Hans J.M. Smit, MD; Ad J.M. Schreurs, MD; Jules M.M. Van den Bosch, MD; and Comelis J.J. Westerman, MD, FCCP
A retrospective study of 21 patients with primary ciliary dyskinesia (PCD) was done. Thirteen had prior resection of bronchiectasis and eight had not. Information about present complaints was obtained by a questionnaire. The prevalence of present respiratory symptoms was the same in both groups. The surgical patients had more severe disease and 85% of them considered the operation beneficial. Selected patients with PCD may have improved conditions with resection of bronchiectasis.
(CHEST 1996; 109:1541-44)
Keywords: bronchiectasis; immotile cilia syndrome; Kartagener syndrome; primary ciliary dyskinesia; thoracic surgery.
Abbreviations: PCD=primary ciliary dyskinesia; VC = vital capacity
Primary ciliary dyskinesia (PCD) is a rare group of diseases with a prevalence between 1 in 14500 and 1 in 34000 in both the United States and western Europe. These are autosomal recessive disorders with a structural abnormality of the dynein arms in the cilia causing dysfunction of ciliary and spermatozoal movement. Due to impaired mucociliary transport, bronchopulmonary and sinus infections frequently occur and may cause bronchiectasis.
Infertility is often present and complete or incomplete situs inversus occurs in 50%. The triad of situs inversus, sinusitis. and bronchiectasis is called Kartagener’s syndrome, named after the Swiss physician. The Dutch physician Siewert, however, was the first to describe the disorder in 1904. The structural abnormality in the cilia was first reported in 1976. In the past, bronchiectatic segments were usually resected. As the underlying disorder was clarified, the indication for resection was disputed, because PCD itself cannot be treated but in a palliative way. To our knowledge, no prospective studies about this problem are available nor are to be expected soon, because of the rarity of the disease and the length of time required for such studies. The aim of this retrospective study was to answer the question whether patients with PCD may benefit from resection of bronchiectasis.
Between 1952 and 1994, altogether 26 patients with PCD were seen in the two participating hospitals. Complete records of 24 patients were available for retrospective analysis. Three patients could not be interviewed by telephone because of language barrier, psychiatric problems, and living abroad. One of them underwent resection of bronchiectasis. Twenty-one patients gave written informed consent to be interviewed by telephone; they form the basis of this study. The diagnosis was based on the presence of the triad in Kartageners Syndrome in the earlier years in 8 patients and on ultrastructural abnormalities of nasal or bronchial cilia combined with functionally impaired mucociliary transport in 13 patients. The questionnaire concerned present complaints about daily cough, phlegm, hemoptysis,respiratoy infections, dyspnea, fitness for work, and the influence of resection on pulmonary complaints.
The records and bronchographic studies were reviewed with respect to the number of ectatic segments, number of thoracotomies and resected segments, histamine threshold, the latest (1991 through 1994) inspiratoy vital capacity (VC), and FEV. Statistical analysis was performed by means of the X2 and the Student’s t test.
Twenty-one patients could be evaluated, 13 with and 8 without resection.
Their characteristics are shown in Table 1.
|resection (n=13)||no resection (n=8)|
|Men. No. (%)||4 (31)||3 (37)|
|Age at present, yr. (range)||46 (32 .. 61)||46 (24 ..66)|
|Age at diagnosis, yr. (range)||16 (5 ..37)||25 (17 .. 38)|
|Year at diagnosis (range)||1965 (’50..’81)||1960 (’64 .. ’89)|
|Tobacco use present(%)||0||1 (13)|
|Stopped No. (%)||1 (8)||1 (13)|
|Never No. (%)||11 (92)||6 (75)|
|Present VC %predicted (range)||91 (70 .. 111)||100 (80 .. 118)|
|Present FEV % predicted range||70 (36 .. 91)||78 (64 ..104)|
|Histamine threshold positive No. (%)||1 (8)||2 (25)|
|Threshold unknown No. (%)||3 (23)||1 (13)|
|Dextrocardia No. (%)||8 (61)||1 (13)|
|No. of ectatic segments (range)||7.2 (2 .. 12.5)||4.9 (0 .. 12)|
|Bilateral bronchiectasis No. (%)||9 (69)||2 (25)|
Both groups are comparable regarding gender, age, smoking habits, pulmonary function, and histamine threshold. The VC and FEV as percentage of predicted normal values of the operated-on patients are slightly smaller, but the predicted values are not corrected for resected segments. The difference in the year of diagnosis of the patients with resection (1965) and those without (1980) is the result of the doubt about the validity of resection that arose in that period. The only significant difference betveen both groups is the high prevalence of dextrocardia in the operated-on patients. PCD was diagnosed in these patients between 1950 and 1981, when ciliary examination was not commonly done or even not possible and dextrocardia often was the main clue to the diagnosis. The younger age at diagnosis, the greater number of ectatic segments, and the higher prevalence of bilateral disease in the operated-on group are not statistically significant. Patients with dextrocardia had more bronchiectatic segments than those without situs inversus.
