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Case Reports in Pulmonology
Volume 2011, Article ID 827591, 6 pages
Case Report

Familial Pulmonary Capillary Hemangiomatosis Early in Life

1University Children's Hospital, University of Wuerzburg, Josef-Schneider-Straße 2, 97080 Wuerzburg, Germany
2Department of Pathology, Faculty of Medicine Mannheim, University of Heidelberg, 68167 Mannheim, Germany
3Harvard Medical School, Department of Pathology, Massachusetts General Hospital, Boston, MA 02114, USA

Received 30 August 2011; Accepted 3 October 2011

Academic Editors: P. Bresser, G. Hillerdal, and H. Kobayashi

Copyright © 2011 Johannes Wirbelauer et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Background. Pulmonary capillary hemangiomatosis (PCH) is a rare disease, especially in infancy. Four infants have been reported up to the age of 12 months. So far, no familial patients are observed at this age. Patients. We report three siblings, two female newborns and a foetus of 15-week gestation of unrelated, healthy parents suffering from histologically proven PCH. The first girl presented with increased O2 requirements shortly after birth and patent ductus arteriosus (PDA). She subsequently developed progressive respiratory failure and pulmonary hypertension and died at the age of five months. The second girl presented with clinical signs of bronchial obstruction at the age of three months. The work-up showed a PDA—which was surgically closed—pulmonary hypertension, and bronchial wall instability with stenosis of the left main bronchus. Transient oxygen therapy was required with viral infections. The girl is now six years old and clinically stable without additional O2 requirements. Failure to thrive during infancy and a somewhat delayed development may be the consequence of the disease itself but also could be attributed to repeated episodes of respiratory failure and a long-term systemic steroid therapy. The third pregnancy ended as spontaneous abortion. The foetus showed histological signs of PCH. Conclusion. Despite the differences in clinical course, the trias of PCH, PDA, and pulmonary hypertension in the two life born girls suggests a genetic background.