Ann Thorac Surg 1997;64:852-854
© 1997 The Society of Thoracic Surgeons
Case Report
Severe Hemoptysis Requiring Lobectomy in an 11-Year-Old Patient With Kartagener's Syndrome
Helmut Schwarzenberg, MD,
Ronald J. Elfeldt, MD,
Erich Schlüter,
Johann Link, MD,
Martin Heller, MD
Departments of Radiology, General and Thoracic Surgery, and General Pathology, Christian-Albrechts-Universität zu Kiel, Kiel, Germany
Accepted for publication April 29, 1997.
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Abstract
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The case of a young girl with severe hemoptysis in a Kartagener's syndrome is described. Because bronchoscopy failed to locate the origin of the bleeding, preoperative angiography was performed. The patient was treated by resection of the middle lobe. The bronchus wall was located as the origin of bleeding.
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Introduction
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Kartagener's syndrome is characterized by the triad of bronchiectasis, sinusitis, and situs inversus. The case of a young girl with severe hemoptysis in a Kartagener's syndrome is reported along with the diagnostic and therapeutic implications.
An 11-year-old female patient suffering from Kartagener's syndrome presented with a sudden onset of recurrent massive hemoptysis at the Department of Surgery. On examination the girl had fever (38°C) and productive cough with hemoptysis. A hemoglobin concentration of 10.5 g/L and a peripheral leukocyte count of 12.2 x 109/L were found. Other routine biochemical tests gave normal results. Bronchoscopy revealed subtotal obstruction of the left middle lobe by compression from the outside. Severe bronchiectasis and purulent secretion were present throughout the lobe. The origin of the bleeding was not located. The biopsy specimen did not show malignancy. Partial atelectasis, thickening of the bronchial wall, and bronchiectasis with amounts of fluid in the left middle lobe were diagnosed (Fig 1A
). Additionally, mediastinal lymph node enlargement and a left prevertebral mass 3 cm in diameter, located directly at the origin of the left middle lobe bronchus and in extension to the origin of the left superior lobe bronchus, were revealed (Fig 1B).
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Fig 1. . (A) Computed tomographic scan shows atelectasis and bronchiectasis of the left middle lobe (arrows). Smaller infiltrations are located in the apical segment of the left lower lobe. (B) Mediastinal enlarged lymph nodes and a left prevertebral mass (T), 3 cm in diameter and located directly at the origin of the left middle lobe bronchus, are presented (arrows). Situs inversus thoracalis. (A = aorta.)
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The decision for explorative thoracotomy was made and preoperative digital subtraction angiography of the arterial and venous vessels of the chest was performed to locate the origin of the bleeding. Digital subtraction angiography revealed an area of small irregular arteries of the left middle zone, which originated from the left bronchial artery (Fig 2). Vessel transformations or arteriovenous malformations were not found.
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Fig 2. . Aorta thoracalis in right oblique projection. Note the area of small irregular arteries of the left middle zone (small arrows), which originated from the left bronchial artery (large arrows).
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Left thoracotomy and resection of the left middle lobe, the mediastinal lymph nodes, and the prevertebral mass were performed. During thoracotomy bleeding was located at the proximal bronchus and treated by ligation. The left middle lobe was resected. Histopathologic examinations showed massive bronchiectasis with acute and chronic bronchitis and peribronchial fibrosis. All of the mediastinal and prevertebral masses were reactively enlarged lymph nodes. Malignancy was not found.
The patient recovered well and was discharged 7 days after the operation in a good general condition. During 6-month follow-up, no recurrence of hemoptysis or pulmonary infection occurred. Lung function tests showed mild airways obstruction, and vital capacity was slightly decreased.
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Comment
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Although the first description was made by Siewert in 1904 [1], the triad of bronchiectasis, sinusitis, and situs inversus is known as Kartagener's syndrome, a name derived from the Swiss physician Manes Kartagener (1897–1975), who published 4 cases of this rare disorder in 1933 [2].
