Congenital Pulmonary Lymphangiectasis

doi:10.1001/archpedi.1970.02100090088006

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Vol. 120 No. 4, October 1970

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Congenital Pulmonary Lymphangiectasis

Jacqueline A. Noonan, MD; Linda R. Walters, MD; John T. Reeves, MD

Am J Dis Child. 1970;120(4):314-319.

Abstract

Three patients with pulmonary lymphangiectasis were studiedby cardiac catheterization. All had evidence of alveolar hypoventilationand pulmonary hypertension. Postmortem injection studies ofthe abnormal lungs demonstrated greatly dilated and intracommunicatingpleural, interlobular, and perivascular lymphatics. Two of thepatients had obstructed total anomalous pulmonary venous return,while the third had a ventricular septal defect. In the latter,a premortem diagnosis of pulmonary lymphangiectasis was madeat 4 months of age by lung biopsy. Forty five cases from theliterature have been reviewed. From the review it is postulatedthat pulmonary lymphangiectasis can occur in the following threeforms: (1) as part of generalized lymphangiectasis; (2) secondaryto pulmonary venous obstruction; and (3) as a primary developmentaldefect of the lung. The clinical picture and prognosis varieswith the type present.

Author Affiliations

Lexington, Ky

From the sections of pediatrics (Drs. Noonan and Walters) and medicine (Dr. Reeves), University of Kentucky Medical Center, Lexington, Ky.

Footnotes

Received for publication April 1, 1970.

Reprint requests to Department of Pediatrics, University ofKentucky Medical Center, Lexington, Ky 40506 (Dr. Noonan).

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