Maranto M, Cigna V, Orlandi E, Cucinella G, Lo Verso C, Duca V, Picciotto F. Non-immune hydrops fetalis: Two case reports. World J Clin Cases 2021; 9(22): 6531-6537 [PMID: 34435022 DOI: 10.12998/wjcc.v9.i22.6531]
Corresponding Author of This Article
Marianna Maranto, MD, Doctor, Fetal Medicine and Prenatal Diagnosis Unit, Villa Sofia Cervello Hospitals, Via Trabucco180, Palermo 90146, Italy. marianna.maranto@community.unipa.it
Research Domain of This Article
Obstetrics & Gynecology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Aug 6, 2021; 9(22): 6531-6537 Published online Aug 6, 2021. doi: 10.12998/wjcc.v9.i22.6531
Non-immune hydrops fetalis: Two case reports
Marianna Maranto, Valentina Cigna, Emanuela Orlandi, Gaspare Cucinella, Clelia Lo Verso, Vincenzo Duca, Francesco Picciotto
Marianna Maranto, Valentina Cigna, Emanuela Orlandi, Francesco Picciotto, Fetal Medicine and Prenatal Diagnosis Unit, Villa Sofia Cervello Hospitals, Palermo 90146, Italy
Gaspare Cucinella, Health Promotion, Maternal and Infant Care Unit, Internal Medicine and Medical Specialties “G. D’Alessandro”, University of Palermo, Palermo 90100, Italy
Clelia Lo Verso, Neonatology and Neonatal Intensive Care Unit, Civico Di Cristina Benfratelli Hospital, Palermo 90100, Italy
Vincenzo Duca, Neonatology and Neonatal Intensive Care Unit, Ingrassia Hospital, Palermo 90100, Italy
Author contributions: Maranto M reviewed the literature and drafted the manuscript; Picciotto F and Cigna V were the pregnant women’s obstetricians and contributed to manuscript drafting as for maternal management; Lo Verso C and Duca V were the babies’ neonatologists, reviewed the literature and contributed to manuscript drafting as for neonatal management; Orlandi E analyzed and interpreted the imaging findings; all authors issued final approval for the version to be submitted.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Marianna Maranto, MD, Doctor, Fetal Medicine and Prenatal Diagnosis Unit, Villa Sofia Cervello Hospitals, Via Trabucco180, Palermo 90146, Italy. marianna.maranto@community.unipa.it
Received: April 7, 2021 Peer-review started: April 7, 2021 First decision: April 23, 2021 Revised: May 6, 2021 Accepted: June 4, 2021 Article in press: June 4, 2021 Published online: August 6, 2021 Processing time: 111 Days and 18.3 Hours
Abstract
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.
Core Tip: We present herein, two rare cases of non-immune hydrops fetalis with severe fetal anemia. Despite the similar onset, the etiology was infectious in the first case and autoimmune in the last one. In particular, we want to emphasize that even if the infectious examinations are not conclusive, but the pregnancy history is strongly suggestive of infection, the infectious etiology must not be excluded. Secondly, transplacental passage of maternal autoantibodies can cause hydrops fetalis and severe anemia. In any case, obstetric management must always be aimed at fetal support up to an optimal timing for delivery by evaluating risks and benefits.