Cases Of Non-immune Fetal Hydrops Research Articles

Non-immune fetal hydrops in late pregnancу: antenatal ultrasound monitoring and pregnancy outcomes in a series of 14 cases

Background. Fetal hydrops is the accumulation of extracellular fluid in two or more fetal cavities, often in combination with subcutaneous edema. An isolated accumulation of fluid only in the abdominal, pleural, or pericardial cavities is described as ascites, pleural effusion (hydrothorax), pericardial effusion (hydropericardium). Features of the pathogenesis of non-immune hydrops fetalis (NIHF) are the follow: high hydrophilicity of fetal tissues, obstruction of the lymphatic vessels, impaired lymph return, congestive heart failure, obstruction of venous return, changes in fetal venous pressure. All these factors lead to the release of fluid from cells and tissues into the «third» spaces – the abdominal, thoracic, pericardial cavities, as well as the subcutaneous space. The oncotic pressure of fetal plasma proteins is not of great importance in the formation of the fetal circulating blood volume. Currently, all the links in the pathogenesis of hydrops fetalis syndrome with various etiological factors are not fully known. The prevalence of NIHF is unknown because it is difficult to collect relevant data; many cases of the disease are not diagnosed until intrauterine fetal death or may spontaneously resolve during the prenatal period. Currently, up to 90% of all cases of fetal dropsy are attributed to NIHF. Purpose – acquaintance of the medical community with the diagnosis and treatment of non-immune hydrops as well as the analysis of clinical features, ultrasound monitoring and perinatal outcomes of 14 cases of non-immune fetal hydrops onset in a second half of pregnancy. Materials and methods. The material for the study were publications and results of clinical trials found in the databases Scopus, Web of Science Core Collection and PubMed for the period 2009–2020 and the analytic report of the own series of 14 cases of non-immune hydrops fetalis (NIHF) of various origins. During the period of 2005–2020 under the supervision in the Ultrasound Department of Kharkiv regional hospital with regional perinatal center there were 14 pregnant women with NIHF diagnosed in a second half of pregnancy. Clinical features are described, ultrasound images and Doppler monitoring are given, perinatal / postnatal results are studied. Results and discussion. The analysis of clinical features, ultrasound monitoring and pregnancy outcomes of 14 cases of non-immune fetal hydrops developed in the second half of pregnancy in presented series was carried out. 6/14 fetuses had structural anatomical defects (lung sequestration, СDH, myasthenia gravis, megacystis and hydronephrosis, epidermolysis bullosa, meconium peritonitis, intestinal atresia). Mortality rate (including perinatal and infant losses) was as high as 9/14 cases (64.2%): 3 of antenatal, 4 of neonatal, 2 of infant death). Surgical treatment was performed on 2 newborns. 5 newborns had apparently a favorable clinical postnatal outcome. In 2 cases, spontaneous resolution with complete regression of hydrops was observed (parvovirus-B19 and idiopathic NIHF). Complete recovery of fetus (spontaneous regression of hydrops without any deterioration and pathological consequences) was observed in 1 case. Conclusions. Antenatal ultrasound monitoring of fetus with NIHF is based on the assessment of PSV CMA, ductus venous, umbilical vein, atrioventricular flow. According to the results of the study, it was revealed that the cardiovascular profile of the fetus with NIHF is disturbed earlier, and the placental profile and arterial Doppler-later. Normal umbilical artery Doppler do not exclude the possibility of an adverse outcome, including intrauterine fetal demise. Extended Doppler monitoring is essential at NIHF. All neonates with NIHF in an antenatal anamnesis require postnatal follow-up.

NON-IMMUNE HYDROPS FETUS IN THE THIRD TRIMESTER: THE EXPERIENCE OF DIAGNOSIS AND TREATMENT TACTICS DELIVERY INFANT

Presented clinical cases non-immune fetal hydrops diagnosed in the third trimester of pregnancy. Analyzed etiological faktory non-immune fetal hydrops, diagnosis and tactics of pregnancy and childbirth, infant treatment non-immune fetal hydrops. Established that cases of non-immune fetal hydrops met in 0.05% of cases, the total number of births. In 50% of etiological factor in the development of fetal hydrops was intrauterine infection. The successful outcome of infants with non-immune fetal hydrops was 33.3%.

