Hennekam syndrome presenting as nonimmune hydrops fetalis, congenital chylothorax, and congenital pulmonary lymphangiectasia
C Bellini, M Mazzella, C Arioni… - American Journal of …, 2003 - Wiley Online Library
C Bellini, M Mazzella, C Arioni, C Campisi, G Taddei, P Toma, F Boccardo, RC Hennekam…
American Journal of Medical Genetics Part A, 2003•Wiley Online LibraryWe 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.© …
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.© …
Abstract
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. © 2003 Wiley‐Liss, Inc.
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