Zuzanna Gorski, *Lidia Zawadzka-Głos
A review of endoscopic sinus surgery in the management of chronic rhinosinusitis and nasal polyposis in pediatric cystic fibrosis patients
Department of Pediatric Otolaryngology, Medical University of Warsaw, Poland
Head of Department: Lidia Zawadzka-Głos, MD, PhD
Introduction. Cystic fibrosis (CF) is an autosomal recessive disease affecting the epithelial lining of the respiratory tract and exocrine glands (1-5). Many children suffering from CF are often diagnosed and treated for various co-morbidities, including chronic rhinosinusitis (CRS) and nasal polyposis (NP) (3, 4, 6, 7), which will remain the focus of this article.
Aim. The aim of this study was to examine the characteristic of patients with cystic fibrosis (CF) admitted to the Pediatric Otolaryngology Department due to coexisting chronic rhinosinusitis (CRS) or nasal polyposis (NP). The study focused on the demographics, symptoms and management of children with CF with coexisting CRS and/or NP. The data was then compared to the results that had been presented in the literature.
Material and methods. A retrospective study of 26 pediatric patients previously diagnosed with CF that were admitted to the Department of Pediatric Otolaryngology of the Medical University of Warsaw between 2010 and 2015 was conducted. Patients’ medical histories were carefully reviewed. Data on patients’ age, gender, symptoms and CF comorbidities were collected. The number and type of procedures performed on each patient were documented. Further assessment of the localization of polyps was performed in all NP-positive patients.
Results. The study included 26 patients (15 males and 11 females). Mean age was 9 years. CRS and NP was present in 100% and 88.5% of the patients, respectively. 23 children underwent a total of 35 sinus surgeries due to CRS and/or NP. 6 patients required one or more revision surgeries, with a total revision rate of 54.1%. Adenoidectomy (AT) and/or adenotonsillectomy (ATT) was performed in 10 patients. 5 children were disqualified from the surgery, due to various reasons. The most common localization of NP was maxillary sinus, followed by ethmoid sinus, sphenoid sinus, frontal sinus, and nasal cavity.
Conclusions. Due to a wide range of clinical findings in many organs and high variability of symptoms in individual cases, there is currently no standardized treatment regimen for pediatric CF patients with CRS or NP. Early intervention and a multidisciplinary approach are highly recommended, due to a positive correlation between the increase in patient’s age and the number of admissions and reoperations. Endoscopic sinus surgery should be considered in CF patients with refractory, chronic or severe acute CRS or NP.
Cystic fibrosis (CF) is an autosomal recessive disease, in which the function of the cystic fibrosis transport regulator (CFTR) gene located on chromosome 7, responsible for chloride ion transport, is compromised. This results in a dysfunction of the epithelial cells lining the respiratory tract, as well as of the exocrine glands (1-5). The organs that are most severely affected are airways, pancreatic ductal system, hepatobiliary system, and male ductus deferens (1, 3). Clinical symptoms may occur as early as after birth or in early childhood, and commonly include pancreatic insufficiency, seen in 90% of infants with cystic fibrosis, vitamin malabsorption, impaired growth and development, and respiratory manifestations, such as recurrent infections, sinusitis, bronchiectasis, and sinonasal polyposis (3, 4, 6, 7). It is important to note that respiratory tract and sinonasal complications due to CFTR malfunction are the most common cause of premature death in CF patients (3). The respiratory symptoms and complications appear to be caused mainly by defective mucociliary drainage, followed by subsequent bacterial colonization – with most common pathogens being Staphylococcus aureus and Pseudomonas aeruginosa (1, 2, 6, 8). The direct pathogenic action of the bacteria, along with the consequent local inflammation and chronic nasal obstruction, play a central role in airway destruction, respiratory failure, and early death in CF patients (1, 3, 8). Two comorbidities that are commonly encountered in CF patients include chronic rhinosinusitis (CRS) and nasal polyposis (NP), which were the focus of the study.
Due to the accumulation of a thick mucus within the sinonasal area, a great number of CF patients consequently develop CRS. CRS is defined as a sinus infection lasting for more than 12 weeks with low-grade signs and symptoms (2, 5, 9-11). The main reasons for the development of CRS are decreased mucociliary clearance, infections, coexisting allergies, edema of the mucosa, and, less commonly, anatomic abnormalities of the sinonasal area (2, 12).
