Rhinosinusosurgery in children with cystic fibrosis

Cystic fibrosis (CF) is a monogenic hereditary disease caused by a mutation of the cystic fibrosis transmembrane regulator (CFTR) gene and characterized by exocrine dysfunction and multi-organ lesion. Chronic rhinosinusitis (CRS), mainly purulent-polypoid, is one of the CF manifestations. Paranasal sinuses are considered as a main reservoir of pathogenic flora and as a source of descending bronchial and lung infection. Surgery sometimes become a first-step treatment. It makes possible to deliver topical drugs. Until now, there are no any standard surgical approaches. Aim. To optimize and standardize of rhinosinusosurgery for CRS in children with CF. Methods. Forty-three patients aged 2 to 17 years (average age 7.9±3.73 years) operated in the period from 2018 to 2020 were included. For all patients standardized protocol for the maxillary sinus surgery (removal of almost the entire medial wall and the whole mucosa of the maxillary sinus in a single block, anterior ethmoidotomy) was performed. Surgical tactics concerning other paranasal sinuses was determined by computed tomography (CT) data. In three patients there was a secondary lacrimal stenosis caused by polypoid transformation of the anterior ethmoid. The children were divided into 2 groups according to the CRS subphenotype: 11 (25.6%) patients with pseudomucopyocele of one or both maxillary sinus and 32 (74.4%) patients with purulent-polypoid CRS with nasal polyposis stage II– III (by Johansen). The age of the patients, the surgery duration and site, cases of poorly controlled intraoperative bleeding were evaluated. The effectiveness of treatment was assessed by the need for re-operation in the period of follow-up from 6 to 34 months. Results. In pseudomucopyocele group (age 4.2±1.54 years) only endoscopic modified maxillectomy and anterior ethmoidectomy were performed according to protocol mentioned above. In group of children with purulent-polypoid CRS (age 9.4±3.65 years) surgery besides mentioned included posterior ethmoidectomy in 27 children, sphenoidectomy – 22, frontotomy – 8, adenoidectomy – 5, septoplasty – 4. In 36 (86%) of patients the suggested technique enabled to remove mucosa fully as fibreoptic control showed.

Surgery duration had a significant difference (43±12.1 and 103±26.2 minutes, p=0.044) in two groups. In 3 patients successful endoscopic dacryocystorhinostomy was performed. Poorly controlled intraoperative bleeding cases correlated with an absence of vitamin K injections in preoperative treatment scheme (4 non-treated with vitamin K patients from 7 bleeding cases). One (2.3%) patient needed re-surgery during follow-up period. Conclusion. Division of CRS in CF into two subphenotype seems to be efficient in terms of surgery planning. Nevertheless the average age difference between the groups can suspect that such a subphenotypes are only the stages of the disease. The suggested technique of modified medial maxillectomy with whole mucosa removal is a way to prevent an early need for re-surgery and on our mind is more effective than standard FESS we published before. In presence of indications, sinus-surgery can be complemented with adenoidectomy, septoplasty. For the first time we described endoscopic dacryocystorhinostomy in children with CF. Cases of poorly controlled intraoperative bleeding once more time showed the necessity of vitamin K in preoperative treatment scheme. Simultaneous rhinosurgery steps performing is rational because of reducing of number and total duration of anesthesia in patients with potentially compromised lung and liver function. The groups are still under the prolonged follow-up to evaluate the CRS treatment effectiveness in children with CF in long-term period.

Key words: cystic fibrosis, chronic rhinosinusitis, pseudomucopyocele, endoscopic sinus surgery, septoplasty, dacryocystorhinostomy, vitamin
Conflicts of interest. There are no any conflicts of interest

Funding. There was no funding for this study.