Exencephaly—Clinical and Ultrasonic Correlation to Anencephaly

Hendricks, Susan K.; Cyr, D R; Nyberg, David A.; Raabe, Rodney D.; Mack, L A

doi:10.1097/00006250-198812000-00018

Cited by 40 publications

(25 citation statements)

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“…Our second and third cases demonstrate that the specific diagnosis of meroanencephaly can be made in utero, and that it is possible to recognize this entity and differentiate it from an exencephaly [4],…”

Section: Discussionmentioning

confidence: 62%

Meroanencephaly: Pathology and Prenatal Diagnosis

Qureshi¹,

Jacques²,

Holzgreve

et al. 1993

Fetal Diagn Ther

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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 ectoptic 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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Section: Discussionmentioning

confidence: 62%

Meroanencephaly: Pathology and Prenatal Diagnosis

Qureshi¹,

Jacques²,

Holzgreve

et al. 1993

Fetal Diagn Ther

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“…Differentiation from massive meningoencepha- locele is based on the identification of the fetal brain without the surrounding flat bones of the calvaria [1], In addition, the marked dysplasia and disorganized devel opment of the rudimentary brain tissue are atypical of meningoencephalocele [1]. This sonographic picture cor relates with the pathologic findings [2], Exencephaly has been associated with other anoma lies consistent with amniotic band syndrome [2]. Other associated defects of spine, abdominal wall and limbs have been noted [6].…”

Section: Discussionmentioning

confidence: 99%

“…The sonographic finding of exencephaly consists of a large amount of disorganized cerebral tissue arising from the base of the cranium [1,2]. Facial structures are pre served.…”

Section: Discussionmentioning

confidence: 99%

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Antenatal Sonographic Diagnosis of Exencephaly

Musa¹,

Hata²,

Senoh³

et al. 1990

Gynecol Obstet Invest

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“…Evidence indicates that exencephaly is a precursor stage of anencephaly. 7 Cephalocele is a defect in the bony skull through which the meninges (superior meningocele) and brain substance (encephalomeningocele) may protrude. The incidence is 1:2000 live births.…”

Section: Neural Tube Defects (Ntd)mentioning

confidence: 99%

Ultrasound and Diagnosis of Fetal Anomalies

Gliozheni¹,

Sylejmani²,

Kati³

2011

Donald School Journal of Ultrasound in Obstetrics and Gynecology

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Background: Congenital abnormalities account 20 to 25% of perinatal deaths. Now, many genetic and other disorders can be diagnosed early in pregnancy. Screening examinations during pregnancy are an essential part of prenatal care. Among the various screening tests that are now offered to pregnant women, ultrasound (US) has the broadest diagnostic spectrum. There is no modality that can detect as many abnormalities during pregnancy as US. A priority goal in screening is the early detection of major fetal anomalies, which are defined as malformations that affect fetal viability and/or quality of life. During the past 10 years, some multicentric studies in Europe and USA show the successfulness of US diagnostics in detecting congenital abnormalities, even in women with low-risk pregnancy. The term sonoembryology designates the description of the embryonic anatomy, the normal anatomic relations and the development of abnormalities as visualized by ultrasound. To confirm the presence of normal anatomy or to make the diagnosis of an anomaly, we need knowledge of the normal embryonic development, including the appearance of the normal embryo.

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Exencephaly—Clinical and Ultrasonic Correlation to Anencephaly

Cited by 40 publications

References 0 publications

Meroanencephaly: Pathology and Prenatal Diagnosis

Meroanencephaly: Pathology and Prenatal Diagnosis

Antenatal Sonographic Diagnosis of Exencephaly

Ultrasound and Diagnosis of Fetal Anomalies

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