Hydrops fetalis: manifestation in lysosomal storage diseases including Farber disease

The authors describe a case of disseminated lipogranulomatosis (Farber disease) presenting as nonimmune hydrops fetalis. This is the tenth lysosomal storage disease which can show this clinical manifestation. The literature is reviewed for all hydrops cases associated with lysosomal storage diseases. Conclusion Although rare, the lysosomal storage diseases collectively are significant causes of non-immune hydrops and appropriate investigations are required in all cases of unexplained hydrops fetalis.