, smoke exposure, and age were independent predictors of success. CONCLUSION: Children who fail medical therapy benefit from surgery. Following certain criteria, one can chose between adenoidectomy alone or endoscopic sinus surgery with adenoidectomy to optimize surgical treatment of these children.     

Surgical management of chronic paranasal sinusitis

An attempt is being made to try to standardize an approach to surgical problems involving the paranasal sinuses, excluding malignancies. This is being done in conformity with a definition proposed by Ferris Smith in 1934 along with certain up-to-date modifications. There are five basic surgical operations commonly and effectively utilized in approaching these sinus problems. Three of these procedures, namely, the nasal antral window, the Caldwell-Luc, and the intranasal ethmoidectomy, are felt to be best utilized in the primary approach to the majority of inflammatory problems. The remaining two procedures, the fronto-ethmo-sphenoid sinusotomy and the osteoplastic frontal flap, are advocated primarily in approaching space occupying lesions, excluding polyps, and secondarily where attempts to manage the problem by the first three lesser surgical procedures have been unsuccessful. The merits, goals, and indications of all five procedures are discussed. In many instances two procedures may have equal effectiveness in handling a patient's particular problem, and the procedure of choice rests with the personal preference of the individual surgeon. At other times an inappropriate procedure may not benefit the patient while a properly selected operation could readily enable a cure. It is in this latter area of selecting the appropriate surgical procedure to attack a particular problem that a serious deficit now exists in the practice of otolaryngology. It is the further contention of this presentation that these five basic procedures can effectively handle the majority of chronically diseased paranasal sinusitis problems. It is unfortunate that the one procedure that is the most effective and the most physiological in handling the majority of the more difficult problems, namely, the intranasal ethmoidectomy, was allowed to fall into near disrepute by a historical misunderstanding.⁰     

Atypical sinusitis in adults must lead to looking for cystic fibrosis and primary ciliary dyskinesia

HYPOTHESES/OBJECTIVES:: In adults, purulent pansinusitis or nasal polyposis starting early in life or that is permanently infected or associated either with chronic bronchial infection, infertility, or situs inversus are uncommon. In these atypical cases of chronic sinusitis (ACS), a primary dysfunction of the mucociliary clearance can be suspected. Adult patients with ACS were therefore investigated to detect primary ciliary dyskinesia (PCD) or cystic fibrosis (CF). STUDY DESIGN: Open, prospective study. PATIENTS AND METHODS: Forty-two patients with ACS were investigated with ciliary beat frequency and ultrastructure analysis in nasal cells and cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation analysis in blood leukocytes. RESULTS: The diagnosis of PCD was confirmed in seven (17%) patients. At least one CFTR gene mutation was detected in 16 (38%) patients. The diagnosis of CF was suggested in three (7%) compound heterozygous patients. Another 13 (31%) patients were heterozygous for a CFTR gene mutation or a complex allele. Comparison of clinical features of ACS showed that only a family history of chronic sinusitis (P <.01) or chronic bronchitis (P <.02) and the presence of diffuse bronchiectasis (P <.0001) or serous otitis media (P <.0001) were significantly more frequent in PCD patients than in patients carrying CFTR gene mutations or those without PCD or CFTR gene mutations. CONCLUSIONS: ACS should be considered a remarkable entity in which congenital abnormalities of epithelial cells are frequently detected (55% of patients). The higher frequency of mutations in ACS patients compared with the general population suggests that heterozygoty for CFTR gene mutation could be a sinusitis-causing status.     

Contemporary management of rhinosinusitis and cystic fibrosis

Historically, cystic fibrosis has been a devastating disease, with patients rarely living beyond childhood. Recent advances during the last two decades, however, have led to an improved understanding of the disease on a molecular level. Such understanding has in turn led to new treatments, longer life expectancy, as well as new challenges in the management of both pulmonary and extrapulmonary manifestations of cystic fibrosis. The otolaryngologist caring for patients with cystic fibrosis is thus challenged with the medical and surgical management of chronic sinusitis and sinonasal polyposis in these challenging circumstances.     

