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Case Reports
. 2010 May 6:2010:bcr08.2009.2206.
doi: 10.1136/bcr.08.2009.2206.

Serious complications of pulmonary biopsy in a boy with chylopericardium and suspected pulmonary lymphangiomatosis

Esther Harnisch  1 , Ram Sukhai, Anne Marie Oudesluys-Murphy
Affiliations
Case Reports

Serious complications of pulmonary biopsy in a boy with chylopericardium and suspected pulmonary lymphangiomatosis

Esther Harnisch et al. BMJ Case Rep. .

Abstract

Lymphangiomatosis is a rare disorder. It is commonly localised in the skeletal system and called Gorham's disease, and in thoracic or abdominal organs. Involvement of the pericardium is rare and has been described in less than 20 patients worldwide. The case of a 14-year-old boy presenting with asymptomatic chylopericardium and interstitial lung disease is presented. After lung biopsy, performed to confirm the diagnosis of pulmonary lymphangiomatosis, he developed chylothorax and massive loss of chyle via chest drains. Thoracic duct ligation did not result in clinical improvement. Treatment with interferon α2b was given and because of clinical deterioration radiotherapy was added to the treatment. This resulted in a rapid decrease of chyle production in the patient.

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Figures

Figure 1
Figure 1
Chest x-ray at presentation showing cardiomegaly and a pattern suggestive of interstitial lung disease.
Figure 2
Figure 2
A. Specimen of lung biopsy: dilated vascular structures with strong immunoreaction to CD 31, CD 34, factor VIII, vascular endothelial growth factor (VEGF) (factor VIII shown here), bronchiole (1), blood vessel (2), lymphatic vessel (3). B. Specimen of lung biopsy: dilated vascular structures with strong immunoreaction to CD 31, CD 34, factor VIII, VEGF (CD 34 shown here), blood vessel (2), lymphatic vessel (3).
Figure 3
Figure 3
Lymphangiography showing a tortuous aspect of the thoracic duct with collaterals and diffuse leakage of lymph to the lungs.
Figure 4
Figure 4
Treatment and effect of treatment regimes on chyle loss in the patient.
See this image and copyright information in PMC

References

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Publication types

Supplementary concepts