Cases reported "Encephalomalacia"

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1/13. arthrogryposis and multicystic encephalopathy after acute fetal distress in the end stage of gestation.

    The natural history of the rare association "multicystic encephalopathy-arthrogryposis" was traced in a fetus carefully followed after artificial insemination. The fetus exhibited normal viability and brain morphology up to the 32nd week. At 36 weeks, active movements diminished and at 37 weeks, hydramnios and signs of fetal distress led to cesarean section. The infant presented with severe arthrogryposis of the limbs and spine, but not with the other elements of a long-lasting akinesia. US showed multicystic encephalopathy. Both the clinical and the neuropathological findings established that multicystic encephalopathy was neither the cause nor the sequential consequence of the fetal akinesia, but the result of a recent diffuse, acute malacic process that also involved the anterior horn cells. Acute fetal distress, responsible for major ischemic damage to CNS but compatible with fetal survival, remains an obscure condition which allows for the development of severe arthrogryposis in a few weeks.
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2/13. A case of pulmonary hypoplasia associated with intrauterine brainstem necrosis.

    An infant with intrauterine brain death accompanied by pulmonary hypoplasia is reported. The fetus was delivered after 36 weeks gestation, 5 weeks after fetal movements ceased. The child died 4 h after birth. Pulmonary hypoplasia and remote brainstem necrosis associated with multicystic encephalomalacia were found at autopsy. CONCLUSION: These findings suggest that damage to brainstem respiratory centres had led to pulmonary hypoplasia through the absence of fetal respiratory movement.
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3/13. Multicystic encephalomalacia.

    Antepartum death of a fetus in a multiple gestation can be associated with mortality or major morbidity in the survivor. This article reports a rare case of multicystic encephalomalacia that occurred in the survivor twin with antepartum death of its co-twin. Its pathology and pathogenesis are discussed with review of literature.
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4/13. Brain damage to the survivor within 30 min of co-twin demise in monochorionic twins.

    Single fetal death in a twin pregnancy in the late second or early third trimester is associated with significant morbidity and mortality rate in the surviving co-twin, especially in monochorionic twin pregnancies. The common causes are twin-to-twin transfusion syndrome, chromosomal abnormalities, and congenital anomalies of the fetus or anomalies of the umbilical cord-placenta. Here we report a case of monochorionic twin pregnancy in which one fetus had a single umbilical artery (SUA) while the co-twin had two umbilical arteries. The twin with SUA died in utero at the 30th week of gestation and the other fetus was delivered by cesarean section immediately due to fetal distress diagnosed by cardiotocography. disseminated intravascular coagulation and multicystic encephalomalacia have been observed in the surviving neonate. This case and review of the literature suggest that neurologic complication rates are also increased in monochorionic twin pregnancies with single fetal demise despite the immediate delivery as in our case.
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keywords = gestation, pregnancy
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5/13. Cardiotocographic and Doppler ultrasonographic findings in a fetus with brain death syndrome.

    OBJECTIVE: The diagnosis of brain death syndrome by cardiotocography (CTG) and Doppler ultrasonography (US) is reported in a fetus at 35 weeks of gestation. CASE REPORT: A 23-year-old, gravida 2, para 0, woman was referred to our hospital because of the absence of fetal movements. CTG showed fixed fetal heart rate (FHR) pattern. A detailed Doppler US examination of the fetus showed extensive cystic lesions of both cerebral hemispheres, polyhydramnios, total absence of neuromuscular parameters of biophysical profile (BPP) and the cessation of cerebral blood flow. umbilical cord artery blood gas analysis showed pH 7.3, PaO2 30 mmHg and PaCO2 35 mmHg. A floppy male infant weighing 2,450 g was delivered vaginally at 36 weeks of gestation and the Apgar scores were 1 and 1 at 5 and 10 minutes, respectively. The neonate died 2 days after delivery. Postmortem examination of the brain showed diffuse, anoxic changes with multicystic encephalomalacia in both hemispheres and the brain stem. No other maternal or placental abnormalities were seen. CONCLUSION: The possibility of intrauterine brain death should be considered in all cases of prolonged fixed FHR pattern, accompanied by absence of neuromuscular parameters of BPP, polyhydramnios and demonstrated cessation of cerebral blood flow by Doppler US. Increased awareness of this event may prevent unnecessary emergency cesarean section.
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6/13. Periventricular leukomalacia: ultrasonic and neuropathological correlations.

