Skip to Main Content


A disorder of neuronal migration characterized by partial or complete agenesis of the corpus callosum.


Corpus callosum agenesis (CCA) is the most common cerebral malformation. It has been estimated that 0.05 to 0.7% of the general population and 2.3% of children with developmental disabilities are affected.


Agenesis of the corpus callosum has been associated with several chromosomal rearrangements. These include autosomal dominant, autosomal recessive, and X-linked inherited syndromes.


Unknown. An insult to the commissural plate during embryogenesis interferes with migration of the cells that form the corpus callosum. This insult can be a result of chromosomal abnormalities, be part of a syndrome, a migration, or a metabolic disorder. CCA may be an isolated anomaly or part of a syndrome with other, more extensive malformations or metabolic or genetic disorders. The corpus callosum is formed between the gestational weeks 7 and 20. Consequently, partial or complete CCA may occur if this process is disrupted. Because major parts of the cortex and cerebellum develop at the same time, associated anomalies must always be excluded. As a rule, formation of the corpus callosum starts in the front and continues to the back (holoprosencephaly is the main exception to this rule), which explains why partial callosal agenesis usually involves the posterior portion of the corpus callosum.


Based on the clinical findings in children undergoing extensive examination for epilepsy, cognitive impairment, or, less often, behavioral problems. Computed tomography or magnetic resonance imaging confirms the absence of the corpus callosum. Frequently, there is also upward displacement and enlargement of the third ventricle (because the corpus callosum normally forms the roof of the third ventricle), widely spaced dorsal horns, and possible evidence of other migration disorders.


Clinically and prognostically, CCA can be divided into two types. Type I is not associated with other disorders. Patients may have mild-to-moderate mental retardation and no or only mild neurologic manifestations, which may include seizure disorders and impaired visual, motor, and coordination skills. A peculiar facies with prominent forehead, macrocephaly or microcephaly, deep-set eyes, and preauricular skin tags is common. However, some patients have no clinical signs, which makes parental counseling difficult in the absence of radiologic and genetic markers allowing determination of future asymptomatic from symptomatic disease. Type II is associated with other migration, genetic, and chromosomal abnormalities, usually resulting in severe neurologic manifestations, which may include severe mental retardation, microcephaly, hemiparesis, spasticity, seizure disorder, and failure to thrive. This condition often is accompanied by recurrent bronchopneumonia and, in the severest cases, early death in infancy.


Check for associated abnormalities, particularly metabolic disorders. Assess for complications such as seizures, paralysis, and bronchopneumonia. Preoperative investigations should include a chest radiograph, pulmonary function tests (if possible), and arterial blood gas analysis. Assess for difficult airway management in case of microcephaly. Mental retardation may affect patient cooperation. Sedative and/or anxiolytic premedication and/or the presence of the primary caregiver during induction of ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.


Create a Free MyAccess Profile

* Required Fields

Note: If you have registered for a MyAccess profile on any of the Access sites, you can use the same MyAccess login credentials across all sites.

Passwords must be between 6 and 40 characters long (no whitespace), cannot contain characters #, &, and must contain:
  • at least one lowercase letter
  • at least one uppercase letter
  • at least one digit

Benefits of a MyAccess Profile:

  • Remote access to the site off-campus on any device
  • Notification of new content via custom alerts
  • Bookmark your favorite content such as chapters, figures, tables, videos, cases and more
  • Save and download images to PowerPoint
  • Self-Assessment quizzes saved for quick review
  • Custom Curriculum access for both instructors and learners

Subscription Options

AccessAnesthesiology Full Site: One-Year Subscription

Connect to the full suite of AccessAnesthesiology content and resources including procedural videos, interactive self-assessment, real-life cases, 20+ textbooks, and more

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessAnesthesiology

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.