The clinical features of primary dyskinesia such as productive cough, pulmonary infections, sinusitis, otitis media, and infertility have been ascribed to the reduced clearance of mucus in the airways and sinuses and impaired motility of the sperm, respectively. Bronchiectasis and chronic infections may result in end-stage pulmonary disease with dyspnea and heart failure [3].
Like other patients suffering from Kartagener's syndrome, our patient had a long history of recurrent bronchial and pulmonary infections, sinusitis, and otitis from early childhood on. Clinical evidence of bronchiectasis with productive cough occurs in fewer than 10% of children aged less than 9 years, versus 75% in affected adult patients [4]. The most frequent symptom of bronchiectasis is a chronic and productive cough. Streaks of blood in the sputum are common. Serious hemoptysis may occur due to exacerbation of chronic bacterial infection with necrosis of the mucosa. The sputum cultures obtained from bronchoscopy were without evidence of bacteria in our patient. Neither clinical nor electrocardiographic signs of pulmonary hypertension, another possible cause of hemoptysis in patients with Kartagener's syndrome, were detected.
Here we report a case of recurrent onset of massive hemoptysis in a young patient with Kartagener's syndrome. Because repeated bronchoscopy did not reveal the location of bleeding and other parts of the lung were also afflicted by (less severe) bronchiectasis, aortography was performed. With the exception of an area of small irregular arteries of the left mid zone originating from the left bronchial artery, no areas suspicious for bleeding were found (see Fig 2
). Therefore embolization of the middle lobe arteries was not performed. Preoperative cavography was additionally performed to exclude possible vessel transformations or arteriovenous malformations of the pulmonary arteries. The decision for explorative thoracotomy was made because computed tomography and magnetic resonance imaging of the chest demonstrated enlarged mediastinal lymph nodes and a suspicious contrast-enhanced left-sided paravertebral mass with a middle lobe syndrome. Obviously, the location of bleeding was not reached by the bronchoscope due to compression of the bronchus. The chronic inflammation of the bronchus wall probably led to erosion of a bronchial artery branch.
In the era of physiotherapy and aggressive antibiotic therapy, surgical treatment as described by Mayo [5] is rarely reported in Kartagener's syndrome in the newer literature. Because repeated bronchoscopy failed in locating the origin of the severe hemoptysis, thoracotomy and resection of the afflicted lobe was the treatment of choice in our patient. However, resection of pulmonary lobes is the ultimate treatment of located chronic bronchiectasis refractory to conservative treatment. Preoperative aortography may play an important role in narrowing down the location of bleeding in case of severe hemoptysis.
In conclusion, physicians should be aware of the possible serious complication of severe hemoptysis in young patients with Kartagener's syndrome. Angiography should be considered as a potential diagnostic tool in such cases.
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Footnotes
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Address reprint requests to Dr Schwarzenberg, Klinik für Radiologische Diagnostik, CAU Kiel, Arnold-Heller-Straße 9, 24105 Kiel, Germany.
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References
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- Siewert A. Über einen Fall von Bronchiektasie bei einem Patienten mit Situs inversus viscerum. Berl Klin Wochenschr 1904;41:139–42.
- Kartagener M. Zur Pathogenese der Bronchiektasien: Bronchiektasien bei Situs inversus viscerum. Beitr Klin Tuberk 1933;83:489–501.
- Rossman CM, Forrest JB, Ruffin RE, Newhouse MT. Immotile cilia syndrome in persons with and without Kartagener's syndrome. Am Rev Respir Dis 1980;121:1011–6.[Medline]
- Nadel HR, Stringer DA, Levison H, Turner JP, Sturgess JM. The immotile cilia syndrome: radiological manifestations. Radiology 1985;154:651–5.[Abstract/Free Full Text]
- Mayo PO. Kartagener's syndrome. J Thorac Cardiovasc Surg 1961;42:39–42.
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Abstract
Introduction
