Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report
Cystic fibrosis is an autosomal recessive genetic disorder that damages the exocrine function of the body, resulting in alterations of multiple organs. In the respiratory system, it is known to cause bronchiectasis, recurrent bronchitis, and pneumonia; however, to the best of our knowledge, there ar...
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| Format: | Article |
| Language: | English |
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Elsevier
2023-03-01
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| Series: | Radiology Case Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S1930043322010925 |
| _version_ | 1811176170529488896 |
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| author | Orito Ikeda, MD Kunihiko Shimizu, MD Yoshitake Yamada, PhD Hiroaki Sugiura, MD Hideaki Suzuki, MD Syuichiro Umetsu, MD Kozo Sato, MD Masahiro Jinzaki, PhD |
| author_facet | Orito Ikeda, MD Kunihiko Shimizu, MD Yoshitake Yamada, PhD Hiroaki Sugiura, MD Hideaki Suzuki, MD Syuichiro Umetsu, MD Kozo Sato, MD Masahiro Jinzaki, PhD |
| author_sort | Orito Ikeda, MD |
| collection | DOAJ |
| description | Cystic fibrosis is an autosomal recessive genetic disorder that damages the exocrine function of the body, resulting in alterations of multiple organs. In the respiratory system, it is known to cause bronchiectasis, recurrent bronchitis, and pneumonia; however, to the best of our knowledge, there are no reported cases of pulmonary arteriovenous malformations associated with this disease. Herein, we report a case of cystic fibrosis with multiple pulmonary arteriovenous malformations. A 16-year-old girl, who has been monitored since childhood for pancreatitis of unknown cause, experienced respiratory symptoms and hypoxemia (PaO2 = 57 mmHg). At 13 years of age, chest computed tomography revealed bronchiectasis, bronchial wall thickening, and tree-in-bud sign. Genetic testing was performed, and the patient was diagnosed with cystic fibrosis. However, the computed tomography scan also showed incidental nodular lesions in the left superior and both the inferior pulmonary lobes, suggesting multiple arteriovenous malformations. Dynamic computed tomography was performed which, confirmed the presence of 3 pulmonary arteriovenous malformations. Coil embolization was performed on all lesions, and the hypoxemia was corrected. Marked hypoxemia in a patient with cystic fibrosis may not be explained only by the presence of bronchiectasis and/or bronchial wall thickening; in such cases, it may be necessary to examine possible additional findings on computed tomography images, such as arteriovenous malformations. |
| first_indexed | 2024-04-10T19:47:55Z |
| format | Article |
| id | doaj.art-e7db19cb7cc343a38bfed863d8d645b2 |
| institution | Directory Open Access Journal |
| issn | 1930-0433 |
| language | English |
| last_indexed | 2024-04-10T19:47:55Z |
| publishDate | 2023-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Radiology Case Reports |
| spelling | doaj.art-e7db19cb7cc343a38bfed863d8d645b22023-01-29T04:20:22ZengElsevierRadiology Case Reports1930-04332023-03-0118310331036Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case reportOrito Ikeda, MD0Kunihiko Shimizu, MD1Yoshitake Yamada, PhD2Hiroaki Sugiura, MD3Hideaki Suzuki, MD4Syuichiro Umetsu, MD5Kozo Sato, MD6Masahiro Jinzaki, PhD7Department of Radiology, Saiseikai Yokohama-shi Tobu Hospital, Shimosueyoshi 3-6-1, Tsurumi-ku, Yokohama-shi, Kanagawa 230-8765, JapanDepartment of Radiology, Saiseikai Yokohama-shi Tobu Hospital, Shimosueyoshi 3-6-1, Tsurumi-ku, Yokohama-shi, Kanagawa 230-8765, Japan; Corresponding author.Department of Radiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, JapanDepartment of Radiology, National Defense Medical College Hospital, Namiki 3-2, Tokorozawa-shi, Saitama 359-8513, JapanDepartment of Radiology, Saiseikai Yokohama-shi Tobu Hospital, Shimosueyoshi 3-6-1, Tsurumi-ku, Yokohama-shi, Kanagawa 230-8765, JapanDepartment of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohama-shi Tobu Hospital, Shimosueyoshi 3-6-1, Tsurumi-ku, Yokohama-shi, Kanagawa 230-8765, JapanDepartment of Radiology, Saiseikai Yokohama-shi Tobu Hospital, Shimosueyoshi 3-6-1, Tsurumi-ku, Yokohama-shi, Kanagawa 230-8765, JapanDepartment of Radiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, JapanCystic fibrosis is an autosomal recessive genetic disorder that damages the exocrine function of the body, resulting in alterations of multiple organs. In the respiratory system, it is known to cause bronchiectasis, recurrent bronchitis, and pneumonia; however, to the best of our knowledge, there are no reported cases of pulmonary arteriovenous malformations associated with this disease. Herein, we report a case of cystic fibrosis with multiple pulmonary arteriovenous malformations. A 16-year-old girl, who has been monitored since childhood for pancreatitis of unknown cause, experienced respiratory symptoms and hypoxemia (PaO2 = 57 mmHg). At 13 years of age, chest computed tomography revealed bronchiectasis, bronchial wall thickening, and tree-in-bud sign. Genetic testing was performed, and the patient was diagnosed with cystic fibrosis. However, the computed tomography scan also showed incidental nodular lesions in the left superior and both the inferior pulmonary lobes, suggesting multiple arteriovenous malformations. Dynamic computed tomography was performed which, confirmed the presence of 3 pulmonary arteriovenous malformations. Coil embolization was performed on all lesions, and the hypoxemia was corrected. Marked hypoxemia in a patient with cystic fibrosis may not be explained only by the presence of bronchiectasis and/or bronchial wall thickening; in such cases, it may be necessary to examine possible additional findings on computed tomography images, such as arteriovenous malformations.http://www.sciencedirect.com/science/article/pii/S1930043322010925Cystic fibrosisPulmonary arteriovenous malformationsHypoxemiaDynamic CT |
| spellingShingle | Orito Ikeda, MD Kunihiko Shimizu, MD Yoshitake Yamada, PhD Hiroaki Sugiura, MD Hideaki Suzuki, MD Syuichiro Umetsu, MD Kozo Sato, MD Masahiro Jinzaki, PhD Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report Radiology Case Reports Cystic fibrosis Pulmonary arteriovenous malformations Hypoxemia Dynamic CT |
| title | Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report |
| title_full | Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report |
| title_fullStr | Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report |
| title_full_unstemmed | Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report |
| title_short | Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report |
| title_sort | cystic fibrosis with multiple pulmonary arteriovenous malformations a case report |
| topic | Cystic fibrosis Pulmonary arteriovenous malformations Hypoxemia Dynamic CT |
| url | http://www.sciencedirect.com/science/article/pii/S1930043322010925 |
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