Ann Thorac Surg 2001;71:2008-2009
© 2001 The Society of Thoracic Surgeons
Case report
Living-donor lobar lung transplantation for primary ciliary dyskinesia
Hiroshi Date, MDa,
Motohiro Yamashita, MDa,
Itaru Nagahiro, MDa,
Motoi Aoe, MDa,
Akio Andou, MDa,
Nobuyoshi Shimizu, MDa
a Department of Surgery II, Okayama University School of Medicine, Okayama, Japan
Accepted for publication April 4, 2000.
Address reprint requests to Dr Date, Department of Surgery II, Okayama University School of Medicine, 2-5-1 Shikata Cho, Okayama 700-8558, Japan
e-mail: hdate{at}nigeka2.hospital.okayama-u.ac.jp
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Abstract
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A ventilator-dependent patient with primary ciliary dyskinesia underwent successful living-donor lobar lung transplantation. The case was a 24-year-old woman who had developed recurrent lower respiratory infection and became ventilator-dependent due to severe bronchiectasis. Transmission electron microscopy of the resected bronchus demonstrated inner dynein arm deficiency.
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Introduction
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Primary ciliary dyskinesia (PCD) is a rare disease which is characterized by systematic ciliary transport failure. The clinical picture is of lifelong sinusitis and recurrent bronchial infection, but the spectrum is broader than that encompassed by Kartagener’s syndrome (dextrocardia, sinusitis, and bronchiectasis). We report a case of PCD with normal situs treated by lung transplantation.
In September 1998, a 24-year-old female with PCD began having severe respiratory insufficiency and right heart failure after multiple episodes of respiratory infection. She was diagnosed with PCD at the age of 12 years by samples of bronchial cilia obtained bronchoscopically. Her pulmonary function test in July 1998 showed a vital capacity of 0.9 L (30% of predicted) and forced expiratory volume in 1 second of 0.48 L (19% predicted). Chest x-ray film revealed normal cardiac situs and diffuse bilateral opacification due to generalized bronchiectasis. Computed tomography (CT) scan of the chest showed severe saccular bronchiectasis (Fig 1). Antral mucosal thickening was present on CT scan of the sinus, however, no evidence of active sinusitis was detected. She had a bacterial colonization of the tracheobronchial tract with strains of Pseudomonas aeruginosa and Staphylococcus epidermidis, managed with antibiotics therapy. On October 13, she required mechanical ventilation and a tracheostomy. Right-sided heart failure was observed with pulmonary artery pressure of 65/35 (47) mmHg. In spite of maximal mechanical ventilation, arterial carbon dioxide tension reached 150 mmHg on October 28, at which time she underwent bilateral living-donor lobar transplantation with her sister’s right lower lobe and her mother’s left lower lobe under cardiopulmonary bypass. The surgical and logistic aspects of the right and left donor lobectomy, the donor lobe back-table preservation technique, and the recipient bilateral pneumonectomy and bilateral lobar implantation have been previously described by Starnes’ group [1].
Except for a short lung edema episode requiring nitric oxide inhalation on the 2nd postoperative day, the further course was relatively uneventful. Postoperative immunosuppression was a triple drug therapy consisting of cyclosporine, azathioprine, and prednisone. The patient was completely weaned from the respirator within 18 days. Two episodes of acute rejection required high-dose methylprednisolone intravenously. The tracheostomy was closed 6 weeks postoperatively, and her pulmonary artery pressure became normal; 30/13 (20) mm Hg. The patient was discharged from the hospital 61 days after transplantation. Five months postoperatively, she has returned to normal life and is able to carry out daily activities. She is in good physical condition with a vital capacity of 1.79 L (60% predicted), forced expiratory volume in 1 second of 1.5 L (54% predicted), and arterial oxygen tension of 95 mmHg at room air.
Transmission electron microscopy was carried out using recipient’s removed bronchus. Groups of cilia were studied in detail. Cilia cut in near perfect cross section showed inner dynein arm deficiency (Fig 2). The outer arms were normal.
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Fig 2. A cilium from the bronchial mucosa of the patient. Inner dynein arms are absent by electron micrograph. Original magnification, x275,000 (uranyl acetate and lead citrate double stain).
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Comment
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There has been growing appreciation of the significant role played by the mucociliary transport system in the body. The mucociliary transport system is an important defense mechanism by which the human body usually maintains its homeostasis by protecting the body against invading particles, including bacteria. This system includes two major functional mechanisms, mucous secretional and ciliary transport systems, each of which is usually complementary and cooperative. Cystic fibrosis (CF) is associated with an abnormality in the secretional transport system, and primary ciliary dyskinesia (PCD) is characterized by systematic ciliary transport failure [2, 3]. The clinical picture of both diseases is similar: lifelong sinusitis and recurrent bronchial infection. CF has been the fourth most common indication for bilateral lung transplantation [4], and most common indication for living-donor lobar lung transplantation [5]. In contrast, PCD has been rarely treated by lung transplant.
About half of the cases of PCD can be classified as Kartagener’s syndrome (dextrocardia, sinusitis, and bronchiectasis). Successful heart-lung [6, 7], en bloc double lung [8], and bilateral lung [9] transplants have been reported for this syndrome. However, we failed to find any previous reports of lung transplantation for PCD with normal situs. The diagnostic difficulties still occur in PCD. Greenstone and colleagues [3] reported that all patients with PCD fulfilled at least the following criteria for in vitro abnormalities: observation of ciliary dyskinesia, reduced numbers of beating cilia, ciliary beat frequency at least two standard deviations from the mean of normal control values, and ultrastructural abnormalities in at least one-third of the cilia examined. In the present case, inner dynein arm deficiency was clearly shown by ultrastructural study of the recipient’s removed bronchus. One hundred and sixty-nine patients with bronchiectasis have undergone bilateral lung transplantation according to St. Louis International Lung Transplant Registry in 1997. We suspect that there might be some unrecognized patients with PCD among them.
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Acknowledgments
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We acknowledge Professor Yuji Ohtsuki, Department of Pathology II, Kochi Medical University, for his electron micrograph courtesy.
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References
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Starnes V.A., Barr M.L., Cohen R.G., et al. Living-donor lobar lung transplantation experience: intermediate results. J Thorac Cardiovasc Surg 1996;112:1284-1291.[Abstract/Free Full Text]
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Miralles A., Muneretto C., Gandjbakhch I., et al. Heart-lung transplantation in situs inversus. A case report in a patient with Kartagener’s syndrome. J Thorac Cardiovasc Surg 1992;103:307-313.[Abstract]
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