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| Diagnosis | |
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Ultrasound can be involved in diagnosis.This anomaly appears during the beginning or end of the fourth week of the fetus' development, when the anterior neuropore closes. The desmocranium normally becomes the epidermis of the scalp, but in acrania the desmocranium remains a membranous coverage. Therefore, the migration of mesenchmal tissue under the calvarium ectoderm does not happen; leading to an absence of the calvarial dermal bones of the skull, related musculature, and dura mater [1]. The brain may still develop normally, but without the walls of the cranium shaping it, it will not differentiate between the two hemispheres and will morph into a single mass that will not be able to sustain life. Acrania is in all cases lethal; no human being can live without the skull and there is no technology that is able to keep victims of this anomaly alive for a longer time. The difference between acrania and anencephaly is that acrania is the absence of the cranium but anencephaly is the absence of the brain. The brain can still be formed in acrania.[2]
[edit] Tags:Cranium,Anencephaly,Edit,Ultrasound,Brain,M,N,S,P,E,B,D,C,A,F,L,G,H,V, | |
| References | |
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^ a b c Acrania: review of 13 cases - Tae-Hee Kwon, MD*, Jim King, MD, Philippe Jeanty, MD, PhD
^ Liu IF, Chang CH, Yu CH, Cheng YC, Chang FM (2005). "Prenatal diagnosis of fetal acrania using three-dimensional ultrasound". Ultrasound in medicine & biology 31 (2): 175–178. doi:10.1016/j.ultrasmedbio.2004.10.005. PMID 15708455.
Acalvaria (Acrania) at NIH's Office of Rare Diseases
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Congenital malformations and deformations of nervous system (Q00–Q07, 740–742)
Brain
Neural tube defect
Anencephaly (Acephaly, Acrania, Acalvaria, Iniencephaly) · Encephalocele · Arnold–Chiari malformation
Other
Microcephaly · Congenital hydrocephalus (Dandy–Walker syndrome)
other reduction deformities (Holoprosencephaly, Lissencephaly, Pachygyria, Hydranencephaly)
Septo-optic dysplasia · Megalencephaly
CNS cyst (Porencephaly, Schizencephaly)
Polymicrogyria (Bilateral frontoparietal polymicrogyria)
Spinal cord
Neural tube defect
Spina bifida · Rachischisis
Other
Currarino syndrome · Diastomatomyelia · Syringomyelia
M: CNS
anat(n/s/m/p/4/e/b/d/c/a/f/l/g)/phys/devp
noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr
proc, drug(N1A/2AB/C/3/4/7A/B/C/D)
This genetic disorder article is a stub. You can help Wikipedia by expanding it.
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Retrieved from "http://en.wikipedia.org/w/index.php?title=Acrania&oldid=468295715"
Categories: Congenital disorders of nervous systemGenetic disorder stubs
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Tags:10,Acalvaria,Nih,Rare Diseases,Congenital,Nervous System,Q00–q07,740–742,Neural Tube Defect,Acephaly,Arnold–chiari Malformation,Congenital Hydrocephalus,Dandy–walker Syndrome,Holoprosencephaly,Lissencephaly,Pachygyria,Hydranencephaly,Megalencephaly,Cns Cyst,Porencephaly,Schizencephaly,Polymicrogyria,Bilateral Frontoparietal Polymicrogyria,Spinal Cord,Rachischisis,Anat,4,Phys,Noco, | |
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