Non-immune fetal hydrops with hepatic hemangioendothelioma and Kasabach-Merritt syndrome: a case report

A premature infant presented with non-immune hydrops fetalis, a liver mass, thrombocytopenia, and hypofibrinogenemia. Histologic examination of the liver tumor showed an infantile hemangioendothelioma. The clinical features of this case can be explained by anemia, hypoalbuminemia, and coagulopathy. The association with Kasabach-Merritt syndrome, the pathophysiology of non-immune hydrops fetalis, and primary hepatic neoplasms of the neonate are discussed.     

Meconium peritonitis and parvovirus B19 infection associated with hydrops fetalis

Two preterm infants with non-immune hydrops fetalis associated with meconium peritonitis are reported. The first presented with a cystic abdominal mass and the second had positive parvovirus B19 serology. The association of meconium peritonitis with hydrops fetalis was through different mechanisms in each patient.     

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     

Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration

Extralobar pulmonary sequestration was found in a newborn premature infait that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphatic vessels in the sequestered lung tissue probably due to impeded lymph drainage. We suggest that not extralobar pulmonary sequestration itself but a subsequent massive unilateral hydrothorax due to severe obstruction of lymph drainage was the cause of the non-immune hydrops fetalis, pulmonary hypoplasia and polyhydramnios. If these symptoms are diagnosed before delivery, a search for extralobar pulmonary sequestration is indicated.     

Hydrops fetalis and fibrosarcoma: case report of an uncommon association

Fetal hydrops associated with neonatal tumours is an uncommon occurrence. The diagnosis can be established prenatally by ultrasound examination. The treatment of choice is surgery which may be curative. We report the case of a male born at 32 weeks gestation who presented with severe hydrops fetalis and a thoracic mass. The child could not be operated upon because of rapid clinical deterioration. The autopsy findings confirmed the diagnosis of congenital fibrosarcoma. This is, to our knowledge, the first case of hydrops fetalis associated with fibrosarcoma. Conclusion The association of hydrops fetalis and fibrosarcoma is an exceptional observation but can be added to the long list of differential diagnoses of non-immune hydrops.     

Hydrops fetalis: manifestation in lysosomal storage diseases including Farber disease

The authors describe a case of disseminated lipogranulomatosis (Farber disease) presenting as nonimmune hydrops fetalis. This is the tenth lysosomal storage disease which can show this clinical manifestation. The literature is reviewed for all hydrops cases associated with lysosomal storage diseases. Conclusion Although rare, the lysosomal storage diseases collectively are significant causes of non-immune hydrops and appropriate investigations are required in all cases of unexplained hydrops fetalis. Received: 25 July 1996 / Accepted: 21 August 1996     

Hydrops fetalis and fibrosarcoma: case report of an uncommon association

Fetal hydrops associated with neonatal tumours is an uncommon occurrence. The diagnosis can be established prenatally by ultrasound examination. The treatment of choice is surgery which may be curative. We report the case of a male born at 32 weeks gestation who presented with severe hydrops fetalis and a thoracic mass. The child could not be operated upon because of rapid clinical deterioration. The autopsy findings confirmed the diagnosis of congenital fibrosarcoma. This is, to our knowledge, the first case of hydrops fetalis associated with fibrosarcoma. Conclusion The association of hydrops fetalis and fibrosarcoma is an exceptional observation but can be added to the long list of differential diagnoses of non-immune hydrops. Received: 28 November 1995 / Accepted: 31 May 1996     

Hydrops fetalis associated with erythrocyte pyruvate kinase deficiency

The authors report a case of hydrops fetalis due to severe pyruvate kinase deficiency, the most unusual clinical manifestation of this disease. Conclusion Pyruvate kinase deficiency, as other erythrocyte enzymopathies, must be considered in the differential diagnosis of non-immune hydrops fetalis. This has important implications for clinical investigations, therapy and genetic counselling. Received: 15 November 1998 / Accepted in revised form: 17 April 1999     

Fetal ebstein anomaly — A rare cause of non-immune hydrops

A rare case of fetal Ebstein anomaly presenting as non-immune hydrops fetalis at 28 weeks of gestation is reported alongwith its review of literature.     

Non-immunologic hydrops fetalis (NIHF)--case report of double partial trisomy 15q and 17q resulting from familial translocation 15/17 and cytogenetic findings in 50 cases with hydrops fetalis

We report the first case of non-immune hydrops fetalis (NIHF) with trisomy 15q11/17q22.5 resulting from a familial translocation 15/17. Furthermore the cytogenetic findings of 50 cases with hydrops fetalis are presented. Of the 30 cytogenetic analyzable cases 7 showed chromosomal abnormalities (4x45,X; 2x47,XY,+21; 1x47,XY,+13). In every case of NIHF a chromosomal analysis should be performed if possible from different tissues.     

