Cases reported "Anencephaly"

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1/3. Sonographic markers of exencephaly below 10 weeks' gestation.

    We report two cases of exencephaly diagnosed by transvaginal ultrasonography at 8 weeks 4 days and at 9 weeks 3 days of gestation. Both cases presented an irregular cephalic pole, and, in the case seen at 8 weeks 4 days, brain vesicles were also absent, whereas in the case seen at 9 weeks 3 days, the midline echo was indistinguishable with disorganized choroid plexuses. In both cases, anencephaly was evident at 11 and 12 weeks' gestation and the postmortem confirmed the diagnosis.
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ranking = 1
keywords = choroid
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2/3. Percutaneous choroid plexus coagulation in hydranencephaly.

    Percutaneous intracranial endoscopy, performed on 3 infants with hydranencephaly and signs of intracranial hypertension, showed normal choroid plexus and choroid arteries in 2 patients. Transendoscopic coagulation of the arteries and plexus alleviated intracranial hypertension postoperatively. Minimal choroid plexus and no choroid arteries were present in the 3rd patient and coagulation did not alter the clinical course. Computerized tomography scans of the infants with normal choroid plexus demonstrated cerebral tissue in the region of the caudate nucleus; the child with minimal choroid plexus had tissue only in the region of the posterior thalamus.
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ranking = 10
keywords = choroid
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3/3. Meroanencephaly: pathology and prenatal diagnosis.

    Meroanencephaly is a rare form of anencephaly characterized by malformed cranial bones and a median cranial defect, through which protrudes abnormal tissue, called the area cerebrovasculosa. Area cerebrovasculosa denotes abnormal spongy, vascular tissue admixed with glial tissue ranging from a thin membrane to a large pseudoencephalic mass simulating cerebral tissue, that is composed of connective tissue, hemorrhagic vascular channels, glial nodules, and disorganized choroid plexuses. There are three types of anencephaly: (1) meroanencephaly, where there is rudimentary brain tissue and partial formation of the cranium; (2) holoanencephaly, the most common type, in which the brain is completely absent, and (3) craniorachischisis, the most severe, where area cerebrovasculosa and area medullovasculosa fill both cranial defects and the spinal column. In meroanencephaly, there is a median defect present, through which protrudes the area cerebrovasculosa. We present 3 cases of meroanencephaly diagnosed prenatally, along with histopathologic analysis. One case showed ectopic glial tissue in the lung and adrenal medullary hyperplasia. Two cases were diagnosed in the early second trimester by ultrasound scanning. Meroanencephaly may be mistaken for encephalocele both at the bedside exam and sonographically, and should be included in the differential for protruding anterior cranial masses.
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ranking = 1
keywords = choroid
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