HIDROPESÍA FETAL NO INMUNE: PROPUESTA DE UN FLUJOGRAMA PARA EL DIAGNÓSTICO ETIOLÓGICO

OBJETIVOS: Proponer un protocolo actualizado para el diagnóstico etiológico de la hidropesía fetal (hydrops fetalis). LUGAR: Centro Médico Especializado Genética. MATERIAL Y MÉTODOS: Elaboración de un protocolo basado en el estudio de 27 casos de hidropesía fetal no inmune (HFNI) referidos para estudio cromosómico durante un período de 10 años. El estudio evalúa el análisis citogenético, la evolución del embarazo, los hallazgos ecográficos y anatomopatológicos. En 25 de los 27 casos, se estudió el cariotipo del producto: 18 en líquido amniótico, 5 en vellosidades coriales del aborto y 2 en sangre periférica. RESULTADOS: En 11 (44%) casos de hidropesía fetal, se encontró alteraciones cromosómicas: 6, síndrome de Turner; 3, trisomía 21 (síndrome Down); una, trisomía 13; y una, trisomía 7. Hubo hallazgos anatomopatológicos de la placenta compatibles con infección en dos casos: el cariotipo no se pudo estudiar en uno y en el otro, fue masculino normal. En otros casos con cromosomas normales, la anatomía patológica demostró malformaciones causantes de hidropesía. No se determinó el factor desencadenante de la hidropesía en 33% de casos. CONCLUSIONES: La alta proporción de casos idiopáticos puede ser atribuida en parte a la falta de uniformidad y sistematización en los criterios y procedimientos utilizados para el diagnóstico. El flujograma propone un esquema que facilitará el diagnóstico etiológico y, por tanto, la asesoría genética para la prevención de recurrencias.

Nonimmune fetal hydrops and lysosomal storage disease: the finding of vacuolated lymphocytes in ascitic fluid in two cases

What's already known about this topic? Nonimmune fetal hydrops (NIFH) is a heterogeneous condition and establishing a diagnosis can be challenging. Inborn errors of metabolism are one of many possible etiologies for NIFH. Inborn errors of metabolism are often missed in utero. What does this study add? We present two cases of NIFH caused by underlying lysosomal storage disease: galactosialidosis and mucopolysaccharidosis type VII. The unique finding of vacuolated lymphocytes in fetal peritoneal fluid lead to the diagnosis.

Diagnóstico prenatal de hidrops fetal no inmune. Serie de nueve casos. Revisión de la literatura

Objetivo: se presenta una serie de casos de hidrops fetal no inmune (HFNI) con el objetivo de revisar la literatura sobre su etiología y pronóstico.Materiales y métodos: serie de casos diagnosticados por ultrasonografía en el Servicio de Perinatología del Hospital Dr. Adolfo Prince Lara, Venezuela, hospital público de referencia de atención perinatal regional que atiende población de medios y bajos ingresos, en el periodo 2005-2009, y se realiza una revisión de la literatura en la base de datos Medline vía Pubmed, y en la base latinoamericana SciELO desde el año 1995 hasta 2011, y en libros de la especialidad. La palabra clave usada fue hidrops fetal.Resultados: se revisaron 2195 historias clínicas encontrándose nueve casos de HFNI, resultando una prevalencia de (0,41%). Hubo dos casos de monosomías, un caso asociado a proceso infeccioso, dos casos de malformaciones cardiacas, un caso de malformaciones múltiples, y en tres no se precisó etiología. Todos los productos fallecieron, ya sea in utero o en el periodo neonatal. Se encontraron 1584 publicaciones, incluyéndose 14 referencias, 6 revisiones, 6 presentaciones de casos y 2 artículos originales.Conclusión: HFNI está asociado a diversas patologías fetales y tiene alta mortalidad.

Hidropesía fetal secundario a hemangioma cavernoso y síndrome polimalformativo

We report a case of non-immune fetal hydrops diagnosed at 32 weeks of pregnancy that ended in foetal death. Following the autopsy an unknown malformation syndrome was found together with a liver tumour that invaded the chest cavity. We analyse the possible causal association of both findings.

