Cystic hygroma of the neck and non-immunologic hydrops fetalis

Fetal cystic hygromas are a manifestation of early lymphatic obstruction. They are mostly associated with nonimmune hydrops fetalis. They often occur in a number of chromosome abnormalities (Turner syndrome and Down syndrome). We report on a prenatally detected case with nuchal cystic hygroma and nonimmune hydrops fetalis without chromosome aberration and without further major malformations. Postnatally hygroma and hydrops regressed.     

Familial non-immune hydrops fetalis and congenital pulmonary lymphangiectasia

We report on three siblings with non-immune hydrops fetalis. Congenital pulmonary lymphangiectasia was diagnosed in two of them. One of these, a girl still alive and suffering from frequent airway infections, has bilateral pleural effusions and distal congenital lymphoedema. Conclusion To our knowledge, this is the first report of non-immune hydrops fetalis and congenital pulmonary lymphangiectasia occurring in siblings. Received: 4 February 1997 and in revised form: 23 September 1997 / Accepted: 23 September 1997     

Nonimmune hydrops fetalis

Hydrops fetalis is a relatively rare phenomenon that presents itself in the delivery room in an extremely acute manner. Prompt resuscitation, an understanding of the condition and its presentation, and immediate treatment can make the difference between life and death in these cases. The focus of this article is on nonimmune hydrops, the type of hydrops seen in the clinical setting. Maternal and fetal pathophysiology, current theories, diagnostic evaluations, neonatal pathophysiology, clinical manifestations, treatment options, pertinent research, and the needs of the family are discussed.     

Alpha-thalassaemic hydrops fetalis.

Concentrations of total protein, albumin, colloid osmotic pressure, and immunoglobulins G and M were measured in the umbilical venous plasma of 4 infants with alpha-thalassaemic hydrops fetalis. Total protein, albumin, and colloid osmotic pressure concentrations were low, and these are likely to contributory factors in the formation of fetal oedema. Immunoglobulin G levels were low suggesting a reduction in placental transfer probably due to placental oedema.     

重症胎儿水肿的急救

胎儿水肿是指胎儿或新生儿生后即发现的程度较重的全身性皮下水肿,常伴有胸膜腔、腹膜腔及心包积液。重症胎儿水肿严重威胁着新生儿的健康和生命,由严重病因导致者病死率高,故需进行急救处理。近年来产前超声波检查对产前即发现胎儿水肿及范围和程度均有帮助,并有助于进一步了解病因,使产科和新生儿科医生能预知胎儿水肿的可能病因并给予及时、相应的处理。1　胎儿水肿的常见病因胎儿水肿按病因分为二大类:一种为免疫因素,如母胎之间血型不合所致的同族免疫性溶血而引起的胎儿水肿,其发生与红细胞破坏所致的严重贫血、心衰、低白蛋白血症及…     

Genetic causes of hydrops fetalis.

A series of 1790 fetal and neonatal autopsies performed between 1976 and 1988 were retrospectively investigated for the presence of hydrops. Thirty (5.5%) and 35 (2.8%) cases of hydrops were found in the groups of fetal and neonatal autopsies, respectively. Genetic causes accounted for 35%. A careful search for previously reported genetic causes of fetal hydrops indicated 64 different etiologies. Twenty-one of them were not mentioned in the previous reviews: these include 9 skeletal dysplasias, 5 inborn errors of metabolism, 3 autosomal recessive, 3 autosomal dominant conditions, and 1 chromosomal abnormality.     

Nonimmune hydrops fetalis in Noonan's syndrome

Nonimmune hydrops fetalis has been reported to be associated with congenital malformations. We describe two newborns with Noonan's syndrome who presented with nonimmune hydrops fetalis that was most likely secondary to a generalized lymphatic vessel dysplasia. Other manifestations of lymphatic abnormalities in Noonan's syndrome, such as pedal edema and pulmonary and intestinal lymphangiectasis, have been observed in children. Nonimmune hydrops represents one end of the spectrum of abnormalities seen in this syndrome.     

Non-immunologic hydrops fetalis and congenital chylothorax

A child was born at the 37th week of pregnancy with hydrops fetalis. Hydramnios and hydrothorax had been proven by fetal ultrasonography. No fetal or maternal etiology was found. At age 4 days, at the beginning of enteral nutrition, the pleural effusion became characteristic of chylothorax. Recovery occurred after 2 weeks of parenteral nutrition. Chylothorax might be an unrecognized etiology of non immune hydrops fetalis. The relationships between both conditions and the interest of prenatal treatment are discussed.     

Intrauterine treatment on non-immune hydrops fetalis.

In 51 cases with non-immunologic hydrops fetalis (NIHF), perinatal management was performed based on our protocol. Twenty-two cases were treated by albumin and/or packed red cell (PRC) injection into the fetal abdominal cavity, and 9 cases by transplacental digitalization. Among the cases treated by albumin and/or PRC injection, 6 of 8 cases without pleural effusion recovered in utero, and all 6 cases are alive. However, of 14 cases with pleural effusion, none recovered in utero, and only one case is alive. Of 9 cases treated by transplacental digitalization, 2 cases recovered in utero, and only one case is alive. All fetuses with congenital heart anomaly died. This evidence indicates that albumin and/or PRC injection into the fetal abdominal cavity is an effective procedure for in utero treatment of NIHF without pleural effusion, but suggests that in NIHF resulting from either congenital heart anomaly and/or heart failure, the survival rate may not be increased by transplacental digitalization.     

The contribution of extramedullary hematopoiesis to hepatomegaly in anemic hydrops fetalis: a study in alpha-thalassemia hydrops fetalis

Massive hepatomegaly is a common finding in hydrops fetalis (HF) arising from hemoglobinopathies. It has been suggested that extramedullary hematopoiesis, which is markedly increased in response to anemia, plays a principal role in hepatomegaly via sinusoidal obstruction and distortion of the intrahepatic architecture. To test this concept, the authors compared 20 cases of α-thalassemia (hemoglobin Bart, 15 cases; hemoglobin H, 5 cases) with 19 cases of HF arising from other causes and 39 control nonhydrops cases. The mean liver weight in α-thalassemia cases was significantly heavier than in control cases and in hydrops fetalis cases due to other causes (P < 0.01). This was not explainable on the basis of extramedullary hematopoiesis (erythropoiesis or myelopoiesis) or hemosiderin deposition, since cases of HF from other causes, especially related to anemia, showed similar findings to α-thalassemia cases. While these processes no doubt contribute to hepatic weight, the major factor would still be high-output cardiac failure causing hepatic congestion.