Non-immune hydrops fetalis:Two case reports

BACKGROUND Fetal hydrops is a serious condition difficult to manage,often with a poor prognosis,and it is characterized by the collection of fluid in the extravascular compartments.Before 1968,the most frequent cause was the maternal-fetal Rh incompatibility.Today,90%of the cases are non-immune hydrops fetalis.Multiple fetal anatomic and functional disorders can cause non-immune hydrops fetalis and the pathogenesis is incompletely understood.Etiology varies from viral infections to heart disease,chromosomal abnormalities,hematological and autoimmune causes.CASE SUMMARY A 38-year-old pregnant woman has neck lymphoadenomegaly,fever,cough,tonsillar plaques at 14 wk of amenorrhea and a rash with widespread itching.At 27.5 wk a fetal ultrasound shows signs of severe anemia and hydrops.Cordocentesis is performed with confirmation of severe fetal anemia and subsequent fetal transfusion.The karyotype is 46,XX,array-comparative genome hybridization(CGH)negative,and infectious tests are not conclusive.In the following days there is a progressive improvement of the indirect signs of fetal anemia.At 33.6 wk,for relapse of severe fetal anemia,further fetal transfusions are necessary and an urgent cesarean section is performed.On the day 12 of life,for the detection of anemia,the newborn is subjected to transfusion of concentrated red blood cells and begins treatment with erythropoietin.Later there is a normalization of blood chemistry values and the baby does not need new transfusions.A 29-year-old pregnant woman,with Sjogren's syndrome and positive Anti-Ro/SSA antibodies,is subjected to serial fetal ecocardio for branch block.At 26.5 wk there is a finding of fetal ascites.Infectious disease tests on amniotic fluid are negative as well as quantitative fluorescent polymerase chain reaction,Array CGH.At cordocentesis Hb is 1.3 mmol/L,consequently fetal transfusion is performed.Also in this case,due to continuous episodes of relapse of fetal anemia with consequent transfusions,at 29.4 wk a cesarean section is performed.On day 9 of life,a treatment with erythropoietin is started in the newborn,but the baby needs three blood transfusions.The search for autoantibodies in the baby found SS-A Ro60 positive,SSA-Ro52 positive and SS-B negative.The hemoglobin values normalized after the disappearance of maternal autoantibodies.CONCLUSION An attempt to determine the etiology of hydrops should be made at the time of diagnosis because the goal is to treat underlying cause,whenever possible.Even if the infectious examinations are not conclusive,but the pregnancy history is strongly suggestive of infection as in the first case,the infectious etiology must not be excluded.In the second case,instead,transplacental passage of maternal autoantibodies caused hydrops fetalis and severe anemia.Finally,obstetric management must be aimed at fetal support up to an optimal timing for delivery by evaluating risks and benefits to increase the chances of survival without sequelae.

When Giant Lymphangioma Meets Hydrops Fetalis: An Uncommon Case Report of Lower Extremity Involvement

Approximately 75%of lymphangiomas manifest in the head and neck region,with limb and bone involvement observed in only 2%of cases.This case report presents a rare instance of fetal lymphangioma associated with hydrops fetalis,affecting the left lower extremity and abdomen.A 22-year-old secundigravida woman was referred to the gynecology department at 31.2 weeks of gestation.Initial fetal ultrasound revealed a live fetus with a large lymphangioma involving the left lower extremity,gluteal region,and abdomen.Additional ultrasound findings included subcutaneous edema,pericardial effusion,ascites,placentomegaly,and tricuspid regurgitation.Postnatal clinical examination confirmed the diagnosis of lymphatic malformation.Notably,only one other similar case has been reported in the English-language PubMed database.This report adds to the medical literature as a rare instance of prenatally diagnosed lymphangioma-associated hydrops fetalis.

