Nasal polyps

Question

Dear expert team,
Our daughter - 3.5 years old- has been diagnosed with CF in december last year. Her main problem are nasal polyps. These have been operated in december last year (removal of a massive endonasal polyposis, preparation of a large window in the maxillary sinus with the middle nasal duct and removal of polypoid material from the maxillary sinus). 4 to 6 weeks after the operation the polyps reoccured in the original extent. Since then breathing through the nose is totally excluded. Until now the treating physicians have not been able to cope with this problem. Our daughter is treated for about 4 weeks with Triamcinolon nasal spray. No improvement until now. We do not want a further operation been done to our daughter at the moment (if possible). We are afraid, that also a second operation would not be sucessful for a longer period of time. Which kind of therapeutic options are there yet resp. could you propose to us? Are there any specialists in the area of Krefeld (Germany) who are specialized in problems with nasal polyps of CF patients or who are experienced?
Many thanks in advance.

Answer

Dear family,

in case of CF even in young children it can come to extensive nasal polyps. These have to be differentiated from the adenoid vegetations (pharyngeal tonsil) often wrongly called "polyps".
Vegetations of the paharyngeal tonsil as a sign of activation of the lymphatic system, play a great role in young children with an occlusion of the upper airways and are a more frequent reason for ENT-operations.
Real polyps are glazed grey vegetations of the mucosa, which often come out of the nasal sinuses and hereby, like also in your daughter, are capable of completely obstruct the nasal ducts. They do occur in about 6% of the whole population and here mostly in adult age. In contrast, up to 40% of CF patients are affected. Also here, more CF adluts suffer from polyps, but also little children can have them, sometimes also as a first symptom, that leads to the diagnosis of CF.
The removal of real polyps in CF patients it followed by high rates of relapse because by an operation the chloride channel defect of the mucosa is not cured. Especially high rates of relapse occur, if only a removal of the outcoming polyps is done.
In case of your daughter, extensive operations in the nasal sinuses with removal of the polyps "with the roots" have been done and the fast and extensive relapse is uncommon in this extension. We try to prevent this with the usage of topic steriods (low dose cortisone as nasal spray) from the early phase of the operation on and we have the impression, that this is frequently successful. Triamcinolon belongs to this group and it has obviosuly not helped your daughter.
The fast and so extensive reoccurence in your young daughter is unusual. I will discuss it with ENT-collegues and give a second feedback.
Yours sincerely,
Dr. J. Mainz

25.10.11
Here comes my second amended answer:
I asked in the meantime two ENT-specialists with experience in CF (Dr. Kasper Aenaes from the CF-center of the Rigshospital in Copenhagen, Denmark and Prof. Ingo Baumann, ENT clinic of the university of Heidelberg, Germany).
Both specialists said, that they do not have any other idea rather than a new and more extended operation (clearing of the sinus ethmoidales, from which the nasal polyps often grow out....). I performed a search of the literature, which did not result in any promising solutions for this question. Admittedly Ibuprofen, Azithromycin, systemic cortisone and others have been discussed, however, never with uncritical success.
Yours sincerely,
Dr. J. Mainz

10.10.2011