Fetal Cardiac Troponin T as a Marker of Poor Prognosis in Nonimmune Hydrops Fetalis

Objective: Severe fetal anemia and cardiac compromise are important causes of nonimmune hydrops fetalis, and fetal recovery also depends on the degree of fetal heart compromise. The aim of this study was to report the fetal cardiac troponin T (cTnT) levels in cases of nonimmune fetal hydrops. Methods: Fetal cTnT was analyzed on 7 occasions in 5 cases of nonimmune fetal hydrops (twice in 2 cases). Results: In 3 of 4 fetuses in which intrauterine death occurred, fetal cTnT levels were increased. The only fetus that survived in this series showed decreased levels of cTnT before birth. Conclusion: Fetal cTnT levels may be a marker of fetal prognosis in cases of fetal hydrops.

Pränatale Ultraschalldiagnostik bei einer seltenen Ursache des Nichtimmunologischen Hydrops fetalis

Nonimmune fetal hydrops can be caused by a large variety of fetal abnormalities. We describe a case of nonimmune fetal hydrops associated with fetal tuberous sclerosis (Bourneville's disease). There were few morphologic abnormalities and the final diagnosis was made at autopsy.

Nonimmune fetal hydrops with generalized lymphangiectasia

The objective of this paper was to evaluavate a case of non-immune fetal hydrops associated with generalized lymphangiectasia. We studied the ultrasound examinations, and clinical and biochemical data of a patient with this syndrome. A bilateral pleural effusion detected by ultrasound in the third trimester of gestation was the first manifestation of the disease. Though rare, generalized lymphangiectasia should be considered in patients with pleural effusion and a progressive increase of the fluids in the fetal cavities and of the amniotic fluid.

Successful ultrasound-guided intrauterine blood transfusion in severe non-immune hydrops fetalis

Non-immune hydrops fetalis has varied etiology, high mortality and few known successful treatment modalities. A rare case of non-immune fetal hydrops with severe anemia diagnosed prenatally and treated successfully by repeated ultrasound-guided intrauterine blood transfusions is presented.

Albumin Transfusion in Non-Immune Fetal Hydrops: Doppler Ultrasound Evaluation of the Acute Effects on Blood Circulation in the Fetal Aorta and the Umbilical Arteries

A case of non-immune fetal hydrops, diagnosed as mucopolysaccharidosis VII with hypoalbuminemia, was treated in utero with albumin transfusions via cordocentesis on five occasions. Blood samples were taken for analysis of full blood count and blood gases before and after the transfusions. Pulsed Doppler ultrasound examinations of the arterial waveform were performed in the umbilical arteries and the descending fetal aorta and analyzed for the pulsatility index (PI). The hemoglobin concentration and the hematocrit decreased from 111 +/- 5 g/l and 0.335 +/- 0.008 to 95 +/- 5 g/l and 0.282 +/- 0.023 (mean +/- SD), respectively, after the transfusions. The calculated blood volume increased more than the given volume, indicating an autotransfusion causing additional plasma volume expansion. The blood gases were not significantly changed by transfusion. The PI decreased both in the umbilical arteries (p less than 0.05) and the descending fetal aorta, indicating peripheral vasodilatation. A positive correlation was found between the umbilical artery PI and the hematocrit before and after the albumin transfusion (r = 0.59; p less than 0.05). This relation could be due to covariation with other factors, e.g. peripheral vasodilatation secondary to the increased blood volume and the puncture of the umbilical vein itself. No improvement of the hydrops was seen after the albumin transfusions. The fetus died in utero during spontaneous labor after 30 gestational weeks.

Nonimmune hydrops fetalis: Antenatal diagnosis and management

An 8-year (1975–1982) study of 26 cases of nonimmune fetal hydrops revealed the main causes to be: anomalies (1126), tachyarrhythmias (426), and twin-to-twin transfusion (426). The perinatal mortality was 1926 (76%). Based on this series, and a review of the literature, a plan for the antenatal diagnosis and management of these cases is outlined.