Encephalocele

Classification according to ICD-10
Q01	Encephalocele
ICD-10 online (WHO version 2019)

Encephalocele (synonym: hernia cerebri , cerebral hernia , external brain prolapse ; English : encephalocele ) describes an inhibitory malformation with a defective brain structure or median skull gap (at the root of the nose, forehead, skull base, back of the head), through which parts of the brain bulge outwards, without the involvement of the Cerebral liquor spaces (kenencephalocele) or with cerebral ventricular parts (also corresponds to encephalocystocele), often including a meningeal sac (encephalocystomeningocele). The word is made up of ancient Greek ἐγκέφαλος enképhalos , German 'brain' and the ending -zele .

Diagnosis

The diagnosis includes the three-dimensional representation of the bony defect using computed tomography (CT) and the cell contents using magnetic resonance imaging .

therapy

The therapy consists in the closure of the meninges, in the case of small encephaloceles by ablation at the base and closure by means of fascial plasty , in the case of large cells repositioning including plastic covering of the skull defect. The repositioning of functional nerve tissue protruding through large gaps rarely succeeds; the malformed tissue is usually removed.

The operation is only urgent if there is a CSF fistula due to the risk of infection or a combined disruption of the CSF circulation ; neurological deficits are not improved surgically.

See also

• Meningocele
• Frontonasal Dysplasia
• Craniotelencephalic dysplasia

literature

• Michael Bömmer: Magnetic resonance tomography diagnostics for cranial and spinal malformations in childhood Inaugural dissertation Ruhr University Bochum

Web links

Wikibooks: Pediatrics Neurology: Encephalocele  - Learning and Teaching Materials

• Encephaloceles Information Page The National Institute of Neurological Disorders and Stroke (NINDS)