title: Effects of ischaemia and hypoxia on the development of the nervous system in acardiac foetus.
abstract: The twin-reversed arterial perfusion (TRAP) sequence and development of an acardius are rare and severe complications in monozygotic twin pregnancy. Haemodynamic disturbances in placental perfusion via abnormal vascular anastomoses allow inter-twin transfusion to occur. Because of blood perfusion, one of the twins is poorly oxygenated and contains metabolic waste products. Retrograde placental perfusion leads to the formation of a non-viable malformed acardiac foetus. We studied the effects of haemodynamic disturbances in acardiac foetus on the development of the nervous system. The acardius was a product of a 32-weeks pregnancy. Caesarean section yielded a skin covered ovoid mass (size, 10 x 8 cm; weight, 220 g). The dissection of the acardiac twin showed a skin with hair and appendages, rudimentary lower limbs, vertebral column and brain mass. The rudimentary brain tissue was considerably disorganised structurally. We distinguished two main morphological forms of various appearances. In the centre, we observed a scarcely vascularised mass of tissue containing mature and immature neurones, glial cells and randomly distributed fibres. The mass of tissue appeared poorly differentiated, although there were some arrangements reminiscent of cerebral structures. Clusters of neurones provided a slight suggestion of nuclear or fibre structure. The cerebellar cortex was the only well recognisable structure. In the other fragment of the tissue, we found a slit cavity with ependymal outline and well-developed choroid plexus, which seemed to represent the 3rd ventricle. The scarcely vascularised disorganised tissue was surrounded by the highly vascularised one. It included many thin-walled sinusoid vessels. In some places, they were so concentrated that they resembled cavernous haemangioma. The spinal cord appeared comparatively well organised with a slightly dilated central canal. The morphological picture of the rudimentary brain tissue was similar to the picture of the cerebrovasculosa area. The effect of ischaemia in the presented case is the anomalous formation of the cerebral structures. The morphological features imply that the failure occurred after neurulation and before the prosencephalic began to grow. The failure of neural tube formation occurred on the 22nd-25th day of gestation. The malformed formation of the nervous system might be caused by impaired induction due to altered gene expression or to the interference of exogenous agents that interrupt normal development. The haemodynamic abnormal placental circulation, which induced lack of oxygen supply and nutritional deficiency, implies the morphological pattern of the anomaly.
authors.affiliation: Department of Experimental and Clinical Neuropathology, Mossakowski Medical Research Centre, Polish Academy of Sciences, Warszawa, Poland. milenak@cmdik.pan.pl
authors.forename: Milena
authors.initials: M
authors.lastname: Laure-Kamionowska
authors.forename: Danuta
authors.initials: D
authors.lastname: Maślińska
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Deregowski
authors.forename: Paweł
authors.initials: P
authors.lastname: Piekarski
authors.forename: Barbara
authors.initials: B
authors.lastname: Raczkowska
mesh_terms: D000015:Abnormalities, Multiple / Q000209:etiology / Q000473:pathology; D000328:Adult; D005260:Female; D005330:Fetofetal Transfusion / Q000150:complications* / Q000503:physiopathology; D006330:Heart Defects, Congenital / Q000209:etiology* / Q000473:pathology; D006801:Humans; D020925:Hypoxia-Ischemia, Brain / Q000503:physiopathology*; D009420:Nervous System / Q000196:embryology*; D009421:Nervous System Malformations / Q000473:pathology*; D010920:Placenta / Q000473:pathology; D011247:Pregnancy; D014310:Triplets