title: Etiology of non-immune hydrops fetalis: An update.
abstract: Hydrops fetalis is an excessive fluid accumulation within the fetal extra vascular compartments and body cavities. Non-immune hydrops fetalis (NIHF), due to causes other than Rh alloimmunization, is the cause in >85% of all affected individuals. Herein we present an update of our earlier systematic literature review [Bellini et al., 2009] using all publications between 2007 and 2013. We excluded most of the initial 31,783 papers by using strict selection criteria, thus resulting in 24 relevant NIHF publications describing 1,338 individuals with NIHF. We subdivided the affected individuals into 14 classification groups based on the cause of NIHF (percentage of the total group): Cardiovascular (20.1%), Hematologic (9.3%), Chromosomal (9.0%), Syndromic (5.5%), Lymphatic Dysplasia (15.0%), Inborn Errors of Metabolism (1.3%), Infections (7.0%), Thoracic (2.3%), Urinary Tract Malformations (0.9%), Extra Thoracic Tumors (0.7%), TTTF-Placental (4.1%), Gastrointestinal (1.3%), Miscellaneous (3.6%), Idiopathic (19.8%). We discuss the results of the review. There may be some shifts in the percentages of etiological categories as compared to the previous review, but the small numbers within each category make drawing firm conclusions hazardous. We highlight the need for multi-center series of NIHF cases collected and classified using the same schemes in diagnostic work-ups to allow for comparisons of larger numbers of cases.
authors.affiliation: Neonatal Intensive Care Unit, Department of Intensive Care, Gaslini Institute, Genoa, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Bellini
authors.forename: Gloria
authors.initials: G
authors.lastname: Donarini
authors.forename: Dario
authors.initials: D
authors.lastname: Paladini
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Calevo
authors.forename: Tommaso
authors.initials: T
authors.lastname: Bellini
authors.forename: Luca A
authors.initials: LA
authors.lastname: Ramenghi
authors.forename: Raoul C
authors.initials: RC
authors.lastname: Hennekam
mesh_terms: D005260:Female; D006801:Humans; D015160:Hydrops Fetalis / Q000145:classification / Q000209:etiology* / Q000503:physiopathology*; D008297:Male; D011247:Pregnancy
keywords: classification; etiology; hydrops fetalis; nonimmune hydrops fetalis; pathophysiology; systematic review