Sinonasal inhalation of dornase alfa in CF: A double-blind placebo-controlled cross-over pilot trial

Objective

The paranasal sinuses are almost always involved in cystic fibrosis, and chronic rhinosinusitis and nasal polyps are very frequent in the disease. Hereby, the patients’ quality of life and their overall health are relevantly impaired. Although dornase alfa, a mucolytic agent, may also be effective in the upper airways, deposition of inhaled drugs into paranasal sinuses is substantially limited. The novel PARI SINUS™ nebuliser has been shown in deposition studies to deliver aerosol into paranasal sinuses but has not yet been clinically tested. This DBPC pilot-trial applying dornase alfa aims to evaluate outcome parameters and sample sizes for a subsequent efficacy trial.

Methods

Primary outcome parameters assessed were the Sino-Nasal Outcome Test (SNOT-20, a disease-specific quality of life assessment tool) and ventilated volume as measured by magnetic resonance imaging. Five CF patients were randomised to inhale either dornase alfa or 0.9% NaCl for 28 days and, after a wash-out period of 28 days, crossed over to the alternative treatment.

Results

Whereas normal saline was not associated with relevant changes in SNOT-20 scores, dornase alfa improved quality of life (p = 0.043). MRI results showed no definite trend.

Conclusion

This first clinical study with the novel device gives promising results for the new therapeutic concept of sinonasal inhalation with vibrating aerosols in regard to further analysis involving larger collectives.     

Evaluation of the results of surgical treatment of patient with paranasal sinusitis and cystic fibrosis

30 patients diagnosed from cystic fibrosis in the Department of Otorhinolaryngology, the Medical University in Warsaw in 1996-1998 participated in this study. In whole group of patients the advanced inflammatory changes in paranasal sinuses were found. In 7 cases endoscopic surgery of paranasal sinuses has been performed. The follow up has been kept by 2 years. In 2 cases reappearance of symptoms and nasal polyps observed after few months post the operation. In the remaining number of patients we noticed the satisfactory result of the treatment and the improvement of the life quality. A good nasal patency and recovery od smell were gained.     

Characteristics of postoperative period in surgical treatment of chronic frontal sinusitis]

During the last 5 years 132 patients underwent frontal-ethmoidal trepanation. Recurrences were observed in 27 (20%) patients with nonspecific immune deficiency, frequent respiratory viral infections and microbial virulence. The major cause of recurrences was inadequacy of the frontal-nasal ostium. Recurrences occurred more frequently in patients with orbital and intracranial complications of frontitis, traumatic frontitis, and abnormal anatomic relations of the frontal and ethmoidal sinuses. It is recommended to carry out postoperative conservative therapy including antibacterial, antiedematous, desensitizing drugs as well as drugs stabilizing immunity and microcirculation. It is also recommended to follow-up patients during a year after frontal-ethmoidal trepanation because recurrent episodes occur mostly during this time period.     

儿童及青少年鼻窦炎内窥镜术后鼻腔黏连的预防与处理

儿童及青少年鼻窦炎内窥镜术后鼻腔黏连的预防与处理谢志刚, 张喜英 (河南省舞阳县人民医院耳鼻喉科, 河南 舞阳 462400) ［摘要］目的：探讨儿童及青少年鼻窦炎内窥镜术后鼻腔黏连的预防与处理方法。方法：对402例儿童及青少年鼻窦炎患者行6个月以上复查和随访,术后发生的鼻腔黏连并分析黏连的原因。结果：64例(82侧)鼻腔黏连中,56例(71侧)一次分离成功,8例(11侧)黏连较重者经多次分离成功。结论：儿童及青少年鼻窦炎内窥镜术后更易发生黏连,黏连与术中动作粗暴损伤鼻腔黏膜,鼻腔狭窄未恰当处理,术后复查不及时,术腔清理不彻底及术后用药不合理等多种因素有关。因此,术后及时换药,定期复查,清理术腔可有效预防黏连,局部应用糖皮质激素也可有效预防黏连。