Short answer · Medically reviewed summary · Last updated: 2026-05-08

Encephalocele, a neural tube defect characterized by a sac-like protrusion of the brain and its membranes through an opening in the skull, is most commonly referred to as a cranium bifidum. While medical terminology has evolved, encephalocele remains the standard term used in modern clinical practice, though it is sometimes categorized by its specific anatomical location, such as occipital or frontal encephalocele. What are the common synonyms and historical names for Encephalocele? In medical literature, you may encounter several terms used to describe encephalocele.

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Encephalocele synonyms

Other names for Encephalocele: synonyms, acronyms and related terms used by doctors and patients.

Encephalocele is also known as...

Encephalocele, a neural tube defect characterized by a sac-like protrusion of the brain and its membranes through an opening in the skull, is most commonly referred to as a cranium bifidum. While medical terminology has evolved, encephalocele remains the standard term used in modern clinical practice, though it is sometimes categorized by its specific anatomical location, such as occipital or frontal encephalocele.



What are the common synonyms and historical names for Encephalocele?


In medical literature, you may encounter several terms used to describe encephalocele. Historically, the condition was frequently referred to as cranium bifidum, a term that describes the defect in the skull bones. Depending on the specific contents of the herniated sac, doctors may use more precise descriptors:



  • Meningocele: A sac containing only the meninges (membranes) and cerebrospinal fluid.

  • Encephalomeningocele: A sac containing both brain tissue and meninges.

  • Encephalomyelocele: A rare term used when the brain and spinal cord tissues are involved.

  • Atretic encephalocele: A small, skin-covered subscalp lesion that contains fibrous tissue and rests of glial cells.



Why does Encephalocele have multiple names in medical records?


The variety of names for encephalocele exists primarily due to historical classification efforts and the need to describe the exact anatomical contents of the protrusion. Older literature often used general terms like "cranial hernia," while modern genetics and neurosurgery prefer specific terminology defined by the site of the skull defect (e.g., occipital, parietal, or basal encephalocele). These distinctions are critical for surgical planning and understanding the prognosis for the 27 members of our DiseaseMaps community and others living with the condition.



How is Encephalocele classified in medical systems?


Major medical classification systems standardize the diagnosis to ensure consistency. Encephalocele is classified under the following codes:


  1. ICD-10/11: Categorized under congenital malformations of the nervous system (e.g., Q01).

  2. Orphanet: Listed as ORPHA:1395, recognizing it as a rare disease.

  3. OMIM: Documented under various entries depending on the associated genetic syndromes (e.g., #236100 for Meckel syndrome).




Next steps



  • Consult with a pediatric neurosurgeon to discuss the specific classification of your or your child's encephalocele.

  • Request a referral to a clinical geneticist to determine if the encephalocele is an isolated finding or part of a broader genetic syndrome.

  • Connect with the 27 encephalocele community members on DiseaseMaps.org to share experiences and resources.



Medical disclaimer: This information is for educational purposes only and should not replace professional medical advice, diagnosis, or treatment.



References



  • NIH Genetic and Rare Diseases Information Center (GARD): Encephalocele.

  • Orphanet: Rare disease database, ORPHA:1395.

  • Online Mendelian Inheritance in Man (OMIM): Clinical synopsis for neural tube defects.

  • The Spina Bifida Association: Resources on neural tube defects and cranial malformations.

Author: DiseaseMaps Editorial Team
Reviewed against authoritative medical sources (NIH GARD, Orphanet, OMIM)
Last updated: 2026-05-08
Medical disclaimer: This information does not substitute professional medical advice. Always consult your doctor before making health decisions.
Source: DiseaseMaps.org
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