Due to the early onset and subsequent adaptation of the patient to the symptoms of CRS, no more than 10% of the CF patients report that they perceive their symptoms as severe (1, 13, 14). The most commonly reported symptoms include: nasal obstruction and discharge, which lead to mouth breathing, post-nasal drip, headache, and localized facial pain or feeling of pressure (1, 9, 12, 15). Adenoid hypertrophy or inflammation may also be present (14). Although a high number of CRS cases in CF patients are of idiopathic origin, other noteworthy non-CF causative factors of CRS include allergy, aspirin sensitivity, and immunodeficiency (13).
Nasal polyposis (NP) can have multiple causes, however, chronic neutrophil-dominant inflammation, commonly present in CF patients, is believed to be one of the major ones (1, 3, 7, 16). The prevalence of NP in CF patients has been increasing over the years (1). Primary appearance of NP in children may be seen in children as young as kindergartners (1, 14). Nasal polyps are present in approximately 86% of CF patients, however, the prevalence has been shown to be highly variable between individual study populations (2, 6, 14). During the first appearance of nasal polyps, it is recommended to refer the patient to an ENT surgeon for further examination (10). If not previously diagnosed with CF, children presenting with nasal polyps should always undergo further testing for CF (10).
Endoscopic examination, as well as radiological imaging, play a central role in diagnostics and staging of CRS and NP in CF patients (1). Although endoscopic examination in CF patients is consistently abnormal, it is essential in determining the presence of nasal polyps (14). Several radiologic imaging techniques for diagnosing CRS and NP have been discussed in the literature, including spiral multislice CT, digital volume CT, and MRI; MRI is the preferred modality in children (1, 2). MRI offers a sensitive and superior visualization of the mucosa, polyps and other intracranial soft tissue abnormalities (1, 17).
Up to date, there has been no standardized treatment for NP in CF patients due to the high variability of the stage of the disease between individual patients and the lack of strong evidence supporting a treatment that would be successful in a large percentage of the patients (1, 2, 18). A recent review conducted by Mainz and Koitschev (1) summarized several conservative treatment options, including topical application of nasal sprays and drops, lavages and inhalations, nasal saline irrigations, topical decongestants, steroids, antibiotics, immunomodulators, antimycotics, mucolytics, bacterial lysates, antihistamines and monoclonal antibodies. No specific topical or systemic conservative therapies were proven to have significant curative outcomes on NP in CF. Therefore, surgical intervention is the treatment of choice once conservative measures are no longer beneficial for an individual, or in instances of chronic and recurrent disease (1, 4, 13, 18).
Surgical intervention is always preceded by imaging of the nasal cavity and paranasal sinuses, in order to confirm the anatomy and localization of the lesions and to design a specific surgical approach for the removal (1). In addition, the severity of sinonasal symptoms and the grade of the coexisting pulmonary dysfunction are the factors favoring surgery (1). Presently, the most widely used surgical intervention is functional endoscopic sinus surgery (FESS), which is minimally invasive and is associated with enhanced recovery from sinus-related symptoms and an improved subsequent quality of life (18). Two definitive indications for conducting FESS are NP and CRS unresponsive to conservative therapy (19, 20). FESS in children is performed under general anesthesia, whereas in adults, it may be performed under sedation with topical and local anesthesia (21). FESS procedures are highly personalized and dependent on the severity and extent of the changes (20, 21). Many types of FESS may be performed, from the isolated infundibulotomy or the resection of the uncinate process, to a complete sphenoeithmoidectomy, which is, however, very rarely performed (19, 21). Contraindications to FESS include the presence of obstructions or stenoses, which may severely reduce the operative field, as well as any signs of meningitis, other intracranial lesions, severe sinus infections, osteomyelitis of the frontal bone, or orbital cellulitis with visual field defects (20, 21). The aim of sinus surgery in CF patients consisting of enlarging the sinus ostia is to allow improved drainage of the sinuses, eliminate mucosal inflammatory changes such as NP, remove any infected tissue, and permit proper healing and epithelialization of the surgically enlarged ostiomeatal complex (14, 22). The procedure is generally focused on the anterior ethmoidal cells and ostiomeatal complex, since these areas are highly associated with the proper functioning of the remaining sinuses (20). Other more rarely performed extensive surgeries include ethmoidectomy, septoplasty, or the now historic Caldwell-Luc procedure (2, 21). In severe and complicated cases of NP presenting with extensive obstruction, more aggressive and extensive surgical approaches are considered (11, 12).
