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Biomedical subjects

F Narcy

Publications and source records attributed to F Narcy.

28 records · Page 2Linked to original sources
PubMed1993

Prenatal management of congenital cystic adenomatoid malformation of the lung.

In utero evolution and postnatal outcome were studied for 18 cases of congenital adenomatoid malformation (CAM) diagnosed by prenatal ultrasound. Five were macrocystic CAM, 9 were microcystic, and 4 were homogeneously hyperechogenic. Three fetuses presented with hydrops. Pulmonary amniotic shunting was performed in 3 patients. Outcome was the following: 4 were aborted, 1 died neonatally, and 13 survived. Four of these infants required no surgery in the neonatal or postneonatal period. In three of these, the size of the mass had decreased spontaneously in utero. Outcome did not appear to be related to the anatomic type of CAM nor to the presence of moderate polyhydramnios, but was related to the degree of mediastinal compression and to the existence of hydrops. A clearer understanding of the natural evolution of CAM is useful to determine the indications for in utero therapy.

Cystic Adenomatoid Malformation of Lung, Congenita↗
PubMed1992

Intrauterine brain damage in nonimmune hydrops fetalis.

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, microcalcifications 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.

Brain↗
PubMed1990

Brain damage in monozygous twins.

A series of 15 monochorionic twins with a great variety of cerebral lesions is reported. Seven cases illustrate the classical situation: the recipient twin was affected and his co-twin, the donor was macerated. In 5 cases, the lesions were described in the donor twin as well and once, as early as 22 weeks. The lesions were usually hypoxic-ischemic, in 2 they were hemorrhagic. In 1 case there was a malformation. Fetal US were performed in 11 cases and the diagnosis of either IUGR, death of a fetus and/or brain lesions in the survivor could be made in 10 cases and once as early as 21 weeks. In fetuses born alive, transfontanellar US or CT scan have confirmed the diagnosis made on fetal US. The pathogenesis of the lesions is not fully understood. Lesions in the recipient twin may result from emboli or thromboplastic material originating from the macerated co-twin. We suggest that blood pressure instability or episodes of severe hypotension might as well lead to brain and/or visceral lesions in the recipient twin. In the donor, the lesions result from hypotension and/or anemia. With improvement and generalization of imaging techniques, the vitality of the fetuses as well as biometric parameters and anatomical structures will be better controlled. However, in case of a fetal death, occurrence of lesions in the survivor is unpredictable and no uniform policy has been proposed yet. Studies with Doppler and continuous monitoring of funicular circulation should improve our knowledge on feto-fetal transfusion and permit to detect hemodynamic fluctuation or impairment.

Brain Injuries↗
PubMed1990

Cerebral midline developmental anomalies: spectrum and associated features.

Cerebral midline anomalies are defects of anatomical relationships between the two hemispheres. They include holoprosencephalies, septal and commissural agenesis. Agenesis of the olfactory tract (arhinencephalies) are often included in the spectrum of holoprosencephalies and the facial phenotype is thought to be affected and characteristic in the midline development abnormalities. This work concerns a review of the literature and personal experience in two units of Fetopathology in Paris. This study confirms the relationships between various cerebral malformations and their frequent association. However, arhinencephaly and moreover agenesis of corpus callosum should be considered as heterogeneous entities, often totally distinct and independent from the malformative process of the holoprosencephaly. In addition, if major facial anomalies such as cyclopia are almost pathognomonic for holoprosencephaly, minor malformations such as lateral facial clefts of cleft palates result from a great variety of malformative processes.

Abnormalities, Multiple↗
PubMed1989

Lateral cricoid cuts as an adjunctive measure to enlarge the stenotic subglottic airway: an anatomic study.

The technique of laryngotracheoplasty, with an anterior approach, with or without a posterior cut, and with or without anterior or posterior cartilage grafts, has been described previously. On occasion, a severely stenotic subglottis or aberrant shape to the cricoid cartilage makes division of the lateral aspects of the cricoid cartilage desirable. In attempting to delineate the relationship of the recurrent laryngeal nerve to proposed lateral cricoid cuts, an anatomic study was conducted. Dissections of neonatal, infant, child and adult larynges and trachea were carried out, with the relative distance of a cut through the lateral cricoid cartilage to the recurrent laryngeal nerve measured and outlined. The distance was very close in the fetal larynx (measuring 1.5 mm in the 23rd week of gestational age), with an increase in dimension in the infant and child, increasing to a distance of over 1 cm in the mature adult. The clinical significance of this relationship to proposed cuts of the lateral cricoid in different age groups is discussed.

Adolescent↗
PubMedDate not supplied

Congenital erythropoietic porphyria: prenatal diagnosis and autopsy findings in two sibling fetuses.

Congenital erythropoietic porphyria is an autosomal recessive disease characterized by a deficiency of uroporphyrinogen III cosynthetase activity, with diffuse tissue accumulation of specific type I porphyrins. The diagnosis of this disease was made in two fetuses, who were siblings, and from a Caucasian nonconsanguinous family. The first fetus died in utero with hydrops fetalis and anemia, but without an etiopathogenic diagnosis. In the second case, the diagnosis was based on pink fluorescence of the amniotic fluid examined fortuitously in sunlight. DNA analysis showed that the fetus was heteroallelic for the mutation C73R. The autopsy showed brown skin, and at histological examination, porphyrin pigment was deposited in many tissues. Retrospectively, similar deposits were found in the tissues of the first fetus.

Adult↗
PubMedDate not supplied

Fetal enteroviral infection with cerebral ventriculomegaly and cardiomyopathy.

Fetal cerebral ventriculomegaly and cardiomyopathy were diagnosed prenatally at 35 weeks and resulted in fetal death. Although there was no post mortem pathological evidence of inflammatory lesions, fetal serum alpha interferon was abnormally high (25 IU/ml). Virologic investigations were therefore carried out on stored amniotic fluid, fetal and maternal blood samples. The presence of enterovirus was retrospectively demonstrated in amniotic fluid. The association of fetal birth defects with enteroviral infection is puzzling and suggests that more attention should be given to virologic studies in the diagnostic workup of fetal malformations otherwise considered as idiopathic.

Adult↗