Pediatrics and Neonatology (2014) 55, 77
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EDITORIAL
Etiology and Outcome of Hydrops Fetalis
The term hydrops fetalis (HF) describes generalized sub-
cutaneous edema in the fetus or neonate, characterized by
a generalized skin thickness of >5 mm. It is usually
accompanied by ascites and often by pleural and/or peri-
cardial effusion. Generally, there are two categories of HF:
isoimmume and nonimmune. HF that develops in the pres-
ence of maternal antibodyefetal antigen-mediated red
blood cell hemolysis is referred to as isoimmune hydrop
fetalis (IHF). HF that develops in the absence of maternal
antibodyefetal antigen-mediated red blood cell hemolysis
is classified as nonimmune hydrops fetalis (NIHF). The
incidence of IHF has markedly declined after the use of
anti-D gammaglobubin prophylaxis. At present, NIHF ac-
counts for 85e90% of HF cases.1,2 Despite the recent im-
provements in perinatal care, however, 45.2% of HF cases
had IHF in Turkey.3
Various possibilities in the pathophysiology of NIHF
include disorders leading to congestive heart failure, dis-
orders characterized by decreased plasma osmotic pressure
and increased capillary permeability, and disorders with
obstructed lymphatic flow, all of which lead to abnormal
water transport between the capillary plasma and extra-
vascular tissues.4 Bellini et al4 performed a systemic review
of 6361 patients with NIHF. They classified the various
causes of NIHF into groups of disorders: cardiovascular
(21.7%), hematologic (10.4%), chromosomal (13.4%), syn-
drome (4.4%), lymphatic dysplasia (5.7%), inborn errors of
metabolism (1.1%), infection (6.7%), thoracic (6.0%), uri-
nary tract malformation (2.3%), extra thoracic tumors
(0.7%), twin-to-twin transfusion syndrome-placental (5.6%),
gastrointestinal (0.5%), miscellaneous (3.7%), and idio-
pathic (17.8%).4
Despite the advances in diagnosis and management,
NIHF still has a high mortality rate (40e50%).1,3,5,6 Huang
et al1 found that HF resulting from lymphatic malformation
had a favorable outcome; however, preterm birth at less
1875-9572/$36 Copyright ª 2013, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rights reserved.
http://dx.doi.org/10.1016/j.pedneo.2013.10.005
than 34 weeks and serum albumin concentration lower than
2 g/dL were two poor prognostic factors for survival. Takci
et al3 found that the presence of two or more serous cavity
effusions and low gestational age were independently
associated with the risk of mortality.
Jia-Yuh Chen
Department of Pediatrics, Chung Shan Medical University
Hospital, Institute of Medicine, Chung Shan Medical
University, Number 110, Section 1,
Chien-Kuo North Road, Taichung, Taiwan
E-mail address: jiayuh@csmu.edu.tw
Oct 20, 2013
References
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factors and clinical features in liveborn neonates with hydrops
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3. Takci S, Gharibzadeh M, Yurdakok M, Ozyuncu O, Korkmaz A,
Akcoren Z, et al. Etiology and outcome of hydrops fetalis:
report of 62 cases. Pediatr Neonatol 2014;55:108e13.
4. Bellini C, Hennekam RC, Fulcheri E, Rutigliani M, Morcaldi G,
Boccardo F, et al. Etiology of nonimmune hydrops fetalis: a
systematic review. Am J Med Genet Part A 2009;149A:844e51.
5. Santo S, Mansour S, Thilaganathan B, Homfray T,
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Diagn 2011;31:186e95.
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