Respiratory failure and pulmonary hypertension associated with Klippel-Feil syndrome.
A 28-year-old woman with a deformed thorax and kyphoscoliosis associated with Klippel-Feil syndrome developed respiratory failure with pulmonary hypertension. Pulmonary 133Xe ventilation and 99mTc-MAA perfusion scintigraphies showed maldistributions of lung ventilation and perfusion, and noticeably delayed 133Xe washout from the lungs. Dynamic breathing MR imaging showed poor and/or asynchronous respiratory movements of the chest wall and diaphragm. These findings indicate that the perfusion-ventilation imbalance, the decreased ventilatory turnover, and expiratory flow from the alveolar space partly derived from the impaired respiratory mechanics may be responsible for the respiratory complications in this patient.
Suga
K
K
Department of Radiology, Yamaguchi University School of Medicine, Ube, Japan.
Motoyama
K
K
Hara
A
A
Kume
N
N
Matsunaga
N
N
Kametani
R
R
Matsuzaki
M
M
D000328:Adult; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology*; D007714:Klippel-Feil Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008168:Lung / Q000000981:diagnostic imaging*; D010102:Oxygen Inhalation Therapy; D013902:Radiography, Thoracic; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals / Q000493:pharmacokinetics; D012129:Respiratory Function Tests; D012131:Respiratory Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology*; D015656:Respiratory Mechanics; D013668:Technetium Tc 99m Aggregated Albumin / Q000493:pharmacokinetics; D014980:Xenon Radioisotopes / Q000493:pharmacokinetics
D019275:Radiopharmaceuticals; D013668:Technetium Tc 99m Aggregated Albumin; D014980:Xenon Radioisotopes