MATERIAL AND METHODS
A retrospective study on 26 pediatric patients previously diagnosed with CF who had been admitted to the Pediatric Otolaryngology Department at the Medical University of Warsaw between the years 2010 and 2015 was conducted. Patients’ medical histories were carefully reviewed. Symptoms of sinonasal disease were gathered from the interviews with the patient and/or parents, depending on the patient’s age. All available pre-operative endoscopic examinations and imaging studies were analyzed. All 26 patients were admitted to the Department due to clinical symptoms of a various degree. The data on patients’ age, gender and comorbidities were collected. The number and type of procedures performed on each patient were documented. Further assessment of the exact localization of polyps in all NP positive patients was performed.
The mean age of the patients was 9 years (with the youngest patient being 3 and the oldest 16 years). There were 11 male patients (42.3%) and 15 female patients (57.7%). The patients were divided into groups according to age, with 7 patients (26.9%) aged less than 6, 12 patients (46.2%) aged 7 to 12 years, and 7 patients (26.9%) aged 13 to 18 years. Patients were admitted to our Department after the referral from primary specialist care or a different hospital department. With a total number of 45 admissions for 26 patients, 17 patients (65.4%) were admitted once, 4 patients (15.5%) were admitted twice, 3 patients (11.5%) were admitted 3 times, 1 patient (3.8%) was admitted 5 times, and 1 patient (3.8%) was admitted 6 times. An increase in the frequency of admissions and the number of surgeries correlated with an increase in patient’s age, as represented in figures 1 and 2.
Fig. 1. Average number of admissions by age of the patient
Fig. 2. Average number of operations by age
Symptoms at the time of admission included nasal congestion and secretion, nasal obstruction, post-nasal drip, headache, and adenoid hypertrophy. All the patients were positive for CRS, whereas 23 out of 26 patients (88.5%) additionally presented with NP. Clinical examination and studies revealed deviated nasal septum in 7 patients, secretory otitis media in 2 patients, allergies in 2 patients, and von Willebrand disease type 1 in 1 patient. 2 out of 7 patients (28.6%) with a deviated nasal septum underwent septoplasty, whereas both cases (100%) of secretory otitis media were treated with tympanocentesis. Both patients (100%) with coexisting allergies underwent maxillary antrostomy. A total of 54 procedures were performed. The most common procedure was FESS, which was performed 35 times (64.8%), followed by adenoidectomy (AT), performed 11 times (20.3%), maxillary antrostomy, performed 3 times (5.6%), tympanocentesis, performed 2 times (3.7%), septoplasty, performed 2 times (3.7%), and sinus puncture, performed once (1.9%). 35 total of FESS procedures were performed on 23 children diagnosed with NP. Among these 23 patients, 17 patients (73.9%) underwent only 1 surgery, whereas 3 patients underwent 2 surgeries, 1 underwent 3 surgeries, 1 underwent 4 surgeries, and 1 underwent 5 surgeries. The overall revision rate for FESS was 51.4%. 84.3% of the polyps were located in the paranasal sinuses, 13.3% in the nasal cavity, and 2.4% were antrochoanal polyps. In the paranasal sinuses, the most commonly affected sinus was the maxillary sinus (31.4%), followed by ethmoid sinus (25.7%), sphenoid sinus (22.8%), and frontal sinus (20.0%). A total of 11 adenoidectomies (AT) and/or adenotonsillectomies (ATT) were performed on 10 patients, and one patient required a revision AT procedure. 3 patients (30%) underwent AT/ATT prior to sinus surgery, compared to 7 patients (70%) in which AT/ATT was performed during the FESS. The revision procedure was undertaken following a primary peri-operative AT.
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the CFTR gene (1-5). Though CF may manifest through a wide range of clinical findings many organs, the aim of this study was to focus on patients admitted due to CRS or NP, who were in need of otolaryngologic consultation or treatment. There is no standard treatment algorithm, as the severity of the disease is different for every patient, therefore, the treatment of each patient is considered individually (1, 2, 18).
Several diagnostic imaging techniques for CRS and NP in CF patients are used, the most important being endoscopy, CT, and MRI. Some authors have suggested that CT study is unable to reliably assess the severity of disease; therefore, the decision on surgical treatment should not be based solely on the results of the imaging studies (14). Patient’s medical history, their complaints, and the quality of life are also important factors that must be taken into account, considering the high individual variability of the severity of the disease in CF patients (1, 18). Only upon gathering both subjective and objective data can a proper treatment modality be considered (1). Prompt involvement of an interdisciplinary team including otolaryngologists, microbiologists and CF specialists is the key to early diagnosis and effective treatment (16).
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