The resections were carried out between 1950 and 1984. The youngest patient was 5 years of age and the oldest was 50 years. The side and extent of the resection of ectatic segments are shown in Table 2.
|No. of resected segments|
|3 .. 4||3|
|5 .. 6||4|
|7 .. 8||3|
|9 .. 10||2|
Only 4 of 13 patients had 1 thoracotomy. Three had two thoracotomies on one side and six had two thoracotomies for bilateral disease. No patient underwent simultaneous bilateral resection. The time between 2 thoracotomies was on the average 8 years ( 1 month to 28 years). Three patients underwent extensive resection of more than eight segments. The mean number of resected segments was 6.6 whereas preoperatively a mean of 7.2 ectatic segments were found. Therefore not all diseased segments were resected.
The present complaints of the patients with and without resection as obtained by the questionnaire are shown in Table 3. There is no statistical difference in the prevalence of daily cough and phlegm, hemoptysis, dyspnea, hospitalization for pulmonary reasons, and ability to work. The hospitalization rate for the entire population is low with a mean of 0.38 admissions per patient in the preceding 5 years. However, 29% of the entire group was considered unfit for work.
|No Resection (%)
|0 .. 1||3(23)||2(25)|
|3 .. 4||3(23)||2(25)|
|Ability to work|
|not willing %||2(15)||0|
Table 4 presents the opinion of the patients about the influence of the resection of ectatic segments on pulmonary symptoms. Productive cough was considered to be improved by 10 patients (77% ) and this improvement lasted for more than 10 years in 7 (54%). Four patients had hemoptysis before the operation and only one of them afterwards, but less severe. Respiratory infections became less frequent in 7 patients (69%), but this effect lasted for more than 10 years in only 5 (39%). Dyspnea improved in 6 patient (46%) after resection. Five ( 38% ) observed no change of: 3 of these had no dyspnea either before or after the operation.
In general, 11 ( 85% ) of the patients felt better after the operation. Two patients were not satisfied. One had 5 segments resected, recurrence of bronchiectasis after 20 years, and asthma. Her FEV was 81% of the predicted value. The other patient had resections of 9.5 segments. Although bronchiectasis did not recur, her complaints became worse. She had no asthma and her postoperative uncorrected FEV was 65% of the predicted value.
|Questionnaire||No (%) of patients
|Daily cough and phlegm|
|Lasting > 10 yr||7 (54)|
|Before resection||4 (31)|
|After resection||1 (8)|
|Respiratory tract infections|
|Lasting > 10 yr||5 (39)|
|Lasting > 10 yr||2 (15)|
|Very satisfied||5 (39)|
|Not satisfied||2 (15)|
The prevalence of present respiratory complaints and fitness for work is the same in patients who had a resection of bronchiectatic segments and those without an operation. The beneficial influence of resection is therefore not apparent. It seems possible, however, that the surgical patients had more serious complaints before the operation because they had more severe and also more bilateral bronchiectasis. This cannot be proved in a retrospective study. The younger age at diagnosis in the surgical group may also point to a greater severity of complaints, but the young age can also be explained by the high prevalence of dextrocardia, which easily raises suspicion of bronchiectasis and thus facilitates the diagnosis. In fact, the diagnosis of PCD was established at the age of 14 years in the patients with dextrocardia and at the age of 19 years if no dextrocardia was present.
The assumption that the surgical patients initially had more complaints is supported by the fact that 85% of the operated-on patients had noticed beneficial influence of the resections. This was most apparent regarding daily cough and phlegm, hemoptysis, and respiratory infections.
