njølstad syndrome njølstad syndrome njølstad syndrome is a syndrome characterized by non-immune hydrops fetalis (nihf), congenital pulmonary lymphangiectasia (cpl). clinical features found in njølstad syndrome include: facial and limbs lymphedema, facial abnormalities (thin upper lip, protruding ears), pectus excavatum and vulvar and labial edema. njølstad syndrome it is named after the norwegian pediatrician pål rasmus njølstad who published a report on three siblings with the condition in 1997.