Successful management of late-onset primary lymphatic hypoplasia.

Journal Article (Journal Article)

Primary lymphedema of the extremities, abdomen, or chest is an unusual and difficult clinical problem with few guidelines for management. A case is reported of lymphedema acquired at the age of 61 years, with associated massive chylous ascites and chylothorax. No underlying condition was discovered and the patient was found to have hypoplastic lymphatics by lymphangiography. Initial management consisted of extremity elevation, diuresis, and repeated paracenteses and thoracenteses. A peritoneojugular shunt provided temporary relief. Surgical pleurodesis combined with intensive diuresis has given prolonged relief of his symptoms allowing him to return to a functional life. Diagnostic and therapeutic guidelines for the management of this unusual condition are suggested.

Full Text

Duke Authors

Cited Authors

  • Rustgi, AK; Rotolo, FS; Peete, WP; Vollmer, RT; Meyers, WC

Published Date

  • June 1, 1985

Published In

Volume / Issue

  • 97 / 6

Start / End Page

  • 714 - 720

PubMed ID

  • 4002118

International Standard Serial Number (ISSN)

  • 0039-6060


  • eng

Conference Location

  • United States