Due to the rarity of PCD , the present study concerns only 21 patients. Yet, in our opinion, it certainly cannot be concluded from the above observations that PCD is a contraindication for resection of bronchiectasis. Patients subjectively benefit from resection.
Resection causes considerable morbidity, particularly when bilateral disease is present. Nine (69%) of the operated-on patients had 2 thoracotomies; no simultaneous bilateral resection was performed. The resections were rather extensive with an average of 6.6 segments per patient. Three patients had a resection of nine or more segments, a functional pneumonectomy, and not all ectatic segments were always removed.
Yet, pulmonary function 29 years after the operation is remarkably good witth a VC of 91 % of the predicted value. This might be caused by the young age at which bronchiectasis developed and resection was performed. The influence of surgery on dyspnea is equivocal as might be expected when nonfunctional segments are removed.
PCD is a disorder affecting mucociliary clearance of the lungs. These patients are therefore prone to respiratory tract infections and development of bronchiectasis. At present, a correct diagnosis can be made with help of nasal biopsy specimens. Screening for bronchiectasis can now easily be done with high-resolution CT. Early diagnosis, modem treatment to improve mucociliary clearance such as inhalation therapy, and proper use of antibiotics may prevent formation of bronchiectasis.
However, if bronchiectasis has developed, resection is a therapeutic option in the properly selected patient. Unilateral or bilateral localized bronchiectasis is a good indication, when frequent febrile episodes or severe hemoptysis is present, despite more conservative measures, such as inhalation therapy, positional drainage, oral or IV antibiotics, and embolotherapy
- Baan S van der, Primaire ciliaire dyskinesie [dissertation], Amsterdam: Free Uniyersity 1985: 9-25
- Afzelius B, A human syndrome caused by immotile cilia, Science 1976: 196:317-19
- Eliasson R, Mossberg B, Camner P, et al, The immotile cilia syndrome: a congenital ciliary abnormality as an etiologic factor in chronic airway infections and male sterility. N EnglJ Med 1977: 297:I-6
- Mygind N, Nielsen MH. Pedersen M, Kartageneis syndrome abnormal cilia, Eur J Respir Dis 1983: 64(suppl 127):11-30. 144-47
- Munro NC, Cunie DC, Lindsay KS, et al, Fertility in men with primary ciliary dyskinesia presenting with respiratory infections, Thorax 1994: 49:684-87
- Halbert SA, Tam PY, Blandau RJ, Egg transport in the rabbit oviduct: the roles of cilia and muscle, Science 1976: 191:1052-53
- Young D, Surgical treatment of male infertility, J Reprod Fertil 197O; 23:541-42
- Afzelius BA. Camner P, Mossberg B. On the function of cilia in the female reproductive tract. Fertil Steril 1978; 29:72-4
- Kartagener M, Zur pathogenese der bronchiektasien: I, Bronchiektasien bei Situs viscerum inversus. Beitr Klin Tub 1933: 83:489-501
- Schidlow DV ..Primary ciliairy dyskinesia (the immotile cilia syndrome). Ann Allergy 1994: 73:457-68 .
- Siewert AK. Ueber einen fall von bronchiektasie bei einem patienten mit inversus viscerum. Berl Klin Wochenschr 1904. 41.139-41
- Report of the Worling Party. Standardisation of lung function tests. Bull Eur Physiopathol Respir 1983: 19 suppl.5
- Mahler DA, Wells CK. Evaluation of clinical methods for rating dyspnea. Chest 1988: 93:580-86
- Laros CD. Westemann CJJ. Dillatation. compensatory growth or both after pneumonectomy. during childhood and adolescence a 30 year follow up J Thorac Cardiovasc Surg 1987 93:570-76
> From the Departments of Pulmonary Diseases. St. Antonius Hospital (Drs. Smit, Van den Bosch, and Westerman), Nieuwegein, the Netherlands, and OLVG (Dr, Schreurs), Amsterdam, the Netherlands. Manuscript received July 6, 1995; revision accepted January 15, 1996, – Reprint requests: CJJ Westennann, St. Antonius Hospital, PB 2500, 3430 EM, Nieuwegein. the Netherlands