    Ultrasound scans of a preterm neonate (27 weeks gestation) at seven weeks of age showed periventricular echo-free cavities, but these were no longer visible at 15 weeks, three weeks before the infant died. At autopsy, a linear glial scar, extending from the periventricular white-matter into the white axis of the parasagittal gyrus, was found in the area occupied by the periventricular cysts. The larger cavity was reduced to a slit-like excavation in the midst of glial tissue. Unsuspected focal infarcts in the cerebral cortex were also found. This observation demonstrates that transient echo-free cavities represent foci of cystic necrosis, which are subject to secondary collapse. In the authors' experience, the linear extension of periventricular leukomalacia (PVL) into the core of parasagittal gyri is a frequent feature of PVL, and one which cannot easily be accounted for by the usual explanations of border-zone ischaemic softening.
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7/13. Congenital microcephaly due to vascular disruption: in utero documentation.

    Death in utero of one member of a monozygotic twin pair has been associated with vascular disruptive phenomena in the surviving twin. It has been hypothesized that this event initiates clot formation in the surviving twin with consequent necrosis of tissues distal to the occluded vessels. This case report describes onset in utero of multicystic encephalomalacia and microcephaly in a surviving twin whose brain appeared normal on ultrasound scanning before death of the cotwin at 21 weeks' gestation. The case provides further support for the hypothesized pathogenetic sequence and illustrates the importance of reviewing all prenatal ultrasound scans in infants with congenital microcephaly.
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8/13. Multicystic encephalomalacia in liveborn twin with a stillborn macerated co-twin.

    Computed tomography showed multiple cystic lesions in the brains of three infants with cerebral palsy. Each patient was the product of a twin pregnancy with a stillborn, macerated co-twin. In two patients angiography was performed and suggested that the cystic lesions were multicystic encephalomalacia due to perinatal arterial occlusion.
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keywords = pregnancy
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9/13. Multicystic encephalomalacia of infancy: clinico-pathological report of 7 cases.

    Clinical follow up and complete neuropathological examination was made on seven cases of multicystic encephalomalacia of infancy. Etiological factors were carefully studied in all the cases. They consisted of prenatal injuries presenting as a cord prolapse, in 3 cases; prolonged labour with marked cyanosis; abdominal trauma during gestation, and various maternal infections at different stages of pregnancy. Pathological interest is centred on the variable involvement of different areas of the brain, generally sparing the cerebellum and brain stem, and being minimal or absent in the occipito-temporal areas. This distribution may be explained by a different effect of the "causal agency" on these different areas, or by a different capacity of these regions to react against injury. Among the etiological factors reviewed in the literature, the anoxic theory appears the most probable, as there was a close parallelism between lesions and vascular areas, mainly the carotid and vertebro-basilar systems.
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keywords = gestation, pregnancy
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10/13. Multicystic encephalomalacia due to fetal viral encephalitis.

    Two children, aged 2 weeks and 2 months, with multicystic encephalomalacia aer described. Although computerized tomography (CT) scan was used for the diagnosis, the value of the simple method of transillumination is stressed. The mother of one child had mumps with meningitis at 26 weeks' gestation. The other child had an echovirus 11 isolated from the cerebrospinal fluid and herpesvirus hominis (HVH) from the skin at 8 weeks. We speculate that the changes in the brains of these babies may be due to the respective viruses causing a fetal encephalitis.
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