Fatal course of ABO hemolytic disease associated with hydrops in a twin pregnancy

Hydrops fetalis associated with ABO incompatibility is an extremely rare condition. We report twin infants both afflicted with significant ABO hemolytic disease but showing different degrees of clinical severity, in which fatal hydrops developed in one of the twins. Hemolysis due to ABO incompatibility is usually difficult to diagnose. All causes of non-immune hydrops should be ruled out in order to identify hydrops due to ABO incompatibility.     

Bilateral subdural effusion (hygromas) in non-immune hydrops fetalis

In addition to body edema fluid accumulation in serous cavities is a component of non-immune hydrops fetalis. A case with bilateral symmetrical subdural effusion is reported. Fluid accumulation in this space appears to be a part of this entity.     

Nomimmune hydrops fetalis due to Diamond-Blackfan anemia

We describe case report of a baby with Diamond-Blackfan anemia, who presented as non-immune hydrops fetalis. The diagnosis was confirmed by measurement of red cell adenosine deaminase activity which is increased in Diamond-Blackfan anemia. At 2 years of age he is dependent on small dose of alternate day steroid to maintain his hemoglobin.     

Hydrops fetalis, hydramnios and hepatic vascular malformation associated with cutaneous hemangioma and chorioangioma.

A premature baby presented with severe hydrops fetalis due to a multifocal angiomatous malformation of the liver. There were two other small vascular tumors: hemangioma of the skin and chorioangioma. Hydramnios and placental edema were also present. The association of severe hydrops fetalis and hydramnios with angiomatous malformation of the liver was not found in reviewing the literature.     

HYDROPS FETALIS, HYDRAMNIOS AND HEPATIC VASCULAR MALFORMATION ASSOCIATED WITH CUTANEOUS HEMANGIOMA AND CHORIOANGIOMA

ABSTRACT. A premature baby presented with severe hydrops fetalis due to a multifocal angiomatous malformation of the liver. There were two other small vascular tumors: hemangioma of the skin and chorioangioma. Hydramnios and placental edema were also present. The association of severe hydrops fetalis and hydramnios with angiomatous malformation of the liver was not found in reviewing the literature.     

De novo translocation t(5;9)(q11.2;p22) associated with hydrops fetalis and cystic hygroma

We report on a case of a prenatally diagnosed non-immune hydrops fetalis and cystic hygroma associated with the balanced translocation t(5;9)(q11.2;p22), an association that to our knowledge has not been reported previously. Both parents had normal karyotypes. The infant was born prematurely at 33 and 3/7 weeks gestation and expired 12 h after delivery.     

Pre- and postnatal treatment of a pulmonary sequestration presenting as a unilateral hydrothorax

We report a case of a neonatal sequestration diagnosed antenatally as a progressive left hydrothorax when ultrasound (US) was done at 30 weeks' gestation for polyhydramnios. Postnatal US and computed tomography failed to demonstrate any lesion; magnetic resonance imaging showed a spherical mass in the left chest adjacent to the left lower lobe. At the age of 4 weeks a thoracotomy was performed and the mass was excised. Pulmonary sequestrations may present with a pleural effusion and polyhydramnios as a part of non-immune hydrops fetalis. In our case hydrops was prevented by insertion of a pleuro-amniotic shunt.     

A case of non-immune hydrops fetalis with congenital cystic adenomatoid malformation of the left lung in a twin

Hydrops fetalis is a rare condition that occurs in one out of every 1,400-4,000 pregnancies. There are two types, immune and non-immune. It can be caused by a maternal-fetal incompatibility due to the Rh factor or, more rarely, the Kell factor or due to other antigens to red corpuscles. Non-immune types of hydrops have a complex multiform pathogenesis and in 50% of the cases they are of idiopathic nature. The causes that could determine non-immune hydrops are manifold and in 50% of the cases they are of an idiopathic nature. Independently of the clinical condition, the presented case could be classified in those forms of pathogenesis in which there is the presence of a congenital cystic adenomatoid malformation (CAM), not encountered in the fetal ultrasound performed at the sixteenth week of pregnancy. The particularity of our case is due to the association of the fetal hydrops of the CAM type in only one of the two twins. The presented case is classified in those forms of hydrops that are expressed with a clinical trend towards a worse prognosis, characterized by the intrauterine death of the fetus or the birth of a hydropic fetus that does not survive because of respiratory insufficiency and cardiocirculatory collapse.     

Cardiac Abnormalities and Nonimmune Hydrops Fetalis: a Coincidental, Not Causal, Relationship

Few conditions associated with nonimmune hydrops fetalis have had a demonstrable causal relationship. Congenital heart disease is often said to be a cause of nonimmune hydrops fetalis and antenatal closure of the foramen ovale is the cardiac abnormality most frequently reported in association with hydrops. In order to examine the role of congenital heart disease in hydrops, and, in particular, that of antenatal closure of the foramen ovale, we reviewed all autopsy cases with hydrops fetalis over an 11 year period and compared cardiac anomalies with those of nonhydropic controls. The incidence of various congenital heart malformations was not significantly different among these groups, suggesting that factors in addition to cardiac anomalies must be considered in the pathogenesis of nonimmune hydrops fetalis.