An updated systematic review of Zika virus-linked complications

Objective: To determine the breadth of Zika virus(ZIKV)-associated brain anomalies in neonates and adults. Methods: Systematic review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses(PRISMA) statement using electronic databases ScienceDirect, Pubmed, Medline, Scopus, and Global Health Library.Only those research articles, case studies, case-control studies, case-cohort studies, crosssectional studies, and organizational survey reports were included in the study that reported any fetal outcomes for pregnant women who had infected with ZIKV during the gestational period and ZIKV-related neurological complications in adults as well. Results: Out of total 72 retrieved articles, 50 met the inclusion criteria. We estimated a significant increase in incidence of neural abnormalities such as Guillain-Barre syndrome and microcephaly in the regions that are experiencing ZIKV outbreaks. Other neurological malformations found in ZIKV patients include hydrancephaly/hydrops fetalis, myasthenia gravis,meningoencephalitis and myelitis. Conclusion: Our systematic analysis provides the broad spectrum of neurological malformations in ZIKV infected patients and these data further support the causal link of ZIKV with neurological disorders.

Lethal Developmental Defects: An Overview

About 3% of all conceptions are associated with major congenital malformations, many of them are lethal developmental defect and genetic in origin or teratogenic (adverse effects of the environment during gametogenesis or early embryogenesis). Genetics with or without adverse environment has role in virtually every developmental defect/malformation disorders in causation, predisposition, susceptibility & modulation of disease. Advances in genetics, introduction of triple marker screening, routine obstetric ultrasound examination into obstetric practice & accesses to prenatal diagnosis helped in secondary prevention (early detection & termination) of lethal developmental defects. Ultrasound detection of fetal developmental defects/malformation is common now and often decision on pregnancy solely based on ultrasonic morphological description. This practice leads to difficulty in providing accurate counseling as well as preventing disorder in subsequent pregnancy, in particular early. Hence an understanding of reproductive genetics of major developmental disorders is important for today’s perinatal care specialists. This overview will outline the various lethal developmental defects observed in an advanced reproductive genetics set up and various approaches adopted to derive diagnosis. Detailed assessment of fetus after termination of pregnancy (spontaneous/induced) for fetal anomalies was carried out in most cases. As most cases was referred after termination in formalin routine chromosomal analysis was not possible however, in selected cases targeted FISH analysis with specific chromosomal probe was carried out to confirm clinical diagnosis. Detailed evaluation of fetus is important as this practice often helped in modification of genetic counseling, as well as course of management in the next pregnancy. No molecular diagnostic or screening work was carried out due to non availability of information and facility in past. However, this is important today as many of the lethal developmental defects are yet to be categorized etiopathologically, and hence immediate need is to start clinical registry along with biorepository of developmental defects cases for future research work on informative families, in particular with multiple affected fetuses/sibs, using genomics, proteomics, metabolomics, platforms.

Parvovirus B19 and Pregnant Women: A Bibliographic Review

Primary infection with parvovirus B19 is an uncommon but serious and treatable cause of chronic anemia in immuno compromised hosts. Widely distributed, it is responsible for a wide range of clinical manifestations, the characteristics and outcome of which depend on the interaction between the viral properties and the physiological and immune status of the infected individuals. Infection during pregnancy can result in fetal anemia, abortion, and hydrops. Pregnancy does not appear to affect the course of the infection, but the infection may affect the pregnancy. The diagnosis of B19V can be made by serological and molecular investigation of the mother, fetus and newborn. In these conditions, it seemed necessary for us to answer in this article the various questions raised by the occurrence of a contagion and/or an infection with Parvovirus B19 during pregnancy. Our objective was to determine at first the nature and the main characteristics of Parvovirus B19 as well as its propagation during the pregnancy and to show its risk for the pregnant woman and her fetus. The importance of the subject is proven by the data on the spread and incidence of the virus. Worldwide, the focus on pregnancy is due to the additional potentially fatal effects on the fetus. This document covers the important aspects of a medical investigation: causes, symptoms, tests and diagnosis.