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(Circulation. 2001;103:1031.)
© 2001 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Chylous Bronchial Casts After Fontan Operation
From University Children’s Hospital (M.I.H., M.M., R.B., S.F., U.B.) and University Hospital (J.E.), Zurich, Switzerland.
Correspondence to Dr Maja Isabel Hug, University Children’s Hospital, Steinwiesstr 75, CH-8032 Zurich, Switzerland. E-mail Maja.Hug{at}kispi.unizh.ch
A 4-year old boy with D-transposition of the great arteries, pulmonary atresia with intact ventricular septum, and a hypoplastic left ventricle with mitral atresia underwent a Fontan operation. He had previously been treated with neonatal balloon atrial septostomy followed by a left modified Blalock-Taussig shunt and, at the age of 2 years, a Hemi-Fontan operation was performed. The Fontan operation was completed with an extracardiac conduit (Goretex) from the inferior vena cava to the superior vena cava. High central venous pressures and bilateral chylothoraces complicated the postoperative course. Despite 6 weeks of conservative treatment with fat-free nutrition and subsequent total parenteral nutrition, pleural fluid loss remained excessive, and a pleurodesis was performed bilaterally. A few days postoperatively, acute respiratory failure occurred due to bronchial casts. Cast analysis showed high triglyceride and protein concentrations (cast triglyceride, 0.96 mmol/L; cast protein, 66 g/L; serum triglyceride, 0.36 mmol/L; and serum protein, 47 g/L). Bronchoscopic attempts to remove the bronchial casts failed, and the child died of cardiorespiratory failure.
At autopsy, the bronchial system was completely plugged by
casts resembling bronchial tree anatomy
(Figure 1
). Thromboses obliterated the left innominate vein
partially and reduced the lumen of the extracardiac conduit by 50%.
These thromboses occurred despite full anticoagulation and were
not detected by previous transthoracic echocardiography. Histopathology
demonstrated massively dilated pulmonary lymph vessels within the whole
lung
(Figure 2). Ruptures of lymphatic vessels and the influx of
chyle into the alveoli could clearly be demonstrated
(Figure 3). The material in the alveoli was proven to be
chyle by lipid staining
(Figure 4). The mechanism of lymphatic leakage leading to the
formation of bronchial casts has been
discussed,1 but it had not
been demonstrated histologically before. This condition must be
distinguished from mucus hypersecretion as an unusual response of the
pulmonary epithelium to elevated venous pressure (classified as type II
or as acellular mucin casts by Seear et
al),2 where no alveolar
flooding with chyle is present.
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Footnotes
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
References
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Languepin
J, Scheinmann P, Mahut B, et al. Bronchial casts in children with
cardiopathies: the role of pulmonary lymphatic abnormalities.
Pediatr Pulmonol. 1999;28:329–336.[Medline]
[Order article via Infotrieve]
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Seear M, Hui H,
Magee F, et al. Bronchial casts in children: a proposed classification
based on nine cases and a review of the literature.
Am J Respir Crit Care Med. 1997;155:364–370. [Abstract]
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