We report a female infant with congenital lymphedema, facial anomalies, intestinal lymphangiectasia consistent with a diagnosis of Hennekam syndrome. At birth the patient presented with severe respiratory distress due to nonimmune hydrops fetalis, a congenital chylothorax (CC), and pulmonary lymphangiectasia. Hydrops fetalis may be present in newborns with the Hennekam syndrome. Lymphoscintigraphy can be useful in explaining pleural-pulmonary involvement of this generalized lymph vessel malformation syndrome.
|Number of pages||5|
|Journal||American Journal of Medical Genetics|
|Publication status||Published - Jul 1 2003|
- Hennekam syndrome
- Nonimmune hydrops fetalis
- Pulmonary lymphangiectasia
ASJC Scopus subject areas