Prognostic Factors and Clinical Features in Liveborn Neonates with Hydrops Fetalis
Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan, Republic of China. American Journal of Perinatology
(Impact Factor: 1.91).
02/2007; 24(1):33-8. DOI: 10.1055/s-2006-958158
The purpose of this study was to delineate the etiology and the clinical features of liveborn neonates with hydrops fetalis, and to explore the prognostic factors for survival. Medical records of 28 liveborn neonates with hydrops fetalis between April 1995 and March 2005 were reviewed retrospectively. Demographic data, clinical manifestations, laboratory findings, and outcomes were analyzed. Most patients presented with pleural effusions (21 of 28) and ascites (22 of 28). The majority of patients had hydrops due to cardiovascular diseases (seven of 28), hematologic disorders (six of 28), lymphatic malformations (six of 28), and idiopathic origins (six of 28). The overall survival rate was 50% and was highest (83%) in infants with lymphatic malformations. By univariate analysis, risk factors for mortality are earlier ages at diagnosis and at birth, low Apgar scores, need for resuscitation in the delivery room, low serum albumin level, and severe acidemia. After using stepwise multiple logistic regression analysis, the most significant factors associated with fatality were younger gestational age at birth and lower serum albumin level. Hydrops fetalis remains a complex condition with a high mortality rate. Hydrops resulting from lymphatic malformations has a favorable outcome. Preterm birth at less than 34 weeks and serum albumin concentration lower than 2 g/dL are two poor prognostic factors for survival.
Available from: Anita J Moon-Grady
- "Historically, hydrops was felt to be a harbinger of fetal demise, but advancements in fetal treatment have improved outcomes for diseases such as primary hydrothorax  and chest masses   . Nevertheless, mortality still approaches 40%–50% when considering hydropic fetuses overall    and predicting survival remains a challenge. "
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Fetal hydrops arises from multiple disease processes and can portend a grim prognosis. We reviewed our experience with hydropic fetuses to understand relevant antenatal anatomic and physiologic predictors of survival.
We reviewed fetal ultrasounds and echocardiograms of hydropic fetuses evaluated from 1996 to 2013.
Overall neonatal survival in 167 fetuses was 44% (range, 0-75%) and was influenced by the underlying disease process. The anatomic distribution of fluid varied and was not significantly different between survivors and nonsurvivors. Univariate analysis indicated that resolution of hydrops and delivery at a later gestational age were predictive of survival (OR: 5.7 (95% CI: 2.5-13.2) and OR: 1.3 (95% CI: 1.1-1.4), respectively). Fetal intervention also improved survival in some diseases. Echocardiograms were reviewed to group fetuses with similar cardiac physiology and defined categories with high or low/normal cardiothoracic ratio (CTR). Among patients with a high CTR, the cardiovascular profile score was predictive of survival (p=0.009).
Survival in hydrops depends on the underlying disease, available fetal therapies to resolve hydrops, and the gestational age of delivery and not on the specific anatomic manifestations of hydrops. In hydropic fetuses with high CTRs, the cardiovascular profile score may be a useful prognostic indicator.
Available from: Eugen Mengel
- "More recent recognition of factors other than isoimmune hemolytic disease that can cause or be associated with hydrops fetalis led to the use of the term non-immunological hydrops fetalis (NIHF). Currently, NIHF (reported incidence of 1 in 2000–3000 pregnancies
) is more common, comprising 85-90% of all described cases
[4,5]. The precise incidence of hydrops fetalis is difficult to elucidate, because many cases are not detected prior to intrauterine fetal death and some cases may resolve spontaneously in utero. "
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Lysosomal storage disorders (LSD) are a rare cause of non immunological hydrops fetalis (NIHF) and congenital ascites. The reported incidence is about 1%. The incidence of idiopathic NIHF is estimated to be about 18%.
Patients and methods
We report four cases with transient hydrops fetalis resulting from LSD and performed a literature review on LSD with NIHF and congenital ascites in combination.
At present, 12 different LSDs are described to be associated with NIHF or congenital ascites. Most patients had a family history of NIHF, where the preceding sibling had not been examined. A diagnostic approach to the fetus with NIHF due to suspected LSD either in utero or postnatal is suggested. Transient forms of NIHF and/or ascites in association with MPS IVA, MPS VII and NPC are described for the first time in this publication.
LSD should be considered in transient hydrops. Enzymatic studies in chorionic villous sample or amniotic cultured cells, once the most common conditions associated with fetal ascites or hydrops have been ruled out, are important. This paper emphasizes the fact that LSD is significantly higher than the estimated 1% in previous studies, which is important for genetic counseling as there is a high risk of recurrence and the availability of enzyme replacement therapy for an increasing number of LSD.
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ABSTRACT: Nonimmune hydrops fetalis (NIHF) is a condition in which excess fluid has accumulated in the fetal interstitial spaces as a result of one or more nonimmune factors. A plethora of maternal, placental, and fetal disease processes have been associated with NIHF. Knowledge of the
various etiologies of NIHF and how the disease process affects fluid homeostasis is important for planning patient care and counseling families of patients diagnosed with nonimmune hydrops fetalis. This article discusses the mechanisms governing fluid distribution in the extracellular spaces,
examines the various etiologies associated with NIHF, and describes the pathogenesis of NIHF for each etiologic category. Part II of this article will appear as the Evidence-Based Practice column in the November/December 2010 issue of Neonatal Network®.
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