Abnormal Development - Anencephaly

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Q00 Anencephaly and similar malformations

  • Q00.0 Anencephaly, Acephaly, Acrania, Amyelencephaly, Hemianencephaly, Hemicephaly
  • Q00.1 Craniorachischisis
  • Q00.2 Iniencephaly

The International Classification of Diseases (ICD) World Health Organization's classification used worldwide as the standard diagnostic tool for epidemiology, health management and clinical purposes. This includes the analysis of the general health situation of population groups. It is used to monitor the incidence and prevalence of diseases and other health problems. Within this classification "congenital malformations, deformations and chromosomal abnormalities" are (Q00-Q99) but excludes "inborn errors of metabolism" (E70-E90).

ICD-10 is the 10th revision, Australian Modification (ICD-10-AM) is currently in use in Australian hospitals for admitted patients. The release date for next version update ( ICD-11) is 2018.


Anencephaly Rates (USA Data)[1]

A neural tube defect, anencephaly is a failure of the neural tube to close cranially. Also called exencephaly or craniorachischisis.

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<pubmed limit=5>Anencephaly</pubmed>

Fetal Anencephaly
Spina bifida front.JPG ZAnencephaly.jpg
ventral view lateral view


Anencephaly ultrasound.jpg

Anencephaly in a fetus (GA week 18) from a diabetic mother. Ultrasound images (coronal) show a complete absence of the cranial vault and brain and enlarged orbits.[2]

Links: maternal diabetes

Historic Anencephaly

These drawings are from a 1921 study of a single embryonic anencephaly.[3]

These drawings are from a 1925 study of 57 cases of anencephaly.[4]

Dodds GS. and Deangelis E. An anencephalic human embryo 16.5 mm long. (1937) Anat. Rec. 67(4): 499-505.


  1. Mathews TJ. Trends in spina bifida and anencephalus in the United States, 1991-2005, National Vital Statistics System.
  2. Alorainy IA, Barlas NB & Al-Boukai AA. (2010). Pictorial Essay: Infants of diabetic mothers. Indian J Radiol Imaging , 20, 174-81. PMID: 21042439 DOI.
  3. Frazer JE. Report on an anencephalic embryo. (1921) J Anat. 56(1): 12-9. PMID 17103933
  4. Nañagas JC. A comparison of the growth of the body dimensions of anencephalic human fetuses with normal fetal growth as determined by graphic analysis and empirical formulae. (1925) American J. Anatomy. 455-494.


Journal of Pediatric Neurosciences - is official publication of the Indian Society for Pediatric Neurosurgery.

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Search term: Neural Development Abnormalities | Anencephaly | Hydrocephalus | Encephalocele | Holoprosencephaly | Autism

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Cite this page: Hill, M.A. (2019, October 16) Embryology Abnormal Development - Anencephaly. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/Abnormal_Development_-_Anencephaly

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© Dr Mark Hill 2019, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G