Hydrops fetalis

Gene: FLT4

Green List (high evidence)

Non-immune fetal hydrops is a rare prenatal manifestation of Lymphedema, hereditary, IA (Milroy diease, OMIM:153100), but in general Milroy disease is not associated with more widespread lymphatic abnormalities.

A Ghalamkarpour et al (PMID:16965327) reported V878M substitution in a 22-week old fetus who was found to have fetal hydrops with bilateral leg edema, pleural effusions, hydrothorax, and pulmonary hypoplasia.

Daniel-Spiegel et al (PMID:16231305) report a case of primary congenital lymphedema (PCL) presenting as hydrops fetalis.

Created: 29 Mar 2022, 12:07 a.m. | Last Modified: 29 Mar 2022, 12:07 a.m.

Panel Version: 0.241

Mode of inheritance
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted

Phenotypes
Lymphatic malformation 1, MIM# 153100

Publications

• 16231305
• 16965327