Successful management of late-onset primary lymphatic hypoplasia.
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.
Duke Scholars
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Related Subject Headings
- Time Factors
- Surgery
- Middle Aged
- Male
- Lymphedema
- Lymphatic System
- Humans
- Chylous Ascites
- Chylothorax
- 3202 Clinical sciences
Citation
Published In
ISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- Time Factors
- Surgery
- Middle Aged
- Male
- Lymphedema
- Lymphatic System
- Humans
- Chylous Ascites
- Chylothorax
- 3202 Clinical sciences