Primary ciliary dyskinesia surgery
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Hafsa Ghaffar, M.B.B.S[2]
Overview
Chronic rhino-sinusitis can predispose to superinfections with Pseudomonas aeruginosa in patients with primary ciliary dyskinesia PCD, endoscopic sinus drainage could facilitate sinus drainage. There is no evidence that suggests the insertion of tympanostomy tubes in cases of recurrent otitis media or otitis media with effusion therefore, regular doctor appointments should be scheduled in order to diagnose conductive hearing loss.
Surgical Treatment
Increased nasal secretions are common in children with PCD. Nasal polyps are reported frequently with improvement with the use of Saline nasal douches and anticholinergic therapy often to treat symptoms, but again there is no evidence base to support their use. Otolaryngology consult consultation for surgery and polypectomy in refractory cases could be performed to facilitate sinus drainage[1]. It has been suggested that some patients with chronic rhinosinusitis may benefit from long-term macrolide therapy and from endoscopic sinus surgery in recalcitrant disease. Chronic rhinosinusitis (CRS) and bacterial sinusitis are troublesome in patients with primary ciliary dyskinesia (PCD). Pseudomonas aeruginosa is found in most of the cultures in these patients, which can cause serious lung infections. CRS-related symptoms after Endoscopic Sinus Surgery and adjuvant therapy show improvement in symptoms. In selected PCD patients, the suggested regimen may delay chronic lung infection with Psuedomonas aeruginosa and stabilize lung function. Around 85% of the children with PCD have chronic and occasionally severe otitis media with effusion. Hearing problems associated with this usually improve spontaneously in the early teenage years. However, hearing loss and otological problems may also be seen later in adulthood. There is inadequate evidence on the management of otitis media with effusion in PCD patients, otitis media with effusion is usually managed conservatively with hearing aids, as treatment with tympanostomy often results in prolonged and unpleasant otorrhoea.[2][3] [4][5][6]
Lung Surgery
In case of severe systemic symptoms such as frequent exacerbations or life-threatening hemoptysis refractory to medical therapy, lobectomy may be considered in areas of localized lung disease. Patients in such situations have undergone successful resection but there is no long-term data available Often the diffuse nature of disease elsewhere in the lung alleviates against the possibility of success in removing more diseased parts of the lung.[7]
Lung Transplant
PCD patients undergoing double lung transplantation usually have good survival outcomes. Multiple drug-resistant organisms, poor nutritional status are the usual concerns that pertain to candidacy for the procedure. Interestingly, patients with situs inversus, do not pose any additional risk to post-transplant outcomes, it could be challenging but not contraindicated.[8]
References
- ↑ Barbato A, Frischer T, Kuehni CE, Snijders D, Azevedo I, Baktai G, Bartoloni L, Eber E, Escribano A, Haarman E, HesselmarB, Hogg C, Jorissen M, Lucas J, Nielsen KG, O'Callaghan C, Omran H, Pohunek P, Strippoli MP, Bush A Eur Respir J. 2009 Dec; 34(6):1264-76.
- ↑ Majithia A, Fong J, Hariri M, et al. Hearing outcomes in children with primary ciliary dyskinesia – a longitudinal study. Int J Pediatr Otorhinolaryngol 2005; 69: 1061–1064.CrossRefPubMedGoogle Scholar
- ↑ Campbell R. Managing upper respiratory tract complications of primary ciliary dyskinesia in children. Curr Opin Allergy Clin Immunol 2012; 12: 32–38.PubMedGoogle Scholar
- ↑ Campbell RG, Birman CS, Morgan L. Management of otitis media with effusion in children with primary ciliary dyskinesia: a literature review. Int J Pediatr Otorhinolaryngol 2009; 73: 1630–1638.CrossRefPubMedGoogle Scholar
- ↑ Bush A, Cole P, Hariri M, et al. Primary ciliary dyskinesia: diagnosis and standards of care. Eur Respir J 1998; 12: 982–988.AbstractGoogle Scholar
- ↑ Hadfield PJ, Rowe-Jones JM, Bush A, et al. Treatment of otitis media with effusion in children with primary ciliary dyskinesia. Clin Otolaryngol Allied Sci 1997; 22: 302–306.CrossRefPubMedGoogle Scholar
- ↑ Smit HJ, Schreurs AJ, Van den Bosch JM, Westermann CJ. Is resection of bronchiectasis beneficial in patients with primary ciliary dyskinesia? Chest. 1996;109:1541–4. [PubMed] [Google Scholar]
- ↑ Christie JD, Edwards LB, Kucheryavaya AY, et al. The Registry of the International Society for Heart and Lung Transplantation: 29th adult lung and heart-lung transplant report-2012. The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation. 2012;31:1073–86. [PubMed] [Google Scholar]