Intrauterine Brain Damage in Nonimmune Hydrops Fetalis

Abstract

Nonimmune hydrops fetalis has been described in a large number of pathological conditions, but brain aspect has been poorly explored. We report the neuropathological findings in a series of 38 fetuses and neonates with anasarca of various origin. Fourteen fetal cerebral ultrasonograms were available; 8 presented some abnormalities. On brain examination, 23 cases showed hypoxic-ischemic lesions. The white matter was the main site of damage that consisted in classical leucomalacia or other features such as: astrocytic glial reaction, micro-calcifications and microthromboses either as isolated finding or in association. Anoxic neuronal damage was much less frequent. Anemia, hypoprotidemia and cardiac failure with hypotension, which often occur in hydrops fetalis, may account for brain perfusion failure and hypoxic-ischemic changes.

Perinatal Lethal Gaucher Disease: A Distinct Phenotype Along the Neuronopathic Continuum

“…Hence, the development of hydrops is generally associated with a poor prognosis and may contribute to the severe neurological deterioration. Pulmonary hypoplasia or liver failure are the common causes of fetal or neonatal demise [14,29]. As expected, there is an association between hydrops fetalis and prematurity and neonatal distress.…”

Non‐immune hydrops fetalis: A prospective study of 53 cases

“…Therefore, unlike of the retrospective series, in the prospective ones it is expected that the systematic study helps to reduce the number of idiopathic. Piraud et al [1996] Fetus (mainly 3rd trimester) 8/54 14.8 ISSD, galactosialidosis (2), Gaucher disease, MPS VII (2), sialidosis (2) Groener et al [1999] Fetus (20-36 weeks) 1/17 5.8 MPS VII Burin et al [2004] Fetus (16-32 weeks) and newborn 5/33 15.0 Galactosialidosis, MPS IVA, mucolipidosis II, NP-A, sialidosis Kooper et al [2006] Fetus (14-36 weeks) 6/75 8.0 Gangliosidosis GM1, galactosialidosis, MPS VII (2), MPS-probable (2) Total 20/179 11.2 IEM in NIHF-general investigation Etches and Lemons [1979] SB, LB 1/22 4.5 LSD-not specified Van Aerde et al [1982] SB, LB 1/10 10.0 Gangliosidosis GM1 Mahony et al [1984] Fetus 1/27 3.7 MPS-not specified Im et al [1984] A, SB, LB 3/20 15.0 Gaucher disease Mostoufi-Zadeh et al [1985] SB, LB-autopsy 1/40 2.5 Gaucher disease McFadden and Taylor [1989] Autopsy 4/90 4.4 Unespecified metabolic disease Larroche et al [1992] SB, LB 3/38 7.9 LSD-not specified Laneri et al [1994] SB, LB 1/45 2.2 Tyrosinemia McCoy et al [1995] Fetus (>20 weeks GA) 2/82 2.4 Gaucher, NP-C Rejjal et al [1996] LB 2/17 11.7…”

“…For instance, the casuistic were not uniform with respect to the included individuals (live births, stillbirths or neonatal deaths). Furthermore, the series of cases were differently studied-some studies included specific tests through of biochemical assays [Piraud et al, 1996;Groener et al, 1999;Burin et al, 2004;Kooper et al, 2006] versus others that used indirect signs like evidence of autopsy [Larroche et al, 1992]. As it was expected the studies whose goal was the specific IEM investigation presented a higher IEM frequency than in those whose purpose was a general evaluation of the NIHF (Table III).…”

Nonimmune Hydrops fetalis: Fetal and Neonatal Outcome during 1983–1992

“…Larroche et al [27] demonstrated that hypoxic-ischemic cerebral lesions are common in NIHF and tend to occur already in utero. Perhaps as a result of selection bias, severity of hydrops being associated with mortality, cranial ultrasound results in the long-term survivors of the present cohort show few abnormalities.…”